Tyldesley_s Oral Medicine 5th Ed - Field

Au th or s: Field, An ne; Lon gman , Lesley Title:

Tyldesley 's O r al Medicine, 5 th E ditio n

Copyri gh t Β©2003 Oxfo rd Un ive rsity P ress > Fro nt of Bo ok > A utho rs

Authors

An n e Field

Sen io r Lectu re r & Ho no rary Co ns u ltan t i n O ral Medi cin e

Depa rtmen t o f Cl in ica l D enta l S cien ce s, Liverpo o l Un ivers ity D enta l Hos pital , P emb roke P la ce, Li verpo ol , U K

Lesley Lo n gman

Con su ltan t & Ho no rary S en ior Le cture r in Resto rative Den tistry

Depa rtmen t o f Clin ic al D en ta l S ci enc es, Liverp oo l Uni versity D en ta l Ho sp ital, Pe mbro ke Pl ac e, Li verpo ol , U K

Secondary Author William R. T yldes ley

Fo rmer Dean o f Den ta l S tu die s Uni versi ty o f Liv erpo ol, UK in co lla bora tion w ith

Specialist advisers Dav id Casso n

Con su ltan t in pae diatric ga stroe ntero lo gy

Li verpo ol Chi ldren s' Hos pital , Live rpoo l, UK Jo hn Coo per

Con su ltan t in ora l a nd m axil lo fac ial su rgery Uni versi ty Ho s pital Ain tree, Liverpo o l, UK Patrick Chu

Con su ltan t ha emato lo gis t

Ro yal Liverpo o l an d B ro adgre en Un iversi ty Hos pital Trus t, Live rp oo l, UK Mark Davies

Con su ltan t an aes th etis t

Li verpo ol Un iversi ty Den tal Hos pital an d Ro yal Li verpo ol an d B roa dgreen Un ivers ity Ho spi ta l Tru st, Liverpo o l, UK Lu ke Daw son

Clin ica l l ectu re r in ora l s urgery

Li verpo ol Un iversi ty Den tal Hos pital , Live rpo ol, UK Jo hn F ield

Pro fess or of m ol ecu la r on co lo gy Th e U ni versi ty o f Live rpoo l, UK

Jo hn Hambu rger

Sen io r lec tu rer an d h on ora ry co n su ltan t in ora l medici ne Th e Un iversi ty of Birm ing ha m Sc h oo l o f D enti stry, UK Eileen M ann in g

Con su ltan t in cli ni cal ch emi stry

Ro yal Liverpo o l an d Bro adg reen Un iversi ty Hos pital Trus t, Live rpoo l, UK Michael M ar tin

Sen io r lec tu rer an d h on ora ry co n su ltan t in ora l mi cro bio lo gy Li verpo ol Un iversi ty Den tal Hos pital , Live rpoo l, UK Sh ev au n M en dels oh n

Con su ltan t derma to lo gis t

Cou ntes s o f Ches ter Ho spi ta l, Che ster, UK Paul Nixo n

Con su ltan t in ora l a nd m axil lo fac ial radi olo gy

Li verpo ol Un iversi ty Den tal Hos pital , Li verpo ol , U K Simo n Ro gers

Con su ltan t in ora l a nd m axil lo fac ial su rgery a nd hono rary reade r Uni versi ty Ho s pital Ain tree, Li verpo ol , U K Jan e S etter field

Sen io r lec tu rer/h on o rary c on su ltan t in derma to lo gy Ki ngs Co lleg e, Lo n don , UK Eileen T heil

Clin ica l l ectu re r in perio do n to lo gy

Li verpo ol Un iversi ty Den tal Hos pital , Live rpoo l, UK Emm a V arga

Ass oc ia te spe cia lis t in ora l s urgery an d le cturer in ora l medi cin e Li verpo ol Un iversi ty Den tal Hos pital , Li verpo ol , U K Ju lia Wo olgar

Sen io r lec tu rer a nd ho n orary co ns ul ta nt i n o ral path ol ogy Li verpo ol Un iversi ty Den tal Hos pital , Li verpo ol , U K Jo ann a Zakr zewska

Sen io r lec tu rer a nd ho n orary co ns ul ta nt

Barts an d the Lon do n, Qu een Ma ry's Sc ho ol o f Med icine an d D entis try, Lo ndo n , UK

Au th o rs : Title:

Field, An n e; Lo n gm an , Lesley

Ty ldesley ' s O r al M edic in e, 5 th E ditio n

Co py rig ht Β © 2003 Ox fo rd U ni ve rsi ty P re ss > F ro nt o f B oo k > Pr e fa ce to F ift h E d it ion

Preface to Fifth Edition Th e 5 t h E di ti o n o f Tyld es l ey's O ra l Me di ci n e h as b ee n th or o ug h ly u pd ate d i n li n e wi th c ur ren t

te a ch in g p rac tic e s. It c o n tin u es to se rve a s a co m pr eh en s iv e te xtb oo k o f or al m edi c in e, n o t

on l y f o r u n de rgra d ua te de n tal s tud en ts , bu t a l so f or c o nti n u in g me di ca l an d de n tal e du c ati on . Th e em ph a s is th ro u gh o u t th e bo o k i s o n c lin i ca l pres en tati o n, in ve sti ga tio n a n d ma n a ge me nt of o ro fa c ia l d is ea s es . A n ew au th o r, Les le y Lon g ma n, d em o ns tra tes th e in va lu a b le

co n trib u tio n m ad e by sp ec i al is ts in o th er di sc ip li nes to mu l tid is ci pl in a ry c li n ic s, in w h ic h

co m pl em en ta ry s ki ll s an d ex pe rie n ce c o mb in e to im prov e th e q ua l ity of p ati en t c ar e i n o ra l me dic i ne .

Sp ec ia li st ad vi so rs , e ac h w ith th e ir o w n a re a of e xpe rtis e, ha ve m ad e a si gn i fic a n t

co n trib u tio n to th e bo o k an d e ns u re d th a t re ce n t adva n ce s in cl in i ca l pra c tic e h a ve b een in co rp o ra ted in to th e text.

Hig h -qu a lity , co l o ur il lu s trati o ns a re in c lu d ed to a dd in te res t a n d de mo n s tra te s ig n ifi ca n t

po in ts ; n o a ttem pt ha s b een ma de to i ll us tra te a l l th e le si o n s an d c on d iti on s d es c rib ed in th e

te xt, th es e w il l b e fo u nd i n th e co m pr eh en s iv e r an ge o f o ra l m ed ic in e c o lo u r a tla s es c u rren tly ava i la bl e.

Th is ed iti o n h as u n d erg on e m ajo r rev is io n i n term s o f l ay o ut an d c o nte n t. Th e ch a pte rs o n

fac i al pa i n, p s yc ho g en i c or o fac i al pro b le ms , s al iv ary gl a nd d is o rde rs a nd o ra l ca rc in o m a ha ve bee n s ig n ifi ca n tly ex ten de d. A s e cti o n o n ren a l di se as e h as a ls o b ee n ad de d, to g eth e r w ith a ch a pte r o n m ed ic al e me rge n ci es i n de n tis try. Ca s e stu di es , d em o ns tra tin g o ral me di ci n e

co n di tio n s , h av e b ee n in tro d uc e d a t th e be gi n ni n g of e ac h re le va nt ch a pte r, w ith a d is c u ss io n of th ei r d iff ere n tia l d ia gn o s is a n d ma n ag em en t o ption s a t th e en d o f th e c ha p te r. Th e se a re

in cl u de d to pro m o te s el f-dir ec ted an d a cti ve le ar nin g bu t w e h op e th at th e y w i ll al so pro m o te dis c u ss io n a n d de ba te. A n u m be r o f pro je cts h a ve a ls o be en ad de d a t th e en d o f ch a pte rs wi th th e p u rpo s e o f e n co u ra gi n g s tu de n ts to co n s u lt o th er ref ere n ce s o u rce s an d β € on lin e β€™ in fo rm ati o n .

Tyld es l ey' s Ora l Me di ci n e ha s be co m e fir ml y e sta bl is h ed a s th e te xtbo o k o f c h o ic e fo r

su c ce s si ve ge n era tio n s o f d en ta l s tu de n ts an d pr ac titi on e rs a nd w e h o pe th at th i s co m pl ete ly revi s ed ed iti on wi ll a cc u ra tely re fle c t cu rr en t c lin ic al pr ac tic e an d id ea s in the ra pi dly ch a n gin g d is ci pl in e o f o ra l me di ci n e.

Au th o rs : Title:



Co py rig ht Β © 2003 Ox fo rd U ni ve rsi ty P re ss > F ro nt o f B oo k > Dis c laim e r

Disclaimer Oxf o rd Un iv ers i ty P res s m ak es n o rep re se n ta tio n , e xp res s o r i mp li ed, th a t th e dru g d os a ge s in th is b o o k a re co r rec t. Rea d ers m u st th e ref o re alwa ys c h ec k the p ro du c t in f or ma tio n a n d

cl in ic a l p ro c ed ur es w ith th e mo s t u p to d ate pu b li sh e d p ro du c t i nf o rma tio n a n d da ta s he ets

pro vi de d by the m an u f ac tu rers a n d the m o st rec en t co de s o f c o n du c t an d s af ety reg u la tio n s .

Th e a u th o rs an d th e pu bl is h ers d o n o t a cc e pt res p ons ib il ity o r le ga l li ab il ity fo r a n y e rro rs i n th e tex t o r f o r th e mi su s e o r m is a ppl ic a tio n o f ma teri al i n th is w o rk.

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Co py rig ht Β © 2003 Ox fo rd U ni ve rsi ty P re ss > F ro nt o f B oo k > De d ic at ion

Dedication An n e Fi el d wo u l d l ik e to d ed ic a te th i s bo o k to th e m em o ry o f h e r la te fa th er, Jo h n E l ton Co stl ey, an d Les le y Lon g ma n to th e m em o ry o f h e r late mo th e r, Pa tri ci a M cLo u gh l in .

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Co py rig ht Β © 2003 Ox fo rd U ni ve rsi ty P re ss > F ro nt o f B oo k > A ck n ow le d g e m e nt s

Acknowledgements Th e a u th o rs are gra tef u l f o r th e su p po rt a n d en c o urag em en t o f th ei r fa m ili es d u rin g th e pre pa ra ti o n o f th i s co m pl ete ly rev is ed ed iti o n o f Tyld es l ey's o ral m ed ic in e .

Ou r s p ec ia li st ad vis e rs h av e m a de a n in va lu a b le co ntri bu tio n to th e b oo k a nd w e a re i n de bte d to the m fo r th e ir pa tie n ce , kn o w le dg e, a n d en c ou ra gem en t d u rin g th e p rep a rati o n o f th is n e w edi tio n . Th a n ks a re al so d u e to co l le ag u es w h o h av e co n trib u ted il lu s tra ti ve ma ter ia ls a n d to ou r en th u si as tic ju n i or s ta ff w h o h av e rea d dra ft ch a pte rs o f th e b o o k a n d ma de h el pf u l su g ges tio n s f or im pr ov em en t.

Fi na l ly, w e w o u ld li ke to th an k M rs Jan Vi ca ry fo r he r e xc el le nt typi n g an d w o rd -pro c es si n g ski ll s du ri n g th e ma n y mo n th s w e h a ve be en rew ri ti ng th e fi fth ed iti o n o f th e bo o k.

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Co py rig ht Β © 2003 Ox fo rd U ni ve rsi ty P re ss > F ro nt o f B oo k > L is t o f a b b re v ia tio ns

List of abbreviations Abbreviations 5-HT

5-hy dro x ytryp tam in e (a ls o kn o w n a s se ro to n in )

ACE

an g io ten s io n -co n ve rtin g en z ym e

ACT H

ad ren o c o rtic o tr o ph ic h o rm o n e

AID S

ac qu i red im m u ne d efi c ien c y sy n dro m e

AN A

an tin u c le ar an ti bo dy

AN UG

ac u te n ec ro tiz in g u lc e rati ve gi n giv iti s

AS A

Am eri c an S o c ie ty o f A ne s th e si o lo g is ts

AZT

az id o th ymi di n e

BM S

bu rn in g m ou th sy n dro m e

BN F

Bri tis h Na tio n a l F o rm u la ry

BP

bu ll o us pe mp h ig o id

CAP D

co n tin u o u s am bu l ato ry pe rito n e al di al ys is

CBT

co g ni tive b eh a vi ou ra l th era py

CD

Cro h n 's di se a se

CMC

ch ro n ic m u co c u tan e o u s ca n di do s is

CO

ce n tr ic o cc lu s io n

CR

ce n tr ic re la tio n CRF

ch ro n ic re n al fa il ur e

CRP

C-rea cti ve pro te in

CRS T o r C RE ST

C, ca l ci fic a tio n ; R, Ray n au d 's ph e no m en o n ; E, o e so ph ag ea l dy sf un c tio n ; S, sc l ero d ac tyl y; T , te la n gi ec tas i a ( sy n dro m e)

CT

co m pu ter iz ed to mo g rap h y

DLE

dis c o id lu p u s eryth e ma to su s

DM

dia b etes me ll itu s

DPT

den ta l pa n o ram ic to m o gra ph

EB

epi de rm ol ys is bu l lo s a

EB V

Ep s te in β €“Ba rr viru s

ED TA

eth yle n ed ia mi n e te tra ac eti c ac id

EM

eryth e ma mu l tifo rm e

EM G

ele c tr om yo g ra ph y

EN T

ea r, n o se , an d th ro at

ES R

eryth ro c yte s ed im en tati o n ra te

FAP A

(pe rio d ic ) fe ver , ap h tho u s u lc e rs, p h ar yn gi tis , an d c erv ic al a de n itis (s yn dro m e)

FB C

fu ll bl o o d c o u n t

FI SH

flu o re sc en t in s itu h ybr id iz ati o n

FN A

fin e n ee dl e as p ira te (bi o ps y)

FT A ( abs )

flu o re sc en t Trep o n em a an ti bo d y a bs o rb ed (tes t)

GF T

glo m er ul ar fi ltra tio n ra te

GO R D

ga stro - oe s op h ag ea l ref lu x di se as e

GP

gen e ra l pra c titio n e r

Ξ³ GT

Ξ³ glu ta ryl tra n sf era se

GT N

gly ce ryl trin i tr ate

GU M

gen i to ur in ar y m ed ic in e

HAD

Ho sp ita l A nx ie ty a n d Dep res si o n (s ca l e)

Hb

ha em o gl o bi n

HH V-8

he rpe s vi ru s 8

HI V

hu m an im mu n o de fi ci en c y v iru s

HLA

hu m an le uk o cy te an ti ge n

HP V

hu m an pa pi ll om a vir us e s

HS V

he rpe s s im pl ex vi ru s

HU

he rpe tif or m ul ce ra tio n

IB D

in fl am ma to ry bo w el d is ea s e

IB S

irri tab le bo w e l s yn d ro me

IM F

im mu n o flu o re s ce n ce

IN R

in tern a tio n a l n o rm al iz e d ra tio

LDE

Lic he n o id dr ug e ru pti on

LE

lu pu s ery th em ato s u s

MAG I C

mo u th a nd g en i ta l u lc ers w i th in fl am ed c a rtila g e (syn dro m e)

MALT

mu c o sa -as so c ia te d l ym ph o id tis s u e

MCV

me an co r pu s cu la r vo lu m e

MiR AS

mi no r rec u rre nt ap h tho u s sto m a ti tis

MjR AS

ma jo r re cu rr en t a ph th o u s sto m ati tis

MM P

mu c o us m em b ran e pe mp h ig o id

MR I

ma gn e ti c re so n a nc e im a gin g

MR S

Me lke rs so n β €“Ro se n tha l sy n dro m e

MR TA

ma gn e ti c re so n a nc e to mo g rap h ic an g io g rap h y

MS

mu lti pl e s c le ro si s

NR L

na tu ra l ru b be r la tex

NS AI D

no n s tero i da l a n ti- in fl am ma to ry dru g

NS U

no n -sp ec if ic u reth ri tis

O FG

or of ac ia l gra n u lo m ato s is

O SC C

or al s qu a mo u s ce ll c ar ci n om a

O SF

or al s ub m uc o u s fi bro s is

O TC

ov er the co u n ter

PAS

per io di c ac i dβ €“Sc h if f (re a ge nt)

PCR

po ly me ras e ch a in rea c ti o n

PP K

pa lm o pla n ta r ke ra to d erm a

PT A

po ly my xin E , to bra m yci n , a nd a mp h o teri ci n (lo z e n ges)

PT H

pa rath o rm o n e

RAS

rec u rren t a p hth o u s sto m a ti tis

RN P

rib on u c le o pro te in

RT -PCR

reve rs e tr an s cr ipta s e po l ym era se c h ai n re ac tio n

SC C

sq u am o u s ce ll ca rc in o m a

SLE

sys te mi c lu pu s e ryth em a tos u s

SN P

si ng le n u cl eo tid e po ly mo rp h is m

SP F

su n p ro tec tio n fa cto r

SS

SjΓ ¶g re n' s sy nd ro m e

SS R I

se le cti ve se ro to n in re u pta ke in h ib ito r

SV 4 0

si mi an v iru s 40

TM J

te mp o ro m an d ibu l ar jo in t

TM PD S

te mp o ro m an d ibu l ar pa in dys fu n c tio n s yn dr o me

TN F Ξ ±

ti ss u e n ec ro s is fa c to r al ph a

TP HA

Trep o n em a pa ll id u m h ae ma g lu ttin a ti o n a ss a y

TP I

Trep o n em a pa ll id u m im mo b il iz a ti o n (te st)

TP MT

th i o pu rin e me th yltra n s fer as e

TS G

tu m o u r s u pp res s or ge n e

UC

ul ce ra tive c o li ti s

US G- FN A

ul tras o u n d- gu id ed fi n e n e ed le a sp ira tio n

VD RL

Ve n ere al Dis ea s e Refe re nc e La bo ra to ry (te st)

VV G

vu lvo v ag in a l g in g iva l (s yn dro m e)

VZV

var ic el la z o ste r v iru s

WCC

wh i te c el l co u n t

TABLE OF CONTENTS

Β†‘

[+] 1 - The oral mucosa [+] 2 - Principles of oral medicine: assessment and investigation of patients [+] 3 - Therapy [+] 4 - Infections of the gingivae and oral mucosa [+] 5 - Oral ulceration [+] 6 - Diseases of the lips and tongue and disturbances of taste and halitosis [+] 7 - Swellings of the face and neck [+] 8 - Salivary glands and saliva [+] 9 - Inflammatory overgrowths, developmental and benign lesions, and pigmentation of the oral mucosa [+] 10 - Precancerous lesions and conditions. Oral carcinoma and carcinogenesis [+] 11 - Mucocutaneous disease and connective tissue disorders [+] 12 - Gastrointestinal disease [+] 13 - Blood and nutrition, endocrine disturbances, and renal disease [+] 14 - Immunodeficiency, hypersensitivity, autoimmunity, and oral reactions to drug therapy [+] 15 - Facial pain and neurological disturbances [+] 16 - Temporomandibular disorders [+] 17 - Psychogenic orofacial problems [+] 18 - Disorders of the teeth and bone [+] 19 - Medical emergencies in dentistry BACK OF BOOK

[-] Resources

[-] Appendix

-

-

Chlortetracycline mouthwash

-

Triamcinolone (plain) mouthwash

-

Triamcinolone with 2 per cent chlortetracycline mouthwash

Suggestions for further reading and reference sources

Β†‘

Au th o rs : Title:



Co py rig ht Β © 2003 Ox fo rd U ni ve rsi ty P re ss > T ab le o f Co nt e nt s > 1 - Th e o ra l m uc os a

1

The oral mucosa Normal oral mucosa Structure

In its ba s ic s tru ctu re th e or al m uc o u s me mb ra n e r esem bl es o th er li ni n g mu c o us m em b ran e s, fo r e xa mp le , tho s e o f th e va gi n a or th e o e so p h ag u s. W ith in th e m o uth , h o w ev er, th e re i s a wi de r ra n ge o f ep ith el ia l thi c kn es s th an th a t s ee n a t o the r m u co s a l s ite s . Th es e va ria tio n s dep en d l arg el y o n d if fer en c es in the d eg ree o f keratin i z ati on sh o w n b y th e mu c o s ae in

dif fe ren t a rea s o f the m o u th . S o m e o f th e rea cti o n s of th e or al mu c o u s me mb ra n e re s em bl e th o s e o f th e sk in , p res u ma bl y b ec a u se o f its po s itio n in th e tra n si tio n a rea b etw ee n th e

ga stro i n te s ti n al tra c t an d th e sk in . A s a res u lt of th is , d is ea se s o f b o th mu c o u s me mb ra n es an d sk in m a y p ro du c e le si o ns in th e m ou th . Th e o ra l m u c o sa , h o we ve r, c h ara c teri sti ca ll y

beh a ve s as a m u co u s me mb ra n e. Its be h av io u r i n d is ea se p ro ce s se s pe rh ap s mo s t c lo s el y res em bl es th a t o f th e va gi n al m u co s a.

Th e e pi th eli u m co v eri ng th e o ral m u co s a s ho w s a w ider va ria tio n i n th ic kn es s a nd i n its pa tte rn o f ke rati n iz ati o n th an m u c ou s m em br an e s at o th e r si tes .

Th e o ra l mu c o u s me mb ra ne c o n si sts b o th an a to mi ca l ly an d fu n c tio n al ly o f tw o l ay ers : o n e

(the c o riu m o r l a mi na p ro pr ia ) es s en tia ll y o f m es o de rma l o rig in a n d on e e pith e li al (Fi g. 1. 1). Wh en c o n si de rin g va ria tio n s o f stru c tu re, th e be h avio u r o f th e co r iu m mu s t b e ta ke n in to ac co u n t e ve n th ou g h th e ma jo r c h an g es m ay a pp ea r to b e w ith in th e ep ith e lia l la ye r.

Fig. 1 .1 A s ec tio n o f no r ma l mu c o us me mb ra n e f ro m the h a rd pa la te wi th a ke rati n iz ed

su rfa c e la yer o f ep ith el iu m o ve rly in g the c o riu m .

The oral epithelium

Th e k era tin o c ytes a re th e ma in c e ll co m po n e nt o f the o ra l ep ith el iu m b ut o th e r c el l ty pe s in cl u de m el an o c ytes , La n ge rh an s c el ls , an d M erk el cel ls .

THE KERATINOCYTES In no rm a l m u c o us m em b ran e th e in teg rity o f th e ep ith el iu m is m a in ta in ed b y th e di vis i on o f

kera tin o c yte s in th e ba sa l la ye r. A s ea c h ce ll d ivide s o ne re su l tin g ce ll re ma in s e ffe cti ve ly in si tu , wh i le o n e m ig ra tes to w ar ds th e su rf ac e, u n d erg o ing va rio u s str uc tu ra l m o di fic a tio n s a s

it p as s es th ro u gh th e ep ith e liu m (Fi g. 1. 2 ). Th es e mo d ifi c ati on s , w h ic h a re de pe nd en t o n th e pro c es s o f k era tin i za tio n , v ary a cc o rdi n g to the p rec is e s ite o f th e mu c o sa in vo l ve d a n d

res u lt i n th e p ro du c tio n o f a s ur fac e la ye r o f ce lls th at a re ei the r f u lly , pa rtia l ly, o r n on -

kera tin i z ed an d w h ic h a re sh e d i n to th e o ra l ca vi ty a t a ra te de pe n de nt on the ra te o f m ito s is in th e ba sa l la ye r. F o r ea c h di vid in g c el l, o n e ce ll is l o st fro m th e s u rfa c e a n d, th u s , th e

in teg rity an d di me n si o n s o f th e ep ith el ia l l a yer are m a in tai n ed . Th e rate o f tur no v er of th es e ce lls , th a t is , th e β€ tr an s it ti me β€™, f o r a ke ra ti n o cy te i n th e b a sa l laye r to rea c h th e

su rfa c e in v ari o us h u m an ep ith el ia h a s be en d eter min ed by a n u mb er o f tec h n iq ue s . Th e r ate in h u ma n s ki n is g en e ral ly qu o ted a s be in g o f th e ord er o f 50 to 70 d a ys a nd th a t in th e

gin g iv al ep ith e liu m m u ch th e s am e, bu t th a t in th e bu c c al ep ith e liu m i s mu c h fa s te rβ€” of th e or der o f 25 da ys .

Th e e pi th eli u m o f th e o ral m u co s a sh o w s w id e va ria tio n s in th e ex ten t o f th e k era tin i za tio n pro c es s. In the f ul ly ke rati n iz ed s itu a tio n , th e r ath er cu b ic a l c el ls f or me d by mi tos i s at o r ne ar th e b a sa l la ye r m ig rate to w ard s th e su rf ac e , be co m in g m or e p o lyh e dra l an d s h ari n g

in terc el lu l ar atta ch m en ts , w h ic h h av e g iv en th e n ame β€ pri ck le ce ll la ye r β€™ (o r s tratu m

sp in o s um ) to th i s z on e . In th e l ig h t mi c ro sc o pe th es e in ter ce ll ul a r β€ pri ck les β €™ ap pe ar as si ng le atta c hm e nts o f th e c e ll wa l ls , bu t b y e le ctron mi cro s c o py the s e i n terc el lu la r ju n cti on s (ref erre d to as d es m os o m es ) a re se en to be o f mu c h gre a ter c o m pl exi ty. It is p ro ba b le tha t th e d es mo s o me s ac t i n a m ec h an ic a l ma n ne r to giv e stre n gth to th e e pi th el iu m. In se ve ral

dis e as es m a rke d b y e pi th el ia l f rag il ity the de sm o s ome a tta c h me nts a re lo s t o r i m pa ire d. It sh o u ld p erh a ps b e a d de d th a t si mi la r, o n e -si de d s tru c tur es , h em id es mo s o me s , atta ch th e

P. 4

pla s ma m em br an e o f th e ba s al ke rati n oc yte s to th e la mi na l u ci da o f th e b as e me n t me mb ra n e co m pl ex. A s th e ce ll s o f th e s tr atu m s pin o s u m mi grate to th e s u rfa c e th e y b eg in to fla tten

an d gra n u la r s tru c tu re s (ke ra toh y al in g ra nu l es ) a p pea r wi th in th em . Th e se g ran u l es gi ve th e

ch a rac te ris tic a pp ea ra nc e to th e β€ stra tu m gra n u lo s um β€™ in ke rati n iz ed e pi th e li a. F ina ll y, at o r n ea r th e su rf ac e, th e e pi th e li al c el ls lo s e th e ir de tai le d i n ne r s tru c tur e, th e n uc le i

deg en e ra te , th e kera to h ya li n gra n u le s fra gm en t a n d di sa p pea r, a n d the i n so lu b le p ro te in

co m pl exe s me n tio n ed ab o ve fi ll th e c e ll, n o w f u lly ke ra tin iz e d ( Fi g. 1. 2). A t th is s tag e th e

des m o so m es h a ve ef fec tiv el y d eg en e rate d al so a n d the f la tten ed ce ll s (β € sq u am es β €™) ar e

eve n tua ll y lo s t in to th e o ral c av ity. A s h a s be en po i nte d o ut, e a ch k era tin i ze d ce ll lo s t i n th is wa y mu s t be m a tc h ed b y a di vid in g c el l i n th e pro life rati n g c o m pa rtme n t o f th e e pith e li um i n or der fo r sta b ili ty to b e ma in ta in e d. Th i s pro c es s o f ren ew a l ap pl ie s on l y to fu ll y ke rati n iz ed epi th el iu mβ €”as s ee n , fo r i n sta n ce , in th e m u co u s mem bra n e ov erl yi ng th e h a rd

pa la teβ€” an d is u s u al ly ref erre d to as o rth o ke ra tiniz a tio n . In o th er ar ea s (a s in s o me p arts o f th e b uc c al m u co s a an d th e flo o r o f th e mo u th ), th is pro c es s o f ker ati ni z ati o n do e s no t ta ke

pla c e, k era to h ya lin gra n u les a re n o t fo rm ed , an d n uc lei a n d o r ga n ell es (a l th o u g h so m ew h a t eff ete) c an b e se en i n th e su rf ac e la ye rs ( Fi g. 1. 3). In an in term e dia te fo rm (pa ra ke rato ti c epi th el iu m) , n uc le i ma y sti ll be s ee n in th e s u rfa ce la ye rs a n d ke rato h ya li n is s p ars e o r

ab se n t. Fo r the p u rpo s e of u n de rs ta n di n g th e c li n ical si gn i fic a n ce o f th e se d if fere n ce s in

kera tin i z ati on , th e y s h o u ld be re ga rde d as be in g part o f a s pe ctru m ra n gi ng f ro m c o m pl ete no n -kera tin i z ati on at o n e ex trem e, th ro u gh va ryin g d eg ree s o f p ara ke ra tin iz a tio n , to fu l l or th o k era tin iz a tio n a t th e o th e r.

Fig. 1 .2 Dia gr am o f a ker ati ni z in g sq u am o u s ep ith el iu m . Co mpa re w ith Fi g. 1. 1.

Fig. 1 .3 A s ec tio n o f no r ma l bu c ca l mu c o s a. C om p are w ith the ke ra ti n iz e d p al a ta l

mu c o sa s h o wn in Fi g. 1. 1 .

Th e d is trib u tio n o f the s e d if fer in g ep ith el ia i n the n o rm al o ra l mu c o sa h as a c lo s e

rel ati on s h ip w ith th e fu n c tio n o f th e tis s u es a t the s i te . In n o rm al mu c o s a, n o n -kera tin i z ed o r pa rak era tin i ze d ep ith el iu m is s e en o n th e bu c ca l muc o s a, th e fl oo r o f th e mo u th , a n d th e

ven tra l su rf ac e o f th e to ng u e, w h er ea s o rth o kera tini z ed ep ith el iu m is s e en o n th e h ar d p al a te an d pa rts o f th e gi n gi vae . Th e do rs a l su rfa c e of the ton g u e i s a ls o o rth o kera tin i z ed , bu t dif fe rs fro m th e o th e r o ra l mu c o sa l su rf ac es i n th at the re a re a n u m be r o f sp ec ia li z ed

stru c tu res p res en t, p red o mi n an tly th e pa pi ll ae . Th ere a re fo u r ty pes o f li n gu a l pa pi ll ae :

an ter io rly th e fil ifo r m a n d fu n gi fo rm ; p o ste rio rl y f ol ia te an d va ll ate pa pi ll ae . Th e fi lif o rm an d fu n gif o rm pa pi ll ae a re o f c li n ic al s ig ni fi ca n ce i n th at th e ir atro p h y i s of ten a n e arl y s i gn o f mu c o sa l ab n or ma li ty.

Th ere a re fo u r typ es o f li ng u al p ap il la e: an te rio rly th e fi lif o rm an d fu n gi fo rm ; p o ste rio rl y fo li ate a nd v al la te p a pil la e.

P. 5

Th ere is c u rre n tl y a g re at d ea l o f re s ea rc h co n c erni n g g en e tic ab n o rma l itie s o f k era tin iz a tio n , pa rtic u la rly di stu rb a nc e s in th e ge n es c o din g fo r sp ec if ic ke rati n sth a t a re res po n s ib le fo r dis e as es , s u ch a s e pi der mo l ys is bu l lo s a ( Ch ap ter 11). In ad di tio n , c el l a d he s io n a n d th e

mo le c ul es a s so c ia ted w ith i t (f or ex am pl e, c a dh e rins a nd i n te gr in s ) a re of g rea t im po rta n ce i n dis o rd ers o f the s ki n a nd m u co u s m em bra n es . In th e pe mp h ig us g ro u p o f i mm u n o bu ll o u s dis e as es , th ere i s fa ul ty a d he s io n b etw ee n ke rati n ocy te s du e to th e d ev el op m en t o f

au to an ti bo d ies a g ai ns t d es m o gle in I an d III, w h ic h ar e me mb er s of th e ca dh e rin fa mi ly of ad h es io n m o le cu le s ( Ch ap ter 11).

THE MELANOCYTES Th e m el a no c yte s ap pe ar in , o r very c lo s e to , th e bas al la ye r a n d o n el ec tro n mi c ro sc o py s ho w gra n u la r s tru ctu re s (me la n o so m es ) th at a re th e pre cu rs o rs o f me la n in β €”th e b la ck pi gm en t th a t m o di fie s th e c o lo u r o f b o th sk in a n d mu c o u s memb ra ne s . Th es e ce ll s, l ik e th e

Lan g he rh an s c el ls , a re de n dri tic w ith c yto p la sm ic pro lo n g ati on s e xten d in g be tw ee n the c el ls

of b as al an d s u pra ba s al a rea s o f th e ke ra tin o cy tes . M el an o c ytes s yn th es iz e b ut do n o t reta in

me la n in ; i t is tra n s ferr ed by th e de nd riti c pro c es ses to a dj ac en t ke rati n oc yte s . Th e m e la n oti c

pig m en tati o n o f o ra l mu c o sa , l ike th a t o f th e s kin , sh o w s g rea t ra ci a l v ari ati o n . Ho w eve r, th is do es n o t d ep en d o n va ri atio n of th e n u mb ers o f me lan o cy tes bu t on the n u m ber a nd a c tivi ty of th e me la n os o m es w ith i n th em . Th e ep ith el ia o f all r ac es c o n tai n a pp ro xim a te ly th e sa me nu m be r o f me la n o cy tes . It is th e ra te o f pr od u cti o n o f m el a ni n a n d i ts di stri bu tio n th a t ar e

dif fe ren t. It i s kn o wn th a t h o rm o na l in fl u en c es a re im po rta n t in th e sti mu l ati o n o f m el an o c yte ac tivi ty, a lth o u gh th e p rec is e me ch a n is ms r em ai n o bs c ur e. In s o me c irc u ms ta n ce s the

me la n oc yte s ma y be s tim ul ate d to pro d u ce ex ce ss m ela ni n b y a w id e ran g e of n o n -ho rm o n al stim u la e ( Ch ap ter 9).

Me la n o cy te s s yn th es iz e bu t d o n o t re ta i n me la n in . It i s tran s fe rred b y th e d en d riti c pro c es s es to ad jac e nt ker ati no c yte s.

LANGERHANS CELLS Th e La n ge rh a ns c e lls w e re firs t id en tif ie d i n 1860, a nd f o r a lo n g tim e we re s om eth i n g o f a

mys ter y. Th e re is a su b s ta n tia l n um be r o f th es e ce ll s pre se n t n ea r th e ba s al c o mp le x o f th e or al ep ith el iu m , w ith d en dr itic p ro c es se s ex ten di n g b etw e en th e ke rati n oc yte s an d w ith

rec o gn iz a bl e ul tra stru c tu ral fe a tu re s . Ov er the l ast 3 β€“ 4 de ca d es re se ar ch h as s h o w n tha t th e se c el ls h a ve an im mu n o lo g ic al fu n c tio n , a cti n g a s pe ri ph era l sc a ve ng in g c el ls o f th e

im mu n e sy ste m, r ath er li ke ma c ro ph a ge s bu t l ac ki n g th e ir ab il ity to ph a go c yto s e eff ec tive ly . It wo u l d s e em th at at l ea s t o n e f u n ctio n of th es e ce lls i s to a ct as a n tig en -pre se n tin g ce ll s

an d sti mu l ate th e ac tiv ati on o f T ly mp h oc yte s ag a ins t th em . Lan g erh a n s ce ll s are th e refo re co n s id ere d to be im mu n o lo g ic a lly c o mp ete nt de n dri tic c ell s , si mi la r to th o s e f o u nd i n th e per ip he ra l l ym ph a tic s ys tem . Th ei r o ri gi n ap pe a rs to be th e bo n e ma rro w a n d no t th e epi th el iu m.

Kera tin o c ytes a n d Lan g erh a n s ce ll s pla y an im po rta nt p art in th e im mu n o s ur vei ll an c e of th e

or al ep ith e liu m , a nd b o th se c rete a nd re s po n d to immu n o lo g ic al ly a cti ve cy to kin e s, i n cl u di ng th e in ter le uk in s a nd i n te rfe ro n s. Th e c o n ce pt o f l oc a li ze d mu c o sa l im mu n i ty i s cu rr en tly

un d er in ve sti ga tio n w ith reg ard to b o th its fu n c tion i n ma i nta in i n g th e i n teg rity o f n o rm al ti ss u e an d i ts r ol e in m u co s a l d is ea s e. T he p ath o gen es is o f l ic h en p la n u s is n o t ye t fu l ly

un d ers to o d, b u t pro b a bly in v o lve s a ce ll -me dia te d imm u n e r es po n s e to an e xte rn al a ge nt, i n

wh i ch La n ge rh a ns c e lls an d ke ra tin o cy tes re le as e cyto kin e s an d a dh es i on mo le c ul es to w h i ch T ly mp h oc yte s ca n b in d . Su b se qu e n t ac tiv ati o n o f c yto xi c ly mp h oc yte s is th o u gh t to be

res po n s ib le fo r d a ma ge to th e ba s al c el ls , w hi c h is a c h ara c teri sti c fea tu re o f th i s co n di tio n . Lan g erh a ns c e lls a re im m u no l o gic a ll y c o mp ete n t de n triti c ce ll s.

The corium

Th e c o ri um (l a mi na p ro pr ia ) o f the o ra l mu c o us m e mbran e is s ep a rate d f ro m th e s u bm u co s a l la yer , by a z on e o f gra du a l tran s iti on rath e r th an a c le a r b ou n d ary . In th e c o ri um a n d

su b mu c o sa l ie th e m in o r sa li va ry gla n ds an d s eb ac e ou s g la n ds o f th e o ra l ca vi ty. Th e se a re

wi de ly va ria bl e in di stri bu ti on , th e m uc o u s gl an d s be in g mo s t fr eq ue n t in the m u co s a o f th e

lip s an d p o ste rio r p a la te, w h il st th e s eb ac e ou s g lan ds a re mo s tly c on c en tra ted i n th e bu c c al mu c o sa w h ere th ey m ay a pp ea r a s ye llo w sp o ts , k no w n a s F o rdyc e 's sp o ts. Th e su b mu c o sa l ti ss u e co m po n e n ts a re a ls o w id el y v ari ab le : b lo o d ve ss el s, fa t, an d fi br ou s ti ss u e be in g pre se n t in d if fer in g pro p o rtio n s ac c o rdi n g to th e pre c is e s i te . Wi thi n th e co ri u m a n d

su b mu c o us ti ss u es a re s ca ttere d ce ll s of th e le u ko cy te s e rie s in v ary in g pro p o rtio n s a nd

co n c en tra tio n s. Du ri n g dis e as e pro c es s es th e se m ay a lter ra dic a ll y, b oth in n u mb er a nd i n ty pe , de pe n din g o n th e b a si c na tu re o f th e pa th o lo gi c al pr oc e ss i n vo lv ed.

Fo rd yc e's s p ots a re th e s e ba ce o u s gl an d s o f th e or al m uc o s a. T h ey are fre qu e ntl y m is ta ken fo r p ath o lo g ic a l l es io n s bu t are c o mp le tely n o rma l.

The basement membrane

Lyin g be tw ee n th e e pi the li u m an d c or iu m o f th e o ra l mu c o u s me mb ra n e i s a co m pl ex

mu lti la ye red s tru ctu re , the b as e me nt me mb ra n e. O n ultra str uc tu ra l s tu dy it is s ee n th at th e co m po n en ts o f the b as a l z o n e ar e m u ch fi ne r th a n s ug ge s te d by li gh t m ic ro s co p y a n d th at, rath e r th an a si ng le me mb ra n e, a n u mb er o f l a yers are vi si bl e, i n cl u din g th e la mi n a lu c ida

an d th e la mi n a de n sa . In th e ba se m en t m em bra n e, a n ch or in g fib res a tta ch th e la m in a de n sa

to the u n de rly in g tis su e a n d h e mi de sm o s o me s atta ch th e b as a l c el ls o f th e ep ith el iu m to th e la mi na l u ci da . B el o w th e l ev el o f th e h em id es mo s o m es is th e su b ba s al d en s e p la te, th ro u g h wh i ch a n c ho r in g fil am en ts c o n n ec t th e l a mi na lu c id a to th e l am in a d en s a. A u to a nti bo d ie s to co m po n en ts o f the l am in a l uc i da h av e b ee n id en ti fied in b u llo u s p em ph i go id , w h ic h is a n im mu n o bu l lo u s sk in di se a se ( Ch ap ter 11).

Function

Al tho u g h th e o ra l mu c o u s me mb ra n e h a s se ve ra l f un c tio n s , se n so r y a n d s e cre to ry a m o ng th e m, i ts ma in pu rp os e is pro b ab ly th at

of a cti n g a s a ba rri er. It pro te cts th e de ep er s truc tur es fr om me c ha n ic a l i n su l ts, s u c h as ma sti ca to ry trau m a an d a ls o p reve n ts the e n tr y o f mi c ro -or ga n is ms a n d so m e to x ic

su b sta n ce s. Th e o ra l mu c o s a h a s a n ex ten s ive se n so ry i nn e rva tio n th a t c an d is c rim in a te

to u c h a n d te mp era tu re. Tas te bu d s are al s o lo c ate d in o ra l ep ith el iu m . In co n s id eri n g th e pro tec tiv e fu n cti on of th e o ral m u co s a , it i s al so n ec es sa ry to d is cu s s o the r f ac to rs , in

pa rtic u la r th e ro le o f sa li va ( Ch ap ter 8). Th e o ral m u co s a is c o n sta n tly ba th ed b y s al iv a,

wh i ch n o t o n l y m ai n tai n s the p h ys io lo g ic al e nv iro n men t n ec es s ary fo r the m a in ten a n ce o f

epi th el ia l i n teg rity bu t a ls o i nc l ud es s ev era l pro te cti ve , an tib a cte ria l co m po n en ts . A n u mb er of th es e h av e b ee n de s cri be d, b u t pe rh ap s th e m o s t im po rta n t a re the s ec re to r y

im mu n o gl o bu li n s, p red o mi n an tl y o f th e Ig A c la ss , that are f ou n d in sa li va a n d th a t a tta c h to si te s o n th e ep ith el ia l su rf ac e.

In sp ite o f th i s ba rrie r f un c tio n o f th e o ral m u co sa , the re is a d eg ree o f pe rme ab il ity tha t,

ap art fro m its th eo re tic a l an d s ci en ti fic i n te re st, i s al so of c li ni c al s ig ni fi ca n ce . Du rin g lo c a l th e ra py w ith m o uth w a sh e s an d s im il ar pre pa ra tio n s, dru g s ma y b e tran s po rte d ac ro s s the

or al m uc o s a an d m ay ex ert e ff ec ts si mi la r to th o s e r es u ltin g fro m s ys tem ic th era py . Th is i s

P. 6

an i mp o rtan t fac to r w h e n co n s id eri n g th e u s e o f h igh- co n c en tra tio n s tero i d m o u thw a s he s fo r ul ce ra tive l es io n s o f th e o ra l mu c o s a. Th e p erm ea bili ty o f th e o ra l mu c o sa i s u tili z ed in th e tr ea tme n t o f an g in a b y g lyc e ryl trin i tr ate . In th ese c i rcu m s ta n c es ra pi d ab so rp tio n o f th e dru g th ro ug h o u t th e o ra l mu c o sa i s an o bvi o u s ad va nta g e. Th e o ra l mu c o sa h a s a n im po rta n t ba rri er fu n cti o n . Al tho u g h th e f u ll si gn i fic a n ce o f th e ro l e o f s al iva i n ma i nta in i n g th e h ea lth o f th e o ra l

mu c o sa i s, a s ye t, n o t fu ll y u n d ers to o d, th er e c a n be n o do u b t th at a f ree s al iv ary fl o w is a n es se n tia l p a rt o f th e o ra l e n vir on m en t. If th e f lo w i s dim in i sh e d, e ith er by d eg en era tiv e

ch a n ge s i n th e sa li va ry gla n ds o r by th e ac tio n o f dru g s, s o re n es s an d a tro ph ic c h an g es i n

so m e a re as o f th e o ra l mu c o s a r ap id ly fo ll o w. Th e to n g ue i s pe rh ap s mo s t m ar ked ly a ffe cte d in th is w a y. In s om e c on d iti on s (f o r e xa mp le , Sj Γ¶gre n' s sy nd ro m e β€”Ch ap ter 8 ), it i s

dif fic u lt to di sti n gu is h b etw ee n pr im ary m u co s al c han g es a n d th os e s ec o nd a ry to dim in i sh e d sa li va ry f lo w , bu t on a cl in ic a l b a si s it i s rea s o na b le to a cc ep t th a t atro p h ic c h an g es i n the

or al ep ith e liu m a re reg u la rly as s o ci ate d wi th dr yn es s o f th e m ou th . Th e m ai n im m un o l og ic a ll y ac tive c o mp o n en t o f s a li va is Ig A, w h i ch i s pre s en t i n a mu c h h ig h er rati o to o th er im mu n o gl o bin s , th a n in th e s eru m .

A fu rth er co m po n en t to be co n s id ere d as p ar t o f th e n o rma l h ea lth y o ral e nv iro n m en t i s th e mi cro b ia l flo ra o f th e mo u th . A w id e ran g e o f m ic ro-or ga n is ms m ay b e p res e nt in th e o ra l

ca vi ty , li vin g in a co m me n sa l rel ati o n sh i p w i th the h o s t. Wh en th i s rel ati on s h ip is u ps et by a ch a n ge in th e lo c a l o r ge n era li ze d co n d itio n s , th e c o m me n sa l mi c ro - or ga n is ms m ay b ec o me pa tho g en i c. O ra l c a n di do s is is a n e xa mp le o f th i s, al th o ug h th e pre ci se c h an g e in th e h o st

lea d in g to cl in i ca l in fe cti o n is n o t ea s y to i de n tify . Th e ae tio lo g ic al fa c to rs fo r s o m e c o m mo n or al in f ec tio n s ar e di sc u s se d in Ch ap ter 4.

A w id e ra n ge o f mi cr o - or ga n is ms m ay b e p res e nt in th e o ra l c a vity , exi s ti n g in a co m m en s al rel ati on s h ip w ith th e h o st.

Age changes

Ch an g es o c cu r in th e o ra l m u co s a o f h ea lth y in di vi du a ls w ith i n cre a si ng a ge . Re du c tio n s in

ov era ll e pi th e li al th ic kn es s , fle xi bi lity o f th e c olla g en f ib res , in n er va tio n , bl o od s u pp ly , an d

per me ab il ity o f th e mu c o sa h a ve be en de sc rib ed . It h a s ge ne ra ll y b ee n as s u me d th at c h a n ges of th is k in d o cc u r w ith c o m pa ra ti ve s u dd en n es s a t a la te a g e, b u t rec e nt evi de n ce i mp li es

th a t, a t le as t i n th e c a se o f the e pi the li u m o f th e to ng u e, a c o n tin u o u s tr en d to wa rd s atro p h y oc c u rs thr o ug h o ut ad u lt l if e. Th e cl in i ca l si gn i fica nc e o f th i s ob s erv ati on is th at su d de n

ch a n ge s i n th e str uc tu re o f th e o ral m u co s a , su c h as de pa pi ll ati on of th e ton g u e, s h o u ld

ne ve r be c o n si de red a s be in g du e to a ge a lo n e w i thou t f u ll in ve s ti ga tio n a n d th e el im in a tio n of o th er fa cto rs s u ch as h a em ato l o gic a l a n d n u tr itio n a l d is o rd ers (s ee Ch ap ter 13).

Ag e ch a n ge s ma y a l so af fe ct th e fu n c tio n o f sa li va ry gla n ds . It h a s be en s h o w n th at

gra du a ll y in c rea s in g de gre es o f atr op h y a n d fib ro s is a ffe ct the s ec re tor y u n its o f bo th th e

su b ma n di bu la r a n d th e l ab ia l sa li va ry gl an d s th ro u gh ou t lif e, e ven in th e ab s en c e o f di s ea se pro c es se s th at mi gh t b e a s so c ia ted w i th su c h ch a n ge.

Abnormal oral mucosa

Ma n y o ra l le si o n s rep res en t th e en d re su l t o f b re akdo w n o r a bn o rm al ity o f th e n o rma l stru c tu rin g o f th e ep ith el iu m . V ari ati o n in th e ra te o f ke rati n fo rm a ti o n , di sp ro po rti o n

betw e en th e di ffe ren t la yer s of th e ce ll s, bre ak do wn o f th e n o rm al in te rce ll u la r b o nd s o f th e pri ck le ce ll s, sp li ttin g o f th e ep ith el iu m fr om th e c on n e cti ve tis su e , an d m an y o th er s i mi la r ab n or ma li ties m a y o c c ur in d if fer en t d is ea s es . Fo r in s tan c e, i n a n u mb er o f mu c o s al

ab n or ma li ties h y pe rker ato s is o c cu rs ( Fi g. 1. 4). Th is m a y a ris e a s a r es u lt o f ab n o rma l

irri tati on of th e mu c o sa o r ap pa re ntl y sp o n tan e o us ly i n so m e co n d itio n s . In o th er l es io n s ,

atro p hy o f the e pi th e li um ma y o cc u r. Th i s rep res en ts a th in n in g o f the n o rm al e pith e li al

la yer , pe rh ap s to o n ly a few la yer s o f c el ls , o ften ac c om pa n ie d by in c o mp le te k era tin i za tio n (Fi g. 1. 5). S uc h

epi th el iu m is e as il y l o st fo ll o w in g a mi n or de gre e o f tra u ma a n d thu s a tro ph i c le si o ns o f th e mu c o sa rea d ily b ec o me u lc er ate d. M a ny o f th e β € so -ca ll ed β€™ ero s ive l es io n s a re of th is ty pe . It s ho u l d b e rem em be re d th a t u lc er ati on is in its el f a q ui te u n sp ec if ic pr o ce ss a n d im pli es o n ly th e lo s s o f e pi th e li u m f ro m the m u co s al su rf ac e fo l lo w ed by in f la mm a tor y

ch a n ge s i n e xpo s ed c o n n ec tive tis s u e. B u ll ae o r bl is ters o f th e m u c os a m ay o c cu r i n o n e o f

tw o w a ys , ei th er by de ge ne ra tio n o f th e c e lls a n d of th e in ter ce ll ul ar li n ks in th e p ric kl e c el l la yer o f th e ep ith el iu m (Fi g. 1. 6 ) o r b y s e pa ra ti o n o f th e w h o le o f th e ep ith el iu m fr om th e un d erl yin g c o riu m (Fi g. 1. 7 ). Fr eq ue n tly, th e re are a ls o c h an g es i n th e s u pp o rtin g tis s ue s an d , in s o me c as e s, th e v is ib le ep ith e lia l ch a n ge s ma y be s ec o nd a ry to ch a n ge s in th e

un d erl yin g co r iu m tha t a ff ec t th e nu tri tio n a n d m e ta b ol is m o f th e ep ith e liu m . Th e gr ea tes t

pra cti ca l s ign i fic a n ce o f thi s fa ct is , pe rh ap s , the n e ce ss ity , w he n ta kin g a bi o ps y o f le s io n s

of th e or al m uc o s a, to in cl u de a re pre se n tati ve thic kn es s o f co r iu m in th e tis s ue re mo v ed fo r mi cro s c op ic e xa mi n ati o n. In ma n y c a se s, a bi o ps y c on s is tin g la rge ly o f e pi th eli u m al o ne i s virtu a ll y u s el es s fo r di ag n o si s.

Fig. 1 .4 Hyp er kera to tic e pi the li u m i n a n al ve o la r m u co s al les io n .

P. 7

Fig. 1 .5 A tr o ph ic e pi the li u m fro m th e do rs u m o f th e to n gu e β€” th e n o rm al c om p lex stru c tu re i s en tir el y lo s t.

Fig. 1 .6 A n in tra -epi th el ia l b u ll a fo rm ed a s a res u lt o f los s o f i n trac e llu l ar co h e si o n in th e p ric kl e c el l la ye r.

Th e i n teg rity o f th e o ra l mu c o sa i s ma i nta in e d by a co m pl ex o f i n tera cti n g f ac to rs

su p eri mp o se d o n th e l o ca li z ed s tab il iz in g m ec h an is ms di sc u s se d a b o ve. T he g en e ral h o rm on a l sta tus o f th e pa tie nt an d a n u mb er o f n u triti o na l an d m eta bo l ic fa c tor s are in vo l ve d i n

ma in ta in in g th e ce ll me tab o li sm a n d the o rd ere d stru c tu re o f th e mu c o u s me mb ra n es . Th e

ro le o f i ro n me ta bo li sm i n th e m a in ten a n ce o f th e str uc tu re o f th e o ral m u co s a h as b ee n th e su b jec t o f m u c h in ve s ti ga tio n . It i s ce rta in ly th e ca s e th a t ir on de fic ie n cy , ev en w h en

rel ati vel y m il d in c li n ic al term s , ca n re su l t in gene ra liz e d or al ep ith e lia l a tr op h y a n d lo s s o f

th e pa pi ll ar y p atte rn o f th e li n gu a l m u c os a . It s eems th a t o the r d ef ic ie n ci es th a t mi gh t a ff ec t

iro n m eta bo l is m an d er yth ro cy te p ro du c tio n , s u ch a s fo l ate a n d vi ta m in B 1 2 de fi ci en c ies , m a y al so c o n trib u te to th i s de sta bi li za tio n o f th e o ra l e pi the li u m. T hi s w ill b e dis c u ss ed a t g rea ter len g th in Ch ap ter 13 . If a n y s in g le fa c to r is d is turbe d, th e n se qu e n ti al ch a n ge s o cc u r a n d cl in ic a lly s ig n ifi ca n t a bn o rm al iti es o f th e o ra l muc o sa m ay fo ll o w . It is o fte n di ff ic ul t to

dec i de w h ic h o f th e va rio u s p o ss ib le fa c tor s ar e i nvo lv ed in in iti ati ng th es e c ha n ge s β €”th e se

ma y e vi de ntl y o c c ur ei th er as a pri ma ry m an if es tatio n o f l o ca li z ed m uc o s al a bn o rm al ity o r a s

a s e co n d ary ef fe ct o f g en e ral iz e d d is ea s e pro c es s es. It is th e ro le o f or al p hy si ci a ns to a s se s s th e p os s ib le a etio l o gic a l fac to rs a ss o c ia ted w ith mu co s a l l es io n s o f th i s kin d an d to e n su re ap pro p ria te in ve sti ga tio n s a nd (i f n e ed s be ) m an a geme n t.

Fig. 1 .7 A s ub ep ith e lia l bu l la fo rm ed b y th e s e pa rati o n o f th e en ti re e pi th el ia l l ay er (in c lu di n g th e b as a l c el l la ye r) f ro m th e u n de rly ing co r iu m.

Th e r ea cti o ns o f th e o ra l mu c o sa a re n o t ex cl u si ve ly tho s e o f a m u co u s m em bra n e. As h a s

bee n po i nte d o ut, a n u m be r o f di se as es o f th e sk in a ls o fi n d exp res s io n i n o ral le si o n s. T hi s is n o t en ti rel y s u rpri s in g o n an a to mi ca l gro u n d s sinc e th e l a rge r pa rt o f th e o ra l mu c o s a i s

der ive d fro m a n em bry o ni c in v ag in a tio n th at ca rri es in w a rds s o me o f the p rec u rs o r ep ith e lia l ce lls f ro m w hi ch bo th fa c ia l sk in a n d o ral m u co s a are d ev el o pe d. A s mi gh t b e ex pec te d, th e les io n s o f o ral m u co s a an d sk in th a t o c cu r in th es e mu c o c uta n eo u s d is ea se s a re o f ten

su p erfi c ia lly di ffe ren t, a lth o u gh th e b as ic h i sto l og ic a l c h a ng es s e en i n th e ti ss u es a re si m ila r. Su c h di ff ere nc e s are s ee n in th e p rim ar y l es io n s a nd , pr es u ma bl y, de pe n d o n th e d iff ere n ce s

betw e en th e s tr uc tu re o f th e mo u th a n d tha t o f th e sk in . Q u ite o ften se co n d ary c h an g es a ls o oc c u r in o ral le s io n s. Th e c o n tin u a lly da m p e n vir onm en t of th e mo u th , in c o m bin a tio n w i th rep ea ted m ild tra u ma o f th e tis su e s by tee th a nd f oo d stu f fs , an d th e p re se n ce o f a wi de

ran g e o f m ic ro bi al fl or a fu rth er mo d ify th e n a tu re o f th e le si o ns p ro du c ed i n a n u mb er of dis e as es . F o r in s tan c e, s h o u ld th e e pi th el iu m be thin n ed b y a tro ph y o r w e ak en ed b y th e

fo rma tio n o f b lis ter s, i t is l ik ely to b e l o st an d th e in iti al le si o n be rep la c ed by a n u lc er. Fo r rea so n s s u ch as th es e , o ral le s io n s, pa rtic u la rly at an ad va n ce d sta ge , ma y sh o w f ea tu res les s c h ara c teri sti c tha n th o s e o f th e eq u iva le n t s kin les i on s o f th e s a me d is ea se . Cl in ic a l dia g no s is i n s u ch ci rc u ms tan c es m a y b e qu ite d iff icu lt s in c e on l y a re as o f u lc era tio n o f a

rel ati vel y n o n -sp ec if ic n atu re m ay b e p res e nt rath er th a n fu ll y d ev el op ed sp ec i fic l es io n s .

Histo pat ho lo gical changes

It ma y be h el pfu l to re ca ll so m e o f th e term s u se d to de sc ri be c h an g es s ee n o n h is to lo gi ca l

P. 8

stu dy o f th e o ral m u co s a .

Hyp erke ra tos is : a n in c rea s e i n th e thi ck n es s o f th e ke rati n la ye r o f th e e pi the li u m, o r th e p res en c e of s u ch a l a yer in a s ite w he re n o ne wo u ld n o rm al ly be ex pe cte d (Fi g. 1. 4). Hyp er or th o k era to si s is th e term u s ed to s pe cify a thi c ken e d, c o mp le tel y

kera tin i z ed la ye r, w h ere as i n h yp erp ara ke ra tos i s the re is i nc o m ple te ke rati n iz ati o n w ith nu c le i rem ai n in g in th e s ur fa ce c el ls .

Ac a nth o s is : a n in c rea s e i n th ic kn es s o f th e p ric kl e c el l la yer o f th e ep ith e liu m . Th i s ma y

or ma y n ot be ac c o mp an i ed b y h yp erk era to si s .

Atro p h y: a de c rea s e i n th e th i ck ne s s of th e ep ith el iu m (Fig. 1. 5 ).

Oe de ma : th e co l lec tio n o f fl u id in o r be twe en th e p ric kl e ce ll s, in tra - or in te rce ll ul a r, th e tw o fo rm s o ften o cc u rrin g s im u lta n eo u sl y. Oe de ma m a y a ls o o c c ur be tw een the epi th el iu m an d th e co ri u m in th e reg io n o f th e b a sal co m pl ex.

Ac a nth o ly si s : lo s s o f th e in te rce ll u la r a ttac h me n ts in th e pri ckle c el l l ay er le ad in g to

se pa ra ti o n o f th e c el ls . Wh en as so c ia te d w i th in terce ll ul a r o ed em a th is le a ds to th e pro d uc tio n o f in tra -epi th el ia l b u ll ae o r b lis te rs (Fi g. 1. 6).

Atyp ia : a term u s ed to d es c rib e v ar ia tio n s in th e m atu ra tio n o f the e pi the li al c el ls th at

ma y b e as s o ci ate d w i th ma li gn a n cy o r p re ma li gn a n t po ten ti al . S uc h f ea tu res a s

ab n or ma l mi to s es a n d la c k o f n or ma l stru c tu re of the ep ith el iu m a re ta ke n in to a c co u n t in th e as s es s me n t o f a typ ia .

The oral mucosa in generalized disease

Ora l le si o ns m a y o c c ur in a w i de va rie ty o f ge n era li z ed di s ea se s. Th is fa c t is i mp o rtan t, n o t on l y b ec a us e o f th e n ee d to tr ea t th e o f ten p ai n fu l or al le s io n s, b u t a ls o in vie w o f th e ir

si gn if ic an c e in p ro vi di ng a d ia gn o s tic in d ic ato r. Th e m o u th is re ad il y a va i la bl e fo r i n sp ec tio n (an d fo r bi op s y) a n d o ral l es io n s ma y ap pe a r ea rl y i n s om e di se a se s. T h us , a n u mb er o f

im po rta nt co n d itio n s m ay be f irs t d ia gn o s ed fo ll o w in g the p ro pe r e va lu a ti o n o f o ra l le si o n s.

Th e r ela tio n s h ip be tw ee n ge n era li z ed a nd o ra l di se as e is a ve ry co m pl ex o n e, b u t it m a y b e he lp fu l to id en tif y th re e ty pe s o f s u ch in terr ela tio n s h ip s ( Tab le 1. 1).

Table 1.1 Interrelationships between generalized and oral disease

In on e g ro u p o f c on d iti on s th e o ral l es io n s a re si mila r i n a eti ol o gy an d

hi sto l o gy to th os e fo u n d el se w he re in th e bo d y, b ein g m o di fie d on l y b y th e

or al en v iro n me n t. M an y o f th e o ral l es io n s o f s ki n d is ea se s fa ll in to th i s gro u p as w el l as th os e in so m e g a stro i n te s ti n al c o n diti o ns. T he s e a re di sc u s se d in Ch ap ters 11 an d 12 .

A se co n d gr ou p o f o ral l es io n s res u lts f ro m ch a n ge s i n th e me tab o li sm o f th e

ti ss u e u n de r th e in fl ue n ce o f ab n o rma li tie s o f n u tritio n , e n do c rin e, an d o th er fac to rs (Ch ap ter 13 ). Th es e ab n o rma li tie s the m se lves a re th e re su l t o f s o me dis ta n t pa th o lo gi ca l pro c es s . Th e o ral l es io n s a ss oci a te d w ith ma la b so rp tio n fal l in to th is g ro u p.

In th i s gro u p bo th o ra l an d m o re ge ne ra liz e d les i ons re s ul t fr om a s ys tem ic (an d di ffe ren t) ab n o rma li ty. S jΓ ¶g re n' s sy nd ro m e, a m an i fes ta tio n o f a

gen e ra liz e d au to im mu n e di s ea se re su l tin g in sa li va ry g la n d h ypo f u nc tio n , is an e xa mp le o f su c h a n as s o ci ati o n . Th is i s fu rth er di sc u s se d in Ch ap ter 8.

Cla s si fic a tio n o f the o ra lβ €“gen e ra l d is ea s e r el atio n s h ip s is d iff ic u lt an d m ay re su l t in

ov ers im p lif ic ati o n. It is e vid en t th a t m o re th a n o ne fo rm o f as s o ci ati o n mi gh t o c c u r in a

si ng le c as e . Fo r in s tan c e, a p ati en t w i th a di se as e o f th e lo w er ga s tro in tes tin a l trac t m ig h t

we ll pr od u ce p rim a ry l es io n s o f the di se a se o n th e or al m uc o s a, a s w el l as s ec o n da ry mu c o sa l ch a n ge co n s eq u en t to ma la b so rp tio n .

The periodontium in generalized disease

Al tho u g h gi n gi va l c h an g es m a y o c cu r in so m e ge n era li z ed co n d itio n s , th e gin g iv ae a re

us u a lly n o t pa rti cu l arl y g o o d di ag n o sti c in di ca to rs o f th e d is ea s e p ro ce s s in vo l ved β €”th e

ch a n ge s a re o fte n cl in i ca ll y n o n -sp ec if ic . Th e hi s to l o gi ca l ch a n ge s in a bn o rm a l g in g iva e ar e al so o ften ver y d iff ic u lt to i n terp ret. A p re -exi sti n g i n fla m ma to ry c e ll in fi ltra te an d , o ften ,

se co n d ary in f la mm a to ry fe a tu re s c on f us e th e p ic tu re an d m ak e gi n gi val b io p si es m u ch l es s

us e fu l th a n m ig ht be ex pe cte d. If th e a lte rn ati ve ex is ts be tw e en ta ki n g a g in g iva l bi o ps y o r on e fro m a n o the r a re a (fo r e xa mp le , th e b u cc a l m u c os a ), th e gi ng iv al s ite is b es t a vo id ed . A few sy ste mi c c on d iti on s h a ve be en des c rib ed i n w hi c h cl in ic a ll y re c og n iz a bl e gi n gi val

ch a n ge s o c c u r. Fo r in s tan c e, i n We ge n er's g ra nu l o mato si s, an u n c o mm o n va sc u li tic d is ea s e wi th w id es pre ad s ys tem ic i mp li ca tio n s , a qu i te c h arac teri s ti c gi n gi val p ic tu re h a s be en

des c rib ed a lth o u gh i t c erta in l y d o es n o t o c cu r in all ca s es . In We ge n er's g ra n ul o ma to si s , the gin g iv al le si o n m ay be a n in i tia l s ig n o f th e di se ase . Cl in ic a lly , th e g in g iva l su rf ac e

ap pe ars g ra n ul ar a nd f lec ke d w ith ye ll o w, an a p pe ara n ce re s em bl in g tha t o f β€ ov er -rip e stra w be rrie sβ €™. T he pro g n o si s o f We ge n er's g ra n ul oma to s is , if u ntr ea ted , is po o r an d th e ref o re e a rly rec o gn i tio n o f the g in g iva l le si o n is i mp o rtan t.

In Weg en e r's g ran u l om a tos i s, th e gi n gi va l l es io n m ay b e an i n itia l si gn of th e di se a se .

Cli n ic al ly, th e g in gi va l s u rfa c e ap pe ars g ra n ul a r and f le ck ed w ith ye llo w , a n a pp ea ra nc e res em bl in g th at of β € ov er -rip e str aw be rri es β€™.

Su c h s pe ci fic a n d rec o gn i z ab le ch a n ge s ar e r are c o mp ar ed to th e n o n -sp ec if ic gi n gi va l

ch a n ge s th a t m ay o c cu r in o the r d is ea s e pro c es s es an d th a t re pre se n t a n ac c en tu ati o n o r

mo di fi ca tio n o f th e w i de sp rea d ch a n ge s o f c h ro n ic peri o do n tal d is ea se o c cu rr in g in a l arg e

P. 9

pro p or ti o n o f th e p op u la tio n . F o r ex am p le, pa tien ts w ith se ve re, u n trea ted di ab ete s me ll itu s

ma y d ev el op a ra p idl y des tru c tive p eri o do n tal d is ease . In th e mu c o c uta n eo u s di s ea se s , su c h as l ic h en p la n us a n d pe mp h ig oi d, th e g in gi va l ch a n ge s ar e th e re su lt o f a β€ co ll is io n β €™

les io n be tw e en th e ge n era li z ed co n d itio n an d pr e-exi sti n g o r su p eri mp o se d ch a n ge s o f

ch ro n ic g in g ivi tis . Th e n o n - sp ec if ic n atu re o f the re su l tin g gi n giv al c h an g es i s ill u stra ted b y th e ad o pti o n o f th e term β € des q ua m ati ve gi n gi viti s β€™, u s ed to d es c rib e th e rea c tio n o f

th e g in gi va e in b oth th e se a n d oth e r c o n di ti o n s (Fig. 1. 8). Gin g iva l le si o n s in d is ea s es o f th e ski n a re di sc u ss e d i n Ch ap ter 11 an d e nd o c rin e -in duc ed c h an g es i n Ch ap ter 13.

Fig. 1 .8 β€ Des qu a ma tiv e g in g ivi tis β€™. Th e de fin i tive d ia gn o s is w as o f li c he n pl an u s .

Projects 1. 2.

Dis cu s s th e f u nc tio n s o f the b as em e nt me mb ra ne i n th e o ra l ep ith el iu m . Des cri be th e di ffe ren t z o n es w i thi n th e b a se me n t me mb ra n e. Wh i ch pro tei n s are k no w n

to be co m e ta rg et a n tig en s in th e a c qu ir ed bl is teri ng d is e as es ? (s ee Ch ap ter 11).

Au th o rs : Title:



Co py rig ht Β © 2003 Ox fo rd U ni ve rsi ty P re ss > T ab le o f Co nt e nt s > 2 - Pr in c ip le s o f or al m e d icine : as se s s m e nt a nd in v e st ig at ion of p at ie nt s

2

Principles of oral medicine: assessment and investigation of patients The speciality of oral medicine

Ora l me di ci n e i s ge n era ll y u n d ers to o d a s be in g th e stu d y a n d n on -su rg ic al tre atm en t o f th e

dis e as es a ff ec tin g th e o ro f ac ia l tis su e s, es pe ci al ly th e o ra l mu c o u s me mb ra n e, b ut al so o th e r as so c ia te d ti ss u es an d s tru ctu re s su c h a s th e s a livar y g la n ds , bo n e , an d th e fa ci al tis s u es .

Ora l me di ci n e is pr ed om in a n tly an o ut -pa tien t sp ec ia li ty. Th e bo u n d ari es o f o ral me di ci n e are po o rly de fi n ed . Fo r in s ta n c e, th e in ve s ti ga tio n o f fa c ia l p a in a n d o the r n e u ro lo gi c al

dis tu rba n c es m ay b e c o n si de red to b e in th e fie ld of o ral me di ci n e or o f o ra l su rg er y. It i s th e res po n s ib ili ty o f th e g en e ra l d en ta l pra c titio n e r to d ia gn o s e a n d ma n a ge s om e o f th e se co n di tio n s . Oth e rs are o fte n be tter tr ea ted in s p ecia li st cl in ic s , bu t th e ge n era l de n ta l

pra cti tio n er, to a ve ry g rea t e xte nt, b ea rs th e respo n s ib il ity fo r th e rec o gn i tio n o f o ral dis e as e at an ea rly s ta g e.

Definit ion o f o ra l med icine

Ora l me di ci n e i s th at ar ea o f sp ec ia l co m pe ten c e c on c ern e d w i th the h e al th o f a n d wi th

dis e as es in vo l vin g th e o ral a n d p a no ra l s tr uc tu res . It i n cl u de s th o s e pri n ci pl es o f me di ci n e

th a t re la te to the m o u th , a s w el l a s re se a rch i n b io lo g ic al , pa th o lo gi c al , an d c li ni ca l s ph ere s . Ora l me di ci n e i n cl u de s th e d ia gn o s is a n d me di ca l man a ge me n t o f d is ea s es s pe ci fi c to the or of ac ia l tis s ue s an d o f o ral ma n if es tati o ns o f s ystem ic d is ea se s . It fu rth er in c lu de s th e ma n ag em en t o f be h av io u ra l d is o rde rs a n d th e o ra l a nd de nta l trea tme n t o f me dic a ll y co m pro m is ed pa tie n ts.

Pro p o se d by th e W or ld Wo rks h o p on Ora l M edi c in e, h el d i n Ch i ca go , U S A, 1998 Th e d ev el op m en t o f the di sc ip li n e o f o ra l m ed ic in e h a s de pe nd ed l arg el y o n th e a do p tio n o f

an an a ly tic al a pp ro ac h b as e d o n th e a pp lic a tio n o f fu n da m en tal p rin c ip le s. It fo ll o w s th a t th e pra cti ce o f o ral m ed ic in e a s a sp ec ia li ty d ep en d s la rge ly o n th e a va il ab il ity o f d ia gn o s tic

fac i liti es , o fte n gre ate r th a n th o se a va ila b le to th e g en e ral d en ta l o r me di ca l pra c titio n e r. Pe rh ap s th e m o s t im po rta n t ro le o f th os e w o rki ng i n th e f ie ld o f o ra l me di ci n e i s in th e

rec o gn iti o n o f c h a ng es i n th e o ra l ca vi ty r es u ltin g fro m g en e ral iz ed d is ea s e p ro c es se s . Ma n y

or al le si o n s th at, i n th e p a st, w er e c o n si de red to b e o f e n ti rel y lo c al o rig in a re n o w kn o w n to

be as s o ci ate d wi th s ys te m ic a bn o rm al itie s . Fo r thi s rea s o n s pec i al is ts in o ral m ed ic in e h av e a cl os e w o rki ng re la tio n s hi p w ith a la rg e nu m be r o f me di ca l an d s u rgi ca l sp ec i al itie s. Th e m o s t po ten t f ac to r i n th e e xp an s io n o f th e s c o pe o f o ra l me di ci n e w a s th e c h a n ge o f e mp h as i s

fro m th e p u rel y d es c rip tive to th e in ve s ti ga tiv e. Th e m o de rn c on c ep t o f the s u bje ct im pl ie s a rec o gn iti o n o f b a si c ae tio l og ic a l f ac to rs , o f th e his to pa th ol o gi ca l an d m o lec u la r c h a n ges

oc c u rrin g in th e in v ol ve d ti ss u es , a n d of th e si gn ific a nc e o f s u c h ma tters a s th e ge ne ra l me dic a l sta tu s of p ati en ts. Th e c h a lle n ge fo r the futu re o f the s pe ci a lity is to de ve lo p evi de n ce -ba se d ma n ag em en t p ro to co l s.

Patient assessment History taking

Th e b a si s of a n y i n ves tig a ti o n is a c a ref ul a n d de tai le d c l in ic a l h i sto ry an d e xa mi n atio n . T he

pa tien t sh o u ld b e a ll o w ed to de sc ri be th ei r c o mp la in t(s ) a n d co n c ern s i n th eir o w n w or ds . It

is , h ow e ve r, o ften ne c es sa ry to a sk th e p a ti en t f o r m o re pre ci se o r deta il ed i nf o rma tio n . Th e sp ec if ic qu e sti o n s as ke d w i ll de pe n d u p o n th e p re sen ti n g co m pl ai n t an d th is w il l be

ad dre ss e d i n th e ap pro p ria te ch a pte rs. Reg ard le s s of th e or o fac i al c o nd iti o n th at th e p atie n t

ha s , it i s im po rta n t th at the c li n ic ia n , w he n q ue s ti o n in g the pa tie n t, do es n o t try to i n flu e nc e th e p ati en t's re sp o ns e . In ad di tio n , th e pa tie n ts mu s t n o t fe el as i f th e y a re be in g h u rrie d. Se n si tivi ty m a y b e es pe c ia ll y re qu i red fo r so m e co ndi tio n s . As w i th al l pa tie nt ca re , co n fi de nti al ity is o f th e u tm o st i m po rta nc e .

It is o f gre at im po rta nc e to o bta in de ta i ls o f th e m ed ic al h is to ry of th e pa tie nt an d o f an y cu rre n t o r re ce n t d ru g th e rap y. S im il a rly, th e p atien t s h o ul d be a sk ed at thi s sta g e a bo u t th e ir us e o f a l co h o l an d to ba c co . S o me p eo p le ar e po o r h is to ria n s w ith re ga rd to th ei r

me dic a l hi s to ry a n d i t is th e ref or e o f ten n e ce ss a ry to as k th e p a ti en t's g en e ral m ed ic al

pra cti tio n er to fi ll in th e de tai ls . Th i s is pa rticu la rl y re le va n t w he n a p ati en t h a s a ch ro n ic

co n di tio n th a t h as b ee n ma n a ge d b y s e ver al s pe ci al is ts. Th e c o rre sp o n de nc e th at th e g en er al me dic a l pra c titio n e r h a s co n c ern i ng s u ch a pa tie nt ca n g ive g rea t i ns ig h t in to th e pa tie n t's

co m pl ai n t an d c ar e. In th e h os p ita l e n vir on m en t, the req u es t fo r an d ca re fu l rea di n g th ro u g h of th e pa tie nt's g en e ra l h o s pi tal c as e

sh e et c a n be , by fa r, th e mo s t p ro du c tive m eth o d o f a s se ss m en t i n co m pl ic a te d ca s es . Wh e n

P. 14

dea l in g wi th th e pa st me di ca l hi s to ry i t is n e ce ss ary to us e di rec t q u es tio n in g o n s om e

po in ts . A s an e xa mp le , s of t-ti ss u e le si o n s of th e mo u th m ay be as s o ci ate d wi th s kin ras h es , eye a n d g en i tal le s io n s. T h e c o n n ec tio n w ith m o u th le si o n s ma y s e em qu i te te n u ou s to th e

pa tien t wh o m a y v ery w el l f ai l to vo lu n te er i n fo rm ati o n o n th es e po in ts u n le ss d ire c tl y a s ke d.

Pat ient assessment : so urces o f help ful informatio n 1.

Ho sp ita l ca s e n o tes

2.

RΓ© su m Γ© of m ed ic al a n d s o c ia l h i sto ry fro m g en e ral me di ca l pra c titio n e r

3.

Co rres p o nd en c e fro m o th er cl in i ci an s i nv o lve d in c a rin g fo r th e pa tie n t

The examination

Wh en e xa mi n in g the m o u th , th e w h o le o f th e o ra l mu co s a m u s t be c ar efu l ly exa m in e d. A ll

rem o va ble a pp li an c es s h o u ld be ta ke n o ut. Th e pra c titio n e r s h ou l d a p pro a ch the e xa mi n ati on

of th e pa tie nt in a s ys tem ati c ma n n er to en s u re that a ll th e rel eva n t ti ss u es h a ve be en s e en . Th e l ip s an d ch e ek s mu s t be g en tly re trac ted to d ispl ay th e fu ll ex ten t o f th e s u lc i an d th e

to n g u e g en tly h el d wi th th e ai d o f a ga u z e na p kin , a n d exte n de d f o rw ard a n d to ea c h si de .

Car e m u s t be ta ken th a t th e w ho l e of th e flo o r of th e mo u th a n d u n de rsu rf ac e o f th e to ng u e is s ee n. T h e p o ste rio r pa rt o f the to n gu e , to ns i llar fa u ce s, so ft pa la te, a n d pa rt o f the

ph a ryn x a re e xpo s ed b y g en tle p res s u re o n th e to n g ue a n d h e lp ed b y p h on a tio n o f β€ ah β€™

by the p ati en t. Th i s e xa m in ati o n o f th e o ra l m u co s a m us t b e co m bi n ed w ith a c ar efu l

as se s sm en t o f th e oth e r de n tal s tru ctu re s, f ac ia l sk el eto n , s al iva ry gl an d s, a n d so f t tis s ue s

of th e fa ce a n d ne c k. A s ea rc h fo r p a lp ab le ly mp h no de s sh o u ld b e m a de , rem em b eri ng th at

no rm a l l ym ph n o d es a re n ot de tec tab le by s im pl e p a lp ati on . S w el li n gs o f th e ly mp h n o de s are fu rth er d is c u ss ed i n Ch ap ter 7. Th e as s es s me n t o f ne ur o lo gi ca l ab n o rma l itie s is d is cu s s ed in Ch ap ter 15 . Pra c titio n e rs sh o u ld b e fa mi lia r wi th th e te sts re qu i red to a s se ss gro s s fu n c tio n of th e cra n ia l n erve s , in p ar tic ul a r th e fif th an d s eve n th cr an i al n erv es .

Th e e xtra o ra l e xa mi n ati on ma y be ex ten de d to in c lu de th e ge n era l ap pe ar an c e an d

dem e an o u r o f th e p ati en t. Th e ey es , s ca lp , n ec k, h an ds , a nd th e sk in o f th e f ac e an d a rm s sh o u ld u s ua ll y be in s pe cte d to o bta in s ig n if ic an t in fo rm ati on ( Tab le 2. 1). E ac h o f the s e vis u al ly a cc es s ib le ar ea s ca n d em o n stra te si gn s th at al ert th e p rac titi o ne r to po s s ibl e

un d erl yin g s ys tem ic d is ea s e. Th e i n trao ra l ex am in a tio n s h o ul d tak e p la c e w i th an ad eq u ate li gh t s o u rc e. S o me tim es

ti ss u es req u ire d ryin g to e n ab le th o ro u gh v is ua l iz ati o n β €”th i s is p arti cu l arl y r el eva n t to

te eth . O c ca s io n al ly , in p ati en ts w ith a β € pa rch m en t β€™ dry o ra l m u co s a , it is h e lpf u l to

al lo w th e pa tie nt to rin s e wi th w ate r b efo r e c o n tinu in g w ith th e in tra o ral e xa mi n ati on β €”th i s

wi ll h el p l u bri ca te th e s o ft ti ss u es a n d pre ve n t you r g lo ve s o r m irr or s ti c kin g to a n d

tr au m ati zi n g th e tis s u es . Co n ve rs ely , in so m e pa tien ts w i th co p io u s vi sc o u s sa l iva , a mu c o lyti c mo u th w as h m ay be h e lpf u l pri o r to i n trao ra l ex am in a tio n .

Table 2.1 Useful information from extraoral examination O bser v atio n

In fo r ma tio n ( exam ples o f ass oc iated c o nd itio n s)

Gen e ral

Was ted , u n de rn o ur is h ed, ca c h ec tic a pp ea ra n ce , (e. g .

ap pe ara n c e, a n d

mo o d, a n xi ety, ag ita tio n (e .g . de pre s si o n)

dem e an o u r, ma n ne r

ma ln u triti o n, ea tin g di so rd er, u n de rlyi n g m a li gn a ncy) ; lo w

Bre a thl es s ne s s

Car di or es pi rato ry pr ob le ms

Fa c e

Sh a pe a n d s ym m etry (m as se teri c h ype rtro p hy , cr an i ofac ia l syn d ro me s ); C us h in g o id a pp ea ran c e (e. g . co r ti c os teroi d th e ra py) ; n eu ro l o gic a l d ef ic its (e . g. B el l' s p a ls y, cr an i al

tu m o u rs) ; c ya n os i s (e. g. ca rdi o res p ira to ry d is ea s e); pa ll o r (e. g. a n xi o us , u n w el l, a n ae mi c ) Sc a lp an d fa c ia l

Sc a nt h ai r (e .g . e cto de rm al dy sp la s ia )

Ey es

Co n jun c tiv al s ca rri n g ( e. g. p em ph i go i d, ke rato c o n junc tiv itis

ha ir

si cc a ); p al e sc le ra (a n ae mi a) ; ye ll o w sc le ra (ja u ndic e) ;

exo p h th a lm ia ( hy pe rth yro id is m ); x an th o ma s o f p er io cul ar ski n (h yp er ch o le s te ro la em ia ) Nec k

En l arg ed l ym ph n o de s (o ra l i n fe cti on , n e o pl as ms ); go i tre

Han d s

Ray n au d 's ph e no m en o n ; k o il on y ch i a an d o th er l es io n s o f th e na il s ; fi n ge rs: jo in t s w el li ng a n d a c qu i red d is fig urem en t

(rh eu m ato id a rth riti s) ; He bd en 's n o d ul es (o s teo a rthro s is );

pa lm ar ke rato s is (P a pi ll on β €“Lef Γ¨v re sy n dro m e); β€ liv er pa lm s β€™; to ba cc o sta in i n g; fin g er cl u bb in g (ch ro n ic

ca rdi o res p ira to ry p ro bl em s, in c lu d in g in fe cti ve en do ca rdi tis ) Wris ts

Pu rp le pa p ul es c o n si s te n t w ith li ch e n pl an u s

Sk in

Pe tec h ia e o r ec c hy mo s es ( e. g. b lo o d dy sc ra s ia ); c yan o si s

(ca rd ia c o r p ul mo n a ry in s uf fic i en c y); j au n di ce ; p igme n tati o n (po s si bl e en do c rin e pro b le ms )

It is e ss en ti al th at ca s e n o te en tri es a re:

leg ib le

co n tem po ra n eo u s

da ted

si gn ed

It is o f pa ra mo u n t i mp o rtan c e tha t a ll th e de ta i ls o f th e h is to ry an d ex am in a tio n o f th e

pa tien t are c o mp reh e n si vel y rec o rde d in th e pa tie n t' s rec o rds . It i s o ften h el pfu l to m ak e a ske tc h o f s o me l es io n s in th e c as e n o te s . Th is w il l in di ca te th e s h a pe an d p o si tio n o f th e

les io n an d h el p c l in ic ia n s at fu tu re ap po i ntm en ts . A to po g ram o f th e o ra l ca vi ty(Fi g. 2. 1 ) is pa rtic u la rly u se fu l fo r th i s pu rp os e . A w ell ta ke n cl in i ca l ph o to gra ph ca n a ls o p ro vid e a

val u ab le re co rd o f an or of ac i al le si o n . It w il l a lso b e us e fu l f o r tea c h in g an d es s en tia l if a ca se re po rt is to b e s u bm itte d

fo r p u bli c ati on . T he p ati en t sh o u l d b e in fo rm ed a s to w h y p h oto g ra ph s a re r eq ui red a n d th e pa tien t's pe rm is si o n is a lw a ys re qu ir ed . It is e ss en ti al to o bta in wr itten c o n s en t i f i t is en vi sa ge d tha t th e ph o to gr ap h w il l b e us e d i n a pu bli ca tio n .

P. 15

Fig. 2 .1 A to po g ram o f th e o ral c a vity . (Rep ro du c ed w i th the p erm is s io n o f Zi la Eu ro p e. )

Investigations

A la rge n u mb er o f d ia gn o s tic te sts a n d pro c ed u res mu s t be a va il ab le to th o se w o rki n g i n th e fie ld o f o ra l me di c in e. Tab le 2. 2 li sts m an y o f th e in v es tig ati on s a n d di ag n os tic tes ts u se d; ho w e ver, th i s li st i s n o t ex h au s tive . Th e ad va n tag es o f cl o se as s o ci ati o n w ith s pe ci a liz e d dep a rtme n ts, s u ch as th o s e o f c lin i ca l im mu n o lo g y, h ae ma to lo g y, m ic ro bi o lo gy , cl in i ca l

ch e mi stry , ga str oe n tero l og y, a n d de rm ato lo g y, a re ev ide n t, an d vi rtu al ly a ll ce n tres i n w hi c h or al m edi c in e cl in i cs a re s uc c es s fu ll y c o n du c ted enjo y su c h a ss o c ia tio n s. Al tho u g h th e r an g e of in v es tig ati on s c a rrie d ou t in th e o ral m ed ic in e c lin i c its el f m ay b e w i de , it is e vid en t th a t,

in m an y in s tan c es , i t is p ro pe r to re fe r th e pa tie nt to a co l le ag u e i n s o me o th er sp ec ia li ty f o r su b se qu en t in ve sti ga tio n f ol lo w in g in i ti a l d ia gn o s is i n the o ra l me di ci n e c l in ic . S o me u n its wi ll h av e sp ec ia l in ter es t o r e xp erti se in ce rtai n d is ea se s an d m ay b e a bl e to o ffe r

so p hi s ti c ate d in ve s ti ga tio n s a n d i n teg rate d mu lti disc ip li n ary c ar e th a t is n o t ro u ti ne ly ava i la bl e i n a ll o ral m ed ic in e c lin i cs .

Th e im po rta n ce o f tak in g a pa tie n t' s tem pe ratu re w hen th e re is a

dis s em in a ted in fe c ti o n s ho u l d n o t g o u n me n tio n ed . Th is w i ll in d ic ate if th ere are a n y sy ste mi c ef fec ts fro m th e in fe c ti o n .

Sp ec ia l in ve s ti ga tio n s w il l be di sc u s se d su b se qu e ntl y, i n the a pp ro pr ia te c h a pter s a n d in

rel ati on to the s u bje ct o f th e in ve sti ga tio n . Ho w e ver , it i s h elp fu l to pre s en t a n o ve rvi ew o f th e tes ts in th i s se c ti o n . It s ho u l d b e rem em be re d th a t ve ry f ew s pe c ia l i n ves tig a tio n s

pro vi de a d efi n itiv e d ia g no s is . It i s mo re u s ua l for th e re su l ts o f s eve ra l i n ve sti ga tio n s to be co m bi ne d w ith th e cl in ic a l h is to ry a n d pre se n tati o n be fo re a de fin i tive d ia gn o s is c an be

ma de β€”th i s is e xem p lif ie d b y th e d ia gn o s tic c rite ria fo r Sj Γ ¶g re n' s sy nd ro m e. Th e m o st co m mo n l ab o ra to r y i nv es tig ati o n req u es ted in the o ra l me di ci n e c li n ic i s a sc re en i ng

pro c ed u re f o r p os s ib le bl o o d a b no rm a li ti es . In v iew of i ts w i de sp rea d ap pl ic a tio n th is m eri ts pre lim in a ry di sc u s si o n.

P. 16

Table 2.2 What to consider in patients assessment Hist or y

In ves tiga tio ns

Co mp la in t a n d h is to ry

Hae m ato lo g y

Fu ll m ed ic a l h i sto ry

Cli n ic al Ch em is try

Sp ec if ic q ue s ti o n s

Im m un o l o gy

S k in i nv o lve me n t

En d o cri n e s tu di es

E y e p ro bl em s

Uri na l ys is

G en ita l sy mp to ms

Bi o ps y

G u t pro b le ms

Mi cro b io l og y

Dru g h i sto ry So c ia l hi s to r y (l ife s tyle )

Im a gi ng P l ai n ra di og ra ph s

T o ba cc o

S a li va ry g la n d im ag in g

A l co h o l

C T s c an

M R sc a n

U ltra s o u nd Clin ic al exam in atio n

Bl o o d pre s su re

Ex tra -or al

Bo d y T em pe ratu re

In tra -or al

Bi o ch e mi stry

Blood examination

Th e a s se s sm en t o f o ra l m ed ic in e pa tie n ts of ten i n volv es h ae m ato lo g ic a l, b io c he mi c al , an d

se ro lo gi c al in ve s ti ga tio n s . Th es e tes ts ma y be d on e e ith er as s c ree n in g tes ts to h elp i n th e dia g no s is o f s om e u n id en tifi ed c o n di ti o n o r to c o n fi rm a dia g no s is b y th e ap pl ic a tio n o f sp ec if ic tes ts . In th e f irs t in s ta nc e it ma y b e h elp fu l fo r th e rea d er to ca teg o riz e tes ts gen e ric a ll yβ€” ac co r din g to th e de pa rtm en t th a t pe rfo rm s th e i n ves tig a tio n .

Haematology

As a s c ree ni n g pro c ed u re fo r p o ss ib le h ae ma to lo g ic al a bn o rm al ity , a ha e mo g lo bi n es tim a tio n ,

wh i te c el l co u n t, a nd bl o o d f ilm e xa mi n ati o n w ere on ce c o n si de red to b e s u ff ic ie nt. O ra l si gn s an d sy mp to ms m a y, h o w eve r, a cc o m pa n y re la tive ly m ino r c h an g es i n th e b lo o d a nd o ra l

les io n s m ay o c cu r ea rly in pa tie nts w i th ha e ma to lo gi c al a bn o rm al itie s , we ll be fo re th es e are sh o w n u p by the s im pl e exa m in a tio n o f pe rip he ra l b lo o d . A si gn i fic a n t lo w er in g o f s eru m o r red c ell f ol ate l eve ls o r (le ss f req u en tly ) lo w er ed s eru m B 1 2 le vel s m ay o cc u r i n th e a b se n ce

of a n y d ete cta bl e ch a n ge i n th e p eri ph e ral e ryth ro cytes , p arti cu l arl y i n p atie n ts w ith

sto m ati ti s an d re cu rr en t u lc e rati o n. It the ref o re fo ll o ws th a t th e si mp le fu l l b lo o d co u n t is a n

in su f fic ie n t p ro ce du re fo r the i ni tia l in ve sti ga tion o f su c h p ati en ts. Ha em a to l o gy rep o rts (a nd al so th o s e of o th er in ve sti ga tio n s ) s h o ul d be in terpre ted w ith th e ai d o f th e n or ma l va lu es o f th e la b o rato ry in vo l ve d. Th e fi gu re s q u o ted in Tab le 2. 3 mu s t b e r eg ard ed a s a gu i de lin e

on l y, p arti cu l arl y i n re la tio n to b o rde rli n e v al u es. In th e ca s e o f bo rd erl in e re su lts o f c lin i ca l si gn if ic an c e it i s rec o m me nd ed th a t a s ec o nd sa m pl e s h o u ld be ta ke n if at al l po s si bl e,

la bo ra to ry f ig ur es a re su b jec t to so m e de gre e o f vari ati o n. It mu s t be re pe ate d tha t s u c h res u lts s ho u l d a l wa ys b e in ter pre ted in th e li gh t of th e ag e o f th e pa tie nt an d th e n o rm a l val u es g ive n by th e la bo ra to ry i n vo lv ed .

Table 2.3 Representative laboratory normal values for haematological tests Tes t

No r m al va lue*

Hae m og lo b in

12. 5 β€“17. 5 g/d l ( ma le );

Me an Cel l V o l um e

80 β€“ 95 fl

ES R

0β€“ 15 mm /h

Red c el l co u n t

4β€“ 6 Γ— 10 1 2 /l ( ma le s) ; 4 β€“5

Wh ite bl o o d c el l co u n t

4β€“ 10 Γ— 10 9 /l

Hae m ato c rit

40 β€“ 50 l/l (m al es ): 34β€“47 l/l

Pl ate le ts

150 β€“400 Γ— 10 9 /l

Se ru m Fo l ate

1. 5 β€“5. 5 Β µg/l

Red c el l Fo l ate

125 β€“600 Β µg/l

11. 5 β€“16. 0 g/d l (fe ma le )

Γ— 10 1 2 /l ( fem a les )

(fem a le s)

Se ru m B 1 2

170 β€“590 Β µg/l

* A ll res u lts s h o ul d be c o mp are d to th e n o rm al val u es o f th e te sti n g la bo ra to ry.

In Tab le 2. 4 th e m ea n in g s o f s o me o f the te rms u se d to de sc ri be va ria tio n s fr om n o rm a l i n th e s iz e an d s h ap e of e ryth ro cy tes ar e g iv en , to ge th er w i th an in di c ati on of s o me o f th e co n di tio n s in wh i ch th e se fo rm s ma y o cc u r.

Hae m ato lo g ic a l i n ves tig a ti o n s are h el pf u l i n th e d ia g no s is o f th e fo ll o w in g co n d itio n s : Leu ko p en ia s An a em ia s Th ro mb o cy top en i as In f ec tio u s mo n o n u cl eo s is My elo m a s Po l yc yth ae mi as Leu ka em ia s

Table 2.4 Variation in size and shape of erythrocytes Descr ipt ion

Er y th ro cy te

ch ara cter istic s

Seen in

Hyp o ch ro m ic

Pa le s tai n in g

Ir on de fic ie n cy a n ae mi a

Hyp erc h ro mi c

Den s e s ta in in g

Pe rn ic io u s a na e mi a

Mi cro c yti c

Sm a ll

Ir on de fic ie n cy a n ae mi a

Ma c ro cy ti c

Larg e (10β€“ 12 Β µ)


Me ga lo c ytic

Ve ry la rge (12β €“25


Β µ)

An i so c yto s is

Mu c h va ria tio n in si z e

Mo s t a n ae mi as

Po i kil o cy tos is

Mu c h va ria tio n in

Mo s t a n ae mi as

Sp h ero c ytic

Sp h eri ca l

Co n ge ni tal h a em o lyti c an a em ia s

Tar get ce ll

Co n ce n tr ic al ly s ta in e d

An y ch ro n i c an a em ia

Ery th ro bl as t

Nu cl ea ted

Den o tes e xc es si ve ery th ro po ie si s

No rm o bl as t

No rma l si z e

Af te r h a em o rrh a ge , ve ry s ev ere

M ic ro bl a st

Sm a ll

Pe rn ic io u s a na e mi a, ca rc in o m a

Reti cu la ted w h en

If h ig h er tha n 1% in ad u lts β€” an

sta in s

dem a n d f o r ery th ro cy te s

(le pto c ytes )

M eg al o bl as t Reti cu lo c yte

sh a pe

Larg e

sta in ed w ith vi tal

an a em ia s an d le u ka em ia s

sto m ac h , af ter tota l ga s tr ec to my

ac tive m a rro w res p on s e to a

A si gn if ic a nt pro p or tio n o f pa tien ts a tten d in g the o ra l me di ci n e c li n ic w ill re qu i re fu ll

ha em a to lo gi ca l sc re en i ng . It i s su g ge ste d th a t th e fo ll ow i n g gro u p s wa rra n t th is e xten d ed sc ree n in g pro c e du re (s ee a ls o Ch ap ter 13):

pa tien ts w ith rec u rren t a p tho u s s to ma titi s

pa tien ts w ith a p er si ste n tl y so re a n d/o r dry mo u th .

pa tien ts w ith or al le s io n s wi th a n aty pi ca l h is tor y o r u n us u a lly re si sta n t to tre atm en t;

pa tien ts co m pl a in in g o f a s o re o r b u rn in g mo u th o r to n g ue , o r a b no rm a l ta s te

al l pa tie n ts wi th pe rs is ten t o ro fa c ia l ca n di do s is .

Pa tie n ts s h o w in g ab n o rma li tie s fo ll o wi n g an i n itia l sc re en i ng (i e h ae mo g lo b in a n d fu ll

se n sa tio n , ev en th o u gh n o mu c o sa l ch a n ge s ca n b e s een ;

blo o d c o un t).

Table 2.5 Useful biochemical investigations In ves tiga tio n

Descr ipt ion

P. 17

Glu c o se

Rai se d in d ia be tes m el li tu s , Cu s h in g 's sy n dro m e

Hyp o gly ca e mi a oc c u rs mo s t c o mm o n ly in d ia be tic p atien ts an d ma y o c c ur in se ver e l iv er d is e as e

Ure a

Rai se d in d eh yd ra tio n , re na l fa il ur e

Crea tin i n e

Rai se d in re n al fa i lu re

El ec tro ly te s S o di u m

El ev ate d i n de h ydr ati on

P o tas s iu m

Rai se d in re n al fa i lu re, dia b etic ke toa c id o si s

Low in c o n diti o n s ca u si n g o v erh yd ra tio n , e. g . ca rd ia c fa il ur e

Hyp erk al ae mi a is c o mm o n ly ar te fa c tu a l du e to h ae mo lys is ,

del a yed s pe ci me n s, or eth yl en e di am in e tetra a ce tic aci d (E DTA ) co n tam i na tio n

Hyp erk al ae mi a is a m ed ic al e me rge n cy a n d mu s t b e c orre cte d im me di ate ly

Hyp o kal a em ia is c o m mo n ly d ue to di u reti cs o r ga stroi n te s tin al lo ss e s of p o ta s si u m Ca l ci um

Hig h in pri ma ry h ype rp ara th yro i dis m , ma l ign a n cy , vitam in D exc es s

Low in ri ck ets , o ste o ma la c ia , h ypo p a rath yr oi di sm P h o sp h ate

Al ka li ne

ph o sp h ata s e

To tal pr ote in

Hig h in ren a l fa il ur e

Low in ri ck ets / os teo m a la ci a Rai se d in co n d itio n s w ith in cr ea se d bo n e tu rno v er su c h a s Pa ge t's di s ea se , ric ke ts/ os te om a la ci a

Al so rai se d in l ive r d is ea s e, p ar tic ul a rly ch o l es tas is Rai se d in d eh yd ra tio n , li ve r d is ea se , m yel o ma , c o nne cti ve ti ss u e di se a se s, a n d sa rc o id o si s

Red u ce d i n o ve rh yd rati o n, en te ro pa thy , ren a l fa il ure Al bu m in

Rai se d in d eh yd ra tio n

Red u ce d w h e n th ere is a n a c ute ph a se re s po n se , e. g . in fl am ma tio n , p o sto p era tive ly , ca rc in o m a

Al so rai se d in s ev ere l ive r d is ea s e, m al a bs o rpti on , n ep h ro ti c syn d ro me , c o nn e cti ve tis s ue d is ea s es Fe rriti n

Rai se d in l ive r d is ea s e, h ae ma c h ro ma to si s, leu k ae mia

Red u ce d i n i ro n de fic ie n cy an a em ia Live r e n zy me s

Dis tur bed i n li ve r di s ea se , s om e dru g th era pi es

En z ym e- in du c in g dr ug s , e. g. c a rba m az e pi ne , ph e n yto in ,

ph en o b arb ito n e, c a u se a m il d e le va tio n i n al ka li ne p h os p h ata se an d Ξ³ glu ta ryl tra n sf era se (Ξ ³GT)

Si n ce th es e gro u p s co n s titu te a s ig n if ic an t p ro po rti o n o f p a ti en ts atte n di ng th e o ral m ed ic in e cl in ic , th e ab o ve sc re en i s al mo s t a ro u tin e pro c edu re in th i s en vi ro nm e nt.

Clinical chemistry

Th ere a re se ver al b io ch e mi c al in ve s ti ga tio n s th a t ar e f req u en tly req u es ted fo r pa tie n ts pre se n tin g wi th o ra l me di ci n e pro b lem s a n d s e vera l o f th e se a re gi ven i n Tab le 2. 5.

Pl as m a glu c o s e m ea s u rem en ts a re req u ire d to di ag n o se d ia be tes m ell itu s (DM ) a n d in th e mo n ito ri ng o f th is s eri o us c o n di tio n . Th e di ag n o stic c ri teri a fo r th e di ag n o si s o f DM w e re

al te re d i n 1999 a n d a re a s fo ll o w s: sy mp to ms p lu s ra n do m pl as m a gl u co s e >11. 1 m m ol /l;

rep ea ted ra n do m bl o o d g lu c o se > 11. 1 m mo l/ l i n a sy mptom a tic in d iv id ua ls ; or fa s ti n g pla s ma glu c o s e > 7. 0 m mo l /l. F o rm al g lu co s e to le ran c e tes ts are n o w p erfo rm e d l es s fre qu e ntl y th a n pre vio u s ly an d a re req u ire d on l y i n c om p lic a ted pa tie n ts.

Immunological tests

A wi de ra n ge o f im mu n o lo g ic al tes ts is a va il a ble to as s is t i n the d ia gn o s is o f di se as e s aff ec tin g th e o r al c av ity an d ma n y su c h tes ts fo rm a n es s en tia l pa rt o f the d ia gn o s tic

pro c es se s o f th e o ra l m e dic i ne cl in i c. Tab le 2. 6 h ig hl ig h ts so m e o f th e te s ts th a t m ay b e

req ue s ted. Ma n y i mm u n o lo g ic al ly ba s ed tes ts ar e n o w m atte rs o f r ou ti ne . O the rs ar e o n l y ava i la bl e i n s pe ci al is t c en tre s.

P. 18

Table 2.6 Useful immunological tests Au to a nti bo d ie s

R h eu ma to id fa cto r A n tin u c le ar fa cto r

S S -A, S S -B an ti bo d ies P a rie tal c el l an ti bo d y A n ti -gli ad en

A n ti -en do m ys ia l

E p ith el ia l in ter ce ll ul a r c em en t

E p ith el ia l ba se m en t me mb ra n e

C1 e ste ras e in h ib ito r (red u ce d in h e red ita ry an g io ede ma )

Vi ral an ti bo d ies H IV

E p ste in β €“Ba rr vi ru s

C-rea cti ve pro te in ( rai se d in i n fla m ma tio n a n d ma lign a n cy )

Ha ema to lo gical screening p ro to co l

Wh en a s c ree ni n g pro c ed u re is d ec id ed u po n , a re as on ab le s c he me o f in ve s ti ga tio n i s as fo ll ow s : 1.

Fu ll b lo o d co u n t a n d fil m ex am in a tio n . F ro m th i s, e vi de nt an a em ia s a re de mo n s tr ate d

by va ria tio n s in red c el l m o rp ho l o gy an d lo w e red h aem o glo b in v al u es . A bn o rm al itie s o f th e w h ite c ell s an d p la tel et n u m ber s are a ls o s h o wn. Ha em a to l o gi ca l in di ce s s uc h a s th e re d a n d w hi te bl oo d c el l c o u n ts, th e me a n co r pus cu la r ha e mo g lo bi n , me an

co rp u sc u la r v ol u me , th e h a em a to cri t a nd p la tel et cou n t a re im po rta n t a n d a b no rm a l val u es m ay i nd ic a te u n d erl yin g s ys tem ic di se a se . 2.

Es tim a tio n o f se ru m ferr itin as a me a su re o f fu ll bo d y i ro n s tatu s . Th is te st ha s a lm o st

en tire ly rep la c ed th e es tim a ti o n s of s eru m i ro n, tota l iro n b in d in g ca pa c ity, an d

sa tu rati o n fo rm erl y u s ed a s a s cre en i ng te st, a lth ou gh th e se a re sti ll u se d in th e 3.

in ve sti ga tio n o f co m pl ex iro n -def ic ie n cy s tate s.

Se ru m B 1 2 , fo la te an d red c el l fo la te es tim ati o ns . Th e se ar e v al u ab le in d ic a to rs o f

ma la bs o rp ti o n a nd , h en c e, o f ga s tro in tes tin a l di se ase s o f m an y ki n ds . Th e red c el l fo la te l ev el is a re la tiv ely s tab le in d ic ato r of fol ate d efi ci en c y, w h er ea s the s eru m

fo la te l ev el s are m o re la bi le a n d i n di ca te th e c u rren t s ta tus . It h a s be en s h o w n th at

th e se m ay s h o w in d ep en de n t c lin i ca ll y si gn if ic an t va ria tio n i n pa tie n ts p re se n ti n g wi th or al si gn s a n d sy mp tom s . Co el ia c di s ea se i s an e xa mp le o f a ga stro i n tes tin a l di se a se

th a t c an res u lt i n m al ab s or ptio n o f h ae m ati ni cs . Low f ol a te l eve ls m a y al s o be d ue to an tic o n vu ls a n t dru g th era p y, p reg na n c y, a n d al c oh o lis m.

A vita mi n B 1 2 de fi ci en c y s h o u ld be su s p ec ted if th ere is a m a croc yto s is β€”in di ca ted b y

a r ai se d me an co rp u sc u la r vo lu m e an d p ac ke d c e ll volu m e. 4.

As a n a dd itio n a l tes t a n er yth ro cy te s ed im en ta tion ra te (E S R) m ea s ur em en t i s us e fu l as

a n o n -sp ec if ic gu i de to u n de rly in g pa th o lo gi ca l pr oc e ss es β€” al te rn a tive ly me a su re me n t of C- rea cti ve pro te in ( CRP ) m a y b e u s ed a s a s im ilar m a rke r fo r pre -exi sti n g d is ea s e (se e Cli n ic al Im mu n o lo g y). Th e E S R i s rai se d in pregn a n cy , c hr on i c in fl am m ato ry co n di tio n s , ac u te in fe cti o n, g ia n t c el l ar teri ti s , an d n eo p la si a.

Th e a s sa y o f ci rc u la tin g au to a nti bo d ie s is a n im po rta n t pr o ce du re in th e o ra l m e dic i ne cl in i c. So m e au to a nti bo d ies a re c lo s el y a s so c ia ted w ith s p ec ifi c di se a se pro c es s es β€” fo r i ns ta n ce , ga stri c pa rie tal c el l an tib o di es w ith p er ni c io u s ana em ia . In o th er a uto i mm u n e d is ea s es ,

ho w e ver, a wi de ra n ge o f a u to an ti bo d ies ma y be pr o du c ed . Fo r thi s rea s o n it i s u su a l to ca rry ou t a r an g e o f au to a n tibo d y te s ts r ath er th an a s i ng le o n e.

Dire ct im mu n o fl u o res ce n t tec h n iq u es , fo r th e d etec tio n o f im m un o g lo b ul in s a n d o th e r

im mu n o lo g ic al ly ac tiv e pro tei n s fix ed w ith in tis su e, h av e a c qu ir ed gr ea t im po rta n ce i n th e dia g no s is o f o ra l m u co s a l l es io n s β€” pa rtic u la rly tho se as s o ci ate d wi th s kin dis e as es a n d

co n n ec tiv e ti ss u e di se as e . Th e p ri nc ip le o f th e te ch n i qu e de pe nd s o n th e fa ct th a t a n tibo d ie s co m bi ne d w ith fl u o res ce in reta in bo th th ei r i mm u n olo gi ca l ac tiv ity an d th e pro p erty o f th e

flu o re sc ei n to fl uo re s ce u n de r u ltra vi o le t li gh t. Th e a nti bo d ie s ca n , th ere fo re , be lo c a te d at th e e xa ct s ite o f co m bi n ati o n w ith th ei r a n tige n ic a n tag o n is ts by mi cro s c o pi c o bs erv ati o n un d er ul tra vio l et i ll um i na tio n . A w i de ra ng e o f h i gh ly s pe ci fic an ti bo d ies to th e va ri o us

im mu n o gl o bu li n s an d c om p lem e nt co m po n e nts i s av ai labl e an d c an be u se d to de mo n s tra te

th e typ e an d s ite o f b o un d c o mp le xes . In s o me co n d itio n s th e res u lts m ay b e h i gh ly s pe ci fi c an d di ag n o sti c (in p em ph i gu s a n d pe mp h ig o id, fo r e xa mp le ). In o th er co n di tio n s (s u ch as lic h en pla n u s) th ey ar e n o t cl ea r. Th e fi n di ng s in a nu m be r o f co n d itio n s a re di sc u s se d in

Ch ap ter 11. Th e s p ec ia l req u ire me n ts f o r th e taki n g of b io ps y ma ter ia l f o r th is te ch n iq u e are me nti o n ed la ter in th is c h a pte r.

Endocrine function

En d o cri n e d is o rd ers a re im po rta n t in o ra l me di ci n e fo r th e fo ll o w in g rea s on s .

Pa tie n ts m a y p res e nt wi th a c o mp la in t th a t le ad s to th e di ag n o si s o f a n e nd o cr in e

dis tu rba n c e. A n e xa mp le is a p ati en t w i th o ral dy saes th es ia o r xe ro sto m ia w h o u po n in ve sti ga tio n i s fo u nd to b e an u n dia g no s ed d ia be tic .

Po o rl y c o n tro lle d h or mo n a l i mb a la nc e s ma y l ea d to oro fa ci a l di se a se .

Th era py o f or of ac ia l di se a se m ay le ad to h o rmo n a l im ba la n ce s. An exa m ple o f th is is

P. 19

th e u s e o f s ys te m ic s tero id s to trea t s ev ere u lc er ati on o r bu l lo u s di s ea se β€” lo n g- te rm th e ra py wi ll in d u ce ad re na l su p pre s si o n an d p red is po s es to w a rds d ia be tes a n d os teo p o ro si s.

Co n cu rre n t en d o cri n e dis e as e o r h o rmo n e rep la c em en t th e rap y m a y i n flu e n ce th e ma n ag em en t o f th e pa tie n t.

Ex am pl es o f h o rmo n e s tu d ie s tha t m a y b e req u es ted in o ral m ed ic in e ar e th y ro id fu n cti o n te s ts a n d pa ra tho rm o n e, g ro w th h o rmo n e , an d c o rtis ol l eve ls .

Urinalysis

Uri na l ys is , w hi c h ca n b e d o n e q u ic kl y a n d co s t-eff ec tive ly , is u s ed to id en tif y th e pr es en c e o f glu c o s e, b lo o d, p ro tei n s, k eto n es , a nd b il e p ro du c ts . Th is c a n al ert the c li n ic ia n to th e

po s si bi lity o f u n d erl yin g s ys tem ic d is ea s e an d , th ere fo re , fu rth er in ve sti ga tio n s w il l b e

req ui red . U rin e s am pl es a re u su a ll y c o ll ec ted fr om th e mi ds tre am fl o w β€”avo i di n g th e u rin e flo w a t th e be gi n ni n g an d en d o f mi ctu ri ti o n .

Biopsy A bi o ps y i n vo lv es th e rem o va l o f p art or a ll o f a les io n s o th a t it c a n be ex am in e d b y hi sto p ath o lo g ic a l te ch n i qu es .

Ma n y le si o n s ma y b e di ag n o se d o n l y a fte r e xa mi na tion o f a n ap pr op ri ate bi o ps y sp ec i me n o f th e a ffe cte d tis su e . Th is i s so , n o t o n ly i n ca s es o f su s pe cte d n eo p la si a, b u t, fo r e xa mp le ,

al so i n th e di ffe ren tia l di ag n o si s o f w h ite p atc h es th at m a y o c c ur in th e o ra l m u c os a a n d o f th e b ul lo u s , u lc era tiv e, a n d de sq u am a tive l es io n s in th e m o uth . M a ny b o ne c o n diti o n s are , si mi la rly , di ag n o se d by ex am in a tio n o f a bi op s y s a mp le . It is g en er al ly ag ree d th at, i n th e

ca se o f su s pe c ted o r p o ss ib le m al ig na n c y o f th e o ral mu c o sa , b io p sy is m an d ato ry an d , w ith si mp le pr ec au ti on s , is u n l ike ly to c au s e di ss em in a tio n o f tu mo u r ce ll s. Th ere a re se ve ral

me th o d s o f o b tai ni n g bio p s ies a n d the s e w i ll be d ealt w ith u n de r th e fo ll o w in g h ea di ng s : exc is io n a l, i n ci si o n al , an d fi n e n e ed le as p ira tio n . Th e de c is io n to ta ke a n ex ci si o na l o r in ci s io n al b io ps y w ill d ep en d u po n th e n atu re o f the le s io n , its s iz e , an d lo c a tio n . Pri o r to b io p sy th e p a ti en t s h o u ld be i n fo rme d ab o ut:

th e re as o n s fo r th e pro c ed u re

wh a t to e xpe c t

an y di sc o mf o rt

po s si bl e co m pl ic a tio n s

A pa tie nt in fo rm ati o n le af le t is a h el pfu l a id in ob tai n in g in fo rm ed c o n se n t.

Bio p sy t echniq ues

In c is io n a l

Ex ci si o n al

Fi ne n e ed le as p ira tio n

Excisional biopsy

If th e les i on in q u es tio n i s sm al l, i t m ay be b es t to rem o ve it en tire ly by l oc a l e xc is io n , in cl u di n g a s m al l a re a of n o rm al tis s u e. Th e sp ec imen ma y th e n be s ec tio n e d a n d its

hi sto l o gy rev ie we d to de term in e w he th er fu rth er trea tme n t w il l b e ne ed ed . Th e bi o ps y i s fa r bette r ta ke n w ith th e k n ife th an wi th th e cu ttin g di a th e rm y, w h ic h m ay ca u s e c o n s ide ra bl e dis to rtio n o f th e ti ss u es . A fte r i ts r em o va l, th e bi op s y s pe c im en s h o ul d be pl ac e d w i th the

mi ni mu m o f de la y i n to a fix ati ve , 10 pe r c en t fo rm ol s al in e be in g th e m o s t u n ive rs al ly u se d. Fu ll c li n ic al d eta ils s h o u ld a lw ay s be g ive n to th e p a th o l og is t w h o i s to exa m in e th e sp ec im en .

Ex ci si o n bi o ps y i s pa rtic u la rl y u s ef ul fo r th e d ia gn o s is o f si n gl e s m al l ul ce rs a n d sm a ll ,

lo ca l iz ed , s of t-ti ss u e sw e ll in gs . In th es e c as es i t is p o ss i ble to co m bi n e pri ma ry trea tme n t wi th bi o ps y.

Incisional biopsy

Th is is th e re mo va l o f a s ec tio n o f a le si o n fo r h isto l o gic a l stu dy w ith o u t a n y a ttem pt b ei n g

ma de to re mo v e th e w ho l e of th e le si o n. In ta ki n g su c h a bi o ps y of th e or al s o ft tis s u es , th e ai m sh o u l d b e to in c lu de w ith i n o n e s p ec im en , i f p oss ib le , a cl in i ca ll y ty pi ca l are a o f th e les io n an d a ls o th e e dg e o f th e le si o n. If th e ch o ic e m us t b e ma de be tw een the tw o

po s si bi liti es , th e cl in ic a lly typ ic a l a re a sh o u ld be c h o se n β€”a la rg e a re a of n o rm al tis s ue bey o n d th e le si o n is q u ite u n ne c es sa ry. Th e s p ec im en sh o u l d b e bi g e n o ug h to a ll o w the

pa tho l o gi st to ma ke a di ag n o si s, as to o s ma l l a bi ops y i s di ffi cu l t to h a nd le a n d to or ien ta te fo r s ec tio n in g .

Th e te ch n i qu e fo r b io p sy o f a l es io n of th e or al epith e liu m i s to ma ke a w ed ge -sh a pe d c u t in to th e c h o s en a rea , to c o mp le te th e tri an g le by a th ird c u t, an d to th en tak e o f f th e

epi th el ia l l ay er, to g eth er w ith a th ic kn e ss o f c or iu m, by s lid in g th e kn if e b el o w an d p ara ll el

wi th th e s u rfa c e. E ve n if i t is th e ep ith el iu m th at is o f pa rtic u la r i n te re st, i t is e ss e nti al th a t a s u ff ic ie nt la ye r o f co ri u m sh o u ld b e i n cl u de d i n o rde r th a t th e su b ep ith el ia l r ea cti o ns m a y

be se en . If th e b io p sy is o f a lu m p, th e n th e w e dg e s e cti on mu s t b e ta ke n in to th e s w el li ng , ma ki ng s u re tha t a n y c a ps u la r ti ss u e is c u t th ro ug h a n d th a t a rep re se n ta tiv e are a o f th e les io n pro p er i s o bta in e d.

An a es th es ia f or th e b io p sy s h ou l d be o bta in e d b y the in je cti o n o f l o ca l an a es th eti c as f ar

fro m th e b io p sy s ite as c o n si s te n t w ith o b tai n in g a sa tis fa c to ry re s ul t. It is c l ea rly u n wi se to in jec t d ire ctl y i n to an are a o f do u b tf u l m a lig n an c y an d , qu i te a pa rt f ro m an y qu e sti o n o f

dis s em in a tio n o f n eo p la sti c ce ll s, th e re i s a da n ger o f di sto rti o n o f th e h is to lo gi ca l pi ctu re i f th e a rea i s in fi ltra ted b y a n ae sth e tic . Th e bi op s y s ite m ay be c lo s ed b y o n e o r tw o su tu re s.

P. 20

If th e sp ec im en is a th in o n e , as i s o ften th e c as e w ith bi o ps ie s o f th e o ral m u co s a, it i s o ften mo s t c on v en ie n t to la y it f la t o n a pi ec e o f c ar d or a sw a b be fo re pl ac in g i nto th e fi xa tive . Th e ti ss u e pra c tic al ly al w ay s ad h ere s to th is b ac king , a n d c u rli n g an d di sto rti o n o f th e

sp ec im en i s pre ve n te d. Mu l ti pl e bio p s ies m a y b e requ ire d o f la rg e m u c os a l l es io n s o r in ca s es wi th w id es pre ad o ra l le si o ns th a t a re cl in ic a ll y d is si mi la r.

Fro z e n se cti o n s fo r ra pi d di ag n o si s are ra re ly req uire d i n o ra l me di ci n e. V e ry o f te n th e

hi sto l o gic a l se cti o ns r eq ui re ca re fu l an d de tai le d stu d y, th is b ei n g d if fic u lt wi th fro z e n tis su e

sp ec im en s . Ra pi d rep or tin g o f fro z en se cti o n s in the u rge nt si tu ati on is p os s ib le , fo r i n sta n ce , du rin g su rg er y w h en it i s es se n tia l to ide n tify m alig n an c y, b u t thi s is n o t a s itu a tio n rel ev an t to the re gu l ar pra c tic e o f o ra l m ed ic in e . Th e ma jo r u s e o f fr o ze n se c tio n s in o ra l me di ci n e i s in i mm u no f lu o res c en t d ia gn o s tic tec h n iq ue s .

Biopsy for immunofluorescence

An tig en β €“an tib o dy c om p lex es i n tis su e s ca n be i denti fie d by va rio u s s tai n in g an d la b ell in g

te c hn i qu e s. F lu o re sc e in i s a co m m on l y u s ed l ab el . Dire ct im mu n o fl u or es ce n t s tud ie s o f b io p sy ma teri al h a ve be co m e an es se n tia l p a rt o f th e di a gno sti c pro c ed u re fo r i mm u n o bu ll o u s or al les io n s (a s d is cu s se d ea rli er in th i s ch a pte r a n d in th e ap pro p ria te la ter ch a pte rs ). In th e

ca se o f n o n- bu ll o us a n d n o n- ero s ive l es io n s th ere are n o pa rtic u la r p ro bl em s . Th e sp ec im en

is tak en f ro m the l es io n a l ti ss u e w ith s o me ma rg in al c li n ic al ly n o rma l tis s ue i f c o n ven i en t. In th e c as e o f b u ll ou s o r ero s iv e les io n s , h o w eve r, the si tua tio n is qu i te d if fer en t i n tha t th e mo s t ch a ra cte ris tic i mm u n o lo gi c al fi n din g s ar e l ikely to b e i n th e cl in ic a ll y n o rm al tis su e

ad jac en t to the l es io n . Wh e n bu l la fo rm a ti o n o r e rosi o n h as o c c u rred , th e bi o ps y te ch n i qu e o f

th e le s io n i ts e lf be co m es v ery di ffi cu l t a nd th e res u lts (la rg el y b ec au s e o f s ec o n da ry in fe c ti o n an d si m ila r f ac to rs ) b ec o me m u ch m o re di ffi cu l t to in te rpre t.

It is e ss en ti al w h en ta ki ng b io p sy sp ec i me ns f o r i mm u no f lu o res c en t s tu di es th a t th e

la bo ra to ry s h o u ld be p re-wa rn ed so tha t th e fre sh , u n fi xe d ti ss u e is p as s ed o n d ire ctl y f or im me di ate pro c e ss in g (o r f o r de ep f ro ze n s to rag e). It ca n b e s a fe ly tr an s po rte d in a

po ly the n e ba g o r pl ac e d o n a n i ce tra y. P ri or to takin g a bi o ps y th e p rac titi o ne r s h o u ld de c ide wh a t typ e of b io ps y is re qu ir ed a nd w h a t is g o in g to h ap pe n to th e bi op s y sp ec i me n. Th is w il l pre ven t u n n e ce ss a ry r ep ea t bi o ps ie s.

Bo th d ire c t an d in d ire c t im mu n o fl u or es ce n t s tud ie s ar e o f va lu e in th e di ag n o si s o f im mu n o bu l lo u s di se as e s (fo r e xa mp le , pe mp h ig u s an d pe mp h ig o id) .

Fine needle aspiration (FNA) biopsy

So ft- ti ss u e le si o n s ca n b e c o ll ec ted fo r m ic ro s c op ic e xa mi n ati o n u si n g a n e ed le as p ira te. T hi s te c hn i qu e is a ls o u se fu l fo r c o ll ec tin g fl u id co n tents o f a le si o n , pa rtic u la rly p us o r cy sti c

flu i d. A 20/21 ga u ge n ee dl e i s em pl o yed f or s am pl ing tis su e a n d u l tras o u n d ca n be u s ed to

gu id e th e p os i ti o n in g o f th e n ee dl e i n a n atte mp t to e ns u re th at th e bi o ps y i s tak en f ro m the ce n tr e o f th e l es io n . S o me tim es i t is n o t p o ss i ble to o bta in a d ef in iti ve di ag n o si s fro m a F NA

bio p sy . O ften th e re is en o u g h in fo rm a tio n to d iff ere n tia te b etw e en m al ig n an t a n d be n ign . A n exp eri en c ed cy to lo gi s t is re qu i red to in te rpre t th e sa m pl e a n d it s h o u ld be re me mb ere d th at th e s pe ci me n h a rves te d m a y n o t a lw a ys b e re pr es en tative o f th e le si o n .

Microbiological investigations

No t al l or o fac i al in f ec tio n s req u ire th e se rvi c es of a d ia g no s tic m ic ro bi o lo g y l ab o rato ry .

Ho w eve r, w h en s u c h se rvi ce s ar e r equ i red , th e cl in ic ia n n e ed s to be a wa re o f th e ra n ge o f

se rvi ce s av ai la bl e an d th e type o f sp ec im e n req u ired. La bo ra to rie s c an l o o k f or th e f o ll ow i n g evi de n ce o f in fe cti on . 1.

Vi ab le o rg an is m s. Mi cro - or ga n is ms c a n so m eti me s be s ee n mi c ro sc o pi ca ll y f ro m a di rec t

sm ea r b u t u su a ll y th e s pe ci me n is c u ltu re d. T hi s w ill th en al lo w th e o rga n is ms i n th e cu ltu re to b e ide n tifi ed a n d u n d erg o se n si tiv ity tes ti n g to an ti mi cro b ia l ag en ts . Fo r

cu ltu re a n d s e ns i ti vi ty tes tin g a n as p ira te o f pu s i s pre fe rab le to a s w ab . Th e la tter

ma y b e co n ta mi n ate d wi th n o rma l o ral fl o ra a nd th e pu ta tive p ath o ge n s ar e l es s li kel y to su rv ive th e jo ur ne y to the l ab o rato ry . 2.

Mi cro b ia l pro d u cts . It is p o ss i ble to d ete ct the p res en c e o f mi c ro -or ga n is ms b y th e

pro d uc ts th at th e y p ro du c e, su c h a s to x in s , o r b y id en tif yin g th ei r DNA . T he a pp li ca tio n

of m o lec u la r te c hn i qu es to ide n tify ge n etic m a teri al h a s al lo w ed fo r the i de nti fi ca tio n o f mi cro -or ga n is ms w ith o u t th e n e ed fo r cu l tu re . Ge n e a m pl ifi ca tio n u s in g th e po ly me ra se ch a in re a cti on (P CR) an d in s itu h ybr id iz ati o n tec h n iq ue s a llo w s fo r th e ra pi d

ide n tifi ca tio n of o rg an i sm s. Th is is p a rtic u la rly be n efi c ia l f o r o rga n is m s th a t a re ha z a rdo u s o r n o t e as il y g ro w n in th e l ab o rato ry . Hep a titis C i s id en tif ie d i n th is

ma n ne r. 3.

An tib o dy de tec tio n . Th e pr es en c e o f c irc u la tin g an ti bo d ies i n th e se ru m, c e reb ro sp in a l

flu i d (C S F), or in s a li va m ay be in d ic a tive o f in fecti o n. S e ro lo g ic al tec h n iq ue s ar e o f ten em plo y ed fo r vir al in f ec tio n s su c h a s h ep ati ti s B .

Bacteriology

If a b ac teri al a eti ol o gy is s u s pe cte d fo r a l es io n , b o th di rec t s me a rs an d s wa b s fo r c u ltu re an d id en tif ic ati o n ma y be tak en . Di rec t s me ar s fro m th e g in g iva l cr evi ce m a y b e o f s o me val u e in th e id en tif ic ati o n o f th e fu s o ba cte ria a n d sp iro c h ae tes i n a cu te u lc era tiv e

gin g iv itis β€” al th o u g h th eir u se i s lim ite d. In ma n y i n sta n ce s o n ly no rm a l o ra l fl or a wi ll be

rep or ted. Th is i s th e c as e , fo r e xa mp le , in v ira l in fe cti o ns . In th es e ci rcu m s ta n c es , h o we ve r,

as i n ma n y o th e r o ra l m u c o sa l dis e as es , th e ba la n c e o f th e o ra l fl or a is s o on di stu rb ed by th e on s et o f a bn o rm al en vi ro n me n tal c o nd iti o ns .

Sp ecimens for the ident ificatio n o f micro -organisms

Wh en a c o ll ec tio n o f pu s is p res e nt an as pi ra te s h ou ld id ea ll y b e tak en . P. 21

Mycology

Can d id a ma y be rec o gn i z ed o n di rec t s m ea rs , by cu l tur e, a nd, i f a n e sti ma te of d en s ity of

or ga n is ms i s req u ire d, b y i mp rin t c u ltu re o r a n o ral rin s e. T he y su rv ive w el l on a dry s w ab o r wh e n pl ac e d i n a n ap pr op ria te tra ns p or t me di u m. It m u s t be rem em b ere d th a t i n so m e fo rm s of c an d id o si s, w h e re th e o rga n is m s are w i th i n th e tis su e s (a s in c h ro n ic h yp erp la s ti c

ca n di do s is ; s ee Ch ap ters 4 an d 6), th ere m ay be ve ry littl e gro w th fro m a sw a b. Th ey ar e bes t i de n tifi ed by h is to lo g ic al m eth o ds . S u sc e ptib il ity tes tin g to va rio u s a n tifu n g al a ge nts ca n be u n de rta ken , b u t th e rep ro du c ib ili ty o f s om e tes ts, pa rtic u la rly th o se re la tin g to az o le s, are q ue s tio n ab le .

Virology

Vi ru s id en tif ic ati o n rem a in s a le n gth y a n d rel ati vely di ffi cu l t pro c es s . Co n fi rma tio n m a y b e

giv en b y dir ec t el ec tro n mi c ro sc o py in th e fe w c en tres w h e re th i s is a va il ab le . Tis s u e c u ltu re , an tig en de tec tio n , a n d i de n ti fi ca tio n o f ge n etic m ateri al a re co m mo n l y e mp lo ye d tec h n iq ue s .

Se ru m an tib o dy s tud ie s ca n a ls o f or m th e b as is f o r th e d ia gn o s is o f vi ral in f ec tio n s . In

he rpe s s im pl ex the b as el in e le ve l o f an ti bo d ies i n a n y i nd iv id ua l be fo re c lin i ca l in fe c ti o n is

var ia bl e, d ep en di n g on th e p a st h i sto ry a nd th e de gre e o f th e im mu n e res p o ns e . At the tim e of a n a cti ve cl in i ca l in fe cti o n th es e l ev el s are rais e d c o n si de ra bly . If p a irs o f se ra fro m th e

pa tien t, ta ke n at an in terv al (o f ab o u t 10 d ay s in th is c a se ), c an be co m pa re d, a s ig n ifi c an t

ris e in titre c o n fir ms th e di ag n o si s. Q u ite c le arl y, th is is n o t a p ar tic ul a rly u se fu l tec h ni qu e , exc ep t i n retro s pe c t, si n ce , in th e ca s e of p rim ar y h e rpe tic s tom a titis , th e le si o ns w i ll h av e go n e in to re mi ss io n be fo re th e c o n fi rma tio n o f th e d ia g no s is i s av ai la bl e.

Ba c teri a an d vi ru se s ca n b e ide n tifi ed ra pi dl y u s ing m o lec u la r tec h n iq ue s

su c h a s po ly me ra se c ha i n rea c tio n (P CR) o r f lu o res cen t in s itu h ybr id iz ati o n (FIS H).

Imaging techniques

Pl ai n fi lm ra di og ra ph y is th e mo s t c o mm o n im a gi ng te ch n iq u e us e d i n d en tal p rac tic e . Th e

var io u s vi ew s ar e s h o w n in Tab le 2. 7 . Th e v al u e of pl ai n ra di o gra ph y li es i n th e s im pl ic ity o f th e m eth o d an d a ls o th e f ac t th a t it is w id el y a va ila bl e. Th e di s ad va n ta g es a re tha t i o ni z in g rad ia tio n i s u se d a n d im ag in g o f so ft - ti ss u e le si o ns is o fte n u ns a tis fa cto ry fo r mo s t

dia g no s tic pu rp o se s. In are a s of c o mp le x b o n y a n atom y th e s up er im po s itio n o f a dja ce n t

stru c tu res o n th e reg io n o f in ter es t c an o f ten l im it th e di ag n o sti c va lu e o f th e im ag e. Th is

pro b lem i s red u ce d by th e te ch n i qu e o f to m og ra ph y whi c h u se s mo v em en t o f th e X -ray tu be an d fi lm to p ro du c e a s li ce th ro u gh the p ati en t w ith bl u rrin g o f th e n ei gh b o ur in g stru c tu res . Th e d en ta l pa n or am ic to m o gra ph ( DPT ) o r pa n o ra mi c vie w u se s th is p rin c ip le.

Table 2.7 Radiographic views Ext ra o ral v iew s Pa n o ram ic ( de nta l pa n o ram ic to mo g rap h , DP T) Late ral vi ew s

O b liq u e la tera l T ru e la tera l

In tr ao r al v iew s Pe ria pi c al Bi tew in g Oc cl u sa l

Oc ci pi to me n ta l

Po s teri o rβ€“ an ter io r ma n di bl e Su b me n to v erte x

Wh en i ma gi n g b o n y s w el li ng s , two vie w s sh o u ld b e take n th at are a t ri gh t an g le s to ea c h oth e r.

Di gi tal ra di og ra ph y u se s co n v en tio n a l ra d io gr ap h ic tec h n iq u es , bu t th e fi lm is rep la c ed w ith a se n so r th a t tra n sm its th e im a ge to a c om p ute r. Th e se s ys tem s al lo w s o m e m a ni pu l ati on o f im ag e to be c arri ed o u t, w h ic h c an e n h an c e th e im a ge qu a li ty. Wh il s t th e e qu i pm en t i s

exp en s iv e, th is te ch n iq u e ha s g ro wn in po p u la rity over rec e nt yea rs . Th e a dva n tag es i n cl u de th e u s e o f a lo w er do s e o f ra di ati on th a n fo r co n v en tio n a l f il ms , th e e li mi n ati o n o f f ilm

pro c es si n g, a n d th e i mm ed ia te vis u a li za tio n o f a n im ag e o n a vis u a l d is pl a y u n it. Tab le 2. 8 lis ts fu rth er s pe ci al iz ed i ma gi n g te c hn i qu e s th a t may be u s efu l in or al m ed ic in e.

Co n tr as t stu d ie s . Th e a p pe ara n c e o f c erta in s o ft-ti ss u e str uc tu res v is u al iz ed b y u si n g i o n iz in g rad ia tio n c a n be e nh a n ce d by al teri n g th e ra di o de nsi ty o f a pa tie n t's tis su e s by th e

in tro du c tio n o f co n tra st me di a. T h e te mp o ro ma n di bu la r jo in t s pa c e an d s al iva ry gl an d s are

pa rtic u la rly a me n ab le to th is te ch n iq u e. Th e im ag ing o f the s e s tru c tu re s w il l b e dis c u ss ed i n gre ate r d eta il in th e ap pr op ri ate c ha p te rs .

Co n tr as t s tu di es a re su c ce s sf ul i n im ag in g :

sa li va ry g la n ds (s ia lo g rap h y)

joi n ts (ar th ro g ra ph y)

blo o d ve ss e ls (a n gi og ra ph y)

ga stro i n te s ti n al tra c t (ba ri um s w a llo w , m ea l, a n d en e ma )

Rad io i so to pe stu d ie s . Cer ta in tis su e s wi ll p refe re nti al ly c on c en tra te sp ec if ic co m po u n d s. T hi s ca n be e xp lo ite d f o r i ma gi n g i f th e c h em ic al i s la be lle d wi th a ra di oa c tive c o mp o u nd . Th i s te c hn i qu e w ill a ll o w fu n cti o n al s tud ie s to be c ar rie d o u t o ver tim e. F o r e xa mp le s a liv ary

fu n cti on of th e ma jo r g la n ds c a n be a ss es s ed by th ei r e ntra p me n t an d re le as e o f te ch n eti u m ( 9 9 m Tc) w h ic h is ca rri ed i n io n ic f or m as p erte ch n e ta te. Th is ra di o is o top e em its g am ma r ay s

bu t th e s h o rt h a lf -lif e o f

99m

Tc (6. 5 h o u rs) mi n im iz es

th e e xpo s u re o f th e pa tie n t to ra di ati o n. Bo n e m ay al s o be in v es tig ate d i n a s im il ar ma n n er

P. 22

us in g m eth yl en e di ph o s ph o n ate a s th e c a rrie r f or the r ad io is o to pe . In th is ma n n er the

ac tivi ty o f bo n e tu mo u rs a n d ma n di bu la r c o n dy la r g ro w th an d in f ec tio n ma y be s tud ie d. T he

dis a dv an ta ge o f r ad io is o to pe s tu die s is th a t th ey su b jec t th e w h o le b o dy to ra dia tio n an d a re ti me -co n s um in g to p erf o rm.

Table 2.8 Specialized imaging techniques Utiliz ing io niz in g r ad iatio n

No t u s ing io niz in g r ad iatio n

Digi tal ra di o gra p hy

Ul tr as o u nd

Co n tr as t stu d ie s

Ma gn e tic re so n a n ce im a gi ng (M RI)

Rad io i so to pe s tu di es

Co mp u teri ze d to mo g rap h y (CT )

Co mp u teri ze d to mo gr ap h y (CT ) u s es a so p h is tic ate d X -ray m ac h in e w ith a ri ng o f X - ray

dete c to rs a ro u n d the p ati en t. A co m pu te r th en ge ne ra tes a n im a ge th at r ep res en ts a s li ce

th ro u g h th e p a ti en t. M an ip u la tio n o f th e d a ta b y com pu ter a llo w s th e im ag e to be a dju s ted to hi gh l igh t bo n e or s of t tis s u e. Co m pu te riz e d to m o graph y is o f va lu e w h en in ve s ti ga tin g

in trac ra n ia l les i on s , s o ft- an d h a rd -ti ss u e tu mo u rs o f th e h ea d an d n ec k, fa ci al fra c tu res , a nd os teo m ye li ti s . It w ill s h o w if th ere is in vo l ve me nt o f a dja c en t s tru c tu re s . CT sc a ns a re

exp en s iv e a n d u se h ig h do s es o f ra di ati on . M e ta l lic o b jec ts ca n c a us e s trea k a rte fa cts th a t ob s cu re in f or ma tio n . Th u s, extra - an d i ntra c o ro n al re sto ra tio n s a nd i mp la n ts ca n b e

pro b lem a tic . Ul tr as o u nd u se s a ve ry h ig h - freq u en c y p u ls ed u ltra s o u nd b ea m th at i s em itte d f ro m a

tr an s du c er tha t i s pl ac e d a ga i ns t th e s kin . Th e s ame tra ns d uc e r pi c ks u p th e re fle c te d be am an d an ec h o pi ctu re im ag e is p ro du c ed . Ul tras o u n d is s af e a n d is u s ef ul fo r ev al u atin g

vas c u la r d is o rde rs a nd s o ft-ti ss u e sw e ll in gs o f th e n ec k a n d fa ce , in c lu d in g sa l iva ry gl an d s

an d ly mp h n o de s. It i s id ea l fo r di ffe ren ti ati ng b etw ee n s o lid a n d cy sti c ma s se s. U ltra s o u nd ca n be u s ed i n co n ju n c tio n w ith f in e n ee dle a s pi rati o n to en s u re co rre c t po s iti on i n g o f th e bio p sy n ee dl e. S i mi la rly , u ltra so u n d ma y be u se d to lo c ate s al iv ary c al cu l i th a t ar e to be bro ke n u p wi th a s ia lo li th o tr ipte r. Th e di s ad va n ta ges o f ul tra so u n d in th e h e ad a n d n e ck reg io n i s th a t i ts u s e is re stri c te d to s up er fic ia l stru c tu res be ca u s e b o n e w i ll a bs o rb th e so u n d wa ve s .

Ma gn e tic re so n a n ce im a gi ng (M RI) u til iz e s the b eh a vio u r of pro to n s in a ma gn etic fi el d to

pro d uc e im a ges . Th i s tec h n iqu e is v al u ab le i n as s ess in g in tra cra n ia l le si o n s, l es io n s o f th e sa li va ry g la n ds , s in u s es , an d ph a ryn x, an d a ls o o f th e tem po ro m a nd ib u la r jo i nt. M RI

pro d uc e s e xc el le n t di ffe ren ti ati o n be twe en s o ft ti ssu e s, h a rd tis su e s, an d n o rma l an d

ab n or ma l tis su e s, bu t g ive s po o r ha rd - ti ss u e de tai l. T hi s im ag in g tec h n iq u e i s mo re tis su e se n si ti ve th an CT a n d do e s n ot us e io n i zi n g r ad ia tio n . M RI i s exp en s iv e an d i s

co n tra in di ca te d i n pa tie n ts wi th c erta in ty pe s of su rgi ca l cl ip s, c a rdi a c pa c em ak ers , a n d

co c h lea r im pl an ts (w h ic h c o nta in ma gn e tic el em en ts ). P a ti en ts w ho h av e c la u s tro ph o bi a are

al so u n s u ita bl e f o r M RI b ec au s e th ey are u n li ke ly to en te r in to th e tu n ne l fo rm ed by th e co re of th e ma gn e t.

Si n ce o ral m ed ic in e re pre se n ts th e w h o le fi el d o f me di c in e as re la ted to th e o ral ca vi ty, i t is evi de n t th at a s w id e a ran g e o f in v es tig ati on s m u st be a pp lie d as in o th e r s pe ci al iti es o f

me dic i ne . It w o u ld , qu ite c le ar ly, b e im po s si bl e to ev en b rie fl y o u tli n e th e se in th e p res en t

co n tex t. Th e ma jo r i nv es tig ati ve pro c ed u res h a ve b een o utl in ed a bo v e. O th ers a re di sc u s se d in th e ap pro p ria te la ter ch a pte rs .

Diagnosis

Th e d ia gn o s is m a y b e o b vi ou s f ro m th e h is to ry an d ex am in a tio n o f th e pa tie n t. Fu rth e r in ve sti ga tio n s ma y n ot be re qu ire d an d th e cl in ic i an m a y m ak e a def in iti ve di ag n o si s.

Th e c li n ic ia n ma y h av e a s tro n g su s pi ci o n o f th e diag n o si s bu t c a n no t co n fi rm th is u n til fu rth er i n fo rm ati o n is re tu rn ed , su c h a s a bi op s y or blo o d re su l ts. In th i s si tu ati o n the

pra cti tio n er ca n m a ke a pro vi si o n al , cl in i ca l, or wo rki n g di ag n o si s an d m ay s tart th era py fo r

th e s u sp ec ted c o nd iti o n. It is n o t u n co m m on , h o w ev er, th a t a fter ex am in a tio n th er e re m ai n s mo re th an on e p os s ib le di ag n o si s. Th es e co n d itio n s ma ke u p th e dif fe ren tia l di ag n o se s. In

th i s si tu ati on th e c li n ic ia n n ee ds to a s se s s th e l ike li ho o d o f ea ch dif fe ren tia l di ag n o si s, tak in g in to a cc o u n t the p ati en t's a ge , ge n de r, a nd ra c e, th e pre s en tin g sy mp to ms , th e me di ca l an d

dru g h is to ry, a n d th e c la ss ic a l fea tu res o f th e va rio u s p os s ib le di a gn o se s . Th is i s a sk ill th a t bec o m es d efi n ed w ith e xp eri en c e an d re qu i res l og ic al th o u gh t a n d kn o w le dg e o f th e v ar io u s co n di tio n s .

Projects 1. 2.

Id en ti fy th e m ed ic al a n d su rg ic a l d is c ip lin e s th at s p ec ia li sts i n o ral m ed ic in e m ay li ai se

cl os e ly wi th an d g ive re as o n s w h y th es e rel a ti o n sh ips a re ne c es sa ry .

Th e p a rtic ip ati on o f a n o ra l m ed ic in e s pe ci a lis t i n s om e mu l tid is ci pl in a ry (j oi n t) c li ni c s

ma y b en e fit pa tie nt ma n ag em en t. W ha t jo i n t cl in i cs d o yo u th i nk w o u ld be a dv is ab le an d li st th e o ro fa c ia l c o n di tio n s th at co u l d b e ma na ged o n th e se cl in i cs ?

Au th o rs : Title:



Co py rig ht Β © 2003 Ox fo rd U ni ve rsi ty P re ss > T ab le o f Co nt e nt s > 3 - Th e ra p y

3 Therapy Principles of therapy

In so m e les i on s o f th e o ra l mu c o sa , s pe ci fi c trea tme n t is d ire ctl y i n di ca ted b y th e di ag n o si s. Fo r in sta n c e, i nf ec tio n s by a n id en tif ia bl e o r ga n ism ma y b e trea ted b y th e u se o f an

ap pro p ria te an tib io ti c. In ma n y or al le s io n s su c h ra tio n a l th e rap y is n o t, h ow e ver , av ai la bl e, of ten be ca u s e o f a la c k o f kn o w le dg e o f th e f ac to rs c au s in g th e c o n di tio n , a n d s o i t is

ne c es sa ry to fa ll ba c k o n to no n -sp ec if ic trea tm en t m o da li ties , s u ch as to pi ca l an tib io ti cs , an tis ep tic s , lo c al a n d s ys tem ic c o rtic o s tero i ds , and im mu n o m o du la tin g dr ug s .

Topical therapy

To pi ca l the ra py h as s ev era l ad va n tag es o ve r th e s yste m ic a dm in is tra tio n o f d ru gs . S id e eff ec ts, a lth o u gh n ot el im in ate d, a re u s ua ll y red u ce d a n d lo c al ap pl ic a tio n a ll ow s f or

ma xi mu m c on c en tra tio n o f th e d ru g at the s ite o f the le si o n . Ag en ts ten d to b e mo re eff ec tive i f th ey c an be reta in e d lo c al ly fo r a s lon g as p o ss i ble .

Covering agents

A nu m be r o f ge ls a n d pa ste s ar e a va il ab le to p ro vide me c ha n ic a l p ro tec tio n o f u lc era tiv e

are as i n th e mo u th . A gen ts s u ch as ca rm el lo s e gel a ti n g el ma y al so be u se d as c a rrie rs fo r mo re a cti ve su b sta n ce s su c h a s ste ro id s o r a n tifu n gal a ge nts . A n u mb er o f p rep a rati o n s o f th i s kin d a re co m m erc ia ll y a va il a ble a n d oth e rs ca n b e ma de u p by a ph a rm ac is t.

Un fo rtu n a te ly , pa tie n ts of ten re po rt di ffi cu l ty a pplyi n g th e se p as tes , p arti cu l ar ly at th e b ac k of th e mo u th .

Topical antiseptics

Ch lo rh ex id in e h as a n tib ac teri a l a n d so m e an ti ca n di da l ac tiv ity an d is c u rre ntl y th e m os t eff ec tive a n tip la qu e ag en t. Ch lo rh e xi din e c an be u se d as a m o u thw a s h, s p ray , o r g el to co n tro l se co n d ary in fe c ti o n in mu c o sa l ul c era tio n an d as a n a dju n c t, or s ho rt -te rm

al te rn a tive , to to o th b ru sh i n g a n d o the r o ra l h ygi ene m ea su re s. Ho w eve r, it fre qu en tl y s ta in s te eth b ro w n a nd m a y c au s e di sc o lo ra tio n o f th e to n gu e. S tai n in g o f th e tee th is rev ers ib le bu t c an be a pro b le m wi th lo n g -te rm u se . S o me p ati en ts do n o t l ik e th e ta s te o f

ch lo rh e xi din e a n d o c c as io n a ll y i t c au s es i di os yn c ratic m u co s al irr itati o n . Pa ro tid s w el lin g h a s bee n re po rte d i n a fe w c as es f o llo w i ng u s e of ch l o rh exi di n e.

Ch lo rh ex id in e h as a n tib ac teri a l a n d an tic a n di da l a ctivi ty a n d is cu rr en tly th e m o s t ef fec tiv e an tip la qu e a ge n t.

Topical analgesics

A to p ic a l a n a lge s ic pr ep ara tio n su c h a s be n zy da m in e (Dif fla m Β ® ) ma y p ro vi de tem po ra ry

sym p tom a tic re lie f fo r ero s iv e an d u lc era tiv e o r al le si o n s an d en a bl e the p ati en t to e at an d

dri nk . To pi c al a na e sth eti c ag en ts s u ch as l ido c a in e ge l ( o r ri ns e ) c an al so b e u se d fo r sh o rt -

te rm s ym pto ma tic r eli ef bu t sh o u ld b e p res c rib ed w ith c a u tio n s in c e se c o nd a ry tra u ma c o u ld

ea si ly be p ai nl es s ly ef fec ted d u rin g th e p eri o d of an a es th es ia . A f ur th e r p rec a u ti o n to b e

ta ke n in c lu d es th e av o id an c e of pre pa ra tio n s o f s u ff ic ie nt stre n gth to a ff ec t th e la ryn ge a l refl ex es . Lid o ca in e rin s es s h o u ld n o t be u s ed fo r lo n g pe rio ds o f tim e be ca u s e o f th e

po s si bi lity o f sy ste mi c a bs o rpti on an d s id e -eff ec ts. In s p ite o f th e se di ff ic ul tie s, i t i s, fro m ti me to ti me , ju sti fia b le to pr es cr ibe tre atm en t o f th is ki n d. Lo c a l an a es th eti cs a re a ls o

in cl u de d in s o me th ro a t lo z en g es a n d m o u th u lc er pas till es (o r p as te ) o n s al e to the p u bl ic . Ma n y c o n tai n s ug a r, h o we ve r, a nd p ati en ts sh o u l d b e wa rn e d a b ou t th e ir ca rio g en i c

po ten tia l . Th e us e o f a n a es the tic mo u th w as h es i s furth er di sc u s se d in Ch ap ter 5 (β € Ora l

ul ce ra tio n β€™).

Topical antibiotics

Th ere a re in h ere n t di sa d va nta ge s a ss o ci a te d w ith the u s e o f to pi c al a nti bi o tic s be ca u se o f

th e p os s ib il itie s o f s el ec tio n f o r res i sta n t str ai ns a n d o f i n du c in g h ype rs en s itiv ity rea c tio n s in th e p ati en t. Th e va lu e o f to p ic al a n tibi o tic s o u tw eig h s s u c h ris ks i n s o me c as es . Te tra cy cl in e (o r c h lo rtetra c yc li ne ) i s a u se fu l top ic a l a n tib io tic . A s a 2 p er ce n t so l u tio n , it i s o ften

eff ec tive i n red u ci n g s e co n d ary in f ec tio n (a n d thu s th e di sc o m fo rt) in c a se s o f a p hth o u s

sto m ati ti s , pri ma ry h erp etic s to m ati ti s , ero s iv e l ic h en pla n u s, an d o th er s e ver e u l ce ra ti ve co n di tio n s . Inte res tin g ly , (c h lo r)tetr ac yc li n e m o u thw a s he s a re p a rtic u la rly ef fec tiv e in

red uc i ng th e di sc o m fo rt o f h erp etif o rm ap h tho u s s toma titi s (se e Ch ap ter 5 ). Us e o f to p ic al

an tib io tic s c an n o t n o rm al ly be a c ce pte d a s lo n g -te rm trea tme n t fo r rec u rre nt co n d itio n s a n d it i s be st r eg ard ed a s trea tme n t re se rve d fo r a c u te e pi so d es . Th e m ou th w as h m a y b e ma de

by the p ati en t d is so l vi ng th e co n te nts o f a 250 m g te tra cy cl in e ca p su le i n 10 m l o f w ate r to

giv e a 2 pe r c en t so l u tio n . It m ay be m o re eff ec tive to h a ve th e so l uti o n ac c u rate ly ma d e u p by th e p h ar ma ci s t, in cl u di n g 10 pe r c en t o f gl yc erol a s a dem u lc e nt (se e Ap pe n di x ).

If th e trea tme n t is n o t u n du l y pro l o n ged , th ere i s mi n im al tro u bl e f ro m o ver gro w th o f

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res is tan t or ga n is ms i n th e m o u th, al tho u g h a ca n di da l in fe c ti o n m ay o cc u r a n d mu s t b e ap pro p ria tel y de al t w ith .

Ma n y o f th e a u th o rsβ €™ pa tie nts h a ve o ra l l es io n s th at a re p ers is ten t a n d se ve re. In s u c h

ca se s , the p ro lo n ge d u se o f an ti bi oti c -ba se d mo u th was h es i s cl in ic a ll y ju s tifi ed , pa rtic u la rl y in th e ch l o rtetra c yc lin e β€“tr ia mc in o lo n e c o mb in a tion (s ee Ap pe n di x) th at is u s ed in s u c h co n di tio n s a s pe mp hi gu s a n d ma jo r ero s iv e lic h en pla n u s.

Tetra c yc lin e m o uth w a sh m a y b e ma de b y th e pa tie n t di s so l vin g th e co n te nts o f o n e 250 mg ca ps u le in 10 m l o f w ate r to g ive a 2 p er c en t so l utio n β €”us e as a m o u th wa s h , thr ee tim es da ily .

Topical corticosteroids

On e o f th e mo s t i mp o rtan t fa cto rs to b e co n s id ere d wh e n u s in g top ic a l s ter oi ds i s th e d eg ree of su p pre s si o n o f a dr en a l f un c tio n th a t m ay o cc u r wh e n th es e d ru gs a re ad m in is tere d. Th e deg re e o f a dre na l s up pr es si o n va rie s n o t o n ly fro m s te ro id to ste ro id a n d a c co rd in g to th e me th o d o f us e , bu t th er e i s al s o co n s id era b le in d ivi du a l var ia tio n . F or in s tan c e, a d o s e o f

sys te mi c pre dn is o lo n e th at ma y ap pa re n tl y ca u se n o s id e -eff ec ts in o n e pa tie n t ma y ren d er

an o th er ma rke dl y Cu s h in go i d. It i s th e a u th or s β€™ pra c ti c e to u s e h ig h -co n c en tra tio n , lo c al ly ap pl ied s tero i ds to re pl a ce s ys tem ic m ed ic ati o n w herev er po s si bl e. E x ce ss iv e us e o f to p ic al ste ro id pre pa ra tio n s c an , h o w ev er, re s ul t in a si gnif ic a nt am o u nt o f sy ste mi c a bs o rpti on .

Ap pl ic ati o n o f m o re po te nt top ic a l s ter oi ds a ls o i ncre a se s th e l ik eli h o od o f a su p eri mp o s ed or al c an d id ia si s an d s o me o ra l p h ys ic ia n s ad vo c ate th e co n c o mi tan t u s e o f a p ro ph yl ac tic , to p ic a l a n tifu n g al ag en t.

Steroid mouthwashes

Ste ro id m ou th w a sh e s c a n be m a de by th e p a tien t di sso lv in g so l u bl e b eta me th as o n e or

pre dn is o lo n e tab le ts in 10β €“15 ml o f wa ter . S te ro id m o u th w a sh e s sh o u l d n o t b e sw a ll ow e d an d pa tie n ts sh o u ld b e m o n ito re d f o r si de -eff ec ts, a s fo r sy ste mi c ste ro id s, b ec a u se o f the ris ks o f sy ste mi c ab s o rptio n .

Al tern a tive ly , a tria mc in o l o ne m o u th w a sh , o f te n i n a ch l o rtetra c ycl in e ba s e ca n b e u s ed , a t a to ta l do s ag e th a t c a n be va ri ed ac c o rdi n g to th e s ev eri ty o f the c o n di ti o n . Mo u th w as h es

co n tai n in g le ve ls o f tria m ci no l o n e v ary in g fro m 0. 75β €“1. 5 mg to 3 β€“6 mg d ai ly ar e m o s t co m mo n ly u se d. A t th e h ig h er e n d o f th is w i de do s e -ran g e th e re is a lm o st c e rtai n to be

si gn if ic an t sy ste mi c ab so rp tio n , a n d p a ti en ts m u st b e ca re fu ll y m o n ito red f o r th e o n s et o f co rti co s tero i d s id e- eff ec ts. T h e c o n ce n trati o n o f ster oi d in th e mo u th w as h c a n be e as il y

var ie d, h o w eve r, a n d s h o u ld be c a ref ul ly titra ted ag a in s t th e c li n ic al re sp o n se (s ee Ap pe n di x fo r fo rm u la tio n ).

Be tam eth a s on e ri ns e : d is so l ve a so l u bl e b eta me th as on e so d iu m ph o s ph a te (0. 5 m g) ta b le t in 10 ml o f H 2 O a n d h o ld in m o u th fo r 3 mi n u te s , be fo re s pitti n g o u t. U se th re e ti me s da il y, i f

req ui red . Do n o t sw a ll o w .

Table 3.1 Examples of steroid sprays that can be used for ulcerative oral lesions Be cl o me tas o n e di pro p io n ate s pra y de liv eri n g a 50 mic ro gra ms m ete red d o se . Bu d es o n id e s pr ay de li ver in g a 100 mi c ro gra ms m etr ed do s e.

Steroid sprays

Ste ro id s pra ys o r i n h al ers s u c h as b ec lo m eth a so n e or bu d es o n ide c a n be u s ed fo r tr ea tin g

on e o r tw o i so l ate d ul c ers o r e ro s io n s, pa rtic u la rly i f th e y o c cu r in th e an te rio r pa rt o f the mo u th w h ic h is a c ce ss i ble (Tab le 3. 1 ).

Intralesional steroids

In tra le s io n al i nje c ti o n s o f de po t p rep a rati o n s o f su ita ble s tero i ds , o fte n u se d by

der ma to lo gi s ts to pro d uc e lo c al iz e d h i gh c o n ce n trati o n s of th e ac tiv e d ru g, m a y b e us e d to

tr ea t o ra l le si o n s. In g en e ral , th ey do no t ap pe ar to be a s ef fec tiv e i n o ra l m e dic i ne pra c tic e an d ar e o n l y o c ca s io n a lly u s ed . Intra l es io n a l s teroi d in jec tio n s h a ve be en u s ed f or trea tin g sw o ll en l ip s in p ati en ts w ith o ro f ac ia l gra n u lo m atos is (O FG ) (s ee Ch ap ter 12).

Steroid pastes

Tria m ci n ol o n e a c eto n id e (0.1 pe r c en t) is a va il ab le in an a d he s ive p as te an d c an b e ap pl ie d

to ap h tho u s u lc e rs 2β€“4 tim es d ai ly . It is m o st eff ec tive ly u se d at n igh t, w h en sa li va ry flo w dec re as e s an d th e pa tie n t is n o t e ati ng . O the r to p ic al s tero i ds (f or ex am pl e, f lu o c in o n id e o r

cl ob eta s o l) m ix ed w ith O ra ba s eΒ ® (equ a l pro p o rtio n s) c an b e u se d fo r is o la ted , u lc era tiv e les io n s , pa rti cu l arl y th o s e in th e an te rio r p a rt o f th e mo u th (s e e Ch ap ter 5).

Steroid lozenges

Loz e n ge s (pe lle ts ) c o nta in i n g 0. 1 pe r c en t h y dro c o rtis o n e c a n be u s ed fo r rec u rre nt ap h tho u s sto m ati ti s (RA S ). Th is s ter oi d is li ke ly to be m o st eff ec tive i f u s ed in the p ro dro m al p h as e o f ul ce ra tio n β€”it s h o ul d be pl a ce d o n (o r n ea r) the ulc e r a n d a ll o w ed to di s so l ve. Th e u se o f

ste ro id lo z en g es i n u lc er- free p eri o ds , a lbe it at a r edu c ed fre qu e nc y, ha s be en rec o mm en d ed by so m e or al ph y si ci a ns f o r RA S . Wh eth e r o r n o t th is re du c es th e o cc u rren c e o f n e w ap h th ae ha s ye t to b e p ro ve n (s ee Ch ap ter 5).

Creams and ointments

Crea m s an d o in tm en t a re u se d as v eh ic le s fo r ac tive in g red ie nts , s u ch a s a n tim ic ro bi al

ag en ts a nd s tero i d p re pa ra ti o n s. T he y ma y b e u se d to trea t a n u mb er o f c o n di tio n s se en in th e o ra l m e dic i ne c li n ic th at af fe ct th e li ps (f o r e xa mp le , an g u la r c h eil iti s) an d p eri o ral ti ss u es .

Crea m s are e mu l si o ns o f o il a n d w a ter an d a re us u a lly w el l a b so rb ed i nto sk in . Ge n era ll y,

cre am s a re co s me tic a lly m o re a c ce pta bl e tha n o i ntm en ts b ec au s e th ey are l es s gre a sy a nd th e ref o re s u ita bl e fo r u s e o n the f ac e. C rea m s us u all y co n ta in p res e rva tive s (fo r exa m pl e, ch lo ro c re so l a nd p ar ab en s ) u n le ss th e ir ac tiv e

in gre di en t h a s s uf fic i en t in tri n si c an ti mi cro b ia l ac tiv ity. P res erv ati ve s ma y c a u se se n si ti z ati o n an d u lti ma tel y a n a ll erg ic c o n tac t derm ati ti s .

Oi ntm en ts a re gre as y pre pa ra tio n s th at are in s o lu ble in w a ter. T he y are p arti cu l arl y us e fu l fo r tre ati n g c h ro n ic , dr y s ki n co n d itio n s a n d are les s ea si ly w as h ed o ff th an cre a ms .

Systemic therapy

Sy ste mi c the ra py w ith n o n -sp ec if ic im mu n o m o du la tin g dr ug s s uc h a s s tero id s a nd

az a thi o pri n e, to ge th er wi th su b s ta n c es s u ch a s th alid o mi de , ar e i n cre a si n gly b ein g u s ed fo r se ver e u lc era tiv e a n d ero s iv e o r al c on d iti on s . Th e se d ru gs h a ve si gn i fic a nt si de -eff ec ts,

ho w e ver, an d pa tie n ts req u ire c are fu l as s es s me nt and m o ni to rin g u n de r h o sp ita l su p erv is io n (se e be lo w ).

Systemic corticosteroids

Th e u s e o f s ys tem ic c o rtic o s te ro id s fo r th e trea tmen t o f or al m uc o s al le s io n s is ju s tifi ab le i f to p ic a l th e rap y h a s fa il ed . P red n is o lo n e h a s pr ed omin a n tly gl u co c o rtic o id a cti vity a nd i s the co rti co s tero i d mo s t c o mm o n ly tak en o ra ll y f o r l on g -te rm di s ea se s u pp res s io n . Or al le s io n s,

su c h as m a jo r a ph th a e o r ero s iv e l ic h en p la n u s, m a y n e ed to b e m a na g ed by a s ho r t (2 β€“ 6 we ek ) re du c in g co u rs e o f p red n is o lo n e. O th er in tra cta b le an d s ev ere c as es ma y ju sti fy an d ne c es si tate lo n g -te rm pr ed ni so l o n e th e rap y, w i th its a ss o ci a te d di sa d va nta ge s a nd s id eeff ec ts. In m a n y o f th es e c a se s th e o ra l le si o n s are p art o f a w i de sp rea d an d m o re

gen e ra liz e d co n di tio n , s u ch a s p em ph ig u s o r B eh Γ §e t's sy n dro m e. S c ru tin y o f a p ati en t's

me dic a l hi s to ry is e s se n tia l b ef o re c o m me n ci ng co r ti c os ter oi d the ra py a nd i t is e ss e nti al th a t reg ul a r m on i to rin g is c a rrie d o u t to de tec t a n y s ide -eff ec ts. M o n ito ri n g s h o u ld in c lu d e

me as u rem en ts o f bl o od pre s su re , w ei gh t, a nd b lo o d gl uc o s e e s tim ati on s . S tero i d tr ea tme nt wa rn in g c ar ds sh o u l d b e is su e d to pa tie nts a n d ca rri ed by th em a t a ll tim es .

Ste ro id trea tm en t w a rn in g ca rd s sh o u ld b e i s su e d to p a ti en ts a n d c a rrie d by th em a t a ll ti me s .

Ba s el in e me as u rem e nts o f bl o o d p res s u re, f as tin g blo o d g lu c o se , a n d w e ig ht are c h ec ke d

P. 27

bef o re co m me n ci n g th era py a nd a c h es t ra di o gra p h may be in d ic ate d in s o me c as e s. P a tie nts sh o u ld be m ad e aw a re o f th e po te nti al a dv ers e eff ects o f th e me di ca tio n (Tab le 3. 2) a nd th e ne ed fo r reg u la r m o n ito rin g . In a dd itio n , co r ti c os ter oi ds s h o u ld n o t be s to pp ed a bru p tl y.

Pa tie n ts w h o a re tak in g s ys te m ic co r ti c os ter oi ds m ay b e at r is k f ro m a hy po ten s iv e ad ren a l cri si s if th ey u nd er go de n tal o r m a xil lo fa c ia l p ro ced ur es , pa rtic u la rl y u n de r g en e ral

an a es th es ia , a nd co n s id era tio n s h o u ld be g iv en to pro vi din g th em w i th ad di tio n al β€ ste ro id

co ve rβ€™. T he re ar e, h o w ev er, n o e vi den c e -ba se d proto c ol s c ur ren tly a va ila b le to in d ic ate

wh i ch p a tien ts u n de rgo i n g d en ta l tr ea tme n t sh o u l d be gi ven a d di ti o n al β€ ste ro id co v erβ €™.

Nei th e r i s the re an y c le ar in di c atio n of th e o ptim um do s e an d tim e o f d el iv ery. Th ere is

evi de n ce th at ad ren a l su p pre ss i on is re du c ed if co rti c os ter oi ds a re gi ve n o n ce a da y (in th e mo rn in g ) a n d po s si bl y o n a lte rn ate da ys .

Table 3.2 Complications of systemic corticosteroid therapy Su p pre ss io n of a dre n al fu n c tio n Hyp erte ns i on

So d iu m an d w a ter r eten ti o n Po ta ss iu m lo s s Dia be te s

Os teo p or os i s

Me n ta l di stu rb a nc e s ( ps yc h o se s, mo o d c h an g es , eu p hori a ) Mu s cl e wa s tin g

Fa t red is trib u tio n Pe pti c ul ce ra tio n

Cu sh i ng 's sy n dro m e

Su s ce pti bi li ty to i nf ec tio n s (c an d id o si s, b a cte ria l an d vi ral i nf ec tio n s ) Gro w th su p pre ss i on in c h il dre n Neo p la sm s , e. g . lym ph o m as

Fi fty p er c en t o f pa tie nts o n l o n g-te rm c or tic o ste ro id s d ev el o p o ste o po ro s is a n d b o n e l o ss i s gre ate st in th e fir st 3β €“6 mo n th s o f s ter oi d th erapy . Pr eve n tive th er ap y, fo r ex am pl e,

dis o di u m etid ro n a te (Di dro n e l P M O Β® ), sh o u ld th ere fo re be c o mm e nc e d a t th e o uts e t o f

ste ro id th era py (p red n is o lo n e 5 mg o r mo re pe r d a y) th a t is l ik ely to l as t fo r mo re th a n 3

mo n th s. A b a se li ne bo n e de n si ty s c an s h o u ld id ea ll y be o rga n iz e d f o r a du lt pa tie nts . If the pa tien t is f ou n d to h a ve o ste o po ro s is p rio r to tr eatme n t w ith s tero i ds , mo re p o ten t

bis p ho s ph o n a tes (f or ex am pl e, a l en dr on a te or ris e dro n a te) ma y b e req u ire d a n d th e p a ti en t ma y n e ed to b e r efe rre d to a m eta b ol ic b o n e d is ea s e cl in i c.

Pa tie n ts o n pre dn is o lo n e β‰ ¥5m g /da y (o r eq u iv al en t) f o r m o re th a n 3 mo n th s s ho u l d b e giv en p ro ph y la cti c bi sp h os p h on a tes .

Azathioprine

Ste ro id -sp ari n g dru g s su c h a s az a th io p rin e ma y be gi ve n to m in im iz e th e l o n g -te rm ef fe cts o f sys te mi c ste ro id s. Az a th io pr in e is a c yto to xic i mm un o su p pre ss a n t w ith p ote n tia ll y s eri o u s

si de -eff ec ts. It sh o u ld n o t b e p res c rib ed u n le ss a de qu a te mo n ito ri n g i s av ai la bl e thr ou g h o ut

th e d ur ati on of th era p y (s e e d ru g da ta sh e et). P a tie nts a re at ris k of d eve lo p in g bo n e ma rro w su p pre ss io n , p ar ti c ul ar ly if the y ha ve a de fi ci en c y o f th io pu ri n e m eth y ltra n sf era se (T PM T) , a cyto p la s mi c en z ym e i n vo lv ed in th e m eta bo li s m o f a za th io p rin e . Pa tie n ts ca n n o w be te ste d

fo r TP M T de fi ci en c y.

Other systemic drugs used in oral medicine

Oth er s yste mi c dr ug s c ur ren tly u se d in o ral m ed ic ine in c lu d e d a ps o n e, c ic lo s po ri n , co l ch i ci n e, th a li do m id e, a n d my co p h en o la te. T h e i n di ca tio n s fo r an d mo n i tor in g o f s id e -eff ec ts as so c ia te d

wi th th es e dru g s ar e d is c us s ed i n the re le va n t ch a pte rs th ro u gh o u t th is b o ok . Th er e i s al so in cr ea si n g i n tere st in th e u se o f an ti- TNF (tis s u e n e cr os i s f ac to r) al ph a th era py fo r se ve re in fl am ma to ry di se as e (fo r exa m pl e, B eh Γ §e ts di se a se). Th es e dru g s ar e u s ed o n a l im ited ba si s o nl y fo r s ev ere o ra l d is e as e an d h av e no pl ace in th e n o n - sp ec ia li st pra c ti c e o f o ra l me dic i ne .

Limitations of therapy

Th ere is a p a uc ity o f o bje cti ve ev id en c e c o n ce rn in g th e eff ec tive n es s o f a n u m be r o f cu rre n t th e ra peu ti c reg im en s fo r o ral m ed ic in e c on d iti on s an d the re is c le ar ly a ne ed fo r th e

dev el o pm en t o f ev ide n ce -ba se d ma n ag em en t p ro to co l s. Cli n ic ia n s sh o u ld al s o be a w are ,

pa rtic u la rly w h en p res c rib in g to pi ca l fo rm ul ati o n s of d ru gs s u ch as s ter o id mo u th rin s es o r sp ray s, th a t m o st are n o t l ic en s ed fo r us e in o ra l co n d itio n s a n d do s ag e sc h ed u les a re

th e ref o re n o t e s ta b lis h ed . P ati en ts s h ou l d be m ad e aw a re tha t th e y a re be in g gi ve a dru g ou ts id e its li ce n se d in di c atio n as th at th e y c a n no t g iv e in fo rm ed c o ns e nt fo r tre atm en t wi th ou t th i s kn o w le dg e.

Project 1.

Vi si t yo u r lo c al ph a rm ac y an d en q u ire w h ic h th erapi es a re av ai la bl e to bu y o ver th e

co u n ter (OT C) f o r m o uth u lc ers . Wh a t a dv ic e w o u ld th e ph a rm ac is t g ive to s o me o n e se eki n g in fo rm ati o n ab o u t m ou th u l ce rs ?

P. 28

Au th o rs : Title:



Co py rig ht Β © 2003 Ox fo rd U ni ve rsi ty P re ss > T ab le o f Co nt e nt s > 4 - Inf e c tio ns of t he g ing iv ae a nd o r al m u co sa

4

Infections of the gingivae and oral mucosa Problem cases Case 4.1

A 28-yea r-ol d la dy atte n ds yo u r de nta l pra c tic e f o r h er 6 -mo n th ly ex am in a tio n . Yo u n o tic e th a t s h e h a s a h ea li n g β € co ld s o re β€™ (h erp es l ab ia li s) o n h er lo w er li p. O n c lo s er

qu es tio n in g th is p ati en t r epo r ts th a t s h e s u ff ers fro m β € co ld s o res β€™ eve ry 2β€“ 3 mo n th s

an d th at th e y a re be c om in g a β € rea l n u is an c e β €™. S h e is p arti cu l ar ly af fec ted w h en on

ho l id ay a bro a d an d ex po s ed to th e s u n . Q1

Wh at a d vic e w o ul d yo u g ive th is l ad y?

Q2

Doe s th e l es io n o n h e r l ip pr es en t a h a z ard to m em be rs o f th e d en tal tea m?

Case 4.2

A 21-yea r-ol d fu ll y d en ta te m a n pre s en ts at yo u r d en ta l p ra cti ce co m pl a in in g o f a s o re mo u th an d th ro at o f 6 w e eks d u ra ti o n . A ph a rm ac is t rec o m me n ded th a t h e us e a na e sth eti c th ro at

lo z en ge s a nd a c h lo rh e xid in e rin s e bu t th e se h a ve not h elp ed . Th e pa tie n t a pp ea rs w ell a n d rep or ts no rel eva n t me di ca l h is to ry. E xtra o ra l e xa mi n ati o n rev ea ls n o l ym ph a de n op a th y .

In tra o ra lly , h is o ra l m u c o sa a pp ea rs e ryth em ato u s an d th e re is ev id en c e o f ex ten s ive w h ite fle ck s o n th e s o ft p a la te a n d bo th b uc c al m u co s a e. Th e se c an ea si ly be w ip ed o ff w ith a

ga u ze n a pki n . Yo u m ak e a c l in ic a l d ia gn o s is o f ps eud o me mb ra no u s ca n di do s is . Th e re are n o oth e r a bn o rm al iti es o f th e o ra l m u c o sa o r gi n gi vae . Q1

Ho w w o ul d yo u ma n a ge th is p ati en t?

Introduction

It is p os s ib le to de m on s tra te a w i de ra ng e o f p o tentia ll y p a th o g en ic o rg a ni s ms i n the n o rm al mo u th . Ma n y of th es e ar e pre s en t i n rel ati ve ly hi gh nu m be rs , bu t i n s pite o f thi s th e o ra l

mu c o sa s h o ws a rem a rka bl y l o w su s c ep tibi li ty to p rim a ry i n fe cti on . T hi s is p ro ba bl y d u e, a t

lea s t in pa rt, to th e a c tivi ty o f s al iva , fr o m w h i ch a n u m be r o f an tib a cte ria l su b s ta n c es m ay be de mo n s trate d. Ig A is i n th e s a li va a nd i t h as b ee n s u gg es ted th at, a s w el l a s a po s s ib le dir ec t ef fec t o f th is o n th e o ral m ic ro -or ga n is ms , a co m pl ex m ay be fo r me d b etw e en th e

im mu n o gl o bu li n a nd th e o ral e pi the li u m i tse lf res u lti n g i n a pr ote c ti ve s ur fa ce c o ati ng o n th e mu c o sa . Th e re i s al s o a fu rth er me c ha n ic a l p ro tec tiv e a c tivi ty o f s al iva i n th at fo rei gn

ma teri al , in c lu d in g mi c ro - or ga n is ms , is w a s he d fro m th e o r al c av ity to the s to ma c h w he re ba cte ria ar e d es tro ye d by ga s tr ic fl u ids .

Th es e lo c al fa c to rs , h o w ev er, rep re se n t n o mo re th an a fi rst lin e o f de fen c e fo r th e pa tie n t

ag ai n st i n va si o n by po te n ti a l p ath o g en s , an d d epe n d fo r th e ir e ffe c tive n es s o n th e i n tegr ity of th e i mm u n e an d o the r g en e ral iz e d p ro tec tiv e r es po n s es o f the h o s t. If th e ba la n c e

betw e en th e h o st an d th e c o m me ns a l mi cro -or ga n is ms i s di stu rb ed by s o me fa cto r tha t

im pa irs th e im mu n e de fe n ce s the n o pp o rtu n is tic o rg an is m s ma y be gin to a ct i n a p ath o ge n ic

ma n ne r. Th i s is a re su l t o f th e ir i n cr ea se i n n um b ers th at e xc ee ds a m in i mu m in f ec tio u s

do s e. It i s in s u ch a wa y tha t m a ny c as e s of o ral ca nd id o si s an d a cu te n ec ro tiz i ng u l ce rati ve gin g iv itis a re th ou g h t to o cc u r. A pa rt f ro m in fe ctio ns o f th e or al m uc o s a bro u gh t ab o ut by a dis tu rba n c e o f a n o rm al h o s tβ €“co m me n sa l rel ati o ns hip th er e a re o th ers , pa rti cu la rl y th o s e

of vi ra l o ri gi n , tha t re pre s en t th e fir st r es po n s e o f th e pa tien t to the in fe c ti ve a ge nt. E v en i n so m e o f th es e (o ro fa ci al h e rpe s in fe cti o ns a re a n ex am pl e) the re ma y be c o mp le x

im mu n o lo g ic al c h an g es in the p ati en t th a t a re o f gre a t si gn i fic a n ce in un d ers ta n din g th e

cl in ic a l c o u rs e o f th e d is ea s e. T he im po rta n ce o f pri ma ry an d s ec o n da ry im mu n e de fic i en c ies in re la tio n to o ra l in fe c ti o n s is d is cu s se d in Ch apter 14.

Th e r ol e o f b ac teri a in th e p ath o ge n es is o f ca ri es , n o n- sp ec if ic gi n gi viti s, a n d pe rio d on ti tis is ou ts id e th e s co p e of th is c h ap ter an d re ad ers sh o u ld re fe r to s pe c ia li st te xts o n th es e su b jec ts.

In pre vi o us e di tio n s o f th i s bo o k, s trep to co c c al s tom a titis w a s in c lu d ed as a ba c teri al

in fe cti on po s si bl y a ff ec tin g th e o ra l mu c o sa . Th e re wo u l d s e em to b e no do u bt th a t th er e i s a rea di ly av ai la bl e res erv o ir of s trep to co c ci i n th e o ra l ca vi ty th a t m ig ht be ex pe cte d to

res po n d to a di stu rb a nc e in th e h o stβ €“co m me n sa l rel ati o ns h ip b y b eh a vi ng i n a pa th o ge ni c

ma n ne r. It i s eq u al ly ev id en t th a t a ge n era li ze d infe cti o n o f th e m ou th a n d o ro ph a ryn x mi gh t

oc c u r a s a res u lt of i nv as io n b y exo g en o u s c oc c i. In s p ite o f th is , i t is b y n o m ea n s cl ea r th a t a tr ue s trep to co c c al s to m a titis d o es , in f ac t, e xist, a lth o u gh u n til rel ati ve ly rec en tl y i t w as gen e ra lly a cc e pted a s a cl in i ca l en tity . Th e co n f usi on ma y h a ve a ris en be ca u se o f th e

si mi la rity be twe en the s ym pto m s as c rib ed to a s trepto co c c al s tom a titis a n d tho s e ca u s ed by

mi ld vi ral in fe c ti o n s. T he s ym pto m s o f s trep to co c c al s to ma titis w e re ge ne ra ll y d es cr ib ed as a gen e ra liz e d eryth e ma o f th e o ra l mu c o sa to g eth er w ith a m ar ked g in gi vi tis , su b ma n di bu l ar lym ph a de n itis , a n d a m i ld de gre e of m al ai s e a n d fe ver .

P. 32

Bacterial infections Acute necrotizing ulcerative gingivitis (ANUG)

Ac u te n e cr o ti z in g u lc era tiv e gi n gi viti s (AN UG) w as , u n til re ce n tl y, re la tiv el y c o mm o n , bu t i s cu rre n tly mu c h le ss s o i n de ve lo pe d co u n trie s . AN UG do e s sti ll o cc u r i n s mo ke rs a nd

im mu n o co m pr om is e d p a ti en ts , pa rtic u la rl y i n th o se with h u m an i m mu n o de fic i en c y vi ru s (HIV ) in fe cti on . W he n i rrev ers ib le pe ri od o n tal de s ctru c tio n h a s o cc u rre d th e c o n di tio n i s no w m o re ap pro p ria tel y te rme d β€ ac u te n e cr oti z in g u lc era tiv e pe rio d o nti tis β €™. T hr ou g h o ut thi s

se cti o n, th e ter m A NU G wi ll be u s ed to de sc ri be bo th c o n di tio n s . Th e e xa c t ca u s e o f A NU G is

no t k n o wn , b u t th ere is n o do u b t th at d u rin g a n atta c k th e re i s a pro l ife ra tio n o f sp iro c h ae tes an d fu s ifo rm b ac te ria . Th er e i s al so a pro l ife ra ti on o f o b li ga te an a ero b ic , n o n -sp o ru la tive ro ds . Th es e ro ds a re th o ug h t to b e i mp o rtan t an d prob a bly m o re si gn i fic a n t th an th e

sp iro c h ae tes . A lth o u gh th e re a re m an y th eo re ti c al sp ec u la tio n s a bo u t th e ex ac t a eti ol o gy o f ANU G, th e re i s n o do u b t th at, f o r pra c tic al p u rpo s es , el im in a tio n o f the o ve rg ro wth o f th e mi cro -or ga n is ms i s co i n ci de n t w ith c li ni c al re mi ss io n o f th e dis e as e.

Clinical features

Th e c li n ic a l f ea tur es o f AN UG are so r en es s a nd b le ed in g o f th e gi n gi vae , to ge th er w i th th e

dev el o pm en t o f cr ate r -lik e u l ce rs , du e to th e ne c rosi s o f th e gi n giv al p ap il la e (Fi g. 4. 1). Th is ul ce ra tio n s u bs eq u en tly s pre ad s al o n g th e gi n gi val m ar gi n. P a tie n ts d ev el o p a m a rked

ha li to si s, wh i ch h a s a c ha ra c te ris tic o do u r ( Tab le 4. 1 ). In a fe w pa tie n ts w i th A NUG th ere a re fev er, m a la is e, a n d lym ph a de n iti s. M o s t, ho w e ver , are y ou n g a du lts a n d, i n th e mo re u s u al , un c o mp li ca ted c a se , th e p ati en t i s in iti al ly pe rfe ctl y h ea lth y.

An y pa tie n t, fo r w h o m pre di sp o si n g fa cto rs c an n o t be id en tif ie d, s h o ul d be s u sp ec ted o f su ff eri n g f ro m an u n d erl yin g s ys tem ic di so rd er an d , in vie w o f th e po s s ib ili ty o f a bl o o d

dys c ras i a in s u ch p a tien ts , h ae ma to lo g ic al e xa mi na tio n sh o u ld b e u n d erta ke n. O n e o f th e

gre ate st cl in ic a l pro b le ms i n th e m a n ag em en t o f A NUG is th a t o f rec u rren c e. It is ev id en t th a t th e pa tie n t w ith p o or o ra l h yg ie n e or w ith g ingi va l co n to u rs di sto rte d by a pre vio u s

atta ck m ay , be ca u se o f the s e l o ca l fa cto rs , b e s u s ce pti ble to re c ur ren t in fe c ti o n , pa rtic u la rly if pre di sp o si n g f ac to rs s till p ers is t.

Fig. 4 .1 A cu te n ec ro tiz in g u lc era tiv e g in g ivi tis , s h ow i n g des tru c ti o n o f th e gi n gi va l pa pi lla e .

Table 4.1 Clinical features of acute necrotizing ulcerative gingivitis (ANUG) So re n es s an d b lee di n g of th e gi ng iv ae Nec ro s is o f th e gi n gi val pa pi ll ae Hal ito s is

Predisposing factors

Th e i n flu e n ce o f po o r o ra l h ygi en e in th e i ni tia tion o f A NU G h a s be en o f ten s tres s ed , bu t th e re is n o d o ub t th a t th ere a re s o m e pa tie n ts wh o se sta n da rd o f h yg ie n e m u s t be

co n s id ere d b y no rm a l c ri teri a to be go o d . Th e mo s t c o mm o n pre di sp o s in g fa cto rs o th er th an

sys te mi c on e s ar e to b ac c o sm o ki n g a n d ps yc h o lo gi c al s tr es s, al tho u g h it i s by n o m ea n s ea sy to se e ho w th es e fac to rs o pe ra te. Ni co ti n e c a us e s va so c o n stri cti o n o f b lo o d ve ss el s w ith a

co n s eq ue n t re du c tio n o f blo o d s u pp ly to the ti ss u es a n d in c rea se d su s c ep ti bi li ty to in f ec tio n an d da m ag e.

Dec rea s ed h o st res is tan c e o r de pre s se d i m mu n e res pon s es a re im po rta n t s ys tem ic fa c to rs pre di sp o si n g to A NU G. A c hr on i c n ec ro tiz in g gi n gi viti s is a ss o c ia ted w ith H IV in fe cti o n.

In no rm a l h e al th y p ati en ts w ith A NU G, s pre a d o f th e i n fe cti on fro m th e gi n gi val m ar gi ns i s rel ati vel y ra re . In a pa tie n t w ea ke n ed by de bi li tati ng di se a se th e in fe cti on ma y sp rea d to

su rro u n di n g ti ss u es . A n e xa mp le o f th e sp re ad o f ANUG in th e de bi lita ted p ati en t i s se en i n ca n cru m o ri s (n o ma ) a co n d itio n no w v irtu a ll y u n kn ow n in Eu ro p e.

Diagnosis of ANUG

Th e c li n ic a l d ia gn o s is o f AN UG ma y be c o n firm ed b y th e de m on s tra tio n o f a fu so s pi ro c ha e ta l co m pl ex in a Gra m -sta in ed d ee p gin g iv al s me ar. Ba c teri a cu l tu re d in A NU G i n cl u de ,

Trep o n em a vin c en ti i, d en tic o la , an d ma cr od en ti um ; Pre vo tel la i n te rm ed ia ; P o rp hy ro mo n a s

gin g iva li s ; a nd Fu s o ba cte riu m n u cl ea tu m.

Management of ANUG

Th e i n itia l trea tme n t o f AN UG co m pri se s s up ra gi n gival pl aq u e co n tro l, a n d the u s e of a sys te mi c an tib io ti c, s u ch as m etro n i da z ol e. S mo ke rs s h ou l d be ad vi se d to ref rai n fro m

sm o kin g . P ati en ts w ith A NU G p res en t w ith va ry in g degre es o f gi ng iv al d is co m fo rt, b u t in mo s t c as es i t is n o t fe as ib le to c ar ry o u t a s c al e an d po l is h a t th e i n itia l vi si t. Gen tle

deb ri dem e nt o f th e gi ng iv al tis s u es s h o ul d be do n e a nd th e pa tie n t gi ve n in s tru cti o ns to us e a c h lo rh e xi din e m o uth ri n se a n d a ttem pt ge ntl e too thb ru sh i ng . A NUG res p o nd s ra pi dly to th e us e o f p en i ci ll in , a nd a n u m ber o f o th e r an ti bi oti cs , b u t me tro n id az o le i s th e u s u al dr ug o f

ch o ic e. A do s e o f 200 m g, th ree tim es d a ily , fo r 3 da ys i s su ff ic ie n t in m o s t ca s es to re du c e th e s ym pto m s dra m ati ca ll y i n 24 h o u rs .

Ho w eve r, if re so l u ti o n do e s n ot o cc u r, a n u n de rly ing co n d itio n s h o u ld be s u sp ec ted a n d

ap pro p ria te in ve s ti ga tio n s u n de rtak en . Wh e n pr es cr ibi n g me tro ni da z o le pa tie n ts sh o u ld b e

wa rn ed to a vo i d a l co h o l. M e tr on i da z o le is s im il ar in its ef fec ts to d is u lfi ram an d b lo c ks th e no rm a l m eta b ol ic p ath w a y o f al c oh o l el im in a tio n . In s o me pa tie n ts, s id e -eff ec ts su c h a s na u s ea , h ypo te n si o n, a n d fl us h in g m ay fo l lo w if al coh o l is ta ke n du ri n g tr ea tme nt. T h e

te ra tog en i c eff ec ts o f m etro n i da z o le ar e u n ce rtai n a nd i t is th e ref or e n o t re c om m en de d in ea rly pre gn a n cy . O nc e th e a c u te ph a se h a s res o lv ed , pe rio d o nta l trea tme n t s ho u l d co m me n ce , to ge the r w i th sm o ki ng ce s sa tio n a dv ic e.

Me tro n ida z o le is th e an ti bi oti c o f c h o ic e fo r th e in i ti al m an a ge m en t o f AN UG. Pa tie n ts s h o u ld be w a rn ed n o t to ta ke al co h o l w h ilst tak in g me tro n id az o le . Me tro n ida z o le is b es t av o id ed du ri n g p re gn a nc y.

Syphilis

Sy ph il is i s a se xu a lly tra n sm itte d d is ea s e an d th e ca u s ati ve ba cte ria l ag en t i s Trep o n em a

pa lli du m . Th is g ai n s en try to the b o dy via m u c ou s m em br an e s an d m in u te ab ra si o n s in th e

ski n . Th er e i s a po p ul a r m is co n c ep tio n th a t sy ph i lis i s a ra re di se a se bu t it i s a co m mo n

in fe cti on in Ru s s ia a n d th e F ar E as t a n d i ts in c id en c e w o rl dw id e is i nc re as in g . O utb rea ks o f

ca se s c o nti n ue to b e rep o rted in th e U K a n d o ral l es io n s o f s yp h il is m ay be s ee n in th e o ra l

me dic i ne c li n ic . E arl y dia g no s is i s es s en tia l as the lo n g - te rm ef fe cts o f un tre ate d sy ph il is a re se rio u s a nd p o ten tia ll y l if e-th re a te n in g .

P. 33

Clinical features of syphilis

Pri ma ry le si o n s of sy ph i lis m a y a pp ea r on an y pa rt o f th e o ra l mu c o sa a n d mu s t a lw a ys be

co n s id ere d in th e di ff ere nti al d ia gn o s is o f or al u lc er ati on . A s i n the c a se o f th e g en ita l le si o n th e o ra l p ri ma ry les i on (ch a n cr e) ap pe ars f o llo w i ng a p eri o d o f 2β€“ 3 w ee ks a fter in fe cti o n.

Us u al ly th e c h an c re pr es en ts a s a pa in le s s i n du ra ted sw e ll in g, d a rk r ed in c o lo u r an d w ith a gla z ed s u rfa ce (Fi g. 4. 2), fro m w h ic h la rg e n u m be rs o f Trep o n em a pa ll id u m ca n b e is o la ted an d de mo n s tra te d o n da rk- gro u n d mi cro s c o py. Ch a n cres ar e m o st li kel y to be fo u n d in th e

rel ati vel y s o ft a n d u n res tric ted tis su e s o f th e to ngu e, c h ee ks , o r l ip s, b u t w h ere th ey o c cu r on th e p al ate o r gi n gi vae th e mo rp h o lo g y o f the l esio n s m ay be m o di fie d, a n d the y ma y

ap pe ar as mo re d iff u se s tru ctu re s. W h ate ver th e s ite , s iz e, o r sh a pe o f the c h an c re , h o we ve r, th e h ea vy in f ec tio n o f the s u rfa c e i s co n s is ten t, th u s pro v idi n g bo th a co n v en ie n t me a ns o f

dia g no s is a n d a co n s id era bl e h az a rd to the u n s u sp ectin g di ag n o sti ci an pa rtic u la rly if g lo ve s are n o t w o rn . A t th e ti me o f the p rim ar y le si o n th ere is a n o n ten d er en la rg em en t o f th e

ce rvi ca l lym ph no d es a ff ec tin g th e s u bm en ta l, s u bm an dib u la r, p re - an d po s t-au ri cu l ar, a n d oc c ip ita l gro u p s β€” th e s o -ca ll ed β € syp h il itic c o ll ar β€™.

Fig. 4 .2 P rim ar y s yp hi li tic le si o n (c h an c re) o f th e to n gu e.

Th is d is ap pe ara n c e o f th e pri ma ry le si on , u s u al ly after a pe rio d o f s o m e 2 w ee ks , m ark s the wi de sp rea d di s se mi n ati on of th e mi cr o- or ga n is ms a n d th e o n s et o f th e s ec o nd s tag e o f th e dis e as e, w h i ch m a y l as t f or m an y yea rs . Th e o ra l symp to ms m o st o ften d es c rib ed a t th is

sta ge o f th e d is ea se a re mu c o u s pa tch e s (a pp ea rin g a s gre y -wh i te u l ce rs c ov ere d by a thi c k sl ou g h ) a n d β€ sn a il tra ck β €™ u lc ers . In v iew , h o w ev er, o f th e p rotea n n a tur e o f th e s ki n

les io n s p ro du c ed du ri n g th i s sta ge o f the d is ea s e, i t w ou l d se em a t le as t a re a so n a bl e

po s si bi lity th at the re ma y be a n eq u iva le n t va ria tio n in th e fo rm o f o r al le si o n s. If th is is s o , it w o u ld s ee m m o re th an l ik el y th a t s ec o nd a ry s yp h il itic u lc ers m a y o fte n p as s u n rec o gn iz e d or be m is tak en f o r s o me le s s s i gn if ic an t n o n -sp ec ific le s io n a n d, fo r th is re as o n , se ro lo g ic al

te s ts f o r s yp hi li s sh o u ld be p art of th e in ve sti ga tio n o f o ral u l ce rati o n o f u n kn o w n o ri gi n. In th i s se c o nd s tag e lym p h n od es m a y a ga i n be pa l pa bl e a s n o n ten de r, d is cre te str uc tu res .

In th e te rtia ry s ta ge o f s yp h il is tw o m ajo r fo rm s of o ra l in vo l vem e nt ma y oc c u r. Th e fir st o f th e se , s yph i li ti c le u ko pl ak ia , w il l be d ea lt w i th in d eta il in Ch ap ter 10 to ge th e r w i th oth e r

leu k op la ki as . In th e in a de qu a tel y tre ate d or u n treated c as es , w h ic h a re n ow se en ve ry r are ly , leu k op la ki a o f th e w h ol e of th e o ral m u co s a bu t e spec i al ly o f th e to ng u e is a n im po rta n t

co m pl ic ati o n, th e m o re s o b ec a us e m al ig na n t tra ns form a tio n c an oc c u r. Th e se c on d typ e o f

or al in v ol ve me n t in th e terti ar y sta g e i s the d ev elo p me n t o f a β€ gu mm a β€™. T hi s is

es se n tia lly a c h ro ni c gra n u lo m a, o f ten i n th e p a la tal tis s ue s , tha t e ve ntu a ll y b rea ks d o w n wi th th e co n s eq u en t p ro du c tio n o f a tis su e d efe ct. T h e u n tre ate d p a ti en t a t th i s s ta ge is lik el y to h a ve o th er mo re w id es pre a d l es io n s , es pe cia ll y o f the n e rvo u s sy ste m, bu t th e

mi cro -or ga n is ms a re by n o m ea n s as re ad il y d em o n stra ted a s in th e ea rli er s ta g es o f th e

dis e as e. A s w el l as th e se m os t c o m mo n ly de s cri be d ma n if es tati o ns o f la te sy ph il is , a n u mb er of o th er or al c h an g es h a ve be en de sc ri be d, i n cl ud ing a fi bro s in g g lo s si tis . Os teo m ye li ti s

aff ec tin g th e j aw i s a ra re co m pl ic ati o n o f s yp h ilis . A w ide ra n ge o f cl in ic a l pre se n tati on s o f

te rtia ry sy ph i lis i s po s si bl e an d th is fa c t em p ha s iz es th e w is do m o f in c lu di n g se ro lo g ic a l te sts fo r th e di se as e in th e in v es tig ati on o f u n u su a l o ral co n d itio n s . E ve n if th e p a ti en t i s o f a n

ad va nc e d a g e w h e n the c o n di ti o n is di ag n o se d an d a lth o u gh n o s ys tem ic e ffe c ts a re o bv io u s , tr ea tme n t is i nd ic a ted . S ym pto ms s u c h as m en ta l co nfu s io n th a t m ig ht be a ttr ibu te d to

se n ile ch a n ge s ma y be , in f ac t, th e res u lt o f th e syp h il itic i n fec tio n a n d ma y res po n d to

P. 34

an tib io tic tr ea tme nt eve n a t a la te sta ge .

Fig. 4 .3 Hu tc hi n so n 's in c is o rs in c o n ge n ita l s yp h il is .

Trep o n em a pa ll id um cr o ss es th e pl ac e nta l ba rri er an d c au s es c o n ge ni tal s yp h ili s in th e fe tus . Th e d en ta l ab n or ma li ties ma in l y a ff ec t th e pe rma n ent d en ti ti o n , as th e de c idu o u s tee th a re

gen e ra lly w el l de ve lo pe d by th e ti me th ei r to o th germ s are i nv ad ed b y th e s pi ro ch a ete s. T he firs t p erm a ne n t m o la rs (β€ Mu l ber ryβ €™ or β€ Mo o n 's β€™ mo la rs ) a n d u pp er ce n tr al

in ci s or s (β€ Hu tch i ns o n 's β€™ in c is or s) ar e u s u al ly in vo l ve d i n co ng en i tal sy ph i lis ( Fi g. 4. 3 ).

The diagnosis of syphilis

Th e d ia gn o s is o f sy ph i lis is g en e ral ly ba s ed o n th e res u lts o f s e ro lo gi c al tes ts . Trep o n em a

pa lli du m ca n n o t be c u ltu re d in vi tr o β€” it i s pro p ag ate d in a n im al s to a llo w o rg a ni s ms to b e

pre pa red fo r se ro lo g ic a l te sts . Cu rre n t s ero l og ic a l te sts i n cl u de th e V e n ere al Dis e as e

Refe re nc e La bo ra to ry (V DRL) tes t, th e Trep o n em a pa ll id u m h ae ma g glu ti n ati on as s ay (TP HA ), th e fl u o res ce n t Trep o n em a an ti bo d y a bs o rb ed tes t (F TA (a b s)) , an d th e Trep o n em a pa ll id um im mo b ili z ati on (TP I) te st ( Tab le 4. 2).

The treatment of syphilis

Pe n ic il lin is th e tr ea tme nt o f c h o ic e an d i s giv en i n h ig h do s e s. In p rim a ry s yp h il is th e co u rs e

of a n ti bi o tic s is u p to 1 m o n th bu t i n la te (o r l ate nt) sy ph i lis th i s is fo r up to 12 we ek s. Pa tie n ts w ho are a ll erg ic to p en i ci ll in c an be pr escr ibe d ery thr om yc in o r tetra c yc lin e .

Gonorrhoea

Pri ma ry o ra l l es io n s o f g o n o rrh o ea a re rel ati vel y rare . Th e y a re th e res u lt of tra n sm is s io n o f th e o rg an i sm (Nei ss er ia go n o rrh o ea ) by d ire ct mu c o sa l co n ta ct.

Table 4.2 Serological tests currently used to diagnose syphilis Tes t

Abbr ev iatio n

Ve n ere al Di se as e Ref ere n ce La bo ra to ry tes t

VDRL

Trep o n em a pa ll id um h ae ma g glu ti n ati on as s ay

TPH A

Fl uo re s ce n t Trep o n em a an ti bo d y a bs o rb ed tes t

FTA

Trep o n em a pa ll id um im mo b il iz a ti o n tes t

TPI

Clinical features

Les io n s ap pe a r p red o mi na n tly in th e p ha ryn x a n d a re in va ri ab ly a res u lt of o ro g en ita l se xu a l co n tac t.

Diff us e ery th em ato u s a nd u lc e rati ve o ra l l es io n s , to ns i lli tis , a pu ru l en t g in gi vi tis , an d o th er

or al m an if es tati o n s ha ve b ee n de sc ri be d. P a ti en ts dem o ns tra ted to h a ve o ral l es io n s a re very few r ela tiv e to the v ery la rge n u m be r kn o w n to h a ve ge n ita l g o n or rh o ea . Th e sy ste mi c

dis tu rba n c es d es cr ibe d as b ei n g a s so c ia ted w i th su ch o ra l les i on s v ary fr om m il d to se ve re

feb ril e sy mp tom s , an d th e de gre e of o ral d is co m fo rt rep o rted is e qu a ll y v ari ab le , w ith s o me pa tien ts c o mp la in i n g o f di ffi cu l ty s w al lo w in g . S ub man d ibu l ar ly mp h ad en o pa th y ma y b e

pre se n t. It is li kel y th a t, in mo s t c as es , th e n ature o f th e in f ec tio n i s n o t i n iti al ly su s p ec ted on cl in ic a l g ro u n ds , bu t b ec o m es c le ar o nl y as a resu l t o f ba c teri ol o gi ca l ex am in a tio n . Th e

tr ue i nc i de nc e o f o ra l go n o rrh o ea l in fe c ti o n s pro b ab ly re ma in s u n rec o g ni z ed be c au s e of th e rel ati vel y n o n -sp ec if ic n atu re o f th e l es io n s .

Cli n ic al fe atu re s o f o ra l go n o rrh o ea i n cl u de di ffu se ves i cu l ar, ery th em ato u s , an d u lc er ati ve or al le si o n s an d a pu ru l en t g in g ivi tis . To n si ll ar in vo l ve me nt ma y al so be a fe atu re .

A dif fu s e f o rm o f g o n or rh oe a s pre ad b y h a em a to g en o us ro u tes m ay ve ry o cc as i on a ll y a ff ec t th e o ral m u co s a , al th ou g h th e p re do m in a nt ma n ife s tatio n s a re o n th e s ki n . Ul ce rs ,

ha em o rrh a gi c le si o ns , a n d o th e r m an i fes ta tio n s o f hyp ers e ns i ti vi ty to th e di ss e mi na tin g

mi cro -or ga n is ms h a ve be en d es c rib ed . O cc as i on a l pa tie n ts w i th go n o c o cc a l i n fec tiv e a rth ri ti s

of th e te m po ro m an d ib ul ar jo in ts h a ve al so be en rep orted . Th e sy mp to ms a re s im ila r to tho s e of o th er type s o f i n fec tiv e ar thr itis β€”pa in , s w ellin g , an d tris m u s. In m o s t de sc ri be d c a se s th e d ia gn o s is h as d ep en d ed o n ba c teri o lo gi ca l s tu d y o f fl u id as p ira ted fro m th e af fe cte d joi n t.

Go no rr ho e a in th e o ro fa c ia l a re a is li ke ly to be u nde rdi ag n os e d.

Diagnosis of gonorrhoea

Mi cro b io l og ic a l c o n fi rma tio n i s es s en tia l to co n f irm th e cl in ic a l s u s pi ci o n. E x am in a tio n o f a

Gram -sta in ed s m ea r o f the o ra l le si o n s ma y s h o w Gram -ne ga tiv e d ip lo c o cc i. Mi cr ob io l o gic a l sw a bs s h o ul d al w ay s be ta ken fo r c u ltu re a nd s en s iti vity .

Treatment of gonorrhoea

As i n u ro ge n ita l g o n or rh oe a , th e tre a tm e nt is b y h ig h d os e s of a n tibi o tic s. Va ryi n g r eg im es ha ve b ee n de sc ri be d, v ary in g fro m a s in gl e, h i gh -dos e i n tram u sc u la r in jec tio n o f pr oc a in e pen i ci ll in to o ra l am o xy ci ll in (o r am pi ci ll in ) to sh o rt c o u rs es o f or al tetra c yc lin e o r c o -

tr im ox az o l e. Th e p ati en t s h ou l d be ref err ed to a s pe ci al is t in g en i tou ri n ar y m ed ic in e (GU M )

fo r fu l l a s se s sm en t. A s in al l s e xu al ly tra ns m itted d is ea s es , it s h o u ld be re me mb er ed th at th e pa tien t mi gh t h a ve ac q ui re d m o re th an o n e in fe c tio n a t th e s a me tim e .

P. 35

Non-specific urethritis

No n -sp ec if ic u reth ri tis (NS U ) is p ro ba bl y th e m os t c o m mo n s ex u al ly tran s m itted d is ea se a n d in m o st c a se s Ch la my di a sp ec i es m ay be i so l ate d f ro m the u re thr a. T he sy mpto ms i n cl u de a

bu rn in g s en s ati on o n mi ctu ri tio n a n d th e re ma y be a pu ru l en t di sc h a rge in ma le s. Ch la my di a

in fe cti on ma y be as ym p to m a tic , pa rtic u la rly i n fe male s. Mi cro b io l og ic a l te sts a re es s en tia l to co n fi rm the d ia gn o s is a n d th e ma jo ri ty o f ca s es re sp o n d to te tra cy cl in e th era py in n o rm a l do s es o ve r a w ee k or tw o, eve n th o ug h n o m ic ro bi a l a ge n t c an b e de mo n s trate d. R eite r's

syn d ro me i s pre do m in a n tly se en i n yo u n g ma le s a nd ma y fo l lo w i n fec tio n w i th go n o rrh o ea ,

ch la m yd ia , o r e nte ric b ac ter ia . In th is c o nd iti o n th ere m ay be p ol ya rth riti s, w hi c h ca n a ff ec t th e tem p or om a n dib u la r jo i n t, ur eth riti s, u v ei ti s (or co n ju n c tivi tis ), a n d m a cu l ar le si on s o n th e p al ms a n d so l es . Or al le si o n s ten d to be e ryth em a tou s a n d of ten re s em bl e e ryth e ma

mi gra n s wi th a w h itis h b o rde r. Th is a pp ea ra n ce h a s be en d es c rib ed a s a β€ ci rci n ate β €™ sto m ati ti s .

Rei ter's s yn d ro me p red o mi na n tly a ffe cts y ou n g m al es a n d c a n pr es en t a s a po l ya rth ritis ,

ure th riti s, u v eiti s, an d ma c u la r l es io n s o n th e pa lm s an d s o les . Or al le s io n s h av e a ls o b ee n rep or ted an d i nc l ud e a β€ ci rci n ate β €™ sto m a ti tis .

Tuberculosis

Th e g en u s My co b ac teri u m in c lu d es pa th o ge n ic a n d n o n -pa tho g en i c sp ec i es . M . tu b erc u lo s is an d M. b o vis ar e e qu a ll y p ath o g en ic f o r m an . Tu b erc u lo s is is a w o rld w id e e n de mi c di se as e

wi th u p to o ne -th i rd o f th e w or ld' s po p ul ati o n be ing a ffe cte d. Th e re is a re su rg en c e o f

res pi rato ry p ul mo n a ry di se as e as s o ci ate d wi th im m uno d efi c ien c y, m a ln u triti on , a n d n o n co m pl ia n ce w ith d ru g reg im es , p arti cu l ar ly as a n A IDS -rel ate d p h en o m en o n . Th e

pa tho g en e si s, c l in ic a l fe atu re s, d ia gn o s is , a n d trea tm e nt of p ul m on a ry tub er cu l os is a re ou ts id e th e sc o p e o f th is b oo k .

Pri ma ry o ra l i n vo lve m en t w ith tu b erc u lo s is ma y, h o we ver, oc c u r, a lbe it rare ly , an d s h o ul d

al wa ys b e co n s id ere d in th e di ffe ren ti al d ia gn o s is o f o ra l u lc era tio n . Th e o ra l mu c o sa i s mo re co m mo n ly in vo l ve d b y b ec o m in g se c on d ar ily in f ec ted by th e sp u tum i n c as es o f a cti ve

pu lm o n ary d is ea se . Tu b erc u lo u s ly mp h ad en iti s fre qu entl y a ff ec ts the c er vic a l ly mp h n o de s.

In i tia ll y, a fi rm bu t m o bi le ly mp h ad en o pa th y is pa lpa bl e. S in u s a n d ab s ce ss f o rma tio n o c c u r la te r w i th ce rvi ca l ly mp h n o de s bec o m in g fix ed (s ee Ch ap ter 7).

Clinical features of oral tuberculosis

Ora l le si o ns o f tu be rcu l o si s u su a ll y p res e nt as p ain fu l u lc era tio n . Th e c la ss ic a l d es c rip tio n o f

a tu be rcu l o us u l ce r i s o f a n ir reg u la r le s io n w ith u n d erm in e d b o rde rs a n d c o ve red by a gr ey sl ou g h . Ul ce ra tio n c o mm o n ly af fec ts th e to n g u e b u t o th er are as o f th e o ral m u co s a ma y be

in vo lv ed , pa rtic u la rl y to w ar ds th e po s teri o r pa rts o f the mo u th . It s h o ul d be a dd ed th at a f ew les io n s h a ve be en d es c rib ed i n w hi ch the pre s en tatio n o f tu b erc u lo s is o f the o ra l mu c o sa i s

qu ite di ff ere nt fro m th e u lc ers d es c rib ed a bo ve . Thes e le si o n s, p re se n ti n g as w h ite pa tc h es

or gra n u la tin g le si o n s, h a ve b een de sc rib ed b o th as p rim a ry l es io n s a n d a s l es io n s se c o nd a ry to pu lm o n ar y i nf ec tio n .

The diagnosis of oral tuberculosis

A tu b erc u lo u s o ri gi n sh o u ld b e co n s id ere d in th e diffe ren ti al d ia gn o s is o f p er si ste n t o ral ul ce ra tio n o f u n kn o wn ae tio lo g y. De fin i tive d ia gn o sis o f su c h a le si o n in v ari ab ly fo l lo w s

bio p sy , w ith h is to pa th o lo g ic al e xa mi na tio n o f fo rm alin -fix ed tis s ue , s ho w i ng n o n -ca se a tin g

gra n u lo ma ta . Th e nu m be r o f a ci dβ €“ al co h o l -fas t b a cilli p res en t i n th e b io p sy s pe ci me n m ay , ho w e ver, be s ma ll a nd th ei r d em o n stra tio n b y Z ie hl β€“Nie ls en s ta in in g , o r b y

im mu n o flu o re s ce n t tec h n iqu e s, ca n be d if fic u lt. Fr es h tis s ue m a y b e n e ed ed to c u ltu re myc o ba c teri a o n Lo we s tein me di um , b u t th is ca n ta ke 6β€“ 8 w ee ks . If th er e i s cl in i ca l

su s pi ci o n o f a tu b erc u lo u s u lc er w he n th e p a ti en t firs t p res en ts th en th e i ni tia l bio p s y c an be div id ed a nd h a lf se n t fo r c u ltu re .

Management

Pa tie n ts w i th o ral tu be rc u lo s is s h ou l d b e fu ll y i n ve sti ga ted fo r pu lm o n ary o r o th e r le s io n s an d it w o u ld be e xp ec ted th at me a su re s su c h a s a ch e st rad io g rap h w o u ld be ta ke n at th e fi rs t

mo m en t o f su s p ic io n . Th e trea tme n t o f tu b erc u lo s is i s o uts id e the p ro vi nc e o f o ra l me di ci n e an d th e a p pro p ria te a c tio n to b e ta ke n w o u ld in vo l ve re ferr al o f th e pa tie n t to a c he s t

ph ys ic ia n (o r sp ec ia li s t in i n fec tio u s d is ea s es ). It s h o ul d be re iter ate d th a t th e in c rea s e o f tu b erc u lo s is i s cl o se ly rel a te d to ge o gra ph i ca l ar ea s af fec ted b y th e HIV e pi de mi c.

Fungal infections Oral Candidosis

Can d id a sp p ar e f u ng i th at h a ve a w id e dis tri bu tio n a n d tha t f req u en tly fo rm p art o f th e

co m me n sa l fl or a of th e hu m a n bo d y. S w ab s tak en f ro m th e s ki n, g u t, va gi n a, o r mo u th o f an ap pa ren tl y h e al thy in d iv idu a l al l ma y s h o w th e pre se n ce o f Can d id a sp ec i es a n d, i n

pa rtic u la r, Can d id a al bi ca n s . Th e o ra l c arri a ge ra te o f o ra l c a nd id al sp ec i es is a b ou t 40 pe r

ce n t o f th e n o rma l po p ul ati o n . Tab le 4. 3 sh o w s c an did al s pe ci es th a t h av e b ee n i so la te d f ro m or al le si o n s. O f th es e, C. a lbi c an s is th e mo s t fr eq ue n t.

Table 4.3 Candidal species involved in oral candidosis C. al bi ca n s * C. tro pi ca li s

C. ps eu d o tro pi ca li s C. gl ab ra ta C. kru s ei

C. pa ra ps il o si s * Th e mo s t fr equ e n tly is o la ted s pe ci es .

P. 36

Predisposing factors for oral candidosis

It ha s l on g b ee n rec o gn i z ed th at di se a se du e to a pro li fera tio n of th is m ic ro -or ga n is m is a ma rk of lo w e red res i sta n ce o r o f m eta bo li c ch a n ge in the p ati en t. Th e o n se t o f ca n di do s is

sh o u ld th ere fo re le ad to a s ea rc h fo r the u n de rly ing c au s e. T he s ig n ifi ca n c e o f pr im ary an d

se co n d ary im m un e d efi ci en c y in re la tio n to c a n did a l i n fec tio n s i s des c rib ed in Ch ap ter 14. It is po i n te d o u t th at, a lth o u gh p rim a ry im mu n e de fi cien c ie s are re la tiv ely ra re, se co n d ary

def ic ie n ci es a re mu c h m o re co m m on . C li ni c al s itu atio n s co m mo n ly l ea di ng to a l o we rin g o f th e im m u ne de fe nc e s an d h en c e to o ral c an d id o si s are s h o wn in Tab le 4. 4.

It sh o u ld b e r em em be red th a t th e o n s et o f ca n di do s is i n a n ad u lt p a tien t r ep res en ts a c h an g e im ply in g a rel a xati o n o f th e n o rma l im mu n e de fe nc e s. It d o es n o t i mp ly a n in fe cti o n by

β€ fo rei gn β€™ ca n di da l str ai ns . A lth o u gh , i n th e pre sen t c on te xt, it is ev id en t th a t th e o ra l co n di tio n s in v ol vi ng Can d id a ar e th o s e mo re fu l ly di sc u s se d, i t m us t b e rem em bered th a t ca n di da l l es io n s m ay i nv o lve o th er are a s an d , in d eed, m ay b ec o me w id es pr ea d a n d

dis s em in a ted in a few d eb il ita ted pa tie n ts. T he c o ndi ti o n s ep ti c ae mi c ca n di da em ia m a y

in vo lv e th e l un g s, my o ca rd iu m, an d o th er v ita l or gan s, a n d is a lw a ys a di se a se o f very p oo r pro g no s is . A pa rt f ro m su c h w id es pr ea d i n fe cti on s , ho w eve r, i nf ec tio n o f th e sk in , h a ir fo ll ic le s, a n d na i ls is re la tiv el y c o mm o n . Lo ca l tis su e tra u ma m ay b e a si gn i fic a n t

pre di sp o si n g f ea tu re a n d, in th e ca s e o f c an d id al pa ro n yc h ia , ci rc u la tor y d is tu rba n ce s an d

dia b etes a re c o mm o nl y th e p rec ip ita tin g fa cto rs . Ther e i s so m e di ffe ren c e of o p in io n a s to th e in c id en c e o f Can d id a in th e va gi n a. M o s t in v es tig ati on s h a ve s ho w n a rel ati ve ly lo w

in ci de n ce i n n o n- pre gn a nt pa tie n ts , b u t a h igh o ne (o f th e o rd er o f 50 pe r c en t) i n pa tie n ts du rin g l ate pre gn a n cy . No t a ll th es e pa tie n ts b y any me a ns s h o w ev id en c e o f c lin i ca l

in fe cti on , b u t it is e vid en t th a t th is va g in a l po p ula tio n o f Can d id a mu s t a ct as a re s erv oi r f or

in fe cti on , p a rtic u la rly o f th e n ew -bo rn b ab y.

C. a lbi c an s n o rma ll y e xi sts i n th e o ra l ca vi ty i n th e fo rm o f ra th er la rg e y ea st -lik e c e lls

(bl as to sp o res ) th a t o c ca s io n a lly el o n ga te to fo rm germ tu be s (ps e ud o h yp ha e ). In th e i n ac tiv e sta te th e ye as t f o rm is pre do m in a n t, bu t w h en pa th ol o gi ca l ac tiv ity o cc u rs th e h y ph a l f o rm is mu c h mo re ev id en t. Th es e ps e ud o h yph a e ca n b e se en , n o t o n ly s up erf ic ia ll y o n th e o ra l mu c o sa , bu t al so p en e trate th e ep ith el iu m , as f ar as th e s tratu m g ran u lo s u m. Th e

me ch a n is m by w hi c h Can d id a ex ert a p a th o l o gic a l e ff ec t o n tis s ue s is n o t k no wn , a lth o u gh i t ha s be en de mo n s trate d th a t p ro tea se s an d ex trac el lul ar pro te in s ma y be p ro du c ed by th e mi cro -or ga n is ms a n d th a t th es e c an in d uc e s kin l es io n s i n th e a b se n ce o f th e o rg an i sm s

th e ms el ve s.

Table 4.4 Predisposing factors for oral candidosis Fact or

Descr ipt ion

Ph ys i ol o gi ca l

Ol d a g e, i nf an c y, pre gn a n cy

Loc a l ti ss u e trau m a

Mu c o sa l irri tati o n, de nta l ap pl ia n ce s, po o r o ra l

An tib io ti c th e ra py

Bro a d sp ec tru m (l oc a l o r sy ste mi c )

Co rtic o ste ro id th era p y

To pi ca l, sy ste mi c , an d in h a le rs

Ma ln u triti o n

Hae m ati ni c de fi ci en c ies β €”hi gh -ca rbo h y dra te die t

Im m un e d efe c ts

AIDS

En d o cri n e d is o rd ers

Dia be te s m ell itu s , A dd is o n' s di se as e, hy po th yro i di sm

Ma li gn a n ci es

Leu ka em ia s , ag a nu l o cy tos i s

Sa li va ry gla n d

Ir rad ia tio n , S jΓ¶g re n' s sy nd ro m e, xe ro ge ni c dru g s

hy po fu n c tio n

hy gi en e

Table 4.5 The laboratory diagnosis of oral candidosis O ral les io n

In ves tiga tio n * Sw ab

Sm ear

O ral

rin se

Bio ps y

Ps eu d o me mb ra no u s

+

Β±

+

-

Ery th em ato u s

+

Β±

+

-

Hyp erp la sti c

Β±

Β±

-

+

An g ul a r c h eil iti s

+

+

-

-

Can d id a -as so c ia te d de n tur e s to ma titi s

+

+

+

-

(pa la te an d de n tur e)

* + , Us ef u l; -, in ap pr op ri ate ; Β± , ma y be h el pf u l.

Laboratory investigations for the diagnosis of oral candidosis Ap pro p ria te la bo ra to ry te s ts a re o u tli ne d in Tab le 4. 5 an d h a ve al rea d y b ee n di sc u s se d in

Ch ap ter 2. A sw a b, mo is te ne d wi th s teri le sa l in e if n ec es sa ry , is w ip ed a lo n g the s u rfa c e o f

th e le s io n a n d p la c ed in a su ita b le tran s po rt me di um. Th e s am p le sh o u ld b e se n t pro m ptl y to th e m ic ro bi o lo gy la b or ato ry fo r cu ltu re a n d s e ns i tivi ty. A s me ar o f th e l es io n m a y a ls o b e

us e fu l a n d fo a m pa ds a re u se d in s o me c en tre s to sam pl e o ral l es io n s fo r Can d id a sp ec i es . A n or al rin s e, wi th a ph o s ph a te-bu ffe re d s a li ne s o lu tio n , w il l de te rm in e th e p res e nc e o f Can d id a sp ec ie s an d a ls o pr ov id e th e c li ni c ia n w ith a qu a n tita ti ve a ss es s me n t o f th e c an d id al c o un t.

Th is h el ps to d if fer en tia te be tw een co m me n sa li s m a nd o pp o rtun i sti c in fe cti o n s. B i op s y o f o ral leu k op la ki a is es s en tia l an d w il l d em o n stra te Can d id a -as so c ia te d le si o n s, s u c h as c h ro n ic hy pe rpl as tic c a n did o si s (c an d id al le u ko pl ak ia ).

Hae m ato lo g ic a l te sts i nc l ud in g a fu l l b lo o d co u n t, e sti ma tio n s o f s e ru m f err itin , v ita mi n B 1 2 ,

fo la te ( an d re d ce ll fo l ate ), a n d a b lo o d gl u co s e tes t s ho u l d b e u nd erta ke n fo r al l c a se s o f

per si se n t o ra l c a n did o si s , pa rtic u la rly if a n u n de rly in g sy ste mi c co n d itio n i s su s p ec ted . Ora l ca n di do s is a ss o c ia ted w ith H IV in fe cti on is d is cu s se d la ter in th is c h ap ter.

Clinical classification of oral candidosis

Th e c li n ic a l p res en ta tio n o f o ral c a nd id o si s is v ari ab le . Th e o rig in a l c la s si fi ca tio n o f o ra l

ca n di do s is b y Leh n er in th e 1960s re co g ni z ed tw o m ajo r gro u ps : ac u te ( ps eu d o me mb ran o u s an d

atro p hi c ) an d c h ro n ic (a tro ph ic a n d h ype rp la sti c). It is , h o we ve r, n o w rec o gn i z ed th at th is

P. 37

or igi n al c la s si fic a tio n h a d l im ita tio n s , an d th e curre n tly ac c ep ted o n e (Tab le 4. 6 ) is b a se d o n cl in ic a lly re le va nt term in o lo g y. P se u do m em bra n o u s ca n di do s is c an be β € ac u teβ €™ bu t,

pa rtic u la rly in im mu n o c o mp ro mi se d pa tie n ts , i s of ten β€ ch ro n ic β €™. β € Atro p h ic β €™ is n o t an en tir el y a pp ro pri a te te rm in th e co n te xt o f o ral c an d id o si s as th e mu c o s a m a y ap pe a r re d as a re su l t o f e ith e r a tro ph y or in c rea s ed va s cu l ari ty, a n d th i s di ffe ren ta tio n i s di ffi cu l t to

ma ke c lin i ca ll y. A n gu l ar ch e il itis , d en tu re sto m a titis , a nd β€ me dia n rh o m bo i d g lo s s itis β €™

ma y b e a res u lt o f c o mb in ed ba c teri al a nd f un g al a eti o lo gy a n d a re th ere fo re c la ss if ie d a s

β€ Can d id a -as so c ia te d β€™ les i on s . S ys tem ic m u co c u tan e o us ca n di do s is f req u en tly h as o ra l

ma n ife sta tio n s a n d th e se a re cl as s if ied a s β€ se co n d ary o ra l c a n did ia s es β€™ (se e Tab le

4. 6).

Table 4.6 The classification of oral candidosis

Pri ma ry o ral c a nd id o se s (gr ou p 1)

Ac u te: ps eu d o me mb ra n ou s , e ryth em ato u s

Ch ro n ic : p se u do m em bra n o u s, ery th em ato u s , h ype rpl a sti c

Can d id a -as so c ia te d l es io n s : Can d id a- as so c ia te d d en tu re -in du c ed s to ma titis , a n gu l ar

ch e ili tis , me di an rh o mb o id gl o ss iti s

Se co n d ary o ra l c a n di do s es (gr ou p 2)

Ora l ma n if es tati on s o f sy ste mi c mu c o c uta n eo u s ca n dido s is

Antifungal agents used to treat oral candidosis

To pi ca l an d s ys te m ic an ti fu n ga l ag en ts s ui tab le fo r th e tre a tm e nt of o ra l ca n di do s is a re su m ma riz e d in Tab le 4. 7.

Th e p o ly en e an tif u n ga l ag en ts , n ys tati n a nd a mp h o teri ci n B , ar e w e ll es ta bli s he d an d

rel ati vel y f ree fro m s id e- eff ec ts wh e n u se d lo c al ly. Th ey a re a va il a ble i n va rio u s fo rm s , su c h as l oz e n ge s, p as til le s, cre am s , an d s u sp en s io n s . Unf or tu n a tel y, p ati en t co m pl ia n c e i s o ften

po o r w ith th e se p rep ar ati o ns , w h ic h m ay ta ke a wh i le to di ss o lv e i n th e mo u th (f or ex am pl e, pa sti lle s a nd l o ze n ge s) a nd h a ve a β€ dis tin c tiv e β€™ ta s te. M ic ro b ia l r es is tan c e to po l yen e

an tif u ng a ls is , h o we ve r, u n co m mo n . Th e n ew e r a z o les h a ve ve ry us e fu l pro p ertie s , al th ou g h

res is tan c e is ra th er mo re c o mm o n ly me t a nd m a y b e pro bl em a ti c in th e fu tu re , pa rtic u la rl y i n th e im m u no c o mp ro m is ed pa tie n t. (C. kru s ei an d a n d C. gl ab ra ta ar e u s u al ly res i sta n t to

flu c o n az o le . ) Th e lo c a lly a cti ve ag en t, m ic o n az o l e i s av ai la bl e as an o ral g el o r c rea m . As

we ll a s its a nti fu n ga l ac tiv ity, i t h a s a l im ite d an ti ba cte ria l ef fec t. Th e s ys te m ic al ly ac tin g

az o le s, itra co n a z ol e, f lu c o na z o le , an d ke to co n a z o le, are o f va lu e in g en e ral iz ed a n d sy ste mi c ca n di da l i n fe cti on s . Th er e m a y b e c h a n ges i n li ve r fu n c tio n , p arti cu l arl y w i th ke to c o n az o l e,

an d pa tie n ts on th e se d ru gs m u st be c are fu ll y m o n ito re d. S ys tem ic th e rap y is ge n era ll y n o t ne c es sa ry in th e ma jo ri ty o f pa tie n ts s u ff eri ng f rom or al ca n di do s is b u t in so m e co n di tio n s , su c h a s ch ro n ic h y per pl as tic c an d id o si s, its u se i s bec o m in g w ide s pre ad a n d is o fte n ver y

eff ec tive . A z ol es h a ve a n u n fa vo u rab le p ha rm ac o ki n etic i n tera c ti o n w ith a n u m be r o f d ru gs

in cl u di n g an ti -co a gu la n ts (fo r ex am pl e, w a rfa rin ), te rfe n ad in e , ci cl o sp o rin , s tati n s, ci s ap rid e, an d as te mi zo l e. C hl o rh ex idi n e so lu ti o n h as a n tib ac teri a l a n d an ti ca n di da l eff ec ts.

Table 4.7 The treatment of primary oral candidoses in immunocompetent patients To pic al th er apy

Sy stem ic t h erap y

Ps eu d o me mb ra no u s , ery the m ato u s h yp erp la sti c ca n di dos is Am ph o ter ic in l o ze n ge s (10 m g)

Fl uc o n a zo l e, 50 β€“100 mg d ai ly fo r 2β€“ 3 w ee ks

or

or

Nys tati n pa s ti ll es (100 000 u n i ts )

Itra c o n az o le 150 m g da il y f o r 2

Dis so l ve sl o w ly in m o u th , a fte r m ea ls ; u s e 4

we ek s

ti me s da il y; u su a l c o u rs e i s 1β€“4 w ee ks . Can d id a -as so c ia te d d en tu re sto m a ti tis* Am ph o ter ic in o r n ys ta tin (as a b ov e) β€”rem o ve de n tur es If c o mp li an c e po o r:

Sy ste mi c th era py is o c c as io n a ll y req ui red (a s a bo ve )

Mi co n a z ol e ge l a p pli ed to p al ata l su rf ac e o f den tu re

4 tim es d ai ly fo r 1 β€“4 w ee ks Mi co n a z ol e la c qu er β€

Ch lo rh ex id in e 0. 2% ri n se , 4 tim es d ai ly (do no t u s e w ith n ys ta ti n )

Can d id a -as so c ia te d a n gu l ar ch e il itis* Nys tati n cr ea m; ap pl y to co rn e rs o f m o u th 3β€“4

Sy ste mi c the ra py ma y be re qu ir ed

ti me s da il y, u n til re so l uti o n

If m ic ro bi al re po rt n o t a va il a ble o r in ca s e of m ixe d in fe cti o n: β€΅

Mi co n a z ol e cre a m (o r g el ); a pp ly 3 β€“ 4 ti me s da il y to an g le s

* In tr ao ra l res er vo irs o f Can d id a sh o u l d b e el im in ate d fro m pa tie n ts wi th an g u la r ch e ili tis a n d d en tu re s to m a titis .

β€ Mi co n a z ol e la cq u er (Du mi c oa t, 50 m g/ g) ca n b e a ppli ed to f ittin g s ur fa ce o f

up pe r de n tur e a fte r th o ro u gh c le a ns i ng a n d dryi n g β€” se e m a n uf ac tu rer sβ €™ in stru c tio n s .

If Sta ph yl o co c c u s au re u s o n ly is o la ted , fu si di c ac i d c re am i s req u ire d (se e Tab le

6. 5 fo r f u rth er de tai ls ).

Pseudomembranous candidosis

Un til re ce n tly, th i s co n d itio n , c o ll o qu ia ll y kn o wn as β€ th ru s h β€™, w a s al mo s t a lw a ys

des c rib ed a s β€ ac u te β€™ ps eu d om e mb ran o u s c an d id os i s, a c co rd in g to Le hn e r's o rig in a l

cl as s ifi ca tio n , e ven tho u g h ma n y le si on s w e re ma n ife s tl y ch ro n ic i n n atu re . Rec o n s ide ra tio n of th e or al le s io n s o f c a nd id o si s in H IV- rel ate d c on di tio n s an d o the r

im mu n o su p pre s si ve sta tes h a s le d to the ro u tin e u s e o f th e te rm β€ ac u teβ€™ be in g

ab an d o ne d. T h e p s eu do m em br an e c on s is ts o f a n e two rk o f ca n di da l h yph a e co n ta in in g des q ua m ate d ce ll s, m ic ro -or ga n is ms , fi bri n , in fl am mato ry c ell s , an d de br is . Th is

P. 38

ps eu do m em b ran e li es o n th e s ur fa ce o f th e tis su e a nd c a n di da l h y ph a e p en e trate su p erfi c ia lly i nto th e e pith e li um to pro v id e a n c ho ra g e.

Fig. 4 .4 P se u do m em bra n o u s ca n di do s is ( th ru s h ).

CLINICAL FEATURES Cli n ic al ly, th i s ap pe ar s as a th ic k, w h ite c oa tin g o r s e rie s of p atc h es o n th e af fe cte d ti ss u e

(Fi g. 4. 4). Th e ps eu d o me mb ran e c an be w ip ed a wa y and , si n ce th e mo re s u pe rfi ci al la ye rs o f th e e pith e li um m a y b e i n cl u de d, a re d an d bl ee di n g ba s e i s le ft b eh in d . Th is m a y b e

co n s id ere d a re a so n a bl e p rel im in a ry cl in ic a l tes t to d is tin gu i sh thr us h f ro m o th e r w h ite

les io n s o f th e m u co s a a nd co n f irm ati o n ma y al so be o bta in e d b y ta ki n g a d ir ec t s me ar fro m th e le s io n . Th is ma y be fi xe d b y g en tl e h e at an d imm ed ia tely s tai n ed , us i ng th e pe rio d ic

ac id β€“Sc h if f (P A S ) re ag en t. Th e h yp h ae a re rea di ly id en tif ie d u n d er th e mi c ro sc o pe . A s wa b sh o u ld b e ta ke n an d s en t to th e m ic ro bi o lo gi ca l la bo ra to ry fo r c u ltu re a n d s e ns i ti vi ty.

An y o f th e mu c o s al s ur fa ce s o f th e m o u th ma y be a ffe cte d by thr us h , a s ma y the p o ste rio r ph a ryn ge al w a ll . In th is la s t in s tan c e, th e c o n ditio n mu s t b e ta ke n p arti cu l arl y s e rio u s ly, si nc e ex ten s io n in to th e o es o ph a gu s a n d tr ac h ea i s po s si bl e a n d ma y pro v e f ata l. T hi s is

lik el y to o c c ur o n ly in th e se ve rel y d eb il ita ted patie n t, al th ou g h th e pre -exi sti n g c o n di tio n ma y n o t h a ve be en pre vio u s ly re co g ni z ed . In a fe w pa tie n ts la ryn ge a l c a nd id o si s o f a l es s ac tive f or m ma y b e as s o ci ate d wi th o ra l le si o n s. T hi s h as b ee n n o ted in p a tien ts ta kin g

an tib io tic s a n d i n th o se ta kin g o ral s tero i d pre pa ra ti o n s. In g en e ra l, th e la ryn g ea l d is c o mfo rt an d h o ars e ne s s res o lv e wi th trea tm en t o f th e o ra l les i on s .

MANAGEMENT Th e tre a tm e nt fo r p se u do m em bra n o u s ca n di do s is i s sum m ari z ed in Tab le 4. 7 . It is e vid en t, ho w e ver, th a t tre atm en t o f s ym pto ms a l on e is i n su f fic ie n t a nd th a t s teps m u s t be tak en to dete rm in e an y pre di sp o s in g ca u se . In s o me p ati en ts th e e ss e nti al u n de rlyi n g f ac to r i s

un d ers to o d a n d u n de r re as o n ab le c o ntro l , bu t i n o thers , in pa rtic u la r th o s e pa tie nts w i th AIDS , th e de bil ita tin g n atu re o f the c o n di ti o n ma y be ex pre s se d in vi rtu al ly c on ti nu o u s ca n di do s is o f th e m o uth . In th e se ci rc u ms tan c es i t is n e ce s sa ry to ma in ta in l o n g- te rm

an tif u ng a l tre atm en t. A pa rtic u la r d if fic u lty a ris es i n th e c a se o f pa tie n ts w i th le u kae m ia o r

oth e r n eo p la sti c di se as es u n d er tr ea tme n t w ith c ytoto xic a n d o the r d ru gs . In th es e ca s es n o t on l y th e u n de rlyi n g dis e as e pro c es s , bu t a ls o th e th era py , ten d to pre di s po s e to w a rds

ca n di do s is .

Erythematous candidosis

Th is c an be β€ ac u te β€™ or β€ ch ro n ic β€™ de pen d in g o n th e du ra tio n o f the o ra l les i on s .

Ac u te e ryth e ma to u s ca n di do s is w a s fo rm erl y k n o wn as β€ ac u te a tro ph i c ca n di do s is β€™ or

β€ an tib io tic s o re m ou th β€™ an d u su a ll y o c c u rred a s a res u lt o f m ed ic al trea tme n t in v o lvi n g

an tib io tic s o r s te ro id pre pa ra tio n s . A si gn i fic a n t n um b er o f pa tie n ts (ad u lts a n d c h il dre n ) a re no w u s in g s tero i d i n ha l ers fo r pu l mo n a ry di se a se a nd th e se c an p re di po s e to th e

dev el o pm en t o f ei the r e ryth em a to us o r ps eu d o me mb ra nou s c a nd id o si s ( Ch ap ter 14).

CLINICAL FEATURES Ac u te e ryth e ma to u s ca n di do s is res e mb le s β€ th ru s h β€™ wi th ou t th e o ve rly in g

ps eu do m em b ran e . Cl in ic a lly , th e mu c o s a i n vo lv ed is r ed an d p ai n fu l. T hi s is , in fa ct, th e o nl y

var ia n t o f o ra l ca n di do s is i n w h ic h pa in an d di s co mfo rt i s ma rke d. A s w o u ld b e e xp ec ted fro m th i s de sc ri ptio n , th e ep ith el iu m is th i n an d a tro phic w i th ca n di da l h yph a e em be dd ed

su p erfi c ia lly i n the e pi the li u m. T he c o n diti o n ma y fo ll o w o r b e co n c u rre nt wi th th ru sh , o r ma y oc c u r a s the o n ly m an i fes ta tio n o f th e in f ec tio n . As ha s b een po in te d ou t ab o ve, th i s fo rm o f ca n di do s is is c o m mo n i n pa tie n ts w ith th e su p pre ss ed i mm u n e fu n cti o n o f A IDS a s we ll a s pa tien ts u n de rg oi n g pro l o n ged a n tib io tic o r ste ro id th era py . It is n o t a lw a ys p o ss ib le fo r

pa tien ts o n s te ro id s to di s co n tin u e trea tme n t a n d th e c on ti n uo u s l ow e rin g o f th e res i sta n ce of th e ti s su e s to in fe cti o n ma ke s trea tm en t b y th e u su a l me an s rel ati ve ly in ef fe cti ve.

MANAGEMENT To pi ca l an tif u ng a l th e rap y (Tab le 4. 7) is u s u al ly ef fec tiv e. E ry the ma to u s ca n di do s is a s a

res u lt o f an ti bi oti c th era py u su a ll y r es o lve s af ter ce ss a tio n o f the c o u rse , al th o ug h th is m a y ta ke s o me tim e . In a pa tie nt kn o w n to be s u sc e ptib le to th is co n d itio n an d fo r wh o m it is

ne c es sa ry to pre s cri be fu rth e r a n tibi o tic trea tme n t, it w o u ld b e wi se to a d d o ra l an ti fu n ga l

tr ea tme n t to th e r eg im e. In th e a u th o rs β€™ exp eri enc e, a m in o ri ty o f th es e pa tien ts a pp ea r to de vel o p a ch ro n i c gl os s o dyn i a (bu rn i n g to n g ue ) th a t pe rs is ts de sp ite th e lin g u al m uc o s a

ap pe ari n g n o rm a l a n d af ter e li mi n ati o n o f c a nd id a l i n fec tio n ( se e Ch ap ter 17 ). Pa tie n ts w ho

reg ul a rly req u ire a s tero id i n ha l er sh o u l d b e in s truc te d to r in s e th e ir m o u th w ith w a ter a fte r us e . Us e o f a s pa c er de vi ce fo r the i nh a le r m a y b e ne c es sa ry ( Ch ap ter 14, Fi g. 14. 2).

Hyperplastic candidosis

Ch ro n ic h yp erp la sti c ca n di do s is i s al s o kn o w n as β € ca n di da l l eu k op la ki a β€™ an d is

as so c ia te d w i th ch ro n ic i n fe cti on of th e o ral mu c o sa

P. 39

wi th ca n d ida l sp ec i es , u su a ll y C. a lbi c an s . Th is c h ro n ic fo rm o f c an d id os i s i s c on s id ere d to be

a p re ma li gn a n t le si o n an d i s mo re l ike ly to o c cu r in p ati en ts w h o sm o ke . Cu rre n tl y, th e re i s a gre at d ea l o f co n tro ve rs y c o n ce rn in g th e ro le o f Can d id a sp ec i es in th e d ev el op m en t o f

epi th el ia l n e o pla s ia . Th e pre m al ig na n t n a tur e o f c an d ida l le u ko pl ak ia is d is c u ss ed f ul ly in Ch ap ter 10.

CLINICAL FEATURES Ch ro n ic h yp erp la sti c ca n di do s is cl a ss ic a lly p res en ts a s a fix ed w h ite pa tc h at the

co m mi ss u res o f th e mo u th . Oth e r a rea s o f th e m o uth , p ar tic ul a rly th e p al a te , m ay be

aff ec ted b ut the to ng u e is le s s co m mo n l y i nv o lve d. C li ni c al ly, th e le s io n (s ) a pp ea r a s ra is ed , irre gu la r wh i te p la qu e s, w h i ch m a y b e β€ sp ec kl ed β €™ or n o du la r in a pp ea ra n ce . Th er e i s

freq u en tly e vid en c e of o ra l ca n di do s is e ls ew h ere , pa rti cu l arl y o n th e pa la te o f p ati en ts w ith fu ll de n tur es a n d/o r an g ul ar c he il iti s.

MANAGEMENT

Defi n itiv e d ia g no s is d ep en d s o n h is to pa tho l o gi ca l exa m in a tio n o f a bio p s y, w h ic h is e ss e nti al to co n fi rm th e pre s en c e o f Can d id a sp ec i es a n d to a s ce rta in th e de gre e o f e pi th e li al

atyp ia β€” if an y. M a n ag em en t in vo lv es th e era d ic ati on o f pre di s po s in g fa cto rs , s u ch a s

sm o kin g a n d i n sti tuti o n o f a p pro p ria te a n tif un g al th era py β€” eith e r to pi ca l o r s ys tem ic . A n

in iti al c ou rs e o f f lu c o na z o le fo r 2β€“3 w ee ks c an be co m bi n ed w ith to p ic al th era p y (s ee Tab le 4. 7), w hi c h ma y be req u ire d o n a lo n ge r -te rm ba s is . A tten tio n m u st be gi ve n to de n tur e hy gi en e an d o th er fa cto rs p red is po s in g to o ra l ca n di do s is , ei the r l o ca l o r s ys tem ic .

Hae m ato lo g ic a l i n ves tig a ti o n s sh o u ld b e u n d erta ke n to ch e ck fo r h ae ma tin i c de fic ie n ci es s u ch as i ro n de fic i en c y, a nd a b lo o d gl u co s e tes t i s a d vis a bl e to exc lu d e dia b etes . E xc is i on o f

per si ste n t le si o n s or th o se w ith a s ig n ifi ca n t d eg ree o f d ys pl a si a is p refe ra bl y c a rrie d ou t wi th a la s er. Lo n g- te rm fo l lo w -up i s es se n tia l.

Candida-associated, denture-induced stomatitis

Th is is b y f ar th e m o st co m mo n fo rm o f o ra l ca n di dos is a n d is a ls o re fer red to a s ch ro n i c eryth e ma to u s ca n di do s is (o r, m o re co l lo q ui a lly in the p as t, a s β€ den tu re s o re

mo u th β€™ β€”a m is n o m er b ec a u se th e co n d itio n i s n ea rly a lw ay s pa in l es s) . Th is f or m o f den tu re s tom a titis r epr es en ts th e en d- res u lt o f se co nd a ry c a nd id a l i n fec tio n o f tis s ue s ,

tr au m ati ze d by a de n tal a pp lia n c e. Th i s n ee d n o t b e il l -fittin g , a lth o u gh m o st of th e de ntu re s in vo lv ed a re ol d. T h e ap pl ia n ce i s co m mo n l y b u t n o t ex cl u si vel y a d en tu re β €”an o rth o d on ti c pla te ma y pro d u ce a s im il ar res u lt.

CLINICAL FEATURES Th e c li n ic a l p ic tu re is o f m a rke d red n es s o f th e pal ata l m u c o sa c o ve red by th e ap pl ia n ce (th e equ i va le nt ma n di bu l ar de n tu re -bea ri n g a re a do es n o t a p pe ar to be co m e in vo l ved ) o f te n w ith a sh a rp ly de fin e d edg e ( Fi g. 4. 5). If a re li ef ar ea is pre s en t o n th e de n tu re , th ere m ay b e a co rre sp o n di ng a re a of s po n gy β€ gra n u la r lo o ki n g β€™ ti s su e , bu t o th erw i se th e af fecte d

mu c o sa i s sm o o th. N ew to n in tro d uc e d a c la s si fi ca tion f or th is c o n diti o n : typ e 1, ch a ra cte riz ed by pi n - po in t h y pe rae mi a o f th e pa la ta l m u c os a ; typ e 2 (th e mo s t c o mm o n typ e), i n w hi c h th e re is d iff us e er yth em a li mi te d to th e a re a o f the de n tu re ; ty pe 3, in w hi c h th ere is a

hy pe rpl as tic n o d ul a r re ac tio n o f th e p a la ta l mu c o s a (Tab le 4. 8). Th ere i s rar ely a n y c o mp la in t of s o ren e ss b y th e pa tie n t in s p ite o f th e in ten s e ery the m ato u s ap pe a ran c e o f th e tis su e s.

Can d id a- as so c ia te d d en tu re sto m a ti tits , be c au s e o f its restri cte d d is trib u tio n to th e de n tur e -

bea ri n g a re a, i s so m eti me s mi sta ke n fo r an al le rgi c rea c ti o n to a cr yli c res in , a c o n di tio n th at is re la ti ve ly ra re.

Fig. 4 .5 Ca n di da -as so c ia te d, d en tu re -in du c ed s to ma titis ( chro n ic e ryth e ma to u s

ca n di do s is ) a ff ec tin g the a rea o f m uc o s a co ve re d b y a p a rtia l de n tur e.

MANAGEMENT By ta kin g s wa b s or c arr yin g o u t di rec t in o c u la tio n fro m th e fi tti n g s u rfa c e of th e de ntu re

in vo lv ed it is p rac tic a ll y al w ay s po s si bl e to is o late a h e av y g ro w th o f Can d id a. S wa b s fro m

th e m u co s al s u rfa ce m ay a ls o p ro vid e a pro l ifi c grow th . B io p sy is ra rel y i n dic a ted bu t ma y b e

do n e in p ers is ten t o r aty pi ca l ca s es to c o n firm th e di ag n o si s. H is to pa th ol o gy o fte n sh o w s fe w ca n di da l h y ph a e w i thi n th e ep ith el iu m .

Si n ce th ere a re two co n tri bu tin g fa c to rs to th i s con d itio n (tis su e tra u ma a n d i n fe cti on ),

tr ea tme n t mu s t b e d ire c te d at bo th i n o rde r to o b tai n ra pi d r es o lu tio n o f sy mp to ms . De ntu re

hy gi en e is a n im po r ta n t pa rt o f th e tre a tm e nt tog eth e r w ith th e el im in a tio n o f trau m a by th e ad o ptio n o f su i tab le p ro sth eti c tec h n iqu e s, th e u s e o f tis s ue c o n di ti o n ers b ei n g pa rtic u la rl y val u ab le a s an in iti al tre atm en t. Th e u se o f an an tif u ng a l c re am o r mi c on a z o le

gel o n th e fi tti n g s u rfa c e of th e ap pl ia n ce m ay pr ove a us e fu l ad ju va n t to tre atm en t a n d he lp s to s pee d th e re s ol u tio n o f the a bn o rm a l ti ss ues (Tab le 4. 7 ). Ca ref ul a n d reg u la r

cl ea n in g o f a ll s u rfa ce s o f th e de n tur e i s al s o o f g rea t i mp o rtan c e. A la cq u er co n ta in in g mi co n a zo l e is n o w av ai la bl e an d pa in te d o n to the fittin g s ur fac e o f th e u pp er de n tur e.

Table 4.8 Newton's classification of Candida-associated denture stomatitis Ty pe

Descr ipt ion

1

Pi n -po in t h y pe rae mi a

2*

Diff us e ery th em a, l im ite d to th e fi tti n g s u rfa c es of de n tu re

3

No du la r a p pe ara n c e o f pa la ta l m u c os a

* Typ e mo s t c o mm o n ly se en .

P. 40

Fig. 4 .6 A ng u la r c h ei li ti s w ith c an d id al in vo l ve me nt.

Candida-associated angular cheilitis

An g ul a r ch e il itis p res e nts a s er yth em a an d c ra ck in g at th e a n gle s o f th e m o uth a n d is

co m mo n ly a Can d id a -as so c ia te d l es io n ( Fi g. 4. 6 ). Th e pa th og en e si s, clin ic a l p re se n ta tio n ,

an d ma n a ge me nt o f a n gu l ar ch e ili tis a re fu ll y d es c rib ed in Ch ap ter 6. Th e tr ea tme nt o f Can d id a- as so c ia te d a n gu l ar ch e il itis i s su m m ari z ed in Tab le s 6. 4 an d 6. 5 .

Median rhomboid glossitis

Me di an r ho m bo i d glo s s itis i s c la ss if ie d a s a β€ Can d id a -as so c ia te dβ €™ les i on an d

ch a rac te ris tic al ly pr es en ts a s an a re a of d ep ap il latio n o n th e mi dl in e o f th e do rs u m o f th e to n g u e, im m ed ia tel y i n fro n t o f the c ir cu m va lla te pa pi ll ae . Th e le si o n is c la s si ca l ly

β€ rho m bo i d -sh a pe d β€™ β€” he n ce th e n am e. Its s u fa ce m ay b e red , w h ite, or ye ll ow in

ap pe ara n c e. Th i s le si o n is fu rth e r d is cu s s ed in Ch ap ter 6 .

Secondary oral candidoses: chronic mucocutaneous candidosis syndromes Ap ar t fro m th e mo re c o mm o n fo rm s o f o ra l ca n di do s is, th ere i s a sp ec tru m o f c o n di tio n s in wh i ch c a n di do s is o f th e o ra l c a vi ty , th e s ki n , an d o th er str uc tu res s u c h as th e fi n ge rn ai ls

ma y o c c ur , w ith o r w i tho u t a s so c ia tio n w i th o the r gen e ral iz e d d is ea s e pro c es s es . Th is g ro u p of c o nd iti o ns i s ge n era ll y k n ow n as c h ro n ic m u co c u tan e o us c a n di do s is (CM C ). Th e o ral les io n s i n CM C m a y b e in i ti a lly th ru sh -lik e, b u t even tu al ly res e mb le le si o n s of c h ro n ic

hy pe rpl as tic ca n di do s is . Th e s kin le si on s m a y i n cl ud e w ide s pre ad a n d dis fi gu ri n g l es io n s o f th e fa c e a n d sc a lp . Gra n u lo ma to u s le si o ns o f th e lip s ma y al so o cc u r, s im il ar to th os e th at aff ec t th e sk in . A va ri an t ( fa mi lia l ch ro n i c mu c o cuta n eo u s ca n di do s is ) is ge n eti ca ll y dete rm in ed a n d i s tra ns m itted a s a n au to s o ma l rec e ss iv e c o n di tio n . In th e

ca n di do s is β€“en do c rin o p ath y sy n dro m e wh i te c a nd id a l pl aq u es i n th e m o u th an d c an d id al in fe cti on s o f th e n a il s ar e a s so c ia ted w ith di so rd er s of th e pa ra th y ro id s or a dre na l s. A cl as s ifi ca tio n o f c h ro ni c mu c o c uta n eo u s c an d id os i s sy nd ro m es is s h o w n in Tab le 4. 9.

Table 4.9 Chronic mucocutaneous candidosis (CMC)

syndromes Ty pe

Featu r es

Fa mi li al CM C

Fi rst de ca de β€” per si ste n t ca n di do s is : mo u th , n ai ls ,

Diff us e CM C

Fi rst 5 ye ars β €”ch ro n ic c a n did o si s : m o uth , n a il s, ski n ,

Can d id o si s -

Hyp o th y ro id is m, h y po a dre n oc o rtic i sm , a nd m il d

(Can d id a gra n u lo m a)

en do c rin o p ath y syn d ro me

Can d id o si s -th y mo m a syn d ro me

ski n . Iro n de fi ci en c y.

ph a ryn x. S u s ce ptib le to ba c teri al in f ec tio n s

ch ro n ic h ype rp la sti c ca n di do s is i nv o lvi n g th e m o uth

Hae m ato lo g ic a l d is o rde rs .

Ps eu d o me mb ra no u s o r h ype rp la sti c ca n di do s is : m o u th, ski n , n ai ls .

* Ta ken fro m B ag g, T . et a l . (1999). Es s en tia ls o f m ic ro bi ol o gy fo r d en ta l stu d en ts. Oxf o rd Un i ver si ty P re ss , Ox fo rd .

MANAGEMENT Ch ro n ic m uc o c u ta n eo u s c an d id o si s is di ffi cu l t to tre at a n d in m a n y c as e s red u cti on , b u t n o t

era di ca tio n , o f Can d id a sp ec i es is a tta in ab le . P red is po s in g fa c tor s fo r o ra l ca n di do s is s h o u ld ,

ho w e ver, be el im in a ted (s ee a bo ve ) w h er eve r p o ss ib le . Tre atm en t i s w ith l on g -te rm s ys tem ic an tif u ng a l th e rap y s u c h as f lu c o na z o le a n d ma y n ee d to b e pre sc ri be d a t d o se s h ig h er th a n th e n o rm al th era pe u tic ra n ge . Reg u la r m o n ito ri ng o f li ver fu n cti o n is es s en tia l.

Viral infections

A nu m be r o f vi ral i nf ec tio n s m ay o cc u r i n a nd a ro u nd th e m o uth a n d ma n y o f th e se p res en t

as ve s ci u la r le s io n s o f th e o ra l m u co s a . Th e ves ic les s h ow a ma rk ed ten d en c y to b rea k do w n wi th th e p ro du c tio n o f u lc ers .

Dia gn o s ti c tes ts fo r v ira l in fe cti o n s h a ve a lre ad y be en d is cu s s ed in Ch ap ter 2. Th e i n iti al

dia g no s is o f vi ral i nf ec tio n i s, h o we ve r, u su a ll y on th e b as is o f cl in i ca l pre se n tati on . T is su e cu ltu re s ys tem s tak e a t l ea s t 24 h o u rs to c on f irm th e pre se n ce o f a vir us a n d se ro lo g ic al te s ts tak e 10 β€“ 14 da ys . Tab le 4. 10 sh o w s th e pri n ci pa l v iru s es th at a ff ec t th e o ral a n d

per io ra l r eg io n . Of th es e, th re e are h u m an h e rpe s vi rsu s es ( Tab le 4. 11). Th ere a re cu rr en tly eig h t h u ma n h erp es v iru s es ( Tab le 4. 11) a nd a ll a re en ve lo p ed , ic o sa h ed ra l v ira l pa rtic le s , wi th a do u b le- stra n de d DNA ge n o me .

Th e h u m an he rp es vi ru se s po s s es s the p ro pe rty o f laten c y (tha t i s, th e y ca n re m ai n do rm a nt

an d be co m e rea c tiva ted l ate r). Th e c lin i ca l di se as e c au s ed b y th e v iru s m ay di ff er d ep en d in g on wh e the r th e in f ec tio n i s p ri ma ry or s ec o nd a ry (th at is , a rea c tiva tio n ).

Table 4.10 Principal viruses that affect the oral and

perioral region Herp es s im p lex vi ru s

Va ric e lla z o ste r vi ru s Ep s te in β €“Ba rr viru s

Gro u p A Co xs a ck ie viru s es Pa ra my xo vi ru se s

Hu ma n p ap il lo m a vi ru se s

Table 4.11 Human herpes viruses Herp es s im p lex vi ru s (typ e 1)

Herp es s im p lex vi ru s (typ e 2)

Va ric e lla - zo s ter vir us ( type 3) Ep s te in - Ba rr viru s (typ e 4) Cyto m eg al ov iru s (ty pe 5)

Hu ma n h er pe s viru s es 6, 7, an d 8 * * A ss o ci a te d w ith Ka po s i's s a rco m a .

P. 41

Herpes simplex virus infections Primary herpetic gingivostomatitis

Th e h e rpe s si mp le x viru s (H SV ) is a DNA vi ru s o f w hic h tw o m ai n g ro up s a re kn o wn in m an , to g eth e r w ith a n u m be r o f sl ig h tly mo d ifi ed tra n si tio n al typ es . He rp es s im pl ex, ty pe 1,

aff ec ts th e o ra l mu c o s a, p h ary n x, a n d s ki n w h ere as H erp es s im pl ex , type 2, p red o mi n an tly in vo lv es th e g en i tal ia . Th e le si o ns p ro du c ed b y th ese tw o typ es o f the v iru s a pp ea r to b e

ide n tic al , bu t th e re are s u gg es tio n s th at th e l on g -te rm c on s eq u en c es o f in fe c ti o n m ay be

dif fe ren t s in c e the typ e 2 vi ru s h as b ee n im pl ic ated in th e pro d u cti o n o f c er vic a l c a rci n o ma . Th e ty pe 1 v iru s is i mp li ca ted i n th e m a jo rity o f ora l an d fa c ia l les i on s .

Pri ma ry h erp eti c gin g iv os to m ati ti s is th e mo s t c o mmo n vi ra l i n fec tio n a ff ec tin g th e m o u th

an d it a ff ec ts pa tie n ts in tw o m a in a ge g ro u ps β €”you ng c h il dre n a nd y ou n g a du lts . In e arl y ch il dh o o d th e in fe c ti o n m ay be s u bc li n ic al . Th e in cub a tio n p eri od i s ab o u t 5 d ay s.

CLINICAL PRESENTATION

Th e p a ti en t w i th pri ma ry h erp eti c gin g iv os to m ati ti s ma y gi ve a h is tor y o f rec e nt exp o su re to a p a tien t w ith a h er pe ti c le si o n . Th e in iti al s ym pto ms a re o f ma la is e w ith tir ed ne s s, gen e ra liz e d mu s cl e ac h es , a nd , s o me tim es , a so r e thro a t. A t th is ea rl y s tag e th e

su b ma n di bu la r l ym ph n o d es a re o ften e n la rg ed an d tend er. Th is p ro dro m al p ha s e ma y be

exp ec ted to l as t f or a da y or tw o a nd i s fo ll o we d by th e a pp ea ra n ce o f o ral a n d, s o me tim es , ci rcu m o ra l l es io n s (Fi g. 4. 7 ). Gro u ps o f v es ic le s fo rm o n th e o ral m u co s a an d ra pi dl y b rea k do w n to pr o du c e s h a llo w u lc ers . A lth o u gh th e ve s ic le s m a y b e rel ati vel y sm al l, th e

bre ak do w n o f co n f lu en t g ro u ps m a y re su l t in th e fo rm a tio n o f la rge a rea s o f u l ce ra tio n . Th e dis tri bu tio n o f th e l es io n s i s v ar ia bl eβ€” an y o f the o ral m u co s al s u rfa c es m ay be i nv o lve d.

Ap ar t fro m th e ul c era ted a rea s , the w h o le o f th e o ra l m u c os a m ay b e bri gh t -red a nd s o re. In yo u ng c h il dre n , pa rtic u la rly , th ere m ay be a m ar ked gi n gi viti s th a t c lo s el y r es em bl es th a t o f ac u te l eu ka e mi a. It i s ev id en t th a t su c h a n a pp ea ranc e s ho u l d i n di ca te sc re en in g fo r

ha em a to lo gi ca l ch a n ge s . Ap a rt fro m th e in tra or al les io n s, th e re ma y be le si o n s of th e lip s

an d ci rc u mo ra l sk in , w h ic h , be c au s e o f th e rel ati ve sta bi li ty o f th e s ki n c om p are d to th e o ra l mu c o sa , m ay reta in a mu c h mo r e o b vio u s ly ve si cu l ar ap pe a ran c e. In a few c a se s , th e

pri ma ry in fe cti o n ma y be co m e wi de sp re ad a nd d is s em in a te d th ro ug h o u t th e bo d y, s o th at

en c eph a li tis , m en in g itis , a n d o th e r li fe -en da n ge ring c o nd iti o ns ma y fo ll o w. Su c h p ote n tia lly fata l ca s es h a ve be en m a in ly re po rte d i n pa tie n ts who are i mm u n o co m pro m is ed .

Fig. 4 .7 P ati en t s u ffe rin g fro m p rim ar y h er pe ti c gi n gi vo stom a ti tis w ith in vo lv em en t o f th e li ps a n d pe rio ra l sk in .

In th e va st ma jo rity o f u n tre ate d ca s es th ere i s a sl o w rec o ve ry f ro m the s ym pto m s o ver a

per io d o f s o me 10β €“ 14 da ys . M o st ca se s o f p ri ma ry he rp etic g in g ivo s to ma titi s ar e d ia gn o s ed on cl in ic a l g ro u n ds . HS V c an be gro w n i n tis s ue c u ltu re s ys te m s bu t th i s ta ke s a t le as t 24 ho u rs . S ero l o gy ca n a ls o b e u s ed to d ete ct eith e r an in c rea s e i n th e IgE a n tib od y ti tre betw e en th e a cu te an d c o nv al es c en t p h as e, o r sp ec i fic Ig M an ti bo di es .

MANAGEMENT Sp ec if ic th era p y f or vi ral i nf ec tio n s is ge n era ll y u n s ati sf ac to ry an d m an a ge me n t is , th ere fo re , sym p tom a tic w ith g en e ral su p po rti ve me a su re s, s u c h as r es t, flu i ds , a nd

an tip yre tic s/ an a lg es ic s . Fo r o ral in fe c ti o n s, th e use o f an an tis e ptic ( fo r e xa mp le ,

ch lo rh e xi din e g lu c on a te) o r tetra c yc lin e m o uth w a sh m a y r edu c e se c on d a ry i n fec tio n a n d an

an a lg es ic m o uth w a sh (fo r exa m pl e, b en z yd am in e ) w il l red uc e di s co m fo rt, p arti cu l arl y w h i ls t th e pa tie n t is e ati n g. S ys tem ic a n al ge s ic s an d a ntiyp reti cs m ay a ls o b e re qu i red . In mo s t

im mu n o co m pe ten t p ati en ts , HS V in f ec tio n s ar e s el f-lim iti n g a n d la st 10β €“14 da ys . P. 42 Ma n ag em en t of m o st v ira l in fe cti o n s aff ec tin g th e mo u th is sy mp to ma tic β€”gen e ra l su p po rtiv e m e as u res i n cl u de :

res t

flu i ds

an tip yre tic s/ an a lg es ic s (s ys tem ic ) an tis ep tic /a n al ge si c mo u th w as h es

Ac ic lo v ir is a cti ve ag ai n st h erp es v irs es b u t do e s n ot era di ca te th em . It c an b e u se d f o r th e sys te mi c an d to pi ca l tr ea tme n t o f h e rpe s si mp le x i nfe cti on s a ff ec tin g the s ki n an d m u co u s

me mb ran e s. Pro p h yla c tic u s e o f a ci cl o vir fo r p re venti on an d re cu rre n ce o f HS V in fe c ti o n s is jus tif ie d i n th e im mu n o co m pr o mi se d.

Sy ste mi c an ti vir al th era py i s e s se n tia l i f th e pa tie n t is i mm u n o co m pro m is ed , a s thi s gro u p is mo re s us c ep tib le to ge n era l di ss em in a tio n o f th e H SV viru s . In th es e ca s es , th e cl in ic a l

pre se n tatio n of th e in fe cti o n ma y be a typi ca l. An ac u te h erp eti c sto m ati tis re pre se n ts th e

pri ma ry in fe cti o n an d , al th o ug h s u bs eq u en t im mu n i ty m ay n o t b e c o mp le te, i t is o n l y ve ry

rare ly th at a se co n d a cu te atta c k fo ll o w s, u n le s s the p ati en t i s im mu n o c om pr is ed . Rec u rre n t in trao r al h erp es in fe c ti o n s are th er efo re ra re in th e im m u no c o mp ete n t pa tie n t bu t c ro p s o f

ves ic le s re stri cte d to pa rt o f the o ra l mu c o sa , p arti cu l arl y th e p al ate , h av e be en d es c rib ed . If th e si gn s a n d s ym pto m s o f p rim a ry h e rpe tic gi n givo sto m ati tis fa il to i mp ro ve a fter 2 we ek s, th e n th e p ati en t s h o u ld be ref erre d fo r a s pec ia li st o p in i on to ex cl u de bl o od dys c ras i as a n d o th e r u n de rlyi n g s ys te mi c co n di tio n s.

Secondary (recurrent) herpes simplex infections

Fo ll o w in g res o lu tio n of th e pri ma ry h erp etic i n fe cti on , th e re is a n ap pr ox im ate ly 30 β€“ 40 pe r ce n t li kel ih o o d tha t re c ur ren t l es io n s w il l f o llo w , reg a rdl es s o f th e in ten s ity o f th e p rim ary

atta ck . Th e us u a l s ite o f the rec u rre n t le si o n is on o r ne a r th e lip s a nd th e le si o n is k no w n a s β€ he rpe s la bi al is β€™ or a β € co ld s o re β€™. Le ss c o m mo n ly , th e s ki n a nd m u co s a o f th e

no s e an d n a sa l pa s sa ge s ar e i n vo lv ed , as m a y b e, o cca s io n a lly , a lm os t a n y s i te o n th e fa ce . In an i n di vid u al p ati en t, h o we ve r, th e are as i n vo lved ten d to re ma in th e sa m e in s u cc es s iv e

epi s od es . Th e re cu rre n ce s m ay be p ro vo ke d b y a wi de ra n ge o f sti mu l ae , in c lu d in g su n l igh t, me ch a n ic al tra u ma , a n d, p arti cu l arl y, m il d feb ril e co n d itio n s s u ch as th e co m m on co l d. Em o tio n a l f ac to rs a ls o pl ay a pa rt i n pre c ip itati n g r ec u rren c es i n m an y pa tie n ts . HS V

pa rtic le s ca n b e d ete cte d in th e sa l iva o f ap pr ox imate ly 30 p er ce n t o f p ati en ts w h o de ve lo p co ld so r es .

CLINICAL PRESENTATION In th e p ro dro m al s tag es o f a rec u rre n ce th e pa tie nt ma y f ee l a m il d de gre e of tir ed ne s s a n d ma la is e. T h is is qu i ck ly fo ll o we d by a pe rio d o f irri tati on an d itc h in g o ve r th e are a o f th e li p in vo lv ed in rec u rren c e an d , w ith in a f ew h o u rs , ve si cl es a pp ea r su rro u n de d by a mi ld ly

eryth e ma to u s are a (a t th is s tag e th e p ati en t i s hi gh l y i nf ec tio u s ). In a sh o rt tim e th e ve si cl es bu rs t a nd a s c ab is f o rme d. F ro m th is p o in t th e proc es s is o n e o f s lo w h e al in g o ve r a pe ri od of s o me 10 d ay s, b u t s ec o n da ry in fe cti on ma y o cc a sio na l ly de la y th e h ea li ng p ro ce s s an d lea d to th e pro d uc tio n o f s ma ll pu s tu le s i n th e area . Hea li n g is w ith o u t s ca rri n g a n d the

aff ec ted a rea re tu rn s, af ter a sh o rt p eri o d of e ryth em a , to an ap pa ren tl y n o rm al s tag e u nti l

th e o n se t o f th e n ex t rec u rre n ce . Th is m ay b e a fte r a p eri o d o f a fe w w ee ks o r e ven da ys in so m e i n di vid u al s, bu t g en er al ly the i n te rva ls b etw een re c ur ren c es a re o n th e o rde r o f

mo n th s, th i s evi de n tly de pen d in g to a la rg e exte n t o n th e d eg ree o f ex po s u re o f th e pa tien t

to the p arti cu l ar s ti mu l u s in vo lv ed .

MANAGEMENT Th ere is n o th e rap eu ti c me as u re av ai la bl e th at p ro vi de s rea s on a b ly co n s is ten t re su l ts an d

th a t e nti rel y p re ven ts fu rth e r rec u rre n ce s. A c ic lo vir c re am , u s ed a s ea rly a s p o s si bl e a n d at

lea s t fi ve tim es a da y in th e ea rly s tag es , m ay pr eve n t pro g res s io n o f th e l es io n . Th i s is n o w per ha p s the m o st eff ec tive a n d ac c epta b le fo rm o f tr ea tme n t fo r r ec u rren t h erp es , a lth o u gh

th e re is n o re al s u gg es tio n th a t a pe rma n en t cu re may be o bta in e d. A s im il ar an ti vir al dr ug , pen c ic l ov ir, c a n be u s ed to pi ca ll y f o r c o ld so r es , (h erp es l ab ia li s) . Th e β€ pro d ru g β€™ of

pen c ic l ov ir is fa m ci cl o vir , wh i ch is a s ys tem ic a n tivi ra l a ge n t re co m me n de d fo r HZ V i n fec tio n s an d ge n ita l h erp es .

To pi ca l the ra py fo r β€ co ld s o res β€™ (h erp es l ab ia li s) sh o u ld b e ap pl ie d in th e pr od ro m al

ph a se o f th e le s io n . A ci cl o vir a nd p en c ic lo vi r c re am s ar e av ai la bl e fo r to p ic al a pp li ca tio n .

Varicella zoster virus infections

Va ric e lla z o s ter vi ru s (VZ V ) is a DNA v iru s m or ph o lo g ic al ly si m ila r to th e HS V a n d ap pa re ntl y

res po n s ib le fo r tw o c o m ple tel y d is s im il ar di se as es i n h u ma n s , n am el y, c h ic ken p o x an d h er pe s zo s ter. T h ere is , h o w ev er, l ittle e vid en c e th a t c o ntac t w ith o n e o f th es e dis e as es i s

res po n s ib le fo r th e in i tia tio n o f the o th er. It se em s mo re li kel y th a t th e z os ter er up tio n

rep res en ts th e rea c ti va tio n o f th e v iru s in a pre vio us l y i nf ec ted p ati en t w ith o n l y p ar ti al

im mu n ity , a si tua tio n p a ral le l to th at i n re cu rre n t h erp es s i mp le x. Th e z o ste r vi ru s is a ls o

th o u g ht to rem a in la ten t i n th e rel eva n t s en s o ry g ang li o n an d to p as s d ow n th e n erv e to the ski n o r m u co u s m em bra n e o n rea c tiva tio n .

Chickenpox

Pri ma ry in fe c ti o n w ith V Z V m an if es ts as ch i ck en p ox , w h ic h is a c o mm o n d is ea se o f ch i ld ho o d . It ha s a n in c u ba tio n p eri o d o f 14 β€“ 21 da ys , du ri n g wh i ch pa tien ts a re in fe c ti o u s. T he

in fe cti on is s pre ad by di rec t c o n tac t o r dro p let infe cti o n an d i s es tab li sh e d f irs t i n th e u p pe r res pi rato ry tra ct.

CLINICAL PRESENTATION Ju st b ef o re th e ra s h, o r al le si o n s ma y a p pe ar o n the h a rd pa la te, p il la r o f fa u ce s, an d u vu la an d ap pe a r a s sm a ll u lc ers , w ith a red h al o . Th e rash i n itia ll y ma n ife s ts a s p in k,

ma cu l o pa pu l ar le si o ns th a t de ve lo p in to i tch y ve si cl es o n th e ba c k, c h es t, fa ce , an d s ca l p. Oth er c lin i ca l ma n if es tati on s i n cl u de ma l ai se , fe ver , an d ly mp h ad en o pa th y. A du l ts ca n b e qu ite s eve re ly af fec ted . Ra re co m pl ic a ti o n s o f ch i ck en p ox i nc lu d e pn eu m o n ia a n d

en c eph a li tis . Tre atm en t i s s ymp to ma tic b u t it is i mp o rtan t f o r th e pa tie nt to av o id c on ta c t wi th an y im m un o c o mp ro m is ed in d ivi du a ls .

P. 43

Shingles

Mo s t p atie n ts w ith h er pes z o s ter are mi dd le -ag ed o r o l de r (70 p er ce n t o ve r th e ag e o f 50), bu t i t ca n o c cu r in m u ch yo u n ge r p ati en ts a nd a fe w n eo n a tal c as e s ha ve b ee n de sc ri be d.

Pre di sp o si n g fa cto rs th a t h a ve be en s u gg es ted i nc lud e a wi de ra n ge o f de bil ita tin g di se a se s an d im mu n o s u pre ss io n , e ith er o f i atr og en i c o r n atu ra ll y oc c u rrin g o rig in .

CLINICAL PRESENTATION Th e c h a rac ter is tic s up er fic ia l le si o n o f h e rpe s z o ste r is a v es ic u la r e ru pti o n in a n a rea o f

dis tri bu tio n o f a se n so r y n er ve. T h e b a nd -lik e d is tri bu tio n o f sh i n gl es o n th e tr un k is w e ll

kn o wn , h a vi ng g iv en th e n am e to th e v iru s (zo s ter , gi rdl e). Wh en th e eru p tio n a ffe c ts th e

tr ige m in al n e rve, th e fa c ia l sk in a n d o ral m u co s a in th e s en s o ry a re a ma y b e af fe cte d. Th e tr ige m in al n e rve is in vo l ve d i n a bo u t 15 p er ce n t of c a se s . Of th e th re e di vis io n s o f the

tr ige m in al n erv e th e o ph th a lm ic is th e m o st f req u ently in v ol ve d, b u t th e o th e r tw o di vis i on s

are a ls o n o t u n co m mo n l y a ff ec ted . Oc c as io n a ll y, w h en th e di s or de r af fe cts o n e di vis io n o f th e tr ige m in al n e rve, th e a dja c en t d ivi s io n be c o me s in vo l ve d a s w el l. C ervi c al n erv es m ay a ls o be af fe cte d. Th e in i tia l s ym p to m s a re o f p ai n a n d te n de rn es s in th e a ffe cte d a re a β€” a

dis c o mf or t mu c h m o re se ver e th a n th at e xp eri en c ed in HS V in fe c ti o n . Th e pro d ro ma l pa in o f sh in g le s is s ev ere a nd is o c ca s io n a lly m is di ag n o se d as to o th ac h e. T he p ro dro m a l p h as e ma y la st f o r 2 o r 3 d ay s an d is s u cc e ed ed by th e ap pe ara n c e o f ve si cl es i n a ra sh , w h ic h m a y b e eith e r s pa rs e o r so de n se a s to be a lm o st c o n fl ue n t. Fr eq ue n tly, th e ve s ic le s ap pe a r o ve r a

per io d o f d ay s rath e r th a n to ge th e r, a n d th e se m ay be c om e se c o nd a ril y i nf ec ted . Wh en th e op h th al mi c di vis io n o f th e tr ige m in al n e rve is in vol ve d th e re m a y b e co rn e al u lc e rati o n,

wh i ch r eq ui res m o s t ca re fu l ma n ag em en t to a vo id p erm an e n t sc a r f or ma tio n . Wi thi n th e

mo u th , the v es ic le s be h ave m u ch as th os e o f h e rpe s s im ple x in fe cti o ns , ra pi dl y b re aki n g

do w n to fo rm u l ce rs. Th e u n ila ter al di s tr ibu ti o n o f th e o ral l es io n s , an d c o nf in e me nt to th e are a o f a si n gl e b ra n ch o f th e tr ige m in a l n e rve, may g iv e a c l ue a s to th e na tu re o f th e

in fe cti on bu t th is c a n no t be rel ie d up o n . If u n tre ate d, th e ves i cl es a n d o ra l u lc era tio n f ad e ov er a va ria bl e p er io d fro m 2 to 4 we ek s, o r ev en lo ng er . Th e sk in v es ic le s ma y fo rm fi rm cru s ts an d , pa rtic u la rl y i f th e se a re dis tu rb ed, mark ed sc a rrin g m ay o c cu r. F o ll o wi n g the

fad in g o f th e ra s h th e m a jo r co m pl ic a tio n o f th e c on diti o n β€”po s th e rpe tic n e ur al gi a β€” ma y

ap pe ar. In th i s co n d itio n an a es th es ia , pa ra es th es ia, a n d tri ge mi n al n eu ra l gia -lik e p a in s ma y aff ec t th e are a a nd m a y p ers is t f or a pe rio d o f years , o cc a si o n al ly rea p pe ari n g af ter a

pro lo n g ed a bs en c e. T hi s is a h i gh ly re fra cto ry c on diti on tha t m a y fa i l to re sp o nd to a n y fo rm of m ed ic al tre atm en t (s e e Ch ap ter 15). In a ve ry f ew p ati en ts th e fa ci al n erv e ma y b ec o m e

in vo lv ed d ur in g an ep is o de o f z o s ter rea c ti va tio n , p ro ba bl y v ia th e gen i cu l ate g an g lio n . F a ci al we ak n es s, lo s s o f ta ste s en s ati on , a n d sy mp to ms s u ch as d iz z in e ss re su l tin g fro m

la byr in th in e di stu rb an c e ma y o c c u r. Th is i s the Ra msa y Hu n t s yn dro m e (Tab le 4. 12).

Ve si cu l ar le si o n s in th is c o n di tio n a re mo s t o fte n se en on the p al ate a n d a ro u n d the e xter na l

au d ito ry me atu s . In mo s t c a se s, th i s is a s el f -lim iti n g c o n di tio n th a t res o lv es w ith r es to rati o n of fu n c tio n , bu t i n so m e pa tie n ts th e re ma y be pe rman en t fa ci al w ea kn e ss .

Table 4.12 Ramsay Hunt syndrome In v ol ve me n t o f f ac ia l n erv e w i th VZ V

Fa c ia l n e rve pa ls y (lo w er m oto r n eu ro n e) Ve si cl es i n ex tern a l au d ito ry me a tu s Ve si cl es o n p a la te

Oth er s ymp to ms , e .g . di z zi n es s , lo s s o f ta s te

MANAGEMENT Trea tm en t o f sh i ng le s is w ith h i gh d o s es o f s ys tem ic a c ic lo vi r o r fa mc ic lo v ir. Th i s is

pa rtic u la rly im po rta n t w h en th e o ph th al m ic di vi si o n o f th e trig em in a l ne rve i s a ff ec ted . Th er e is s om e ev id en c e th at e a rly u se o f sy ste mi c an ti vira l th era py m ay red u ce th e po s tin fe cti ve

co m pl ic ati o n o f p o sth e rpe tic n eu ra lg ia (Ch ap ter 15). In th e c a se o f the o ra l le si o ns , to pi c al

tr ea tme n t as f o r h erp es s i mp le x s to ma titi s (tetra c yc li ne m o u th w a sh ) is u s ed , w ith th e sa m e

rati o na le a n d wi th s im ila r, i f sl o w er, re s ul ts. Pe rh ap s o n e o f th e m o s t im po rta n t fu n c tio n s o f th e c li ni c ia n fa ce d w ith a c as e o f h e rpe s z os te r is to b e aw a re o f th e po s s ib ili ty o f c or ne a l

in vo lv em en t a n d to pl ac e th e p a ti en t u n d er ap pro p ria te ca re if a ny s ym pto m s, h o we ve r m il d, ap pe ar.

Sh i ng le s (pa rti cu l arl y i f a ff ec tin g th e tri ge mi n al n er ve) sh o u l d b e trea ted a s so o n a s p os s ib le by sy ste mi c an ti vira l th era py ; s u ch a s a ci c lo vi r o r fa mc ic l ov ir.

Epsteinβ€“Barr virus infection

Th e E p ste in β€“ Ba rr vi ru s (E BV ) pl ay s a p a rt in th e p ath o ge n es is o f a n u mb er of c o nd iti o ns

aff ec tin g th e o ro f ac ia l reg io n , i nc lu d in g in f ec tio us mo n o n u cl eo s is (g la n du l ar fev er) . Th e E BV is c on s id er ed to be a n o n co g en i c viru s a n d as s oc i ate d w i th th e f o rma tio n o f ma li gn a n t lym ph o m as ( Bu rk itt's l ymp h o ma ) i n A fri ca a n d lin k ed to n a so p ha ry ng ea l ca rc in o m a, pa rtic u la rly in so u th ern C h in a . Th e as s oc i ati on of E B V w ith o ra l h ai ry leu k op la ki a in

im mu n o co m pr om is e d p a ti en ts , pa rtic u la rl y th o s e w i th HIV in fe c ti o n , is d is cu s s ed in th e se cti o n o n A IDS .

Infectious mononucleosis

Th is is a r ela tiv el y c o mm o n in fe c ti o n , u su a ll y m a nife sti ng s ym pto m s in e ar ly ad u lth o o d.

Ch il dre n ma y su f fer a su b cl in i ca l in fe cti o n. Th e d ia g no s is i s co n fi rm ed by la b o rato ry tes ts ,

wh i ch sh o u l d i n cl u de a bl o o d f ilm a n d di ffe ren ti al w h ite ce ll c o un t. Du ri n g th e a cu te ph a se s

of th e in fe cti o n, p a tien ts p ro du c e β€ atyp ic a l β€™ mo n o n u cl ea r c el ls (Da u nc y ce ll s) an d ther e wi ll be a l ymp h o cy to si s. In th e p as t, th e P a u lβ€“ Bun e ll tes t w a s us e d to d ete ct the

he tero p h ile a n tib od y in p ati en t's s eru m , w h ic h ag glu tin a te s s he ep e ryth ro c ytes . In m o st la bo ra to rie s th i s tes t h a s b ee n rep la c ed by a s li de ag gl u tin a tio n tes t c a lle d the β€ mo n o sp o tβ €™.

CLINICAL PRESENTATION Th e p a ti en t w i th gl an d u la r fe ver fe el s ill a n d h a s a fe ver a nd e n la rge me n t o f th e ly mp h n o de s th a t m ay ex ten d o ver th e w h o le

of th e bo dy . Th e se ve rity o f th es e sym p tom s is v ery w id el y v ari ab le , ra n gin g fr om a m in o r atta ck th at ma y pa s s al mo s t u n n o ti c ed to a c on d iti on req u iri ng h o s pi tal iz ati o n .

In th e e arl y s ta ge s o f th is i n fec tio n a s o re th ro at an d o ra l u l ce ra ti o n ma y be ve ry

tr ou b le so m e. T h e u l ce rati o n is q u ite n o n- sp ec if ic , bu t m a y b e w id es pre ad . A s in s im il a r

ci rcu m s ta n c es s ym pto m ati c tr ea tme n t o f th e o ra l u l ce rs w ith a n tibi o tic m o uth w a sh e s is th e

on l y h e lp fu l p ro c ed ur e. P ete ch i ae m ay b e vi si bl e a t th e ju n cti on o f th e h ard a n d s o ft pa la te.

Ma n ag em en t of th is co n d itio n is s ym pto ma tic b u t re co ve ry ca n b e qu ite pr otr ac ted in se ve re ca se s .

Coxsackievirus infections

Th is g ro up o f vir us e s is di vi de d i n to Co xs a ck ie grou p A a n d g ro u p B vi ru se s. G ro up A v iru s es are p rin c ip al ly res po n s ib le fo r two in fe c ti o n s tha t a ff ec t th e o ro p h ar yn ge al re gi on : β€ ha n d,

fo o t, an d m ou th di se a se β €™ an d h erp a ng in a . Th es e a re r el ativ el y u n c o mm o n bu t te n d to

oc c u r a s mi n i -epi de mi c s bec a u se th ey a re hi gh l y c o ntag io u s . Th e pu tati ve vi ru se s, cl in i ca l

pre se n tatio n , a n d trea tme n t o f th e se i nf ec tio n s a re s u m ma riz e d in Tab le 4. 13. Trea tm en t fo r bo th th es e in fe cti o ns i s sy mp to ma tic .

Hand, foot, and mouth disease

P. 44

Han d , fo o t, a n d-mo u th di s ea se (n o t th e sa m e as β€ fo o t an d m o u th di se as e β €™) is c au s ed

by a Co xs ac ki e A vir us , u s u al ly type 16 b ut le ss c omm o n ly typ es 5 a n d 10. A n um b er o f

mi no r ep id em ic s o f th is co n d itio n ha ve o c cu rr ed an d b ee n de sc ri be d i n de ta il, o n e in v ol vi ng stu de n ts an d sta ff a t a de n tal h o sp ita l. T he o ra l le si o ns i n th is o u tbre a k c o n si ste d of s ma ll

ul ce rs , res e mb lin g m in o r a p hth o u s u lc ers , re la tiv ely fe w in nu m be r a n d di stri bu te d o v er th e or al m uc o s a. H o we ve r, o ra l l es io n s , o bs erv ed b y o n e o f th e au th o rs , a ls o in d en ta l ho s pi tal

per so n n el , a nd c o n firm e d b y a f u ll ra ng e o f in ve s tiga tio n s , h av e, i n fa c t, c o n s is ted o f b u ll ae th a t l ate r ru p tu re d to pro d u ce tra n si en tly p ai n fu l e ro s io n s o f th e mu c o s a. T he l es io n s o f th e ha n ds a n d fe et c o n si st o f a re d ma c ul ar ra sh , e ac h m a cu le a pp a ren tly s u rro u nd in g a de ep se ate d v es ic le . Th e c on s titu tio n a l sy mp tom s e xpe rien ce d are o f a mi ld n atu re , a sl ig h t ma la is e w ith m in im a l p ai n a n d d is c o mfo r t. T h e s ym pto m s res o lv e in a b ou t a we ek .

Table 4.13 Clinical features of Coxsackievirus infections Han d , fo o t, a n d m o u th di se a se β€” Co xs ac ki e A 16 ( oth e rs: A 5, A10)

In tra o ra l ve si cl es /u lc e rs

Ma c ul ar ra sh wi th ve si cu l ati o n o n p a lm ar su rf ac e o f h an d s p la n tar s ur ac e o f fe et

Ma la is e

Dur ati on : 7 da ys Mi n i-epi de mi c s

Herp a ng in a β€” Co xs ac ki e A ( mo s t typ es ) So re th ro a t an d m al ai se

Ve si cl es /u lc e rs o n pa la te an d p ha ry nx Dur ati on : 3β€“ 5 da ys

Herpangina

Herp a ng in a i s pre do m in a ntl y c a u se d by Co xs a ck ie A vi ru se s. It is a m il d i n fec tio n , s ee n

pre do m in a ntl y ( bu t n o t en tir el y) i n c hi ld ren , th a t ten d s to o cc u r i n m in o r e pi de mi cs . Th e

pa tien t co m pl a in s o f a m o de ra te d eg ree o f ma la is e an d o f a so re th ro a t, wh i le o cc a si o n al ly

th e re is a ls o a m in o r d eg ree o f mu s cl e we a kn es s an d p ai n . Sm a ll ve si cu l ar le si o ns a p pe ar in th e p os ter io r p a rt o f th e m ou th , i n pa rtic u la r, o n th e s o ft p a la te. Th e se l es io n s a re n o t

pa rtic u la rly c ha ra c te ris tic o f th e co n d itio n , b u t re se mb le o th er viru s -in du c ed l es io n s an d a re rec o gn iz a bl e o nl y b y th eir typ ic al d is trib u ti o n . This i s a se lf -lim iti n g c o n di tio n . Th e le si o ns fad e af ter so m e 3β €“5 da ys a n d co m pl ic ati o ns a re extrem el y ra re.

Paramyxovirus infections

Pa ra my xo vi ru se s in c lu de th e mu m ps v iru s a n d th e mo rb il li vir us th a t c au s es m ea s le s. T he s e in fe cti on s a re in c lu d ed in th i s ch a pte r be ca u s e th ey b o th h av e o ro f ac ia l ma n if es tati o ns .

Mumps

Mu m ps i s ch a ra cte riz ed b y b il ate ra l s w el li ng o f th e p a ro ti d gl an d s, a lth o u gh un i la tera l

gla n du l ar sw e ll in g ca n o c cu r. In vo lv em en t o f the s ubm a nd ib u la r g la n d i s le ss c o m mo n . Th e

sa li va ry g la n d du c ts u su a ll y a pp ea r red a nd i n fla m ed a nd p ati en ts o cc a si o n al ly rep o rt a d ry mo u th . Tri sm u s ma y be pr es en t a n d th e g la n ds a re extre m ely p ai nf u l a n d ten de r to to u c h .

Co mp li ca tio n s o f mu m ps i nc lu d e pa n cre a ti tis , en c ep hal iti s, o rc h iti s, o o p h or itis , an d d ea fn es s . Th e e ld erl y a re a t gr ea ter r is k o f de ve lo pi n g co m plic ati o ns . M an a ge me n t is s ym pto m ati c w ith

gen e ra l s u pp o rtiv e m ea s u res (s ee a ls o Ch ap ter 8).

Mumps

In c u ba tio n p eri o d: 14β€“ 21 da ys

Tra ns m is si o n : re sp ira to ry se cr etio n s a n d s a li va

Sa li va ry gla n d en l arg em en t, u s u al ly bi la tera l pa ro tid in vo l ve me nt Tris m us

Dia gn o s is : u s ua l ly on cl in ic a l pre s en tati o n

Measles

Me as le s u su a ll y p re se n ts a s a s ys tem ic , fe bri le il ln es s w ith a n i n itia l n as a l d is c ha rg e,

(ca ta rrh al s tag e). Ko pli k's sp o ts ar e b lu i sh -wh i te, p in po i n t sp o ts, w i th da rk -red a veo l ae , th at ap pe ar o n th e b u cc a l m u c os a e an d di sa p pe ar af ter 3β€“4 da ys . A m ac u lo p ap u la r s ki n ra sh

th e n a pp ea rs bu t r es o lve s af ter a few d a ys β€”dis a ppea ra n ce o f th e ra sh h era ld s rec o ve ry. In

a f ew c a se s th ere a re po ten ti al ly se rio u s c o mp li ca tio n s re su l ti n g fro m in f ec tio n w ith me as le s, in cl u di n g e n ce ph a li tis a nd p n eu m on i a. M a n ag em en t is sy mp to ma tic w i th ge ne ra l s u pp o rtiv e me as u res . A n tib io tic s ma y be re qu ir ed fo r co m pl ic a tio n s su c h a s se c on d ar y o titi s o r pn eu m o n ia .

P. 45

Human papillomavirus infections

Th ere a re ov er 100 di ffe ren t hu m a n pa pi ll om a vir us e s (HP V ) a n d th e y c a n ca u s e w a rty les i on s on th e sk in a n d mu c o u s me mb ra ne s . Th e co m mo n w a rt (v erru c a vu l ga ris ) i s co m m on l y s e en on th e s ki n an d o ra l m u c os a , w h ere it is in d is tin g ui s ha b le fro m a s qu a mo u s c el l pa pi ll o ma

(Ch ap ter 9). Th e cl in ic a l p re se n ta tio n is o f a β€ ca u li flo w er - lik eβ €™ les i on . In tra o ra l l es io n s

or th o se a ffe cti n g th e li ps a re o ften th e re su l t o f a u to in o c ul a ti o n by c h ew in g w ar ts o n th e ha n ds . He c k's di se a se is a ra re o ra l m a n ife sta tio n o f HP V i nf ec tio n i n th e m o u th an d i s

dis c u ss ed i n Ch ap ter 9. Ve n ere al w a rts (co n d ylo m a ac u m in a tu m ) m a y o c c ur in th e m ou th , a s a r es u lt o f o ro ge n ital s ex u al c o nta c t an d ap pe a r a s so f t, pin k pa p ill a ry l es io n s , pa rtic u la rl y on th e p al ate a n d to n g ue . Ca n ce r o f th e c er vix h as b ee n a ss o c ia ted w ith H PV i n fec tio n .

Human immunodeficiency virus and AIDS

Th e h u m an im mu n o d efi ci en c y v iru s (H IV) is a re tro vir us r es po n s ib le fo r th e a cq u ire d im mu n e def ic ie n cy s yn dro m e (A IDS). Th e f irs t h u m an r etro viru s, h u m an T-ce ll ly mp h otro p h ic vi ru s

ty pe I, w as i s ol a te d in 1980 f ro m a p a ti en t w i th T-ce ll le u ka em ia . Th e cl in ic a l ma n ife s ta tio n s

of HIV i n fec tio n w e re fir st r ec o gn iz e d i n 1981. Th e co u n try o f o ri gi n o f th e vi ru s i s u n kn o w n, al th o u g h th ere is f ai rly str on g p res u mp tive e vid en ce th at th e in i tia l c e ntre o f ac tiv ity wa s i n ce n tr al A fric a . HIV i nf ec tio n i s, h o w e ver, a rap id ly ex pa n din g w o rld w id e p ro bl em , al th o ug h ,

qu ite c le arl y, p ro gn o s ti c ati on in th es e c irc u ms ta nce s i s a ve ry di ffi cu l t pr oc e ss a n d the re ca n be n o de gre e o f c erta in ty a bo u t th e ma n y pro je c ti o ns of fu tu re pa tie n t n u mb ers th a t h a ve

bee n m ad e. T ran s m is si o n o f th e vi ru s is c ur ren tly th o u gh t to b e pre do m in a ntl y v ia b lo o d an d

blo o d p ro du c ts a n d se m en . P eri n ata l an d po s tn ata l tran s mi s si o n o cc u r i n a bo u t 20 p er ce n t o f in fa n ts bo rn to i n fec ted m o th ers .

Th e p a th o g en es i s of d is ea se ca u s ed by HIV i n fec tio n is c om p lex a n d o u ts id e th e s co p e of th is bo o k. Th e m o st si gn i fic a n t ef fec t o f th e v iru s i s in th e in fe cti o n an d c o n se qu en t in a cti vati o n of CD4-exp res s in g ce ll s, th a t i s, th e h el pe r a n d d el ay ed -ty pe h yp ers en s iti vity T ce ll s.

In f ec tio n w ith th e vi ru s is a s lo w p ro ce s s, b u t ev en tu al ly th e h o s t's im mu n e s ys tem is fu n cti on a ll y d is a bl ed .

A cl as s ifi ca tio n of th e s ta ge s of HIV i n fec tio n ha s be en pro p o se d a n d is s h o wn in Tab le 4. 14 .

Th e m a n ag em en t o f pa tie n ts wi th HIV i nf ec tio n i s com pl ex bu t i n vo lv es a n tire tr o vira l th era py an d th e tre a tm e nt o f o p po rtu n is tic i n fec tio n s (f or ex am pl e, c a n di do s is , h erp es vi ru s

in fe cti on s , an d Pn e um o c ys ti s c ari n ii ) a nd m a lig n an c ie s as s o ci ate d w ith A IDS . Az i do th ym id in e (AZ T) w as th e fi rs t an ti retro vi ra l d ru g to be li c enc ed fo r tr ea tin g HIV a nd i s a pro te as e

in h ib ito r. Th e pr og n o si s fo r p a ti en ts h a s im pro v ed w ith th e in tro d uc tio n o f m ul tip le dr ug

th e ra pie s . Trip le th er ap y co n s is ts o f a c o m bin a tio n o f dr ug s , in c lu di n g a p ro tea s e i n h ibi to r

(fo r e xa mp le , A ZT ), n u cl eo s id e r eve rs e tra n s cri pta se i n h ibi to rs, an d n o n -nu c le o si de re ver se tr an s cr ipta s e i n h ib ito rs . Th e co s t o f th e se d ru g r eg im en s is h i gh a n d co n s id ere d to be

un a ff or da bl e b y Th ird Wo rl d c o u n trie s. T h eir lo n g -te rm ef fe ctiv en e ss h a s ye t to be p ro ve n.

Table 4.14 Classification of the stages of HIV infection* Gr o u p

Descr ipt ion

I

Se ro co n v ers io n i ll ne s s

II

As ym pto m ati c

III

Pe rs is ten t g en er al iz ed ly mp h ad en o pa th y

IV A

Co n sti tu ti o na l di se a se

IV B

Neu ro lo g ic a l di se a se

IV C

Se co n d ary in fe c ti o u s di se as e

IV D

Se co n d ary c an c er s

IV E

Oth er c on d iti on s

* A s pro p os e d b y th e Ce n ter fo r Di se as e Co n tro l, USA .

Oral manifestations of HIV infection

Th e o ra l ma n if es tati o ns o f HIV i n fec tio n a re c o ns ider ed to be o f gre a t si gn i fic a n ce a n d h av e

bee n c la s si fie d as tw o g ro u ps : g ro u p 1, l es io n s c o mm o n ly as s o ci ate d wi th HIV i nf ec tio n ; a n d gro u p 2, le si o ns l es s c o mm o n ly as s oc i ate d w i th HIV in f ec tio n (Tab le 4. 15 ). Th e mo s t

si gn if ic an t co n s id era tio n c o n ce rn s th e c h a ng es th a t m ay a pp ea r i n the o ra l ca vi ty o f a pa tie n t wi th HIV i nf ec tio n th at h a s n o t, as y et, b ee n di ag no se d. T he l ike ly o ra l c h an g es a re

dep en d en t o n th e i n cre a si n g re du c tio n i n im m un e s u rve ill an c e, pre se n tin g ei th e r a s in fe c tio n s or as n e o pl as m s. T he m o st lik el y i n fec tio n i s ca n dido s is .

Table 4.15 Oral manifestations of HIV infection

Di se a se s stro n g ly as s oc i ate d w ith HIV in fe c ti o n Can d id o si s

E ry th em ato u s

P s eu d o me mb ra no u s

Hai ry le u ko pl ak ia

Pe rio d o nta l di se as e

Li n ea r gi n gi val e ryth em a

N ec ro tiz in g u lc er ati ve gi n gi viti s

N ec ro tiz in g u lc er ati ve pe rio d o nti tis

Kap o si 's s arc o m a Lymp h o ma

Les io n s le ss co m m o nl y a s so c ia ted w ith HIV in fe c ti o n My co b ac teri a l in fe c ti o n s Me la n o ti c pi gm en ta tio n

Nec ro tiz in g (u l ce rati ve ) sto m a ti tis Cys tic s al iv ary g la nd d is ea s e Th ro mb o cy top en i c pu rp u ra No n -sp ec if ic u lc era tio n

Vi ral i nf ec tio n s in c lu d in g Herp es s im p lex , He rpe s z os te r, an d h u ma n p ap il lo ma v iru s in fe cti on

P. 46

ORAL CANDIDOSIS IN HIV INFECTION As h a s be en p o in ted o u t p rev io u sl y in th i s ch a pte r, o ra l c a nd id a l i n fec tio n s h o u ld a lw ay s be

co n s id ere d a s a n in d ic ato r of g en er al iz ed i ll h ea lth a n d, i n th e c a se o f th e e ar ly AIDS p a ti en t, it m ay b e th e ea rl ies t p res e nti n g s i gn . O the r v ira l, f u ng a l, a n d b a cte ria l or al in fe c ti o n s are

mu c h le ss l ik ely , bu t d o o c cu r. T h e o th er wi se u n ex pl ai n ed o n se t o f a n i nf ec tio n , p arti cu l arl y

wh e n as s o ci ate d wi th o th er pe rsi ste n t s ign s a n d sy mp to ms , s u ch a s g en er al iz ed l ymp h n o de en la rg em en t, m al ai se , in te rmi tten t fe ver s, a n d we igh t lo s s , sh o u ld a ro u se s u sp ic io n s . O ral ca n di do s is h a s al rea dy be en d es c rib ed in thi s c ha p te r a n d an g u la r c h eil iti s is fu rth e r

dis c u ss ed i n Ch ap ter 6. Er yth em ato u s c an d id o si s co mmo n l y o c cu rs i n HIV - in fe cte d i n di vid u al s an d af fe cts th e h ard a n d s o ft pa la te, to n gu e , an d bu c c al m uc o s ae . P se u do m em bra n o u s ca n di do s is is f req u en tly c h ro ni c a nd a n gu la r ch e il itis m a y a ls o b e pre se n t.

HAIRY LEUKOPLAKIA AND HIV INFECTION β€ Hai ry le u ko pl ak ia β €™ ha s b ee n de sc ri be d i n a la rg e n u m be r o f pa tie nts w h o a re HIV in fe cte d. It o c c ur s on the l ate ral m ar gin s o f th e to n g ue a n d h a s a fo l de d, co r ru ga ted , o r β€ ha ir y β€™ ap pe ara n c e a n d ge n era ll y i s as ym pto m a ti c . Al th ou g h Can d id a ma y be

as so c ia te d w i th the l es io n , it do e s n o t se em to b e a β € ca n di da l l eu k op la ki a β€™ or c hr on i c hy pe rpl as tic c a n did o si s , as de sc ri be d e a rli er i n this c h ap ter. Th e l es io n a p pe ars to b e as so c ia te d w i th the E ps tei n β€“ Ba rr viru s (h e rpe s , type 4), a s d em on s tra ted bo th b y

hi sto c h em ic a l a n d im mu n o lo g ic a l s tu di es . It is s u gges te d th a t h ai ry le uk o pl ak ia is a p o w erfu l cl in ic a l i n di ca to r o f im mu n o s up pr es si o n in H IV- in fecte d p a ti en ts a n d th a t i t is a pre di cto r o f th e e ven tu a l o n s et o f A IDS. Th is d oe s n o t s eem to be a le si o n w ith s ig n ifi ca n t p rem a lig n an t po ten tia l . No c as es o f m al ig na n t tra n sf o rma tio n i n h ai ry leu k op la ki a h av e a s ye t b een des c rib ed .

PERIODONTAL DISEASE IN HIV INFECTION Th ree pe rio d o nta l ma n if es tati o ns o f HIV h a ve be en des c rib ed : a l in ea r g in g iv al ery th em a th a t

ap pe ars a s a re d b a nd o n th e ma rg in a l g in g iva a n d is n o t pa rtic u la rl y a s so c ia ted w ith p o o r or al h yg ien e ; a n e cro ti zi n g ul ce ra tive gi n gi viti s, a s so c ia ted w ith p a in a n d sp o nta n eo u s ble ed in g ; a n d a n e cr oti z in g u lc era tiv e p er io do n titis th at i s de stru c tiv e a n d ra pid ly pro g res si ve .

KAPOSI'S SARCOMA Th e n e o pl as m th at i s mo s t l ike ly to o cc u r i n th e AIDS p ati en t i s Kap o s i's s ar co m a (Fi g. 4. 8 ).

Th e m o u th an d , in pa rtic u la r, th e mu c o sa o f the h a rd pa la te is a c o mm o n s ite fo r th is le s io n , wh i ch i s , in f ac t, a fo rm o f di ffu s e l ym ph o m a rath er th a n a di sc re te n e o pl as m. Th e l es io n i s des c rib ed a s a pi gm en ted , n o n pa in fu l , sl ig h tly n o dula r l es io n o f th e mu c o sa w ith a

ch a rac te ris tic h is to lo g ic al a pp ea ra n ce . B efo re th e rec o gn i tio n o f AIDS th i s wa s c o n si de red to be a ra re l es io n , c o n fin e d to el de rly pa tie n ts o f sev era l res tric ted ra c ia l gro u ps ( Ba n tu ,

Jew is h , a n d Ital ia n ) o r to pa tie nts o n i mm u n o su p press iv e th e ra py. Its a pp ea ra n ce i n pa tie n ts

of o th er kin d s is n o w s ai d to be vi rtu al ly pa th o gn omo n i c of a cti ve A ID S. Ho m o se xu al m en a re co n s id ere d to be mo re a t ri sk fro m Ka po s i' s sa rc o ma th a n o the r g ro u ps w ith HIV in fe c tio n .

Th e h e rpe s vi ru s 8 (HH V- 8) is n o w c o n si de red to h ave a r ol e in th e ae tio l og y of th is s a rco m a .

Fig. 4 .8 Ka po s i' sa rc o ma o f th e g in g iva te

LYMPHOMA No n- Ho dg kin ' s l ym ph o m a is c o mm o n ly as s o ci ate d wi th HIV i n fec tio n a n d ma y pre se n t a s a sw el li n g o r u lc era tiv e l es io n in th e m ou th .

Gro u p 2 le si o ns i n cl u de a w id e r an g e o f o pp o rtun i sti c in fe cti o n s (Tab le 4. 12) in c lu d in g HP V -

as so c ia te d l es io n s th at ma y af fec t th e o ral c a vity. Sw e lli n g of th e sa li va ry gl an d s, x ero s to mi a (Ch ap ter 8), an d m el an o tic p ig me n tati on h as a ls o b ee n a ss o ci ate d w ith HIV i n fec tio n .

Discussion of problem cases Case 4.1 Discussion Q1

Wh at a d vic e w o ul d yo u g ive th is l ad y?

Th e p a ti en t c a n be ad vi se d to u se a to pi ca l an ti viral pr ep ara tio n , s u ch as a ci c lo vi r o r

pen c ic l ov ir cr ea m. T h is s ho u l d b e ap pl ie d fiv e ti mes da il y s ta rtin g in th e pr od ro m al s ta g e o f

th e le s io n , th at is , w he n s h e f irs t fe el s a β€ ti n gl in g β €™ or β€ pri ck in g β€™ se n sa tio n of th e lip s . In res is ta nt ca s es , o r w h en th e l es io n s rec u r a t re gu l ar in ter va ls , th en i t w o ul d be jus tif ie d to pre sc ri be a pro p h yla c tic c o ur se o f sy ste mi c a ci cl o vir (fo r do s e, s ee

ma n uf ac tu rer 's da ta s he et). E x po s u re to su n ap pe a rs to pre c ipi tate th is l ad y's l es io n s a n d sh e sh o u l d u s e a su n bl o ck l ip pre pa ra tio n w ith a high s u n p ro tec tio n fa c to r (S P F) . It is

ad vis a bl e to rem in d th e p a ti en t th a t c o ld s or es a re hi gh l y i n fec tio u s a n d s h e sh o u ld b e ca ref u l n o t to s ha re to w el s, c u ps , o r c u tle ry wi th o th er f am il y m e mb ers . O ro ge n ital tr an s mi ss i on is a ls o a ris k du ri n g s ex u al a cti vity . Q2

Doe s th e l es io n o n he r l ip po s e a h az a rd to m em bers o f th e de n tal tea m ?

Herp eti c w hi tlo w s w ere c o n si de red to b e a n o c cu p a tio n al h az a rd fo r de nta l h ea lth -ca re

wo rk ers , p rio r to th e u s e o f pr ote cti ve gl o ve s, a n d res u lte d f ro m co n ta ct w i th a pa tie n t pre se n tin g wi th a h erp eti c le si o n o n th eir li ps . P ati en ts w h o bi te th e ir n ai ls m ay

au to in o c u la te th e ir H SV an d de ve lo p a h erp etic w h i tl o w , w hi ch is ex tem ely p ai n fu l a n d dif fic u lt to trea t.

P. 47

Case 4.2 Discussion Q1

Ho w w o ul d yo u m an a ge th is p ati en t?

Yo u sh o u ld in fo rm th e pa tie n t a bo u t h is c a n did a l i nfec tio n a n d ex pla i n th at i t is i mp o rta nt to ch e ck fo r a n u n d erl yin g c au s e or pr ec ip ati ng f ac to r. Deta il ed q ue s tio n in g ab o u t h is m ed ic a l hi sto ry a n d g en e ral h e al th is e ss en ti al , w ith p articu l ar em ph a s is o n re ce n t a nti bi o ti c o r

ste ro id th era py (i nc lu d in g s tero id i n ha le rs fo r as th m a). T h ere m ay be a fa m ily h is to ry o f dia b etes a n d the p ati en t s h o u ld be as ke d sp ec if ic qu es tio n s, rel ati ng to s ym pto m s o f

dia b etes . A t th is s ta ge , the pa tie n t c an b e as ke d if he k no w s a n y re a so n w h y he m ig h t be

su s ce pti bl e to in fe c ti o n s. H e m a y th e n vo lu n te er tha t h e is i n a ris k gro u p fo r H IV in fe cti o n . It is n o t a lw a ys a pp ro pri ate in th e d en tal s u rge ry to as k pa tie nts a b ou t th e ir li fes tyl e or

se xu al ity , bu t th e pa tie n t ma y w is h to di vu lg e thi s in fo r ma tio n . P ati en ts sh o u ld b e ab le to

dis c u ss th e ir m ed ic a l h i sto ry in pri va cy a nd i t mu st al w ay s be c o ns id er ed c on f ide n tia l, b y a l l den ta l sta ff.

Th e pa tie n t s ho u l d b e pre sc ri be d to p ic al o r sy ste mic an tif u ng a ls (fo r ex am pl e, fl uc o n az o l e),

wh i le aw a iti n g th e re su lts o f mi c ro bi ol o gi ca l sa m plin g, a n d a to pi ca l an a lg es ic m o u th w a sh to red uc e o ra l d is c om fo rt. H e sh o u ld b e a dv is ed to ma in ta in hi s fl ui d in tak e an d ea t s o ft f o od s .

Fu rth er in ve s tiga tio n s , in c lu d in g a fu ll ra n ge o f blo o d tes ts , an d fo l lo w -up n e ed to be c ar rie d ou t a t a sp ec i al is t o ra l m ed ic in e (o r or al s u rge ry) un i t. Th e pa tie nt ma y req ue s t HIV tes tin g bu t th is s h o u ld o n ly be do n e a fter pro f es si o n al c oun s el li ng β€” th i s is u su a ll y a va il ab le i n a dep a rtme n t o f g en i to u ri n ary me di ci n e.

Projects 1.

Wid es pre a d a n d o ften in a pp ro pri ate u s e o f a n ti bi otic s ha s re su l ted in th e em e rge n ce o f

ba cte ria th at a re re si sta n t to th er ap y. It i s we ll re co g n iz ed th a t res is ta n ce to an ti bi oti cs

is tra ns fe rre d f ro m o ne b ac ter iu m to a no th e r. Fi n d ou t ab o ut the di ffe ren t me tho d s o f gen e tic e xc ha n ge i n ba c teri a.

2.

Wh at l a bo ra tor y te sts a re cu rr en tly u se d fo r th e di ag n o si s o f H IV in fe c ti o n ? Wh y i s

pro fe ss i on a l co u n s el lin g re co m me n de d fo r p ati en ts pr io r to HIV tes tin g ?

Au th o rs : Title:



Co py rig ht Β © 2003 Ox fo rd U ni ve rsi ty P re ss > T ab le o f Co nt e nt s > 5 - O ra l ulc e r at ion

5 Oral ulceration Problem cases Case 5.1

A 45-yea r-ol d ex - sm o ker is re fe rred to y o u in th e ora l m ed ic in e c li ni c by a c ard io l o gis t. Th e gen tl em an ha s a h is to ry of se ve re, r ec u rren t o ra l ulc era tio n a ff ec tin g th e l ate ra l b o rde rs o f

to n g u e, la bi al m u co s a e, a n d s o ft pa la te. O n e or tw o u lc ers o c c ur a t a tim e, th ey u s ua l ly la s t fo r a bo u t 8 we ek s an d h e is ra rel y u l ce r-free . Th is p a tien t firs t d ev elo p ed u lc ers 9 mo n th s

pri or to th e co n s u ltati o n , af ter b ei n g dis c h arg ed fro m h o s pi tal fo ll o w in g a se ve re m yo c a rdi al

in fa rc ti o n . He is c u rren tl y o n m ed ic a tio n , in c lu d ing a po ta ss iu m -ch a n ne l ac tiv ato r ( n ic or an d il) fo r u n s ta b le an g in a a n d, si n ce th e my o ca rd ia l i n fa rcti o n, ha s b ee n o n as p iri n 75 m g/d ay . Th e pa tien t rep o rts di ffi cu l ty w i th ea tin g an d s w al lo w in g . He h as n o e ye , sk in , o r g en i tal le si o n s. Al l p re vio u s a tte m pts to c o ntr ol h is o ra l u lc era tion w i th to pic a l th era py h av e fa ile d. O n

exa m in ati o n h e ha s la rg e β€ ap h th o u s β €“lik e β€™ ul c ers w ith e vid en c e o f m u co s a l s c arri n g. Q1

Ho w w o ul d yo u m an a ge th is g en tle ma n 's s ev ere RA S a n d w h a t th era pe u tic o p ti o n s are a va il ab le ?

Tw o da ys a fte r yo u r co n s u lta tio n th is p ati en t p res ents w i th se ve re to o th a ch e a nd re qu i res

th e ex trac tio n o f a ma n di bu l ar fi rst mo la r. H e i s ex trem el y a n xi ou s a bo u t d en ta l trea tme n t. Q2

Ho w w o ul d yo u ma n a ge th is d en tal e me rge n cy ?

Case 5.2

A 20-yea r-ol d tea ch e r a tten ds y o ur de n tal pra c tic e, w i th a 5-yea r h i sto ry o f r ec u rren t

ap h th o u s s tom a titis th a t is be co m in g a n u is an c e. The u l ce rs o c cu r ma in ly i ns id e h er ch e eks an d li ps . Th ey u su a ll y l as t 10 da ys a n d tw o o r thr ee o cc u r a t a tim e . Ov er -th e -co u n ter

rem ed ie s h av e b ee n tri ed bu t a re n o t ve ry h e lp fu l. S h e do e s n ot wa n t to b e ref erre d to th e ne ar es t s pe ci al is t u n it, w h ic h i s 50 mi le s aw a y. Q1

Ho w w o ul d yo u m an a ge th is p ati en t i n yo u r pra c tice?

Introduction

Ul ce rati o n o f th e o ra l m u co s a is a co m m on rea s on for p ati en ts to s ee k m ed ic a l o r de n ta l ad vic e . Pa tie n ts ma y r ep or t rec u rre n t ep is o de s o f ora l ul c era tio n , s in gl e ep is o de s of

ul ce ra tio n , o r pe rs is ten t o ra l ul c era tio n . Ca u se s of o ra l u lc era tio n ( Tab le 5. 1) in c lu d e tr au m a, rec u rren t a p hth o u s s tom a titis ( RAS ), m i cro b ia l in fec ti o n s (Ch ap ter 4 ), mu c o cu ta n eo u s

dis e as es (Ch ap ter 11 ), dru g th era p y (Ch ap ter 14 ), an d s qu a mo u s c el l c a rc in o ma ( Ch ap ter

10). Ul ce ra tio n d ue to tra um a , RA S , an d B eh Γ §e t's dis ea se w il l be di sc u s se d in th is ch a pte r.

Table 5.1 Principal causes of oral ulceration

Tra um a

Rec u rren t ap h th o u s sto m a ti tis (R AS ) Mi cro b ia l in fe cti o ns

Mu c o cu ta n eo u s di se a se s Sy ste mi c di so rd ers Dru g th e rap y

Sq u am o u s ce ll ca rc in o m a

Traumatic ulceration Aetiology

Tra um a tic u lc er s c a n be d u e to ph ys ic a l ( me ch a n ic al, th erm a l, el ec tric a l) or c he mi c al tra u ma . Co mm o n c au s es o f me c ha n ic a l tra u m a are s h arp , bro k en d o w n tee th , o rth o d on ti c an d pro s the tic a pp li an c es , a n d n u m b lip s o r to ng u e be ing bi tte n a fter a lo c al a n ae sth e tic in jec tio n .

Ch em ic al tra u ma c a n be c au s ed b y a s pi rin o r β€ to o th a ch e β €™ rem ed ie s be in g pl ac e d dir ec tly o n the o ra l mu c o sa .

As pi rin pl ac ed d ire ctl y o n th e o ra l m u co s a , as a rem ed y fo r to oth a c he , ca n c a us e a c he m ic al bu rn .

Clinical features

Tra um a tic u lc er s a re s o re, p ai n fu l to to u ch , a n d ten d to h av e a n i rreg u la r b o rde r w ith

eryth e ma to u s ma rgi n s an d a ye llo w b a se . Du rin g th e he a lin g p ha s e th e y f req u en tly de ve lo p a β€ kera to tic h al o β€™.

Management

Ma n ag em en t in vo lv es th e el im in a tio n o f th e s u sp ec ted c au s e an d u s e o f a n an ti se pti c

mo u th wa s h (fo r ex am pl e, 0. 2 p er ce n t c hl o rh ex id in e) o r a s im pl e co v eri n g a g en t s u ch a s Ora ba s eΒ ®

du rin g th e h ea lin g p ha s e of u lc era tio n . A ll tra u ma tic u l ce rs sh o u ld b e rev iew e d. If th ey per si st th e n a bi o ps y s h o u ld be c a rrie d ou t to exc lu d e sq u am o u s ce ll c arc in o m a.

If a pu ta tive tra u ma tic u l ce r p ers is ts fo r mo re th an 10β€“ 14 da ys a fte r el im in a tio n of th e

ae tio lo g ic al fa c to r, th en th e pa tie n t sh o u ld b e referre d fo r a s pe ci a lis t o pi ni o n a nd p o ss ib le bio p sy to e xc lu de th e po s s ibi li ty o f an or al ca rc ino m a.

Fa c ti tio u s (s el f-in fl ic ted) o ra l u lc era tio n c a n a lso o cc u r ( se e Ch ap ter 17) a nd m a y b e di ffi cu l t to di ag n os e a nd m an a ge . P sy ch i atri c ad vi ce i s req u ire d i n s o me ca s es .

Recurrent aphthous stomatitis

Rec u rren t ap h th o u s s to ma titis ( RA S) is th e mo s t co mm on o ral m u co s al d is ea s e a ff ec tin g

P. 52

hu m an s a n d h as b ee n rep o rted a s af fec tin g 20β€“ 25 pe r c en t o f th e g en e ra l p o pu la tio n at

an y tim e. A h i gh er pre va le n ce h a s be en r ep or te d in N or th Am er ic an stu de n ts, p a rtic u la rly a t exa m in ati o n tim es , a n d in u p pe r s o ci o -ec o no m ic g ro ups . In tere sti n gly , RA S is i n fre qu en tl y fo u nd i n B ed o ui n A ra bs .

Clinical features

Th e c li n ic a l f ea tur es o f RA S co n s is t o f rec u rren t bo u ts o f o n e o r s ev era l, sh a ll ow , o v o id,

pa in fu l u lc ers , o c cu rr in g at i n terv al s o f a f ew d ays o r u p to 2 β€“3 mo n th s . Th ree c li n ic al

pre se n tatio n s o f RA S ar e rec o gn i z ed : m in o r re c ur rent a ph th o u s sto m ati tis (M iR AS ), m a jo r rec u rren t ap h th ou s s to ma titi s (M jRA S) ; a n d h e rpe tifo rm u l ce ra ti o n (HU ). Th e c li n ic al

pre se n tatio n of th es e type s o f R AS are s h o wn in Table 5. 2. P ati en ts ma y so m eti me s pre se n t wi th a mi xe d p a tte rn o f RA S bu t th i s i s rel ati ve ly u nc o m mo n .

Minor recurrent aphthous stomatitis (MiRAS)

Th is is th e m os t c o m mo n fo rm o f RA S an d a pp ro xim a tely 80 p er ce n t o f p a tien ts h a ve le si o n s

of th is typ e. It is re po rte d tha t 56 pe r c en t o f the p ati en ts a re fem a les a n d th at th e pe a k ag e of o n se t o f the u lc e rs is i n th e s ec o n d de ca de (10β€“19 ye ars ). H ow e ve r, ma n y pa tie nts

exp eri en c e the ir fir st u l ce rs a t a n ag e we ll o u tsi de th es e li mi ts. In de ed , it i s by n o me a ns un c o mm o n to fi n d th i s fo rm o f ul ce ra tio n i n mu c h you n ge r c h il dre n .

In its m os t c h ar ac teri s ti c fo rm M iRA S pre s en ts th e p ic tu re o f a n u mb er o f s m al l u l ce rs (o n e to fiv e) ap pe ar in g o n the bu c c al m uc o s a, th e la bi a l m u co s a , the f lo o r o f th e m o u th, or th e

to n g u e. M or eo ve r, th e ul ce rs a re us u a ll y c o n ce n tr ate d i n th e an ter io r p a rt o f th e m ou th . Th e ph a ryn x a n d to ns i lla r f au c es a re ra rel y i mp li ca te d i n th is fo rm o f u lc era tio n . Th e p ro dro m al

sta ge o f ul ce ra tio n i s va ria bl e, b u t th ere is u s u ally a se n sa tio n des c rib ed a s β€ bu rn in g β€™

or β€ pri ck in gβ €™ fo r a s h o rt pe rio d be fo re th e u l ce rs a pp ea r. F o llo w i ng th is p h as e ,

ul ce ra tio n o c cu rs d ire c tl y b y lo s s o f th e ep ith el ium . Th e u lc ers a re u su a ll y l es s th an 1 cm i n

dia m ete r a nd , in m o s t in s tan c es , th ei r s iz e is a pp ro xi ma tel y 4 o r 5 m m in d ia m eter . Ho w eve r, th e c la ss i fic a tio n o f β€ mi no r β€™ RA S do es n o t d ep en d o n th e di me n si on s o f th e le si o n s

al on e , bu t o n a n u m be r o f cl in i ca l fea tu res . It i s qu i te p o ss ib le to h av e l a rge m in o r u lc e rs

an d sm a ll m ajo r on e s. Th e a p pe ara n ce o f th e u l ce r bas e is g rey -yel lo w , o fte n w ith a re d a n d sl ig htl y ra i se d m a rgi n , an d , u n les s in fl u en c ed by th e si te ( as i n th e d ep th o f th e b uc c al

su lc u s w h ere th ey a pp ea r el o ng a ted) , the y are u s u ally o va l i n s ha p e ( Fi g. 5. 1). Th e u lc ers

are p ai n fu l, p arti cu l ar ly if the to n gu e is i n vo lv ed, an d m ay m ak e e ati n g o r s pe ak in g di ffi cu l t. If th e lip s a re i m pli c ate d, th er e m a y b e a m in o r degre e o f o e de ma i n th e s u rro u n di n g a re a bu t th is i s n ot co m mo n . Ly mp h n o de en l arg em en t is see n o n ly as a r es po n s e to se co n d ary

in fe cti on in s ev ere ly af fe cte d p a ti en ts . Th e co u rs e o f th es e u lc ers v ar ies f ro m a few da ys to a littl e o v er 2 w ee ks , bu t u su a ll y th e ir d u ra tio n is o f th e o rd er o f 10 da ys . A fter th is pe ri od th e

ul ce ra ted a rea s re- epi th el ia liz e a n d h e al o ve r i n an in terv al o f so m e da ys , bu t res o lu ti on ma y or ma y n ot be s im u lta ne o u s in a ll th e ul c ers o f a gro u p. Mi n or ap h th ae h ea l w ith o u t sc a r

fo rma tio n . T hu s , if s ca rs s h o ul d fo rm , it i s pro b able th at th e c o nd iti o n is n o t M iRA S , b ut the ma jo r typ e. F o ll o w in g h ea lin g o f the u lc ers , th er e i s a va ria bl e ul ce r -free i nte rva l β€”3β €“4 we ek s is m os t co m m on β€” bu t m an y pa tie n ts are a bl e to pre di ct w i th so m e de gre e o f

ac cu ra c y th e pe rio d ic ity o f th e c on d iti on . In a f ew p ati en ts , h o we ve r, th e r ec u rren c e o f th e ul ce rs a pp ea rs to b e e n tire ly ran d o m an d , in s o me ca se s, th e re ma y no t b e an u lc er -free per io d be twe en atta ck s, w i th n ew a ph th a e d ev el op in g b efo re e xis tin g o n es h a ve h ea le d.

Table 5.2 Clinical features of RAS Featu r e

Ty pe o f RA S

Min o r ( M iRAS ) Pe ak a ge o f

Maj or ( Mj RAS )

Her petifo r m (H U)

Se co n d

Fi rst an d s ec o n d

Th ird

Nu mb er of

1β€“5

1β€“3

5β€“ 20 (u p to 100)

Si z e o f

< 10

>10

1β€“ 2

Dur ati on

7β€“14 da ys

2 w ee ks β€“ 3

7β€“ 14 da ys

Hea l wi th

No

Yes

No *

Si te

No n -kera tin i z ed

Kera tin iz e d pl us

No n -kera tin i z ed

la bia l /bu c ca l

mu c o sa

pa rtic u la rly fl oo r o f

on s et

(de ca de )

ul ce rs /bo u t

ul ce rs (m m)

sc ar rin g

mu c o sa e sp ec ia ll y mu c o sa .

Dor su m a n d

la te ra l b o rde rs o f

mo n th s

no n - kera tin i z ed

pa rtic u la rly s of t pa la te

mu c o sa , bu t

mo u th a nd v en tra l su rfa c e of to n gu e

to n g u e

* Un l es s a n um b er of u lc er s co a le s ce .

P. 53

Major recurrent aphthous stomatitis (MjRAS)

Ma jo r RA S v ari es f ro m th e m in o r fo rm in a n u m be r o f im po rta n t c lin i ca l fe atu re s. T he u lc e rs

are ge n era ll y l ar ge r th an tho s e o f M iR AS ( Fi g. 5. 2) a nd th e y ar e o f gr ea ter d u ra tio n , u p to a per io d o f m o n ths i n s o me c as es . A s a res u lt o f th e l o n g p eri o ds o f tim e i n vo lv ed , th ere is

pro b ab ly a ma rke d ten de n cy to th e pro d u cti o n o f a he ap ed -up m ar gin wh i ch , w h e n a si n gl e

ul ce r i s se en in is o la tio n , m ay le ad to th e s us p ic io n th a t th e l es io n i s ma l ign a n t. O n eve n tu al

he al in g , th e u l ce rs ma y le av e a s u bs ta nti al s c ar and th is , to ge the r w i th the tis su e d es tru cti o n th a t m ay o c cu r du rin g th e ac tiv e p h as e o f u l ce ra tion , ma y le ad to g ro ss d is to rtio n o f th e

in vo lv ed tis s u es (Fi g. 5. 3). Mj RAS ma y p ro du c e le sio n s th ro u g h ou t th e e nti re o ral c av ity,

in cl u di n g th e s of t pa l ate a nd to n s ill ar a rea s, an d u lc era tio n of ten e xten d s to th e o ro p h ary n x

(Fi g. 5. 4). Th e in vo l vem en t of th e po s teri o r o ra l ti s su e s is s o ch a ra cte ris tic o f M jRA S as to b e dia g no s tic , e ven th ou g h th e ul c ers m ay in i tia ll y b e s m al l.

Fig. 5 .1 A ph th o us sto m a ti tis (m in o r ty pe ) o n to ng u e.

Fig. 5 .2 A ph th o us sto m a ti tis (m a jo r typ e), o n l a bia l mu c o s a, o f 6 w ee ks du ra tio n .

Fig. 5 .3 S ca rri ng o f th e la tera l ma rg in o f th e to n g ue fo l low in g re cu rre n t m ajo r ap h tho u s sto m ati ti s .

Fig. 5 .4 A ph th o us sto m a ti tis (m a jo r) o n th e so f t pa la te.

Th e n u m be r o f u lc ers p res e nt at on e tim e va rie s from o n e to 10 in MjR AS . F req u en tly , a

P. 54

si ng le u lc er w ill p ers is t fo r a lo n g pe rio d , w hi le o th er (u su a ll y s m al le r) u lc e rs fa de . A ffe cte d pa tien ts s h o w a sl ig h t ma le p rep o n de ran c e an d th ere i s a wi de va ri ati on in th e a ge o f

in ci de n ce , a lth o u gh m o st pa tie nts e xp eri en c e th e ir f irs t u l ce rs du ri n g th e fi rst two de ca de s o f lif e. U n lik e Mi RA S , th e re do e s no t ap pe ar to be a cy cl ic a l p atte rn i n MjR AS an d th e u l ce rs a re us u a lly u n pre di cta bl e in th e ir o n se t. Lo ng p eri o ds o f rem is s io n m ay b e f o llo w e d b y i n terv al s

of in te n se u lc er ac tiv ity, wi th ou t an y o bvi o u s pre ci pi tati ng f ac to r. Th e pro l o ng ed a n d pa in fu l

ul ce ra tio n m ay pr es en t s ig n if ic an t p ro bl em s to th e pa tien t. In p a rtic u la r, ea tin g m ay be co m e extre me ly di ffi c ul t a n d th e ge n era l h ea lth o f th e pati en t m a y su f fer a s a c o n s eq ue n ce .

Herpetiform ulceration (HU)

Th is d is ti n cti ve fo rm o f RA S di ffe rs in m a n y w ay s fro m bo th M iR AS an d M jRA S an d it is le s s co m mo n . Th e ter m β € he rpe tif or m β€™ is n o t u n li ke th at of d erm ati tis h erpeti fo rm is i n

der ma to lo gi c al pr ac tic e, i n th at i t re fer s to a mo rp h o lo gi ca l res e mb la n ce to l es io n s c au s ed b y he rpe s vi ru s in fe c ti o n s. H o we ve r, it is a n u nf o rtu na te o n e, c au s in g c o nf u si o n w ith vi ra l

in fe cti on s . In HU th e u lc ers a re s ma ll (1β €“2 mm ) an d mu l tip le (a s ma n y a s 100 u lc ers m a y be pre s en t a t th e sa me ti me ). A lth o u gh a n y n o n -keratin i se d o ral mu c o s a m a y b e in vo l ved ,

ch a rac te ris tic al ly th e a ff ec ted s ite s a re th e la tera l ma rg in s a nd v en tra l s u rfa c e o f th e to n g u e an d th e f lo o r o f th e m o u th (Fi g. 5. 5). In div id u al ulc ers a re g rey an d w ith o u t a d eli n ea tin g

eryth e ma to u s bo rd er, m a kin g th em q ui te di ffi cu l t to vi su a li ze . In s pi te o f th ei r sm a ll s iz e,

th e se u lc e rs ar e v ery pa in f ul a n d ma y m a ke ea tin g and s pe a kin g di ffi cu l t. A si n gl e c ro p o f

ul ce rs m ay la s t fo r ap pro x im ate ly 7β€“14 da ys , a nd th e p eri o d of re mi ss i on be tw e en a tta cks is va ria b le. In so m e in s ta n c es th ere m a y be re pe ated epi s od es o f u lc era tio n wi th ou t an y in terv al o f rem is si o n . Wh ere m an y u lc ers a re pr es ent th ey m ay c o al es ce to f o rm la rge r co n fl u en t a rea s o f u l ce r, u su a ll y w i th ma rke d ery the ma . Th e p ati en ts af fe cte d a re

pre do m in a ntl y f em al e (2. 6:1), th e mo s t c o mm o n a ge of o n s et b ei n g i n th e 20β €“29 -yea r -ol d gro u p. Fo r tu n a tel y, th is f or m o f re cu rre n t u lc era tio n is re la tive ly sh o rt -liv ed , mo s t pa tie n ts

exp eri en c in g s po n tan e o us r em is si o n w ith in 5 ye ars of o n se t. He al in g w ith s ca r fo rm ati on ha s bee n d es cr ibe d in H U bu t th is i s pro b ab ly a re su lt o f co a le s ce n ce . No th in g res em b lin g th e gro s s tis su e di s to r ti o n c au s ed b y th e sc a rs o f M jRAS i s se en a s a n af term a th o f HU .

Fig. 5 .5 A ph th o us sto m a ti tis (h e rpe tifo rm ty pe ) u n de r th e tip o f th e to n gu e β€” mu lti pl e,

very sm a ll , an d , as i s o ften th e c as e , di ffi cu l t to s ee . Th e pa tien t wa s ev en tu al ly fo u n d to ha ve c o el ia c di se as e a nd th e u lc era tio n w a s el imin a ted by a gl u ten -free d ie t.

Recurrent aphthous stomatitis (RAS) in children

RAS is n o t, by an y me a ns , a c o n diti o n res tri cte d to ad u lts . Th e au th o rs rev ie we d o ver 100

pa tien ts w ith w el l -es tab li sh e d p atte rn s o f RA S f ro m the a ge o f 7 ye ars o n w a rds . Th e in i ti a l ap pe ara n c e o f th e u l ce ra tio n w a s, i n th e c a se o f a n um b er o f pa tie n ts, be lo w th e ag e o f 3 yea rs , al th o ug h th es e di d no t i n cl u de c as es o f m ajor ap h tho u s u lc era tio n . Th e fi rs t

ap pe ara n c e o f ma jo r ul ce ra tio n i n th is gr ou p w a s at th e ag e o f 7 yea rs . Th e di stri bu ti on of ty pe s o f u lc e rati o n an d th e pre va le n ce o f h ae ma to log ic al a bn o rm al iti es w a s en tire ly

equ i va le nt to th at d es c rib ed i n mi xe d ag e an d a du l t gro u p s. T he o n s et o f u lc era tio n , c o n trar y to fre qu en tl y e xpr es se d id ea s , di d n o t s ee m to be , in an y w ay , as s o ci ate d w i th pu b erty.

With in th i s gro u p th ere w a s o nl y o n e pa tie n t w ith an a bn o rm al je jeu n a l m u co s a typ ic al o f co e lia c d is ea se .

Aetiology of RAS

Th e c a u sa tiv e f ac to rs o f R AS a re u n kn o w n bu t a re prob a bl y mu l tifa c tor ia l, w ith pre ci pi tati ng

sys te mi c, l o ca l, m ic ro b ia l, a n d ge ne tic fa cto rs a ll b ei ng i mp li ca ted ( Tab le 5. 3). β € Ap h tho u s -

lik eβ €™ ul c ers c an be a ss o ci a te d w ith a n u mb er o f sys tem ic co n d itio n s , bu t th e se m a y n o t exh i bit the c la s si ca l be h av io u r o r pa ttern o f RA S .

Genetic factors

It is h ig h ly li kel y th a t g en eti c fa cto rs p red is po s e to RA S , wi th th e pre va len c e o f th e c on d iti on bei n g mo d ifi ed by e nv iro n m en ta l fa cto rs . A pp ro xi ma tel y 40 β€“ 50 pe r c en t o f RA S pa tie n ts giv e a f am il ia l h is tor y o f R AS . Th e p ro ba bi lity o f a si bl in g de vel o pi ng RA S i s in fl u en c ed by th e ir pa re nts β€™ RA S sta tu s an d th ere i s a h i gh c o rre la tio n o f RA S in i de n tic al tw in s . A nu m be r o f stu d ie s ha ve a ttem pte d to co rre la te as s oci ati on s b etw ee n p arti cu l ar h u ma n leu k oc yte a n tige n (H LA) h ap lo typ es a n d RA S bu t th e ev id en c e i s eq u ivo c a l.

Table 5.3 Possible aetiological associations with RAS

Tra um a

Sm o ki ng c es s ati o n Mi cro b ia l ag en ts Gen e ti c fa cto rs Stre ss

Me n stru a tio n

Fo o d h ype rs en s iti vity

P. 55

Trauma

RAS pa tien ts o fte n re po rt a p hth o u s u lc ers a t si tes o f trau m a , pa rtic u la rly d ue to to o th br us h in g , o r a t th e si te o f a lo c a l an a es th etic in jec tio n .

Smoking cessation

Th ere h a ve be en s ev era l rep o rts o f a n e ga tive as s o ci ati o n be tw een sm o ki ng a n d RA S. A si gn if ic an t nu m be r o f pa tie n ts r ep or t th at u l ce ra tio n a pp ea red a t th e tim e th e y s to pp ed sm o kin g . To ba c co ma y in c rea se mu c o s al ke rati n iz a ti on , w hi c h ma ke s th e m u co s a le s s

su s ce pti bl e to u l ce ra tio n . In s us c ep tib le pa tie n ts , n i co tin e re pl ac em en t th e ra py (fo r exa m ple , wi th tra ns d erm al p atc h es ) h a s be en tri ed w ith var iabl e o u tc o me s. A l tern a tive ly , str es s, a fte r sto pp in g s mo k in g, m a y p la y a r o le in th e in c rea s ed s u sc ep tib il ity to RA S .

Stress and menstruation

Th es e fac to rs h a ve bo th b ee n im pl ic ate d in pl ay in g a si gn i fic a n t ro le in the a eti o lo gy o f R AS . A rec en t d eta il ed re vie w o f th e l ite ratu re w a s un a bl e to es tab li sh a de fin i te a s so c ia tio n

betw e en R AS a n d th e m en s tru a l c yc le , a lth o u gh th er e a re m an y an e cd o tal re po rts o f wo m en wh o rel ate th ei r R AS to th e lu te al p ha s e of th ei r cyc le . Th er e i s al so n o co n vi n ci n g ev id en c e th a t p sy ch o lo g ic a l i ll ne s s or s tres s is re la ted to RA S .

Microbial factors

Th ere h a ve be en n u m ero u s s tu d ie s to in ve s ti ga te the ro le o f mi cro -or ga n is ms i n RA S . Lin ks

ha ve b ee n pro p o se d wi th o ra l s tre pto co c c i, h erp es sim pl ex vi ru s, v ar ic el la z o ste r vi ru s , an d cyto m eg a lo vi ru se s bu t f ur th e r stu d ie s are re qu i red to p ro ve o r re fu te su c h a ss o ci a ti o n s.

Food hypersensitivity

Th ere is n o p ro ve n a ss o c ia ti o n be tw ee n h ype rs en s itivity to c erta in f o od s a nd RA S , o th er tha n in g lu ten -se n si ti ve en te ro pa thy (c o el ia c di se as e ).

Histopathology and immunopathogenesis of RAS

Th ere ar e th re e ph a se s in th e de ve lo pm en t of a n a phth o us u l ce r, th e pre u lc era tiv e,

ul ce ra tive , a nd h e al in g ph a se s . His to lo g ic al ly , ap htha e a re m u c os a l ul ce ra tio n s w ith a m ixe d in fl am ma to ry in fi ltra te. C D4 + T l ym ph o c ytes a n d la rge gra n u la r l ym ph o c ytes p red omi n ate in th e p reu lc e rati ve an d h ea l in g ph a se s an d CD8 + ce ll s pre do m in a te in th e u lc era tiv e p h as e.

Nu me ro u s s tu di es h a ve be en c o n du c ted o n th e im mu n o pa th o ge n es is o f RA S an d th ere i s go o d

evi de n ce th at pa tie n ts wi th RA S s ho w e vi de nc e o f i mm u n e dys re gu la tio n . It h a s be en

po s tu l ate d th at, i n th e p res e nc e o f a l o ca l o r s ys tem ic s tim u lu s , the e pi the li al c el ls be co m e ta rg ets fo r the c yto to xi c ac tio n o f ly mp h oc yte s an d m o no c yte s an d a re su b s equ e n tly

des tro ye d. C u rren t e vi de nc e w o u ld su g ge st tha t RA S i s n ot an au to i mm u ne d is ea s e. Th e im mu n o pa th o ge ne s is o f th i s co m mo n d is o rd er, h o w ev er, i s ye t to be e lu c id ate d.

Table 5.4 Systemic conditions and β€ aphthous-likeβ€™ lesions Be h Γ§e t's d is ea se

Nu tr itio n a l de fi ci en c ies

Gas tro in te sti na l di so rd er s Cyc li ca l n eu tro pe n ia HIV in fe c ti o n

MA GIC sy n dro m e FA P A sy n dro m e Dru g rea c tio n s

Systemic conditions and β€ RAS-likeβ€™ lesions

Tab le 5. 4 gi ve s a l is t o f sy ste mi c co n d itio n s a s so cia ted w ith RA S . Be h Γ§e t's d is ea se w ill b e dis c u ss ed i n th e n e xt se c tio n o f th i s ch a pte r.

Nutritional deficiencies

Hae m ati ni c (ir on , f o lic a c id , o r vi tam in B 1 2 ) de fi ci en c ie s h ave b ee n rep o rted to b e tw i ce a s

co m mo n i n RA S pa tie n ts as i n co n tro l s an d u p to 20 pe r c en t of RA S p ati en ts ma y h av e a ha em a tin ic d ef ic ie nc y. B- co m pl ex de fic ie n cy (B 1 , B 2 , an d B 6 ) h as b ee n re po rte d i n a S co tti sh co h o rt o f RA S pa tie n ts a n d z in c de fi ci en c y h a s be en i mp li ca ted i n a fe w pa tie n ts .

Rep la ce me n t th era p y i n RA S pa tie n ts wi th n u tr itio n al d ef ic ie n ci es h a s n ot, h o w ev er, m e t w ith un i fo rm su c c es s.

Up to 20 p er ce n t o f R AS p a tien ts m ay h a ve a h ae ma tin i c de fi ci en c y.

Gastrointestinal disorders

Th e a s so c ia tio n o f RA S w ith c o el ia c di se as e an d Cr oh n 's d is ea s e is fu l ly di sc u ss e d i n Ch ap ter 12. Th e p re va len c e o f c o el ia c di se as e in pa tien ts wh o pre s en t w ith R AS is n o w th ou g h t to b e les s th an 5 pe r c en t. P a tie nts w i th es ta bli s he d Croh n 's di se a se fr equ e n tly rep o rt o ra l ul ce ra tio n th a t is β € ap h th o u s -lik e β€™.

Neutropenia

A la rge p erc en ta ge o f pa tien ts w ith cy cl ic a l n e u tr open i a pre se n t w ith β€ ap h th o u s -lik e β€™

ul ce ra tio n th a t o cc u rs a t in ter va ls (o fte n m on th ly ), re fle c ti n g th e ir ne u tro pe n ic s tatu s . Oth e r ma n ife sta tio n s o f n eu tro pe n ia in c lu d e f ev er, m a la ise, a n d su s ce pti bi li ty to ba c teri al a nd

fu n ga l i n fec tio n s . P ati en ts w h o ar e f u nc tio n a ll y n eu tr op en i c (fo r ex am pl e, th o s e w i th ch ro n i c gra n u lo ma to u s di se as e o r b en ig n fa m il ia l n eu tro pe n ia ) a re a ls o s us c ep tibl e to β € ap h th o u s -

ty pe β€™ u lc era tio n .

HIV-associated aphthous stomatitis

Th es e ul ce rs ten d to o cc u r i n c ro ps o f fiv e or fe w er u l ce rs , o n n o n -kera tin i z ed mu c o s a a n d

ma y res e mb le mi n o r o r m a jo r a ph th a e. Th e y a re fre que ntl y v ery pa i nf u l a n d la st fo r se ve ra l

mo n th s. T hi s typ e o f u lc era tio n ca n b e e xtre me ly debi lita tin g in the s e pa tie n ts an d c an c a u se pro b lem s w ith e ati n g. C are m u st b e tak en to e xc lu d e H SV o r c yto m eg al ov iru s i nf ec tio n i n HIV- in fe cte d p a ti en ts . In a dd itio n , s o me o f the drug s u se d to

tr ea t HIV i nf ec tio n m a y p red is po s e pa tie n ts to or al u lc er ati on eith e r di rec tly o r i n di rec tly, by

P. 56

ca u si n g m ye lo s u pp res s io n . Th a lid o mi de h a s su c c es sf ull y b ee n u se d to trea t HIV -as so c ia te d RAS .

Other systemic conditions and factors

Oth er s yste mi c co n d itio n s a s so c ia ted w ith β € RAS -lik e β€™ ul c era tio n i nc l ud e MA GIC (m o uth an d ge n ita l u l ce rs w ith i n fla m ed ca rti la ge ) s yn dr ome , FA P A (p eri od ic f eve r, a ph th o u s u lc ers ,

ph a ryn gi tis , an d c erv ic al a de n itis ) sy nd ro m e, a n d rela tiv e Ig A de fi ci en c y. Dru g s su c h a s

no n s tero i da l a n ti- in fl am ma to ry dru g s (NS AIDs ), n ic oran d il , a po ta ss iu m - ch a n ne l ac tiv ato r, an d me th o tr exa te h av e b ee n im pl ic a ted in β € RAS -lik e β€™ ul c era tio n .

Management of RAS History and examination

Th e c o rre ct dia g n os is o f a pa tie n t w ith R AS i s de pen de nt o n a de tai le d a n d ac c u rate c li ni ca l hi sto ry a n d e xa mi n ati o n o f th e u lc er( s). Im po rta n t fa c to rs to be e lic ite d in th e h is to ry a re sh o w n in Tab le 5. 5.

Wh en e xa mi n in g pa tie nts w i th o ral u lc era tio n i t is e s se n tia l to c a rry o ut an e xtra o ra l

exa m in ati o n in c lu d in g pa lp a ti o n o f th e c erv ic al l ymp h n o de s (s ee Ch ap ter 7). Th e o ral

mu c o sa , in c lu d in g th e a re a of u lc er ati on , s h o u ld then b e sy ste ma tic a lly ex am in e d. Tab le 5. 6 lis ts th e i mp o rta nt fea tu res to be n o te d w he n e xa mini n g a p a tien t wi th o ra l u l ce rati o n .

Cli n ic al ev al u ati o n o f a n u lc er in c lu de s in s pe cti o n a nd p al pa tio n , w h ic h c o mp le me n t ea c h

oth e r. Th e ba s e o f th e u lc er ca n b e ne c ro ti c , gra n ula r p u ru len t, o r co v ere d w i th mu c u s. T h e co n s is ten c y o f th e b as e (s o ft, fi rm, o r h ard ) a n d fi xati o n to u n de rly in g str uc tu res c a n be

as ce rta in ed b y p a lpa tio n . Th e e dg es o f th e u lc e r c an b e irr egu l ar , stra ig h t, o r u n de rm in ed

an d ma y fe el h ard i n co n tra s t to th e su rr o un d in g tis su e . Th is i s th e c h a rac ter is tic in d u rati o n, as so c ia te d w i th n eo p la sti c in fi ltra tio n . A n oth e r fea tu re o f a ca rc in o m a is its ro l le d b o rde r. Th e ti ss u e su rro u n d in g the u l ce r m ay b e w h ite , s pe ckl ed , er yth em ato u s , o r n o rm al in ap pe ara n c e.

Th e d ia gn o s is o f RA S is n o t u s u al ly di ffi cu l t an d may b e d ed u ce d, i n m os t c a se s, fro m th e hi sto ry a n d c h a rac teri s ti c c lin i ca l ap pe a ran c e. If th ere i s an y do u bt ab o u t th e d ia gn o s is ,

ap pro p ria te dia g no s tic tes ts sh o u ld b e arra n ge d to ex cl u de o th er c a u se s o r o ra l u lc era tio n .

Further investigations of RAS

Pa tie n ts w i th pe rs is ten t a n d tro u b le so m e RA S s ho u l d u n d erg o s cre en i ng to c h ec k fo r a n

un d erl yin g h a em ati n ic de fi ci en c y ( Tab le 5. 7). Th is i n cl ud es a f u ll bl o od c o u n t an d fi lm a n d me as u rem en t of in f la mm a to r y m ar kers a n d h ae ma tin i cs (s e ru m ferr itin , s er um B 1 2 , se ru m

an d red c el l fo la te) (Ch ap ters 2 an d 13 ). S cre en i ng f or de fi ci en c ie s of vi tam in B co m pl ex es o r zi n c de fic ie n ci es i s n ot ro u ti n el y c a rrie d ou t, b u t ma y be in d ic a te d in c er tai n gro u p s o f

pa tien ts . P ati en ts w ith RA S a s so c ia ted w ith a s ys tem ic c o n di tio n sh o u l d b e ref erre d to the

ap pro p ria te sp ec ia li st fo r f u rthe r i n ves tig a ti o n s an d ma n a ge me nt. If th ere i s an y su s pi c io n o f co e lia c d is ea se , ei th er du e to th e pa tie n t's h is tory o r evi de n ce o f ma la bs o rp tio n o n ro u tin e

te s ti n g, th e n se ro lo g ic a l te sti n g f o r a pp ro pri ate IgA au to an ti bo d ies s h o u ld be c a rrie d ou t (s ee

Ch ap ter 12) a nd th e pa tie n t ref err ed to a ga s tro en tero l o gi st f o r e nd o sc o p y a n d b io p sy o f the sm al l in tes tin e .

Table 5.5 RASβ€”important points in the history Ag e of o n se t

Fa mi ly h is to ry

Fre qu e nc y o f u l ce ra ti o n Dur ati on of u lc era tio n Nu mb er of u lc e rs

Si te o f u lc ers ( no n - kera tin i z ed o r ke rati n iz ed ) Si z e a n d sh a pe o f ul c ers

As s oc i ate d m e dic a l co n d itio n (s ) Gen ita l u lc era tio n Sk in pro b le ms

Gas tro in te sti na l di stu rb a nc e s Dru g h is to ry

Table 5.6 Oral ulcerationβ€”important features to be noted Nu mb er of u lc e rs

Si z e a n d sh a pe o f ul c er(s ) Si te o f u lc era tio n Ed ge o f u lc er Ba s e o f u lc er

Su rro u n d in g tis su e Sc a rrin g

Co n si ste n cy

Table 5.7 Investigations for patients with persistent RAS Hae m og lo b in a n d fu ll bl o o d co u n t ES R/C RP Se ru m B 1 2

Se ru m/r ed c ell f ol ate

An ti -gli ad in an d an ti -en do m ys ia l au to a nti bo d ie s *

* If gl u ten -se n si ti ve en te ro pa th y (c o e lia c di s ea se ) i s su s pe c te d (s ee Ch ap ter 12).

Therapy for RAS

Tab le 5. 8 sh o w s s o me o f th e th era pe u tic o pti o ns a vail a ble f or th e m a n ag em en t o f pa tie n ts wi th RA S . So m e o f th es e me dic a tio n s a re a va i la bl e ov er th e c o un te r a n d o th e rs req u ire a

pre sc rip tio n . P a ti en ts re qu ir in g sy ste mi c im mu n o m o du la to rs a re b es t m a na g ed in a h o sp ita l se tti n g. T he c h o ic e of th era p y fo r RA S de pe n ds o n th e se ve rity an d fre qu e nc y o f u l ce rati o n bu t th e o bje cti ves o f trea tm en t a re to rel ie ve di sco m fo rt, re du c e s ec o n da ry

in fe cti on , p ro m ote h ea l in g o f ex is tin g u lc era tio n , a n d p re ven t n e w u lc ers o c c ur rin g .

Table 5.8 Therapeutic options for RAS* Ty pe

Th er apy

To pi ca l an ti se pti c

Ch lo rh ex id in e gl u co n a te (m ou th w a sh )

To pi ca l an a lg es ic s

Be n z yda m in e h ydro c h lo ri de (m o u th w a sh )

To pi ca l co rti co s tero i ds

Hyd ro co rti so n e h em is u cc i na te (pe ll ets )

Lign o c ai n e rin s e

Tria m ci n ol o n e ac e ton i de (i n ad h es iv e p a ste ) Be tam eth a s on e va le ra te (m ou th w a sh ) Be cl o me th as o n e d ip ro pi o n ate (s pra y) Bu d es o n id e (s pra y)

Tria m ci n ol o n e ( wi th o r w ith o u t c h lo rte tr ac yc li n e) mo u th wa s h To pi ca l an tib io ti c

Ch lo rte tr ac yc li n e m o u thw a s h

Sy ste mi c

Pre dn i so l o ne

im mu n o mo d u la tor s

Az a th io pri n e Co lc h ic in e

P. 57

Cic lo s po ri n

Th al id om i de Mi sc el la n eo u s

Cim ete di ne

Car be no x o lo n e ( mo u th w as h a n d s ys tem ic ) 5 am in o -sa li cy li c a c id Dap so n e

Pe n to x ph yl li n e

Low -en erg y la s er Leva m is o le

*S e e Ch ap ter 3 fo r f u rth er d eta il s o f to pi ca l th e rapy.

ANALGESIC AND ANAESTHETIC PREPARATIONS To pi ca l an a lg es ic s p ray s o r ri ns e s su c h a s be nz y da mi n e h y dro c h lo ri de (Dif fla m Β ® ) ca n be

us e d to r ed uc e di sc o m fo rt. Ho w ev er, 2 pe r c en t l ig noc a in e ge l, u s ed d ire ctl y o r di lu ted a s a

rin s e, i s mo re ef fe cti ve fo r s ev ere c as e s of RA S . Ca re mu s t b e tak en i f u s ed i n th e p o ste rio r

pa rt o f th e m o uth as s tro n ge r a n al ge si c pre pa ra tio ns c an a ff ec t th e la ryn g ea l re fl exe s . Lo ng te rm u se o f to pic a l li gn o c ai n e i s n ot ad vi sa bl e, a s it ma y be ab s o rbe d a n d ca u s e s ys tem ic eff ec ts. β € Ove r -th e -co u n ter β€™ th ro a t l o ze n ge s co n ta in in g a loc a l a n a es the tic , o fte n co m bi ne d w ith a n a nti se pti c, c a n be u s ed to re li eve d is co m fo rt f ro m RA S bu t m o s t

pre pa ra ti o n s co n ta in s u ga r a n d sh o u ld n o t b e u s ed co n tin u o us l y i n de n tate pa tie n ts. So m e pa tien ts m a y a ls o re qu ir e s ys tem ic a n a lg es ic s su c h a s ib u pro fe n o r p a rac e ta m o l,

al th o u g h it i s im po rta n t to re me mb er th at th ere is a p o ss ib il ity, al be it ra re , th at N SA IDs c an pre ci pa te RA S .

COVERING AGENTS Se ve ral p as tes a n d ge ls c an b e u se d to c oa t th e s urfa ce o f th e u lc er s an d to fo rm a

pro tec tiv e ba rrie r a ga in s t se c o nd a ry i n fec tio n a n d fu rth er me ch a n ic a l i rrita tio n ( Ch ap ter 3).

So m e dif fi cu lty m ay be e xpe ri en c ed in a p ply in g c erta in o f th es e pre pa ra tio n s , pa rtic u la rly to la rge u lc er s an d to th o se a t th e ba ck o f th e mo u th . It i s al so aw kw a rd to ke ep th es e

β€ co ve rin g s β€™ in p la ce o n th e to ng u e an d l ips w h e re co n s tan t m o ve me nt ten ds to wi pe

th e m o ff. In s p ite o f th es e di sa d va nta ge s , h ow e ve r, su c h s im pl e r em ed ie s are o fte n h el pfu l in co n tro ll in g th e s ym pto m s o f m in o r ap h tho u s u lc e ratio n.

ANTISEPTIC AGENTS An tis e ptic s u bs ta n ce s tha t m a y b e h el pfu l in te m po ra ril y re du c in g s ec o n da ry in fe cti o n are ava i la bl e i n w id e va rie ty a n d su i tab ly fo rm u la ted as m o u th wa s h es , ge ls , a nd p as til le s

(Ch ap ter 3). Th e res po n s e is va ri ab le , as m ig h t be e xpe c ted in vi ew o f the w id e ra ng e o f or ga n is ms i nv o lve d, bu t i n ma n y c a se s s om e de gre e of rel ie f i s ac h ie ve d. C hl o rh exi di n e

mo u th wa s h es a re wi de ly u se d fo r th e sy mp to ma tic treatm en t of RA S a n d a re c o ns id er ed

he lp fu l by ma n y pa tie nts , pa rtic u la rl y i f o ra l h ygien e is d if fic u lt to ma in ta in b ec a us e o f o ra l

ul ce ra tio n . E xtri ns ic s ta in in g o f te eth as so c ia te d w i th lo n g-te rm u se o f ch l o rh exi di n e m a y b e a p ro bl em .

TOPICAL ANTIBIOTICS A mo re ef fe cti ve me as u re in th e rel ie f o f s ymp to ms c a u se d b y se co n d ary i nf ec tio n i s the

ap pl ic ati o n o f to p ic al an ti bi oti cs . A m o u thw a s h co nta in in g 2 p er ce n t tetra c yc li ne o r

ch lo rte tra cy cl in e is o fte n h ig h ly eff ec tiv e i n re duc in g the p ai n c au s ed b y s ev ere u lc e rati o n

an d , as a r es u lt o f the m u ch les s h ea vi ly c ol o n iz ed e n viro n m en t, th e ul c ers o fte n h ea l mo re

rap id ly th an o th er wi s e (Ch ap ter 3 ). Th e trea tme n t of HU de pe n ds la rg el y o n th i s th e ra py, a n d res po n s e to th e a n tibi o tic mo u th w as h i s o ften r ap id a nd c o mp le te. T he re ar e o b vio u s

dis a dv an ta ge s, h o w ev er, in th e u se o f bro a d -sp ec trum an tib io ti cs f or th is p ur po s e, th e ris k o f hy pe rse n si tiv ity r ea cti o n s a n d th e e n co u ra ge me n t o f gro w th o f r es is tan t o rga n is m s bei n g th e

mo s t im po rta n t. Lo ca l se c o nd a ry i n fec tio n b y op po rtu n i sts su c h a s Can d id a se em s to b e m u c h

les s o f a pro b lem , bu t, a s be en p o in ted o u t i n Ch apter 4, an β € an tib io tic so r e to n gu e β€™

(ac u te eryth e ma to u s ca n di do s is ) m a y v ery o cc a si o n ally de vel o p. In vi ew o f the d es ira b ili ty o f lim iti n g th e lo c a l u s e o f a n tibi o tic s, th i s is a form o f trea tme n t th at s h o u ld be u s ed s pa rin g ly an d , in th e ma jo rity o f ca s es , as a s i ng le c o ur se of trea tme n t fo r a n i so l ate d atta ck o f ul ce ra tio n .

Th e r es po n s e o f HU to a 2 p er c en t c h lo rtetra c yc li ne m o u thw a s h is o fte n ra pi d.

TOPICAL STEROIDS To pi ca l ste ro id s ca n b e e ffe c tive dru g s in th e treatm en t o f RA S. Pa tie n t res p on s e is va ri ab le an d th ere a re so m e in d ivi du a ls w h o g ai n li ttl e o r no re li ef fro m th ei r u s e. S tero i ds u s ed in

th i s ma n n er h av e tw o m o de s o f a c ti o n . Th ei r a n ti -infl am ma to ry ac tio n m o di fie s , in a mi n o r

wa y, th e pr og re ss o f the u l ce rati o n a t al l sta ge s an d , to s o me ex ten t, red u ce s th e d is c o mf or t exp eri en c ed . Th e se c on d e ffe ct of s tero i ds , n am el y, th e sp ec i fic b lo c kin g e ffe ct o f th e T

lym ph o c yteβ €“epi th el ia l c el l in tera c tio n , is mu c h mo re im po rta n t in th e pr es en t c o n text.

Si n ce th e co n c en tra tio n o f se n si tiz e d l ym ph o c ytes oc c ur s bef o re an d du ri n g th e e arl y s ta ge s of

ul ce ra tio n , it f o ll ow s th a t th e d ru gs e xe rt th ei r max im u m e ff ec t a t th is tim e. W ith th e

es tab li sh m en t o f th e u l ce ra ti o n a nd th e fa ll in se nsi tiz ed ly mp h o cy te co n c en tra tio n , th e

P. 58

sp ec if ic bl o ck in g ef fec t o f th e s ter oi d be co m es l ess im po rta n t, an d o n ly th e a n ti -in fl am ma to ry ac tio n o f th e d ru g op er ate s. F o r th i s rea s o n it i s im po rta n t th at the p ati en t s h o u ld

un d ers ta nd th a t, wh a tev er pre pa ra tio n i s u se d, i t is o f ma xi mu m v al ue a t th e tim e th a t th e ea rli es t pro d ro ma l s ign s o f u lc era tio n a re n o tic ed .

If u s in g a top ic a l s ter oi d fo r R AS , a im to u s e a pre pa ra tio n th at is le ss l ik ely to g ive s id e eff ec ts be ca u se o f sy ste mi c ab s o rptio n .

Th e d ru gs m o s t co m m on l y a do p ted fo r lo c al o ra l ap plic ati o n in R AS a re h yd ro co rti so n e

he mi s uc c in a te ( as p el lets o f 2. 5 mg ) a n d tria mc i no lo ne a c eto n id e ( in a n a dh es i ve pa s te

co n tai n in g 0. 1 pe r ce n t o f th e s tero i d). T he re is little ri sk o f a d ren a l s u p pre ss io n p ro vi de d

th a t th e r ec o mm en d ed do s e (fo u r ti me s da il y) is a dher ed to . Little i s kn o wn ab o u t th e lo n g te rm ef fe ct o f s ma ll do s a ge s o f to pi c al s tero id th er ap y i n c h ild ren bu t pre s cri bi n g o f th es e sh o u ld b e a pp ro a ch e d w i th ca u tio n , i n thi s gr ou p o f pa tie n ts.

Hyd ro co rti so n e s uc c in a te ( 2.5 mg ) ta bl ets (Co rl a n Β®) are d is so l ved f ou r tim es d ai ly in vic in i ty o f ap h tho u s u lc e r. S tart in th e pro d ro ma l ph a se o f RA S .

Tria m ci n ol o n e ( Ad co rty lΒ ® ) in o ra ba s e p a ste s ho u l d b e ap pl ie d on a mo i st f in g er to dri ed

ul ce rs , fo u r ti me s da il y. M a ny p atie n ts fin d th is pr ep ara tio n d if fic u lt to u se bu t it a pp ea rs to be reta in e d bette r, if ap pl ie d at n i gh t.

In Mi RA S u n res po n s ive to th e se pre pr ati on s a n d in MjRA S it ma y be n ec es s ary to u s e a m o re po ten t s ter oi d pre pa ra tio n , s u ch a s a be tn es o l ( o r tria m ci n o lo n e) rin s e or s te ro id s pra y (s ee Ch ap ter 3).

SYSTEMIC THERAPY FOR RAS In se ve re c a se s o f R AS , pa rtic u la rl y M jRA S , it m a y b e n ec es sa ry to u s e s o me f or m of

sys te mi c th e ra py. Ho w ev er, a ll d ru gs h a ve si de - eff ec ts an d ri sk s th a t m u st b e w eig h ed

ag ai n st th eir be n ef its . Ap a rt fro m p red n is o lo n e, a n u m be r o f sy ste mi c dru g th era pi es h av e bee n a dv oc a ted fo r the tre atm en t o f M jRA S (Tab le 5.8) an d , in so m e c a se s , B eh Γ§e t's

dis e as e (se e n ext se cti o n ). Th a lid o mi de h a s be en u se d su c ce s sf ul ly fo r se ve re R AS th a t h a s fai le d to res po n d to o th er trea tme n t mo d al iti es . It h a s a l so p ro ve d us e fu l i n HIV -as so c ia te d

or al u lc era tio n . Th a lid o mi de i s a T NF Ξ ± in h ib ito r th a t h as a n ti- in fl am ma to ry eff ec ts. Its u s e is li mi ted be c au s e o f its te rato g en i c eff ec ts. T h alido m id e mu s t n o t b e u s ed fo r w o me n o f ch il d- bea ri n g a ge . P eri ph e ral n e ur op a th y is a s id e-eff ec t o f th e ra py an d re gu l ar ne rve

co n du c tio n te sts (e le ctr om yo g ra ph y, E M G) mu s t be c ar rie d o u t. P ati en ts sh o u ld re c eiv e

co u n se ll in g , pri o r to s tarti n g dru g trea tme n t. Co lch ic in e a ffe c ts th e fu n c tio n o f po ly mo rp h s by in h ib itin g th ei r m ig ra ti o n to si tes o f in fl am m ati o n. T h e u s e o f c im eti di ne (H 2 -rec ep to r

blo c ke r), ca rb en o xo l o ne s o di u m, a n d ma n y o th er treatm en t m o da li ti es h a ve a ls o be en tr ied fo r RA S (Tab le 5. 8 ) w ith l ittle s u cc e ss .

Th al id om i de ca n b e us e d f o r s eve re RA S a nd B e h Γ§e t's s yn dr om e bu t pa tie n ts mu s t rec e ive co u n se ll in g . Th al id o mi de m us t n o t b e us e d f o r w o me n o f ch i ld - bea ri n g a ge . P eri ph e ral

ne u ro pa th y i s a rec o gn i ze d si de -eff ec t o f th e ra py an d p ati en ts mu s t h a ve n erv e c o n du c tio n stu di es o n a re gu l ar ba s is .

BehΓ§et's disease

Be h Γ§e t's (p ro n ou n c ed β€ Be tch e t's β €™) sy n dro m e w a s fi rst rec o gn i ze d in Tu rke y a n d w as

or igi n al ly th o ug h t to b e a d is ea s e o f M ed ite rran e an or ig in . Ho w ev er, a l arg e n u mb er of c as e s ha ve s in c e bee n re po rte d i n Ja pa n a n d oth e r co u n tries . Wo me n a re mo re c o mm o n ly af fec te d th a n m en . B eh Γ §e t's s yn dr om e is a ra re dis e as e ch a ra cte riz e d b y a c l as s ic al tria d o f R AS

(an y o f th e th ree cl in i ca l va ria n ts), ge ni tal u lc e rati o n, a n d in fl am m ato ry ey e l es io n s . O the r

ma n ife sta tio n s i nc lu d e sk in , jo in t, n e ur ol o gi ca l, vas c u la r, a nd i n te s tin al di so rd er s. U p to 90

per c en t o f af fec ted p ati en ts h av e RA S. G en ita l u lcers a re si m ila r to th o s e o f th e o ral m u co s a

an d h ea li n g m a y ta ke pl ac e w ith s c ar fo rm ati o n an d l ea d to res id u al tis s u e l o ss a n d de fo rm ity si mi la r to th a t in th e o ral c a vity . Th e oc u la r i n volv em en t i n itia ll y ta ke s th e f o rm o f a n teri or

uv ei ti s , a su p erf ic ia l i n fla m ma to ry les i on o f th e an te rio r p a rt o f th e e ye, w hi c h ma y be co m e

mo re s eve re in l ate r e pi so d es a n d, p erh a ps , p ro gre ss to in vo lv e o the r s tru c tur es o f th e e ye. Th is m ay le ad to p erm a n en t d am a ge by s ca r f o rma tio n o r, ev en , to b li nd n es s .

Th e s e qu en c e o f i nv o lve m en t o f th e o ral a n d ge ni tal m u co s a is va ri ab le , an d th e two ma y no t be in vo l ved si m ul tan e o us l y. In de ed , th ere ma y be a c o ns i de rab le in te rva l betw e en th e

in vo lv em en ts . Oc u la r in vo lv em en t, h o w ev er, i s us u a ll y l ate , o cc u rrin g s o me tim es af ter ma n y

yea rs o f in term itte n t o ral a n d gen i tal u lc er ati on . In th e g en er al iz ed fo rm o f the d is ea s e, s kin les io n s o f va rio u s ki n ds m ay a pp ea r, th e mo s t ch a racte ris tic b ei n g p a pu le s th at p ro c ee d to

pu s tu l e fo rma tio n . It i s in ter es tin g to n o te th a t in so m e pa tien ts w ith sk in l es io n s th ere is a ma rke d sk in r ea cti o n to tra um a . Th e ten de n cy o f steril e b li ste rs to de ve lo p at ve ne pu n c tu re

si te s is k n ow n a s β€ pa the rg yβ€™, a n d it m a y b e u s ef u l a s a di ag n o sti c in di ca to r. P a th e rgy

is le ss c o m mo n ly re po rte d i n U K pa tie n ts wi th B eh Γ §e t's d is ea se .

Th e n e u ro lo gi c al di se a se th at ma y oc c u r i n th es e patien ts i s the re su l t o f th e a pp ea ra nc e o f ce n tr es o f in fl am ma tio n a n d n ec ro s is w ith in th e c entra l ne rvo u s s ys tem . Th e sy mp to ms a re var ia bl e, d ep en di n g u po n th e lo c ati o n o f th e le si o ns, b u t in th e e arl y s ta ge s the y m a y

res em bl e th os e o f m u lti pl e s c le ro si s. T h ere is a v as c u liti s, p er ha p s co m pl ic ate d by th ro mb o tic epi s od es , th a t ma y be ei th er lo c al iz ed a n d m i no r o r in v o lve m ajo r ve ss el s . Th e e ff ec t o n th e joi n ts is th at o f a n o n -sp ec if ic a rth ro pa thy . It h as be en s u gg es ted th a t m ajo r an d mi n o r

cri teri a sh o u ld b e u s ed to a rri ve at a di ag n o si s o f B e h Γ §e t's d is ea se . Th e ma jo r c ri teri a are or al u lc era tio n , g en i ta l u lc era tio n , e ye le si o n s, an d sk in l es io n s . Th e mi no r cr iter ia in c lu d e

les io n s o f th e n e rvo u s s ys te m , va sc u la r s ys tem , jo in ts , ga s tro in tes tin a l trac t, a nd pu l mo n a ry

sys te m. H ow e ve r, th ere is n o a g ree me n t as to th e n u mb er or typ es o f le si o n n ec es s ary to a rri ve at th e d ia gn o s is . A n in tern a tio n a l

P. 59

wo rk in g pa rty ha s red ef in ed th e cr iter ia fo r the d ia gn o s is o f Be h Γ§e t's a s the p res en c e o f RAS pl u s an y two o f: re c ur ren t g en i ta l u lc era tio n , e ye le si o n s, s ki n le si o n s or a po s iti ve pa the rg y te st ( Tab le 5. 9). It is th e au th o rs β €™ vi ew po i n t th at, a s th is is a p ro gre s si ve

co n di tio n , o fte n be gi n ni n g a s RA S w ith o u t an y o th er sy ste m i n vo lv em en t, i t is i mp o ss ib le a t

an y gi ven ti me to di ffe ren ti ate w ith a n y d eg re e o f ac c u rac y be twe en un c o mp li ca te d R AS a n d RAS th a t mi gh t e ve n tu a ll y p ro c ee d to or og en i tal u lcer ati on or B eh Γ §e t's di se a se .

Table 5.9 Criteria for the diagnosis of BehΓ§et's disease RAS pl u s an y two o f:

R ec u rren t g en i tal u lc era tio n

E y e l es io n s (a n teri o r u ve itis , po s teri o r u ve iti s, re tin a l v as c u li ti s ) S k in le si o n s (ery the ma n o d u su m , pa pu l o pu s tul ar le si o n s) P o s itiv e pa th erg y te st (rea d by ph ys i ci an 24 β€“48 h )

Management

Th e m a n ag em en t o f B eh Γ§e t's s yn dr om e u su a ll y re qu i res a m u ltid is c ip li na ry ap pr oa c h .

Ho w eve r, th e lo c al m an a ge me n t o f o ral a ph th a e i n B eh Γ§e t's s yn dr om e is e xa ctl y th a t fo r al l oth e r fo rm s o f RA S an d i s si mi la rly li mi ted in eff ec t. S ys te m ic th era py i s th e ref o re r eq ui red in mo s t c a se s an d dr ug s u se d in c lu de : s ys tem ic s te ro ids , a z ath i op rin e , cy cl o ph o s ph a mi de , co lc h ic i ne , c ic lo s po ri n, an d , mo re re ce n tly, a n ti-TNF Ξ± th e ra py an d m yc op h en o la te .

Th al id om i de ap pe a rs to be s u cc es s fu l in s o me c a se s w ith m u co c u tan e ou s i n vo lv em en t b u t its us e is re str ic ted be ca u s e o f its tera to ge n ic ity an d s id e- eff ec ts (se e ab o ve ).


Ho w w o ul d yo u ma n a ge th is g en tle ma n a n d w h a t th era p eu tic o p ti o n s are av ai la bl e?

Th e h i sto ry a nd c li n ic al ex am in a tio n o f the u l ce rs in th i s ca s e a re c on s is ten t wi th m ajo r RA S. It is im po rta n t s pe ci fic a ll y to e n qu ir e a b ou t ge ni tal u lc e rati o n an d o th er sy mp tom s , w hi c h mi gh t b e s u gg es tiv e o f B eh Γ §e ts di se a se . Th e sh o rt h i sto ry o f u l ce ra tio n is , h o w eve r,

su g ges tiv e of a re ce n t pr ec ip ita ti n g ca u se o r fa ctor, w h ic h s h o ul d be s o u gh t. It is i mp o rta nt to es tab li sh w h e the r th e pa tie n t s to p pe d sm o ki ng after hi s my o ca rd ia l i n fa rc ti o n a nd th en

dev el o pe d R AS , a s sm o ki n g c e ss a ti o n c an pre c ip itate RA S i n so m e in d ivi du a ls . S tres s ma y al so h a ve b ee n a co n tri bu to ry fa cto r. T h e p o ss i bil ity o f H IV in fe cti o n mu s t a ls o b e

co n s id ere d, b u t is u n lik el y i n th is c as e. Ora l u lc era tio n h a s be en r ep or te d in p a ti en ts o n

ni co ra n di l, a p o tas s iu m -ch a n ne l ac tiv ato r th a t i s us e d f o r u n sta bl e an g in a , an d it i s im po rta n t to ru le th is o u t a s a ca u se o f thi s pa tie n t's o ral u l ce rati o n . (Th is m u s t n o t be s to pp ed be fo re lia is i ng w ith th e c ar dio l o gi sts . ) B lo o d tes ts sh o u ld b e arr an g ed to c he c k f or ra is ed

in fl am ma to ry ma rke rs , h ae ma tin i c de fi ci en c ies , a n d an ti -en do m ys ia l au to a nti bo d ie s. A f u ll bio c h em ic a l a n d im mu n o lo g ic a l p ro fi le is a ls o a dv isab le .

In vie w o f th e se ve rity o f th e RA S a nd th is p a tien t's p o o r q ua l ity of li fe , so m e fo rm o f sys te mi c th e ra py n ee ds to b e c o n si de red a n d the o p ti o n s in c lu de s ys tem ic ste ro id s,

az a thi o pri n e, c ic lo s po ri n , th al id om i de, an d c ol c hi ci n e. In th e c as e o f a yo u n g pa tien t wi th a hi sto ry o f co ro n a ry a rte ry d is ea s e, l o ng -te rm pre dni so l o ne i s co n tra in d ic ate d a l th o u g h a

sh o rt, re du c in g co u rs e o f pre dn i so l o ne m ay g ive s h ort-te rm re lie f o f u lc er ati on . R AS d o es ,

ho w e ver, ev en tu al ly rec u r a fte r s to pp in g th e s ter oid. T o pic a l an a lg es ic s ma y be re qu ire d an d an a n tifu n g al s h o ul d al so be c on s id ere d, pa rtic u la rly if th ere i s a h i gh o ral c a rria ge ra te o f Can d id a al bi ca n s .

Co lc h ic in e h as b ee n re po rted a s s uc c es s fu l th e rap y fo r RA S a n d az a th io p rin e ma y be w o rth co n s id eri ng , ei th er al o ne o r as a s tero i d-sp ari n g ag en t. A n o th er tr ea tme n t o ptio n fo r th is

ma le pa tie n t is th a li do m id e, a lth o u gh th is is a s so cia ted w ith a n u m be r o f si gn i fic a n t si de eff ec ts an d th e pa tien t w ill n e ed co u n s el li ng a n d cl os e m on i to rin g. Q2

Ho w w o ul d yo u m an a ge th is d en ta l em erg en c y?

Th ere a re a nu m be r o f fa cto rs to be c o n si de red w h en a rra n gi n g e xtra c tio n o f th i s ma n 's

to o th , in c lu d in g h is me di ca l a nd d ru g h i sto ry (p revio u s my o ca rd ia l i n fa rcti o n, as pi rin ) an d an xi ety ab o u t de n tal tre atm en t. He i s pro b ab ly be st trea ted u n de r l oc a l an a es th es ia , w ith

se da tio n i f re qu i red . Th e as p irin ma y pre di sp o se to p o ste xtra cti o n h ae mo rr ha g e b u t th is i s

un l ike ly to b e s ig n ifi c an t a n d the re is n o i n dic a tio n to sto p th e me di ca tio n . It i s im po rta n t to ch e ck fo r h a em o s tas is a fte r th e ex trac tio n a n d, i f ne c es sa ry, su tu re an d /o r p ac k the s o ck et.

If th e pa tie nt de ve lo ps a n gi n a the n h e sh o u ld u s e hi s gl yc ery l tri n itra te s p ray s u bl in gu a ll y. If th e re are m o re se ve re c a rdi a c co m pl ic ati o ns , th en th e p ati en t m u st be trea ted ac c o rdi n gl y (se e Ch ap ter 19 o n β€ Me di ca l em erg en c ie s β€™).


Ho w w o ul d yo u ma n a ge th e p a ti en t i n yo u r pra c ti c e?

Al tho u g h th e p a ti en t a p pe ars f it a n d we ll , s he s h o ul d be qu e sti o n ed ab o u t a ny g ut, e ye , o r

ski n p ro bl em s an d a sk ed if s he h a s su f fere d fro m g en ita l u lc era tio n . H er d o cto r ca n a rra n ge

fo r b lo o d tes ts bu t i t is i mp o rtan t to di pl o ma tic ally po i n t o u t th at th e se s h o ul d in c lu de es tim ati on o f fe rri tin , fo la te, an d B 1 2 le vel s , as a f ul l bl oo d c o u nt an d fi lm a re in s u ffici en t. (It is im po rta n t to e sta bl is h a g oo d w o rki n g r ela tio n s hip w ith ge n era l me di ca l pra c titio n e rs in yo u r a rea . )

If th is p ati en t's cl in i ca l exa m in a tio n is c o n s is tent w ith a d ia gn o s is o f M iRA S a n d th e re i s n o

in cl in a tio n o f sy ste mi c di se as e , the n th ere a re a nu mb er of tre atm en t o p tio n s av ai la bl e tha t th e d en tis t c an pre sc ri be . An a lg es ic r in se s c an b e h elp fu l , pa rtic u la rl y b ef or e m ea ls , a n d an an tis ep tic ri n se w il l red u ce s ec o n da ry in fe c ti o n a nd a id p la qu e c on tro l , pa rtic u la rl y i f

to o th br us h in g is p a in fu l . Hyd ro c o rtis o ne p el lets c an be u s ed to pi ca ll y i n th e pro d ro ma l ph a se of u lc era tio n a n d ma y sp ee d up re s ol u tio n o f the u lc e rs.

Tria m ci n ol o n e p a ste is di ffi cu l t to a pp ly bu t m a y be us e fu l, i f a pp li ed la s t th in g a t n ig ht. A n y ob vi o us c a u se o f me ch a n ic al tra u ma d u e to bro ke n tee th o r d en ta l a p pli a nc e s sh o u l d b e

eli mi n ate d, i n c as e the s e a re pr ec ip ita tin g the a phth o u s u lc ers . M o re po te nt top ic a l s ter o ids , in th e fo rm o f r in s es o r i n ha l ers (s ee Tab le 5. 8 ) may be re qu i red . In mo s t c a se s, th e se

si mp le m ea su re s ca n re du c e the d is c om fo rt an d du ra tio n o f RA S . It do e s, ho w e ver , ne ed to be po i nte d o ut to th is pa tie n t th at the re is n o t, at th e p res e nt tim e, a n y sa tis fa c to ry

β€ cu re β €™ fo r th is c o n di tio n a n d th e ri sk s of s ys tem ic th era p y pro b ab ly o u tw eig h th e

ben e fi ts i n h er pa rtic u la r c a se .

Project

P. 60

1.

Fi nd o u t w hi c h sy ste mi c dru g s h av e b ee n re po rted a s c au s in g o ra l u l ce ra ti o n , in c lu d in g

RAS .

Au th o rs : Title:



Co py rig ht Β © 2003 Ox fo rd U ni ve rsi ty P re ss > T ab le o f Co nt e nt s > 6 - Dis e as e s o f t he lip s and to ng ue and d ist ur b an ce s of ta st e and halit os is

6 Diseases of the lips and tongue and disturbances of taste and halitosis Problem cases Case 6.1

A 20-yea r-ol d fem a le s tu d en t a tten d s yo u r su rg er y co mp la in i n g o f red p atc h es o n h e r to n gu e th a t m o ve fro m o n e are a to an o th er . Th es e h av e b ee n pr es en t f or a n u mb er o f ye a rs bu t

pre vio u s ly ga ve n o sy mp to ms , p ro vid ed s h e a vo i de d sp ic y f o od s . Rec e ntl y, s h e h a s n o tic ed

th a t h er to ng u e is p erm an e n tl y so re . Th is s tu de n t al s o rep o rts fe eli n g ti red a ll th e tim e an d lo o ks ra th er p a le . Q1

Ho w w o ul d yo u ma n a ge th is c as e ?

Case 6.2

A 7-yea r-ol d gi rl, s u ffe rin g fro m Do w n 's sy n dro m e is br o ug h t to yo u r su rg ery fo r ro u ti n e

den ta l ca re . Her mo th e r is co n c er ne d be ca u se th e girl ha s a p ers is ten t c ra c k i n th e c en tre o f he r l o we r li p. S h e h as a ls o n o tic ed th a t h er da u gh ter' s ton g u e l o o ks β € to o big f or h er

mo u th β€™. T he m o the r w a n ts to kn o w if th es e pro b le ms a re as s o ci ate d wi th h er Do w n' s syn d ro me a n d as ks w h eth e r th ey c an b e trea ted b y s urge ry. Q1

Wh at a d vic e w o ul d yo u gi ve to th is g irl 's mo th e r?

Q2

Wh at o th e r fa cto rs m u s t be c o ns i der ed w h en p ro vidin g d en tal tre atm en t f o r a p ati en t wi th Do w n' s sy nd ro m e?

Case 6.3

A 60-yea r-ol d reti red ga rd en e r a tte n ds y ou r pra c tice req u es tin g n ew d en tu res . O n

exa m in ati o n th ere is c ru s tin g o f h i s lo w er li p. P a lp ati on rev ea ls a rea s o f th i ck en in g a n d s o m e

in du ra tio n . O n c lo s er qu e sti o ni n g th i s ge ntl em a n rep o rts th at h i s lip h a s be en l ik e th i s fo r th e la st 4β €“5 ye ars a n d it g ets w o rs e i n th e co l d w e ath e r. He h as b ee n a pp lyi n g V as el in e Β ® to th e lo w e r l ip. Q1

Ho w w o ul d yo u m an a ge th is c a se ?

Q2

Wh at i s th e d if fer en tia l di ag n os i s o f th e lip co nd itio n ?

Introduction

So m e les i on s th a t a ffe ct th e l ips a n d to ng u e me rit s e pa ra te d es c rip ti o n . A lth o ug h m an y

co n di tio n s th at in vo lv e th e o ral m u co s a in g en e ra l m a y a ls o a ffe c t th e l ip s an d to n gu e, th e re are o th ers i n w hi c h th es e s tru c tur es a re sp ec if ic ally in vo l ve d. P a ti en ts w ith th e se c o mp la in ts ma y b e di ffi cu l t to di a gn o se a n d, i n pa rti cu la r, i t m ay be d iff ic u lt to di ffe ren ti ate be tw ee n

sym p tom s th at are e ss en ti al ly ph ys i ca l in n a tu re a nd th os e th at a re p sy ch o lo g ic al ly in d u ce d.

Diseases of the lips

Th e l ip s rep res e nt a tran s iti on a l ar ea be tw ee n th e o ra l mu c o u s me mb ra n e a n d the s ki n o f th e fac e a nd m a y, th ere fo re , be in v ol ve d i n pa th o lo gi c al pr oc e ss es a ff ec tin g bo th s tru ctu re s .

Swelling of the lips

Th ere is c o n si de ra bl e i n di vid u al an d ra c ia l v ar ia tio n in th e s iz e o f l ip s. A b n or ma l sw e lli n gs o f th e li ps c a n ei the r b e di ffu s e or lo c a liz e d to a partic u la r a rea o f th e lip . Tab le 6. 1 h ig hl ig h ts

so m e o f th e ca u se s o f b o th lo c al a n d di ffu s e li p s wel li ng (r ea de rs ar e e n co u ra ge d to co n s u lt a te xtb oo k o f or al pa th o lo gy fo r fu rth er in fo rm a tio n c o nc e rn in g lo c al iz ed l es io n s ). P ati en ts w ith or of ac ia l gra n u lo m ato s is (O F G) f req u en tly p res en t to o r al m edi c in e cl in ic s w ith li p s w el li n g th a t i s co s me tic a lly di s fig u rin g (Ch ap ter 12). A ng io e de ma , ei th er al le rgi c or n o n -al ler gi c, ma y p res e nt in iti al ly w ith l ip sw e ll in g an d b e m is ta ken fo r a l oc a l or al pr ob le m, su c h a s in fe cti on . A ng io e de ma i s di sc u ss e d f u rthe r i n Ch ap ter 14 .

Angular cheilitis (angular stomatitis, cheilosis, perlΓ¨che) An g ul a r c h eil iti s, th e i n fla m ma tio n o f o n e ( or bo th) o f th e co rn e rs o f th e mo u th , is a

rel ati vel y co m m on co n d itio n . Its re la tio n sh i p to can di da l in fe cti o ns h a s be en f u ll y di sc u s se d in Ch ap ter 4.

An g ul a r c h eil iti s is a mu l tifa c tor ia l c o n di tio n w ith a n u m be r o f lo c al a n d s ys tem ic pre di sp o si n g fa cto rs ( Fi g. 6. 1).

Table 6.1 Causes of lip swelling Diffus e

Loc alized

An g io ed em a (a lle rg ic /n o n -al ler gi c)

Mu c o co e le

Oe de ma (tra u ma o r in fe c ti o n )

Ab sc e ss

Oro fa c ia l gra n u lo m ato s is (O F G)

Hae m ato m a

Cro h n 's di se as e

Sa li va ry ad en o m a

Hae m an g io ma

Ba s al c el l c a rci n o ma

Lymp h an g io m a

Sq u am o u s ce ll ca rc in o m a

An g ul a r c h eil iti s is a m ul tifa c to ria l co n di tio n w i th a nu m be r o f lo c al an d s ys tem ic pre di sp o si n g f ac to rs .

In mo s t c as es , de ep fo l ds a t th e an g les o f th e mo u th be c o me tra um a tiz ed a s a res u lt of co n tin u a l w e tti n g by sa li va . Th es e a ng u la r f o lds a re th e res u lt e ith er o f an a to mi ca l

P. 64

co n fi gu ra tio n s o r, in ma n y c a se s, un s a tis fa cto ry den tur es , pa rtic u la rl y th o s e w i th a

dec re as e d v erti ca l di me n si on . S e co n d ary in f ec tio n by C. a lbi c an s (Fi g. 6. 2), S. a u reu s (o r bo th ), a nd o th er ba c teri a, i n cl u din g B -ha em o ly tic strep to co c ci , fo l lo w s. In so m e c a se s

an g ul ar c he il itis i s th e res u lt of a n u n de rly in g syste mi c co n d itio n o r de fic i en c y th a t res u lts i n th e h o st's d ef en c es , be in g c om p ro mi se d (Tab le 6. 2). A sy ste ma tic d ia gn o s tic a pp ro a ch m u s t be u se d in th es e pa tie n ts (Tab le 6. 3) a nd m a na g em ent i s di cta ted by th e res u lts o f

in ve sti ga tio n s (Tab le 6. 4). A nti mi cr ob ia l th era py fo r an g u la r c h ei liti s is s u mm a riz ed i n Tab le 6. 5. An ti fu n ga l th era py h as b ee n fu l ly di sc u s se d i n Ch ap ter 4 .

Fig. 6 .1 Fa c to rs in vo l ved in th e pa th o ge n es is o f an g u la r c heil iti s. ( Ad ap ted w ith

per mi ss io n f ro m Fi g. 26. 13 i n B a gg , J. , et a l . (1999). Es s en tia ls o f m ic ro bi ol o gy fo r den ta l stu d en ts . Ox fo rd Un i ver si ty P re ss , O xfo rd . )

A few o th e r c o n diti o ns m a y r es u lt i n a mo re s pe ci fic fo rm o f a n gu l ar ch e il itis . F o r i ns ta n ce ,

fac i al ec z em a m ay pre s en t a t th e an g le s o f th e mo u th an d m im ic a s im pl e an g ul ar c he il iti s. In th i s si tu ati on the d ia gn o s is l arg el y de pe n ds o n th e h is tor y o f th e

pa tien t. It i s u n lik el y th a t a pa tie n t w o ul d in iti al ly pre s en t w ith e c ze m a a ff ec tin g th e a n gl es

P. 65

of th e mo u th o n ly. T h e ra p id res p on s e to lo c a l s tero i d ap pl ic ati o ns ( su c h a s 1 pe r c en t

hy dro c o rtis o n e c re am ) h e lp s to co n f irm th e dia g no s is . In C ro h n' s dis e as e an g u la r ch e il itis i s

a s i gn if ic an t d ia g no s tic i n dic a to r w h en a s so c ia ted w ith oth e r ma rk ers o f the d is ea s e ( Ch ap ter 12). Th is i s a pa ra lle l to th e a n a l f is su re s se en in th is c o n di tio n a n d, s im il ar ly, re s po n ds rea di ly to lo c al s tero i d th e ra py.

Fig. 6 .2 A ng u la r c h ei li ti s w ith Can d id a pre s en t.

Table 6.2 Angular cheilitis as a marker of systemic disease An a em ia Ir on , B 1 2 , o r fo l ate d efi ci en c y

OF G ( in cl u di n g C ro h n' s di se as e) HIV in fe c ti o n

Dia be te s m ell itu s

Sj Γ¶g re n' s sy nd ro m e ( an d o th er ca u s es o f sa li va ry gla n d h ypo f u nc tio n )

Table 6.3 The investigation of patients presenting with angular cheilitis His to ry: key qu e sti o ns Gen e ral iz ed i ll h ea lth ?

Kno w n s ys tem ic d is ea s e? Xe ro ge n ic me di ca tio n ?

An tib io ti c or s te ro id m ed ic a ti o n ?

So c ia l hi s to r y, in c lu d in g tob a cc o u s e? Il l -fittin g d en tu res ?

Den tu res w o rn a t n ig ht? Th era pe u tic ra di ati o n? Cyto to xic dru g s?

Ex am in a tio n : c h ec k fo r Si gn s o f an a em ia

Lymp h ad en o p ath y

Sa li va ry gla n d sw e ll in g In tra o ra l ca n di do s is

Den tu re- in du c ed e ryth em a to u s c an d id o si s

Co mm is s u ral le u ko pl ak ia (c h ro n ic h yp erp la s ti c c an d ido s is ) Ora l dry n es s

Ma n ife s tati on s o f OF G

Il l -fittin g d en tu res /re du c ed ve rtic al d im en s io n Sp ec ia l in ve s ti ga tio n s Mi cro b io l og y

S a mp li n gβ €”te c hn i qu e (s me ar , sw a b, a n d o ral ri nse , if av ai la bl e).

S a mp li n gβ €”si te (a n gl es , p al ate , fi tti n g -su rfa c e of d en tu re, a n teri o r n a res )

Bl o o d tes ts

In d ic ate d if su s pi ci o n o f u n d erl yin g sy ste mi c facto r o r l oc a l th e rap eu ti c me as u res

fai l to res o lv e an g u la r c h ei liti s

F u ll b lo o d co u n t S e ru m B 1 2 , fer ritin , s er um a n d red c el l fo la te ( o r w h o le bl ood f ol a te ) B l o od gl u co s e

Oth er tes ts

A s i nd ic a ted by c li ni ca l fi nd in g s

Table 6.4 The management of patients with angular cheilitis El im in a tio n o f pre di sp o si n g l o ca l fa cto rs C h ec k d en tu re s an d de n tu re h ygi en e

In s tru ct pa tie nt to le av e o u t d en tu re s at n igh t

Refe rra l fo r tre atm en t of u n de rly in g de fic ie n ci es or sy ste mi c di se a se

Pro v is io n o f ap pro p ria te an ti mi cro b ia l the ra py (to pi ca l o r s ys tem ic )β €”as i nd ic a ted by mi cro b io l og y (se e Tab le 6. 5)

Table 6.5 Antimicrobial therapy for angular cheilitis*

Can d id a is ol a te d

In tra o ra l β € : ny sta tin p a sti lle s o r am p ho te ric in lo z en ge s (e limin a te o ral re se rvo i r

of in fe c ti o n )

A n g les : ny sta tin oi n tme n t

S. au re u s is ol a te d

A n g les : fu si di c ac i d cr ea m

A n ter io r n a res : mu p iro c in c re am o r fu s id ic a ci d cre am .

Mi xed i n fec tio n s

Mi co n a z ol e ge 1 o r cr ea m β € ΅ * S ys te m ic fl u co n a zo l e ma y b e req u ire d to trea t a n gu la r ch e il itis r es is tan t to to p ic a l th e ra py o r fo r im mu n o c om p ro mi se d pa tie n ts.

β€ Ch lo rh ex id in e so l u tio n h a s an tib ac te ria l a n d anti ca n di da l ac tiv ity an d c an be us e d a s a rin s e. Ch lo rh e xid in e ma y pa rtia ll y i n h ib it th e ac tio n of n ys tati n .

β€΅ Mi co n a z ol e ca n a ls o b e u s ed i f i n fec tin g mi c ro -or ga n is ms a re u n kn o wn β€”it h a s an ti -sta ph yl o co c c al a cti vity . M ic o na z o le is n o t th e dr ug o f ch o i ce fo r Can d id a

in fe cti on s be ca u s e o f va ria b le se n si tiv ity an d po s sibl e de vel o pm en t o f a zo l e res is tan c e.

Lip fissures

Ve rtic a l c ra ck s in th e li ps a re le ss co m m on l y s e en th a n an g u la r c h ei liti s an d th e mo s t

co m mo n s ite i s in th e mi dl in e o f th e lo w er li p. S u ch fi ss u res a re o fte n rem a rka bl y re si s ta n t to co n s erv ati ve trea tme n t a n d s u rg ic al ex ci s io n h a s been o c ca s io n a lly a dvo c a ted fo r th e m o re res is tan t les io n s . Th e ma jo ri ty o f the s e f is su re s are i n fe cte d b y Sta ph yl o co c c u s au re u s an d

th e m o st s a tis fa cto ry fo rm o f trea tme n t c o ns i sts o f the e li mi na tio n o f th e se co n d ary in f ec tio n (by th e u s e o f su i tab le a n ti bi o tic c rea m s) fo ll o we d by th e u s e o f s tero i d cre am s . With su c h a

reg im e m o s t o f th e se c rac ks c a n be p ers u a ded to h e al , a t le as t te mp o rari ly . A sh o rt co u rs e of a s ys te mi c an tib io tic m a y b e req u ire d i n s o me pe rs is ten t ca s es β €”th e d ru g o f c ho i ce i s in di ca ted b y c u ltu re a nd s en s iti vity o f th e pu ta tive b ac teri a . Sh o u ld th e pre do m in a n t

or ga n is m in vo lv ed n o t b e a Sta ph yl o co c c u s , bu t Can d id a , th e n th e tre a tm e nt sh o u ld b e

mo di fi ed s ui tab ly . Th e ba si c pr in ci pl e, h o w ev er, o f th e el im in a tio n o f se co n d ary in f ec tio n

fo ll ow e d by the u s e of to pi ca l s te ro id i s th e o n e mo s t li kel y to be su c c es s fu l. Li p c ra c ks o f th i s n atu re s ho w a m a rke d ten d en c y to

reo c cu r an d a pe rm an e nt cu re i s un l ike ly . Lip fi ss ure s are c o mm o n ly se en in p ati en ts w ith

P. 66

Dow n 's s yn d ro me , to ge th e r w i th an g ul a r ch e il itis β €”po s si bl y a s a res u lt of m o uth bre ath in g . Lip fis s u rin g is a ls o a fe atu re o f O F G ( Ch ap ter 12).

Ch ro n ic li p fis s u res a re o ften r es is tan t to co n s ervati ve trea tme n t a nd ma y req u ire s u rgi ca l ma n ag em en t.

Allergic cheilitis

Ir rita tio n a n d s c al in g o f th e li ps m a y re su l t fro m a co n ta ct al le rgy to a w id e va rie ty o f

su b sta n ce s, in p arti cu l ar, to the co n s titu en ts o f li ps tic ks . Ho w ev er, o th er to pi ca l pre pa ra tio n s us e d o n th e li ps , su c h a s li p o i n tme nts o r mo i stu rize rs , to o th p as tes , a n d, o c c as io n a ll y, fo o d s, ma y c a u se a n a lle rgi c c he il itis . In g en e ral , th e i rri ta tio n is c on f in ed to th e ve rmi li o n o f th e lip , bu t ma y e xte nd b ey on d i t in a n e cz e ma -lik e i rri ta tio n of th e p eri o ra l s ki n . Th is fo r m o f co n tac t s e ns iti vi ty i s o f ten d iff ic u lt to di ag n o se a n d r eq ui res d eta il ed h is to ry ta ki n g. F o r

in sta n c e, a li ps tic k al le rgy ma y be c au s ed b y th e mi n im al a mo u n ts tra ns fe rre d i n ki ss i ng a n d so m a y re ma i n qu i te u n s u sp ec ted . In s om e in s tan c es th e irr ita ti o n m ay fo ll o w th e a c tio n o f su n li gh t o n th e li ps . Th i s ma y n o t b e a pri ma ry ac tin i c rea c ti o n , bu t m a y b e se c o nd a ry to a ph o to se n si tiz in g ef fec t o f s ub s ta n c es , s uc h a s th o se in li ps tic ks , w h ic h mi gh t i n o th er

su b jec ts ca u se d ire ct co n ta ct ch e ili tis . Th is i s rathe r d if fere n t fro m th e ac tin i c ch e il itis th a t

ma y o c c ur in so m e p a tien ts w h o w o rk i n fu ll s u n lig ht fo r ma n y y ea rs (s ee b elo w ). In th e c as e of a s im pl e co n ta ct ch e il itis , tre atm en t c o n si sts of tra ci n g th e s en s itiz i ng s u bs tan c e an d

eli mi n ati n g i t. P atc h -te s ti n g ma y b e n ec es s ary to co nf irm th e pu ta tive a ll erg en . Te mp o rary

rel ief m ay be g ive n b y u s e of to pi ca l s te ro id s , bu t th es e s ho u l d n o t be c o n ti n u ed o n a lo n g te rm ba s is .

Actinic cheilitis (solar keratosis)

In a nu m be r o f pre do m in a n tly ma le p ati en ts , lo n g expo s u re to su n li gh t, o fte n th e r es u lt o f ou td o or w o rk i n a s un n y cl im a te, is f ol lo w ed b y th e o n se t o f a ch e ili tis i n w hi ch ep ith el ia l

atyp ia m a y o c cu r w ith p ro gre ss i on to ca rc in o m a in so me c as e s. T hi s is a lm o st al w ay s mo s t ma rke d in th e lo w e r li p β€” th e s o -ca ll ed β € lip a t ri sk β €™. C ru sti n g a n d in du ra tio n o f th e

verm il io n m a rgi n o cc u rs , th e i n du ra tio n b ei ng d u e, i n pa rt, to a fi br oti c rea c tio n o f the co n n ec tiv e ti ss u es ( Fi g. 6. 3). B io ps y is n ec es s ary fo r a fu ll a ss es s me n tβ €”th e c li ni c al ap pe ara n c e a lo n e is a n in s u ffi ci en t g u id e to th e p res en c e of w o rryi n g ch a n ge s in th e

epi th el iu m. If , o n bi o ps y, s ig n ifi ca n t d ys pl as ia i s fo u n d, th en exc i si o n o f th e ve rmi li on are a by a li p s h a ve o pe rati o n o r l as er trea tme n t s h ou l d be co n s id ere d. C h em ic al e xfo l ia n ts ca n al so b e u se d.

Pro l o ng ed e xp os u re to s un l ig h t, eith e r o c cu p ati o na l o r re cr ea tio n al , c an res u lt in th e dev el o pm en t o f an ac tin i c c h ei li tis .

Exfoliative cheilitis

Ex fo li ati ve ch e il itis i s an u n co m mo n c o n di tio n a ffec ti n g on l y th e ve rmi li o n bo rd ers o f th e l ip s

an d ch a ra cte riz e d by the p ro du c tio n o f exc e ss iv e a mo un ts o f ke rati n . Th is fo rm s bro w n s c al es th a t m ay b e s po n ta n eo u s ly sh e d o r ma y be re mo ve d by th e p ati en t ( Fi g. 6. 4). It h as b ee n

su g ges te d th a t th is c o n di ti o n is ex cl u si vel y se en in fe ma le p ati en ts , bu t s ev era l ca s es h a ve

rec en tly b ee n rep o rted i n ma le s . Th e hi sto l o gy o f th e le si o n is o f a si mp le h yp erp a rak era to si s an d it i s n o t c on s id ere d to be a p rem al ig n an t l es ion . Th e re is n o a pp ar en t sy ste mi c

ba ck gro u n d to th is c o nd iti o n bu t s o me p ati en ts re port a n a ss o c ia tio n w ith s tres s , th e d eg ree of ke ra tin fo rm a tio n a pp ar en tly in c rea s in g at tim es o f in c rea s ed a nx ie ty. A ll s ys tem ic

in ve sti ga tio n s in th e se p ati en ts pro v e n e ga tiv e an d a ll fo rm s o f tre a tm e nt ha ve a s ye t pr ov ed un h e lpf u l. Lo c al a n d s ys tem i c ste ro id s, ca u tery , cry o su rg ery , a nd m an y o th er fo rms o f

tr ea tme n t h av e b ee n atte mp ted w ith o u t s u cc es s . In th o se w ith a n a pp ar en t a n xie ty-rel ate d

co n di tio n , mi ld tra n qu il li z atio n ha s b een rep o rted a s he lp fu l, al tho u g h th e m ec h a ni s m fo r th is is fa r f ro m cl ea r. A n tide pr es sa n t m ed ic ati o n h as a ls o b ee n pr es cri be d fo r a fe w o f th es e

ca se s , w ith s o me de gr ee o f s u c ce ss . Th e a u th o rs β€™ exp eri en c e wi th th is c o nd iti o n is th a t

aff ec ted i nd iv id ua ls o f ten a pp ea r to ha ve a p ers o n al ity di so rd er . Th e e xte n t to w h ic h th ere is a f ic titi ou s e le me n t in vo l ve d i s di ffi cu l t to q ua n tif y. M o st pa tien ts te nd to p ee l of f th e

exf ol ia tin g s kin , b u t th is c o ul d be a n o rma l res p ons e u nd er th e c i rcu m sta n c es . E ven tu a ll y,

th i s co n d itio n ap pe ars to res o lve s po n ta n eo u sl y bu t, w h ile p res en t, i t is s u ffi c ien t to c au s e th e p ati en t c o n si de rab le d is tres s.

Fig. 6 .3 S ol a r ke ra to s is o f th e l o w er l ip .

Fig. 6 .4 E xfo l ia tive ch e il itis .

P. 67

Perioral dermatitis

Th is re la tive ly u n co m mo n co n d itio n is o fte n a co n s id era bl e di ag n os tic p ro bl em a n d the

pa tien ts a re fro m tim e to tim e s e en i n th e o ra l me di ci n e c l in ic f or in ve s tiga tio n . T he p ati en ts are a lm o st al wa ys y o un g a du l t fe ma le s an d c o mp la in o f a n ery the ma to u s ra sh o n th e f ac ia l

ski n a ro u nd th e mo u th (Fi g. 6. 5 ). In s om e ca s es , b ut no t al l, th er e a re pa p ul es p res e nt. In

so m e p a tien ts th er e i s a h is to ry o f th e u s e o f s tero i d cre am s o n th e fa ce , bu t th i s is n o t b y an y me an s in va ri ab le . P eri or al de rm ati tis c an a l so b e du e to a c on ta c t al le rgy (s ee al ler gi c ch e ili tis ).

Fig. 6 .5 P eri or al de rm ati ti s in a y o un g fe m al e pa tie n t.

Th e d ia gn o s is is e ss en ti al ly cl in i ca l β€” th e re are no s ys tem ic c h a ng es d ete cta bl e. M a n y o f

th o s e pa tie nts w i th pa pu l es p res en t re s po n d to a lon g- te rm lo w -do s e re gi me o f tetra c yc lin e s, mu c h as i n a cn e . P ara do x ic al ly , so m e pa tie nts re s pon d we ll to lo w - po ten c y s ter o id cr ea ms ,

su c h a s 1 pe r c en t h y dro c o rtis o n e. A s in a l l c a se s w he re th e f ac ia l s kin i s in vo l ve d, c au ti o n

mu s t be e xer ci se d in th e u se o f mo re po te nt ste ro id pr ep ara tio n s b ec au s e o f th e po s si bi li ty o f atro p hi c c ha n ge s . In vi ew o f the w id e ra ng e o f p resen ta tio n a n d rea c ti o n to th era p y, it is

po s si bl e th at p er io ra l d erm a titis r epr es en ts a re actio n p atte rn ra th er th a n a s in g le en tity , an d th at m o re th an o n e b as ic a eti o lo gi ca l pr oc e ss may b e in vo l ve d.

β€ Lick eczemaβ€™

Th is is a f u rthe r p eri o ra l c o n di tio n th at o ften ca us es d ia gn o s tic c on f u si on . T h e p ati en ts a re

yo u ng β€” of ten c h il dre n β€”an d co m pl a in o f a sh a rpl y del in e ate d z o n e o f i rrita b le, sc a ly sk in

aro u n d the m o u th. Th is c o n di tio n , as i ts n am e im pl ie s, i s si mp ly th e r es u lt o f a li ck in g h ab it th a t th e pa tie n t ma y stro n g ly de ny , ev en w h en ac tu al ly pe rfo rm in g th e lic ki n g ac tio n du rin g

th e e xa mi na tio n ! T he tre atm en t i s to s top th e h ab it. If th is is do n e , the l es io n ra p idl y f ad es . Su c h a w el l-in gra in e d ha b it (o f w h ic h th e pa tie n t ma y be en ti rel y u n c o ns c io u s ) m ay ,

ho w e ver, be di ffi cu l t to e rad ic a te . A rem o va bl e ap pl ia n ce w ith a ro u g h o r s h ar p e dg e to in terf ere w ith th e to n gu e ac tio n is o fte n su c c es sf ul.

Cheilocandidosis

Th ere h a ve be en s ev era l rep o rted ca s es i n w h ic h th e l ip s be co m e the s ite fo r a he a vy

ca n di da l i n fe cti on . T he s e le si o n s are re po rte d a s oc cu rri n g bil ate ra ll y, pr ed om in a n tly o n th e lo w er lip , an d a pp ea r a s u lc er ate d g ra n ul ar a rea s fro m w h ic h C. al bi ca n s ca n b e fre ely

cu ltu re d. T h is is re po rte d to o cc u r i n ge n era ll y h ea lth y pa tie nts , b ut the re ar e s tro n g

su g ges tio n s o f pri o r l oc a l ab n or ma li ty th at m ig h t le a d to a se co n d ary c an d id al in f ec tio n , fo r exa m ple , s o la r i rrita tio n in s o m e A u s tral ia n p ati ents. In so m e ca s es th e ch e ili tis h a s be en

as so c ia te d w ith c h ro n ic in tra o ra l c a n did o s is . Th es e le si o ns h a ve b een co n s id ere d by se ve ral au th o rs to re pre se n t c an d ida l in fe c ti o n a ffe cti n g in trin s ic al ly u n sta bl e ep ith el iu m a n d i t is

su g ges te d th a t, as i n c h ro ni c h yp erp la sti c ca n di do sis (c an d id al le u ko pl ak ia ), e arl y tr ea tme nt by an tif u n ga ls m ig ht le ad to re so l u ti o n , w he re as d el ay mi gh t l ea d to in c re as in g ep ith e lia l dys pl a si a.

Diseases of the tongue

Th e to n g ue m ay b e i n vo lv ed in a w i de ra n ge o f o ra l mu c o s al di se a se s. Ap ar t fro m th is

in vo lv em en t i n ge n era li z ed o ra l c o n di tio n s , se ve ra l l es io n s a re qu ite s pe ci fic to th e to n gu e .

Ma n y o f th es e de pe nd o n al tera tio n s i n the s pe c ia liz e d ep ith el ia l c o ve rin g o f th e to n gu e a nd ,

in pa rtic u la r, i n th e f il ifo rm p ap il la e. T he s e s truc tur es a pp ea r to b e pa rtic u la rly su s c ep tibl e to ch a n ge s b ro u gh t a b ou t by sy ste mi c ab n o rma li tie s. F or i n sta n c e, in an a em ia s (i n w h ic h th e

or al m uc o s a as a w h o le m ay u n de rgo s o m e c h a ng e) , the pre do m in a n t o ra l a bn o rm a lity m ay be o f th e to n gu e. W h en a l es io n o f an y ki nd i s pre sen t o n th e to ng u e, i t m ay be b ro u gh t

rap id ly to th e n o ti ce o f th e p ati en t be ca u s e o f th e m o bi li ty o f th e o rg a n an d its ri c h su p pl y o f se n so ry n erv e en d in gs . Th i s, h o w ev er, is by n o m ea ns in va ri ab ly the c a se a n d th e re are

ma n y ex am pl es o f le si o ns ( fo r e xa mp le , th e w i de sp rea d u lc era tio n o f m ajo r ero s ive li ch e n

pla n u s) in wh i ch th e p ati en t c o mp la in s o f fa r le s s pa in th a n m igh t be ex pe cte d. O f g re ate r

si gn if ic an c e is th e fa ct th a t n e op la s ms o f th e to n gu e m ay gro w to a co n s id era bl e si ze b ef or e se co n d ary in f ec tio n l ea ds to s ym pto m s o f p ai n (a n ea rly c ar ci n om a o f th e to n gu e ma y be qu ite pa i nl es s ).

Developmental abnormalities and morphological variations Si gn if ic a nt de vel o pm en ta l a b no rm a li ti es o f th e to n gue a re ra re. A nk yl og lo s s ia (to n gu e tie ) i s us u a lly c o ng en i tal a n d m a y b e a s so c ia ted w i th mi c roglo s s ia . Co n tra ry to p o pu l ar o pi ni o n ,

th e re is n o e vid en c e tha t an k ylo g lo s si a in ter fer es w ith s pe ec h , a lth o u gh p ati en ts m ay fi n d th a t th e res tri cte d m o bi li ty i nte rfe res w i th the m ec h a ni ca l c lea n s in g ac tio n o f th e to ng u e. Ma c ro glo s s ia c an o cc u r i n a n um b er o f s ys tem ic c o nditi o ns , s u ch as co n g en ita l hy po th yro i di sm , Do w n' s sy n dro m e, a c ro me ga ly , an d amyl o id o si s.

Tongue fissures

Th e n o rm a l g ro ss s tru c tur e o f th e to n g ue i n cl ud es fi ss u res , w h ic h m ay s ho w var ia tio n i n

nu m be r, d epth , a n d arr an g em en t. Th e re i s co n s id era ble do u b t as to th e e xten t to w h i ch th e pa tte rn o f fi ss u res m a y c h an g e du ri n g d is ea s e pro c es s es , al th o ug h it do e s

se em th at in a f ew c o n diti o n s, fo r e xa m ple , c h ro ni c mu c o c uta n eo u s c an d id os i s, th e fi ss u res ap pe ar so ex ag ge ra te d an d o f su c h a bn o rm al fo rm th at th ey mu s t b e c o n si de red a s pa rt o f

P. 68

th e p ath o lo g ic al c h an g e ( Fi g. 6. 6). In ge n era l, h o wev er, th ere is l ittle e vid en c e o f c h an g es i n fis s ur e p a tte rn d ue to l es s ex ag ge ra te d pa th o lo gi es. Th e re is n o d ou b t, h o we ve r, th at

fis s ur es o f no rm a l f o rm an d di stri bu ti on ma y pla y a pa rt i n th e m o di fic a tio n , o r e ve n th e

in iti ati on , o f pa th o lo gi c al pr oc e ss es , s in c e co n di tio n s o f a n a ero b ic s tas is a re pr es en t i n th e dep th s o f th es e s truc tu re s an d pre s en t o p po rtu n ities fo r se le cti ve ba c te ria l gro w th . Th i s

bei n g so , i t is c o mm o n to f in d su p erf ic ia l i n fe cti on s a n d si mi la r co n d itio n s c o n ce n trate d at an d al o n gs id e th e fi ss u re s. S i mi la rly , in a s itu a tio n th at m a y l ea d to a so re a n d irri tate d to n g u e, th e fis s u res m ay b e th e fi rst an d th e m o s t se ve rel y a ff ec ted pa rt. Th e u s e o f a

ch lo rh e xi din e m o uth w a sh ma y b e h el pfu l fo r pa tie nts w i th a sy mp to ma tic fi ss u re d to n g ue .

Fig. 6 .6 Dee p fis s u res o f th e to n g ue i n a pa tie n t w ith c h ron ic m uc o c uta n eo u s ca n di do s is .

Th e so -ca ll ed β€ sc ro tal to n gu e β €™ is a n o rma l mo rp h o lo g ic al va ri an t w ith mu lti pl e fi ss u res

on th e d o rsu m o f the to n gu e . Th e ap pe ar an c e o f a n irr eg ul ar bo rd er o f th e to n g ue (o f te n s ai d to res em bl e a β€ pie -cru s t e dg eβ €™) is kn o w n a s a β€ cre n ate d to n g ue β€™ an d c an b e o ne

of th e fea tu res o f br ux is m in s o me pa tie n ts.

Coated tongue

Th e to n g ue s o f a ll n o rm al in d iv id ua ls h a ve a c o ati ng co n s is tin g o f a l a yer o f m u cu s ,

des q ua m ate d ep ith el ia l c e lls , o rg an i sm s , an d de bri s. In the h e al thy in d iv idu a l th e to n gu e i s mo bi le , th ere is a r ap id fl o w o f sa l iva , a n d th i s co a tin g is ke pt to a mi n im u m. Wi th th e

sl ig hte st di stu rb an c e to th e h e al th o f th e in d ivi dua l, h o w ev er, th e b al an c e is u ps et an d th e

co a tin g ma y qu i ck ly be co m e very m u ch th i ck er. A l ack o f m o bi li ty o f the to n gu e , w hi c h ma y be ca u s ed by th e mo s t m in o r p ai n fu l les i on s , a d is tur ba n ce in sa li va fl o w , an ex ce ss o f

to b ac c o o r o f a lc oh o l , a ga s tr ic o r re s pi rato ry u pset, o r a feb ri le co n d itio n , m a y re su l t in a

bu il d-up o f the to n gu e c o ati ng s u ffi c ien t to pro d uce a w hi te or c ol o u red p la qu e. Th e c o lo u r o f su c h a c oa tin g d ep en ds o n a v ari ety o f fa cto rs , s u ch as to ba c co u sa ge a n d d ie tar y h a bi ts ,

an d is o f ve ry l ittle di ag n o sti c si gn if ic a nc e . Su c h a c o ati ng m a y, h o w eve r, b e u n p lea s an t to

th e pa tie n t an d i ts r em o va l m a y b e q u ite di ffi c ul t s in c e v ery o fte n it i s fi rml y ad h ere n t to th e to n g u e. P ati en ts fre qu e n tly be co m e ov erl y a w a re a n d se lf -co n s ci o us a b ou t th e ir co a ted

to n g u e a n d ne ed re as s u ran c e. In a few pa tien ts th eir c oa te d to n gu e b ec o me s an ob s es si o n . Vi go ro u s br us h in g o f th e to n gu e is o ften ad vo c ate d a n d to ng u e sc ra pe rs ar e n o w

co m me rc ia lly a va il ab le . Us e o f th es e is s o me tim es fou n d to b e m o s t u np le a sa n t by th e

pa tien t. E ff erv es c en t mo u th w as h es , s u ch as th o s e c on tai n in g as c o rbi c ac id , m ay be h el pf ul , pa rtic u la rly if c o mb in ed w ith bru s h in g o f th e ton g ue.

Hairy tongue

In ha i ry to n gu e th e le si o n do e s no t co n s is t s im pl y o f a c o atin g o n th e su rf ac e o f th e to n gu e bu t re pre se n ts an el o ng a tio n o f th e fi li fo rm pa p illae , o fte n to m an y tim es th ei r o ri gi na l

len g th . With th i s el on g ati o n th e p a pi lla e o ften ta ke o n a d ar k c o lo u r, b la ck o r b ro w n be in g

co m mo n ( Fi g. 6. 7). Th e me ch a n is m fo r th e fo rm a ti o n o f th e se c o lo u red h a iry to n gu es i s qu i te un k no w n , th ere a pp a ren tly be in g m an y in i ti a ti n g facto rs . F or in s tan c e, h a ir y to n gu e

freq u en tly fo l lo w s a co u rs e o f an ti bi oti c th era py and m a y re so l ve qu i te ra p id ly on co m pl eti on of tre atm en t. Oth e r h a iry to n gu es a pp a ren tly a pp ea r c o mp le tel y s po n ta n eo u sl y an d n o c au s e is ev er fo u nd f or th em . E qu a ll y d o ub tfu l is th e so urc e o f th e pi gm en t i n vo lv ed . It is u s u al to rel ate th is to p ig me n t- pro d uc in g o rg an i sm s en tra pp ed w ith i n th e pa pi ll ae b u t, in fa c t, n o

su c h o rga n is m s ha ve e ver be en de mo n s tr ate d. In th e pa s t, the p res en c e o f h a iry to n gu e w as of ten a s cri be d to ca n di da l in fe cti o n bu t, ag a in , it ha s n ev er be en s h o w n th at th e re is a ny tr ue a s so c ia tio n b etw ee n ca n di do s is a n d the p ro du c tio n o f the e lo n ga ted p ap il la e.

Trea tm en t o f ha i ry to n gu e i s rem ark ab ly d iff ic u lt. Th o s e c a se s as s o ci ate d wi th a nti bi o tic

th e ra py fre qu en tl y, b ut no t in va ria b ly, re s o lve w h en th e m ed ic a tio n i s f in i sh e d. Th e u s e o f

eff erv es ce n t a nd m u cu s -so lv en t mo u th w as h es m a y b e he lp fu l in re du c in g s ec o n da ry irri tati on an d th ere by pro d u ci n g s u ita bl e co n d itio n s fo r the re s ol u tio n o f the a bn o rm al ity bu t, a ga in , th e re su l ts are va ri ab le . Th e au th o rs h av e us e d c h em ic a l c a u teri za tio n , w ith tric h lo ra c etic ac id , to trea t h a iry to n gu e , bu t o n ly s ma ll a rea s can b e ma n a ged at a tim e, b ec au s e o f dis c o mf or t af terw a rds . Lo n g- te rm

P. 69

res u lts o f th i s ch e mi ca l co u n ter iz a ti o n w ere d is ap po i n ti n g. β € Su c ki ng a d ry p ea c h s to n e β€™

ha s be en ad vo c ate d fo r th e m an a ge me n t o f h a iry to n gu e bu t th i s ap pro a c h do e s se em to b e po ten tia l ly ha z a rdo u s ! S o me pa tie n ts wi th th is c o n diti o n u se to n gu e s cra p ers o r bru s h th ei r to n g u e v ig or ou s ly b ut the s e m ea s u res a re ra rel y e ffe cti ve .

Fig. 6 .7 B la ck h a iry to ng u e.

Atrophy of the lingual epithelium

At th e o pp o si te ex trem e fro m th e p ro li fer ati on o f th e fil if o rm pa pi lla e s ee n in h a iry to n gu e are th e a tro ph i c ch a n ge s in th e pa p ill ae f ou n d in a nu m be r o f ge n era li z ed di se a se s bu t, i n pa rtic u la r, in ha e ma to lo gi ca l a nd n u triti o na l a bn o rma l itie s. Atro p h ic ch a n ge i n th e o ra l epi th el iu m as a w h o le m ay b e th e c on s eq u en c e of a wid e ran g e o f a bn o rm a liti es (f o r

in sta n c e, i ro n de fic i en c y, m ega l ob la s tic a n ae mi as , a n d n u tr itio n a l d ef ic ie n ci es o f va rio u s

kin d s). Th es e atro p hi c c ha n ge s a re so m eti me s , bu t no t i nv ar ia bl y, fo u n d pre do m in a n tl y in

th e fi li fo rm pa p ill ae , a n d th e s u rfa c e o f th e to n g ue m a y b ec o me re d, s h in y, an d pa in f ul . It i s no w a c ce pte d th at a w i de s pe ctru m o f ge ne ra li ze d abn o rma li tie s ma y b e res p on s ib le fo r the pro d uc tio n o f s im il ar ep ith el ia l c h a n ges . C on v ers ely, a w id e ran g e o f o ra l si gn s an d

sym p tom s m ay o cc u r i n d iff ere nt pa tie n ts w i th si mi la r c o n di tio n s. It is ce rta in ly tru e,

ho w e ver, th a t a ll p atie n ts w ith p ai nf u l, d ep ap il la ted , o r red de n ed to n gu e s s h o u ld u n de rgo blo o d tes ts in c lu d in g es tim ati o n s of s eru m , fe rritin, B 1 2 , fo la te, a n d gl uc o s e to eli mi n ate th e

po s si bi lity o f a n u n de rlyi n g h a em a tin ic d ef ic ie nc y o r u n di ag n o se d dia b etes . A so r e to n gu e m ay be f ou n d in as s oc i ati on wi th m an y ge n era li ze d co n d itio n s , pa rtic u la rl y th e an a em ia s an d s al iv ary g la nd h yp o fu n c ti o n . Fo r in s tan c e, i n rh eu m ato i d a rth ri ti s , wh e re the re ma y b e an an a em ia o f ch ro n ic d is e as e, i t is n o t u n us u a l to f in d a so re to n gu e . S im ila rl y, i n SjΓ ¶g re n' s sy nd ro m e, i n w hi c h th e s a li va ry f lo w is r ed uc e d, th e ton g u e m a y b ec o m e d ry,

red de ne d, o r pa in f ul (Ch ap ter 8). Th ere a re, ho w e ver , so m e pa tie nts i n w h om n o ca u s e c a n

be fo u n d fo r th e atro p h ic c h an g es . In o ld er pa tie nts th e a tr o ph y m a y be re ga rde d as a n a ge

ch a n ge , bu t i t is n o t c le ar to w h at exte n t th is m ay rep re se n t su b cl in i ca l sy ste mi c ch a n ge s. In so m e c o n di tio n s in w h i ch de pa pi ll ati o n o f th e to ng ue ha s ta ken pl ac e, th e o rig in a l

mo rp ho l o gy o f th e li n gu a l e pi the li u m ma y n o t r etu rn o n h ea l in g. F o r i n sta n ce , in ma jo r

ero s ive li ch e n pl an u s a l arg e are a o f th e to n gu e may be u lc era ted . O n h ea li n g, th e ep ith el iu m is o ften vir tu a ll y d ev o id o f f il ifo rm p ap il la e ap peari n g as a fl a t, fe atu re le ss tis s u e.

In ad di tio n to th o se p ati en ts in wh o m a rec o gn i z ab le pa th o lo g ic al c o nd iti o n ex is ts, th e re i s a gro u p, l a rge ly o f o l der fe ma le p ati en ts, wh o c o m pl ai n o f te n der ne s s an d s or en es s o f the

to n g u e, a n d w h o sh o w n o o b vi ou s s ig n s to a cc o u nt for th e se s ym pto m s. A ll te sts m ay s h ow

ne ga tiv e res u lts a n d it i s tem ptin g to c la s si fy su ch s ym pto m s as b ei ng p sy ch o g en ic i n o rig in . Th ere is n o d o u bt th a t in so m e pa tie nts th i s m a y b e so , b u t th e po s s ibi li ty o f h ab it -in du c ed

irri tati on mu s t a lw ay s be c o ns i de red in su c h c as es . Glo s s o dyn i a an d bu rn i n g m o u th s yn dro m e (BM S ) a re c o ns i de red fu l ly in Ch ap ter 17.

Traumatic irritation of the tongue

Th e m o bi li ty o f the to n gu e m ak es i t pa rtic u la rl y sus c ep tibl e to trau m a , w hi ch ma y be ac u te

(as th a t fo l lo w in g th e f rac tu re o f a ca ri o us to o th ) o r ch ro n i c (as th a t c au s ed , fo r exa m ple , b y th e a do p ti o n o f a h ab itu a l a c tivi ty s u c h as ru b bi n g th e to n gu e o ver th e e dg es o f th e a n teri o r te eth o r a de n tu re ). Th e le s io n s pro d u ce d va ry g reatly , fro m m il dly e ryth em a to u s pa tc he s of ten s ee n o n th e tip o f th e to n gu e to fra n k u l ce ratio n . Th es e trau m a tic u lc ers a re o fte n

extre me ly pa i nf u l a n d, a lth o u gh the y ma y b e evi de n tly rel ate d to th e s h a rp ed ge in q u es tio n , th e y m a y s h o w so m e de gre e o f i nd u ra ti o n du e to lo c al iz e d i n fla m ma to ry ch a n ge s to g eth e r

wi th w h iten e ss o f th e s u rro u n di n g ep ith el iu m β €”ch a nge s th a t a ro u se i mm ed ia te s u s pi ci o n o f

ma li gn a nc y. It h as b ee n am p ly de mo n s trate d, h o w ev er, th a t s u per fic ia l u lc ers o f th e to n g ue , su rro u n de d by a w h ite ma rg in , a re virtu a ll y a lw a ys b en ig n a n d o f tra u ma tic o ri gin . T he

ea si es t w a y to a ll ay th es e su s pi c io n s is to re mo v e th e o ff en di n g to o th o r ap pl ia n ce , o r to dea l w ith i t in s o m e o th e r s ui tab le w a y i n o rde r to e li mi n ate th e tra u m a c o m pl etel y.

Tra um a tic u lc er s r es o lve v ery ra pid ly a n d v irtu a lly a ll tra ce m ay be e xpe c te d to h av e

dis a pp ea red w i th i n a w ee k o f rem o vi ng th e irr itan t. If th is re so l u ti o n h as n o t o c c ur red in the exp ec ted tim e , the n th e le si o n mu s t b e vie w ed w ith s u sp ic io n a n d a bi o ps y ta ke n . In the

to n g u e, a s in o the r a re as , th ere i s littl e e vi de n ce o f a r ela tio n s h ip be tw ee n ph ys i ca l trau m a an d ma l ign a n cy , bu t th e p os s ib ili ty m u s t o bv io u sl y b e co n s id ere d.

Mo re d iff ic u lt to d ia gn o s e are th e mo re c h ro n ic fo rm s o f l in g ua l irr itati o n res u lti n g f ro m

ha b its o f v ari o u s kin d s (fo r exa m ple , to n gu e bi tin g) . Th es e o ften res u lt i n n o o bv io u s o ra l les io n , b u t fre qu e n tl y c a u se th e pa tie nt a gre a t deal o f co n c ern . In th e se c as e s it m ay b e

dif fic u lt to de ci de w h eth er a ny sy ste mi c fa cto r is i nv o lve d in th e pro d u cti o n o f th e s ymp to ms des c rib ed b y th e pa tie n t an d i t ma y be n ec es s ar y to c a rry ou t a fu ll s eri es o f ha em a to lo gi ca l in ve sti ga tio n s be fo re c om in g to th e co n c lu s io n th a t a h ab it is th e on l y f ac to r in vo l ve d. In

pa rtic u la r, th ere a re a nu m be r o f pa tie n ts in w h o m the i rrita tio n o f th e tip o f th e to n gu e a nd , of ten a ls o th e lo w er la bi al m u co s a , fo ll o ws th e mo st m in o r c h a ng e in m o rph o l o gy o f th e an ter io r d en titi o n , fo r e xa mp le , by th e rep la c em en t o f a cr ow n o r th e i n se rtio n o f a rep la ce me n t pa rti al de n tu re. Fr eq ue n tly, th e re is no d is ce rn ib le a bn o rm al ity in th e

res to rati o n to a cc o u nt fo r th i s. N on e th ele s s, i t is e vi de nt fro m th e mi ld ery the m a of b oth th e

ti p o f th e to n g ue a n d th e m u co s a o f th e lo w er li p th a t tra u ma i s o cc u rrin g . It is o f ten di ffi cu l t to he lp s u ch pa tien ts i n th e a b se n ce o f an y di sc er ni bl e fau l t in the res to ra tio n .

Enlargement of the foliate papillae

Th e f o lia te pa pi ll ae a pp ea r a s bi la tera l , pi n k n o dul es o n th e s ide o f the to n gu e , a t th e

jun c tio n o f th e a n teri o r tw o -th i rds a n d po s teri o r th ird . Th ey c an be co m e e n la rg ed an d

in fl am ed (f ol ia te pa pi ll itis ) an d c au s e so re n es s to th e p a ti en t. Th e a pp ea ra nc e o f th e se m ay ca u se co n c er n to pa tie n ts, w h o s h o ul d be a de qu a te ly re as s ur ed .

P. 70

Geographic tongue (β€ erythema migransβ€™, benign migratory glossitis)

Depa p ill a ti o n o f th e to n gu e is a ls o a m ar ked fe atu re o f th e c o nd iti o n u su a ll y d es c rib ed a s geo g ra ph ic to n g ue o r β€ eryth e ma mi gra n s β€™. T he p atte rn o f d ep a pi lla tio n is qu i te

ch a rac te ris tic w ith th e af fe cte d a re as o c c ur rin g as r ed pa tc he s s ur ro un d ed b y w h ite bo rd er s. Th e p a tc h es ar e d is trib u ted o ve r th e su rf ac e o f th e to ng u e in a β€ ma p -lik e β€™ fa sh io n

(h en c e th e n am e β€ geo g ra ph ic to n g ue β€™) an d ten d to v ary po s iti o n , appa re ntl y mo v in g

ab o ut the s u rfa ce o f th e to n gu e , h en c e th e n a me er yth em a mi gra n s (o r be n ig n mi gra to ry

glo s s itis ) (Fi g. 6. 8). Th e ap pe ar an c e of th e l es io ns m ay be th e pa tie n t's o nl y co m pl ai n t bu t so m e p a tien ts c o mp la in o f ten d ern es s o f the to n gu e , pa rtic u la rl y w h en ea tin g h igh l y

fla vo u re d f o od . Th i s co n d itio n of ten p ro ve s extre m ely w o rryi n g to th e p ati en t i n vie w o f its of ten qu i te s pe c ta c u la r a pp ea ra n ce . Th e ag e ran g e of th e pa tie nts i n vo lv ed is v ery w id e.

Al tho u g h ge o gra p hi c to ng u e is ge n era ll y co n s id ere d to be a co n d itio n af fe cti ng a du l ts, th e

au th o rs h av e s e en p ati en ts fro m th e ag e o f 3 y ea rs u pw a rds . Th e a ss o ci a ti o n o f g eo g ra ph ic to n g u e w i th ps o ria s is h as b ee n re po rte d in a n u m be r o f stu d ies . A f ur the r s u gg es ted

as so c ia tio n is be tw ee n ge o gra ph i c to ng u e an d de ep fis su ri n g. A ga in , th e re is n o c le ar

evi de n ce th at thi s is o th er th an a r an d om a s so c ia tio n o f tw o rel ati ve ly co m mo n co n di tio n s .

Ery th em a mi gra n s ca n o c c ur el se w h ere o n th e or al m uc o s a an d h as b ee n re po rte d a ff ec tin g th e pa la te an d la b ia l m u c os a .

Th e a e ti o lo g y o f ery the ma m ig ra n s is u n kn o wn . It is ra rel y a s so c ia ted w ith an y u nd erl yi n g

sys te mi c dis o rd er al tho u g h , sh o u l d th e re b e a h ae matin i c de fic ie n cy s u ch a s a n ae mi a, th e re ma y b e an in c rea s e i n th e ten de rn es s o f th e to n gu e . Dia gn o s is is i n g en era l s im ple , b ein g

ba se d on the c h ar ac teri sti c ap pe a ran c e an d be h av io ur o f le si o n s, a lth o u gh it m a y so m eti me s be n ec es s ary to s ee th e pa tie nt o n se ve ral o c ca s io ns to c o nf irm th e mi gra to ry n atu re o f th e

co n di tio n . If th e to n gu e is s ym pto m ati c, th e n ap pr op ri ate bl o o d te sts s h o u ld be c ar rie d o u t to eli mi n ate a n y u n de rly in g h ae ma tin i c de fic ie n cy . In s o me pa tie n ts the l es io n s ma y be

so m ew h at le ss d ef in ed an d w ith a m a rke d w h i te b o rder, a lth o u gh , in mo s t c as es , a t le a st a sm al l are a o f w h ite d em arc a tio n m ay b e s e en . In o ther pa tie nts th e le si o n s ma y o c c u r in a

mo re s ta tic m a n ne r o r in a s in g le s ite an d th is m ay c au s e co n fu s io n w i th a tra um a tic le s io n .

Th ere a re, h o w ev er, f ew c a se s o f g eo g rap h ic to n gu e th at ca n n o t ev en tu al ly be d ia gn o s ed o n a c l in ic a l b as i s al o ne . B io p sy is ra re ly n ec es s ary to co n f irm d ia gn o s is , bu t s h o u ld be

co n s id ere d i f th e re is a po s s ib ili ty o f si n is ter path ol o gy . No s u cc es s fu l trea tme n t is k n ow n f o r a p a in fu l ge o gra ph i c to ng u e, b u t a n an a lg es ic m o u thwa s h ma y pro v id e s ym pto m ati c rel ie f. Mo s t p atie n ts le arn to av o id fo o ds tu ff s th a t i rritate th e ir ton g u e. S o me a u th or itie s h av e

su g ges te d th e u se o f z in c s up pl em en ts fo r ge o gra ph ic ton g u e b u t th e evi de n ce fo r th is be in g eff ic ac io u s i s ma in l y a n ec do ta l.

Fig. 6 .8 Geo g ra ph ic to n g ue (er yth em a mi gra n s ).

Median rhomboid glossitis

Th e to n g ue m ay b e i n vo lv ed in c a n di da l i n fec tio n (Ch ap ter 4). Th is i s pa rtic u la rly s o in th e ch ro n ic i n fec tio n s c o n se qu e nt to im mu n o lo g ic a l d ef ec ts (su c h a s ch ro n i c mu c o c uta n eo u s

ca n di do s is ) o r in d eb ili tati n g c h ro n ic d is ea s es . In s uc h c a se s th e to ng u e ma y no t o n l y b e

co ve red b y p la qu e s o f c a nd id a l p se u do m em bra n e, bu t m ay al s o be co m e de ep ly fis s u red a n d

ap pa ren tl y f ibr os e d. It s h o u ld be s tres s ed th at ca ndi do s is o f th e to n g ue , a s in th e o ral c a vity in g en er al , ma y we ll s ig na l u n de rlyi n g s ys te mi c dise as e, s u c h as d ia b etes o r iro n d ef ic ie nc y.

Les io n s o f su p erf ic ia l mi dl in e gl os s iti s are u s u al ly se en i n a pp are n tly h ea lth y pa tie nts . Th es e are , by de fi n itio n , i n the m id li n e o f th e to n g ue i n si tes v ary in g fro m im me di ate ly in fro n t o f th e va ll a te p ap il la e (w he re th e c o n di tio n i s k n o wn as β € me dia n rh o m bo i d g lo s s itis β €™

bec a u se o f the c h ara c teri sti c sh a pe o f the le si o n in th a t s ite ) to w ard s th e a n ter io r d o rsa l su rfa c e of th e ton g u e. M ed ia n rh o mb o id gl o ss iti s h as f o r lo n g be en co n s id ere d to be a

dev el o pm en ta l ab n o rma l ity i n s o me w a y c o n ne c te d w ith th e si te o f th e em bry on i c tub er cu l um im pa r. It h a s no w b ee n re co g n iz ed th a t th es e l es io ns a re o ften a s so c ia ted w i th th e p res e nc e of Can d id a .

Me di an rh o mb o id gl o ss iti s ap pe a rs as a n a rea o f de pa p ill ati o n o n th e d o rs um o f th e to ng u e

an d ma y h av e a re d, w h i te, o r y el lo w a pp ea ra nc e (Fig. 6. 9 ). Lo n g-te rm th era py w ith to p ic al an tif u ng a l a ge n ts, f o r e xa mp le , n ys tati n pa s ti ll es , m ay be e ffe cti ve in r edu c in g th e si z e o f th e le s io n , a nd rec e nt su c c es s ha s b ee n rep o rted w ith s ys tem ic a n tif un g al a ge n ts (fo r

exa m ple , fl uc o n az o l e). N ot al l l es io n s d is ap pe a r ful ly wi th th is tre atm en t. A n y d o u bt a s to

th e n a tu re o f th e c o n di tio n s h ou l d be res o lv ed b y bio ps y an d h is to pa tho l o gi ca l exa m in a tio n .

In ge ne ra l, h o w ev er, th e ch a ra cte ris tic ap pe a ran c e, s i te , a n d te xtu re o f th e le si o n is s u ch as to en a bl e a c o n fid en t in i ti a l d ia gn o s is to b e ma de o n c li n ic al

gro u n ds . P ati en ts pr es en tin g w ith th is l es io n s h o u ld u n de rgo b lo o d tes ts to ex cl u de a n

un d erl yin g h a em ati n ic de fi ci en c y o r di ab etes . M ed ian rh o m bo i d g lo s si tis m ay a ls o b e s ee n i n pa tien ts in fe c te d w ith HIV .

Fig. 6 .9 M ed ia n gl o ss iti s.

Disturbances of taste and halitosis Disturbances of taste

β€ Hyp o geu s ia β€™ is a re du c ed tas te se n sa tio n a n d β € ag eu s ia β €™ is a c om p lete l os s o f

ta s te. A n u n pl ea s an t o r al tere d tas te se n sa tio n i s kn o w n as β € dys ge u si a . Pa tie n ts

co m pl ai n in g o f di stu rb an c e o f ta s te s en s a ti o n a re am o n g th e mo s t d iff ic u lt to ma n ag e. T he re is o ften no th i ng o th er th an th e pa tie n t's o w n de sc ri ptio n to ch a ra cte riz e th e co n d itio n an d

ob jec tiv e te s ts a re o fte n n o t h elp fu l . Ph y si o lo gi cal tes ts to de term in e th e pa tien t's a b ili ty to dif fe ren tia te the b as ic ta s te s en s a tio n s o f sw e et, s o u r, bi tter, a n d sa lt ma y b e do n e, bu t

th e se i n th em se lv es m ay gi ve li ttl e in fo rm a ti o n , sin ce th e se n se o f tas te as a w h o le de pe n ds on a mi xtu re of as s o ci ati o n s of ta ste a nd s c en t.

Dist urbances o f t ast e Dys geu s ia β €”un p le as a nt o r a lter ed tas te se n sa tio n Ag eu s ia β€” co m pl ete lo s s o f tas te

Hyp o geu s ia β €”red uc e d ta s te se n sa tio n

P. 71

Tru e ne u ro lo g ic al d is tur ba n ce o f th e s en s e o f tas te is e xtrem e ly rar e, b ut ma y oc c u r i n so m e gen e ra liz e d ab n o rma l itie s o f th e ce n tra l n e rvo u s syste m. A so m ew h a t mo re c o mm o n

(al th ou g h sti ll ra re) n eu ro lo g ic a l d is tu rba n c e i s tha t d u e to su rg ic a l tra u ma to th e ch o rd a ty mp an i fo l lo w in g o pe ra ti o n s o n the m id dl e e a r. If th e n erv e is di stu rb ed , ei th er d u rin g

op era tio n or by p os to pe ra tive o ed em a , an are a o f tas te di stu rb an c e ma y de vel o p on the s id e of th e to n g u e c o rre sp o n di ng to th e term in a l di stri bu ti on o f th e ch o rd a tym pa n i w i th the

lin g u al n erv e. In a f ew p ati en ts w ith B el l's p a ls y ( Ch ap ter 15) th ere i s a si mi la r l os s o f tas te se n sa tio n d u e to i n vo lv em en t o f th e c h o rda ty mp an i o n th e af fec ted si de .

Me di ca tio n c a n af fe ct ta s te, a n d dru gs c o m mo n ly im pl ic a te d in c lu de th e an ti rh eu m ati c (fo r exa m ple , a ll op u rin o l an d ph e n ylb u taz o n e) an d a n ti meta bo li te ( fo r e xa mp le , me th o tr exa te)

dru gs . P en ic il la m in e an d c ap to pri l (a n a n gi o ten s in -co n ve rtin g en z ym e (A CE ) i n hi bi to r) h av e

bee n re po rte d a s c au s in g ta ste di s or de rs. M e tro ni daz o le c an a ls o c a u se a n u n ple a sa n t ta ste . An y dru g s ca u s in g xer os to m ia c an a l so i n di rec tly a ffe ct ta s te pe rc ep ti o n .

Th e m o s t co m m on ca u se o f a di stu rb an c e o f ta ste se nsa tio n or o f a f o ul ta ste i n the m o u th is

th e p res en c e of a p yo ge n ic in fe c ti o n , mo s t u s ua l ly the re su l t o f p er io do n ta l d is e as e, i n fec tio n in th e n os e o r s in u s es , o r a d is ch a rg e f ro m a les i on su c h a s an i n fe cte d d en ta l cy st. A n in fe cti ve fo c us m a y b e very d iff ic u lt to d ete ct, p artic u la rly w he n p res en t i n a rel ati ve ly

in ac c es s ibl e are a s uc h a s a s al iva ry gl an d . Th e cl in i ca l exa m in a ti o n o f s u c h a pa tie nt sh o u ld al wa ys i n cl u de a c are fu l se ar ch f o r p us -co n tam i na ted sa l iva f ro m an y o f th e ma jo r s a li va ry gla n ds a s w el l a s ra di o gra p hi c as s es s me n t fo r th e pres en c e of p os s ib le in tra bo n y in fe c te d

les io n s , s in u si tis , a nd s im il ar po s s ibl e in fe cti ve fo c i. C lea rl y, s u ch p a ti en ts a re su f feri n g no t fro m a tru e di stu rb an c e o f ta s te s en s a ti o n , bu t f rom th e c h ro n ic im po s iti o n o f u n p lea s an tl y ta s tin g ma ter ia l i n to the o ra l ca vi ty.

Tab le 6. 6 li sts s o me c o n di ti o n s th a t m ay g ive ri se to a lte ra ti o n s or lo s s o f tas te. An o s mi a (ab se n ce o f sm e ll) c an e ith er be tra n si en t d u e to upp er res p ira to ry tra c t in f ec tio n s o r

per ma n en t a s a res u lt o f te ari n g o f th e o lfa c to ry ne rve fo l lo w in g ma xi ll o fac i al o r h e ad

in ju rie s an d w il l r es u lt i n so m e di mi n uti o n o f ta s te. Th e u n de rly in g ca u se f or th e a l te ra tio n i n ta s te m a y b e el ic ite d f ro m the p ati en t's h is to ry and ex am in a tio n o f th e o ra l c avi ty, b u t it is ad vis a bl e to ch e ck fo r z i n c de fic ie n cy .

Pa tie n ts w i th ev id en c e o f cr an i al n erv e def ic its o r rep o rtin g n eu ro lo g ic al s ym pto m s sh o u ld b e se n t fo r s p ec ia li st e va lu a tio n

an d ap pr op ri ate im a gin g . Wh en al l su c h ex am in a tio n s h a ve be en p erf o rme d, th e re rem ai n s a

P. 72

gro u p o f p ati en ts in w ho m n o a b no rm a li ty o f an y kind i s de te c te d, bu t w h o c o nti n ue to

co m pl ai n o f a n u n ple a sa n t ta ste . Th es e pa tie n ts a re o fte n o bs es s io n al , bu t it i s very d iff ic u lt to de te rm in e w he th er thi s is th e ca u s e o r the c o n sequ e n ce o f th e ir co m pl ai n t (s ee Ch ap ter

17). A ltera tio n s i n tas te ca n b e o n e o f th e sy mp to ms o f o ral d ys ae s th e si a. So m e o f th e mo s t

dif fic u lt pa tie nts to tre a t am o n g tho s e w ith d is turba n ce s o f ta ste s en s ati o n a re th o s e wh o a re

we ar in g rel ati ve ly rec en tl y a c qu ire d de n tu re s . Fe w ta ste bu d s ar e c o ve red b y th e de n tur e an d th e o lf ac to ry ne rve e nd in g s ar e i n n o w ay a ffe c te d bu t, n o n eth el es s , so m e su c h p ati en ts

co m pl ai n o f a n a bn o rm al ity o r e ven co m pl ete lo s s o f ta s te se n sa tio n . T he e xpl a na tio n s fo r

th i s are n o t c o n vin c in g , bu t i t w o u ld se em th a t va ria tio n s in textu re b etw ee n th e u n c o ver ed pa la te a n d th e d en tu re ma y pl ay a pa rt.

Table 6.6 Conditions associated with alterations in taste Den tal c on d itio ns

Pe rio d o nta l di se as e s Car io u s le si o n s

Dis ch a rgi n g den ta l si n us

Res to rati o n s w i th ma rg in a l def ic ie n ci es Dry s o c ket

As s oc i ate d str uc tu res Sa li va ry gla n ds

Sa li va ry gla n d h ypo f u cti on Si al ad en i tis

Si n us e s

Si n us i ti s

Lun g s

Res pi ra to ry di se a se

Sto m ac h

Gas tro -oe s op h ag ea l

re flu x di so rd er

Sy stem ic d iseas e Ura em ia

Neu ro lo g ic a l d is o rde rs (e .g . B el l's pa ls y, b ra in tum o ur s, d a ma ge to c h o rda tym pa n i) An a em ia

Deficien cies e. g. z in c Dr ug s

AC E in h ib ito rs , li th iu m sa lts , g o ld, ca rb im az o l e, metro n id a zo l e, p en i ci lla m in e, xero g en i c dru g s Sm o kin g

Psy ch o gen ic

Ora l dy sa es th es ia

Ps yc h o si s (de lu s io n s , h ypo c h o n dri as is )

Th e d en ti st's re s po n si bi li ty i n su c h c a se s as th o s e d es c rib ed a bo ve i s to el im in a te, b y

deta il ed e xa mi na tio n , a n y o f th e lo c al c au s es . Th e pa tie n t m ay th en n e ed to be re fe rred

ap pro p ria tel y to e li mi n ate n o n -or od en ta l ca u se s . If th is ex am in a tio n i s n eg ati ve an d if a ll co n c ern e d a re s ur e th a t th ere i s no n as a l o r si mi lar a eti o lo g y, th en th e re is li ttle po i nt in

attem p ti n g tr ea tme n t. M ou th w a sh e s ma y o c c as io n a ll y h e lp bu t, i n ge n era l, th e pa tie n t w h o is ob s es si o n al ly co n c ern e d wi th a pe rs is ten t ta ste is c o m pl etel y r es is tan t to lo c al trea tme n t.

Halitosis

A cl o se ly rel ate d pro b le m i s th at of h a lito s is β€” bad b re ath . Th i s ma y o cc u r i n th e he al th y, o r in th o se w ith si gn if ic an t or al o r g en e ral iz e d d is eas e. It is o f te n a tra n si en t p ro bl em , bu t, i f no t, w a rra n ts i n ves tig a tio n . Th e ca u s es o f h a li tos is ma y b e di vid ed i nto lo c al (Tab le 6. 7 ),

sys te mi c (Tab le 6. 8), an d d ru g -in du c ed (Tab le 6. 9 ) c au s es . P ati en ts w h o s mo ke o r ea t a

gre at d ea l o f ga rl ic a nd h i gh ly s pi ce d f o o ds w il l ha ve a c h ara c teri sti c od o u r o n th ei r b rea th .

Loc a l c a u se s o f h a li tos i s in cl u de p oo r o ral h yg ie n e, c h ro n ic pe ri od o n tal o r de n tal di s ea se , a nd ea r, n o se , an d th ro a t (E NT) pro b le ms , s u ch a s s in u si tis o r ch ro n i c ton s il li tis ( Tab le 6. 7). Sy ste mi c di se as e, as i n ren a l o r h ep ati c fa il ur e, re sp ira to ry di se as e , an d u n co n tro ll ed

dia b etes l ea di n g to ke to si s, i s a ra re c a u se o f ha lito si s bu t s h o u ld be c o n si de red ( Tab le 6. 8). So m e dru g s are re po rte d a s c au s in g h al ito s is (Tab le 6. 9 ). Th e mo s t c o mm o n dru g -in du c ed

fo rm o f h a li tos i s is pro b ab ly th at du e to th e l a ck o f o ra l h ygi en e re su l ti n g fro m ia tro ge n ic d ry mo u th (d is cu s s ed in Ch ap ter 8 ).

Th e pro b le m o f d el us i on a l h al ito si s is f ur th e r d is cus s ed in Ch ap ter 17.



Th e h i sto ry is su g ge sti ve o f e ryth e ma m ig ran s (g eo gra ph ic to n g ue ). E xa m in ati o n o f th e to n g u e w ill c o n fir m th e di a gn o si s a nd e xc lu de o th er pa th o lo gi c al c on d iti on s .

Th e r ec en t h is to ry o f i n cre as i ng s o ren e ss s u gg es ts a po s s ib le u nd er lyi n g fa cto r, s u c h as

an a em ia , du e to i ro n de fic i en c yβ€” pa rtic u la rly in vie w o f h e r rep o rted tir ed ne s s. T hi s gi rl

sh o u ld b e a s ked f ur th e r q u es tio n s ab o u t h er ge n era l he a lth , in c lu d in g an y ga s tro in tes tin a l sym p tom s a nd d ru g hi s to ry . S ig ns o f i ro n de fi ci en c y a n a em ia in c lu d e pa ll or o f th e m uc o u s

me mb ran e s, an g ul ar ch e il itis , a ph th o u s sto m ati tis , gl o ss iti s, o ra l c an d ido s is , a n d ko il o n yc hi a .

Table 6.7 Local causes of halitosis Mo u th

Po o r o r al h yg ien e

Fo o d pa c kin g a n d sta g na tio n Ch ro n ic pe rio d o n tal di s ea se

Ac u te n e cr o ti z in g u lc era tiv e g in g ivi tis Dry s o c ket

Pe ric o ro n itis

Ch ro n ic de n tal s ep si s In f ec tio n s

Ma li gn a n t tu mo u rs Hae m or rh ag e

No se a n d ph a ryn x Ph a ryn g itis To ns i lli tis

Si n us i ti s (po s tn as a l dri p) Ma li gn a n t tu mo u rs Fo re ig n bo d ie s

Table 6.8 Systemic diseases causing halitosis

Low e r re sp ira to ry trac t i n fec tio n s Dia be ti c ke toa c id o si s Kid ne y fai lu re Live r fa i lu re

Fe bri le il ln es s

Table 6.9 Drugs that may produce halitosis Pa ra ld eh yd e

Is o s o rbi de di n itra te Dis ul fi ram Al co h o l

Cyto to xic d ru gs (in d ire ctl y)

Xe ro ge n ic d ru gs (in d ire ctl y)

Bl o o d te sts s h o u ld be u n de rtak en i n cl u di ng a f ul l blo o d c o un t (FB C ), se ru m B 1 2 , red c el l a n d

se ru m fo la te, a n d se ru m fer ritin es tim ati o ns . It m ay a ls o be w o rth w hi le to c ar ry o u t a s er um zi n c as s ay . Iro n de fic i en c y an a em ia i n a fe ma le s tude n t w ith n o

P. 73

ob vi o us g a stro i nte sti n al di se a se is m o s t li kel y to be du e to e xc es s me n stru a l lo s s an d /o r p oo r die t. Th e β€ geo g ra ph ic to n g ue β€™ ca n b e tre a te d sy mp to ma tic al ly w ith a n a n al ge si c rin s e (fo r e xa mp le , be n z yda m in e) an d a vo id a nc e o f β € irri tati ng β €™ fo o ds . A n y h ae ma tin i c

def ic ie n cy s ho u l d b e in ve sti ga ted a n d a p pro p ria tel y ma n a ge d.


Wh at a d vic e w o ul d yo u g ive to th i s gir l's m o the r?

Q2

Wh at o th e r fa c tor s mu s t be c o n si de red w h en p ro vi di ng d en ta l trea tme n t fo r a pa tie nt wi th Do w n' s sy nd ro m e?

En l arg em en t of th e ton g u e ( ma c ro glo s s ia ) i s a we ll re co g n iz ed fe a tu re o f Do w n' s sy nd ro m e. Th e to n g ue m ay b ec o me u lc er ate d a s a re su l t o f o c c lu sa l tra um a . A n en l arg ed to n gu e a nd co n s ta n t d rib bl in g ma y pre di sp o se to th e de ve lo pm e nt of an g u la r c h ei liti s in Do w n 's

syn d ro me , p arti cu l arl y i f th e re is m o uth bre ath in g . M ic ro bi ol o gi ca l sa m pl in g sh o u ld b e c a rri ed

ou t a n d an ap pro p ria te an ti mi cro b ia l ag en t ap pl ied . Sta ph yl o co c c u s au re u s is c o mm o n ly

is ol a te d fro m li p fis s u res , w h ic h m ay res p on d to top ic al fu c id in w i th the ad di tio n o f a lo w -

po ten c y s ter o id pre pa ra tio n . Na sa l c arri a ge o f S. a u reu s n ee ds to b e a d dre ss e d, i f re in fe c ti o n is to b e av o id ed . Pe rs is ten t l ip fi ss u res o f ten re qu i re s u rg ic al e xc is io n , a lth o u gh cr yo s urg er y ha s a ls o be en ad vo c ate d. Wh e n c on s id er in g el ec tive s u rgi ca l in ter ven ti on in a p ati en t w i th Dow n 's s yn d ro me , a ris k as s es sm e nt sh o u ld b e u n de rtak en i n rel ati o n to a na e sth e si a an d su s ce pti bi lity to i nf ec tive en d o ca rdi tis (s e e b el o w) .

Th ere a re a nu m be r o f fa cto rs th a t n ee d to be co n s id ere d w he n p ro vid in g de n tal tre atm en t

fo r p ati en ts w ith Do w n 's sy n dro m e. C o ng en i tal c ard ia c a no m a lie s a re pre se n t in a b ou t 40 pe r ce n t o f in fa n ts w ith Do w n 's s yn dro m e bu t m o st ca n be s u c ce ss fu l ly co rr ec ted by s u rge ry. An tib io ti c pro p hy la xi s ma y, th er efo re , be n e ed ed for den ta l pro c ed u res l ike ly to cr ea te a ba cte rae m ia . Oth e r p ote n tia l p ro bl em s fo r th e d en tis t i nc l ud e in c rea s ed su s c ep tibi li ty to

in fe cti on an d pe rio d o n tal di s ea se . Th er e m a y b e reduc e d co o p era tio n a n d u nd er sta n di ng o f th e n ee d fo r o ra l h ygi en e , bu t th e ma jo ri ty o f pa tie n ts wi th Do w n' s sy nd ro m e c a n be

su c ce s sfu l ly trea ted i n ro u tin e de nta l pra c tic e. T he c a rria ge ra te o f h ep a titis B i s rep o rted ly hi gh e r in pa tie nts w h o ar e i n sti tuti o n al iz ed .



Q2

Wh at is th e di ffe ren ti al di a gn o si s o f th e li p co nd itio n ?

Th e m o s t li kel y d ia g no s is o f th is pa tie n t's c hr on i c li p c o n di tio n i s ac tin ic c h ei li tis du e to

su n li gh t da m ag e, a s a res u lt o f h is o u td oo r o cc u pa tio n . S o la r U V B ra di ati on is m o st da m ag in g an d res p on s ib le fo r pre ma l ign a n t a n d a g ein g d am a ge. It is i mp o rtan t to ch e ck th e fa ce ,

sc al p, a n d o the r e xp o se d sk in fo r ac tin i c ker ato s es, pa rtic u la rl y i n fa ir -ha ir ed in d ivi du a ls .

Oth er c on d iti on s to b e co n s id ere d in th e d if fer en tia l dia g no s is a re li c he n p la nu s a n d lu pu s

eryth e ma to su s . A n al le rgi c ch e il itis i s al s o po s si bl e bu t l es s li kel y u n l es s it i s su p eri mp o se d on an al rea dy s u n -da ma ge d li p. B io p sy is e s se n tia l a n d ma n ag em en t de pen d s o n th e hi sto p ath o lo g y an d de gre e o f e pi th e li al d ys pla s ia .

Ac tin i c ch e il itis i s co n s id ere d to be a p rem al ig n ant c o n di tio n a n d c a n tra ns fo rm i n to

sq u am o u s ce ll ca rc in o m a, w h i ch c a n be q u ite ag gre s si ve co m pa re d to o th e rs a t th is s ite. In

ca se s w h ere th ere i s si gn if ic a nt dys p la si a bu t n o fran k c arc in o m a , o n e f o rm o f tre atm en t i s a verm il io n e cto m y, in wh i ch th e ve rm ili o n bo rd er is co m pl ete ly ex ci se d an d th e la bi al m uc o s a

ad va nc e d to joi n th e sk in . La se r ab la tio n c a n a ls o pr od u ce g o od c o s me tic re su lts w i th few e r po s to p era tiv e co m pl ic a tio n s. C h em ic al e xfo l ia tio n with 5 -flu o ro u ra ci l c a n be u s ed b ut

so m etim e s ca u se s qu i te s ig n ifi c an t d is c om fo rt an d in f la mm ati o n u n til h ea lin g tak es p la ce . A ll

pa tien ts w ith ac tin ic c h ei li tis o r e vi de nc e o f s u n -da ma ge el se w h ere o n th e sk in m u st b e gi ven ad vic e ab o u t s u n av o ida n c e ( if po s si bl e) an d th e u se o f s u n sc re en s (li p sa lv es o r cre am s )

wi th a h ig h su n p ro tec tio n f ac to r. Ol de r m en in p arti cu l ar ca n b e en c ou ra g ed to w ea r a h a t ou td o or s, e sp ec i al ly if th ey h av e th i nn i n g h a ir o r a re ba ld . P ati en ts di ag n o se d w i th ac tin i c ch e ili tis sh o u l d b e fo ll o we d u p o n a re gu l ar ba s is .

Project 1.

Th ere is a w i de ra n ge o f m o u thw a s he s c om m erc ia ll y ava i la bl e f o r th e ma n a ge me nt o f

ha li to si s (ba d bre a th) . Wh at are th e co n s titu en ts of the s e pre pa ra tio n s ? Is th e re an y evi de n ce th at the y are e ffe cti ve in tre a ti n g h al itos is ?

Au th o rs : Title:



Co py rig ht Β © 2003 Ox fo rd U ni ve rsi ty P re ss > T ab le o f Co nt e nt s > 7 - Sw e llin g s of t he f ac e a nd ne ck

7

Swellings of the face and neck Problem case Case 7.1

A 65-yea r-ol d ma n a tten ds y o ur s ur ger y f or th e p ro vi si o n o f n e w de n tur es . Th e pa tie nt h as sm o ked (30 ci ga re tte s pe r d ay ) si n ce h e w as a te en age r. Wh en qu es tio n i ng th is g en tl em an ab o ut hi s me di c al h is to ry y o u n oti ce th a t h e ha s a β € cro a ky β€™ vo ic e. H e rep o rts a 2-

mo n th h is to ry o f in c rea s in g h o ars e ne s s. O n e xa mi na tio n y o u c an p a lpa te a h ard , fi xe d, no n ten d er ju gu lo - om o h yo id l ym ph n o de . Q1

Wh at o th e r q ue s ti o n s wo u l d y o u as k th is ge n tlem a n?

Q2


Facial swelling Differential diagnosis

Al tho u g h de n tal in f ec tio n s ar e b y far th e m o s t co m mon ca u s e o f sw e ll in gs a b ou t th e fa c e

se en b y th e de n tal s u rge o n, th e re ar e ma n y oth e r p oss ib il itie s th at m u s t be c o n si de red in a dif fe ren tia l di ag n o si s. Th es e in c lu d e f ac ia l sw e ll in gs d u e to al le rgy o r a n gi o ede m a (o ften

mi sd ia gn o s ed as a p u rel y d en ta l pro b lem ) an d th e s wel li n gs in or al Cro h n 's d is ea s e ( or of ac i al gra n u lo ma to s is ), w h ic h ar e e qu a ll y s u s ce pti ble to a m is ta ken in iti al di a gn o si s . S im ila rl y,

fac i al s we ll in g du e to i nf ec tio n s o f n o n -od o n tog en ic o rig in β€”su c h a s th at, f or in s tan c e, i n

ac u te s ia l ad en iti sβ €”is c om m o nl y a s su m ed to b e o f den ta l o rig in . M a ss ete ric h yp er tr op h y c a n giv e ris e to so m e di ag n os tic p ro bl em s in pa tie n ts pre se n tin g wi th fa c ia l s w el li ng a n d is dis c u ss ed i n Ch ap ter 16 (β € Tem po ro m an d ib u la r d is o rde rs β€™).

Th ere a re a la rge n u mb er o f c o n di tio n s, b o th lo c a liz e d an d ge n era li z ed , th at c a n c au s e fa ci al sw el li n g ( Tab le 7. 1). S om e o f th e se co n d itio n s a re co n s id ere d el se w he re in th i s bo o k.

Rea de rs a re, h o w ev er, a dv is ed to c on s u lt te xtb o ok s o f p ath o lo g y, m ed ic in e , an d s u rge ry f o r deta il s o f o th e r co n d itio n s .

Swellings in the neck

Cerv ic al s w el lin g s ge n era ll y p res e nt ei th e r a t th e si de o r th e mi dd le o f th e n ec k an d th ei r

dif fe ren tia l di ag n o si s is o u tli ne d in Tab le 7. 2 . Cer vic a l l ym ph a de no p a th y w il l b e di sc u ss e d i n mo re de tai l.

Table 7.1 A β€ surgical sieveβ€™ for the differential diagnosis of facial swelling

Co n ge ni tal (e . g. l ymp h an g io m a, h ae ma n gi o ma ) In f ec tio u s

O ro d en ta l in fe c ti o n s

S a li va ry g la n d in fe cti o n s ( Ch ap ter 8) C u tan e ou s in fe c ti o n s

Neo p la sti c

S a rc o ma s

C ar ci no m a (Ch ap ter 10)

Tra um a tic

P o s t-in ju ry

P o s to pe rati ve

S u rg ic al em ph y se ma

Im m un o l o gic a l A l le rgi c

H ere di tary ( Ch ap ter 14)

En d o cri n e a n d me tab o li c (Ch ap ters 13 an d 18 ) C u sh i ng 's sy n dro m e A c ro m ega l y

M y xo ed em a

N ep hr oti c sy n dro m e

Oth er s, e . g. ;

Oro fa c ia l gra n u lo m ato s is (Ch ap ter 12 )

Id io p ath i c or dr ug -in du c ed a n gi oe de ma (Ch ap ter 14) Co rtic o ste ro id th era p y ( Ch ap ter 3)

Ma s se teri c hy pe rtro ph y (Ch ap ter 16)

Cervical lymphadenopathy Differential diagnosis

No rma l lym p h n od es a re n o t pa l pa bl e. E n la rg em en t o f a n o de o r a c h an g e i n tex tu re, s o th a t it m ay b e p al pa ted th ro u gh th e s ki n, in d ic ate s tha t i t is e ith e r th e pri ma ry si te o f a

pa tho l o gi ca l pro c es s (fo r ex am pl e, l ym ph o m a) o r s eco n da ril y i n vo lv ed (fo r exa m pl e, i n an in fe cti ve o r n eo p la sti c pro c es s el se w h ere in the b ody) . Diff ere n tia tio n be tw ee n th es e tw o si tu a tio n s is i m po rta nt. T h e

vas t m a jo rity o f c erv ic a l l ym ph n o de en l arg em en ts are du e to ei th er in fe cti o n o r n eo p la s ia

(Tab le 7. 3). Ho w ev er, l ym ph n o d es c an b e en l arg ed in so m e co n n ec tiv e ti ss u e di so rd er s (fo r

exa m ple , s arc o id o s is ) a n d ( rare ly ) a s a si de -eff ec t o f d ru g th era py (fo r ex am pl e, p h en yto i n) .

Table 7.2 The differential diagnosis of swellings in the neck Si de o f n e ck

Cerv ic al ly mp h ad en o pa th y

Sa li va ry gla n d en l arg em en t ( Ch ap ter 8) Ac tin o m yc o si s

P. 78

Car oti d bo d y tu m ou rs Bra n c h ia l cy st

Ph a ryn g ea l p o u ch Cys tic h yg ro ma

Mi dd le o f th e n ec k

Th yro id e n la rge me n t (m ul tin o du l ar go i treβ €”ne o pl as ia , ec to pi c) Th yro gl o ss a l c ys t Derm o id c ys t

β€ Pl u ng in g β€™ ra nu l a

Lud w ig 's an g in a

Table 7.3 Cervical lymphadenopathy: important infective and neoplastic causes In f ec tive ca u s es Lymp h ad en i tis O ro d en ta l A n tra l

T o ns i lla r A u ra l

N as o ph a ryn g ea l S c a lp

F a ci a l

Tu be rcu l o si s Bru c el lo s is

In f ec tio u s mo n o n u cl eo s is (g la n du l ar fe ver) HIV in fe c ti o n

To xo pl as m o si s (c at sc ra tch f ev er) Sy ph il is

Neo p la sti c ca u s es Lymp h o ma Leu ka em ia

Se co n d ary n eo p la si a

Examination of lymph nodes

Lymp h n o de s ma y be ex am in e d b y pa lp ati o n, u l tras o u nd- gu id ed fi n e n e ed le a sp ira tio n ( US GFNA ) an d ap pr op ri ate im a gin g te ch n iq u es . Hi sto p ath olo g ic al e xa mi n ati on of a n ex ci s ed n o de is o n ly in di c ate d i n s pe ci fic s i tu a tio n s , su c h a s occ u lt pri ma ry h ea d a n d n ec k tu mo u rs a n d lym ph o m a.

Wh en e xa mi n in g the l ym ph n o de s o f th e h ea d an d n ec k (Fi g. 7. 1), ea c h o f th e ma in gro u p s

mu s t be pa lp a te d in tu rn , u s in g a sy ste ma tic a p pro ach . F ro m a po s iti o n be h in d the pa tie n t,

th e p re-au ri cu l ar, p a ro ti d, f ac i al , su b ma n di bu l ar, s u bm e nta l, d ee p, ce rvi c al (u pp er , mi d, a n d

lo w er), s u p rac la vi cu l ar , po s teri or tria n gl e, a n d o cc ip ita l gro u p s of n o de s ar e p al pa ted i n tu rn on ea ch si de . Wh en e xa mi n in g th e ce rvi c al n o de s it i s h elp fu l to re la x th e su rr ou n d in g tis su e

by be nd in g th e pa tie n t's h ea d fo rw ard a n d la tera ll y to w a rds th e si de e xa mi ne d. If a p al pa bl e no d e is fo u n d, i ts textu re i s no te d a n d it i s mo v ed be tw ee n tw o fin g ers to di sc o ve r a n y atta ch m en t to s ki n o r u n d erl yin g tis s u e.

In ad di tio n to a d eta il ed ex tra - an d i ntra o ra l exa min a ti o n , the e xa mi n er s h o u ld ch e ck th e

ski n o f the s c al p, fa c e, a n d n ec k. P la in rad io g rap hs o f th e h ard tis su e s ma y be re qu ire d to ide n tify a n y o d on to g en ic i n fla m ma tio n re la ted to the pa tie n t's de n ti tio n . S ec o n da ry

ne o pl as m s in b o ne u s u al ly pre se n t a s an il l -def in e d ra d io lu c en c y. If th ere a re no fo c i o f

in fe cti on o r ev id en c e o f mu c o s al le si o n s res po n s ib le fo r th e c erv ic al l ym ph a de no p ath y , the n

th e p ati en t sh o u l d b e ref erre d to a ma xi llo f ac ia l (o r E NT ) s u rge o n fo r f u rthe r e xa mi n ati o n o f th e e ar, n o s e, a n d thr oa t. F le xib le n a so e n do s co p y or rig id o es o ph a go l ary n go s co p y m a y b e

req ui red , to ge th er wi th s pec i al iz ed i ma gi n g tec h n ique s su c h a s M RI a n d u l tras o n o gra ph y (s ee Ch ap ter 2 ).

Inflammatory causes Acute infections

Lymp h ad en i tis a ris in g fro m a n a cu te in f ec tio n s u ch as a p er ia pi ca l ab sc e ss o r a p er ic o ro ni tis

is u su a ll y u n i la tera l. T he a pp ea ra n ce o f th e n o de s is ra pi d, a n d the y a re s o ft a n d a re p ai nf u l wh e n to uc h ed . Th e re ma y be o ed em a o f th e s o ft tis s ue s u rro u n di n g th e n o de s gi vin g th e

vis u al i mp res s io n o f g rea ter en l arg em en t th a n i s, in fa c t, th e c as e . Th e fa ci al ly mp h n o de , lyi n g ju st an ter io r to th e a n te ri or bo rd er o f m a ss ete r a t th e le vel o f th e o c cl u sa l pl an e , is co m mo n ly i nv o lve d in c h il dre n .

Chronic lymphadenitis

In ch ro n ic i n fec tio n s th e af fe cte d n o d es a re fir m bu t m ay n o t be te nd er . In tu ber cu l os i s th e in vo lv ed n o de s bec o m e a tta ch e d to th e s kin an d th is p ro du c es th e so - ca ll ed β€ co ll a r s tud

ab sc es s β€™. In l o n gs tan d in g ca s es o f ch ro n i c in fe cti o n, c a lc if ic ati o n o f a n o de ma y pre se n t as a s o li ta ry h ard , n o n fix ed s w ell in g . Mo re w id es p rea d sm a lle r ca l ci fic a tio n s in th e c erv ic al no d es a re mo re c o mm o n . Th ey a re us u a lly di sc o ve red i nc i de nta ll y d u rin g ra di o gra ph y , are

as ym pto m ati c, a n d ca n n o t be pa lp a te d. W id es pre a d l ym ph n o d e e n la rge me n t m ay be th e fi rst cl in ic a l s ig n o f in fe c ti o n w ith th e HIV vi ru s β €”th e s u bm an d ib ul a r n o de s are o fte n p ro mi ne n tly aff ec ted (Ch ap ter 4).

P. 79

Neoplastic causes of lymph node enlargement Lymphoma

Th e l ym ph n o d es o f the n e ck ar e o f ten in vo l ve d e ar ly in l ym ph o ma s , bo th o f th e H o dg kin 's an d n o n- Ho dg kin ' s ty pe s. A ffe cte d no d es a re in i ti ally di s cre te, ru b be ry in c o n si ste n cy , an d

pa in le ss . Dia gn o s is i s by h is to pa tho l o gi ca l exa m in atio n . B ur kitt's l ym ph o m a mo re c o mm o nl y

ari se s as a c e ntra l le si o n o f th e ja w s, a lth o u gh the c erv ic al l ym ph n o de s ma y be af fe cte d f irs t in a fe w pa tie n ts.

Fig. 7 .1 P rin c ip al gr ou p s o f l ym ph n o d es in the h e ad a n d n ec k.

Leukaemia

Th e o ro f ac ia l ma n if es tati o ns o f le u ka em ia ar e f u lly d is cu s se d in Ch ap ter 13. Pa tie n ts wi th a n

un d erl yin g d ia gn o s is o f leu k ae mi a ra rel y p res en t with a n ec k m a ss . It i s, h o we ve r, im po rta n t to ap pre c ia te th a t de n tal in f ec tio n s a nd n o n -od o n tog en i c in fe c ti o n s co m mo n l y o c cu r in

leu k ae mi c pa tie n ts a n d gi ve ris e to ei th er f ac i al sw e ll in g an d /o r c erv ic a l l ym ph a de no p a th y .

Secondary neoplasia

Sp rea d to ly mp h n o de s o f th e n ec k ma y o cc u r a t a n y s ta ge in th e gr ow th o f a ma l ign a n t

ne o pl as m o f th e o ra l c a vity , ph a ryn x, an tru m , o r a dja c en t s tru ctu re s. T h e n o d es a re in iti al ly pa in le ss , h a rd, an d u s u al ly u ni la tera l. Th e p re se n ce o f pa in le s s e n la rg ed ly mp h n o de s te n ds to be m o re su g ge stiv e of a p ath o lo g ic al p ro ce s s othe r th a n in fe cti o n. β € Tro is ie r's si gn β€™

is th e p re se n ce o f a lef t s up ra cl av ic u la r n o d e i n th e p re se n ce o f ga s tric c an c er β €”so -ca ll ed bec a u se a s a ph ys ic i an i t w a s th e fi rs t si gn th a t ca u s ed h im to s u sp ec t h i s ow n g a stri c ca rc in o ma .

Discussion of problem case Case 7.1 Discussion Q1

Wh at o th e r qu e sti o ns w o u ld y ou as k th i s ge n tl em a n?

Pe rs is ten t h o ar se n es s is h ig h ly s ug ge sti ve o f m a li gn a nt di se as e o f th e la ryn x a nd m u st be exc lu d ed , as a m a tte r o f u rge n cy . Th e h ard , fi xed ju gu lo -om o h yo id l ym ph n o de i s a

pa rtic u la rly w o rryi ng f ea tu re a n d su g ge sti ve o f me tas ta tic s pre ad . Th e pa tie n t s ho u l d b e sp ec if ic al ly as ke d ab o u t o the r sy mp to ms , s u ch a s d iff ic u lty sw a ll ow i n g (d ys ph a gi a ) o r

bre ath in g (d ys pn o ea ), d ry co u g h , ea ra ch e , h ae mo p tysis (c o u gh in g u p bl oo d ), a n d w e ig ht

lo ss . Q1

Ho w w o ul d yo u m an a ge th is ca s e?

It is im p or ta n t to e xa mi n e th e m o uth an d o ro p ha ry nx fo r o bvi o u s mu c o sa l le si o ns . Th e

pa tien t sh o u ld b e i mm ed ia tel y ref erre d fo r f u rthe r i n ves tig a tio n b y a n E NT or h ea d an d n ec k su rg eo n . A d ire ct ref erra l c an b e fa xed to th e h o spita l un i t c on c er ne d o r a rra ng ed b y th e

pa tien t's d o cto r, w h o s h o u ld a ls o be tel ep h o ne d. It is e ss en ti al to c h ec k th a t yo u r re fe rra l ha s be en rec ei ve d a s a dm in is tra tive e rro rs a re al ways po s s ibl e.

Sq u am o u s ce ll c arc in o m a o f th e la ryn x is mu l tifa c tor ia l b u t c o ns u m ptio n o f to ba c co a n d

al co h o l are re co g n iz ed a s ris k fa cto rs . In th e E ur opea n U n io n , c arc i no m a o f th e la ryn x is i n th e to p 10

mo s t c om m o n ma li gn a n ci es f o r m al es . O th e r c a us e s of p ers is ten t h o a rs en es s in c lu d e ch ro n ic la ryn gi tis d ue to i n fla m ma to ry c o n di tio n s o r v oc a l fati gu e (o ve ru se ), vo c a l c o rd pa ra lys i s, an d , les s c o mm o n ly, s yp h il is , tu be rcu l o si s, h y po th yro i dis m , my ae s th e n ia g rav is , an d fu n cti on a l dys p h as ia . B en i gn tu m o ur s rare ly c au s e ho ars en e ss .

Project 1.

Ma gn e tic re so n a n ce im a gi ng (M RI) a n d ul tas o n o gra phy a re tec h ni qu e s tha t c a n be u s ed

to in ve sti ga te so f t-ti ss u e sw e ll in gs i n th e he a d a nd n ec k. Wh a t a re th e i nd ic a tio n s, co m pa ra tive a dv an ta ge s, a n d di sa dv an ta ge s o f th es e tw o tec h n iq ue s ?

P. 80

Au th o rs : Title:



Co py rig ht Β © 2003 Ox fo rd U ni ve rsi ty P re ss > T ab le o f Co nt e nt s > 8 - Sa liv a ry g land s a nd s aliv a

8 Salivary glands and saliva Problem cases Case 8.1

A 48-yea r-ol d ma le a tten ds f o r a 6 -mo n th rev ie w ap po i n tm e nt at yo u r p rac tic e. On u p da tin g

hi s me di ca l h is to ry y o u le arn th at h e i s ab o ut to sta rt a c o u rs e o f ex tern a l b ea m ra di o th e ra py fo r to n si ll ar ca rc in o m a. Q1

Wh at a d vic e w o ul d yo u g ive to th i s pa tien t co n c ern i ng h is f u tur e o ra l h ea lth ?

Case 8.2

A 55-yea ro ld f em al e p re se n ts to yo u r p rac tic e fo r rou ti n e re vi ew . S h e rep o rts xe ro sto m ia . O n exa m in ati o n yo u n o tic e th at s h e h as a d ry or al m uc os a. New ca rio u s l es io n s , o n th e p ro xi ma l su rfa c es o f pre vi o us ly s o u nd l o we r in c is o rs , ha ve dev elo p ed s in c e he r l as t v is it. Q1

Wh at q u es tio n s w o u ld yo u a s k th is l ad y?

Q2

Wh at a d vic e w o ul d yo u gi ve ?

Q3

Wh at i s th e m o s t fre qu e nt ca u se o f xe ro sto m ia ?

Case 8.3

A 45-yea r-ol d ma le p ati en t p res en ts to y ou r pra c tice an d c om p la in s th at ov er the p as t m o n th he h a s be en e xp eri en c in g pa in an d s w el lin g u n de r h is to n g ue a n d lo w er jaw β€”on th e l eft si de . Th e pa in a n d sw e ll in g is w o rs e ju s t b efo r e e ati n g a n d im pro v es c o ns i de rab ly a fter a me al . Q1

Wh at f u rth er qu e sti o ns w o u ld y ou as k th i s pa tie n t?

Q2

Cli n ic al ly h ow wo u l d y o u ex am in e th is p ati en t?

Q3

Ho w w o ul d yo u m an a ge h im ?

Q4

Is th i s pa tie nt lik el y to c o m pla i n o f a dry m o uth?

Saliva and the salivary glands

A nu m be r o f c on d iti on s a ff ec tin g the sa l iva ry gl an ds are m an a ge d su rg ic a ll y. Th es e in c lu d e cys ts a n d n e o pl as ms ar is in g in th e gl a nd s a nd a ls o c o n diti o n s ari si n g a s a re su l t o f th e pre se n ce o f ca l cu l i o r oth e r o b stru c tio n s in th e g la nd s o r d u cts . Ho w ev er, w i th th e

eli mi n ati o n o f th e se tw o gr ou p s o f i mp o rtan t s a li vary g la n d di se as es th e re rem a in s a n u mb er of c o nd iti o ns th a t s h ou l d be c on s id ere d w ith in th e s co p e o f o ral m ed ic in e . Th is c h ap ter w ill

co n s id er s i al ad en iti s, si al o si s, n e cr o ti z in g si a lo me ta pla s ia , a nd d is tu rba n ce s in s a li va ry f lo w

rate . Th e cl in ic i an s h o u ld be co g n iz a n t w ith th e fun cti o ns o f s al iva a n d the a n ato m y o f th e

sa li va ry g la n ds . Th is i n fo rm ati on is h el pf ul i n u nder sta n di ng th e n atu re o f th e i nv es tig ati o ns

req ui red to s tu dy s al iva ry gl an d d is ea s e, th ei r e n su in g s eq u el ae , an d th e th era pi es a va il ab le .

Saliva

Sa li va is a g la n du l ar se c retio n th a t i s es se n tia l fo r th e ma in te na n c e o f h ea lth y o ro de nta l

ti ss u es . S a li va is a c o mp le x f lu id a n d ma n y o f th e f u nc tio n s o f s a li va h av e a p ro tec tiv e r ol e,

so m e o f w h ic h a re s h o w n in Tab le 8. 1. Th e p h ys ic a l p ro pe rtie s o f s a liv a va ry a c co rd in g to th e dif fe ren t typ es o f s al iva ry gl an d s, wi th pa ro tid s ec re ti o n s ha vi n g a se ro u s (w ate ry)

co n s is ten c y. Th e s ub m an d ibu l ar an d s u bl in gu a l gl an ds se cre te a mo re vi sc o u s sa li va d ue to th e ir hi gh e r g lyc o pr ote in c o n ten t. A s ev ere re du c tio n i n sa l iva ry flo w r ate c an h a ve

dev as ta tin g co n s eq u en c es o n o ra l he a lth a n d, su b s equ e n tly, o n th e p sy ch o lo g ic al a n d so c ia l we ll -bei n g of th e su f fer er.

Table 8.1 Functions of saliva Fu n ctio n

Descr ipt ion

Lub ric a n t

Co ats a n d pro tec ts th e mu c o sa a ga in s t m ec h an i ca l , th erm al ,

Cle an s es th e

Cle ars f o o d f ro m th e o ra l ca vi ty a n d o ral m u co s a

Io n res e rvo ir

Fa c ili tate s rem in e ral iz a tio n o f the tee th

Bu f fer

Neu tra li ze s pl aq u e pH af ter e a ti n g

An tim ic ro b ia l

Se cr eto ry im mu n o g lo bu l in s , en z ym es , a n d o th er s al iva ry

Pe ll ic le

A pro tec tiv e la yer o f s a li va ry p ro tei n th at f o rms ov er en a me l

Dige s ti o n

Sa li va ry am yl as e in iti ate s th e d ig es tio n o f s ta rc h

Fa c ili tate s

Sa li va is a s o lv en t a n d the ref o re al lo w s th e i n teracti o n o f

Wate r b al an c e

Deh yd rati o n c au s es a red u cti o n in s a li va ry f lo w ra te wi th a n

te eth

fo rma tio n

ta s te

an d ch e mi c al irr ita ti o n

pro tei n s h elp re gu l ate th e o ra l fl or a

ac ts as a d if fu si o n ba rri er

fo o ds tu ff w i th tas te bu d s

as so c ia te d o ra l dry n es s; th is s h ou l d sti mu la te a n ee d to in cr ea se fl u id in ta ke

P. 84

Sa li va is i mp o rta nt fo r th e ma in te n an c e of o ra l h e al th .

A dim i nu ti on o f sa li va ry f lo w c an h av e a d etri me n tal im pa c t o n th e qu a lity o f lif e o f a pa tien t.

Th e c o m po s itio n o f s al iva i s af fe cte d b y a nu m be r of fa cto rs , i nc lu d in g th e ty pe o f sa li va ry gla n d. Th e ma jo ri ty o f am yl as e is p ro du c ed by th e pa ro tid gl a nd s bu t th e b lo o d gro u p

su b sta n ce s ar e s ec re ted m ai nl y by the m in o r m u c ou s g la n ds . Th e u n sti mu la te d f lo w ra te i s

mo re im po rta n t th a n the s tim u la ted fl o w ra te f or o ra l c o mf o rt. Ho w ev er, th e s tim u la ted fl o w

rate is i mp o rtan t to fa ci li tate c he w in g an d s w al lo win g du ri n g m a sti ca tio n . Th e s u bm an d ib ul a r gla n d co n tri bu tes a pp ro xi ma tel y 65 pe r ce n t o f th e re sti n g w h o le s al iv ary fl o w ra te. O n ly 15 β €“20 pe r c en t i s de riv ed fro m th e pa ro tid , w ith th e su b lin g u al a n d m in o r gl an d s bo th del iv eri n g 7 β €“8 pe r ce n t. In c on tra s t, the p ar oti d pro vi de s 45β €“50 pe r c en t o f th e

stim u la ted w h o le sa l iva ry flo w r ate . S al iva ry flo w rate s exh i bi t di u rn al va ri ati on wi th a n

ave ra ge u n sti mu l ate d f lo w ra te thr o ug h o ut the d ay of ap pro x im ate ly 0.3 ml /m in u te β€” in

sl ee p th i s ma y f al l to 0.1 ml /mi n u te. M an y tex tbo o ks m en tio n th a t ap pr ox im ate ly 1500 m l o f

sa li va is p ro du c ed e ac h d ay . Mo re re c en tly , ho w e ver, it h as b ee n s ug ge s te d th at th i s fig u re is an o v ere sti ma tio n o f to ta l da il y sa li va ry fl ow rate, an d a d ai ly flo w rate o f 500 β€“ 600 ml /da y ma y b e a mo re re al is tic es tim a te . Th e br ea kdo w n o f s al iva ry fl ow ov er a 24-ho u r pe rio d is sh o w n in Tab le 8. 2 .

The salivary glands

Th e p a ro ti d gl an d s are th e la rge s t sa l iva ry gl an d s. Th ey a re we dg e -sh a pe d a n d si tu ate d i n

fro n t o f th e ea r an d be h in d th e r am u s of th e m a nd ible . Th e ap ex o f th e w ed ge i s th e d ee pe st pa rt o f th e g la n d. Th e pe rip h era l bra n c he s o f th e fa c ia l n e rve (CN V II) a re in tim ate ly

as so c ia te d w i th th e p a ro ti d gl an d . Th is re la tio n s hip is i na d verte n tly de mo n s trate d wh e n a n

in fe rio r d en ta l ne rve a n ae sth e tic b lo ck i s ad mi n is tere d i n co rr ec tly, a n d ca u s es a tem po ra ry dro o pi n g of th e up pe r e ye li d.

Pa ro tid s al iv a is tra ns fe rre d a lo n g th e pa ro tid du ct i nto th e o ra l c a vi ty . Th e th ic k -wa ll ed

pa ro tid du c t (S ten s o n 's du c t) e me rge s a t th e a n terio r b or de r o f th e p aro ti d gl an d a n d r un s ov er the s u rfa ce o f th e m a ss ete r b ef or e h o o ki n g m edia ll y ov er the an te rio r mu s cl e bo rd er.

Th e o ri fi ce o f th e d uc t i s co v ere d by a sm a ll fla p o f mu c o s a c a ll ed th e p a ro tid pa pi ll a an d th is is s itu ate d op po s ite th e ma xi ll ary se c o nd p erm a ne n t m ol a r.

Th e tw o s u bm a nd ib u la r g la n ds a re ap pr ox im ate ly h al f th e s iz e o f th e pa ro tid s. Th e s u pe rfi c ia l pa rt o f th e su b ma n di bu l ar gl an d is w e dg ed be tw ee n th e b o dy o f th e m an d ibl e an d th e

myl o h yo id mu s c le , wi th th e s m al le r d ee p p a rt h o o king a ro u nd th e po s teri o r b or de r o f th e mu s cl e to lie o n th e fl o or o f th e m ou th β€” ab o ve the m yl oh y o id. Th e s u bm a n dib u la r

(Wh ar to n 's ) du c t ru n s fo rw ar d, a lo n g th e f lo o r o f th e m o u th to o pe n in to th e su b li gu a l

pa pi lla , ju s t la ter al to th e lin g u al fre n u m. T he s ec re tio n s ar e a mi xtu re o f s e ro us a n d mu c o u s flu i ds .

Th e s u bl in g ua l gl an d s ar e th e s m al le st of th e th re e pa irs o f s al iva ry gl an d s an d a re lo c ate d jus t b el o w th e f lo o r o f th e m o u th be n ea th th e s u bl in g u al fo l ds o f mu c o us m em b ran e . Th er e are n u me ro u s su b li ng u al d u cts th a t o pe n in to th e mou th a lo n g th e s u bl in g ua l fo ld s . Th e se cre tio n s o f th e se g la n ds a re pre do m in a n tl y mu c o us.

Table 8.2 Salivary flow over a 24-hour period Sl ee p

40 ml s al iv a wi ll be p ro du c ed o ve r 7 h o u rs

Aw a ke

300 ml o f un s tim u la ted sa l iva o ve r 16 h ou rs

200 ml o f sti mu la te d s a liv a du ri n g me a ls β €”ov er 54 mi n u te s

Th e m in o r sa li va ry gla n ds c o n s is t o f n u m ero u s s ma ll m u co s al g la n ds si tu ate d on th e to n gu e , pa la te, b u cc a l a n d la bi al m uc o s a. T h ey pro d uc e pr imari ly a mu c o u s se cr etio n .

Assessment of the salivary glands Examination

Th e p a ro ti d gl an d s, l yi ng p ar ti al ly co n c ea le d by the a s ce n di n g ra m u s of th e ma n di bl e, a re n ot pa rtic u la rly ea s y to pa lp a te . Te n de rn es s an d s we ll in g a re b es t d ete cte d b y sta n di n g i n fro n t

of th e pa tie n t an d b y p la c in g two or th ree fi n ge rs o ver th e p o ste rio r bo rd er o f th e as c en d in g ram u s o f th e ma n di bl e. B a ck w ard s a nd i n wa rd s mo ve men t o f th e fi n ge rs w ith l igh t pre ss u re

is a lm os t a lw a ys a ll th at is n ee de d to de tec t ten d ern e ss i n th e s u pe rfi ci al p art o f th e pa ro tid . Th is m an o eu v re is n ec es s ar y to di ffe ren ti ate pa ro tid te n der ne s s fro m th at of th e

te mp o ro m an d ibu l ar jo in t o r th e ma s se ter w ith w h ic h it is o fte n co n f us e d. It m u st al so be rem em be red th a t th e pa in f ul s ig n s res u ltin g fr om temp o ro ma n di bu l ar jo in t/m us c le

dys fu n c tio n m ay ex ten d to th e u p pe r p ol e o f th e pa ro tid w ith w h i ch th e jo in t i s in c lo s e an a tom ic a l rel ati on s h ip . S we ll in g o f a p ar o ti d gl and ma y al s o be vi su a li z ed by sta n di n g

beh i n d th e pa tie n t w h o is s ea ted i n a rec l in ed d en tal c h ai r. Wh e n ex am in i ng a p aro ti d gla n d

th e d uc t p ap il la m u st al so b e ex am in e d i n trao ra ll y fo r si gn s o f in fl am m ato ry c ha n ge . P ar oti d

sa li va c an b e vis u a li ze d by li gh tly co m pr es si n g th e s kin o verl yi n g th e du c t w ith th e fi n ge rs. If th e c h ee k i s he ld retra c ted , th e s a liv a ex pre ss ed by th i s ma n o eu vre w il l be s een co u rs in g do w n wa rd s o ver th e b u cc a l m u c os a fr om th e du c t p ap il la . It is h e lp fu l fo r th e cl in i ci an to kn o w tha t p a ro ti d sa li va c an exp re ss ed , bu t th i s meth od i s no t of va lu e in q u an ti tati vel y as se s si n g th e pa ro tid f lo w ra te o f s al iv a.

Th e s u bm a n dib u la r g la n d ma y b e fe lt b el ow th e a n gl e a n d bo d y o f the ma n di bl e, th i s si mp le pa lp ati on be in g rei n fo rc ed by bi m an u a l p al pa tio n w ith a fi ng er in th e fl o or o f th e m o uth ,

gen tl e p re ss u re be in g ex erted b etw ee n th e ex am in i ng h a n d ( be lo w th e ma n di bl e) a n d th e

fin g er. A s in the c a se o f th e pa ro tid g la n d, th e sub ma n di bu l ar (Wh a rton ' s) du c t s ho u l d b e

ob s erve d fo r s i gn s o f i n fla m ma tio n a n d a su b jec tive a s se ss m en t m ad e o f th e qu a li ty o f the sa li va . It is s im il a rly di ffi cu l t to s pe ci fy the β € no rm a lβ €™ pa lp ati o n fea tu res o f th e

su b ma n di bu la r g la n d. In so m e e n tire ly

no rm a l i n di vid u al s it i s po s s ibl e to pa lp ate th e glan d , w hi le in o th e rs it is n o t.

Measurement of salivary flow Sialometry

Sa li va ry flo w ra te is m ea s ur ed by si a lo me try an d eith er res tin g (u n sti mu l ate d/b as a l) o r

P. 85

stim u la ted s a liv a ma y be co l le cte d. W ho l e s a li va , rep res e nti n g c o n trib u tio n s fro m a ll th e

sa li va ry g la n ds o r s ec re ti o n s fro m in d ivi du a l ma jo r g la n ds , c an b e me as u re d. F o r e xa mp le , a

Car ls o nβ €“Critte n den co l le cto r ( a sm a ll cu p pl a ce d ov er the o ri fic e o f th e pa ro tid g la n d d u ct) ca n be u s ed to c o ll ec t s al iva f ro m the p ar oti d gla nd. Th e pa ro tid o r s u bm a n di bu la r d u cts c a n

al so b e ca n n u la ted to m ea su re f lo w ra tes . Th es e tech n iq ue s ar e r ela tiv el y i n va si ve an d m ak e me as u rem en t of u n sti mu l ate d fl ow rate s u nr eli a ble for in d ivi du a l gl an d s. S tim u la ted w h o le

sa li va c an b e co l le cte d an d me a su re d by a va rie ty of vo lu m etri c an d g rav im etri c tec h ni qu e s. Si al o me try s h o u ld be m ea s ur ed u n de r s tan d ard iz e d c on di tio n s . Co n si de ra ti o n m us t be g ive n to the tim e o f d a y, th e ty pe o f sti mu la n t u s ed , an d th e pre -co ll ec tio n in s tru cti o ns , fo r

exa m ple , a sk in g th e p ati en t to r efr ai n fro m s mo k in g, d rin k in g, an d e ati ng f or a de fi ne d

per io d. D es pi te th e me a su re me n t o f w h o le s al iv ary fl o w ra tes be in g a re la tive ly s im ple a n d

no n -in va si ve a pp ro ac h , it is n o t u su a ll y u n de rtak en i n ge n era l de nta l pra c tic e. S ia lo m etry i s

pro b ab ly mo s t b en ef ic ia l fo r th e lo n g itu di n al a ss e ss me n t o f flo w ra tes f o r i nd iv id ua l pa tie n ts as th er e is c o n si de rab le v ari ati o n w ith in th e po p u la tio n .

A wi de ra n ge o f f lo w ra tes h a ve be en q u o ted fo r bo th b as a l a n d sti mu la ted s a liv a in th e

gen e ra l p o pu la tio n , b u t it ha s b een sh o w n th at ch a nge s in s a liv ar y f lo w a re pro b ab ly m o re im po rta nt in di c ato rs o f sa li va ry fu n cti on tha n a s in g le fl o w rate me a su re me n t. Vo l u nte ers

giv en a tro pi n e rep o rted th at a d ry mo u th d eve lo p ed w h en th eir re sti ng f lo w fe ll to a bo u t 50 per c en t o f the ir n o rma l flo w r ate . Th e n o rma l rate o f flo w o f u n sti mu l ate d w h o le s a liv a is

ap pro xi m ate ly 0.3 ml /mi n u te an d fo r sti mu l ate d wh o le s a liv a 1β €“2 ml /m in u te. S tim u la ted

sa li va is m a in ly se c rete d i n re sp o n se to m as tic a tory a n d gu s tato ry sti mu l i a n d the f lo w ra te wi ll ris e si gn i fic a n tly (4β€“ 6 ml /m in u te) wh e n c he win g a po w er fu l si a lo go g u e.

Salivary gland imaging Plain radiography

Cal c ul i ca n s o me tim es b e s e en o n p la in f il ms , bu t it s h o u ld be re me m be red th at no t al l

ca lc u li a re rad io -op a qu e. W h en in ve s ti ga tin g a s u spec ted s al iv ary c al cu l us , tw o v iew s s h o ul d

be tak en a t 90Β °. Fo r th e p a ro ti d gl an d , a pa n o ram ic o r o bl iqu e l ate ral vi ew c a n be c o mb in e d wi th ro tate d an ter io rβ€“ po s te rio r o r p o ste rio rβ €“anter io r v ie w. F o r th e s u bm an d ib ul ar gl an d , pa n or am ic a n d lo w er oc c lu s al vi ew s (tru e a n d o b li que) a re a p pro p ria te. In th e i n tere st of

rad ia tio n d o se re du c tio n , it is a dvi s ab le to ta ke a se cti o na l pa n o ra mi c fil m wh e re po s si bl e fo r th e se p ati en ts , li mi te d ju st to the sy mp to ma tic s ide.

Sialography

Si al o gra ph y is a n i ma gi n g te ch n i qu e u se d to de mo n s tra te th e du c tal s ys tem o f the p ar oti d or

su b ma n di bu la r g la n d. A w ate r -so lu b le ra di o -op a qu e co n tra st me di u m i s in je cte d in to th e d u ct or ifi ce a n d β € po s t co n tra s tβ€™ rad io g rap h s ar e th e n tak en i n tw o di ffe ren t pla n es .

Fo ll o w in g the re mo v al o f the c an n u la , a f in al β € dra in a ge β€™ fil m is u s ua l ly tak en to a s se s s

th e c le ara n c e o f th e c o n tra st m e diu m . Th i s giv es s ome i de a of th e g la n d fu n cti o n an d w il l

of ten h i gh li gh t a n o b stru c tio n w ith i n th e d u cta l syste m. Tra diti o n al ly, s i al o gra ph y w as c a rrie d ou t u s in g a n o il -ba se d co n tra st me di u m, b u t th is c ou ld ca u s e p ro bl em s if ex trav as a ted

ou ts id e th e d uc ta l s ys tem an d i t co u l d a l so d a ma ge th e gl an d in p a tien ts w ith l ittl e o r n o sa li va ry f lo w ra te. Wa ter -so lu b le , pre fe rab ly n o n -io n ic , c on tra s t me di a ar e n o w u s ed ro uti n ely in m o s t ce n tres .

Si al o gra ph y de m on s tra tes a m ajo r sa li va ry gla n d in th re e ph a se s :

pre op er ati vel y

th e fi ll in g ph a se

th e e mp tyin g ph a se

Si al o gra ph y is h e lp fu l i n de mo n s tra ti n g stru c tu ral a bn o rm al iti es o f th e d uc t s ys tem .

Stri ctu re s o f th e su b ma n di bu l ar gl an d a n d d u ct dil ata tio n s a re cl ea rl y s ee n . Wh en th e re is

atro p hy o f th e s al iva ry ac i ni , a n ex tr av as a ti o n o f c o n tra st m e diu m w il l be ev id en t i n th e b o dy of th e gla n d (s ia le cta s is ). In S jΓ¶g re n' s sy nd ro m e th e re is s o me ti m es a ch a ra cte ris tic β€ sn o w sto rm β€™ ap pe ara n c e ( pu n cta te si al ec ta si s) .

Si al o gra ph y s ho u l d n o t b e u nd er ta ke n in the p res en ce o f a c ute i nf ec tio n o r w h en a c a lc u lu s is

kn o wn to be c lo s e to th e d uc t o p en in g . Th er e i s a ris k th at th e c o ntra s t m ed iu m ma y di sp la c e th e c al cu l u s fu rth er in to th e du c t.

Su s pe cte d β€ ma ss β€™ les i on s w i th i n th e s a li va ry g la n ds sh o u l d no t b e in ve sti ga ted w ith si al o gra ph y as s u c h le si o n s ca n n ot be s ee n di rec tly. Dis pl ac e me n t o f th e du c tal s ys tem ,

giv in g th e β € ba ll in h a n d β€™ ap pe a ran c e, m a y s u gg es t th e pre s en ce o f s uc h a l es io n , b ut

th i s pa ttern i s n o t al w ay s se en a n d ma y res u lt i n le s io n s be in g mi s se d. P a tien ts w ith dis c rete ma ss e s wi th in th e sa li va ry gl an d s sh o u ld b e s en t for u ltr as o u nd a n d po s si bl y a ma g ne tic res o n an c e i m ag in g (M RI) s c an .

Scintigraphy (radioisotope imaging)

Sa li va ry gla n ds , in c o m mo n w ith th e th yro i d, h a ve th e a b ili ty to co n c en tra te c er ta i n

rad io i so to pe s . In sa li va ry sc in ti sc a nn i n g l ab el le d te c hn e tiu m pe rtec h n eta te ( 9 9 Tc m ) is

freq u en tly u s ed be c au s e i t h a s a sh o rte r h al f-lif e th a n io d in e . An in tra ven o u s in je cti o n o f th e rad io i so to pe i s fo ll o we d by s ca n n in g o f th e sa l iva ry gl an d s at in terv al s o f 30 s e co n d s.

Sa li va ry gla n d fu n c ti o n is th e n as s es s ed w ith c o mp uter -as si s te d qu a n tita ti ve pr og ra mm es . Th e ra te o f co n c en tra tio n o f th e i so to p e i n th e gl an d s is pl o tted an d , a fter 30 mi n u te s ,

sa li va ry s e cre to ry a c tivi ty i s sti mu l ate d b y dro p ping c itri c ac id so l u tio n o n th e ton g u e. Th e res u ltin g a cti vity o f th e g la n ds is a g ai n fo ll o w ed by s ca n n in g at in terv al s o f

30 se c on d s. Tim eβ €“ac tivi ty pro f ile s o f th e g la nd s a re th e reb y p ro du c ed a n d ca n b ui ld u p a

P. 86

fu ll pi ctu re o f sa l iva ry se c reto ry ac tiv ity ( Fi gs 8. 1 an d 8. 2). Th is p ro ce du re m ea s ur es g la nd

up tak e of th e rad io i so to pe an d g ive s an in di c ati on of i ts c l ea ran c e. Th e c li n ic ia n c an vis u a liz e an d co m pa re th e ac tiv ity of a ll th e m a jo r s al iv ary gl a nd s . Sc i nti gra ph y is th e ref or e be n efi c ia l

fo r c o mp ar in g the f un c tio n o f a di se a se d g la n d (a s in a l oc a li ze d ch ro n i c si al ad en i tis ) w ith th e rem ai n in g h ea lth y gla n ds o r to de tec t a ge n era li z ed lo s s o f g la n du l ar fu n cti o n (a s se en i n

SjΓ ¶g re n' s sy nd ro m e). S ci n ti gra p h y p ro vid es n o i n form ati o n ab o u t sa l iva ry gl an d a na to m y. Sc in ti gra ph y is u s ed to a ss e ss s a liv ar y g la n d f u nc tio n in s al iv ary g la nd di se a se s su c h a s SjΓ ¶g re n' s sy nd ro m e.

Fig. 8 .1 Tim e β€“ac tivi ty c u rve o f n or ma l sa l iva ry gl an d du ri n g sc in ti sc an n i n g β€”se e te xt

fo r d eta il s of th e pro c ed u re. Pa rt a o f th e c u rve re pre se n ts the i n itia l is o top e u pta ke

an d pa rt b th e res po n s e to sti mu l u sβ €”th e w a sh -ou t e ff ec t. Pa rt c s h o w s co n ti nu i n g s lo w up tak e wh i ch r ea ch e s a ma xi mu m a n d th e n de cl in e s.

Fig. 8 .2 Th e co m pu te r s cr een du ri ng a sc i nti sc a n o f a p a ti en t w i th po o r up tak e an d

res po n s e to sti mu lu s o f th e le ft s u bm a nd ib u la r g la nd.

Ultrasonography

Ul tr as o u nd m a y b e u s ed f or su p erf ic ia l so f t-ti ss u e sw e ll in gs a n d the i n itia l in ve sti ga tio n o f

su s pe cte d ma s s le si o ns . U ltra s ou n d is p a rtic u la rly u se fu l fo r d if fer en tia tin g be tw een so l id a n d cys tic l es io n s b u t th e s o u n d w a ve s are b lo c ke d b y bo n e. U ltra s o un d m ay be u s ed in ca se s o f ch ro n ic i n fec tio n w h e re si al o gra ph y is c o n trai n di cated a n d ma y id en tif y th e pre s en c e o f si al ec tas is o r o f a c a lc u lu s, th o u g h su b tle c ha n ge s ar e d if fic u lt to se e.

Magnetic resonance imaging

MRI is u s ed to in v es tig ate s pa ce -oc c u pyi n g le si o n s su c h a s sa li va ry g la n d tum o u rs . Ho w ev er,

on l y l im ite d i n fo rm ati on is gi ve n o n ad ja ce n t h ard ti ss u es . Th i s te c hn i qu e pr ov id es g oo d s o ftti ss u e de tai l an d l oc a li za tio n of m as s es β €”th e fa c ia l ne rve is u s u a lly id en ti fia bl e. Im a gi ng te c hn i qu e s are d is cu s s ed mo re f ul ly in Ch ap ter 2 .

Table 8.3 Clinical conditions in which saliva may play a diagnostic role Mo n ito r ing

Co nd itio n

Bl o o d gro u p s

Us ef ul i n fo re ns ic m e dic i ne

Dru g l ev els

Lith iu m , me th ad o ne , di go xi n

Detec tio n o f dr ug s

Al co h o l, am ph e tam in e s, b en z o di az e pi ne s , o pi o ids

Ho rmo n e s

Co rtis o l, te sto s tero n e

An tib o dy de tec tio n

HIV in fe c ti o n , me a sl es , mu m ps , ru be ll a

P. 87

Sialochemistry

Th e m ea s u rem en t o f th e b io c h em ic a l co n s titu en ts o f sa li va h a s be en u n d erta ken fo r m a ny

dis e as es o f th e s a li va ry gl an d s; h o we ve r th e res u lts a re fre qu en tl y m is le a din g o r d if fic u lt to in terp ret. S ia lo c he m is try i s n o t ro u tin el y u s ed fo r di ag n o sti c pu rp os e s bu t i t rem a in s a

po ten tia l ly us e fu l res ea rc h to o l. It c a n al so be o f v al u e in m ea s uri n g dru g , an ti bo d y, a nd

ho rm o n e lev el s. Tab le 8. 3 id en tif ie s so m e co n d itio ns i n w hi c h th e u s e o f s al iv a ca n c o n tr ibu te to the di ag n o si s.

Biopsy

Op en b io p sy o f a m a jo r s al iv ary gl a nd c a n be h az a rdou s a n d is b es t av o id ed . Pa ro tid g la n d

bio p sy c ar rie s a ris k o f fa c ia l s c arr in g, d a ma ge to th e fa ci a l n e rve , or th e es tab li sh m en t o f a sa li va ry f is tu la . It is c o mm o n p rac tic e to a ss es s th e hi s to p ath o l og y of th e sa li va ry gl an d s b y

an i n trao ra l bi o ps y o f th e m in o r gl an d s, n o ta bl y the la bi al g la n ds . Fi n e ne ed le a sp ira tio n ( se e Ch ap ter 2) o f th e m ajo r sa li va ry gl an d s ma y b e u n dertak en .

Labial gland biopsy

Th is en ta il s ma ki n g a s m al l i n ci si o n in s id e the l o we r li p, th ro u gh no rm a l m u c os a , u n de r lo c a l an a es th es ia a n d h a rve s ti n g a nu m be r o f sm a ll m uc o s al gl a nd s fo r hi s to p ath o l og ic a l a n a lys is .

Th is b io ps y is p arti cu l arl y u se fu l in e sta b lis h in g a di a gn o si s o f S j Γ¶g re n' s sy nd ro m e, a n d th e

pa tho l o gi ca l ch a n ge s mi rro r th e fo c a l l ym ph o c ytic sia la de n iti s th a t o c cu rs i n th e ma jo r gl an d s in th is c o n di tio n . Th e pa tie nt sh o u ld a lw a ys b e w a rn ed o f the p o ss ib il ity o f l o ca li z ed li p

pa ras th es ia ( or a na e sth es i a) fo ll o wi n g bio p sy o f the l ab ia l gl an d s. T h is is a w e ll rec o gn i z ed po s to p era tiv e co m pl ic a tio n .

Salivary gland disease Sialadenitis

Si al ad en i tis is th e term u s ed to de sc ri be in f la mm ati o n o f s a li va ry g la n ds , m o st c o m mo n ly th e res u lt o f vi ral o r ba c teri al in f ec tio n , bu t o c c as ion a ll y d u e to oth e r c au s es ( fo r e xa mp le ,

al ler gi c rea c tio n s, irra di ati o n ). B ac teri a l s ia la d en iti s is u s ua l ly a se co n d ary co n s eq u en c e of eith e r a lo c a li ze d o r s ys tem ic c au s e o f re du c ed s aliv ary fl o w an d is ra re ly the p rim a ry

pa tho l o gi ca l pro c es s . Vi ra l i n fec tio n s , h o w eve r, fre qu e ntl y a ff ec t p revi o u sl y n o rm a l s a liv ar y gla n ds . Ge n era ll y s p ea kin g , si a la de ni tis i s a cl in ic a l p ro bl em a ff ec tin g the m aj or s al iva ry

gla n ds . Ho w e ver , it m a y a ls o o c c ur in m in o r sa li va ry gl an d s, e ith er a s a p ri ma ry ph en o m en o n (as in S j Γ¶g re n' s sy nd ro m e) or a s a s e co n d ary fe ature o f so m e oth e r c o n diti o n s uc h as p ip e-

sm o ker 's pa la te, i n w h ic h th e pa la tal s al iv ary gl a nd s o ften be co m e in fl am e d a n d en la rg ed (se e Ch ap ter 9).

Of th e v ira l in fe cti o ns o f th e s a li va ry g la n ds b y far th e m o s t co m m on is mu m ps , w h ic h m o st co m mo n ly a ffe c ts th e pa ro tid g la n ds b u t al so fro m tim e to tim e in vo l ve s th e o th er ma jo r gla n ds . Th e m u mp s viru s ( an R NA pa ra my xo vi ru s) is tra ns m itted b y d ire ct co n ta ct or by

dro p let in fe cti o n, an d a cl in i ca l in fe cti o n is h e ral de d by a fee li n g o f m al ai se a n d o f ten b y

ab do m in a l p ai n . Th e sa li va o f pa tie n ts dev el o pi ng mu mp s is i nf ec tio u s fo r se ve ral d ay s b ef o re pa ro titis de ve lo ps a n d fo r a pp ro xi ma tel y 2 w ee ks after the p res en ta tio n o f cl in i ca l sy mp to ms . Fo ll o w in g thi s , the re is s w el lin g o f o ne o r bo th paro tid g la n ds , so m eti me s as s o ci ate d wi th a

sw el li n g o f th e s u bm a n di bu la r g la n ds . In m an y c as es, o n e o f th e p a ro tid gl an d s is b y f ar the

mo re s eve rel y aff ec ted . Wi th the sw e ll in g o f th e glan d th ere i s pa in a n d ten de rn es s to ge th er wi th a ge n era l ma la i se a n d a f eve r. P a ti en ts o fte n h av e tri s mu s . Th e sw e lli n g of th e sa li va ry gla n d is a t its m a xim u m w ith in 1 o r 2 d ay s o f th e on se t o f s ym p to m s a nd i s fo ll o w ed by a gra du a l res o lu tio n o v er 1 o r 2 w e ek s. M u mp s o fte n occ u rs in mi no r ep id em ic fo r m a n d is us u a lly re co g n iz ed o n c li n ic al s ym pto ms a l on e . Ra rel y, co n f irm ati o n ma y be o bta in e d b y an tib o dy me a su re me n ts. T rea tme n t o f m u mp s is en tir el y s ym pto m ati c, w i th the u s e of

an tip yre tic s, si mp le a na l ges i cs , a mp le fl ui d in tak e, a n d res t. Is ol a ti o n is i mp o rtan t si nc e th e dis e as e is ve ry co n ta gi o us . M u mp s is a ls o d is c us s ed in Ch ap ter 4 .

A we ll -es tab li sh e d c o m pli c ati on of m u mp s is th e o n se t o f o rc h iti s o r o o ph o ri tis β€” oc c as io n a ll y ste ril ity m a y e n su e . Th es e co n d itio n s a re ca u s ed , not by di rec t v ira l i n fe cti on , b u t by th e

pro d uc tio n o f au to a n tibo d ie s di rec ted a ga in s t th e tis su e s o f th e rep ro du c tive o rg an s . A fte r a n atta ck o f m u m ps , im mu n i ty i s us u a lly c o mp le te a n d, th e ref or e, a re cu rre n t sw e ll in g o f th e

pa ro tid reg io n i s n o t li kel y to be du e to a re cu rren ce o f mu m ps . It s ho u l d b e rem em b ere d, ho w e ver, th a t, ra rel y, s a liv ary g la n d i n fec tio n s m ay b e ca u s ed by o th er vir us e s, i n cl u din g

cyto m eg a lo vi ru s an d Co xs a ck ie viru s es . Th e w id es pre ad u s e of i mm u ni z ati o n ag a in s t mu m ps wi ll re su lt in a f al l i n in c id en c e. Si al ad en i tis c an :

be ch ro n i c or a cu te

aff ec t th e mi n or o r ma jo r s a li va ry g la n ds

ha ve a s ys tem ic o r l o ca l ae tio l og y

ha ve a n in f ec tive o r n o n -in fe cti ve ae tio l og y

A red u cti on in s a liv ary g la n d fl ow rate , ei the r f rom a lo c al iz ed o r sy ste mi c ca u se , is a

pre di sp o si n g f ac to r in a c u te s ia la d en iti s. A s h a s be en p o in ted o u t, ba c teri al in f ec tio n o f the

sa li va ry g la n ds i s co m m on l y s ec o n da ry to a n o bs tru cti ve co n d itio n su c h a s ca l cu l us f o rma tio n or du c t s tric tu re; it i s th ere fo re a lo c al iz e d c o n di tio n . Th is b ei n g s o , it is e vid en t th a t a si ng le

gla n d is n o rm al ly af fe cte d. M os t o f te n th is i s a paro tid gl a nd . Th e red u ce d sa l iva ry flo w

P. 88

pre di sp o se s the gl an d to a n a sc en d in g in f ec tio n from b ac ter ia w ith in the o ra l ca vi ty. In th is co n di tio n th e gl an d is p a in fu l, te n de r, a nd s w o ll en, a nd p ai n is r ad ia ted to th e ea r a n d the te mp o ra l a re a. In tra o ra lly , th e d u ct of th e af fec ted g la n d ma y b e se en to be s wo l le n a nd

red de ne d, a n d th e d u ct pa pi ll a e n la rg ed. Pu ru l en t sal iv a ma y be m ilk ed fro m th e du c t b y

ma n ua l pr es su re . Wh en pu s c an b e exp re ss ed f ro m th e d u ct it s h o u ld be s en t f or c ul tu re an d se n si ti vi ty te sti ng . In th e ab se n ce o f s en s iti vity tes ts , flu c lo x ac il li n is th e an ti mi cro b ia l o f ch o ic e in th e pa tie n t w h o is n o t s en s iti ve to pe n icil lin . T he re w ill o fte n be a n a ss o c ia ted lym ph a de n o pa thy o f the c erv ic a l n o d es .

SjΓ ¶g re n' s sy nd ro m e i s a pre di sp o s in g fa cto r fo r b ac teri a l i n fec tio n s , bo th a c u te a n d ch ro n ic .

In pa tie n ts in w h o m ill h e al th h as l ed to lo w e red re si sta n ce , a sc en d in g in f ec tio n o f a n y o f th e

sa li va ry g la n ds m a y o c cu r fro m th e mo u th vi a a sa liva ry gl an d du c t. Th is , o n c e a c o mm o n

se qu el to g as tro in te sti n al s u rge ry, e sp ec ia ll y i n old er pa tien ts , is n o w n o t s o o fte n s ee n du e

to im pro ve d pe rio p era tiv e c a re. In th es e ca se s th e tr ea tme n t is e vid en tl y n o n su rg ic a l, b ei ng

dep en d en t o n an ti bi oti c th era py , the m a in ten a n ce o f a co rre ct flu i d ba la n ce , an d , if po s s ibl e, th e re so l u ti o n o f th e pr ed is po s in g co n d itio n .

Trea tm en t o f an a c u te p ar oti tis ten d s to be s o me w h at p ro tra cte d. B ac teri a l s w ab s s ho u l d b e

ta ke n a n d a n tib io tic s en s iti viti es d ete rmi n ed w h en ev er po s s ibl e. It is c le ar, h o w ev er, th a t th e us e o f a n tib io tic s s ho u l d b e reg a rde d o n ly as f irs t-lin e tre atm en t. A n y p red is po s in g fa c to r

mu s t be i de nti fie d. It is po s s ibl e tha t s u rgi c al trea tm en t m ay b e n e ce ss a ry, s u c h as r em ov al of a du c t ca l cu l us o r, o c c as io n a ll y, th e exc i si o n of a s ev ere ly da m ag ed gl a nd .

Ch ro n ic s ia la de n itis , e ith er o f th e pa ro tid o r th e s u bm an d ib ul ar gl an d s , ma y fo ll o w th e

res o lu tio n o f a n ac u te in fe cti o n o r m ay o cc u r w i thou t a n y evi de n t pri ma ry a cu te ph a se . In

th e se c irc u m sta n ce s sy mp to ms a re rel ati ve ly lo w -grade w ith te nd ern e ss a n d a mi n o r de gr ee

of s w ell in g o f th e a ffe cte d gl an d , an d o cc a si o n al ly wi th s o me de gr ee o f s w el li ng a n d red ne s s of th e du c t an d th e pa pi ll a. A s i n ac u te si al a den i tis , pu ru l en t s al iv a ma y be ex pre ss ed f ro m

th e du c t. F req ue n tly in th e se c as e s, m in o r dil a ta tio n s o f the d u cta l sy ste m o f th e gl an d s ma y be de tec ted o n s ia lo g rap h y a n d, p re su m ab ly , the s e pro vi de fo c i of in fe c ti o n a nd s ta gn a ti o n .

Su c h a re cu rre n t c hr on i c si al a de ni tis m ay o c cu r fo ll o w in g rad io th e rap y a ff ec tin g th e g la n ds o r ma y f o llo w th e m in o r d am a ge du e to th e p re se n ce o f a ca lc u lu s . Th e pri n ci pl es o f trea tme n t

are e xa ctl y th e sa m e as th o s e of a cu te s ia la de n itis. In th e pa tie n t w ith re cu rr en t s ia la de n itis

of a su b ma n di bu l ar gl an d , ex ci si o n o f th e gl an d m igh t be re qu ir ed. Tab le 8. 4 su m m ari z es th e ma in c a u se s o f si a la de ni tis .

Sialosis

Si al o si s (s ia la de n os i s) im pl ies a p a in le ss , n o n -in fl am ma to ry, n o n- ne o pl as tic s w el lin g o f the

sa li va ry g la n ds . Th e re are m an y pr ec ip ita ti n g fa ctors f o r th is c o n di ti o n , in c lu di n g th e e ffe ct

of a n ti rh eu m ati c dru g s, dru gs co n ta in i ng i od in e , and a dre n erg ic d ru gs . S im il ar ex pe rim en ta l si al o si s ma y be sti mu l ate d in a n im al m o de ls by th e ad mi n is trati o n o f i so p ren a li n e. O n th e wh o le th es e dru g -in du c ed e nl ar ge me n ts a re re ver si ble, a lth o u gh , i n so m e ca s es , a cu te

si al ad en i ti s h as b ee n re po rted f o llo w in g w ith d raw a l o f th e dru g . S ia lo s is m ay al s o o cc u r i n

ho rm o n al a bn o rm al iti es (d ia be tes m el litu s , a cro m eg aly) an d i n n u tr itio n a l d ef ic ie n cy s ta te s, in cl u di n g a n o rex ia n er vo sa a n d ch ro n i c al c oh o l is m. T h e p ar oti d gl an d s are m o st f req u en tly

aff ec ted , a nd th e sw e ll in g is co m m on l y b il ate ra l. Th e m ec h an is m i nv o lve d is n o t u n de rsto o d , bu t, h is to lo gi ca l ly, th e re is h yp ertro p h y o f se ro u s ac in i . In ves tig a ti o n o f p a ti en ts w ith

po s si bl e s ia l os is s h o u ld in c lu d e th e id en ti fic a tio n o f pre di sp o si n g c a u se s. Th u s, liv er fu n cti o n te s ts a n d tes ts fo r b lo o d gl u co s e an d gr o wth h o rm o ne m a y b e in d ic ate d. A d eta il ed d ru g

hi sto ry s h ou l d be rec o rd ed a nd th e po s si bi li ty o f ea tin g di s or de rs co n s id ere d ( Ch ap ter 17). Ap pro p ria te im ag in g s h o ul d be u n de rtak en . Ho w ev er, bio p sy i s rare ly in d ic ate d.

Table 8.4 Causes of sialadenitis Ba c teri al

A s c en di n g si a la de ni tis

R ec u rren t p a ro titis o f ch i ld h oo d

Vi ral

M u m ps H IV

C yto me ga l ov iru s

Sj Γ¶g re n' s sy n dro m e

Ir rad ia tio n

Necrotizing sialometaplasia

Th is re la tive ly u n co m mo n , β € tu m o u r-lik e β€™ co n di tio n o c c ur s mo re fre qu e n tl y in m al es ,

es pe ci al ly sm o ke rs , an d i s of u n kn o w n a etio l o gy. It ap pe ars to be th e r es u lt o f a va sc u li tic ph en o m en o n th a t o cc u rs in mi no r sa l iva ry gl an d s, u su al ly in th e p al ate . It is po s s ibl e tha t is ch a em ia l ea ds to i n fa rcti o n o f s a li var y ti ss u e. The c o n se qu e nc e is re la tiv ely p ai n les s

ul ce ra tio n o f rap id o n s et. Th e m arg in s a re o ften e ve rted an d m ay b e i n du ra ted , res em b lin g a ca rc in o ma . A n ae s th e si a o f th e pa l ata l m u c os a h as b ee n re po rted a s a n ea rly in d ic a to r o f th i s fo rm o f u l ce rati o n . Hi sto lo g ic a lly , th e s q ua m o us m eta p la si a fo u n d i n th e sa li va ry d u cts ,

to g eth e r w ith p se u do ep ith e lio m a tou s h yp er pla s ia o f the s u rro u n di ng p al ata l ep ith el iu m , ma y giv e an i n co rre c t im pre s si o n o f m a lig n an c y. T hi s is a s el f-lim iti n g co n d itio n th a t re so l ve s in ab o ut 8 w ee ks tim e w ith o ut an y th i n g o th e r th an sy mpto ma tic tre atm en t. Le si o n s of

ne c ro ti z in g si a lo me tap la s ia a re o ften e xc is ed fo r dia g no s tic p u rpo s es . A s ye t, th e re is n o re al in fo rm ati o n ab o u t th e im me di ate c a us e o f th i s co n ditio n o r of i ts i mp li ca tio n s i n rel ati o n to sys te mi c fa cto rs .

Sarcoidosis

Sa rc o id os i s i s a c hr on i c gra n u lo m ato u s di so rd er th at m a y r are ly pre s en t a s pa in l es s, per si ste n t en l arg em en t o f th e ma jo r s a li va ry

gla n ds . Th e re is o fte n an as s o ci ate d r ed uc tio n in sal iv ary fl o w an d th ere m ay be a n

ac co m pa n yi n g β € Sj Γ¶g re n' s -lik e β€™ co n di tio n . S ar co i do s is is de sc ribe d m o re fu ll y in Ch ap ter 12.

Table 8.5 Causes of salivary gland swelling In f ec tive

Dru g -as so c ia te d

Ba c teri al

Al co h o l

A s c en di n g s i al ad en i ti s

Io d in e c om p ou n d s

P. 89

R ec u rren t p a ro titis o f ch i ld h oo d Vi ral

Th io u ra ci l Su l fo n am id es

M u m ps

Ph e no th i az in e s

H IV pa ro titi s

Ch lo rh ex id in e

In f la mm a to r y

En d o cri n e

Ob stru c tiv e si a la de ni tis

Ac ro m eg al y

Sj Γ¶g re n' s sy nd ro m e

Dia be te s

Sa rc o id os i s

Me tab o lic

Si al o si s

Al co h o li c ci rrh o s is

Neo p la sm s

Ma ln u triti o n

Pl eo m o rph ic sa l iva ry ad en o m a

Oth er s

Ad en o ly mp h o ma

Sa rc o id os i s

Mu c o ep id erm o id ca rc in o m a

Nec ro tiz in g si a lo me ta pla s ia

Ac in i c ce ll c arc in o m a

Ad en o id c ys tic c a rci n om a , etc .

HIV-associated salivary gland disease

Pa tie n ts w i th HIV i nf ec tio n c a n de ve lo p sa li va ry gla n d pro b le ms a n d xer os to m ia . Th e sa li va ry

gla n d sw e ll in g ma y be du e to a β€ Sj Γ¶g re n' s -lik eβ €™ co n di tio n w i th lym ph o c yti c in fi ltra tio n

an d a dry m o uth . H ow e ve r, th ere m ay be o th er pa th o lo gy pr es en t i n th e s a li va ry g la n d su c h

as Ka po s i' s sa rc o ma o r a l ym ph o m a. It is a ls o po s s ib le th at s a li va ry g la n d sw e lli n g ma y b e a co n s eq ue n ce o f o the r v ira l in fe cti o ns s u c h as c yto meg al o vi ru s or E ps tei n β €“Ba rr viru s (Ch ap ter 4). Ch ro n ic p ar oti tis i n ch i ld ren i s h ig hly s u gg es tiv e of HIV in fe c ti o n .

Salivary gland tumours

Sa li va ry gla n d tu mo u rs c o mp ro mi se a bo u t 3 pe r c en t o f al l tum o u rs . Th e ma jo rity o cc u r i n th e p aro ti d g la n ds a n d on l y 10 pe r c en t af fe ct th e m in o r s a liv ar y g la n ds . Th er e i s a gre at var ie ty o f sa li va ry g la n d tu mo u rs a n d i n 1991 th e WHO p ro po s ed a c la ss i fic a tio n a n d no m en c la tu re fo r th e se (s ee Tab le 8. 6 fo r s u m ma ry).

Th e cl in i ca l pre se n tati o n, h i sto p ath o lo g ic a l f ea tu res , a nd m an a g em en t o f the s e sa l iva ry gl an d tu m o u rs is o u tsi de th e sc o pe o f th is bo o k , an d re aders a re ad vi se d to co n s u lt o th er re fere n ce so u rc es (s ee th e pro je ct at the e n d o f c ha p te r).

Tu mo u rs af fe cti ng th e mi n o r s al iv ary gl a nd s w il l b e bri efl y d is c u ss ed , a s th e se p res en t

in trao r al ly. S i nc e th e g re ate st c o n c en tra tio n o f the se g la n ds i s in th e are a o f th e ju n c tio n o f th e h a rd an d s of t pa l ate s, th is i s th e r eg io n in whi ch th e se n eo p la sm s a re m o s t o ften se en . Ab o u t 20 pe r c e nt o f m in o r s a li va ry g la n d tu m o u rs occ u r i n th e up pe r l ip . Th e ma jo rity o f

th e se l es io n s a re p le o mo rp h ic a de n o ma s bu t m o re ag gr es si ve le si o n s su c h a s ad en o c ys tic ca rc in o ma s m ay o cc u r.

Table 8.6 Classification of salivary tumours* Aden o m as

Car cin o ma s

Pl eo m o rph ic ad en o m a

Mu c o ep id erm o id c arc in o m a

Warth i n 's tu mo u r (a d en o ly mp h om a )

Ac in i c ce ll c arc in o m a

Ba s al ce ll a de n om a

Ad en o id c ys tic ca rc in o m a

On c o cy to m a

Car ci n om a a ris in g in pl eo mo rp h ic

Can a li cu l ar a den o m a

Po l ymo r ph o u s lo w -gra de a de n oc a rc in o ma

Duc ta l pa pi ll o ma s

Oth er c arc in o m as

ad en o ma

*S o u rc e: S oa m es , J. V . an d S o u th a m , J. C. (1988). Ora l pa th ol o gy , 3rd edn . O xf o rd

Un iv ers ity P res s , Ox fo rd.

Ten p er ce n t o f al l s a li var y g la n d tu m o u rs af fe ct th e m in o r s a liv ary gl an d s. T h e m a jo rity o f th e se a re pl eo m o rph ic a d en o ma s o c cu rri n g in th e pa la te.

Clinical features

Th e g ro w th o f p le o mo rp h ic a de no m a s is u s ua ll y sl o w an d p ai n les s a n d u l ce ra tio n is u n u s u al un l es s th ere h as b ee n s o me de gr ee o f tra um a . Th e textu re o f su c h a tu mo u r i s u su a ll y f irm an d th e o v erl yin g m u co s a ma y ap pe ar vi rtu al ly n or mal ( Fi g. 8. 3).

Th e o u ts tan d in g ch a ra cte ris tic o f th es e g ro w th s is th ei r u n pre di cta bi li ty , bo th i n h is to lo gi c al ap pe ara n c e a n d in c li n ic al b eh a vio u r. M or e a g gre ss iv e g ro w th m ay o cc u r i n s om e tu mo u rs wi th u lc era tio n o f th e u n de rlyi n g m u c o sa . Th es e fe atu re s mu s t b e c o n si de red a s po s s ib le

ma rke rs o f a ma li gn a n t le s io n s u ch a s a n a de no c ys tic c a rci n o ma , w h ic h ma y res u lt in d ea th, bo th by l oc a l ti ss u e in va s io n a n d th e p ro du c tio n o f dis ta n t me tas ta se s. Th e o n l y cl u e as to

th e p ro ba bl e e ve n tu a l be h av io u r o f a sa li va ry ne o plas m is f ro m th e h is to lo gi ca l ex am in a tio n

of tis s u e, a n d th i s mu s t fo rm th e ba s is o f th e s u rgi ca l trea tme n t o f th e l es io n .

Fig. 8 .3 A la rg e p le o mo rp h ic a de n om a o f th e p a la te .

Sa li va ry gla n d tu mo u rs , be ca u s e o f th ei r o fte n sl o w a nd p ai n le ss g ro w th , a re o fte n ve ry

dec e ptiv e, a n d la ck o f co m pl ai n t fro m th e pa tie nt ma y pro d uc e a qu i te u n wa rra n ted s en s e o f se cu ri ty. A n y s u s pec te d s a liv ar y tu m o ur sh o u l d b e ref erre d at o nc e fo r in ve sti ga tio n a n d tr ea tme n t w ith o ut an y a tte mp t a t m in o r s u rgi ca l in ter ven ti on , w h i ch m ig h t m ak e l ate r

def in iti ve trea tm en t d iff ic u lt. M o st pl eo m o rph i c a den o ma s a ffe c ti n g th e m in o r s a liv ar y g la n ds are e xc is ed w ith a ma rg in o f su rr o un d in g n o rma l tissu e .

An y su s pe c ted sa l iva ry gl an d tu mo u r sh o u ld b e re fe rred f or in v es tiga tio n an d tre atm en t wi th ou t a n y atte mp t a t mi n o r s u rgi ca l in terv en tio n , w h ic h m ig h t ma ke l ate r d efi n itiv e tr ea tme n t di ffi cu l t.

Disturbances of salivary flow Xerostomia

Xe ro sto m ia i s the s u bje cti ve fe el in g o f o ra l dry ne ss, w h ic h m ay o r m a y n o t be a ss o c ia ted wi th h yp of u nc tio n o f th e sa li va ry g la n ds . A l ac k o f s al iv a ma y be d ue to e ith er a lo s s o f se cre to ry ti ss u e in th e s al iva ry gl an d s, or a di sturb an c e in th e s ec reto ry in n e rva tio n

me ch a n is m bro u g ht ab o ut by th e a c tio n o f x ero g en ic d ru gs o r, m u c h le ss c o mm o n ly , by ne u ro lo g ic al d is ea se . A si gn i fic a n t pro p o rtio n o f pa tie n ts c o m pl ai n in g of xe ro s tom ia a re

fo u nd , a fter in ve s ti ga tio n s , to h a ve so m e sy ste mi c fa cto r r es po n s ib le fo r th e re du c tio n in sa li va ry f u nc tio n . T he s e s ys tem ic f ac to rs m ay be a ss o c ia ted w ith a w id e ra ng e o f di se a se

pro c es se s (s u ch a s re n al a nd e n do c rin e di stu rb a nc e s) bu t m a ny o f th e se pa tie n ts su ff er fro m SjΓ ¶g re n' s sy nd ro m e; th i s w ill b e d is c u ss ed i n mo re d eta il be lo w . Th e c au s es o f xe ro sto m ia are s u mm ar iz ed i n Tab le 8. 7.

Xe ro sto m ia i s a sy mp to m th a t s h o ul d be in v es tiga te d b ec a us e it ma y b e in d ic ati ve o f un d erl yin g s ys tem ic d is ea s e.

Th era pe u tic ra di ati o n to th e h e ad a n d ne c k re gi o n, fo r th e trea tme n t o f ma li gn a n cy , c an a ls o

P. 90

ca u se a m ark ed d im in u tio n i n sa l iva ry flo w a n d se vere o ral dry n es s. Pa tie n ts tr ea ted by

wh o le -bo dy ra di ati o n (fo r exa m pl e, p rio r to bo n e marro w tra n s pl an ta ti o n fo r le uk ae mi a ) a n d

th o s e gi ven rad io a cti ve io d in e (I 1 3 1 ) fo r thy ro id c an c er ca n a ls o s u ffe r f ro m xero s to mia . Ea rl y

dim in u tio n of s al iv ary fl o w du e to ra di ati o n ma y be du e to d am a ge to th e blo o d su p pl y o f th e gla n ds , b u t la ter eff ec ts ar e th e re su lt o f d es tru cti o n o f th e gl an d 's s ec reto ry a pp ara tu s.

Neu ro lo g ic a l d is ea s e, e ith er ce n tra l o r pe rip he ra l, m ay b e r es po n s ib le fo r a d ec re as e in th e se cre to mo to r sti mu l ati o n o f th e sa l iva ry gl an d a nd , h en c e, th e d ryn es s o f th e m o uth .

Ho w eve r, th e m o s t co m m on ca u se f or th is is th e a ctio n o f d ru gs w ith th e s ite o f a c tio n be in g

eith e r c en tra l or in th e a u to n o m ic p ath w ay . Gro u ps o f dr ug s im pl ic a ted as h av in g th is ki n d o f ac tio n i n cl ud e an ti hi s ta m in es , a n tih yp erte n si ves , an d se da tiv es . Ho w ev er, th e m o re c o m mo n on e s to ca u s e x ero s to mi a ar e th e ps yc h o tro pi c dru g s an d , in p ar ti c ul ar , the a n tid ep res sa n ts an d tra nq u il liz e rs . Ov er 400 dr ug s h av e bee n i de ntifie d as h a vi ng th e po te nti al to c a us e var yin g de gre es o f o ra l d ryn e ss , a nd th e ef fec ts m ay be p o te n tia ted in pa tie nts ta ki ng

mu lti pl e x ero g en ic d ru gs . Dru g -rel ate d o ra l dry n es s sh o u ld b e a r eve rs ib le si de -eff ec t, w ith res o lu tio n o c c ur rin g fo ll o w in g ce ss a tio n o f the drug .

Table 8.7 Causes of xerostomia De ve lo pm en ta l

Ap la s ia o r a tres ia

Sa li va ry gl an d di s ea se

Sj Γ¶g re n' s sy nd ro m e ( pri ma ry, se c o nd a ry) Sa rc o id os i s

HIV in fe c ti o n Ia tro ge n ic

Dru g-in du c ed

Th era pe u tic irr ad ia tio n (e . g. e xtern a l be am ra di o thera py , to ta l bo d y irr ad ia tio n Graf t-vers u s -ho s t di se a se Ps yc h o ge ni c

Ora l dys a es th es ia

Bu rn i ng m o u th sy n dro m e An xi ety /de pre ss i on De h ydr ati on

Fe bri le il ln es s


Dia be te s in s ip id u s Ren a l fa il ur e Dia rrh o ea Al co h o l

Ma y ca u se s al iv ary g la n d di se a se , li ver di se a se , and de h ydr ati on Loc a l

Mo u th -bre ath in g

Co mmonly used drug s that cause xero st o mia An tid ep re ss a nts An tih i sta m in es Dec o ng es ta nts An tip a rkin s o n ia n ag en ts

Tra nq u il liz e rs a nd h yp n o tic s An tip s yc ho ti cs Diu reti cs Ap pe tite su p pre s sa n ts

Sa li va ry hy po fu n c tio n c a n al s o be d ue to a n u n de rlyin g co g n itiv e dis o rd er, s u c h as d ep res s io n or c hr on i c an x ie ty . Th e ab il ity o f a c ute a n xie ty to c a u se a tra n si en t re du c tio n i n s al iva ry fl ow

is w el l k n ow n to stu de n ts tak in g exa m in a tio n s an d th o se e ng a ge d i n p ub li c sp ea ki n g. P a tien ts

wi th a se n so ry o r c o g ni tive d is o rde r m a y a ls o h a ve a pe rc ep ti o n o f o ra l dr yn es s , bu t o b jec tive me as u rem en ts o f sa li va ry flo w m a y b e n o rma l an d th e mo u th a pp are n tly mo i st. T he s e in di vid u al s fre qu e ntl y c o m pl ai n o f o the r s ym pto m s , su c h a s a ba d ta s te an d ab n o rma l se n sa tio n s in th e

mo u th . X ero s to mi a is fre qu e n tly rep o rted by p atie n ts w ith b ur ni n g m o u th sy n dro m e.

P. 91

Th ere a re co n fl ic tin g rep o rts o n th e e ff ec t o f a g e o n s al iva ry gl an d fu n c tio n , bu t th e re is so m e e vi de nc e th at sti mu la te d s a liv ar y fl ow rate s ar e u n im p ai red w ith a g e i n h ea lth y,

un m ed ic a te d in d ivi du a ls . Un s tim u la ted w h o le sa l iva ry flo w r ate s, h o w ev er, h a ve b een sh o w n to de cre a se w ith a ge in h ea lth y n o n -me dic a ted s u bjects . A ge , in a dd iti o n to dru g s an d

dis e as e, i s im po rta n t in red u ci n g th e s ec reti o n o f re s ti n g wh o l e s a liv a. Iatro g en i c ca u se s a nd

sys te mi c dis e as e are ri s k fa c to rs fo r x ero s to mi a tha t a re mo re l ike ly to b e e n co u n tere d in th e mi dd le -ag ed o r e ld erl y po p u la tio n .

Investigation of xerostomia

Sy mp tom s o f a dry mo u th in c lu d e th i rst, d if fic u lty in e a ti n g dry fo o ds a n d sw a ll o wi n g,

dif fic u lty in sp ea ki n g an d w ea rin g d en tu res , a nd the n ee d to ta ke f req u en t s ip s o f w a ter w h i le

ea tin g. P a tie nts m a y a ls o c o mp la in of a b ur ni n g s e ns a tio n i n th e m o u th an d ab n o rma l tas te o r ha li to si s β €”freq u en tly th ey re po rt c ra ck ed li ps o r so re n es s at th e c o rn er s of th e mo u th . Th e qu es tio n s li s te d in Tab le 8. 8 ar e f req u en tly h el pf ul i n id en tif yin g p ati en ts w ith o ra l

dys fu n c tio n as s o ci ate d wi th a re du c ed sa l iva ry flo w. O ro fa c ia l s ig n s an d s ym pto m s as s o ci ate d wi th sa l iva ry gl an d h yp o fu n cti on are g ive n i n Tab le 8. 9.

Cli n ic al s ig ns a s so c ia ted w ith sa li va ry g la n d h ypo fu nc tio n i n cl u de dr yn es s o f th e o ra l m u c os a (wh i ch of ten a p pea rs l ike pa rc h me n t) , fis s u rin g a nd l o bu la tio n o f th e to n g ue ( Fi g. 8. 4), an d evi de n ce o f or al c an d id os i s, p ar ti c ul ar ly in th e form o f an g u la r c h ei liti s. Th e s a li va m ay

ap pe ar stri n gy an d th ic k an d ten d s to ac c u mu l ate a s sm al l be ad s o n th e m u co s a . Th ere m a y

be di ffi cu l ty i n β€ mi lki n g β€™ sa li va fro m th e ma jo r d u c ts a n d the re is o fte n d en tal c ar ie s a t

si te s n o t u su a ll y s u s ce pti bl e to d ec a y. Th e s al iv ary gl an d s ma y al so be s wo l le n , ei the r d u e to ch ro n ic i n fec tio n o r in vo l ve me nt in a n a u toi mm u n e si al ad en i tis ( Fi gs 8. 5 an d 8. 6 ).

Table 8.8 Questionnaire to identify patients with salivary gland hypofunction 1. 2. 3.

4.

Doe s th e a m o un t of s al iv a i n yo u r mo u th fe el to o li ttl e, to o m u c h o r d o yo u

no t n o ti ce it?

Doe s yo u r m o u th fe el dry w h en y o u ea t a m ea l?

Do yo u fr eq ue n tly si p liq u id s w he n yo u ea t a me a l? Do yo u h a ve di ffi cu l ties s w a llo w i ng a n y fo o ds ?

Table 8.9 Signs and symptoms suggestive of salivary gland hypofunction Sy mp tom s re po rte d Ora l dry ne s s

Bu rn i ng , tin g lin g s en s ati o n o f to n gu e

Th e n e ed fo r fre qu en t d ri nk s to be tak en w h il s t ea tin g o r ta lk in g Diff ic u lty i n c h ew in g a n d sw a ll o w in g dry fo o ds Al tere d ta s te ( dys g ue s ia ) a n d sm e ll

Rec u rren t sa li va ry g la n d sw e lli n gs /i nf ec tio n s In c re as e in r ate o f d en ta l de ca y

Dry, s o re, c ra ck ed li ps a n d an g le s of mo u th Diff ic u lty i n ta lki n g ( dys p ho n i a)

Gen e ral iz ed m u co s a l so r en es s a nd u l ce rati o n o f d en tu re - bea ri n g a re as Ora l ex am in a tio n re ve al s Sw o ll en s a li va ry g la n ds

Ab se n ce o f sa l iva ry fil m o ver o ra l m u co s a

Dry, p ap er -th i n β€ pa rch m en tβ €™ ap pe a ran c e o f o ra l mu c o sa o r ap pe ar an ce o f sm al l am o u nts o f fro th y sa l iva i n a n o th e rw is e dry m ou th Fi ss u rin g a n d l o bu la tio n o f th e to ng u e Dry, c ra ck ed li ps , a ng u la r c h ei li ti s

Ev id en c e o f ch ro n i c o ral c an d id o si s

Deve lo p me n t o f n e w ca ri o us l es io n s , es pe c ia ll y o n inc i sa l o r c u sp al su rf ac e s

Fig. 8 .4 A dr y a n d s o m ew h at lo bu l ate d to ng u e as s oc i ate d w i th xe ro sto m ia .

Sa li va ry gla n d h ypo f u nc tio n i n cr ea se s a pa tie n t' s su s ce pti bi li ty to :

ca rie s

ca n di do s is , in c lu d in g an g u la r c h eil iti s

or al m uc o s itis

Management of xerostomia and salivary hypofunction

Ma n ag em en t dep en d s o n th e u n d erl yin g c au s e o f th e xe ro sto m ia a n d th e de gre e o f s a liv ar y

gla n d im pa ir me nt. P a tie n ts w h o h a ve de tec ta ble s a liv ary fu n c tio n c a n be en c o u ra ge d to u se

so m e f o rm o f l o ca l sti mu la tio n , f o r ex am p le, c h ew i ng s u ga r-free g u m o r su g ar -free p as til le s. Th e a n tib ac ter ia l p ro pe rtie s po s se s se d by xyl ito l, wh i ch is o fte n u s ed as a s w ee ten e r in

su g ar-free g u ms , m ay h av e a s ig n if ic an t ad di tio n a l a n ti -ca rie s ef fec t. De nta te pa tie nts m u s t be di sc o u ra ge d f ro m us i ng su g ar -co n tai n in g o r a c idic s we ets a s th es e c a n ex ac e rba te th e

te n de n cy to de mi n era li z ati on o f th e tee th an d c au s e de nta l ca ri es . S ys tem ic m ed ic ati o n to stim u la te sa li va ry flo w h a s be en as s es se d in a n u mber o f

cl in ic a l tri al s a nd , to d ate , pi lo c ar pin e s ee ms to h a ve be en the m o st eff ec tive d ru g te s te d.

Ho w eve r, s u ch sti mu l ati n g th e rap y is o f l ittl e u s e if the x ero s to mi a is du e to n ea r tota l lo s s

P. 92

of s ec reto ry u n its in the s a liv ary g la n ds . P ilo c a rpi n e a ls o h a s di sa dv an ta ge s: it h a s a n u mb er of in te rac tio n s w ith oth e r d ru gs a n d m a y h a ve a dve rs e e ff ec ts on th e c a rdi o va sc u la r s ys tem . Mi n or s ide -eff ec ts su c h a s sw e ati ng a n d th e u rg e to u rin a te h a ve b een rep o rted by p ati en ts ta ki n g s ys te mi c pil o ca rp in e an d a re du e to its c h o li ne rgi c a go n is t a cti vity .

Fig. 8 .5 P aro ti d s w el li ng i n a pa tie n t w ith a u toi mm u n e si alad en i tis .

Fig. 8 .6 P us e xp res s ed fro m th e pa ro tid du c tβ€” th e re su l t of i n fec tio n s e co n d ary to au to im mu n e s ia la de n itis a s pa rt o f S jΓ¶g re n' s sy nd ro m e.

Sympt omat ic relief o f dry mo uth In tri n si c (in c rea s e g la n d ac tiv ity)

su g ar- free g u m,

pil o ca rp in e

Ex trin s ic

sa li va s ub s titu tes

Th e u s e o f sa l iva s u bs titu tes m a y b e of s o me h el p to pa tie n ts c o m pl ai ni n g of a d ry m o u th

an d ca n b e of fe red to p ro vid e sy mp to ma tic re li ef for p ati en ts w h o h av e in s u ffi ci en t s a liv ar y

fu n cti on to be n efi t fro m s tim u la tio n . A la rg e nu m ber o f di ffe ren t β € arti fic ia l s al iva s β €™ are no w c o m me rci a lly pr o du c ed, an d m os t a re ba s ed o n c arb o xym e th y lc el lu lo s e o r m uc i ns . If po rc in e mu c in p re pa ra ti o n s are pre fe rred b y th e c lin i ci an , c a re mu s t b e ta ke n n o t to

pre sc rib e th em to ve ge ta ri an s a n d var io u s rel ig io u s gr ou p s. A nu m be r o f pre pa ra tio n s c o nta in flu o ri de an d th is c o u ld be b en e fic ia l as a p ro tec tiv e m ea s u re ag ai n st ca rie s . Cl in ic a l tri al s ha ve i nd ic a ted th at a rti fic ia l sa l iva s c an b e u se ful fo r th e ma n a ge me nt o f x ero s to mi a bu t

pa tien ts f req ue n tly di sc o n tin u e the ir u se . E ith er th ey do n o t l ik e th e tas te o r c o n si ste n cy o r fin d th at freq u en t a p pli c ati on is req u ire d, m a kin g u s e i n co n ve n ie nt an d e xpe n si ve . Ma n y

pa tien ts p ref er to tak e fre qu e nt si ps o f w ate r fro m a s m al l c o n tai n er ca rri ed ar o un d w ith

th e m. T h e u s e o f s u ga r -free p as til le s ma y so m eti me s be mo re h e lp fu l th a n s pra ys i n a s oc ia l se tti n g. C rac ki n g an d dry in g o f th e li ps c a n be c o ntr ol le d by a pe tro leu m -ba se d o in tm en t

su c h a s Va s eli n e, a n d an g u la r c h eil iti s is tre ate d a c co rd in g to th e mi c ro - or ga n is ms c u ltu re d. Ora l ca n di do s is c a n u su a ll y b e co n tro ll ed w ith to p ic al an ti fu n ga l ag en ts . Den ta te p ati en ts

req ui re reg u la r d en ta l i n sp ec tio n s , w h ic h ma y n ee d to b e at in terv al s o f 3 mo n th s o r le s s.

Ad vi ce s ho u l d b e gi ven ab o u t ma in te n an c e of o ra l h ygi en e, a vo i da n ce o f su g ar y f oo d s, an d us e o f f lu o rid e su p pl em en ts . Th e u se o f as trin g en t m o u thw a s he s , es pe ci al ly th o se th at co n tai n al c oh o l , are b es t a vo id ed . F lu o rid e -co n tai nin g ge ls m ay b e to l era ted m o re

su c ce s sfu l ly by pa tie n ts wi th s al iva ry gl an d h yp o fun cti o n . Th ey o ften la st lo n ge r a n d thi s m ay

ren de r th e m mo re c o st-eff ec tive . It i s o f ten d iff icu lt f o r pa tie n ts to al ter th e ir di et w h ic h

te n ds to b e se ve rel y re str ic ted to bl an d , so f t fo o dstu ff s. M a n y f oo d s ar e to o a s trin g en t a n d

ha rd fo o d s ca n tra u ma tiz e th e fra gi le o ra l m u c os a . Ne verth e le ss , in d iv idu a l di eta ry a n a lys is is a dvi sa b le fo r pa tie n ts wh o h a ve a h ig h c ari es i nci de nc e .

P. 93 Ch ew in g s u ga r-free g u m:

in cr ea se s th e f lo w o f sti mu l ate d s a li va to le ve ls abo u t 3 to 10 ti me s res tin g va l ue s

ca n h el p pre ven t ca ri es : s tim u la ted s al iv a h a s en h an ce d bu ff eri n g c a pa ci ty a n d a

wi ll in c rea s e th e re sti n g s a li va ry f lo w ra te fo r u p to 30 m in u tes be yo n d the p eri o d o f

ha s a n an ti mi cro b ia l ac tiv ity if it c o n tai n s xyl itol

ma y b e pro b le ma tic i n pa tie n ts w i th h ia tus h e rn ia or ga stri c an d in tes tin a l u lc era tio n s

gre ate r rem in e ra liz i n g p o ten tia l tha n re s ti n g sa li va ch e wi n g

Pa tie n ts w h o co m pl a in o f xer os to m ia , bu t h a ve n o c li ni ca l o r o b jec tive e vid en c e o f a d ry

mo u th , o ften h a ve o th er o ral s ym pto m s su g ge sti ve o f o ra l dys a es th es ia a n d ma n y co m pl a in of a bu rn i n g s e ns a tio n i n th e m o u th. Th es e pa tien ts c a n be st be ma n a ge d by c o un s el li ng , in cl u di n g p sy ch o th er ap y i f a p pro p ria te. P s yc h otro p ic m ed ic a ti o n , pa rtic u la rl y th e u se o f

tr ic yc lic m e dic a tio n , is b es t a vo id ed a s it ma y ex ac er ba te x ero s to mi a. T h is s ub jec t i s fu rth er dis c u ss ed i n Ch ap ter 17.

SjΓ¶gren's syndrome

SjΓ ¶g re n' s sy nd ro m e ( SS ) is a n au to i mm u ne d is ea s e of th e e xo c rin e gl an d s th at p a rtic u la rly in vo lv es th e sa li va ry an d la c rim a l g la n ds . Tra di tion al ly , th e s ym pto m s o f S jΓ ¶g re n' s

syn d ro me w e re th o u gh t to res u lt f ro m th e d es tru c ti on o f s a li va ry a n d la cr im al g la nd ti ss u e. Mo re re ce n tly, i t h a s be co m e cl ea r th a t th is i s no t th e c as e a nd th a t ma n y Sj Γ ¶g re n' s

syn d ro me su f fer ers h a ve su b sta n tia l res er ves o f h isto lo gi ca l ly no rm a l a c in a r ti ss u e th a t

si mp ly do e s no t fu n cti on pro p erl y. Re -eva lu a tio n o f S jΓ¶g re n' s sy nd ro m e i n th is li gh t g ive s a mu c h mo re o p tim is tic o u tlo o k f o r tre atm en t d ev el op men t. Wh er ea s th ere c an b e n o

po s si bi lity o f r ec o ver y i f th e ac i na r ce lls h a ve b ee n de stro y ed , rev ers a l o f a ci n ar ce ll hy po fu n c tio n m ay b e a ttai n ab le i f th e ca u s e o f th e h ypo f u nc tio n c a n be d ete rmi n ed .

Pri ma ry S jΓ¶g re n' s sy nd ro m e co n s is ts o f d ry eye s (xe ro ph th a lm ia ) a n d dry mo u th a n d

Se co n d ary S jΓ¶g re n' s sy nd ro m e c o n si s ts o f dr y e ye s a n d dry m o uth an d is a s so c ia ted

Sy ste mi c lu pu s e ryth em a tos u s , sy ste mi c sc l ero s is , pri ma ry bi li ary c irrh o s is , a n d m ix ed

is n o t a ss o ci a te d w ith a co n n e cti ve tis su e di s ea se

wi th a co n n e cti ve tis su e di s ea se , mo s t c o mm o n ly rh eu m ato i d a rth ri ti s

co n n ec tiv e ti ss u e di se as e ma y al so be as s o ci ate d with s ec o n da ry S j Γ¶g re n' s sy nd ro m e

Th is p ro ce ss m a y o c cu r as a n i so l ate d p h en o m en o n , in w h i ch c a se i t is term ed p rim a ry

SjΓ ¶g re n' s sy nd ro m e ( 1Β °S S) , o r in co n ju n c ti o n w ith a c o n ne c tive tis su e o r c o ll a gen di se as e , in w h ic h c as e it i s ref err ed to a s se co n d ary S jΓ¶g re n' s sy nd ro m e ( 2Β °S S) . Th e pre va le nc e o f th i s sy n dro m e i s u n kn o wn an d it is th o u gh t th a t m any ca s es re ma in un d ia gn o s ed . Re ce n t

da ta ha s s u gg es ted th at th e e s ti ma ted p rev al en c e of S S is b etw e en 1 β€“ 3 pe r c en t o f th e U K

po pu l ati o n. M idd le -ag ed a nd e ld erl y w o m en a re ma i nly a ff ec ted a n d p res e nt w ith a c o mp la in t of d ry m o u th (xe ro sto m ia ) a n d dry ey es (x ero p h th a lmia ), w i th or w ith o u t ev id en c e o f a

co n n ec tiv e ti ss u e di se as e . In pa tie n ts w i th se c on d ar y S S , rh eu m ato i d ar thr itis i s the m o st

co m mo n ly a ss o c ia ted c on d iti on . O th er as s o ci ate d dise as es in c lu d e s ys tem ic l u pu s

eryth e ma to su s , p rim ary b ili a ry c ir rh os is , a n d sy stemi c sc l ero s is . In c o mm o n w ith m an y au to im mu n e di s ea se s S S sh o w s a se xu a l dim o rp hi s m w ith a fe ma l e to m a le ra tio o f

ap pro xi m ate ly 9 to 1. X ero s to mi a is o fte n s eve re in p rim a ry S S , b ut so m e stu d ies s u gg es t

th a t i t is l es s ma rke d in s ec o n da ry S S. A h is to ry of s al iva ry gl an d s w ell in g is c o m mo n a n d is eith e r d ue to i n fil trati o n o f th e gl an d b y l ym ph o epith el ia l tis su e o r to r ec u rren t i n fe cti on s

(Fi gs 8. 5 an d 8. 6). S we ll in gs d u e to the rep la c em ent o f sa l iva ry gl an d tis s ue d o n o t ten d to be pa in f ul . S al iva ry gl an d sw e ll in gs o f a n in fe cti ve n atu re a re pa in fu l , an d th ere i s an

as so c ia te d l o ca l o r s ys tem ic ri se i n tem pe ra tu re a nd pu s m ay be e xpr es se d fro m th e d u ct or ifi ce (Fi g. 8. 6 ). X ero p hth a lm ia (d ry eye s ) i s th e m o st c o m mo n co m pl a in t b ut ma n y

su ff ere rs a ls o co m pl a in o f β€ itch i n g β€™ or β€ gri tti n es s β €™ in th e ey es o r giv e a hi sto ry

of re cu rre n t ey e in fe cti o ns . Th i s ca n g ive ri se to ke rato c o n ju nti viti s si c ca . U ntre a te d, th i s ca n eve n tua ll y lea d to c o rn ea l d a ma ge an d l os s o f si gh t. P ati en ts w ith S S m ay a ls o c o mp la in o f a dry sk in o r va gi na l dry n es s.

Th e e a rly de tec tio n o f Sj Γ¶g re n' s sy nd ro m e c a n pr eve n t s eri ou s o c u la r di se a se . Th e a s se s sm en t a n d ma n ag em en t of p ati en ts w ith s al iv ary g la nd e n la rge me n t c o nti n ue s to be a p ro bl em i n th e S jΓ ¶g re n' s pa tie n ts. P e rsi ste n t glan d u la r s w el lin g in the s e i n di vid u al s is a wo rry in g fe atu re a s th e y h a ve a n in c rea s ed ri sk o f d ev el o pin g n o n -Ho dg kin ' s l ym ph o m as . Th es e are o fte n lo w -gra de B -ce ll ly mp h om a s an d a pp ea r to b e si mi la r to l ym ph o m as th at

dev el o p i n o th er mu c o sa -as so c ia te d l ym ph o id tis s u e ( MA LT) . Lo w -gra de B - ce ll ly mp h om a s o f oth e r M A LT s ite s (MA LTo m a s) in c lu d e th o s e o f s tom a ch . th yr oi d, a n d lu n g. Sa li va ry gla n d

im ag in g tec h n iqu e s, in cl u di n g C T a n d MR , ca n p ro vide im po rta n t d ia gn o sti c in fo rm a tio n b ut bio p sy i s in di ca ted i n so m e ca s es . Hi sto p ath o lo g y of en l arg ed s al iv ary g la nd s (p arti cu l arl y

pa ro tids ) in S S u s ua l ly rev ea ls a b en ig n l ym ph o ep ith el ia l le si on , b u t th is i s no t al wa ys th e

ca se . M ajo r gl an d b io ps y is , h o we ve r, n o t in d ic ate d fo r ro u tin e d ia gn o s ti c ev al u ati o n o f S S .

Cli n ic al s ig ns , s u ch as a ra pi d an d p ro gre ss iv e un ila tera l o r a sy mm etri ca l gl an d en l arg em en t or /an d a c h an g e i n th e c o n s is ten c y o f the g la n d f rom a so f t sw e ll in g to a h ar d o r n od u la r

on e , sh o u l d p ro mp t m ajo r gl an d b io ps y. O p en p ro ce dure s gi ve mo re u s ef ul i nf o rma tio n a n d bette r ti ss u e sa mp li n g th a n a n ee dl e bio p sy . Th er e ar e f ew l ab o ra to ry m ar kers th a t w il l ide n tify th o se p ati en ts at ris k fro m ma li gn a n t ly mph o ma , a lth o u gh a f al l i n s eru m im mu n o gl o bu li n s sh o u ld p ro mp t fu rth er in ve s ti ga tio ns .

P. 94

SDiagnosis of SjΓ¶gren's syndrome

Pa tie n ts s h o u ld be qu e sti o ne d ab o u t th e o ra l sy mp toms a s o u tlin e d in th e se cti o n a bo ve , bu t

sh o u ld al s o be a sk ed a bo u t o th er sy mp to ms ( Tab le 8.10) a nd dry n es s el se w he re in th e bo d y. th e re is a g en era l iz ed e xo cr in e hy po f un c tio n . M ea sure me n t o f s a li va ry g la n d flo w s h o u ld be

dete rm in ed b y s ia lo m etry , a nd gl an d u la r fu n c tio n i s fu rth er as s es se d by s ci nti sc a n n in g wi th

la be lle d pe rtec h n eta te. S ia lo g ra ph y, u s in g a w ate r -ba se d dye , m ay be i nd ic a ted w h ere th ere

is a h is to ry o r cl in i ca l si gn s in d ic a ti n g po s si bl e s tru c tur al da m ag e o f th e s a li va ry g la n ds . Th is ma y o c c ur a s a res u lt o f s ec o nd a ry c h ro n ic i nf ec tion (Fi g. 8. 6 ). Pe rs is ten t s w el li ng o f th e

gla n ds c a n be in ve s ti ga ted b y s ia l og ra ph y, b u t m o dern tec h n iq ue s , pa rtic u la rly M RI, gi ve a mu c h be tter pic tu re o f s o ft-ti ss u e le si o n s an d a re e s se n ti a l i f a tu m o ur is su s p ec ted . Grea t

em ph a si s h as b ee n pl ac ed o n th e di ag n o sti c im po rta nce o f a la bi al g la n d b io p sy fo r pa tie n ts wi th su s p ec ted S jΓ¶g re n' s sy nd ro m e. Th e n u mb er o f lym ph o c ytic f oc i w ith in th e g la nd s is

th e n m ea su re d an d gr ad ed a cc o rdi n g to th e s c h em e p ro po s ed b y C h is h ol m an d M as o n . Th e re is in c rea s in g ev id en c e, h o we ve r, th at a n u m be r o f pa tie n ts m a y h a ve a n eg ati ve la bi al g la n d bio p sy res u lt wh i le , o n o the r c ri te ria , b ei ng d ia gno s ed a s su f feri n g fro m S S.

A pro b lem i n th e de fin i ti ve di a gn o si s o f S S h a s be en th e va ria b le cr iter ia u se d in d if fere n t ce n tr es a n d th e fa c t th at s o m e o f th es e ha ve b ee n un du ly n ar ro w, in th e se n se th a t th ey

ha ve h ig h s pe ci fi ci ty b u t lo w s en s iti vity . In a dd itio n , m an y re lie d o n li p b io p sy fo r a de fin i te dia g no s is o f S S an d , a s a co n s eq u en c e, th e c on d iti on ma y h a ve b ee n u nd er dia g no s ed i n th e

pa st. S o m e pa tie n ts are n o t p rep are d to u n de rgo l abia l gl an d bi o ps y, w h ic h th e y p erc ei ve a s

a r ela tiv el y u n p lea s an t p ro c ed u re, a lb ei t mi n o r s u rge ry. A b io p sy po s iti ve co n f irm ati o n o f S S rare ly a lter s th e m an a ge me n t o f th e p ati en t a n d, a s a co n s eq u en c e, m a ny a re pre pa red to

ac ce pt a pro vi s io n al d ia gn o s is o f SS . Cu rre n tl y, h ow ev er, l ab ia l gl an d b io ps y is th e pro c ed u re wi th th e g rea te st sp ec i fic ity a nd d ia gn o s tic va lu e fo r th e s a liv ar y c o mp o n en t o f SS . A n

Am eri c an β€“ Eu ro p ea n S S c on s en s u s gro u p h a s n o w r evise d the c rite ria f o r th e cl a ss if ic ati o n of th is s yn d ro me , in c lu di n g th e c o n ce pt o f bi o ps y-ne ga tiv e S S (pr ov id in g th at c e rtai n

au to an ti bo d ies a re pr es en t; s e e Fi gu re 8. 7). Th e cri teri a fo r th e c la ss if ic a ti o n a nd d ia gn o s is of S S ma y, h o we ve r, u n de rgo f u rthe r m o di fi ca tio n s in fu tur e. A u to an tib o di es a re u su a ll y

pre se n t in S S , a n d a ro u ti ne i mm u n e p ro fi le fo r r heu ma to id a n d a n tin u c le ar fa cto rs , a s w ell as a n ti-SS -A an d a nti -SS -B an tib o di es s h o u ld be a rra ng ed , to ge th e r w i th me as u rem e nt o f

se ru m im mu n o gl o bu li n s. S a li va ry d u ct au to a n ti bo d ie s ma y a ls o b e pre se n t in SS , b u t th eir si gn if ic an c e rem a in s u n cl ea r; th e y a re n o t in c lu d ed in th e di a gn o sti c cr iter ia o f S S . A fu l l ha em a to lo gi ca l an d b io c he m ic al sc re en s h o u ld a ls o be u n de rta ken to el im in ate s ys tem ic dis e as es , s u ch a s di ab ete s. P a tie n ts w i th ey e s ym pto m s mu s t b e e xa m in ed b y a n

op h th al mo l og is t. A pre li mi n ary e sti ma te o f xe ro ph tha lm ia c an be ma d e b y i n se rtin g a s ma ll

stri p o f ab s o rbe nt pa pe r i n si de th e l o w er eye li d ( Sc h irm e r tes t) to as s es s the v ol u me o f tea rs pre se n t (F ig . 8.8) . Ind iv id ua l s w h o h a ve c li ni ca l ev id en c e o r sy mp to ms su g ge sti ve o f a

co n n ec tiv e ti ss u e di so rd er sh o u ld b e a s se s se d by a rh eu m ato l o gis t. O ra l s ym pto m s ar e

ma n ag ed a s fo r o th er c as es o f xe ro sto m ia , bu t ma n y pa tie nts w i th Sj Γ¶g re n' s sy nd ro m e h a ve littl e o r n o sa l iva ry fu n cti o n an d ca n o n ly b e g iv en s ym pto m ati c rel ie f i n th e f o rm o f s a liv ar y su b sti tu te s. Rec u rre n t in fe c ti o n s, p a rtic u la rly o f th e pa ro tid g la n ds , c an b e pa rtic u la rly

tr ou b le so m e an d m ay res u lt ul tim a te ly in su rg ic al re mo v al , w ith th e atte nd a nt ris ks to th e fac i al n erv e. If th ere is a n y cl in ic a l s u s pi ci o n o f a tu m ou r de ve lo pi n g i n a n y g la n d, th en

im ag in g an d b io ps y are m a nd a to ry . Ca lc u lu s fo rm ati on is o fte n tro u bl es o me a s a lo n g -te rm res u lt o f ch ro n i c in fe cti o n an d sta s is i n the m a jor s al iva ry gl an d s. A su m ma ry o f th e ma n ag em en t o f a pa tie n t w ith S jΓ¶g re n' s sy nd ro m e i s gi ven in Tab le 8. 12.

Table 8.10 Extra-orofacial signs and symptoms associated with SjΓ¶gren's syndrome Loc atio n of

Descr ipt ion

sym p to m

Oc u la r

Pe rs is ten t, tro u bl es o me dr y e ye s, re c ur ren t s en s a tio n o f

Res pi ra to ry tra ct

Dryn es s o f u p pe r a n d lo w er res pi ra tor y tra c t, dys p ho n i a,

Va gi n al

Va gi n al d ryn es s , bu rn i ng , h is to ry o f rec u rre n t fu n ga l

Sk in

Dry sk in , b u tte rfl y ra s h , va sc u li tis

Gas tro in te sti na l

Dys ph a gia , co n s tip ati o n

tr ac t

sa n d o r gra ve l in th e ey es , n ee d to us e tea r s u bs titu te s da il y

dis tu rba n c es to s en s e o f s me ll

in fe cti on s , p ai n fu l in ter co u rs e

Gen e ral

Fa tig u e, w ea kn e ss , d ep res s io n Sl ee p d is tu rba n c e, l os s o f se xu a l d es ir e, de pre s si on

Th ere is , to d ate , n o ef fe cti ve cu re fo r S jΓ¶g re n' s sy nd ro m e, b u t sy mp to ms c a n u su a ll y b e co n tro ll ed to s o me e xten t b y th e m ea su re s o u tl in e d ab o ve . Pa tie n ts are o fte n re lie ve d at

ob tai n in g a de fin i tive d ia gn o s is a n d m a y be n efi t from m em b ers h ip o f a S j Γ¶g re n' s sy nd ro m e su p po rt g ro u p.

Fig. 8 .7 Th e Sc h ir me r tes t to a s se s s th e ac tiv ity o f th e lac rim al g la n ds .

Table 8.11 Revised international classification criteria for SjΓ¶gren's syndrome* I.

Oc u la r s ym pto m s: a po s iti ve res po n s e to at le as t o n e o f th e fo ll o wi n g

qu es tio n s :

Hav e yo u h ad d ai ly , pe rs is ten t, tro u bl es o me d ry e yes fo r m o re tha n 3 mo n th s?

Do yo u h av e a r ec u rren t s e ns a tio n o f sa n d or gr av el in th e ey es ? II.

Do yo u u s e te a r s u bs titu tes m o re th a n 3 ti me s a da y?

Ora l sy mp to ms : a p o si tiv e re s po n s e to a t l ea s t one o f th e f o llo w i ng qu e sti o ns :

Hav e yo u h ad a d ai ly fe el in g o f d ry m o u th fo r mo re th an 3 mo n th s?

Hav e yo u h ad re c ur ren tly o r p ers i ste ntl y s w o ll en sal iva ry gl an d s as a n a du l t? III.

Do yo u fr eq ue n tly dri n k l iq ui ds to ai d in s w al lo w ing d ry f o od ?

Oc u la r s ig n s, i . e. o b jec tive ev id en c e o f o cu l ar in vo l vem e nt de fin e d a s a

po s itiv e r es u lt f or a t le as t o n e o f th e fo ll o w in g two tes ts:

Sc h irm er 's I tes t, pe rfo rm ed w ith o u t a n ae sth e si a (< 5 m m in 5 mi n ute s )

Ro se be n ga l sc o re o r o th er oc u la r dye s co r e (> 4 a cc o rdi n g to va n IV .

Bi jste rve ld 's s co ri n g s ys tem )

His to pa th o lo gy : i n mi n or sa l iva ry gl an d s (o bta ined th ro u gh no r ma l -ap pe ari n g

mu c o sa )

Fo c al l ymp h o cy tic s ia la de n itis , e va lu a te d by a n ex per t h is to pa tho l o gi st, w i th a fo cu s s c or e > 1, de fin e d a s a n u mb er o f l ym ph o c ytic f oc i (w h ic h a re ad jac e nt

to no r ma l- ap pe ari n g m u c o us ac i ni a n d c o n tai n m o re th a n 50 l ym ph o c ytes ) p er V.

4 mm 2 o f g la n du l ar tis su e

Sa li va ry gla n d in vo l vem e nt: o bje cti ve ev id en c e o f sa l iva ry gl an d in v ol ve me n t

def in e d b y a p o si tiv e res u lt fo r a t l ea st on e o f the fo l lo w in g d ia gn o s ti c tes ts: Un s tim ul ate d wh o l e s a li va ry f lo w (< 1. 5 m l in 15 min u te s )

Pa ro tid s ia lo g rap h y s h o w in g th e p res e nc e o f d if fu se si a lec ta si as ( pu n ctu a te,

ca vi ta ry , o r d es tru cti ve pa ttern ) wi th ou t ev id en c e o f o bs tru cti o n in th e m ajo r du c ts

Sa li va ry sc i nti gra p hy s h ow i n g d el ay ed u pta ke , red u ce d co n c en tra tio n , a n d/o r VI.

del a yed e xc reti on of tra ce r

Au to a nti bo d ie s: pre se n ce i n th e se ru m o f th e fo llo w in g au to a n tibo d ie s:

An tib o di es to R o( SS A ) o r La( SS B ) a n tig en s , o r b o th

Fo r th e d ia gn o s is o f pri ma ry S S:

In pa tie nts w i th o u t a n y p o ten tia ll y as s oc ia te d di sea se , pri ma ry S S ma y be de fi ne d as fo l lo w s :

Th e p res e nc e o f a n y 4 o f th e 6 i te m s is in d ic ati ve o f p rim a ry S S , a s lo n g as

Th e p res e nc e o f a n y 3 o f th e 4 o b jec tive c rite ria item s (th at, is i tem s III, IV ,

Th e c la s si fi ca tio n tre e pro c ed u re rep re se n ts a v al id a lter na tiv e me tho d o f

eith e r i te m IV (h is to pa th o lo gy ) o r V I ( se ro lo g y) i s po s iti ve V, V I)

cl as s ifi ca tio n , a lth o u gh it s h o u ld be m o re pro p erl y u s ed i n cl in ic a lβ €“epi de mi o lo g ic al su rv ey

Fo r th e d ia gn o s is o f s e co n da ry S S:

In pa tie nts w i th a po ten ti al ly as s o ci ate d d is e as e (fo r in s ta n c e, a n o the r w e ll

def in e d c o n n ec tive ti ss u e dis e as e) , th e p res e nc e o f i tem I o r ite m II pl us a n y 2 fro m a mo n g ite ms III, IV, an d V m ay be c o n si de red a s in di c ati ve o f se co n d ary S S

Ex cl u si o n cr iter ia :

Pa s t h ea d an d n ec k ra di ati on tr ea tme n t Hep ati tis C in fe cti o n

Ac qu i red im mu n o d efi ci en c y d is e as e (A ID S) Pre -exi sti n g ly mp h om a Sa rc o id os i s

Graf t ve rs us h o s t d is ea se

Us e of an ti ch o l in erg ic d ru gs (s i nc e a tim e sh o rter th an 3 -fo ld th e h al f- lif e o f th e dru g) .

*S o u rc e: V ital i, C . , Bo m ba rd ie ri, S . , Jo n ss o n , R. , et al . A n n Rh eu m D is

(2002):61:554β €“558. Cla s si fic a tio n c rite ria fo r S jΓ¶g re n' s sy nd ro m e: a revi s ed

vers i on of th e E uro p ea n c rite ria pro p o se d by th e A meri ca n β€“Eu ro p ea n Co n s en s u s Gro u p.

Table 8.12 SjΓ¶gren's syndrome: assessment and clinical investigations His to ry

Dry mo u th

Diff ic u lty e a tin g

Diff ic u lty s w a llo w i ng

Sw e lli n g o f sa l iva ry gl an d s Dry s ki n

Va gi n al d ryn es s

Co n ne c ti ve tis s u e di se a se Ex am in a tio n

Mu c o sa l dry n es s

No po o l in g o f sa l iva o n f lo o r o f th e m o u th Den tal s tatu s Bl o o d tes ts

Fu ll b lo o d co u n t ES R

Im m un o l o gy

In f la mm a to ry ma rk ers Bi o ch e mi ca l pro f ile

Sa li va ry g la n d im a gin g Sc in ti sc a nn i n g Si al o gra ph y Bi o ps y

Lab ia l gl an d b io ps y. Refe rra l to

Op h th a lm o lo g is t

Ph ys i ci an an d /o r rh eu m ato l o gis t

P. 95 P. 96

Typical result s of investig atio ns in S jΓ¶g ren's syndro me

Si al o me tryβ€” lo w s al iva ry fl ow ra te

Lab ia l g la n d bi o ps yβ€” lym ph o c ytic in fi ltra tio n

Au to a nti bo d y s c ree n β€”po s itiv e au to a n tibo d ie s, in parti cu l ar rh eu m ato i d f ac to r,

Sa li va ry gla n d im ag in g β€”sc in tis c an wi ll de mo n s tra te re du c ed tra ce r u p tak e a n d

Sc h irm er tes tβ€” red uc e d l ac ri ma l fl ow rate

an tin u c le ar, SS -A, S S -B

se cre tio n ; s i al o gra ph y w ill s h o w si a lec ta si s

Excessive saliva

An i n cr ea se d sa li va ry flo w ra te is a ls o kn o w n a s sia lo rrh o ea o r ptya li s m a n d, in c o n tras t to xero s to mi a, it i s an u n c o mm o n co m pl a in t. Hyp er sa li va tio n m ay be tra n s ien t o r a ch ro n i c pro b lem . Th e re are se ve ra l re a so n s w h y p ati en ts m ay c om p la in o f an in cr ea se i n th e

pro d uc tio n o f sa l iva , b ut the y are d ue to tw o m a in c au s es : hy pe rs ec reti on an d n eu ro m u sc u la r dys fu n c tio n . E xc es s ive s al iv a i s a fre qu e nt co m pl ain t o f p a tien ts w h o a re w ea rin g a n in tra o ral pro s the s is fo r the f irs t tim e. In fa ct, o n e o f th e co m m on l y u se d me tho d s o f s tim u la tin g

sa li va ry f lo w fo r ex pe rim en ta l p u rpo s es i s to u se an in e rt fo re ig n bo d y w i th i n th e mo u th . Mo s t pa tien ts e ven tu a ll y b ec o me u s ed to th eir n ew d en tu re s or a pp lia n c e a n d du ri ng th is p ro c es s th e e xc es s sa li va ry flo w u su a ll y d is a pp ea rs. In a few pa tie nts , h o w eve r, th is m a y p ro ve an in trac ta bl e pro b le m. In f ec ted o r u lc e rati ve le si o n s in th e mo u th m ay tem p or ari ly ca u s e a n

in cr ea se i n sa l iva ry flo w , w h ic h a dd s to th e d is c o mf or t o f th e in i ti a l co n d itio n . T hi s ca n b e a fea tu re o f p rim ar y h e rpe tic gi n gi vo s to m a titis . A sim ila r e ff ec t is o f te n s ee n in c a rc in o ma o f

th e m o uth , i n w h ic h th e i n cre a se d sa l iva ry flo w m a y b e ac c o mp an i ed by a re du c ed s wa ll o w in g refl ex a nd a c o n sta n t dri bb li n g o f s al iva . It c a n be d iff ic u lt to d is tin g ui s h be twe en hy pe rsa l iva tio n an d d ro o lin g β €” th e ter ms a re n ot syn on y mo u s . In pa tie n ts wi th

hy pe rsa l iva tio n sa l iva i s n or ma ll y c le a red fro m th e mo u th b y s w al lo w in g . Dro o li n g o c c ur s du e to a fa il ur e to s w a llo w s a li va a nd i s co m mo n in i nfan ts a n d a l so i n th o se w ith p o o r

ne u ro mu s c ul ar c oo rd in a tio n . Dro o li n g is n o t n ec es s ar ily c au s ed b y a n o ver pro d u cti on of sa li va , bu t i t c an o c c u r be c au s e of i t.

Ve ry fe w dru g s in du c e ex ce ss iv e sa li va tio n , a s ta rk c on tra s t to th e nu m be r o f dru g s th at

red uc e s al iva ry fl ow rate . A nti ch o l in es ter as es , w h ic h e nh a n ce n e ur om u s cu l ar tra ns m is si o n

an d ar e u s ed i n th e tre a tm e nt of m ya sth e ni a gra vi s , ca n c au s e h ype rs al iv ati on . In te res tin g ly, th e a n ti ps yc h o tic d ru g c l oz a pi n e h a s be en im pl ic a ted in c a us i ng a d ry m o u th a nd hy pe rsa l iva tio n .

Ex ce ss i ve sa li va tio n m ay b e d u e to :

hy pe rse c reti on (fo r e xa mp le , th e p ro vi si o n o f a n e w in tra o ral p ro sth e si s, d ru gs )

ne u ro mu s c ul ar dy sf un c tio n ( fo r e xa mp le , ce reb ra l pals y)

or al dy sa es th es ia ( pa tien ts w ith or al dy sa e sth e si a are m o re lik el y to re po rt xe ro sto m ia )

Sy ste mi c co n d itio n s , mo s t n o ta b ly n eu ro lo g ic al d is tu rba n ce s su c h a s pa rki n so n i sm , c ere bra l pa ls y, a n d ep il ep sy , ca n c au s e pa tie n ts to co m pl ai n o f e xc es s ive s a liv ati o n. In the s e si tu a tio n s th ere m ay b e n o i n cr ea se i n th e p ro du c tio n o f sa l iva b u t sw a ll o wi n g is

un c o o rdi n ate d a n d in e ffi ci en t. M erc u ry po is o n in g a nd ra b ies a re ex trem el y ra re dis e as es th a t ha ve h yp ers a li va ti o n a s a sy mp tom .

Trea tm en t fo r ex ce ss iv e sa li va tio n d ep en ds l ar ge ly on the e lim i na tio n o f (o r h a bi tu atio n to )

th e c au s a ti ve fa c tor , wh e th er i t b e a f o rei gn b o dy o r a n in fe c ti ve le s io n . Th e u se o f dru g s to

su p pre ss s a liv ar y f lo w is ra re ly in d ic ate d si n ce v irtu a lly a ll dru g s w ith a m ark ed s al iv ary

su p pre ss iv e e ff ec t a ls o ex ert o the r, a n d o f ten m o re si gn i fic a nt, e ff ec ts. A n tic h o li ne rg ic dr ug th e ra py is so m eti me s u se d in p ati en ts w ith c er eb ral p al sy w h o dro o l ex ce s si vel y. O n e freq u en t o ra l si de -eff ec t o f s u c h me di c atio n is a n in c rea s e i n c ar ies ra teβ€” of ten i n a pre vio u s ly ca ri es -free d en titi o n. Al tern a tive ly , the m a jor sa l iva ry gl an d du c ts c an b e

red ire cte d to the o ro p ha ry nx to tre at dro o li n g. In a fe w pa tie n ts c o m pl ai ni n g of e xc es si ve sa li va tio n n o i nc re as e in f lo w ra te ca n b e de tec ted. In th is s itu a ti o n th ere m ay b e a n un d erl yin g c o gn iti ve o r p sy ch i atri c di stu rb an c e, and , in de ed , s om e pa tie n ts ma y

dis p la y o b se ss i on a l trai ts. T h e cl in i ci an ca n re as sur e th e pa tie n t th at the re is n o s er io u s

P. 97

mo rb id ity as s oc ia te d w ith th is c o n di tio n a n d s ia l ome tr y m ay b e h e lp fu l in d em o ns tra tin g

sa li va ry f lo w ra tes w ith in th e n o rm al ra n ge . In a mi n o rity o f p ati en ts , be ha vi o u ral th er ap y ma y b e be n efi ci al .


Wh at a d vic e w o ul d yo u g ive to th i s pa tien t co n c ern i ng h is f u tur e o ra l h ea lth ?

Th era pe u tic ra di ati o n ca n i n du c e ch a n ge s to m uc o s al, mu s c ul a r, va sc u la r, a n d os s eo u s

ti ss u es . A lte ra tio n s to sa l iva ry gl an d fu n c tio n a re co m mo n a s i s a s h if t in or al m ic ro bi al fl o ra, res u ltin g in a hi gh e r c o n ce n trati o n o f c a rio ge n ic bac ter ia . Th e irra di a te d pa tie n t h as a n

in cr ea se d ris k of d eve lo p in g ca ri es . Cl as s ic al ly thes e o c c u r a t th e c u s p ti ps , in c is a l e dg es ,

an d ce rvi c al th ird s o f th e cr ow n . Th e o n c ol o gi st, ra di oth e rap is t, a n d d en tis t c o m pri se a tea m

of h ea lth -ca re pro f es si o n al s wh o s e co o rd in a ted ef forts a re req u ire d f o r e ffe cti ve ma n a gem e nt of th e rad io th er ap y pa tie n t. A pre -th e ra py de nta l ev al u atio n is es s en tia l fo r th e p ati en t

un d erg o in g ra di ati on th e ra py tha t m a y a ff ec t th e o ra l c av ity a n d /o r s al iva ry gl an d s . Th e ob jec tiv e sh o u ld b e to e s ta b lis h g o o d o ra l h ea lth for th e p a tien t pri or to ra di ati on tr ea tme n ts β€” fai lu re to d o s o co u l d r es u lt i n co m plic a tio n s.

Tee th th at r eq ui re ex tr ac tio n s h o u ld be re mo ve d as s o o n a s po s si bl e. It is a dv is ab le to l ia is e wi th th e o n c o lo gy u n it to di sc u ss th e tim in g o f ex trac tio n s . Ra di oth e ra py to th e ja w s ca n

ma ke a pa tie n t s us c ep tib le to o ste o rad io n e cro s is f ollo w i ng o ra l su rg ery (i nc l ud in g ex o do n tia , es pe ci al ly w h en ma n di bu l ar tee th req u ire ex trac tio n). T ee th w ith a lo n g -te rm qu e sti o n ab le pro g no s is s h o u ld be re mo v ed pre fe ra bly b efo re ra di oth era py c om m en c es .

Th e p a ti en t s h o u ld be w a rn ed th at sa li va ry flo w m a y re du c e dra ma tic a ll y fo ll o w in g

rad io th era p y a n d th e c o n se qu e nc e s of th is o n o ro d enta l h e al th sh o u l d be d is cu s s ed w ith th e pa tien t. T he p ati en t s h o u ld em ba rk u po n a n in te n si ve pre ve n ti ve p ro gra mm e β €”or al h ea lth

edu c a tio n is o f pa ra m ou n t i mp o rtan c e. Nu triti o na l gu i da n ce s h ou l d be o ffe red a n d th e u s e o f

flu o ri de th era py co n s id ere d. T h e c li n ic ia n s h ou l d rea li z e th a t c o mp lia n c e w i th su c h a re gi me n ma y be a p ro bl em , es pe ci a lly if th e pa tie nt di d n o t m a in tai n a n o ra l s el f -ca re pro g ra mm e

pri or to ra di o the ra py. Th ere fo re trea tm en t p la ns s ho u ld be re a lis tic . Ro o t c an a l trea tme n t is no t c o n tra in di ca ted i n pa tie n ts wh o a re to u n de rgo ra di oth e rap y. S i al og o gu e s ca n b e

ben e fi ci al in c lu d in g th e u s e o f s ys tem ic pi lo c ar pine . Th e do s e reg im en a n d si de -eff ec ts fo r th i s dru g c an b e o bta in ed fro m th e Bri tis h Na tio n a l F o rm u la ry o r th e ma n u fa ctu re r's d ata sh e et.

Lon g -te rm pa tie n t fo l lo w -up i s n ec es s ary o n th e pa rt o f bo th th e de n ti s t an d h yg ie n is t. Th e pa tien t ma y a l so ex pe rie nc e n o n -sp ec if ic o ra l m u co siti s fo ll o w in g rad ia tio n a n d w ill b e

su s ce pti bl e to o r al c an d ido s is . P o s t- irra di ati o n sia la de ni tis i s al so a po s si bl e si de -eff ec t.

SCase 8.2 Discussion Q1

Wh at q u es tio n s w o ul d yo u a sk th is l ad y?

Qu es tio n s s h o ul d be a sk ed to a ss e ss th e se ve rity o f th e dr y m o u th . th e qu e sti o n s lis ted i n Tab le 8. 8 ca n h e lp id en tif y i f a p a ti en t h a s o ra l dys fu n c ti o n as s o ci ate d wi th s al iva ry gl an d hy po fu n c tio n . A fu l l d en ta l a n d me di ca l h is to ry sh ou l d b e take n . It is a l so h e lp fu l to do a

die tar y a n al ys is β€” th e in c id en c e o f ca ri es c o u ld inc rea s e a s a re su l t o f m o re fre qu e nt in tak e of c arb o h yd rate . P ati en ts w ho de vel o p a dry mo u th may e rro n eo u sl y us e mi n ts o r c itru s -

fla vo u re d sw e ets to o b tai n sy mp to ma tic re li ef. A full m ed ic al h is to ry sh o u ld b e ta ke n a nd sp ec if ic qu e sti o n s th a t e lic i t th e s ym pto m s o f S jΓ ¶g re n' s sy nd ro m e s h o u ld be u se d, f o r

exa m ple , d o yo u h a ve dry ey es ? do yo u h av e rh eu ma toid a rth riti s? A th or o ug h d ru g h i sto ry

sh o u ld b e o b tai n ed. If yo u are i n do u bt as to th e si de -eff ec ts o f a n y m ed ic a tio n , a ref ere n ce bo o k s u c h as th e Bri tis h Na tio n a l F o rm u la ry sh o u l d b e co n s u lted . Th e lo c al an d re gi o na l UK Me di ci n es In fo rm ati o n se rvi ce w i ll al so be h el pfu l . Q2

Wh at a d vic e w o ul d yo u gi ve ?

Th e tre a tm e nt o f th is p ati en t w i ll de pe n d u p o n th e a e ti o lo g y. If sa li va ry g la n d h ypo f un c tio n is s us p ec ted , the n th e pa tie n t s ho u l d e mb a rk u p o n an in ten s iv e p rev en tiv e reg im e ( or al hy gi en e in s tru cti o n, die tar y a dv ic e, f lu o rid e su p plem en ts , si al o go g ue s , an d p os s ib ly

pil o ca rp in e) . Th e pa tie nt ma y m e rit re fe rra l to an o ra l me di ci n e d ep a rtme n t if yo u s u sp ec t un d erl yin g s ys tem ic d is ea s e. Q3

Wh at i s th e m o s t fre qu e nt ca u se o f xe ro sto m ia ?

Th e m o s t fre qu e nt ca u se o f xe ro sto m ia i s th e in ta ke o f dru g s w i th an ti ch o l in erg ic a c tivi ty.

Case 8.3 Discussion

Th e p rec i pita tin g a n d r eli ev in g fa cto rs i n th e pa in h i sto ry ar e s u gg es tiv e o f a n o bs tru cti o n in

a s u bm a n di bu la r du c t. Th e ac c u mu la tio n of s al iv a p ro xi ma l to th e b lo c ka ge i n th e d u ct ca u se s pa in a n d sw e lli n g. Th e s ym pto m s are w o rs e j us t p rior to a me al , w h en th e re i s a ris e in th e pro d uc tio n o f s al iva . Th e o bs tru c tio n i s mo s t li ke ly to be du e to a sa l iva ry sto n e (c al cu l u s, si al o lith ). H o we ve r, o th er ca u se s co u l d b e a mu c o u s pl u g, d u cta l s te no s is , o r a n e o pl as m . Q1

Wh at f u rth er q u es tio n s w o u ld yo u a s k th is pa tie n t?

Yo u w ou l d ne ed to e xp an d u po n th e pa in hi s to ry a n d e s ta b lis h i f th e pa in is p rec ip ita ted by

an y o th e r g u sta to ry o r o lfa c to ry s tim u li . It is u n li ke ly tha t th i s pa in h a s a sp o n tan e ou s o n s et. Is th e pa in bil a te ra l? S ia lo l ith s ar e u s u al ly u n ilater al β€” aff ec tin g o n ly o ne s a liv ar y gl an d . Q2

Ho w w o ul d yo u ex am in e th is p ati en t?

Yo u sh o u ld p al pa te th e s u bm a n dib u la r g la n d an d du c tβ€”bo th ex tra - an d in tra o ral ly

(bi ma n u al pa lp a ti o n ). Th e si z e o f th e sa li va ry g la nd sh o u ld b e c o m pa red w ith th a t o f the co n tra la tera l gl an d an d th e du c t s ho u l d b e pa lp ate d fo r a s to n e o r an y irr egu l ar itie s o f co n to u r o r c o n si s te n cy . A pl a in o c cl u sa l rad io g ra ph m ay b e u se fu l

P. 98

in id en tif yin g c al c ul i. H o we ve r, th ey are n o t a ll ra di o -op a qu e. S i al o gra ph y ma y be req u ire d to ide n tify th e lo c ati on o f th e o bs tru cti o n. A sc in ti sc a n is a fu n c ti o n al a ss es s me n t o f s a li va ry

gla n d ac tiv ity an d is o f li mi ted di a gn o sti c va lu e . Ul tras o n o gra ph y c an b e u se fu l in i de nti fyi n g th e s ite o f a n y ob s tr uc tio n an d th e pre se n ce o f ca lcu li . It m ay a ls o in d ic ate th e pre se n ce o f an e xtra - or in tra gl an d u la r tu mo u r. Q3

Ho w w o ul d yo u m an a ge h im ?

Th e m a n ag em en t w o u ld de pe n d u p o n th e c a u se o f th e obs tru c tio n . Th e pa tie n t req u ire s

refe rra l fo r sa l iva ry gl an d im a gi ng a n d rem o va l o f th e o b s tr uc tio n . A s a liv ar y d u ct c a lc u lu s ca n s o me tim e b e su c ce s sf ul ly β€ mi lke dβ €™ al o ng th e du c t a n d m a ni pu l ate d o u t o f th e

or ifi ce . A c al cu l u s lyi n g i n th e su b ma n di bu la r d u ct tha t ru n s s u pe rfi ci al ly al o n g th e fl o o r o f

th e m o uth c a n be re mo v ed by i nc is i ng th e o ver lyi n g mu c o s a β€” ta ki n g c a re n ot to da m ag e th e

lin g u al n erv e. T he i nc i si o n is o fte n le ft u n su tu red to m in i mi z e p o ss ib le d u ct o b str uc tio n d u e to sc a rrin g . Lith o trip sy h a s be en u s ed f or la rg e s ali va ry c a lc u li. S u b ma n di bu la r sa li va ry

gla n ds r eq ui re su rg ic a l e xc is io n i f th e y a re s eve rely da ma ge d an d a ss o c ia ted w ith p ers is ten t sym p tom s . Q4

Is th i s pa tie nt li kel y to co m pl ai n o f a d ry mo u th?

In a h ea lth y pa tie nt, x ero s to mi a is u n li kel y to ac co mp an y a co n d itio n th a t a ffe c ts o n ly o ne ma jo r s al iv ary g la nd . It i s us u a lly a s ym pto m o f m ulti gl an d ul a r di se a se .

Project 1.

Fi nd o u t a bo u t th e c lin i ca l pre s en tati o n , h is top ath o l o gic a l f ea tu res , a n d m a n ag em en t o f

tu m o u rs th at m a y af fe ct th e s al iv ary gl an d s .

Au th o rs : Title:



Co py rig ht Β © 2003 Ox fo rd U ni ve rsi ty P re ss > T ab le o f Co nt e nt s > 9 - Inf lam m at o ry ov e r g r ow t hs , d e v e lo p m e nt al and b e nig n le s io ns , and pig m e nt at io n of th e o ra l m uc o sa

9 Inflammatory overgrowths, developmental and benign lesions, and pigmentation of the oral mucosa Problem cases Case 9.1

A 30-yea r-ol d Ca uc a si a n ma n a tten ds y o ur de n tal pr ac tic e fo r th e fi rst tim e. He i s wo rr ie d

ab o ut a pi gm en ted p atc h th at h e h a s n o tic ed o n th e ro o f o f h is m ou th β€” th i s h as c au s ed n o sym p tom s . Th e pa tie n t is f it a n d we ll a nd n o t ta ki ng a n y m ed ic a tio n . Hi s fa th e r, h o w eve r,

die d o f m a lig n an t me la n om a o n th e sk in w h en th e p ati en t w a s a bo y. E x am in a tio n rev ea ls a n is ol a te d, sm al l (3β€“4 mm i n di am e te r), b ro w n pi gm en te d m a cu l e, o n th e p a la ta l mu c o s a. Th e r es t o f th e o ra l m u c os a a pp ea rs c om p lete ly n o rma l an d th ere a re no pa lp ab le ly mp h no d es . Q1

Wh at i s th e d if fer en tia l di ag n os i s o f th is l es io n a n d ho w w o u ld yo u m an a ge th is c a se ?

Case 9.2

A 75-yea r-ol d ed en tu lo u s la dy a tten ds f o r pro v is io n o f rep la c em en t f ul l de ntu re s. Her exi sti n g d en tu re s are 20 y ea rs o ld an d i ll -fittin g . In th e pa st, s h e h ad s u ffe red s o me

dis c o mf or t β€ wh e re th e to p de n tur es h a d d ig ge d in β€™, b u t th is ha d e ve n tu a ll y s e ttl ed

do w n . At the ti me o f yo u r e xa mi n ati o n sh e is a s ym pto m ati c. H er exi sti n g d en tu re s are

dis c o lo u red a n d th e te eth b ad ly w o rn . E xa mi n atio n of th e o ral m u co s a rev ea ls a s o ft -ti ss u e les io n , e xten d in g a lo n g th e b uc c al s u lc u s, a d jac en t to the l eft ma xi lla ry tu be ro si ty re gi o n. Th is c o ns i sts o f mu lti pl e fo ld s o f β € hy pe rpl as tic -lo o kin g β€™ mu c o s al tis s ue . Q1

Wh at i s th e m o s t li kel y cl in ic a l d ia gn o s is o f th is la d y's le s io n ?

Q2

Ho w is th is l es io n ma n a ge d?

Q2

A mo re s in is ter o ral l es io n c a n o cc a si o n al ly pre se n t in a si mi la r w a y. Wh a t is th i s an d ho w c a n it be di ffe ren ti ate d f ro m the m o re co m mo n , b en i gn o ra l le si o n ?

Inflammatory overgrowths

Al l n e w gr ow th s o f th e o ra l ti ss u es s h o u ld be trea ted w ith s u s pi ci o n an d m u st b e fu ll y

in ve sti ga ted . Th ere a re so m e w el l -def in e d c o n di tio ns (fo r in s ta n c e, d en tu re gra n u lo m as ) o f

wh i ch th e c li ni ca l di ag n o si s ca n b e a c cu ra te in p racti ca ll y a l l c a se s, bu t a pp ea ra n ce s ca n b e

mi sl ea di n g a n d it c a nn o t b e to o stro n g ly stre s se d th a t a bs o lu te c erta in ty as to th e n a tu re of an y le si o n ca n o n ly c o me a fter h is to lo gi ca l ex am in atio n .

Epulides

An e pu l is (p lu ra l, e pu l ide s ) i s def in e d a s a so f t-ti ss u e sw e ll in g o f th e gi n giv al m ar gi n. T h e te rm is m o re sp ec if ic a lly u s ed to de s cri be a ra n ge o f h y pe rpl as tic i n fla m ma to ry l es io n s

ari si n g f ro m th e pe rio d o nta l tis su e s. Th ey rep res e nt an e xa gg er ate d i n fla m ma to ry r es po n s e of th e pe rio d o nti u m, a lth o u gh the s o u rce o f irri tati o n is n o t a lw a ys o bv io u s . Th e

in fl am ma to ry pro c es s a ris es i n itia ll y i n th e in terden ta l ti ss u es , a n d th e re is o ften an

as so c ia te d l o ss o f al ve o la r c res t-bo n e th a t m ay b eco me s eve re in ad va n ce d ca s es . Th re e ty pe s, th e fi bro u s e pu li s, th e p yo ge n ic g ran u lo m a (pr eg na n c y e pu l is ), an d th e gi an t-ce ll epu l is , a re c o m mo n ly s ee n , ref lec tin g di ffe rin g s tag es o f th e i n fla m ma to ry a n d bo n e -

res o rbi n g p ro c es s. M o s t ep u li de s ar e m o re co m m on in f em al es th a n ma le s a nd u s u al ly o cc u r an ter io r to th e m ol a r tee th.

Fibrous epulis

Th e f ib ro u s e pu l is p res en ts a s a pe du n c ul a te d o r ses si le s we ll in g o n th e g in g iva e a nd

es se n tia lly c o n si sts o f h ea vil y f ib ro se d gra n u la tion ti ss u e. Its c o n ten t o f co l la ge n fi bre s gi ves it a fi rm , ru bb ery tex tu re a n d its c ol o u r is u s u al ly pa l e p in k. T h e d eg ree o f fib ro s is d ep en ds , ho w e ver, on the s tag e o f m atu ri ty o f th e e pu l is , and a l es io n i n its e arl y sta g es m ay be s o ft in tex tu re a n d w ith a h is to lo g ic al a pp ea ra n ce th at sh o w s ve ry ma n y ce ll s. In a l o n gs tan d in g les io n , f o ca l mi ne ra li za tio n a n d/ o r bo n e fo rm a tio n ma y o cc u r. A cu te in f la mm a to ry c h an g es

ma y f o llo w tr au m a or in f ec tio n a n d, i n su c h c a se s, th e ep u li s ma y bec o m e r ed an d s o re ( Fi g. 9. 1).

Pyogenic granuloma and pregnancy epulis

Th es e are b oth va sc u la r ep u lid es a n d pre s en t c li ni cal ly as re dd is h -pu rpl e sw e lli n gs o n th e

gin g iv ae . A pre gn a nc y ep u lis i s h is to lo gi ca l ly id entic a l to th e py o ge ni c gra n u lo m a bu t o cc u rs in p reg n an c y ( se e Ch ap ter 13 ). In th e p yo g en ic gra nu lo m a an d p reg na n c y e pu l is th e

gra n u la ti o n tis s u e re m ai ns v as c u la r a n d im ma tu re. It is m u ch r ed der in co l ou r tha n th e

fib ro u s ep ul is p ro pe r a n d ha s a ten d en c y to b le ed ea s il y b ec a us e o f i ts h igh va sc u la r c o n ten t.

The giant-cell epulis (peripheral giant-cell granuloma)

Th e gi an t -ce ll ep u lis i s a le si o n in w h i ch th e gr anu la tio n ti ss u e is o s teo ge n ic i n n atu re . Its pre do m in a nt hi s to l o gi ca l fea tu re is th e

pre se n ce o f mu lti n u cl ea ted gi an t c e lls d is p ers ed in a va sc u la r s tro m a. Wi th m atu ri ty th e

les io n ma y b ec o m e le ss v as c u la r a n d m o re fi bro s ed an d m a y i n cl ud e so m e are a s of b on e fo rma tio n . In its i mm atu re fo rm , th is e pu l is is c h arac ter is tic al ly red - pu rpl e in co l o u r.

P. 102

Fig. 9 .1 A rel a ti ve ly ea rly an d i mm atu re fi bro u s e pu li s .

Ep ulid es Fi bro u s ep u li s Va s cu l ar ep u lid es Py og en i c gra n u lo m a Pre gn a n cy ep u li s Gia n t ce ll e pu li s (pe rip h era l gi an t-ce ll gra n u lo m a)

Management

Trea tm en t o f al l o f th es e fo rm s of e pu li s is b y l o ca l ex ci si o n. Th e o ri gi n o f th e le s io n i s o f ten in terd en ta l a n d, in m o re ad va n ce d ca s es , th e per io do n ta l m e mb ran e m ay be q u ite de ep ly

in vo lv ed . If ex ci si o n is n o t c o mp le te, th er e m a y b e rec u rren c e o f th e le si o n a nd s o , a lth o ug h rad ic a l s u rgi c al tec h n iq ue s a re n o t c a ll ed fo r, th e in i ti a l re m ov al s h o ul d in c lu d e a ll a ffe c te d

ti ss u e. With re pe a te d rec u rre nc e , it i s so m eti me s nec es s ary to re mo ve th e ad ja ce n t tee th in or der to s ec u re the e li mi na tio n o f th e ti s su e o f o rig in . In th ei r te n de nc y to rec u r, e pu li de s

ma y a p pe ar to be n eo p la sm s , bu t th e rec u rre n ce is du e on l y to p er si ste n ce o f th e c o n diti o n s th a t c au s ed th e in i ti a l ab n o rma l res p o ns e . Th e ti min g fo r th e ex ci si o n o f a p reg n an c y epu l is is fu rth er di sc u s se d in Ch ap ter 13.

Al tho u g h th e cl in i ca l di ag n os i s ma y b e a co n fi de n t o n e, i t s ho u l d a lw a ys b e co n fi rme d by hi sto l o gic a l exa m in a ti o n . Oc c as io n a ll y, a n eo p la s m m a y p res e nt in a f or m res e mb li ng a

si mp le ep u li s an d in a l ik ely s ite fo r on e . It s h oul d al so b e rem em b ere d th a t a c en tra l gia n t ce ll gra n u lo m a ma y pe rfo ra te a lv eo l ar bo n e an d ap pea r a s an e pu l is . A pp ro pri ate

in ve sti ga tio n s (in c lu d in g bl o od te sts f or pl as m a calc iu m , p ho s ph o ru s , a nd a lk al in e

ph o sp h ata s e) sh o u ld b e c a rrie d o ut o n al l pa tie nts p res e nti n g w i th a h is tol o gi ca ll y co n fi rme d gia n t-ce ll ep u lis to ex cl u de h yp erp ara th yro i di sm . An y s ym pto m s o r h is to ry th a t m ig h t im pl y un d ia gn o s ed h yp erp ar ath yro i di sm (s u c h as r en a l c a lcu li) s ho u l d b e tak en i nto co n s id era tio n (Ch ap ter 18).

Fibroepithelial polyp

Th is le s io n , si m ila r i n str uc tu re to th e m a tur e f ibro us e pu l is , is e ss e nti al ly s ca r ti ss u e

pro d uc e d a s a res p on s e to tra um a , su c h a s rep ea ted i rrita tio n o f th e b u cc a l o r la bi al m u co s a , al on g th e o cc lu s a l p la n e o f th e te eth , o fte n c au s ed b y a b ite . It is u s u al ly se en in a du l ts an d th e re is n o s ex di ffe re nti ati o n. Th e l es io n a p pe ars a s ei th er a se s si le o r a p ed u nc u la ted

sw el li n g ( Fi g. 9. 2) a nd i s qu i te f ree o f sy mp tom s un le ss s ec o n da ri ly tr au m ati z ed . Th e u s u a l si ze o f su c h a le si o n w h en th e pa tie n t pre s en ts fo r tre a tm e nt is o f th e o rd er of 1 c m in

dia m ete r, bu t o c c as io n a l l o n gs tan d in g le si o n s are see n th at are ve ry mu c h la rg er. Th e c o lo u r of th e les i on is p in k a n d th e te xtu re va rie s fro m so ft to ru bb ery , de pe n di ng o n th e ma tu rity of th e co n s titu en t f ib ro us ti ss u e. Si n ce th is le si on is s im pl y an e xa gg era ted a n d ch ro n ic a ll y irri tate d m a ss o f sc a r ti ss u e, trea tme n t n ee d o nl y b e co n s erv ati ve , exc i si on to th e l im it o f th e s w ell in g o r to th e b a se o f th e p ed ic le be in g a ll tha t i s req u ire d. Re c ur ren c e wi ll o c cu r

on l y if tra um a is r ep ea ted an d i s, i n fa c t, un c o mm on . A s wi th a ll o the r ti ss u e o ver gro w th s, ab so l ute c erta in ty o f d ia gn o s is c a n co m e o nl y afte r h i sto lo g ic a l ex am in a tio n , a lth o u gh a

cl in ic a l d ia gn o s is c a n o ften be ma d e w i th a fa ir d eg re e o f c o n fid en c e. An i n tere sti n g c l in ic a l var ia n t is o n e in w h ic h a f ibr oe pi th el ia l le si o n (th e so -ca ll ed β € lea f fib ro m a β€™) de vel o ps un d er the p al ate o f an u p pe r de n tur e. In s u ch ca s es th e no rm a l s h a pe o f th e le s io n is

dis to rted a n d fla tten e d in to a th in dis c th a t fits i n to a sh a ll o w de pre s si o n in th e pa la te. It

reta in s a pe di cl e an d m ay be d is pl ac ed d o wn w a rds o n i t li ke a h in g ed fl ap .

Fig. 9 .2 A la rg e fi bro e pith e li al po l yp o f th e bu c c al mu c o s a.

P. 103

Denture granuloma

Th is is a l es io n e s se n ti a lly si m ila r to th e fi bro e pi th eli a l p o lyp , th e irri tati ng fa c to r in thi s c as e bei n g th e f la n ge o f a n o ve re xten d ed o r i ll -fittin g d en tu re. A s i n th e c a se o f the po l yp,

pro li fe rati ve s ca r ti ss u e is fo rm ed f ol lo w in g c h ro nic tra um a . Th e typi c al fi ss u red s h ap e o f th e den tu re g ran u lo m a re su lts f ro m the i nd en ta tio n c a use d b y th e f la n ge o f th e de n tu re ( Fi g.

9. 3). Th es e le si o n s a re ra rel y p ai n fu l an d , in de ed , o fte n ca u s e a s to ni sh i n gl y l ittle tro u bl e to th e pa tie n t. Th is b ei ng s o , o cc a si o n al le s io n s are s ee n th at are v ery la rg e i n de ed , w ith

mu lti pl e f o ld s of p ro lif era tiv e ti ss u e. Th e d en tu re gr an u lo m a is a b en ig n le si o n a nd i s trea ted by si mp le e xci s io n a fter rem o vi n g th e o ffe n di n g d entu re o r dra s tic al ly trim m in g it a w ay fr om th e a ffe cte d are a . Th e rem o va l o f th e s o u rc e o f c h ro n ic i rrita tio n i s in m an y c as es s u ff ic ie nt

to red u ce c o ns i de rab ly th e si z e of th e les i on wi th in a re la tiv ely s h o rt tim e an d ev en to m a ke exc is io n u n ne c es sa ry.

Focal epithelial hyperplasia (Heck's disease)

Th is c o nd iti o n (o th erw is e kn o w n as H ec k's d is ea s e) h as b ee n fu ll y d es c rib ed a n d i n ve sti ga ted

on l y i n re ce n t ye ars . Th e p ati en ts ar e p red o mi n an tly c h il dre n fro m B la c k A fr ic an , E s kim o , a n d Am eri c an In d ia n gr ou p s, al tho u g h a ve ry f ew c a se s hav e b ee n re po rted i n Wh ite E u ro pe an s .

In th i s co n d itio n , m u ltip le ra is e d s es s il e l es io n s a pp ea r o n th e bu c c al a nd l ab ia l mu c o sa . Th e mu c o sa re tai n s a rel ati vel y n o rm a l p in k ap pe ar an c e an d th e textu re o f the l es io n s is s o ft. Th ere is n o u l ce ra tio n (e xc ep t in th e ca s e of s ec o nda ry tr au m a) a nd th e le si o ns a re q ui te pa in -free . Hi sto l og ic a ll y, th e e pi th eli u m, o v erl ying a re la tive ly n o rma l co ri u m, a p pe ars

hy pe rpl as tic , w ith ma rk ed ce ll u la r i rreg u la riti es . Th is c o n di tio n i s as s oc ia te d w i th sp ec if ic ty pe s o f th e h u ma n p ap il lo ma vi ru s , al tho u g h th ere is th o u gh t al so to be a ge n etic ba s is

res po n s ib le fo r i ts h ig h in c id en c e i n th e gro u ps m en ti o ne d ab o ve . No trea tme n t is n e ce s sa ry. It is a s el f-lim iti n g c o n di tio n a n d r eg res se s c om p lete ly af ter a va ria bl e p er io d.

Fig. 9 .3 A de n tur e gra n u lo m a in th e lo w er bu c c al su l cu s .

Developmental lesions Hamartomas

A ha m arto m a is a l o ca li z ed n o n- pro g res si ve tis s u e abn o rm a lity re su l ti n g fro m a d efe ct in dev el o pm en t. It i s ne ith e r in f la mm a to ry n o r n e o pla stic in n atu re b ut, s in c e it m a y b e

co n fu s ed w ith c o n di tio n s o f e ith er typ e, i t sh o u l d be c o n si de red in rel ati on to th em . Wh en

pre se n t o n s kin o r mu c o u s me mb ra n e th e term β € na ev u s β€™ is o fte n u se d eve n w h en th e

na ev u s ce ll s (th e m el an o c yte s) ar e in n o w a y in vo l ved . In s u ch a l es io n , a s in g le el em en t o f th e m u co s a, ep ith el ia l, v as c u la r, o r l ym ph a tic , is p red o mi na n tly in v ol ve d.

Angiomatous naevae

Th es e are th e res u lt o f de ve lo p me nta l ab n o rma li tie s in ei the r th e ly mp h ati c o r va s cu l ar

co m po n en ts o f the m u co s a . Va s cu l ar n ae va e (h ae m an g io ma s ) a re rel a ti ve ly co m mo n les i on s

of th e or al m uc o s a res em bl in g th e β€ stra w be rry ma rk β€™ of s ki n. De pe n di n g o n th e de gr ee

of d ila ta ti o n o f th e ab n o rma l bl o o d v es se ls , th e lesi o n ma y ap pe ar a s a f in e n etw o rk of

ca pi ll ari es o r a s a mo re p ro n ou n c ed n o du l ar stru c ture, u s u a lly fi ll ed w ith s lo w - mo vi n g ve no u s blo o d a n d, th ere fo re , da rk- blu e in co l o ur . Th e ca vern o us f o rm ma y be mi s ta ke n c li ni c al ly fo r a m e la no m a , bu t c a n be q ui c kly di ffe re nti ate d by its ten d en c y to b la n ch on pre ss u re . Th is

ca n be s ee n b y p res s in g a gl as s s lid e do w n o n to the su rf ac e. It sh o u ld b e e mp h as i ze d tha t

th e va s cu l ar n ae vu s is a s ta ti c de ve lo pm en ta l ab n o rma li ty th a t is a s ym pto ma tic a n d is b es t lef t u n di stu rb ed . A si m ila r n a ev u s in vo l vin g th e l ym ph ves s el s is th e l ym ph a n gi o ma . It

co n s is ts of a co l le cti o n o f d il ate d l ym ph a tic ve s sels a n d s p ac es i n a c o nn e cti ve tis su e s tro ma

an d is v ery si mi la r to th e va s cu l ar n ae vu s in stru ctu re. A lth o u gh it c a n ap pe a r o n a ny p art of th e o ra l m u c os a , it is s ee n mu c h m o re c o m mo n ly o n th e ton g u e th a n el se w h ere . If th e le si o n is s up er fic ia l it a p pe ars a s a tra ns l uc e nt wh i te str uc tu re o n th e m u c os a . If i t is d ee pl y

si tu a ted th e o ver lyi n g s u rfa c e o f th e to ng u e ap pe ars gre yis h a n d no d u la r. Th is i s al s o an en tire ly in n o c uo u s le si o n .

Th ere m ay be p ro bl em s o f b le ed in g as a re s ul t o f tra um a to th es e le si o ns a n d ac tio n ma y b e ca ll ed fo r, a lth o u gh the b le ed in g ra rel y re a ch e s dan ger o us p ro po rti o ns i n th es e

ci rcu m s ta n c es . Cry o su rg ery h as be en u s ed to d ea l w ith th is s itu a tio n i n the p as t, a lth o u gh

no t w i tho u t s o m e di ffi cu l tie s. T he re ca n b e pro b lems w ith p o stc ryo th e rap y oe de ma o f th e to n g u e. Th e de vel o pm en t o f la s ers h a s, ho w e ver , provi de d an a n s we r to th i s pro b le m. If

th e re is a n y d o ub t a s to w h eth er o r n o t a le s io n i s a h a em a n gio m a , bi o ps y ex am in a tio n m ay be n ec es s ary , bu t a n gi o ma to u s les i on s o f te n b lee d co p io u s ly. N o su s p ec t va s cu la r les i on s sh o u ld ev er be s u bje cte d to an y fo rm o f s u rg ery ex ce pt in h o s pi tal .

Developmental white lesions

A nu m be r o f ge n etic a ll y d ete rmi n ed w h ite le si o ns o f th e o ra l mu c o sa e xi st, s ev era l o f w h ic h are a ss o c ia ted w ith l es io n s o f o th e r mu c o u s me mb ra nes o r o f th e s ki n . Th es e ar e ch a rac te riz ed b y d is tu rba n ce s o f ke rati n iz ati o n a nd a re o ften cl as s ifi ed a s

β€ gen o k era to se s β€™ or β€ gen o d erm a to s e s β €™ in th e de rma to lo g ic al l iter atu re . P. 104

Oral epithelial naevus (white sponge naevus)

Th e b es t-kn o wn les i on af fe cti ng th e o ral m u co s a a lon e i s th at of th e or al ep ith e lia l n ae vu s

(wh i te sp o n ge n a evu s ). Th e de s ig na tio n β€ wh i te f o lde d gi ng iv o sto m ati tis β€™ ha s a ls o be en

us e d w i th ref ere nc e to th is c o n di ti o n , bu t th e fi rst us e o f th i s term w a s to de s cri be th e o ral

les io n in a m u ltip le a bn o rm al ity in vo l vin g o th er muc o u s me mb ra n es . Th es e term s a re,

ho w e ver, of ten u s ed i nte rc ha n ge a bl y, a nd i t ma y w ell be th a t co n d itio n s a ffe c ti n g th e o ra l mu c o sa a lo n e rep res e nt an in co m pl ete ex pre ss io n of a m o re g en e ra liz e d m u c o sa l ab n or ma li ty.

Wh ite sp o n ge n a evu s i s a lo c al iz ed d eve lo p me n tal tis s ue a bn o rm a lity th at is n ei the r

in fl am ma to ry no r n eo p la sti c. W hi te sp o ng e n ae vu s a pp ea rs as a f o ld ed w hi te le si o n o f th e or al m uc o s a th a t m ay b e s o e xten s iv e as to a ff ec t th e w h o le o f th e o ral c a vity . It ma y be

pre se n t fro m b irth , bu t in c erta in ca s es th e co n d itio n ma y be co m e evi de n t o n ly in l ate r l ife . Th ere h a s b ee n , in th e p as t, s om e do u b t as to th e exa ct g en e tic m ec h an i sm i nv o lve d in th e pro d uc tio n o f th is l es io n , bu t it h a s n o w be en d em on stra te d th a t th e co n di tio n d is pl ay s

cl as s ic al d om in a n t tra ns m is si o n c ha ra c te ris tic s i n so m e, b u t n o t a ll pa tie n ts. In o th er c as es th e re is n o f am il y h i sto ry a nd th e co n d itio n a p pe ars to a ri se i n is o la tio n . Th e re a re n o

rep or ted in s tan c es o f ma l ign a n t tra n sf or ma tio n i n th es e les i on s , a n d th e y a re ge n era ll y

reg ard ed a s be in g en ti rel y b en ig n . Hi sto lo g ic a l e xami n ati o n o f th e af fe cte d o ra l mu c o s a

sh o w s the c o n si ste n t pr es en c e o f a ca n th os i s wi th para ke rato s is a n d in tra ce llu l ar o ed em a.

Ch ara c teri sti c o f th is l es io n a n d al s o o f m a ny s im il ar le si o ns i n th e mo re c o mp le x m u co s a l syn d ro me s is th e pr es en c e o f ma n y la rge c le ar ce ll s exte n di n g th ro u gh o u t th e s tr atu m sp in o s um a n d to the su rf ac e o f th e ep ith e liu m .

Pachyonchia congenita, benign intraepithelial dyskeratosis, and dyskeratosis congenita

In pa ch y on c h ia c o n ge n ita, an a u to so m a l d o mi n an t in her ited c o n di ti o n , w hi te l es io n s o f the

or al m uc o s a an d re ctu m a re a s so c ia ted w ith d efe c ti ve fo rm a tio n o f th e n a ils o f th e h a n ds a n d fee t. In h ere di tary be n ig n in tra ep ith el ia l dys ke ra tos i s, th e ab n o rma l ity is re stri cte d to the or al mu c o u s me mb ra n e a n d co n ju n cti va , bu t th e h is tolo g y o f the o ra l le si o ns r eve al s a deg re e o f dy sk era to si s th at w o u ld b e co n s id ere d a l arm in g in oth e r l es io n s o f th e o ral

mu c o sa . In s pi te o f th is , h o we ve r, th e c o n di tio n , as its n am e im pl ie s, rem a in s es s en tia ll y

ben i gn . Dy sk era to si s co n g en ita i s ch a ra cte riz ed b y h ype rke ra tos i s of th e mu c o u s m e mb ran e s (in c lu di n g th e o ra l c a vi ty ), dy str op h ic n ai ls , a nd a bn o rm a l p ig me n tati on of th e sk in . Pe rio d o nta l ma n if es tati on s o f th is co n d itio n ha ve bee n rep o rted .

Tylosis (palmoplantar keratoderma)

In th e 1950s a n as s o ci ati o n w as fo u n d be tw e en w h ite l es io n s o f th e o ra l m u c os a ,

oe s op h ag ea l ca rc in o m a, a n d tylo s is ( hy pe rke rato s is o f th e p a lm s an d s ol es ). In th i s exa m ple of a c o mp le x de rm al β€“ mu c o sa l sy nd ro m e, th e ch a ra cte ris tic o ra l le si o ns c li n ic al ly a nd

hi sto l o gic a ll y re s em bl e th o s e o f a le u ko ke rato s is . Th i s la tter s yn d ro me , li ke mo s t o th er

der ma lβ €“mu c o sa l sy nd ro m es o f th i s type , is tra n s mitte d as a d o mi n an t co n d itio n . Li n ka ge

an a lys i s h a s re ce n tl y be en u n de rta ken in a l arg e fami ly o f tylo ti c pa tie nts a n d the l o ca tio n o f

th e o es o ph a ge a l c a n ce r g en e is l ike ly to be id en tifie d i n th e ne a r fu tu re . It w ou l d se em l ike ly th a t a w id e sp ec tru m o f s u ch co n d itio n s e xis ts a n d, i nd ee d, i so l ate d exa m pl es o f va ryin g

deg re es o f co m bi ne d de rm al a nd m u co s a l a bn o rm a liti es h a ve fre qu e ntl y b ee n re po rte d. Th e cl as s ifi ca tio n o f pa l mo p la nta r k era to de rm a (PP K), of w h ic h tyl o si s is o n e ty pe , h as re c en tly

bee n re de fin e d. T he te rm β€ pa lm o pla n ta r ec to de rm al d ys pla s ia β€™ h as n o w b ee n pro p o se d

to em ph a si z e th e m ul tip le ec to de rm al s tr uc tu re s in vo lv ed in s o m e p a tte rn s o f P P K.

Darier's disease

Dari er' s dis e as e, a l so k n ow n a s β € fo ll ic u la r ke rato s is β €™, i s a ge n etic a ll y d eter mi n ed

dis e as e. It is u s ua ll y firs t s ee n in yo u n g ad u lts and g en e ral ly ma n if es ts as g rea s y b ro w n

pa pu le s o n th e ch e st a n d sh o u l der s. P i tti n g o f th e sk in o f the p al ms o f th e h a n ds a n d na i l c a n

oc c u r. Th e o ral m u co s a is a ff ec ted in a s i gn if ic an t p ro po r ti o n o f c a se s a nd th e di se as e u su a ll y pre se n ts a s c o al es c en t w h ite le si o n s o n the b u cc a l m u co s a e o r pa la te.

Benign neoplasms

Neo p la sm s o f m a n y ki n ds , b oth p ri ma ry an d s ec o nd a ry, m ay o c cu r in th e mo u th . It i s po s si bl e to d es c rib e typi ca l cl in i ca l fea tu res f o r m an y o f th e se le si o n s, b u t it mu s t be

rem em be red th a t mi s lea d in g an d a typi ca l fo rm s ma y al so pre s en t a n d m a y l ea d to di ag n o sti c erro r. T he f in al d ia gn o s is o f an y tis su e o ver gro w th d ep en ds e n tire ly o n h is tol o gi ca l

in ve sti ga tio n a n d th i s mu s t in va ri ab ly be u n de rta ken i f an y do u bt at al l e xi sts a s to th e tru e

na tu re o f a le si o n . Th e mo s t c om m o n n eo pl a sm s af fe cti ng o ra l mu c o sa a re th e s q ua m o us c e ll pa pi llo m a , wh i ch is a be n ig n tu mo u r, a n d sq u am o u s ce ll c arc in o m a, wh i ch i s a ma li gn a n t

tu m o u r (s ee Ch ap ter 10). Th e ro le o f th e h u ma n pa pill o ma vi ru s (HP V ) in the d eve lo p me n t o f bo th o f th e se n e op la s ms re ma i ns sp ec u la tiv e. A n u m be r o f co n n ec tiv e -ti ss u e n eo p la sm s (fo r exa m ple , fi bro m a s, li po m a) ca n o c c ur in th e m ou th bu t th es e are l es s co m m on .

Squamous cell papilloma

Th is b en ig n ep ith e lia l n eo p la sm i s rel ati vel y c o m mon . It m ay a pp ea r a n yw h ere o n th e o ra l

mu c o sa , bu t is mo s t c o mm o n ly fo u n d at th e ju n cti o n o f th e h ar d a n d so f t pa la tes . Its typ ic al

ap pe ara n c e i s β€ ca u li flo w er -lik eβ €™ an d pe du n c u la ted w ith a p al e colo u r va ryi ng fro m th at of n o rm al m uc o s a to wh i te. It i s a pa in l es s le si o n th at r are ly gi ve s tro u bl e. M al ig n an t

tr an s fo rm ati o n h as n o t b ee n de sc ri be d i n th es e le sio ns a n d, in th i s res pe ct, th e o ra l l es io n s beh a ve q ui te dif fe ren tly fro m th o s e i n th e lo w er parts o f th e g as tro in te sti na l trac t, w h ic h ha ve a d efi n ite ten d en c y to u n de rgo m a lig n an t ch a n ge .

Trea tm en t o f a pa pi llo m a is b y l o ca l ex ci si o n . Th is, h o we ve r, m us t b e su ff ic ie n tly wi de a n d

dee p to in c lu d e a n y ab n o rma l c ell s th at m a y e xte nd b eyo n d th e are a o f th e pe di cl e. It is n o t su ff ic ie n t

to si mp ly s eve r th e pe di cl eβ €”th i s wi ll le a d to recu rren c e. Rem o va l w ith a l as er is a n

P. 105

al te rn a tive .

Mu l ti pl e vira l w arts , cl in i ca ll y s i mi la r to th e so lita ry p a pi llo m a , oc c as i on a ll y o c c u r in the

mo u th o r, m or e c o m mo n ly , o n an d a ro u nd th e li ps . In c h ild re n the s e a re o fte n the res u lt of

au to in o c u la tio n by c h ew in g w a rts on the h a n ds . S exua l tran s mi s si on be tw een ge ni tal a n d o r al si te s h as a l so b ee n d es cri be d. V e n ere al w ar ts (c o ndyl o ma a cu m in a tu m), w h i ch

ch a rac te ris tic al ly o cc u r i n th e an o ge n ita l reg io n an d m ay b e a m a ni fe sta tio n o f HIV in f ec tio n (se e Ch ap ter 4 ), ca n b e se en o n th e o ra l m u co s a . A la rg e n u m be r o f pa pi ll om a vir us e s h as bee n d es cr ibe d an d it is p o ss ib le to c la ss i fy or al w ar ts /p ap il lo m as i n va rio u s w a ys . Fo r

pra cti ca l pu rp o se s , ho w e ver , the d ivi s io n is c l ea r b etw ee n so l ita ry les i on s w i th no evi de n t in fe cti ve ae tio lo g y a n d th os e th at are th e res u lt of i no c u la tio n . M an a ge m en t i s th e s am e,

al th o u g h, cl ea rly , the β € wa rt- ch e wi n gβ €™ ha b it o f af fec te d c h il dre n s h ou l d be di sc o u ra ge d.

Vi ral w a rts ge n era ll y s p on ta n eo u s ly reg res s a fter a pe rio d o f a y ea r o r so .

Miscellaneous benign conditions Traumatic keratoses

Tra um a tic ke ra to s es o n th e o ra l m u c o sa a re ca u s ed by a lo c al re ac tio n to ea rli er ph ys ic a l,

me ch a n ic al , el ec tric a l, o r th e rm al c au s es an d a re rev ers ib le . Ch em ic a l tr au m a ca u s ed by a n irri tan t s u bs ta nc e , su c h a s to pi ca l us e o f a n a s pirin , c an c a u se e ith er o ral u lc e rati o n (s ee

Ch ap ter 5) o r h y per ker ato s is , w h ic h ma n if es ts as a w h ite le s io n o n th e o ra l mu c o s a. A c u te ch e mi ca l in s ul ts, s u c h a s i n ge sti o n o f a c a us tic o r ac i d s u bs ta nc e , ten d to c au s e or al

ul ce ra tio n . Les io n s s ee n o n th e or al m uc o s a o f i n divid u al s w ho sm o ke c iga re ttes , ci ga rs , o r pip es a re du e to a c o mb in a tio n o f c h em ic a l a n d th erm al i ns u lts . Th e ro le o f tob a cc o i n th e

dev el o pm en t o f pre m al ig na n t l es io n s is f ul ly di sc u sse d in Ch ap ter 10. Nic to n in ic s to m atiti s du e to pi pe -sm o kin g i s d is c u ss ed b el ow .

Frictional keratosis

An i mp o rta nt fa cto r in th e pro d u cti on o f w h ite pa tche s o f th e o ra l m u co s a is th e ef fe ct o f

me ch a n ic al tra u ma . It i s we ll kn o w n th at s u c h tra uma m ay h av e a w i de va ri ety of e ffe cts o n th e m u co s a, d ep en d in g o n th e e xa ct n atu re o f th e tra u ma , its ra te o f a pp li ca tio n , a n d an

in di vid u al 's res p on s e. Wh en th e tra um a is a c u te a n d lo c al iz ed , th e e pi th el iu m is d es tro ye d

an d an u lc er is pr od u ce d. If , h o we ve r, th e tra u m a is le s s ac u te a n d le ss lo c al iz e d, th en the re wi ll be a ra n ge o f res po n s es d ep en d in g o n the p rec is e cl in i ca l co n d itio n s . If th e irr ita tio n is rel ati vel y c h ro n ic i n ch a ra cte r a n d of lo w i n te n si ty, th e ma jo r c h a ng e in du c ed i n th e o ra l

mu c o sa m ay w el l be an ab n o rma l ity i n th e ker ati ni z ati o n pa ttern , w h ic h m u st be as s o ci ate d wi th so m e de gre e o f c h an g e in th e u nd erl yi n g c el ls . Th e re is n o d ou b t th at su c h tra u ma tic kera to s es ex is t a n d th a t th e la rge m ajo ri ty a re re ver si bl e, c o mp le tely di sa p pe ari n g i f th e

tr au m a is re mo ve d. It is o f in tere s t to rec a ll th at th e mu c o s al re sp o ns e to tra u ma m o st of ten se en i n th e o ra l c avi ty i s th at of th e de n tur e g ra nu l o ma . Th e pr in ci pa l ch a n ge i n thi s

pa rtic u la r l es io n i s in th e co n n e cti ve tis su e c o mp one n t o f th e m uc o s a rath e r th a n in th e epi th el iu m.

Nicotinic stomatitis (pipe-smokers' palate)

Nic o tin ic s to ma titi s is u s ua ll y fo u nd , as th e n a me im pl ie s, in p ip e-sm o ker s, b u t a ls o ,

oc c as io n a ll y, i n ci ga re tte - an d ci ga r -sm o ker s. T he c h ar ac teri sti c h is to lo gi ca l fe atu re o f th i s co n di tio n i s the co m bi n ati o n o f e pi the li al a c an th o si s an d h yp erk era to si s w ith i nf la mm a tor y ch a n ge s i n th e mu c o u s gla n ds o f th e pa la te. A s a resu lt o f th es e c ha n ge s , the pa la te

bec o m es w h ite a nd a n u m ber o f n o d u les p ro jec t f ro m the s u rfa ce , ea c h rep re se n ti n g th e s ite of a m uc o u s gl an d a n d i n di vid u al ly be ar in g a sm a ll red sp o t a t th e ce n tr e th a t m ark s the

op en i n g o f th e d u ct o f th e gl an d (Fi g. 9. 4 ). In ad va n ce d ca s es th e mu c o u s gl an d s ma y b re ak do w n to fo rm q ui te la rge c h ro ni c u lc ers . Th e c o nd iti o n u su a ll y a pp ea rs m o st ma rke d on th e ha rd pa l ate , al tho u g h th e s o ft pa la te m a y a ls o b e in vo l ved . It h a s be en su g ge ste d tha t th e

si te o f in vo l ve me nt de pe nd s u po n th e pr oje c tio n o f pip e sm o ke di rec tly o n to th e pa la te, b u t

th i s is n o t e as y to pro ve . In s pi te o f th e co n s tan t an d c hr on i c exp o s ur e to th e ir rita n t fa cto r, ma li gn a nt tran s fo rm a ti o n is n o t a fe a tur e o f th is le s io n , w hi c h is n o t c o n si de red to b e

pre ma li gn a n t. M an a ge me n t is d ire cte d at per su a di n g th e pa tie n t to s to p s m o kin g . If th is i s

do n e, re s ol u tio n o f the l es io n (o f ten c o mp le te) ca n b e r ea so n a bl y e xp ec ted . An ad de d be n efi t is th at th e o ra l mu c o sa i s no lo n ge r e xp os e d to c h em i ca l ca rc in o g en s th at ma y p re dis p o se to th e d eve lo p me n t o f p rem a li gn a nt les i on s f o r ca rc in om a el se w he re in th e m ou th o r ae ro di ge sti ve trac t.

It sh o u l d b e po in te d o u t th a t th e ab o ve de sc ri ptio n of th e co n d itio n a pp li es o n ly to th at

ch a rac te ris tic al ly s een in E u ro pe a n an d No rth A m erica n p ip e-sm o ker s. Oth e r to ba c co -in du c ed pa la tal le s io n s h av e b ee n de s cri be d tha t h a ve a ve ry di ffe ren t pro g no s is ( se e Ch ap ter 10 fo r

pa la tal le s io n s as s o ci ate d wi th re vers e s mo ki n g).

Leukoedema

Leu ko e dem a is th e n a me gi ve n to a fi lm y g rey c o ati ng o f th e b u cc a l mu c o s a th a t i s fo u nd i n a hi gh p ro po rti o n o f p a ti en ts . Th er e h a ve be en wi de ly di ve rge nt es tim ate s o f b o th th e in ci de n ce

an d th e s ig n if ic an c e o f th is co n d itio n , o n e o f th e di ffi c ul tie s in th is b ei n g th a t o f de fin i tio n .

P. 106

Ho w eve r, th ere c an b e n o do u b t th at s u c h a fi lm y c oati n g a p pe ars o n th e bu c c al m uc o s a o f a la rge n u mb er o f c o mp le tel y a s ymp to ma tic p a ti en ts w hen v ie we d in a de qu a te li gh tin g co n di tio n s .

Fig. 9 .4 S mo ke r's k era to si s o f th e pa la te.

His to lo g ic al ly, th i s le si o n h as b ee n de sc ri be d a s bein g p ara ke ra toti c, w ith l arg e sw o ll en c e lls in th e su p erf ic ia l l a yer s o f th e ep ith el iu m. Th is his to lo gi ca l a ppe a ran c e is q ui te co m pa tib le wi th th e o b se rva tio n s th a t the g rey su rf ac e fi lm (co n si sti n g o f th e s u pe rfi ci a l o e de ma to us

ce lls ) ma y ea si ly be sc ra pe d aw a y f ro m th e m u co s a , le av in g an ap pa ren tl y i n ta c t s u rfa ce th at ag ai n ra pi dly a cq u ire s th e s u pe rfi ci al ly gr ey ap pe ar an c e. Le u ko ed em a is n o t a s so c ia ted w ith epi th el ia l a typ ia a n d s h o u ld n o t be re ga rd ed a s a pre ma li gn a n t le si o n .

Pigmentation of the oral mucosa

Pi gm en ta tio n o f th e o ra l mu c o sa c a n o cc u r a s a res ult o f e n vir on m en ta l or o cc u pa tio n a l

exp o su re to h e av y m eta l sa lts , s uc h a s bi s mu th , le ad , a nd m erc u ry, so m e of w h ic h w ere u s ed in th e pa st as th e rap eu ti c a g en ts fo r a n u mb er of dis ea se s s uc h a s s yph i li s. F o ll o wi n g

ab so rp tio n o f th e m eta ls , th ey ar e d ep o si ted a s me tal li c su lf id es a s a gre y (o r b lu e/b la c k) lin e a lo n g the ma rg in a l g in g iva e .

Amalgam tattoos

Am a lga m ta tto o s a re a res u lt o f fra g me n ts o f th e mater ia l b ei n g e m bed de d in th e o ra l

mu c o sa . Th e se a pp ea r a s is o la ted p igm e nte d le si o n s (li gh t -bro w n to d ark -blu e /bl ac k in

co lo u r) . Am a lg am ta ttoo s m a y b e ra di o -op a qu e, b u t th is i s no t al wa ys th e c as e ( Fi g. 9. 5).

Th ey u su a ll y o c cu r o n th e f lo o r o f th e m o u th o r a lveo la r mu c o sa , n ea r to ex is tin g o r p revi o u s

am al ga m re sto ra tio n s . Ma rke d am a lg am ta ttoo s m a y b e s e en o n th e a ttac h ed g in g iva e, ov erl yin g te eth th a t h av e b ee n a pi ce tom i ze d an d a pica ll y se a led w i th am a lga m .

Oth er fo re ig n su b sta n c es s u ch a s ro a d gri t c an be im pl an te d in th e o ral m u co s a an d

oc c as io n a l pa tie nts p res e nt wi th me s sa ge s (o fte n rude !) ta tto o e d on th e ir la bi al m u co s ae .

Melanotic pigmentation of oral mucosa

Th e si gn i fic a n ce o f or al pi gm en ta tio n i n en d o cri n e d is tu rba n c es , a nd p arti cu l ar ly in A dd is o n' s dis e as e, i s di sc u s se d in Ch ap ter 13. An as s o ci ati o n be tw een o ral m el an o s is (p arti cu l ar ly of th e s o ft p al ate ), s mo ki n g, an d b ro n ch o ge n ic c ar ci n om a h a s al so b ee n re po rte d. O ra l

me la n os is m a y o c c ur in HIV i nf ec tio n ( Ch ap ter 4). A n u mb er of d ru gs m ay a ls o sti mu l ate in cr ea se d pi gm en ta ti o n β€”in cl u di n g o ra l co n tra c eptive s, a n tim al a ria ls , an d m ajo r

tr an q ui ll iz er s. T he re h av e b ee n re ce n t rep o rts o f min o c yc li n e c a us i ng pi gm en tio n of th e sk in

an d o ra l m u co s a . Inc re as e d m el an i n pr od u cti o n ma y al s o o cc a si o n al ly be s ee n in a s so c ia tio n wi th th e o ra l le si o n s of li c he n p la nu s a n d w ith l euko p la kia s β€”th i s is re ac tiv e a n d o f n o

cl in ic a l s ig n if ic an c e. H o we ve r, in m a n y p ati en ts , pa rtic u la rly th o se w ith h e av il y p ig me n ted

ski n s, or al pi gm en ta tio n i s qu ite n o rma l . Th is m ay b e p atc h y or di ffu s e, bu t th e gi ng iv ae ar e al mo s t a lw a ys in vo l ve d, e ven wh e n th e s ki n p igm e ntatio n i s mi n im al .

Fig. 9 .5 (a ) A ma l ga m tatto o . (b ) Ra d io gra p h sh o w in g a ma lg a m de bri s .

Th e P eu tz β €“Jeg h er s yn d ro me i s an a u to so m a l d o mi n ant s yn d ro me , c om p ris in g me la n o tic ma cu l es p eri o ral ly an d o ra ll y to g eth er w ith i nte stin al p ol yp o si s. C li ni ca l ly, th is s yn dro m e

ma n ife sts a s m ul tip le fre c kle s o cc u rri ng a ro u n d th e n o se a n d e ye s an d o n th e li ps a n d th e

or al m uc o s a. T he i n te s ti n al p o lyp s ca n b e pre se n t th ro ug h o u t th e g u t a nd m a y g ive ri se to sym p tom s , su c h a s ab do m in a l pa in o r ca u s e i n tes tin al o b stru c tio n . Th e se p o lyp s do n o t us u a lly b ec o me ma li gn a n t.

Th e La u gi erβ €“Hu n zi ke r s yn dr om e (id io p ath i c le n ti c ul a r m uc o c u ta n eo u s p ig me nta tio n ) is a rare c o n di ti o n in wh i ch th e re is w id es pre ad m el an i n pi gm en ta tio n a ffe c ti n g th e o ra l an d gen i tal m u co s a an d lo n gi tu di na l pi gm en ta tio n o f the n a il s.

Th e r ec o gn iti o n o f a lo c al iz e d a n d sy mp to ml es s β € me la n oti c -lik e β€™ les i on o n the o ra l

mu c o sa m ay ca u s e c o n s ide ra bl e p ro bl em s . Su c h a l es io n m ay b e e n tire ly be n ign an d s ta tic , bu t th e in iti al s tag es o f ma li gn a n t m ela n o ma m a y h ave a s im ila r ap pe ara n c e to th at o f

ben i gn l es io n s . Th e fi rst ste p m u s t be to e lim i na te the p o ss ib il ity o f an am a lga m ta tto o (se e ab o ve).

Id io p ath i c me la n oti c ma c u les ar e r ela tiv el y c o mm o n , be n ig n le si o n s o f th e me la n oc yte s , wh i ch a p pe ar as s m al l bro w n o r bl ac k pa tch e s on the o ra l mu c o sa o r the l ip s. O ra l me la n oc yti c n ae vi ar e m u c h le ss c o mm o n a n d s u bd iv ided in to β € jun c tio n a lβ €™,

β€ co m po u n d β€™, β € in tram u c o sa l β€™, a n d β€ blu e β€™ va ria n ts. O ra l m e la no c yti c n ae vi an d me la n o tic m ac u le s sh o u ld b e e xc is e d to c o n fi rm the d ia gn o s is

an d , in pa rtic u la r, to e xc lu d e th e po s s ib ili ty o f a ma l ign a n t m el an o m a. Tab le 9. 1 su m m ari z es th e c au s es o f me la n o tic p ig me nta tio n o f th e o ral m uco s a .

Table 9.1 Melanotic pigmentation of the oral mucosa: principal causes Rac ia l

Ad di so n 's di se a se HIV di se a se Rea cti ve

Li c he n pl an u s a n d l eu ko p la ki a

Dru g-rel ate d

Pe u tz β€“Jeg h er sy n dro m e

Is o la ted m el an o tic le si o n s

Id io p ath i c m e la n oti c ma c u le M e la n oti c n ae vu s

M a li gn a n t me la n o ma

Oral melanoma

In tra o ra l m el a no m a s are h ig h ly ma li gn a n t o ra l tu m o urs tha t u s u al ly o cc u r a s is o la ted d ar k bro w n o r b la c k pa tc he s o n th e o r al mu c o s a. A m el an o tic m el an o m as c a n m an if es t a s red

les io n s . Or al m ela n o ma s a re rar e m a li gn a nc ie s a nd te nd to o c c u r o n th e pa la te. In th e ea rly sta ge s the y ma y b e sy mp to ma tic . Th e a ppe a ran c e is us u al ly o f a n o du l ar o r ma c u la r l es io n

P. 107

th a t i s firm to p a lp ate . Th is u lti ma tel y u l ce ra tes a n d c a u se s di sc o m fo rt o r b le ed s. Ea rl y

dia g no s is o f a ny le s io n s u sp ec ted o f be in g a ma li gna n t m el an o m a i s es s en tia l as m eta s tas is

to the l ym ph n o de s a nd o th er o rga n s (lu n g s, l iv er, a n d bo n e) o cc u rs ea rl y. Th e pr og n o si s fo r or al m ela n o ma i s po o r. S u pe rfi ci a l s pr ea di ng m el an om as a re ra rel y s e en i n th e m o u th an d th e ir ap pe a ran c e ca n b e q u ite s pe cta cu l ar ( Fi g. 9. 6).

Fig. 9 .6 Dif fu se a dv an c ed o ral m el an o m a.

Ea rl y di ag n o si s an d tre atm en t o f o ral m al ig n an t m ela no m a s is es s en tia l, as th ey me tas ta si z e ea rly to ly mp h n o de s an d o th er or ga n s.


Wh at i s th e di ffe ren ti al d ia gn o s is o f th i s les i on an d h o w w o ul d yo u m an a ge th is ca s e?

An a m al ga m tatto o w o u ld b e u n u s u al at thi s s ite, a lth o u g h the a u th or s ha ve s ee n a fe w su c h

ca se s . Ma l ign a n t me la n o ma m u st be in c lu d ed in th e diff ere n ti a l d ia gn o s is o r po s si bl y a n e arl y Kap o si 's s arc o m a. T he m o st lik el y d ia gn o s is o f thi s is o la ted l es io n i s an id io p ath ic m el a no ti c

ma cu l e b u t e xc is io n al b io ps y is i nd ic a ted . Th e bio ps y sh o u l d b e arr an g ed as s o o n a s po s si bl e, pa rtic u la rly in vie w o f th e pa tie n t's fa mi ly h is to ry.


Wh at i s th e m o s t li kel y, c li n ic al d ia gn o s is o f thi s la dy 's le si o n ?

Th is is l ik ely to b e a d en tu re gr an u lo m a , ca u se d by c hr on i c trau m a fro m th e b u cc a l f la n ge o f th i s la dy 's il l-fittin g u p per de n tu re. Q2

Ho w is th is l es io n ma n a ge d?

Th e f la n ge o f th e de n tu re sh o u ld be tri mm ed b ac k so th a t it is n o l on g er trau m a ti z in g th e

mu c o sa a n d th e p ati en t sh o u l d b e di sc o u rag ed fr o m we ar in g he r d en tu re s at n i gh t. S u rgi ca l

exc is io n of th e r em ai n in g hy pe rpl as tic ti ss u e ma y be n ec es s ar y b u t sh o u ld n o t b e ca rri ed o u t un ti l th e res p o ns e to m od if yin g th e de ntu re h a s be en a s se ss e d. Ne w de n tur es sh o u l d b e

pro vi de d in th e lo n g term . Q3

A mo re s in i ste r o ra l les io n ca n o c ca s io n al ly pr esen t i n a si m ila r w a y. W ha t is th is a n d ho w c a n it be di ffe ren ti ate d f ro m the m o re co m mo n ben i gn o ra l le si o n ?

Car ci n om a o f th e m ax il la ry a n tru m ma y in fi ltra te the m ax il la ry b o n e a n d o cc a si o na l ly

pre se n ts a s a p ro lif era tiv e le si o n in th e b uc c al s ulc u s. T he a pp ea ra n ce o f a ti s su e ma s s in th i s si te, w ith o u t an y ev id en c e o f tra u ma fr om th e de n tu re, w o u ld im m ed ia tely a le rt th e

cl in ic ia n . P ati en ts w ith a n tral c a rci n o ma m ay a ls o pr es en t w ith sy mp tom s s u ch a s bl o ck ed no s e, n a s al d is ch a rge , p ai n, or pa ra es th es ia o ve r th e c he ek . A ntra l ca rc in o m a ca n a ls o

pre se n t as a s w el li n g i n th e p a la te, w h ic h ten d s to u lc era te . Ra di og ra ph y rev ea ls o p ac ity o f th e a n tr um , o fte n w ith e ro si o n o f th e a ntr al w al ls . If th e re is a n y d o ub t w h ats o ev er,

im me di ate bi o ps y o f th e les i on mu s t be ca rri ed o u t. A ntr al c arc in o m a h as b ee n de s cri be d in pa rtic u la r ri sk g ro up s , fo r ex am pl e, w o rk ers i n the w o o d o r s h oe i nd u s tr ies a n d in d ivi du a ls wh o u s e sn u f f.

Au th o rs : Title:



Co py rig ht Β © 2003 Ox fo rd U ni ve rsi ty P re ss > T ab le o f Co nt e nt s > 1 0 - Pr e ca nc e r ous le sio ns an d c ond it ion s. O ra l c ar cin om a and c ar cin og e ne sis

10 Precancerous lesions and conditions. Oral carcinoma and carcinogenesis Problem cases Case 10.1

A 60-yea r-ol d ed en tu lo u s ge n tlem a n pr es en ts to yo u r d en ta l su rg ery fo r the f irs t tim e

req ue s tin g a ne w s et o f de n tu res . Hi s me di ca l h is tory i s cl ea r b u t h e is a li fe - lo n g s m o ker ,

ave ra gi n g 20β €“30 ci ga re tte s pe r d ay . Hi s up pe r d entu re is b ad ly s tai n ed w ith s ev era l te eth eith e r fra c tu re d o r m is si n g.

Dur in g yo u r o ra l exa m in a ti o n yo u n o te an is o la ted ulc er o n th e lef t la ter al bo rd er o f th e

to n g u e, w h ic h is a p pro xi ma tel y 1 c m in d ia me ter. Palp a ti o n o f h i s n ec k re ve al s n o o bv io u s ab n or ma li ty. Q1

Wh at q u es tio n s w o u ld yo u a s k th is pa tie n t?

Q2

Wh at f ea tu res o f th e u lc er ar e i mp o rtan t to n ote ?

Q3

Wh at i s th e m o s t li kel y d if fe ren tia l di ag n o si s in th is pa rtic u la r c a se ?

Q4

Ho w w o ul d yo u m an a ge th is p ati en t?

Introduction

Th ere a re a nu m be r o f o ral l es io n s th at h av e a p o ten tia l fo r m a li gn a nt ch a n ge . Ho w ev er,

rel ati vel y f ew c a rci n o ma s de ve lo p w ith in a re c og n iza bl e p re ca n ce ro u s le si o n o r i n a pa tie nt wi th a pre c an c ero u s c o n diti o n .

A pre ca n c ero u s le si o n is a mo rp h o lo g ic al ly al tere d tis su e in wh i ch c a n ce r i s mo re li ke ly to

oc c u r th an in its a pp ar en tly n o rma l co u n ter pa rt, that i s , the l es io n i tse lf u nd er go es ma li gn a n t tr an s fo rm ati o n. E x am pl es o f pre c an c ero u s o ra l les ion s i n cl ud e le u ko pl ak ia a n d e rth yro p la kia . A pre c an c ero u s l es io n i s a mo rp h ol o gi ca ll y a l te red ti ss u e in w h ic h c an c er is m o re li kel y to oc c u r th an in i ts ap pa ren tl y n o rm al c o u nte rpa rt.

An o ra l pre c an c ero u s c o n diti o n is a g en e ral iz ed s tate a s so c ia ted w ith a si gn i fic a n tly in c rea se d ris k o f c an c er de vel o pi n g s o me w h ere in the m o u th , th a t is , n o t n ec es s ar ily in a pre -exi sti n g

les io n . In p re ca n ce ro u s co n d itio n s th e li n kin g fa c to r ap pe a rs to be e pith e li al a tro ph y su c h a s th a t w h ic h o c cu rs i n o ral s u bm u co u s fi bro s is a n d side ro pe n ic d ys ph a gi a. S o m e a u th or itie s

pre fer th e term β€ po ten tia l ly ma li gn a n tβ €™ to pre ca n c ero u s be c au s e not al l p rec a n ce ro u s les io n s u n de rg o ma li gn a n t tra ns fo r ma tio n a n d a p ro po rti o n reg re ss o r sta y th e sa m e.

A pre ca n c ero u s c on d iti on is a ge n era li z ed s tate a sso ci a te d w ith a si gn i fic a n tly in c rea se d ris k of c an c er de ve lo pi n g.

Precancerous lesions Leukoplakia Definition of leukoplakia

Leu ko p la kia i s cu rr en tly de fi ne d as a pre do m in a n tly wh i te l es io n of th e or al m uc o s a tha t ca n n ot be c ha ra c te ri ze d as an y o th er de fin a bl e l es io n β€”it i s th e ref o re a d ia g no s is o f

exc lu s io n . V a rio u s atte mp ts h av e b ee n m ad e by the Wo rl d Hea l th Org a ni sa tio n to de fin e o ra l leu k op la ki a fo r u s e in e pi de mi o lo gi ca l s ur vey s an d th ere h a ve be en a n u m be r o f rev is ed

def in iti o n s. N o do u bt the c u rre nt de fin i ti o n o f l euk op la ki a w ill c h an g e as o u r u nd ers ta n din g o f its ae tio l og y an d pr og re ss io n i n cre a se s.

Leu ko p la kia i s cu rr en tly de fi ne d as a p red o mi n an tl y w h ite le si o n o f th e o ral m u co s a th at ca n n ot be c ha ra c te ri ze d as a n y o th e r de fi n ab le le sio n .

It is im p or ta n t to n o te th at th e ter m o ra l le u ko pl ak ia is a c l in ic a l te rm w ith n o h is to lo g ic al co n n o tati on s . F ro m a cl in i ca l po in t of vi ew , i t is u s ef ul to d iv ide th es e le si o n s in to

ho m o ge n eo u s an d n o n -ho m o ge n eo u s le si o n s. No n -ho m o gen eo u s le u ko pl ak ia s, i n cl u di ng th o s e wi th a β€ sp ec kl ed β €™ ap pe ara n c e, h a ve a w o rse p ro gn o s is w ith re ga rd to m al ig n an t

tr an s fo rm ati o n tha n h o m o ge ne o u s les i on s . Le uk o pl ak ia s in p arti cu l ar s ites , s u ch as th e fl o o r of th e mo u th a n d th e ve n tr al s u rfa ce o f th e to n gu e , ar e c o n si de red to be m o re at r is k o f ma li gn a nt tran s fo rm a ti o n th an th o s e el se wh e re o n the o ra l mu c o s a.

Incidence of leukoplakia

Th e i n ci de n ce o f or al le u ko pl a kia i s di ffi cu l t to as c erta in bu t re po rte d p re va len c es th ro u g ho u t th e w o rld ra n ge fr om 0. 2 to 4 pe r ce n t. Ho w ev er, the re a re m ark ed va ri ati on s i n pr eva le n ce betw e en d if fere n t ge o gra ph i ca l ar ea s (We ste rn E u ro pe a nd A m eri ca ) th a t p ro ba bl y re fl ec t

dif fe ren t eth n ic a n d c u ltu ra l gro u ps a n d ae tio l og ica l f ac to rs , su c h a s sm o ki n g a n d ch e wi n g to b ac c o . Or al le u ko pl ak ia u s ed to o c cu r pre do m in a n tl y in m al es a n d af fe ct o l de r a ge g ro u ps bu t g en de r a n d ag e rati o s are n o w ch a n gi n g.

Aetiological factors associated with oral leukoplakia

Tw o gro u p s o f le u ko pl ak ia s ma y be re co g ni z ed : i dio pa th i c le uk o pl ak ia s, i n w h ic h n o

ae tio lo g ic al fa c to rs h av e b ee n re co g ni z ed , an d le u ko p la kia s in wh i ch an e vi de nt pre di s po s in g

fac to r m a y b e pre se n t. It is p ro ba bl e th a t th e id iopa th ic g ro u p ma y in c lu d e a c er ta i n n um b er of le s io n s in w h ic h th e ae tio l og ic a l f ac to r re m ai ns u n rec o gn iz e d, b u t, ev en w ith th i s

res erv ati o n, it w o u ld s ee m rea so n a bl e to ac c ep t th e po s si bi lity o f th e e xis ten c e o f i di op a th i c les io n s .

It is c le arl y e s ta b lis h ed th a t th ere a re a n um be r of c lin i ca ll y si gn i fic a n t ae tio l og ic a l f ac to rs

th a t m ay c o ntr ibu te to th e de vel o pm en t o f o ra l le u ko pl ak ia . Th e mo s t i mp o rtan t o f th es e is th e u s e o f to ba c co , e ith er w he n sm o ke d or c he w ed in o ne o r o th e r o f the l arg e n um be r o f

to b ac c o -us in g h a bi ts th at h a ve b ee n rep o rted . Th e pre ci s e n a tu re of th e ac tio n o f to ba cc o o r

of its sm o ke o n th e o ral m u co s a is n o t ye t k no w n , but the re ca n b e no do u bt th a t a pro f o un d eff ec t is o f te n e xer te d. A p ar t fro m th e to b a cc o itse lf, o th er su b s ta n c es in v o lve d in to ba c co ch e wi n g h a bi ts (s uc h a s be tel n ut an d li me ) m a y al s o be im p lic a ted in th e p ro du c tio n o f

leu k op la ki a. Th ere h a ve be en m a ny su rv ey s co n c ern e d w i th th e e ffe c t o f to b ac c o h ab its o n th e o ra l m u c os a a n d, i n vi rtua l ly al l c a se s , it w o uld a pp ea r th a t th o se in d iv idu a ls w h o u s e

to b ac c o in an y o f i ts fo rm s ar e m u ch mo re li ke ly to de ve lo p o ral l eu ko p la kia . It i s al so w el l es tab li sh e d th a t th e pre ci se n a tur e o f th e to b ac c o h ab it is o f gre at si gn i fic a n ce in the

dete rm in a ti o n o f th e ex ac t f o rm of th e le si o n pro d uce d an d al s o o f i ts eve n tua l pr og n o si s. In an i n ves tig a ti o n o f th e re la tio n sh i p be tw e en th e i nci de n ce o f leu k o pla ki a an d th e se x, a ge ,

P. 112

an d to ba cc o h a bi ts o f a B o mb a y (re na m ed β € Mu m ba i β€™) po p ul a ti o n , it w a s sh o w n q u ite

cl ea rly th at th e m o st im po rta n t ex trin s ic f ac to r b y f ar wa s th ei r s mo k in g h ab it. F u rth erm o re,

th e in c rea s ed in c id en c e o f l es io n s in m a le s wa s d ire ctl y a ttrib u ta b le to th ei r in c rea s ed u s e o f to b ac c o c o mp are d to th at o f fe ma le s . It is n o t d iffic u lt to ex trap o la te th e se fi n di n gs to o th er se rie s in w h ic h th er e h a s be en a c o n s ta n t ma rk ed pre do m in a n ce o f le uk o pl ak ia in ma le

su b jec ts. T he re is n o d o u bt th a t th os e le u ko pl ak ia s th at a re d ire ctl y a ttrib u tab le to to ba c co ha b its ar e, to a la rg e d eg ree , re vers i ble , a n d i t ha s b ee n c le arl y dem o n s tr ate d o n m a n y

oc c as io n s th at r eg res s io n o f a l es io n ma y b e in du c ed b y th e c es sa tio n of th e ha b it, al th o ug h th e re gre ss i on ma y n o t b e pe rma n en t.

In d iv idu a ls w h o u s e to b a cc o in an y o f i ts fo rm s are m u ch m o re li kel y to de ve lo p o ral leu k op la ki a.

A se co n d im po rta n t ae tio l og ic a l f ac to r i n th e p ro duc tio n o f o ra l l eu ko p la ki a is th at o f

in fe cti on an d , o f th e o rg an is m s in vo l ved . It a pp ea rs th at Can d id a al bi ca n s is b y f ar th e m o st im po rta nt. It is w el l e s tab lis h ed (Ch ap ter 4 ) th at the re ma y be a h ea vy in fi ltra tio n o f th e ps eu do h y ph a l f o rm of Can d id a in to th e ep ith el iu m o f o ral l eu ko p la ki as , a nd i t has b ee n

sh o w n th at su c h le si o n s (ca n di da l le u ko pl ak ia s) a re a s so c ia ted w i th an i n cr ea se d in c ide n ce o f ma li gn a nt tra ns fo rm a tio n . Th e pre ci s e re la tio n s h ip be tw een the c a nd id al i nf ec tio n a n d the

pro d uc tio n o f th e leu k op la ki a is n o t kn o w n a n d, i n pa rti cu la r, i t h a s n o t y et be en d ete rm in ed wh e the r th e in fe c tio n is a p rim a ry o r a se c on d ar y eve nt. Hi sto lo g ic a l s tu di es w o u ld s ee m to

pro vi de e vid en c e fo r e ith er vi ew po i nt, a n d it w ill p ro ba bl y b e as th e re su lt o f i mm u n ol o gi ca l stu di es th a t th e fin a l d ec i si o n as to th e e xa ct a e ti o lo g ic al fa c to r in th e se c a se s is m ad e.

Ho w eve r, re ce n t w o rk h as s h o w n tha t, o n a n e xpe rim en ta l ba si s, ca n di da l in fi ltra tio n i nto

epi th el iu m ma y o f i tse lf pro d u ce c h an g es re se mb li n g th o s e of le u ko p la kia , a n d th e re wo u l d

al so s e em to b e e vi de nc e th at co m pl ex im mu n e de fi cien c ie s ma y o c c ur in so m e pa tie n ts wi th leu k op la ki a, in cl u di n g a d ef ic ie nt im mu n e res p on s e to Can d id a al bi ca n s .

Th ere is a ls o s o m e ev id en c e o f an ad di tive e ffe ct be tw e en th e a cti o ns o f to ba c co a n d Can d id a in th e fo rm ati on o f l eu ko p la kia s . It is c u rre n tl y su g ge ste d tha t h e av y s m ok ers a re a t

in cr ea se d ris k, f irs tly, of d eve lo p in g ca n di da l le uko pl ak ia a n d, s u bs eq u en tly , o f d ev elo p in g ca rc in o ma w i thi n it. T hi s is n o t a p ro ven as s o ci ation bu t th er e i s a co n s id era b le bu l k o f an ec d o ta l in fo rm a tio n to s u pp o rt th e co n c ep t. Th e rol e o f v iru s es , s uc h a s th e h u m an

pa pi llo m a viru s (H PV ), in th e ae tio lo g y o f o ra l l eu ko p la ki as re m ai ns u n c erta in . Ho w ev er, h a iry leu k op la ki a, wh i ch o c c u rs in H IV an d o th er i m mu n o sup pre s se d pa tie n ts, i s as s o ci ate d wi th th e E ps tei n β€“Ba rr viru s (E B V ).

Clinical features of leukoplakia

Leu ko p la kia a pp ea rs a s a n in trin s ic w hi te are a o f th e o ra l m u co s a , so m eti me s h o mo g en eo u s (Fi g. 10. 1), so m eti me s w rin kl ed , an d s o me tim es w ith a v err uc o u s (Fi g. 10. 2 ) o r f is su re d

su rfa c e. T he w h ite p atc h m ay va ry fro m be in g tra ns pare n t a nd f ilm y in a pp ea ra n ce to b ei ng

den s e an d th ic k. In s o m e ca s es , th e leu k o pla ki a is a s in g le , di sc rete , a nd w e ll -ci rcu m s cri be d pla q ue ar ea , w he re as i n o the r p a ti en ts th ere m ay b e w i de sp rea d a bn o rma l ity of th e mu c o s a wi th a n u mb er of le s io n s di stri bu te d i n va ryi n g s i te s . In a Dan i sh po p u la tio n th e hi gh e st

in ci de n ce o f le uk o pl ak ia h as b ee n fo u n d to be o n the bu c c al m uc o s a an d c om m is su re s , an d

th e n , in d es c en di n g or der o f f req u en c y, o n th e a lv eo l ar rid ge s, to n g ue , bu c c al s u lc i, f lo o r o f mo u th , la bi al m u co s a, an d pa la te. Ho w eve r, in co m mon w i th al l oth e r s tati sti cs r ega rd in g

leu k op la ki a an d as s o ci ate d le si on s , i t mu s t b e r em em be red th at the f igu re s re fer to a s pec i fic po pu l ati o n an d th at o th e r p o pu la tio n s i n di ffe ren t a re as a n d wi th di ff ere nt in fl u en c in g fa cto rs (su c h a s tob a cc o h a bi ts) ma y sh o w d ive rs e c li n ic a l fe atu re s.

Histological features of oral leukoplakia

Th ere is a w i de ra n ge o f h is to lo gi ca l c ha n ge s th at c a n o c cu r in o ra l leu k o pla ki as , a n d th e se

ran g e f ro m h ype rke ra tos i s wi th ou t ep ith el ia l d ys pl as i a, to s q ua m o us e pi th eli a l d ys pl as ia an d

sq u am o u s ce ll ca rc in o m a in s itu . Kera tin o c ytes sh o w a n u m be r o f cy tol o gi ca l ch a n ges

(ce ll u la r a typi a) in ep ith el ia l d ys pl as i a. Th e va rio u s h is to lo g ic al c h an g es a re dis c u ss ed i n

deta il i n sta n da rd tex tbo o ks o f o ra l pa th o lo gy . No t a ll th e i n di vid u al c el lu la r a typ ia o c cu rs i n on e le s io n . It is c o n ve nti o n al fo r or al pa th o lo g is ts to

su b jec tive ly gr ad e th e de gr ee o f d ys pl as ia a s m il d, m o de rate , o r s ev ere d epe n di n g o n th e pro p or ti o n o f ke rati n oc yte l ay ers s h o wi n g c e llu l ar a ty pi a.

Fig. 1 0 .1 Ho m o gen e o u s le u ko pl ak ia o f th e fl o o r o f the m o u th an d la b ia l mu c o s a.

Fig. 1 0 .2 V erru c o u s le u ko pl ak ia a ffe cti n g f lo o r o f m ou th , a lv eo la r r id ge, an d ve n tr al su rfa c e o f to n gu e . (Hi sto p ath o l og y sh o w ed m o de rate dy sp la s ia ).

Erythroplakia

P. 113

As i mp li ed by its n a me , er yth ro pl ak ia is e ss en ti al ly a red l es io n o f th e m u co s a . A n al tern a tive te rm β€”eryth ro p la si a β €”is tak en f ro m the n a me g ive n to a s o me w ha t s im il a r le s io n o f th e

pen i s (ery th ro pl as ia o f Qu e yra t), w h ic h h as f o r s o me tim e be en r ec o gn iz e d as a p re ma li gn a nt les io n . T he o ra l le si on is re la tive ly u n co m mo n a n d ap pe a rs as a bri gh t r ed pa tc h tha t i s w ell def in e d f ro m the s u rro u n din g m u co s a an d h a s a ve lvet -lik e s u rfa c e te xtu re . Th ere m ay b e

oc c as io n a l wh i te a re as a djo i n in g th e le si o n a nd , in so m e ca se s , pa tch e s o f e ryth ro p la kia m a y be in ter sp ers ed w i th le u ko pl ak ic l es io n s. It is m o st of ten s ee n o n th e bu c ca l mu c o sa ,

al th o u g h it m a y o cc u r o n o th e r s ites o f th e or al m uc o s a, s u c h as th e s o ft p al ate , ve ntra l su rfa c e of th e ton g u e, a n d flo o r of th e mo u th .

Th e m o s t im po rta n t fe atu re o f thi s le si o n is th e h ig h in c id en c e o f c el lu la r a typ ia s h o wn o n hi sto l o gic a l exa m in a ti o n , an d o fte n th is is a s so c iated w ith a tro p hy o f the ep ith el iu m . A si gn if ic an t nu m be r o f th es e l es io n s d is pl ay ep ith e lia l aty pi a th ro ug h o u t al l ce ll ul a r

la yer sβ €”kn o wn as c a rci n o ma in si tu , a dia g no s is th a t w a rran ts i mm ed ia te su rg ic a l

in terv en tio n . P ara do x ic al ly , it h a s be en p o in te d o ut th a t s om e o f th e se l es io n s di sp la y

virtu a ll y n o a typ ia a n d, i n dee d, ma y b e o f a s im pl e in fl am ma to ry n atu re . It is qu i te e vi den t

th a t b io ps y ex am in a tio n i s es se n tia l i n a ll th es e ca se s . As i n th e ca s e of l eu ko p la kia , i t is b y no me an s c le ar as to wh e the r th e Can d id a is a p rim ar y o r a se co n d ary fe atu re o f su c h

les io n s . Ho w ev er, i t is g en e ral ly fe lt ( per ha p s wi th o ut an y cl ea r e vi de nc e ) th at the pre s en c e of Can d id a in th e ep ith e liu m i s lik el y to co i n ci de w ith a n i nc re as e d te n de nc y to ma l ign a n t

ch a n ge . Wh ate ver th e ae tio l og ic a l f ac to rs in v ol ve d, th e re s e em s littl e do u bt th a t a h ig h pro p or ti o n o f l es io n s c li ni c al ly di ag n os e d a s e rythro pl ak ia p res en t s u ff ic ie nt ep ith el ia l

ab n or ma li ties to be cl a ss if ie d o n h i sto l og ic a l e xa mi n ati o n as i n ci pi en tly ma li gn a n t. It w o ul d se em th at ery thr op la ki a wa rra n ts th e d es c rip tio n tha t h as be en a p pli ed to i t o f b ei n g th e mo s t s eri ou s o f th e o ra l pre c an c ero u s le si o n s.

Speckled leukoplakia

Th is le s io n c an be c on s id ere d as a v ari a nt of ei ther l eu ko p la ki a o r er yth ro pl ak ia . It a pp ea rs

as a s eri es o f w hi te no d u la r pa tc he s o n a n ery the m ato u s ba c kgr ou n d a n d s o , in ap pe ara n c e, is m idw a y be tw e en th es e tw o c o n di tio n s . An y a rea o f the o ra l mu c o sa m a y b e i n vo lv ed , bu t ma n y le si o n s ap pe ar o n th e b u cc a l m u c os a n ea r to the c o mm is s u res ( Fi g. 10. 3).

His to lo g ic al ly, th e se l es io n s s ho w a h i gh i n ci de n ce o f atyp ia a n d, in m a n y c as e s, th e

pre se n ce o f ca n di da l hy ph a e in th e ep ith el iu m . Th e sa m e do u b ts h a ve b ee n exp re ss ed a bo u t th e e xa ct r ol e o f Can d id a in th e se l es io n s as i n th e ca s e o f h o mo g en eo u s le uko pl ak ia s, bu t

th e re is n o d o ub t th a t th ere a re f req u en tly tw o c o in ci de n tal fa cto rs i n a ll th es e l es io n s β€” th e pre se n ce o f Can d id a an d th e pre se n ce o f ep ith el ia l aty pia . Wh e the r th ere is a c au s al e ffe ct

rem ai n s to be d ete rmi n ed . Th e les io n s s h o u ld al w ay s be c on s id ere d as p o ten tia ll y ma li gn a n t. Al tho u g h a cc u ra te i nf o rma tio n i s n o t a s yet av ai la bl e, a ll a va ila b le su rv ey s su g ge st th a t sp ec kl ed le u ko pl ak ia s h av e a s ig n if ic an tly h ig h er rate o f ma li gn a n t tra n sf or ma tio n th a n ho m o ge n eo u s le si o n s.

Fig. 1 0 .3 Ca n di da l s p ec kle d le u ko pl ak ia a t th e c o m mi ss u re β €”a c h a rac ter is tic si te.

P. 114

Candidal leukoplakia

Can d id al l eu ko p la kia i s al s o kn o w n a s ch ro n ic h y perpl as tic c a nd id o si s. Th e i mp o rta nt ro le o f Can d id a as a n a eti o lo gi c al fa c to r in th e pr od u cti o n o f o ral le u ko pl ak ia h a s al rea dy b een

dis c u ss ed . It i s no t ea sy to a sc ri be a ch a ra cte ris tic a p pea ra n ce to c a n did a l l eu ko p la ki as a n d, in de ed , a w id e v ari ety o f l es io n s m ay be s h o wn to in c lu de th e o rga n is m s. H o we ve r, it i s gen e ra lly ac c ep ted th at ca n di da l leu k o pla ki a s are fr equ e n tly sp ec kl ed a nd o f te n h a ve a so m ew h at irre gu l ar an d n o du l ar ap pe ar an c e. A h i gh pro po rti o n o f c o mm i ss u ra l le si o n s

exte n din g to th e ex te rn a l a n gl e of th e lip s a re ca ndi da l leu k op la ki as ( Fi g. 10. 3). Dia gn o s is o f a c a n di da l l eu k op la ki a ca n n o t be ma d e b y ta ki n g s u pe rfi ci a l s w ab s β€” th e h yp h ae a re wi th in th e le s io n a n d v ery fe w ma y be o n th e su rf ac e. F o r a r eli a ble d ia gn o s is i t is n e ce ss a ry to

sta in se c tio n s fro m a bi o ps y sp ec im en wi th pe rio d ic a ci d Sc h if f ( PA S ) re a gen ts . In th is w ay th e h yp h ae a re rea di ly s een wi th in th e su p erf ic ia l la ye rs an d s tratu m co r ne u m o f th e

epi th el iu m. In vie w o f th e in c re as ed ra te o f m al ig nan t c h a ng e in ca n di da l le u ko pl ak ia s, i t i s

of ev id en t i mp o rtan c e tha t th e se le s io n s sh o u ld b e rec o gn i z ed a nd re ga rd ed w ith h e igh te n ed su s pi ci o n.

Malignant transformation of precancerous lesions

Ora l le u ko pl ak ia is a m a rke r o f an i n cr ea se d ris k of c an c er an y wh e re in th e o ral ca vi ty, b u t to da te th e re a re n o re li ab le c lin i ca l o r h is to lo g ic al fe a tur es th at ca n b e us e d to pre di ct w h e th e r th e le s io n w il l reg res s s po n tan e o us l y, re ma in th e sa m e, o r pro g res s to c an c er. It h as b ee n rep or ted th at m a li gn a nt tran s fo rm ati o n o cc u rs in betw e en 4 an d 8 p er ce n t o f o r al

leu k op la ki as a n d ab o u t 15 p er ce n t w ill s po n ta n eo u sl y reg res s . Th e si te o f in vo l ve me nt is

im po rta nt an d in su b li n gu a l l eu ko p la ki as th e ra te of tran s fo rm a ti o n is a s h ig h a s 40 pe r c en t. Ery th ro pl aki a h as th e gr ea tes t ten d en c y to d ev el o p i n to a ma li gn a n t le si o n , w ith a

tr an s fo rm ati o n ra te a s h ig h as 80 pe r c en t a n d ab o ve.

The management of precancerous lesions

It is n o t w ith in th e re mi t o f th i s bo o k to de al in an y de tai l w i th the s u rgi ca l trea tm en t o f

leu k op la ki as . It s h o u ld be p o in ted o u t, h ow e ve r, that o n e o f th e p ro ble m s in th era py li es i n ac cu ra te ly de fin i ng th e ar ea a t ris k. It is e vid en t th a t th e o ral m u co s a a s a w h o le m u st, to

so m e e xte nt, b e ex po s ed to th e pa rtic u la r ae tio l og ic a l a g en t, w h eth er th e l atte r b e i n trin s ic or ex trin s ic . Th u s, it i s o ften dif fi cu lt to co m e to a n ar bitra ry de c is io n a bo u t th e li mi ts of

su rg ic al o r la s er e xc is io n ne c es sa ry fo r o p tim u m trea tme n t. It is b y n o m ea n s u n co m mo n f o r

an a pp a ren tly g en ero u s e xc is io n to p ro ve in a de qu a te o r fo r en tire ly n ew f ie ld s o f a bn o rm a lity to ap pe ar . Ind ee d, i t is c l ea r th a t le u ko pl ak ia o fte n rep res e nts mo re th a n a lo c al iz e d

ab n or ma li ty a n d th a t th e w ho l e o f th e mu c o sa m ay b e in vo l ve d, e ven th o u g h lo c al iz e d l es io n s ma y b e the e xp res si o n o f th i s. S im ila rl y, th e rec u rren c e o f l es io n s re pa ire d by mu c o sa l gra fts

or al lo w e d to ep ith el ia li z e c a n be e as il y e xp la in e d b y th e re al iz a tio n th at su c h a m an o e uv re is eff ec tive o n ly in rem o vin g s pe ci fi c ep ith el ia l c e lls th a t h a ve be co m e in vo l ve d i n a c om p lex

ab n or ma li ty. If th e p re-exi sti n g a e ti o lo g ic al fa c tor s rem a in , th en r ec u rren t a b no rm a li ty m ay be ex pe cte d e ve n th ou g h th e ae tio lo g y m a y rem a in u nre co g n iz ed . It m ay w el l b e, i n rec u rren t c a se s o f th i s na tu re, th a t ab n o rma l me s oder ma l β€“epi th el ia l re a cti o ns a re

res po n s ib le fo r th e in i tia l le si o n a nd a re ma in ta ine d b y th e p res en c e o f th e in fl u en c in g co ri u m afte r su rg er y. Th e re i s in c rea s in g do u b t reg a rdi ng th e rec u rre n ce o f cry o su rg ic al ly trea ted

leu k op la ki as a s c ar ci no m a , eve n th o u gh i t s ee ms th at so m e de gre e o f u n de rtrea tme n t mi gh t be in vo l ved i n s uc h c a se s. C u rren tl y, ex ci s io n o f th e le si o n s us in g a CO 2 la s er i s fe lt to b e a

sa fe r a lte rn ativ e. Ce rtai n ly, th i s te c hn i qu e gr ea tly red u ce s the n e ce ss i ty fo r gra fti ng o f

exc is e d ar ea s, th e re is li ttle tis s ue d is to rtio n fol lo w in g re -epi th el ia liz a tio n , a n d, i n term s o f po s to p era tiv e dis c o mf o rt, it i s gre atl y p ref era b le to cr yo th era py .

Ap ar t fro m s u rgi ca l trea tme n t it is po s s ib le to o bta in a m a rke d re gr es si o n o f a s ig n if ic an t

nu m be r o f to ba cc o -in du c ed le u ko pl ak ia s by di sc o n tinu in g th e ha b it. B io p sy s tud ie s o f s u ch

reg res s io n le si o n s ha ve s h o wn ma rke d red u cti on s i n th e i n ci de n ce o f atyp ia a s w el l a s i n th e exte n t o f th e le si o n s. T hi s ma n o eu vr e s h o u ld be th e fir st em pl oy ed in the ma n a ge me nt o f

les io n s i n w hi c h a to ba cc o -me dia te d a eti o lo gy i s sus pe cte d. S im ila rl y, in tho s e le si o n s wi th

as so c ia te d c a nd id a l i n fec tio n s , it is o fte n po s s ible to ef fec t a m ar ked i mp ro ve me nt by th e u s e of s ys tem ic a n ti fu n ga l s. It is u n li ke ly tha t a c o mple te r eg res s io n w il l b e o bta in ed i n th is w ay ,

bu t th e red u cti o n in th e s iz e o f th e le si o n ma y ma ke th e eve n tu al s ur gi ca l ma n ag em en t mu c h si mp le r. Th e de vel o pm en t o f s af er s ys te mi c an tif u nga l a ge n ts, s u c h as fl uc o n az o l e, h a s fac i lita ted th ei r u s e in th e ro u tin e ma n ag em en t o f c an d id al le u ko pl ak ia .

Leu ko p la kia s w ith l ittle o r no dys p la si a ar e o ften lef t u n trea ted , pa rtic u la rl y i f p ati en ts a re ab le to re du c e o r sto p sm o ki n g o r ch e w in g tob a cc o . Les io n s a re mo n i to re d o n a lo n g -te rm ba si s, b o th cl in i ca ll y a n d h is tol o gi ca ll y, a s a pp ropri ate . Th is p o li cy o f β € wa tc hf u l

wa iti n gβ€™ is c le arl y u n s ati sf ac to ry an d re lie s o n th e e xp eri en c e an d s ub jec tiv e as s es s me n t of th e cl in ic ia n . Th e ke y to m a n ag in g o ra l l eu ko p laki a is li ke ly to be th e es tab li sh m en t o f

mo le c ul ar a ss a ys th at ca n re de fin e th e as s es sm en t of th e ris k o f m al ig n an t tra n s fo rm ati on

an d h o pe fu ll y l ea d to su c c es sf u l m eth o ds o f ch e mo p rev en tio n , s u ch as th e u se o f reti no i ds . Mo l ec u la r m ark ers a re di s cu s se d in th e n ex t s ec tio n o f th i s ch a pte r.

Precancerous conditions Oral submucous fibrosis (OSF)

Th is is a d is ea s e th a t w a s fir st r ec o gn iz e d i n th e 1950s , d es pi te p res u mp tiv e e vi de nc e th at it

mu s t h av e b ee n p res en t l o n g be fo re th at ti me . Th e va s t ma jo ri ty o f ca s es h a ve be en f ou n d in th e In di an su b c on ti ne n t, al th o ug h s im il ar exa m pl es h a ve be en re po rte d in o th er A si ati c co u n trie s as w e ll as a n i n cre as i ng n u m be r o f p a tients i n th e U K.

Ora l su b mu c o u s fib ro si s (O SF ) is a c o nd iti o n in w h ic h f ib ro us ti ss u e is la id d o wn in th e

co ri um o f th e o ra l mu c o s a. Si mu l ta n eo u s c h an g es o c cu r in th e o ra l ep ith el iu m . In th e e a rly ph a se v es ic le s an d s ma ll

P. 115

ul ce rs m ay be f or me d, bu t th i s sta ge is s o o n s u pe rse de d by o n e o f ge n era li z ed ep ith e lia l

atro p hy . Th e ef fec t o f th e f ib ro si s is a s tiff en in g o f the o ra l mu c o sa l ea di n g to dif fic u lty in

op en i n g th e mo u th a n d to a bi nd in g d ow n o f th e to n gu e. Th e a pp ea ra nc e o f th e mu c o s a i s o f a b la n c he d, β € ma rbl ed β €™ na tu re, wh i ch s e em s to be q ui te ch a ra cteris tic o f th e c o n di tio n .

It is p os s ib le to pa l pa te b a nd s o f f ib ro u s tis su e with i n th e m u co s a a nd i t is re po rte d tha t eve n tua ll y th e s ca r fo rma tio n w ith in th e s o ft p al ate is s u ff ic ie nt to ca u s e th e n ea r dis a pp ea ra n ce o f th e u vu la .

Th e c a u se o f OS F is n o t k n ow n w i th ce rta in ty, b u t rec e nt wo rk s u gg es ts tha t b o th a ge n etic su s ce pti bi lity a nd a f ibr o bla s tic res p on s e to a rec a (be tel ) n u t c h ew in g m ay be i n vo lve d.

Ep id em io l og ic a l s tu di es i n dic a te th a t th is c o n di tion is i n du c ed by ch e w in g ar ec a nu t an d it is th e ref o re p a rtic u la rly c om m o n in A s ia n c o mm u ni tie s. S tu d ie s in di c ate th ere i s p ro b ab ly a

gen e tic p red is po s iti on fo r O S F an d th e ro le o f a u toim mu n ity is a ls o b ei n g i n ves tig a ted. Th e

in fl ue n ce o f n utri tio n a l fa cto rs , i f a n y, re ma in s un c le ar. P a tie nts w i th OS F h a ve an in cr ea se d ris k o f de ve lo pi n g o r al c arc in o m a, wh i ch h a s be en es tim ate d to be a s hi gh a s 10 p er ce n t

ov er 10 to 15 y ea rs . So fa r, th ere do e s n o t se em to b e an y sa tis fa c to ry trea tme n t fo r OS F al th o u g h in tra le si o na l s te ro id s h av e b ee n u s ed . Pr im ary pre ve n ti o n by re du c in g th e u s e o f

are ca n u t p ro du c ts w ou l d ap pe ar to be th e be st w ay fo rw a rd to red u ce th e in c id en c e o f O SF . A co n di tio n , kn o w as β€ bete l- ch e we rs β€™ mu c o sa β €™ ha s b ee n de sc ri be d i n w hic h th ere is

bro w n is h -red di s co lo r atio n of th e o ral m u co s a (pa rtic u la rl y th e bu c c al m uc o s a) to ge th e r w i th des q ua m ati o n o r p ee lin g o f the m u co s a . Th e l a tte r ma y b e p a rtly du e to tra u ma f ro m ch e wi n g. In a dd iti o n, th e m u co s a o ften h a s a w rin k led ap pe a ran c e. T h is is n o t per s e

co n s id ere d to be a pre c an c ero u s l es io n b u t ma y pro gres s to o ra l s u bm u c ou s f ib ro si s an d /o r or al le u ko pl ak ia . P ati en ts ar e o f te n re lu c tan t to sto p ch e w in g qu id , a lth o u gh th is i s th e k ey to su c ce s sf ul m an a ge m en t.

Sideropenic dysphagia

Si de ro pe n ic dy sp h ag ia i s al so kn o w n as th e P ate rso nβ €“Kel ly (o r P lu m me rβ €“Vi n so n )

syn d ro me a n d pre do m in a ntl y a ff ec ts mi dd le -ag ed fe male s w h o h av e i ro n de fi ci en c y ( se e a l so Ch ap ter 13 ). Th e o ral a n d p h ar yn ge al m u co s a ma y ap pe ar atr op h ic a n d sh in y re d. Or al

leu k op la ki as a n d mu l tipl e sq u am o u s ce ll c arc i no m a s ca n d eve lo p in th i s co n d itio n bu t i t is pa rtic u la rly a ss o ci a te d w ith p os tc ric o id ca rc in o m a.

Lichen planus

Th e p rem a li gn a nt po ten ti al o f or al li ch e n pl a nu s i s di sc u s se d in Ch ap ter 11 an d th e ma li gn a n t tr an s fo rm ati o n ra te i s ci ted a s 0.4 β €“3. 3 per c en t. Wh eth e r o r no t p a tien ts w ith atro p hi c o r ero s ive f or ms o f o ral li c he n p la n us a re m or e s u s ce pti ble to m a lig n an t ch a n ge h as ye t to be pro ve d by lo n g- te rm pr os p ec tive s tu di es .

Other precancerous conditions

Dis co i d l u pu s ery th em ato s u s ha s b een cl as s ifi ed a s a pre ma l ign a n t c o n diti o n bu t o n l y a fe w ca se s o f m a li gn a nt tran s fo rm ati o n o f l ip le s io n s h av e b ee n re po rte d. Te rtia ry sy ph i lis i s

rare ly re po rted th e se da ys b ec a u se o f ea rly rec o g nitio n a n d trea tme n t. In th e p a st, o ra l leu k op la ki as a n d sq u am o u s ce ll c ar ci no m a s de vel o pe d in a s so c ia tio n w i th th e a tro ph i c

glo s s itis o f terti ar y s yp h ili s bu t th e u se o f po te nti al ly c arc in o g en ic a g en ts su c h a s ar se n ic to tr ea t th is c o nd iti o n ma y h av e pre di s po s ed to th is tra n sf o rma tio n . O th er r are c o n diti o n s th a t ha ve o ra l ma n if es tati o ns h a ve b een rep o rted a s pre ca n c ero u s a nd i n cl ud e xe ro de rma

pig m en to su m a n d e pi de rm ol ys is b u llo s a . Ov era ll , c ond iti on s i n w h ic h th ere is ep ith el ia l atro p hy a pp ea r to b e as s o ci ate d wi th an in c rea s ed ris k o f m al ig n an t tra ns fo rm a tio n .

Oral carcinoma and carcinogenesis

Ma li gn a n t tu mo u rs o f th e h ea d a nd n e ck in c lu d e sq u am o u s c ell c ar ci n om a (S CC ) o f th e o ra l

ca vi ty , la ryn x , an d p ha ryn x , sa l iva ry/ gla n d ul ar c anc ers , m al ig n an t m el an o m as , l ym ph o ma s , an d sa rc o ma s . S CC is th e m o s t co m m on ne o pl as m o f the h ea d an d n e ck an d a cc o u n ts fo r mo re th an 90 pe r ce n t o f al l o ra l ma li gn a n ci es . Wo rl dw id e, th e a n nu a l in c id en c e o f S CC

exc ee ds 300 000 w i th ap pro x im ate ly 2000 n ew c a se s be in g reg is ter ed pe r y ea r i n th e U K.

Ea c h ye ar n ea rly h al f th a t n u mb er di e fro m o ra l S CC (O S CC). T h e h i gh m o rbi di ty ra te i s du e to a nu m be r o f fa cto rs i n cl u di ng l ate pr es en ta tio n , fa il ur e to res p on d to tre atm en t r eg im en s

cu rre n tly av ai la bl e, a n d a l a ck o f s u ita bl e ma rk ers fo r ea rly d etec tio n . T he d en ta l p ro fe ss io n ha s a c ru ci al ro l e i n th e ea rly d etec tio n of S CC, wh i ch , i f tre ate d ea rly , h as th e be st

pro g no s is . Cu rr en t ap pr oa c h es fo r co n tro l lin g th is c a nc e r in c lu d e im pro ve d pre ve nti o n (ri sk

fac to rs su c h a s to ba cc o a re w el l re c og n iz e d) an d earl y d ete cti on of p ati en ts w ith su s p ic io u s or al le si o n s.

Aetiological factors for oral squamous cell carcinoma (OSCC)

Th e a e ti o lo g ic al fa cto rs i mp li ca ted i n OS C C a re to ba cc o u s e, a lc o h o l c o n s um p ti o n , su n l igh t

(ca n c er o f th e lip ), d iet an d n u triti on a l sta tu s, ch ro n ic c a nd id a l i n fec tio n s , vi ral i nf ec tio n s , an d im mu n e de fi ci en c y. O f th e se , to ba c co u s e an d a lc o h ol a re co n s id ere d to be th e mo s t

im po rta nt. C o n si de rati o n o f a ll th es e fa cto rs i s clea rl y o u ts ide th e sc o p e o f th is b oo k bu t th e

ro le o f to b ac c o, al co h o l co n s u mp tio n , a n d d ie t w il l b e bri efl y co n s id ere d. Th e p o ten tia l ro le o f viru s i n o ra l c a rci n o ge ne s is is d is cu s s ed in a la ter s e cti on o f th is c h ap ter.

Th e s p ec u la tive re la tio n sh i p be tw e en Can d id a sp ec i es a n d m a lig n an t tr an s fo rm ati o n o f o ra l

leu k op la ki a h as a lre a dy be en d is c us s ed in Ch ap ter 4.

Tobacco use

To ba cc o u s e, i n a n y o f its fo rm s (c ig are ttes , c ig ars , pi pe -sm o kin g , to ba cc o -ch e wi n g, re ve rse

sm o kin g ), is o n e o f th e m o st i mp o rta nt ae tio l og ic a l f ac to rs in the d eve lo p me n t o f O S CC. Th e rel ati ve ris k

of de ve lo pi n g o r al c arc in o m a fro m c ig are tte- sm o kin g d ep en ds o n a n u m be r o f fa cto rs ,

P. 116

in cl u di n g l ev el o f c o n su m pti o n an d w h eth er th e c i garette s are h ig h o r l o w tar . Ho w ev er, a

rea so n a bl e es tim ate is th a t a n in d ivi du a l wh o s m o kes m o re th an 20 ci ga re tte s a da y h as a

ris k o f de ve lo pi n g or al c arc i no m a 10 tim es h i gh e r th an th at o f a n o n sm o ke r. Pi pe - an d ci ga r sm o kin g h a ve be en l in ke d wi th c an c er o f th e li p. In o n e reg io n o f In di a, w h e re r eve rs e

ch e ro ot sm o ki ng i s pra c ti s ed a mo n gs t w o me n, a gre atl y i n cre as e d i n ci de n ce o f ca rc in o ma o f th e p al ate in fem a le pa tie n ts ha s b een rep o rted . Rev ers e sm o ki n g i s al so co m mo n in o th er

pa rts o f th e w o rld . Th e in c rea se d in c id en c e o f o ra l c a nc e r in Ind ia i s li kel y to be du e to th e co m mo n p ra cti ce o f sm o ki ng b id is β€”a c h ea p typ e o f c ig are tte ( ma de fr om lo c al to ba c co ro lle d in a l ea f)β €”an d rev ers e sm o ki n g.

Th e ch e w in g o f b ete l q u id is e n de mi c thr ou g h o ut the In di a n su b co n tin e n t, So u th -ea st A si a,

an d la rg e p ar ts o f th e we s te rn P a ci fic . O n ly th ree s ub s tan c es , n ic o tin e, eth a n ol , a nd c a ffe in e, are c o ns u m ed m or e w i de ly th a n b ete l. Q u id co n s u mp tio n i s h ig he r i n fe ma le s a nd th er e i s

co n s id era bl e r eg io n al va ri ati o n in i ts co n s titu ten ts . Q ui d h as b ee n de fin e d as β€ a s u bs ta n ce

or mi xtu re o f s u bs ta n ce s pl ac e d i n th e m o u th o r c h ew e d a n d rem a in in g in co n ta ct w i th the

mu c o sa , u su a ll y co n ta in i ng o n e o r b oth of th e two ba si c in g red ie nts , to ba c co or a rec a n ut, i n raw o r an y m an u fa c tu re d o r p ro ce ss e d f o rmβ €™. In g red ie nts c o m mo n ly u se d in th e

pre pa ra ti o n o f b ete l qu id a re li ste d i n Tab le 10. 1. Pa n ma s al a h as a ll th e in gr ed ien ts o f the

bete l qu id , ex ce pt th e b etel l ea f, a n d i s co n v en ie ntly pa ck ag ed i n sm a ll s ac h ets a n d ti n s (Fi g 10. 4).

A nu m be r o f c as e co n tro l stu d ie s ha ve re po rte d an in c rea s ed rel ati ve ris k o f d ev elo p in g

ca n ce rs o f th e o ra l c a vity d ue to th e u se o f be tel qu i d, w ith o r w ith o u t to ba cc o . O ral

su b mu c o us f ib ro si s an d o ra l le u ko pl ak ia , bo th c o n side red to b e pre ma li gn a n t, are a s so c ia ted wi th be tel -ch e wi n g ( se e β€ Pre c an c ero u s le s io n s β €™ an d β€ Pre c an c ero u s c o nd iti o ns β €™).

It is re po rte d th a t th is h a bi t im pro v es di ge sti o n an d s al iv ati on , d im in i sh e s hu n g er pa n gs , a nd pro d uc e s a f ee li ng o f eu p h or ia , an d s o me c la im it has a ph ro d is ia c po w e rs. C h ild re n as y o un g as 3 y ea rs o f a ge p ar ti c ipa te in th i s pra c ti c e. It i s an e s se n tia l e le me n t in s o c ia l, cu l tu ral , an d ec o n o mi c lif e in m an y pa rts o f the w o rld .

Ch ew in g o f be te l qu id i s as s o ci ate d w i th o ral l eu kop la kia , o ra l su b mu c o u s fib ro s is , an d sq u am o u s ce ll ca rc in o m a.

To ba cc o -ch e wi n g i s tra diti o n al ly pra c tis ed by m in ers, a s s mo ki n g i s o bv io u sl y d a ng er ou s

un d erg ro u n d. M in e rs w ho ad o pt th i s pra c ti c e a re , the ref o re, su s c ep tibl e to de ve lo pi n g o ra l leu k op la ki a, wh i ch m a y u n d erg o ma l ign a n t tra ns fo rm atio n .

It is im p or ta n t to a pp re ci ate th at tob a cc o u sa ge i s n ot o nl y a n i mp o rtan t r is k f ac to r f or o ra l ca rc in o ma b u t a c om m o n ae tio l og ic a l f ac to r o f o ra l l eu ko p la ki a (se e ab o ve) .

Alcohol consumption

Al co h o l co n s u mp tio n a s a ris k fa cto r fo r o ra l ca rc in o m a is d iff ic u lt to q u an ti fy, p ar ti c ul ar ly as ma n y pa tie n ts wh o d rin k h ea vi ly al so h av e a h i gh c on su m pti o n o f c ig ar ettes . Th e ge n era l

co n s en s us , h o w ev er, i s tha t a lc o h o l i s an in de pe n den t ris k fa cto r bu t th e e ffe c t o f a lc o h o l an d to ba cc o to g eth er is m u ltip li ca tiv e, th a t is , g rea ter th an th e a dd ed ris k o f a lc o h o l o r to b ac c o .

Table 10.1 Ingredients commonly used in the preparation of betel quid Be tel le af (a ls o k no w n a s pa n )

Are c a nu t ( su p ari )β €”se ed o f a re ca c a tec h u tree Lim e (c al ci u m hy dro x id e)

Cate c hu (res in o u s ex trac t f ro m ac a ci a tree ) To ba cc o

Fig. 1 0 .4 P an m a sa la , o n sa l e to th e p ub li c.

Diet and nutritional status

Th e r ol e o f d ie t an d n u triti on a l sta tu s in th e p reven tio n of o ra l c a n ce r h a s be en th e s ub jec t

of a n u mb er o f s tu di es a n d i t is g en e ral ly co n s id ere d tha t d ef ic ie nc i es o f vita mi n s A, C, a nd E ma y p red is p os e to th e d ev el o pm en t o f o ral c ar ci n om a. Lac k o f d ie tary ir on ma y lea d to

ae ro di ge sti ve (in c lu d in g o ral ) c a rci n om a s as i s in th e P lu m me rβ€“Vi n so n sy n dro m e. H ig h

co n s um p ti o n o f f ru it a n d fre sh veg eta bl es m ay l ow e r th e ris k, a n d rec e nt stu d ies s u gg es t th a t p ati en ts w ith h e ad a nd n ec k c a n ce rs h av e a h i gh f at an d re d m ea t i n take .

Clinical features and diagnosis of oral squamous cell carcinoma

P. 117

No t al l or al c arc i no m a s are p rec ed ed b y a rec o gn i z ab le pr em al ig n an t l es io n , a lth o u gh i n

ma n y c a se s evi de n t a bn o rm al ity ma y h av e b ee n p res ent f o r s o me tim e ( Tab le 10. 2). Th e

si gn if ic an c e o f l eu ko p la ki a as a p rem al ig n an t l es ion h a s be en dis c u ss ed a bo v e bu t i t m u st b e rep ea ted th at th e c li ni c al c rite ria b y w h ic h a w h ite pa tc h ma y be ju dg ed a s pre ma li gn a n t a re far fro m c le ar , an d th at it i s o nl y a fte r a s tu dy of th e h i sto l og ic a l a p pe ara n ce o f th e l es io n th a t a n y a ttem pt at p ro gn o s is c a n be ma d e.

Th e c la s si c al s ig ns o f a ma l ign a n t c o nd iti o n, wh i ch sh o u l d a ro u s e i mm ed ia te su s pi ci o n , ar e: 1. 2. 3. 4.

per si ste n t u lc era tio n : an y u ne xp la in e d u l ce r th a t la s ts fo r l o n ge r th an 10 da ys s h o u ld

be trea ted w ith s u s pi ci o n;

in du ra tio n : th i ck en in g a n d h a rd en in g o f th e tis sue s; pro li fe rati ve gr ow th o f tis su e a bo ve its n o rm al le ve l, o fte n w ith c h an g es i n th e s u rfa c e

te xtu re a nd c o lo u r ch a n ge s;

fix ati on o f th e af fec ted ti ss u e to the u n de rlyi n g s tru c tur es .

Pa in i s n o t a lw ay s pre se n t in th e e ar ly sta ge s o f a c a rci n o ma a n d th e p ati en t m ay b e q u ite

un a w are o f an y ab n o rma l ity u n ti l th e le si o n h as b eco m e l ar ge an d s ec o n da ril y i n fe cte d. If the aff ec ted a rea i n cl u de s th e tee th , th es e m a y b ec o me m o bi le du e to th e rep la c em en t o f th e

per io do n ta l m e mb ran e b y tu m ou r. Th u s, u n ex pl ai n ed ra pi d l o o se n in g o f th e tee th sh o u ld b e ca ref u lly i nv es tig ate d.

Lymp h n o de i nv o lve me n t ma y o cc u r e ar ly in o ra l ca rcin o ma . H ow e ve r, en la rg ed re gi on a l

lym ph no d es m ay i n fa ct sh o w o n ly n o n -sp ec if ic in f la mm ato ry c h an g es in so m e ea rly c as es .

Un fo rtu n a te ly , th is c an n o t b e r eli ed o n a n d, i n s ome ca s es , m al ig n an t d ep o si ts in th e ly mp h no d es a re fo u n d wh i le th e p rim a ry l es io n is s ti ll sma ll . It is ce rta in ly tru e tha t p ro gn o s is i s mo re fa vo u ra bl e i f tr ea tme n t is i ns titu te d be fo re th e l ym ph n o d es a re in vo l ved .

Les io n s sh o w in g th e si gn s o u tl in e d a b ov e are i mm ed ia tel y s u sp ec t b u t s uc h a l es io n i s at a rel ati vel y l ate sta g e ( Fi g. 10. 5). In its e arl y s tage s, a c a rc in o ma m a y sh o w n o n e o f th e se

si gn s a nd m a y b e d ete cta bl e o nl y by a ch a n ge in co lou r o r su rf ac e tex tur e o f th e m u c os a . It ma y b e im po s si bl e to di ffe ren tia te cl in i ca ll y be tw een le uk o pl aki a o r e ryth ro pl a kia a n d ea rly

ca rc in o ma a n d, th u s , bi o ps y ex am in a tio n i s ma n da to ry i n e very c as e in w h i ch th e po s s ib ili ty of c arc i no m a ma y ar is e. A n y pro lo n g ed u lc er , ery th ema , o r u n e xpl a in ed w h ite pa tc h sh o u l d be in ve s ti ga ted i n th is w ay . A di a gn o si s o f c a rci n oma s h o u ld be fo l lo w ed a s ra pid ly as po s si bl e b y tr ea tme nt an d th u s it i s es s en tia l th a t th e su rg eo n w h o c a rrie s o u t bi o ps y

pro c ed u res s h o ul d be in a p o s itio n to a rra n ge fo r im me di ate tre atm en t if req u ire d. F o r th i s

rea so n , i t is o f te n b etter fo r s u c h in ve s ti ga tio n to be ca rri ed o u t i n sp ec ia li s t ce n tres ra th er th a n i n the d en ta l p ra cti tio n er' s su rg er y. P ro gn o s is i n la te o ra l c arc in o m a is p o or a nd m u ch

dep en d s o n th e e a rly de tec tio n o f th e l es io n b ef o re th e c erv ic al n o de s be co m e in vo l ved , th e ov era ll 5-yea r s u rvi va l rate be in g e ffe cti vel y d o u bl ed if th e les i on is di s co ve re d ea rly . It is

al so tru e th a t th e p ro gn o s is b ec o me s w or se th e fu rth er ba ck in the o ral c a vity th e l es io n l ie s. Th us , i t is q u ite cl ea r th a t th e pa tie nt' s su rvi va l ma y de pe n d o n a th o ro u gh sy ste ma tic

exa m in ati o n o f th e o ra l m u c os a . Th e u se o f to lu id ine bl u e d ye h a s be en a dv o ca ted a s an

ad ju nc t to th e c lin i ca l di ag n o si s o f su s p ic io u s o ral l es io n s . Pr od u cts s u c h as O ra Te st Β® are

no w a p pro ve d in th e UK fo r o ra l s cre en i ng , d etec tion o f s ec o n d p rim a ry l es io n s , a nd d efi n in g ma rgi n s o f le si o n s fo r b io ps y an d s u rge ry an d a re th e s u bje ct o f o n go in g c li n ic al tria l s.

Su s pi ci o u s or al le s io n s sh o u ld a lw a ys b e b io p si ed re ga rdl es s o f th e re su l ts o f to lu id in e bl u e sta in in g .

Table 10.2 Oral carcinoma: suspicious clinical features

Pe rs is ten t, u n exp la in e d u lc era tio n In d u rati o n

Pro l ife ra tive g ro wth

Ch an g es i n tex tu re a n d co l ou r Fi xa ti o n to u n de rly in g tis su e s Su d de n lo o s en in g o f teeth Lymp h n o de i nv o lve me n t

Pa in β €”of ten a l ate fe atu re

Car ci n om a o f th e l ip is a ra th er di ffe ren t l es io n in th at it i s cl ea rly vi si bl e a n d so is n o tic ed ea rly . It h as a m u ch bette r p ro gn o s is th an in tra o ral ca rc in o m a an d ea rl y d ia gn o s is o fte n im pli es s u c ce ss fu l a nd re la tiv ely sm a ll -sc al e su rg ery . Th e lo w er li p i s pra c tic al ly a lw ay s

aff ec ted , th e p a ti en ts be in g pr edo m in a n tly o ld er m ale s (Fi g. 10. 6). Th e ca rc in o m a is o fte n mi sta ke n in i ts ea rly s tag es fo r a h erp eti c l es io n , b u t its p ers is ten t n atu re s h ou l d aro u s e

su s pi ci o n an d th e sa m e c ri te ri a f o r i nv es tig ati o n sh o u ld b e ad o pte d as fo r in tra o ral l es io n s . Pa tie n ts w i th ac tin i c ch e il itis a re at in c rea s ed risk o f d ev el op in g c arc i no m a o f th e li p (se e Ch ap ter 6 ).

Fig. 1 0 .5 Ca rc in o ma o f th e pa la ta l m u co s a .

P. 118

Staging systems for oral carcinoma

In or de r to a id u n ifo rm ity in s tu di es o f th e e pi de mi o lo gy o f or al c arc i no m a a nd th e pro g n o si s of th os e s uf fe rin g fro m it, th e re ha ve b ee n a n u mb er of a ss e ss me n t m eth o ds in tro d uc e d. Th es e are g en er al ly kn o w n as s ta gi ng s ys tem s . Th e mo s t w id ely u s ed is th e TNM s ys tem

(tum o u r, n o de s, m e ta s tas is ). In th i s sy ste m sc o res a re gi ve n to ea c h pa tie n t de pe n de n t o n th e s iz e o f th e pri ma ry tu mo u r, th e ex ten t o f reg ion a l l ym ph no d e in vo lv em en t, a n d th e

pre se n ce o f dis ta n t me tas ta se s. Sta gi n g sy ste ms h a ve b een fo u n d o f c on s id er ab le va lu e in

se le cti ng th e mo s t a pp ro pri a te ma n a ge me nt an d a ss ess in g th e o v era ll p ro gn o s is o f p a tien ts in e ac h gro u p .

Fig. 1 0 .6 Ca rc in o ma o f th e lo w er li p.

The management of oral carcinoma

Th e m a n ag em en t o f o ral c a rci n o ma s h o ul d be c oo rd in ated b y a m u ltid is c ip lin a ry tea m,

in cl u di n g ma xi ll o fa ci al s u rge o ns , o n c ol o gi sts , ra dio the ra pi sts , s pe ec h th era pi sts , de di ca ted

on c o lo g y n u rs es , a n d o th er pe rs o nn e l in vo lv ed in reh a bi li ta tio n . In so m e ce n tres , a c li ni ca l ps yc h ol o gi st ma y a l so b e in vo l ved i n th e a s se ss m en t a n d reh a bi li tati on o f p ati en ts

un d erg o in g trea tme n t fo r o ral c an c er . Sp ec ia l is ts in pa ll ia tiv e c a re ma y a l so b e in vo l ved i n th e m an a ge me n t o f th e se p ati en ts w h o h av e a d va nc e d d is ea s e or fo r wh o m tre atm en t h a s pro ve d un s u c ce ss fu l . Th e trea tme n t o f ch o ic e de pe n ds o n a n u mb er o f f ac to rs in c lu d in g

pa tien t pre fer en c e, bi o lo gi c al a ge , ge ne ra l h e al th , an d s ite an d s tag in g o f th e tu mo u r. O ve r th e la s t 2 d ec ad es , th ere h a ve be en g re at a d va nc e s in th e rec o n stru c tio n o f pa tie n ts w ho ha ve u n de rgo n e s u rgi ca l trea tme n t fo r or al c arc in o ma an d th es e h av e l ed to a gre a tl y im pro ve d qu a li ty o f lif e.

Th e m a n ag em en t o f o ral c a nc e r s h ou l d be co o rd in a ted by a mu l tid is ci pl in a ry te am . Th e tre a tm e nt o f o ra l ca rc in o m a is c le arl y a c o m pl ex ma tter, w h i ch i n th e ma jo rity o f c a se s in vo lv es su rg er y (w i th rec o n stru c tio n ), ra d io th era py , o r a c o mb in a tio n o f bo th .

Ch em o the ra py is n o t ro u tin e ly u se d fo r th e trea tme nt o f or al c arc i no m a a t the pre s en t tim e . Ma n ag em en t reg im en s fo r ca rc in o m a m u s t fo c u s on lo nge vi ty a n d qu a li ty o f li fe. Ma n ag em en t reg im en s fo r ca n ce r m u s t fo c us o n lo n ge vity a nd q u al ity o f li fe.

Radiation mucositis

Dur in g the c o u rse o f rad io th e rap y ( wh i ch is li ke ly to la s t fo r se ver al w ee ks ) th e pa tie n t

dev el o ps a p ro gre ss iv e an d ge n era li z ed ery th em ato u s a nd u lc e rati ve res p o ns e o f th e o ra l

mu c o sa (ra d ia tio n m u co s iti s) (s ee Tab le 10. 3). Ra diati on mu c o si tis ge n era l ly sta rts ab o u t th e

se co n d w ee k a n d ma y b e ac c o mp an i ed by a c o mp la in t abo u t a n a lte red tas te. It is e xtrem el y pa in fu l , the p ro bl em be in g c o mp li ca ted b y th e fa ct tha t th e s a li va ry g la n ds a re al s o vir tua ll y al wa ys a ff ec ted , w ith c o ns e qu en t le ss e ni n g o f s al iva ry s ec reti o n an d xe ro s to m ia . S ec o n da ry ca n di do s is a lm o st in va ri ab ly o cc u rs , an d Gra m - ne ga tiv e o rg a ni s ms m ay p la y a s ig n ifi ca n t

ro le. Fo l lo w in g th e co u rs e of ra di ati o n th e m u c os i ti s gra d ua l ly su b si de s le av in g an atr op h ic

or al ep ith e liu m o n a re la tiv ely a va sc u la r s u bm u c os a. Th e sa l iva ry flo w ma y be pe rm an e n tl y red uc e d a n d th e p ati en t w i ll de vel o p si gn s a n d s ym pto m s rel ate d to sa l iva ry gl an d

dys fu n c tio n (s ee Ch ap ter 8). In th e l o n g te rm, rad ia tio n th e rap y m a y r es u lt i n tris mu s d u e to ti ss u e fi bro s is .

In th e a cu te p ha s e, s im pl e n on -as trin g en t mo u th w as hes ( fo r e xa mp le , so d iu m bi c arb o n ate o r ta b le sa l t) ma y h av e a s o o th in g ef fec t o n th e o ra l m u co s a . B en z yd am in e h yd ro ch l or ide

mo u th wa s h h as an ti -in fl am ma to ry, a n d an ti mi cro b ia l pro p erti es a nd m a y re du c e the s ev eri ty of th e m u c os iti s. Mi co n a z o le ge l m a y b e us e d to co mba t c an d id o si s. P a tie nts m a y o n ly be

ab le to to le rate a s o ft, bl a nd d ie t. In the l o ng term, th e c o mb in a ti o n o f d ry mo u th a n d a n atro p hi c mu c o s a ma y c a u se c o nti n u in g di sc o mf o rt fo r wh i ch ar tifi ci al s al iv as (a n d sa li va

rep la ce me n t ge ls ) are th e o nl y a va il a ble trea tme n t, to ge th e r w i th be nz y da mi n e h y dro c h lo ri de rin s e. Th o s e co n ta in in g a dd ed fl uo ri de m ig h t w ell be c o n s ide re d. Th e se p ati en ts be co m e

un u s u al ly su s c ep ti bl e to c ari es a ga in s t w h ic h to pi cal fl u or ide s m ay be e ffe cti ve. Pa tie n ts

sh o u ld b e en c o u rag ed to m a in tai n th ei r o ra l h y gi en e as m u ch as p o ss ib le . Ra di ati o n ca ri es

sh o u ld b e m a na g ed by a c o mb in a ti o n o f o ra l h yg ien e p ro ce du re s, r es to rati o n o f ea rly l es io n s , an d th e a p pli c ati on of to pi ca l fl uo r ide s . Th e ma n agem en t o f sa l iva ry gl an d h yp o fu n cti on h as bee n d is cu s s ed in Ch ap ter 8 .

Rec en t s tu di es h a ve sh o w n th a t s ele c ti ve de co n ta mi nati o n o f o ra l flo ra w i th PT A (po l ym yxi n E, to b ra myc i n, a n d am p ho te ric in ) lo z en g es re du c es th e du ra tio n a n d de gre e o f m uc o s itis i n pa tien ts irr ad ia ted fo r or al c arc i no m a β€” th i s sh o u ld b e s ta rted b efo re a n d d u rin g rad io th era p y (Tab le 10. 4 ).

Osteoradionecrosis

Rad io th er ap y f o r o ra l m a lig n a nc y al s o af fe cts th e va sc u la rity o f th e b on e , w h ic h be co m es no n vi tal . It is , th ere fo re, hi gh l y s u sc e ptib le to in fec tio n a n d the e ffe cts o f tra um a .

Os teo m yel iti s o cc u rs a s a c o n s eq ue n ce o f in fe cti o n an d re su l ts in a p ai n fu l n ec ro s is

(o ste or ad io n ec ro s is ) w i th sl o u gh in g o f th e o ve rly ing so f t tis su e s. M od er n me th od s o f

rad io th era p y h a ve red u c ed th is c o mp lic a tio n . Ho w ev er, ex trac tio n s in pre vio u s ly ir rad ia ted bo n e mu s t be c ar rie d o u t w i th gre a t ca re an d c o n si de rati o n sh o u l d b e gi ven to

an tib io tic c o ve r. Hy pe rba ric o xyg en th e ra py is n o w be in g in c re as in g ly u se d to ma n ag e the s e pa tien ts . It ap pe a rs tha t o s teo ra di o n ec ro si s a fter extra c tio n s is m o re li kel y w h e n a lo n ge r ti me h a s el ap se d si n ce ra di o the ra py (Tab le 10. 5).

Table 10.3 Oral complications of radiotherapy Ear ly o ns et

Later o ns et

Mu c o si tis

Rad ia tio n c a rie s

Al tere d tas te

Os teo ra di o n ec ro si s

Xe ro sto m ia

Tris m us ( fib ro si s o f mu s c le s a n d o the r s o ft ti ss u es)

P. 119

Se co n d ary in f ec tio n s

Dem in era l iz ati o n o f tee th Hyp ers en s iti vity o f te eth

Table 10.4 Prevention and management of radiation mucositis Ma in te na n c e o f o ra l h yg ie n e

Se le cti ve de co n ta mi n ati on (e. g . lo z en g es c o n tai ni n g po ly my xin E, to b ra my ci n, an d am ph o ter ic in )

Dieta ry ad vi ce (b la n d, n o n -irri tan t f oo d s an d a vo id al c oh o l ) Bl an d m o uth w a sh e s (e. g . sa lt/b ic a rbo n a te o f so d a ) Be n z yda m in e h ydro c h lo ri de (to pi c al u se ) Mi co n a z ol e ge l (o ra l c a n did o s is ) To pi ca l ste ro id s *

Ma n ag em en t of s al iv ary gl a nd h yp o fu n cti o n (Ch ap ter 8) * M ay pre di sp o s e to o ra l ca n di do s is .

Table 10.5 Prevention and management of osteoradionecrosis Arr an g e e xtra cti o n s be fo re rad io th er ap y co m m en c es Av oi d trau m a to o ra l m u c os a

Co n se rve tee th po s tra dio th e rap y β€”if po s si bl e

Ex trac tio n s s h o ul d be a tr au m ati c an d u n de r a n tib io tic c o ve r Rin s e w i th 0. 2% c h lo rh e xid in e , pri o r to ex trac tio n Co n si de r th e us e o f h y pe rba ric o xyg en

The role of the dental team in the management of patients diagnosed with oral carcinoma

Cli n ic al gu i de li ne s (s ee pro je ct 1 at the e n d o f c ha p te r) rec o mm en d th a t o nc o lo g y p ro to co l s fo r p ati en ts di ag n o se d wi th o ra l c a rci n o ma s h o ul d in c lu de a n e arl y p retr ea tme nt o ral

as se s sm en t a n d th e o rg a ni z ati on o f o ra l ca re , in c ludi n g a n y de nta l trea tme n t req u ire d.

Id ea ll y, a h o s pi tal de n tis t s ho u l d b e an in teg ra l par t o f th e o n co l og y tea m an d c an lia i se w ith th e p ati en t's o w n de n tis t to o rg an iz e d en tal tre atmen t pri o r to , d ur in g, a n d af ter th e ra py fo r or al c arc in o m a. An y pro p h yla c tic req u ire me n ts ne ed to b e in s ti tu ted pr io r to r ad ia tio n

th e ra py. E s s en tia l de nta l pro c ed u res a ls o n e ed to be c a rrie d o ut as s o o n a s po s si bl e, s o n o t to de la y ca n c er th e ra py. Pa tie n ts w h o h a ve u n de rgo ne ra di oth e rap y ma y b e at lif el o ng ri sk fo r o ra l dis e as e, pa rtic u la rly de n tal c ari es a n d perio d on ta l di se as e.

In p u t fro m a s pe ci al is t p ro sth o d on ti st is re qu ire d fo r th o s e p a tien ts w ho h av e u n d erg o ne

ab la tive p ro ce du re s in vo l vin g re co n s tru cti o n. En d o ss eo u s im pl an ts m ay b e u s ed to re tai n

bri dg es o r d en tu re s. T he d en ta l h yg ie n is t h a s an impo rta n t ro le i n as s is tin g th e p a tien t wi th

die tar y a dv ic e, th e ma i nte n an c e o f o ral h yg ie n e, a nd ap pl ic ati o n o f to p ic al fl u o rid es . P ati en ts us u a lly n ee d to be mo n i tor ed by th e d en ta l tea m at 2 - or 3- mo n th ly in terv al s a fter rad io th era p y.

Prevention of oral carcinoma

Th is c an be di vid ed i n to pri ma ry, s e co n d ary , an d terti ary p rev en tio n . Th e de n tal s u rge o n h as a p iv o tal ro le i n pre ve nti ve a sp ec ts o f o ra l ca rc ino m a an d is i n a n id ea l po s itio n to giv e ad vic e c on c er ni n g li fes tyl e (fo r e xa mp le , to ba c co ce ss a ti o n a nd d ie tary a dvi c e) a n d to ide n tify h ig h -ris k o ra l le si o n s ( se e Tab le 10. 6).

Oral carcinoma as a genetic disease

Ora l sq u am o u s ce ll c ar ci n om a s an d th o se a t o th er sites in the h ea d a nd n e ck a ris e as a co n s eq ue n ce o f mu l tipl e mo l ec u la r e ven ts . Th es e a re i n du c ed b y th e ef fec ts o f va rio u s

ca rc in o ge n s fro m h ab its s u ch as to ba c co an d in f lu enc ed b y e n vir on m en ta l fa cto rs , p o ss ib ly viru s es i n s o me in s tan c es , a ga in s t a b ac kg ro u n d o f h eri tab le res i sta n ce o r s u s ce pti bil ity . Hu ma n c an c er s

dev el o p b y ac c um u la tin g a ra ng e o f s o ma tic g en e tic c h an g es th ro u gh o u t th ei r p ro gre ss io n ;

P. 120

ho w e ver, th e mo l ec u la r b as is f o r th es e ch a n ge s rem ain s u n cl ea r i n th e m a jo rity o f c a nc e rs. It ha s be en po s tul a te d tha t g en e tic da m ag e af fec tin g th e ma jo rity o f a u to so m a l ch ro m o s om e arm s , in c lu di n g a c tiva tio n o f o n co g en e s an d /o r i na ctiva tio n o f tu mo u r su p pre s so r ge ne s

(TS Gs ) p os s ib ly w or kin g in co n ju n cti o n w ith a n im p ai red ca p ac ity o f DN A rep ai r m ec h a ni s ms , ma y b e a fa cto r in th e de vel o pm en t o f sq u am o u s c ell c ar ci n om a o f th e h ea d a nd n e ck . It is gen e ra lly a cc e pted th a t th ere a re th re e ma in cl as s es o f ge ne s in v ol ve d i n

tu m o u rge n es is β€” TS Gs, o n co g en es , a n d DNA rep a ir g ene s β€” al th o u g h di sti n cti on s b etw ee n

th e p arti cu l ar ge n es a n d th e ir pro p ertie s , an d th eir c la s si fic a tio n w ith i n a ce rta in g ro up , a re su b jec t to c o ntr ov ers y. O n c og en e s in c lu de s gr ow th fac to rs , gro w th fa c to r rec e pto rs , si gn a l

tr an s du c ers , a n d n u c le ar pro te in s . Ho w ev er, m u tati ons , am p lif ic ati o n , o r re arr an g em en t o f pro to -on c o ge n es m ay re su l t in th e a cti va ti o n o f o n co ge ne s , w hi ch le ad s to ma li gn a n t

tr an s fo rm ati o n. T h e r an g e o f o n co g en es th at h a ve b ee n stu d ie d i n o ra l c a rci n o ma (f o r

exa m ple , m yc a nd ra s g en e fa mi lie s an d e rB -1) a nd ac tiva ted o n c og en e s pro d u ce ei th er

ab n or ma l (mu ta ted ) p ro te in s o r ab er ran t e xp res s io n o f pro te in s at an in ap pr o pri ate s ta g e i n

th e c el l c yc le . A se c o nd g ro u p o f ge n es i s in vo l ved i n ma li gn a n t tra ns fo rm a tio n , TS Gs . O ne o f th e mo s t s tu di ed TS Gs is p 53, w h ic h is l o ca ted o n ch ro mo s o me 17p . Th e p53 g en e c o de s fo r

a 53 kDa n u cl ea r p h o sp h o pro tei n th a t h ol ds a k ey pos iti on in th e co m pl ex n etw o rk co n tro ll in g gen o m e sta bi li ty, c ell c yc le , a nd a po p to si s. p 53 a cts a s a TS G b y a rre s ti n g ce ll s ca rryi n g DNA da ma ge i n th e G 0 /G 1 ph a se , p ro vid in g a de qu a te ti me fo r the c el l's DN A rep ai r m ec h an i sm to fu n cti on an d , if u n su c c es sf u l, le a ds c el ls to a po p toti c de ath . p 53 m uta tio n s h a ve be en

pre vio u s ly rep o rted i n ma n y c a n ce r s tu di es in c lu d ing o ra l c a nc e r a n d a re co n s id ere d to be

th e m o st f req u en t g en e ti c ev en t c o n trib uti n g to th e m o le cu la r pa th o ge ne s is o f or al c an c er. O f cru c ia l im po rta n ce is th e fa c t th at ab n o rma l itie s of p 53 h a ve be en i de nti fi ed in o ra l c an c er, as so c ia te d w i th sm o ki n g a n d dri n kin g , an d i n so m e dy sp la si c le u ko pl ak ia s. Th e r ol es o f

on c o ge n es , TS Gs , an d DNA r ep ai r m ec h an i sm s a re c u rre n tl y very a cti ve a rea s o f res e arc h i n or al c an c er, a s th ey a re co n s id ere d to be th e b a si c me ch a n is ms i n th e a eti o lo g y o f the

dis e as e as w e ll as h a vi n g e n o rmo u s p ote n tia l a s ea rl y de tec tio n m ar ker s an d fo r us e as ta rg ets fo r the ra py .

Table 10.6 Summary of methods for the prevention of oral carcinoma* Pri ma ry pre ve n ti o n

Ad vi ce o n s top pi n g (o r red u ci n g) sm o ki ng a n d ch e wi ng are ca ( be te l) n u t Ad vi se m od er ati on o f a lc o h o l i n tak e

Dieta ry ad vi ce (e sp ec ia l ly en c ou ra g in g in ta ke o f f res h fr ui t a n d v eg eta bl es ) Se co n d ary p reve n tio n (po te n tia lly m al ig na n t l es io n s)

Al w ays s c ree n e nti re o ral mu c o s a

Rec o gn i ze a bn o rm al iti es (e .g . c h an g e o f c ol o u r)

If ca n di da l in fe c ti o n pr es en t, id en ti fy ca u se a n d tr ea t

Refe r a n y su s p ic io u s le si o n to s pe ci al is t, es pe c ia lly i f th er e i s n o im pro v em en t wi th in 2 w e eks o f re mo vi n g p o s si bl e c a u sa tiv e fa ctor Terti ary p reve n tio n (p rev en tio n o f rec u rre n ce )

Im p or ta n c e o f ro u tin e ex trao ra l an d in tra o ra l ex am in a tio n Reg u la r re vie w

Low th re s ho l d fo r re -refe rra l

Dieta ry ad vi ce (f res h fru i t, ve ge ta b le s)

Ch em o pre ve nti o n ma y pl ay a ro le i n the fu tu re *R ep ro du c ed (w ith sl ig h t m od if ic ati o ns ) fro m Ta bl e 1 in O gd en , G .R . an d

Ma c lu s key , M. (2000). A n o ve rvi ew o f th e p rev en tio n o f o ra l c a n ce r a n d d ia g no s tic ma rke rs o f m a lig n an t ch a n ge . De n tal U pd ate 27 , 95β€“ 9 by pe rm is si o n o f G eo rg e

Warm a n P u bli c ati on s (U K) Ltd.

Ora l ca rc in o m a is a ge n eti c dis e as e. On e em erg in g c o n ce pt, h o w eve r, i s tha t a n u m be r o f a c tiva tin g a n d i n ac tiv ati n g ge n etic

eve n ts mu s t o cc u r f o r th e in iti ati o n an d p ro gre ss ion o f o ra l c a rc in o ma an d th a t th es e g en e tic ch a n ge s m o s t pr ob a bly o c cu r as a m u ltis te p p ro c es s (Fi g. 10. 7).

Gen e ti c ch a n ge s in vo l ve d i n th e i n iti ati on an d pr ogre ss io n o f c an c er o cc u r i n a m ul tis tep pro c es s.

The potential role of viruses in oral carcinogenesis

Th ere is c o n si de ra bl e in ter es t in th e p o ss ib le in terac tio n s i n ca rc in o g en es is b etw e en H PV s , si mi an vi ru s (SV 40), a n d la rge T an ti ge n. Th e r ea son f o r th is re n ew ed i n te re st i s ba s ed o n th e e xpe rim en ta l evi de n ce s u rro u n din g th e p53 T SG 's in vo l ve me nt in c a nc e r a n d ev id en c e th a t i ts a b erra n t e xpr es si o n ma y be a lter ed by p53 ge n e m u tati o ns a s a co n s eq u en c e of

ca rc in o ge n s, r ad ia tio n , o r o th er mu ta ge n s, o r by o th er me c ha n is m s, s u c h as i n ac tiv ati o n o f th e p 53 pro te in b y v ira l o r o th er pro te in s. S ign i fica n t in th i s res pe c t ma y be vi ra l p ro tei ns

su c h a s SV 40, la rg e T a n tige n , HP V typ e 16 E 6 pro tein , a s w ell a s th e c e llu l ar pro te in s s uc h as M DM -2 (m ur in e do u b le mi n u te -2) . Th e pre se n ce o f h igh -ris k H PV ty pe s in s qu a m ou s c el l

ca rc in o ma o f th e h ea d an d n ec k ma y res u lt i n in a c tiva tio n o f p53 TS G fu n c tio n b y n o ng en e tic me ch a n is ms . HP V h a s be en f o un d in p53 m u tate d o ral ca rc in o m as .

Fig. 1 0 .7 P ro gre ss io n fro m le u ko pl ak ia to c a us e s o f th e or al c av ity. R ep ro du c ed fro m

Lipp m an ( 2001) . M ol ec u la r m a rke rs o f th e ris k o f o ra l ca n ce r. New E n gla n d Jo u rn al o f

Me di ci n e, 34 4 , p. 1323. Co py rig h t Β© 2001 Ma s sa c h us e tts M e di ca l So c ie ty. A ll ri gh ts res erv ed .

Molecular techniques to identify genetic changes/alterations in the oral mucosa Gen e ti c al tera tio n s c an be s tu d ie d in c el lu la r DNA , RNA , a n d p ro tei n fro m tu m ou r tis su e sp ec im en s ; b io p si es ; ti ss u e sc ra pe s; an d a ls o i n plas m a o r s eru m fro m p ati en ts w ith pre ma li gn a n t an d m al ig n an t di se a se .

Mo l ec u la r tec h n iq u es c u rren tl y a va il ab le fo r ge ne tic an a ly si s in c lu d e: a t th e DNA l eve l, a ll el ic im ba la n ce (l o ss o f h eter oz y go s ity an a ly si s) , DNA s eq u en c in g, s in g le -stra n de d co n fo rm a ti o n al an a lys i s, m eth yl a ti o n pro f ili n g, a n d mo l ec u la rβ€“ cyto g en e ti c a pp ro ac h es ; a t th e RNA l eve l,

No rthe rn bl o tti n g an d rev ers e tra ns c rip tas e po l ym era se c h ai n re ac tio n ( RT- PC R); a n d, a t th e pro tei n le ve l, We s te rn b lo ttin g , im mu n o h is to ch e mi cal an a ly si s, in s itu h ybr id iz ati o n , an d

pep tid e se qu e nc in g . Th e pu b li ca tio n o f th e H um a n Geno m e Se qu e n ce n o w m ak es la rg e-sc al e

ap pro a ch e s to ca n c er ge ne tic s in p a tien t g ro u ps a p pli c ab le , by u si n g e xp res s io n c h ip a na ly si s wh i ch c o n tai n s mo s t o f al l o f th e k n o wn hu m a n ge n es, as w e ll a s a p pro a ch e s

P. 121

su c h a s CGH c hi p an d m eth yl ati o n ch i p a n a lys is . F u rth erm o re, th e id en ti fic a tio n o f mi ll io n s o f si ng le n u c leo ti de po l ym o rph is m s (S NP s) in th e h u ma n g en o me h a s ma de ge n o ty pi n g a n a lys is a r ea li ty . Deta il s o f th e se tec h n iq u es a re cl ea rly o u tsi de th e s c o pe o f th i s bo o k b u t it is

im po rta nt to ga in an u n d ers tan d in g o f th e sc i en tif ic ba s is o f the s e te sts , th ei r l im ita tio n s,

an d ap pl ic a tio n s.

Tumour markers

In fu tu re, m o le cu l ar ge ne tic m a rker s of p rem al ig an cy an d m al ig n an c y w i ll be u s ed a lo n gs id e co n ve n ti o n al h is to pa th o lo gi c al tec h n iq ue s to a id in th e di a gn o si s o f o ra l ca n c er as w el l as bei n g us e d i n th e ma n ag em en t o f th e se p ati en ts to opti mi ze tre atm en t. M a ny o f the s e ma rke rs ar e o n l y u s ed a s res e arc h to o ls a t th e pre se n t tim e bu t a re c ur ren tly b ein g dev el o pe d to b ec o m e a pa rt o f ro u tin e di ag n o sti c tes ti n g.

Molecular markers for malignant transformation of oral leukoplakia

Th ere a re cu rre n tly n o rel ia bl e cl in ic a l o r h is to logi ca l fe atu re s o f o ra l leu k o pla ki a th at c a n be us e d to p re dic t w h eth e r a le si o n w il l re gre s s o r prog re ss to c a rci n o ma . Th e de ve lo pm e nt of mo le c ul ar ma rk ers th a t w ill d ete rmi n e th e ris k o f ma lig n an t tra n s fo rm ati on is th ere fo re o f

fu n da me n tal im po rta n c e. F u rth erm o re, m o le cu l ar ma rkers m ay m ak e i t p os s ib le to mo n i tor ca n ce r re c ur ren c e a s w el l as p ro vid in g th e key fo r the d ev elo p me n t o f ef fec tiv e ch e mo pr eve n tive a n d ch e mo th era p eu tic a ge n ts.

Th ere h av e b ee n re ce n t im po rta n t a dv an c es i n th e m ole cu l ar as s es s me n t o f th e ris k o f

ma li gn a nt tran s fo rm a ti o n in pa tien ts w ith or al le u ko pl ak ia . Th e se s tud ie s h av e in vo l ve d

as se s sm en t o f po te n tia lly p red ic tive m ar ker s s u c h as th e e sta bl is h me n t o f p lo i dy sta tu s,

dete c ti o n o f a l lel ei c im ba la n ce (o r lo s s o f h e te ro zyg o si ty), mu ta tio n a l p ro fi lin g o f TS Gs su c h as p 53, o r de term i ni n g a b erra n t e xpr es si o n o f a ra ng e o f g en e s in vo lv ed in ca rc in o ge n es is

Ho w eve r, it is n o w c le ar tha t a si n gl e g en e tic m arker o r c la ss o f m ark ers c a nn o t b e u se d to

pre di ct th e o u tco m e o f e ve ry c a se o f o ral l eu ko p la kia . Co m pl ex m od el s to pr ed ic t th e r is k o f ca n ce r in p a ti en ts w ith o ra l le u ko pl ak ia a re the re fo re be in g de vel o pe d.

Mo l ec u la r a ss a ys m ay , in f utu re , de fi ne p ati en ts w ho a re a t a l o w ris k o f o ra l ca n c er

dev el o pm en t a n d fo r w h o m n on -in va si ve re vie w o f th eir or al le u ko pl ak ia s an d m o dif ic a ti o n o f lif es tyle (f o r e xam p le, to b a cc o c es s ati on ) ma y be re as o n ab le a n d b a se d on sc ie n tifi c

evi de n ce . Fo r th os e pa tie n ts id en tifi ed a s β€ hi gh r is k β€™, re s ec tio n o f the ir le si o n s w i th

veri fi ab le c le ar res ec tio n ma rg in s m ay be th e mo s t a pp ro pri ate m an a ge me n t w ith o r w ith o ut

ch e mo pr eve n tio n . A w id e r an g e o f n ew c h em o pre ve n ti on a ge nts a re c ur ren tly b ein g a ss e ss ed

an d mo l ec u la r b io m ark ers w i ll be u s ed a s i n term ed iate en d- po in ts to a ss e ss th e ir va li di ty . We are e nte rin g a n ew e ra o f mo le c ul a r di a gn o si s a nd ta rge ted trea tme n t m o da li ti es th a t w il l b e ba se d on the f in di n gs fr om th e po s t-gen o m ic rev o lu tio n .

Ea rl y d ete cti on an d tar ge t dru g th era p y i n o ra l c a rci n o ma w il l be ba s ed o n fu tu re ge n etic pro fi li ng .


Wh at q u es tio n s w o ul d yo u a sk th is p ati en t?

Th e p a ti en t sh o u l d b e as ke d if he h a s n oti ce d th is u lc er an d , if s o , fo r h o w l on g a n d w he th er

it h a s be en s o re. It is a ls o im p or ta n t to f in d o ut if th ere h as b ee n a n y so r en es s a nd i f h e h as a h a bi t o f bi ti n g hi s to ng u e o r c atc h in g it w i th the de n tur e. He s h o ul d be qu e sti o n ed a s to wh e the r h e h a s s u ff ere d fro m mo u th u l ce rs in th e pas t. Yo u s h ou l d rec h ec k h i s me di ca l

hi sto ry , w ith p arti cu l ar em ph a s is o n sk in , e ye, geni tal , o r g u t pro b le ms a n d as c erta in i f h e ha s ge n era l ly fel t w el l o r s u ffe red a n y w e ig ht lo s s. T he p ati en t's d ru g h i sto ry s h ou l d be

revi ew e d to ch e ck fo r an y pr es cr ibe d o r o ve r th e cou n ter dru g s. He sh o u ld a ls o b e a s ke d ab o ut hi s in ta ke o f a lc o h o l.

Q2

Wh at f ea tu res o f th e u l ce r a re im po rta n t to n o te ?

Th e f o llo w i ng s ig n ifi c an t f ea tur es o f th e u lc er sh ou ld b e no te d b y vis u a l i n sp ec tio n a n d

pa lp ati on : in d ur ati on ; pro l ife ra tive g ro w th ; ch a n ges in s u rfa c e te xtu re an d c o lo u r; fix ati on to th e af fe cte d ti ss u e o r u n de rly in g stru c tu res . Q3

Wh at i s th e m o s t li kel y d if fe ren tia l di ag n o si s in th is pa rtic u la r c a se ?

Th is p atie n t a pp ea rs to h av e p re se n te d w ith a n a sy mp tom a tic u lc er bu t m a y, w h en

sp ec if ic al ly as ke d, rep o rt s o ren e ss o r di sc o mf o rt on e atin g . Th e bro k en d en tu re co u l d in di ca te the u lc er is tra u ma tic a lly in d u ce d, p a rticu la rly if it ha s n o c li ni c al fe atu re s

su g ges tiv e of m al ig n an c y. A n i so l ate d a p hth o u s u lc er is ra re ly sy mp tom -free a n d th e p a ti en t is li ke ly to h av e g iv en a h is to ry o f RA S i n th e p a st. Dru g s, su c h a s n ic o ran d il o r NS A IDS c an pre di sp o se to o ra l ul c era tio n b u t le si o ns a re u s ua ll y pa in f ul a n d th e p ati en t w o u ld rep o rt a

po s itiv e d ru g h is tor y. In th e ca s e o f th is pa tie n t w h o h as o n e (o r p o ss i bl y m o re) si gn i fic a n t ris k f ac to rs (th at is , h e i s a h ea vy sm o ke r) a n d pre se n ts wi th a n is o la ted , as ym p to m a tic ul ce r o n th e la tera l bo rd er o f th e to ng u e (ris k si te), th e li ke lih o o d o f a s qu a m ou s c el l ca rc in o ma i s h ig h. Q4

Ho w w o ul d yo u ma n a ge th is p ati en t?

If yo u s u s pe ct th a t th e l es io n i s a trau m a tic u lc er an d th ere i s an ob vi o us s o u rc e o f tra um a , th i s sh o u l d b e el im in ate d by ad ju s ti n g th e d en tu re. T he p ati en t m u st, h o w ev er, b e rev ie we d in 10β€“ 14 da ys , to c h ec k i f th e re are a n y s ig n s o f h e al in g.

Th is g en tle ma n s h ou l d be to ld a t th is s tag e w h y it i s im po rta n t fo r h im to be f ol lo w ed u p . If, ho w e ver, th e u lc e r h as a n y su s pi ci o u s fea tu res , h e sh o u ld be re fer red to th e n ea res t sp ec ia li st ce n tre ( tha t i s, o ra l me di ci n e or o ra l an d ma xi ll o fa ci al s u rge ry) fo r f ur th e r in ve sti ga tio n s, in c lu di n g b io p sy . B io ps y o f an o ral le s io n th a t yo u s u sp ec t m ay b e a

ca rc in o ma , s h o ul d be u n de rtak en b y th e sp ec i al is t wh o w ill d ec id e on de fin i tive tre atm en t

an d n o t th e g en e ral d en ta l pra c titio n e r. A re ferr al le tter, d es c rib in g th e p a ti en t's h i sto ry an d

fu ll de sc ri ptio n of th e u lc er, s h o u ld be fa xe d (as w e ll a s po s te d) to th e sp ec ia li st ce n tre, w i th a f o ll ow -up te lep h o n e c a ll to c he c k th a t th is h a s be en re ce iv ed . Th e pa tie n t sh o u ld b e ad vis e d o f th e i mp o rta nc e o f a tten d in g h is s pe ci al is t ap po i n tme nt. P a tie n ts

ma y a s k i f th e ir mo u th u lc er co u l d b e a β€ ca n ce r β€™ or β€ ma li gn a nt β €™ or a

P. 122

β€ tu m o u rβ €™, a n d de nti sts m a y fi nd th is q u es tio n a w kwa rd to an s w er. In th e m ajo ri ty o f

ca se s it is be tter to in fo rm th e pa tie n t th at th i s is a p o ss ib il ity an d th at it m u st be ru le d ou t by fu rth er in ve s ti ga tio n s . It m ay be h e lpf u l, a t this s tag e, to po in t o u t th a t th ere a re a

nu m be r o f di ffe ren t c a u se s o f m o u th u lc ers . If the p ati en t's u lc er is f ou n d to b e c a rci n o ma , th e d en tis t i s lik el y to h a ve a ro le to p la y i n po sttrea tme n t re ha b ili tati o n .

Projects 1. 2.

Rea d the cl in i ca l gu id el in e s, Th e o ra l ma n a ge me nt o f o n c o lo gy pa tie n ts, pu bl is h ed b y

th e F ac u lty o f De n tal S u rge ry, R oy al Co l le ge o f S urge on s o f E n gla n d.

Wh at i s me a nt by th e te rm β€ he al th -rel ate d q u al ity o f l ife β €™? Ho w h a s th is b een

me as u red i n pa tie n ts w ho h av e u n d erg o ne s u rge ry fo r th e trea tme n t o f or al ca rc in o m a?

Au th o rs : Title:



Co py rig ht Β © 2003 Ox fo rd U ni ve rsi ty P re ss > T ab le o f Co nt e nt s > 1 1 - Mu c oc ut ane o us d is e as e and c o nne c t iv e t is su e d iso r d e r s

11

Mucocutaneous disease and connective tissue disorders Problem cases Case 11.1

On e o f y o ur ed en tu lo u s p ati en ts, a 62- yea r-ol d la dy, tele ph o n es th e su rg er y re qu e sti n g a n urg en t ap po i ntm en t b ec a u se s h e i s af ra id sh e m ay h av e mo u th c a nc e r. Wh il st cl ea n in g h er den tu re s the n i gh t b efo re , s he h a d no ti ce d so m e w hite pa tch e s in s ide h e r m o uth a n d

rem em be rs rea d in g in a m a ga z in e th a t th es e ca n b e a s ig n o f mo u th c an c er. Th is la d y h a d

bee n u n ab le to s le ep d u e to w o rry. S h e is s ee n la ter tha t d a y a n d o n exa m in a ti o n yo u n o te a

ne tw or k o f w hi te β€ la ce - lik eβ €™ les i on s o n b o th bu c c al m uc o s ae , w hi c h ar e p ai n le ss . Th e re

are n o o the r o ra l le si o n s. O n fu rth e r q ue s tio n in g th e p ati en t r epo r ts a n i tch y ra sh o n h er wri s ts . Q1

Wh at i s th e m o s t li kel y dia g no s is o f th is l ad y's o ra l les i on s ?

Q2

Are h e r s kin sy mp tom s rel ate d?

Q3

Wh at in ve s ti ga tio n s w o u ld yo u c a rry ou t?

Q4

Wh at a d vic e an d tre atm en t w o u ld yo u giv e to thi s la dy ?

Case 11.2

A 41-yea r-ol d Jew i sh m a n pre s en ts w ith a 9 -mo n th h is to ry o f m ou th u lc era tio n , w h ic h h a s

bec o m e p er si ste n t an d c o ns i de rab ly w o rse . It i s a l so ca u s in g gre a t di sc o mf o rt o n e ati ng . He

ha s n o tic ed th at la rge b li ste rs o cc u r in s id e h i s lips b ut the s e ra p id ly bu rs t. Ov er the l as t fe w da ys h e ha s d eve lo p ed sk in l es io n s a n d h e s h o ws y o u so m e ero d ed s kin les i on s o n h i s ba ck .

Th ere a re no o cu l ar o r g en ita l sy mp to ms . E xa mi n ati on o f th e mo u th re ve al s wi de s pre ad o ra l ul ce ra tio n a n d th e Ni ko ls ky s ign is p os iti ve . Q1

Ho w w o ul d yo u in v es tig ate a nd m a na g e th i s ge n tlema n ?

Case 11.3

A 2-yea r-ol d bo y is re fer red to yo u b y a de rma to lo gis t fo r den ta l ca re . Hi s pa ren ts tel l yo u th a t h e w as d ia gn o s ed a t bi rth a s h avi n g ep ide rm o lys is bu l lo s a (E B) . Q1

Wh at a re th e o ro de n ta l ma n if es tati o ns o f th is c o ndi ti o n ? D is cu s s th e d if fic u lti es yo u mi gh t e nc o u n ter wh e n pr ov id in g de n ta l trea tm en t fo r th is bo y .

Mucocutaneous disease

It wa s p oi n ted o u t in Ch ap ter 1 th at th e o ra l m u c ous m em br an e , al th ou g h s im ila r to o the r lin i ng m u co s a e i n str uc tu re an d g en er al be h av io u r, a ls o re se mb le s th e s ki n in s o m e w a ys .

Th is is in k ee pi ng w i th th e tra n si tio n a l p o si tio n an d f u nc tio n of th e o ral m u co u s me mb ra n e,

lyi n g a s it do es i n a n in te rme di ate p os iti o n be tw een the s ki n an d th e gu t m u c os a p ro pe r. On e res u lt o f th is s itu ati o n is th a t ge n era li z ed di s ea se s, bo th o f mu c o s ae a nd o f sk in , m ay a ffe ct

th e m o uth . In s u c h sk in d is ea s es , h o we ve r, th e o ra l le si o n s, b ec a us e o f th e m o dif yin g e ffe ct of th e en vi ro n me nt, ma y be ar li ttl e su p erf ic ia l r esem bl an c e to th os e o f th e s kin . T he

co n tin u a l pre s en c e o f s al iva , s ec o n da ry in fe cti o n by o ra l o rg an i sm s , an d th e rep ea ted

tr au m as o f th e o ra l en vi ro n me n t pl ay th ei r re s pec tiv e pa rts in th e m od if ic ati o n o f th e o ra l les io n s . P ar ti c ul ar ly go o d ex am pl es o f thi s fo rm o f mo d ifi ca tio n are sh o w n i n th e g ro u p of dis e as es (i m mu n o bu l lo u s) in w h i ch b li ste rs o r b u llae a re fo rm ed . Wh en th i s o cc u rs in th e mo u th th e b u ll ae ra pi dl y b rea k do w n to f or m u lc era ted a rea s . S u ch b eh a vi ou r af fe cts th e ma n ag em en t o f th es e co n d itio n s . It is o fte n n ec es s ar y to tre at the s ec o n da ry in fe cti o n

pro d uc e d i n th is w a y b efo r e pro c ee di n g to mo re s ys tem ic tre atm en t. A d iff ere n t fo rm o f

mo di fi ca tio n i n o ra l le si o n s oc c u rs in s o m e d is ea s es (f or ex am pl e, l ic h en p la n u s) in wh i ch th e in iti al o ra l l es io n m a y b ea r l ittle m o rph o l og ic a l res em bl an c e to th e s ki n le si o n , eve n th o u gh th e b as ic h i sto p ath o lo g ic al c h an g es a re si mi la r. The se va ria n ts pre s um a bl y d ep en d o n th e dif fe rin g stru c tu res o f th e s ki n a n d th e o ra l mu c o sa r ath e r th an on the e ffe ct o f th e en vi ro nm e nt.

It is n o t ea s y to u n d ers tan d w h y s o m e s ki n di s ea se s co m m on l y p ro du c e o ral l es io n s w hi le oth e rs do n o t. F o r e xa mp le , ps o ria s is , o n e o f th e tw o m o s t co m m on sk in d is ea s es se en in

Eu ro p ea n c li ni cs ( the o th er is e cz e ma ), p ro du c es o ra l les i on s v ery ra rel y. In fa c t, th ere is

so m e d if fer en c e o f o pi n io n a s to w he th er a ch a rac teris tic o ra l l es io n do es e xi st. O n th e o th e r ha n d, l ic h en p la n u s pro d u ce s o ral l es io n s in a hi gh p ro po r ti o n o f th e pa tie n ts w ith s kin in vo lv em en t.

Ora l le si o ns h a ve o c ca s io n al ly be en d es c rib ed i n a ve ry w i de ra n ge o f s ki n di se a se s, eve n

th o u g h su c h b eh a vio u r m a y n o t b e ch a ra cte ris tic o f th e c o n di tio n . Th u s , in th e di ag n o si s o f

or al le si o n s o f do u b tf u l a e ti o lo g y, a h is to ry o f s kin dis e as es i s al w ay s o f i nte res t. P erh a ps th e mo s t im po rta n t c o n si de rati o n in th i s co n tex t is th e fa ct tha t, i n so m e di se a se s o f th e sk in (in c lu di n g s o m e

P. 126

of th e mo s t s eri o us s u c h as pe mp h ig u s), th e o ra l l es io n s m ay a pp ea r b ef or e th o s e o f th e sk in

an d ma y thu s pro v id e a n o p po rtu n ity fo r e ar ly di ag nos i s an d th e r ap id in i tia tio n o f trea tme n t. Defi n itiv e d ia g no s is o f th e o ra l le si o n s in s ki n dise a se s de pe nd s la rg el y o n b io p sy

exa m in ati o n a nd th is s h o u ld be c a rrie d o u t o n an y do u b tfu l les i on . T hi s is n o t, h o w eve r,

al wa ys a n e as y ma tter, p a rtic u la rly in th e c as e o f b u llo u s l es io n s in wh i ch th e m an i pu la tio n s of in c is io n a l o r ex ci si o n al bi o ps y ma y c a u se vi rtu al d is in teg ra tio n o f th e l es io n . If s u c h a

les io n ca n n ot be re mo ve d in ta ct f o r e xa mi na tio n , then the e dg e o f th e les i on ma y o ften sh o w th e c h ara c teri sti c ch a n ge s be tte r th a n a c en tra l a re a wh e re se co n d ary ch a n ge s ma y o bs cu re th e p ic tur e. A b io ps y tak en f ro m an u l ce ra ted ar ea m ay s ho w n o th i n g b u t n o n -sp ec if ic in fl am ma to ry ch a n ge s. Im m un o fl u o res c en t tec h n iq u es a n d, in p a rtic u la r, di rec t

im mu n o flu o re s ce n t stu d ie s of b io ps y ma teri a l h a ve rev o lu tio n iz e d th e m eth o ds o f di ag n o si s of im m u no b u llo u s s ki n di se a se s an d th ei r o ral l es ion s . Fo r th is tec h n iq ue a p eri le si o na l bio p sy i s ta ke n a n d f res h (u n fix ed ) ti ss u e se n t to th e p a th o l o gy la bo ra to ry.

In th i s ch a pte r th e c on d iti on s c h o se n fo r di sc u ss i on are th o s e th a t a re rel ati vel y co m m on l y se en i n th e o ra l me di c in e cl in ic o r tho s e o f p ar ticul a r in te res t o r im po rta n ce i n th e d en ta l co n tex t. Oth e r c o nd iti o ns th a t li n k o ra l me di ci n e an d de rm ato lo g y i n cl u de th e

gen a to de rma to s es (Ch ap ter 9), P eu tz β €“Jeg h er's s yn dro me ( Ch ap ter 9), an d We ge n er's gra n u lo ma to s is (Ch ap ter 1).

Lichen planus and lichenoid reactions Skin lichen planus

In th i s di se as e th e s ki n le s io n s tak e th e fo rm o f du s ky pi n k p ap u le s tha t m a y o c cu r in a n y

si te , b ut are m o st co m mo n l y fo u n d o n th e fl exo r su rf ac es o f th e wri s ts ( Fi g 11. 1 ), o n th e

gen i tal sk in , o n th e ab do m en an d lu m ba r r egi o n s, a nd o n th e a n kl es a n d s h in s . F in e w hi te stri ati o ns o v erl ie th e p a pu le s. Th es e are th e so -call ed Wi ck ha m 's s tr ia e th a t ar e

ch a rac te ris tic o f th e c o n diti o n (Fi g. 11. 1). In ge nera l, th e s kin les i on s a re rel a ti ve ly sh o rt liv ed , the a ve ra ge du ra tio n b ei n g o f th e o rde r o f 9 m o nth s . A fter th is th ey fa de , le a vin g beh i n d a f ai n tly pi gm en te d p a tc h th a t m ay tak e a c on s ide ra bl e ti me to d is ap pe a r. In a

si gn if ic an t pro p o rtio n o f th e p ati en ts , h o we ve r, there m ay be a re cu rr en c e o f th e l es io n s . Th e ma jo rity o f s ki n le si o n s ca u se l ittle tro u bl e ap ar t fro m itc h in g , w hi c h is ve ry v ari a ble i n its in ten s ityβ €”in s o me in s ta nc e s it i s so i n si gn i fic a nt th at th e l es io n s a re n ot no ti ce d by th e

pa tien t. In a f ew c a se s the KΓ ¶b n er ph e n om en o n m a y b e se en w i th le si o ns d is trib u ted in a lin e ar pa ttern al on g a sc ra tc h ma rk o n th e s ki n . Sca lp le s io n s ma y al so oc c u r i n a few

pa tien ts (u s ua ll y fem a le) . Of ten th es e a re n o t p a pula r, b u t ar e r epr es en ted b y p a tc h es o f

al op ec i a. T he re ar e w i de sp rea d va ria tio n s in the c li n ic al p ic tur e, s h ow n b y o cc a si o n al pa tie n ts wh o m a y p res e nt wi th bu l lo u s , h ype rtro ph i c, o r an n ula r le si o n s, a n d, v ery ra rel y, th e di se as e ma y f irs t o c cu r in a n a cu te fo rm w i th in i ti a l s ym pto m s mu c h m o re s e ver e th a n th o se

des c rib ed a bo ve . Th e h is to lo gy o f a sk in l es io n i s qu i te c h a rac teri s ti c a nd , in al l e s se n ti a ls ,

res em bl es th a t o f th e o ra l le si o n s. T he m o st ob vi o us f ea tu re is ba s al c el l l iq u efa c tio n a n d th e pre se n ce o f a na rro w d en s e ba n d o f in f la mm a to ry c ells , pre do m in a n tly ly mp h oc yte s, wi th in

th e d erm is a n d l yi n g ju s t b el ow th e e pith e li um . In th e sk in th e e ffe c t ap pe a rs to be a p us h in g up o f the e pi the li u m to fo rm th e p a pu le s, al th o ug h th is m a y n o t, in f ac t, b e th e a ctu a l

me ch a n is m o f p ap u le fo rm ati o n . Th e o ver lyi n g e pi th el iu m ma y un d erg o a va ri ety o f c h an g es , hy pe ro rth ok era to s is be in g th e m o s t co m m on fin d in g in th e c as e o f th e sk in l es io n s .

Fig. 1 1 .1 Typ ic a l s ki n le si o n s of li c he n p la n us .

In th e K Γ ¶b n er ph e no m en o n i n di vid u al s kin le si on s dev el op o n a s ite o f tr au m a o r a n

op era tio n sc a r. Th is i s se en i n a n u mb er o f d erm a tol o gi ca l co n d itio n s i nc lu d in g li ch e n pl an u s an d ps o ria s is .

In v ol ve me n t o f th e n ai ls i s se en i n a bo u t 10 p er cen t o f p a tien ts a n d th i s ma n if es ts as f in e

rid gin g o r g ro o vi ng , s ev ere dy stro p h y, a n d, i n a few ca s es , de s tr uc tio n of th e n ai l b ed . Th e cl as s ic n ai l ch a n ge o f lic h en pla n u s is th e pte ryg iu m, in w h ic h ad h es io n b etw e en th e do rs a l na il fo l d an d th e n a il b ed le ad s to th e pa rtia l de stru c tio n o f th e n a il .

Th e f le xo r s u rfa c es o f wri s ts , a n kle s , an d s h in s and l um b ar reg io n s a re th e u s u al s ite s of in vo lv em en t i n s kin lic h en pla n u s .

Th e g en i tal m uc o s a al so ca n b e i n vo lv ed in l ic h en pla n u s bu t m a y b e as ym p to m a tic . Th e

vu lvo v ag in a l g in g iva l (V V G) s yn d ro me is a r are va rian t o f li ch e n pl an u s , c ha ra c te riz e d by

ero s io n s o f v u lva l, v ag in a l, a n d gi ng iv al mu c o s ae with a p red il ec tio n f or s ca rri ng a n d stri ctu re fo rma tio n . T hi s ca u s es co n s id era b le pro b le ms to th e pa tie n t a nd i s di ffi cu l t to m an a ge .

Th e v u lvo v ag in a l g in g iva l sy n dro m e is a ra re va ria nt o f l ic h en p la n u s, w hi c h is d iff ic u lt to ma n ag e.

Aetiology of lichen planus

Lic he n p la n us i s an im mu n o lo g ic a lly m ed ia ted di s ea se b ut in th e ma jo ri ty o f ca s es d oe s n o t

ap pe ar to be c o n si ste n tl y p a rt o f an a u to im mu n e di stu rb an c e. A nu m be r o f fa cto rs h a ve b een im pli c ate d i n th e ae tio lo g y o f li ch e n pl an u s a n d a sso c ia tio n s , o fte n u n su b sta n tia ted , ma de

wi th sy ste mi c di se a se s su c h a s di ab ete s me ll itu s and li ve r di se a se . Les io n s th at cl in ic a ll y a n d hi sto l o gic a ll y

res em bl e th o s e o f o ra l lic h en pl an u s (l ic h en o id re ac tio n ) ca n b e p rec i pita ted b y a n e ver -

P. 127

in cr ea si n g r an g e o f dru g s. Lic he n o id re ac tio n s w ill b e d is c u ss ed i n th e n e xt se c tio n o f th i s ch a pte r.

Oral lichen planus PREVALENCE

Ora l li ch e n pl an u s a ffe cts f ro m 0.1 to 4 pe r c en t of in d ivi du a ls d ep en d in g o n th e p o pu la tio n stu di ed a n d i s ge n era ll y a di se a se o f th e m id dle - aged a nd e ld erl y, w ith a f em al e

pre po n de ra nc e o f 2: 1. Th e ag e ra ng e is s im il ar to th at i n p ati en ts rep o rtin g w ith s ki n le si o n s

on l y, al th o ug h , in th e c as e o f p ati en ts w ith o ra l le si o ns , th er e i s a ra th e r h i gh er pro p o rtio n o f pa tien ts i n th e 60 yea r plu s g ro u p. Th e a u tho rs h a ve s ee n a sm a ll n u mb er of p ati en ts w ith co n fi rme d o ral l es io n s at a ver y e arl y ag e, th e yo un ge st be in g 7 ye a rs o ld . Th is w o u ld be co n s id ere d a g rea t ra ri ty i n th e c a se o f sk in le si on s.

Ora l li ch e n pl an u s a ffe cts 0. 1 to 4 pe r c en t o f in divi du a ls , de pe n di n g o n th e p o pu l ati on stu di ed .

Ora l le si o ns o c c ur in a co n s id era b le pro p o rtio n o f pa tie n ts w i th li ch e n pl an u s a n d a re s ee n in th e o ra l me di ci n e cl in ic f ar mo re fr eq ue n tly tha n th e o ra l l es io n s o f oth e r s ki n di se a se s. T h e

tr ue i nc i de nc e o f o ra l le si o n s i s a li ttle di ffi cu lt to de term in e si n ce th e fi gu res a va i la ble v ary

wi de ly, a si gn if ic an t fac to r b ei n g w h e the r th e pa tie n ts are fi rs t s een in a d erm a to l o gi ca l cl in ic (in w h ic h c a se o ra l l es io n s ar e p res e nt in a bo u t 70 pe r c en t o f c as es ) o r in a d en ta l c l in ic . In th e se la tter pa tie nts , p res en ti ng b ec au s e o f o ra l le si o ns , th e in c id en c e o f s kin le si o n s is o f th e o rd er o f 40 p er ce n t. Th is v ari ati o n is p ro ba bly d u e to a n u mb er of fa c to rs, th e m os t im po rta nt be in g th e a s ym pto ma tic n a tu re o f th e o ral le si o n s in m an y c as es . A s ec o n d

im po rta nt fa cto r is th e in co n s tan t se qu e nti al rel ati o n sh i p o f th e l es io n s β€” th e o ra l l es io n s

ma y o c c ur be fo re , af ter, o r at th e s a me tim e as th e s kin les i on s . In g en er al , h o we ve r, o ral

les io n s l as t m u ch lo n ge r th a n s kin les i on s . A m ea n d ur ati on o f 4. 5 ye a rs h as b ee n s ug ge ste d bu t th ere is n o d o u bt th a t in ma n y c a se s th is p eri od m a y b e g re atl y ex ce ed ed .

CLINICAL PRESENTATION

Th e o ra l le si o n s are u s ua l ly bi la tera l an d in vo l ve th e b u cc a l m u c o sa i n ab o u t 90 p er ce n t o f al l c a se s. In de sc en d in g o rde r o f f req u en c y th e to ng u e, g in g iva e , al veo l ar rid ge , li ps , a n d

les s c o mm o n ly the pa la te ma y al so be a ffe cte d. In or al li c he n p la nu s , th e bu c ca l mu c o sa a n d ton g u e are th e mo s t c o mm o n si tes o f in vo lv em en t a n d le si o ns te n d to be bi la tera l.

A ran g e of c li ni ca l pr es en ta ti o n s ca n o c cu r in o ra l li ch e n pl an u s a nd ma y co e xis t i n th e sa me

pa tien t. T he re is , h o w eve r, s o me c o nf u si o n in th e li tera tu re co n c ern in g th e term in o lo g y u s ed to de sc rib e th e c li n ic al va ri an ts o f o ral l ic h en p la n u s (Tab le 11. 1 ).

Reti cu la r, p la qu e -lik e, a n d pa pu la r va ria n ts te n d to be a sy mp to ma tic . In th es e var ia n ts th e

epi th el ia l c h a ng e is o f hy pe rpa ra ke rato s is o r, o c c as io n a ll y, h ype ro rth o ke rato s is . Th e re is n o atro p hy o f the e pi th e li um a n d, he n ce , n o u lc era tio n. Th e c h ara c teri sti c ap pe ar an c e is o f wh i te s trea ks o n th e o ra l mu c o s a, a rra n ge d i n a re tic ul a r β€ la ce -lik eβ €™ pa ttern ( Fi g.

11. 2). In s om e pa tie n ts th e le s io n s ma y be m or e c o nfl ue n t (p la qu e -lik e) an d re se mb le a

leu k op la ki a, wh e rea s in oth e rs th ere m ay be p ap u la r, li n ea r, o r an n u la r arra n g em en ts o f th e wh i te a re as . Th e se a re of ten d es c rib ed a s be in g si mila r to th e Wi ck h am 's s tria e o f th e sk in les io n s , bu t are , in fac t, m u ch m o re cl ea rl y d ef in ed . In th e c o u rs e o f th e dis e as e the

mo rp ho l o gy o f th e le si o n s ma y ch a n ge , a nd th er e m a y a ls o b e var ia tio n s in th e ir e xte n t an d in ten s ity. In ge n era l, th e se l es io n s ar e o f ten q u ite sy mp to m -free a n d are o fte n n o tic ed

in ci de n tal ly by th e p a ti en t, a lth o u gh a s e ns a tio n of β€ ro ug h n es s β €™ ma y b e pre s en t. In th e

stri ate d f o rms th e di a gn o si s ma y in iti al ly be m ad e w ith s o me co n f ide n ce o n a pp ea ra n ce

al on e , bu t i n th e ca s e o f th e c o n fl ue n t p la qu e- lik e le si o n s th e d ia gn o s is m ay be m ad e o nl y afte r b io p sy .

Table 11.1 Clinical variants of oral lichen planus Reti cu la r

Pl aq u e- lik e Pa pu l ar

Atro p h ic

Ero s iv e (u lc er ati ve) Bu l lo u s

Des qu a ma tiv e gi n gi viti s

Fig. 1 1 .2 Reti cu l ar , n o n -ero s ive l ic h en p la n u s o f b uc c al mu co s a.

In oth e r fo r ms o f o ra l li ch e n pl an u s , th e o ra l ep ith el iu m u n de rgo e s atro p h ic c h an g es a n d i s ea si ly lo s t fro m th e w ea k a n d o ed em ato u s ba s al a reas . Th is m a y re su l t in th e fo rm ati o n o f ul ce ra tive l es io n s o n th e m u c o sa , th es e o ften b ei n g a s so c ia ted w ith n ea rby a rea s in wh i ch

retic u la r o r atr op h ic l es io n s o cc u r ( Fi g. 11. 3). In a fe w pa tie n ts th e ba s al a n d su b ep ith el ia l

oe de ma m a y l ea d to se pa ra tio n o f th e e pi th eli u m wi th c on s eq u en t b u ll a fo rm ati on , b u t s uc h

bu ll ae , de ve lo pe d in i n itia ll y a tro p hi c ep ith el iu m , ar e v ery fra g ile a n d r ap id ly di si n teg rate to pro d uc e th e c h a rac ter is tic u lc era tiv e les i on s o f the c o n di tio n . In pu b li sh e d s tu di es o f o ral lic h en pla n u s th e te rms β € ul ce ra tive β €™ an d β € ero s ive β €™ ha ve b ee n u se d

syn o n ym o u sl y to de sc rib e a cl in i ca l ap pe ar an c e in wh ic h th ere h a s be en e pi th eli al l o ss . Th is var ia tio n i n de sc ri ptiv e term in o lo g y u s ed i n o ral med ic in e le ad s to c o nf u si o n an d d oe s n o t

ne c es sa ri ly refl ec t th e h is to pa tho l o gy o f th e le si on : i n ad di tio n th e res u lts o f cl in i ca l tria ls

are d iff ic u lt to e va lu a te. In th is c h a pter , the c lin ic al ly de sc ri ptiv e term β€ ero s ive β €™ wi ll

be u se d to de sc ri be th e i rre gu la r a re as o f o ral e pithe li al d es tru cti o n (s ha ll o w o r d ee p) wh i ch are c o ver ed in a y el lo w la ye r o f fib rin . A tro p hi c le si o n s, i n w h ic h th ere h a s be en e pi th e li al th i n ni n g, pre s en t a s irre gu l ar ar ea s o f e ryth em a tous m u co s a β €” bo th er os i ve an d a tro ph ic

var ia n ts te n d to be s ymp to ma tic . U n lik e th e s itu a tio n in th e re tic u la r o r pl aq u e -lik e f o rms o f th e d is ea se , th ere i s of ten c o n si de ra ble d is c om fo rt to the pa tie n t, pa rtic u la rly w h en e ati n g sp ic y o r ac id f oo d s . Th e mu c o sa i s al so su s c ep tibl e to me c ha n ic a l i rri ta tio n a n d th e i n itia l

sym p tom s m ay o cc a si o n al ly ap pe a r a s de ntu re tra u ma b ef or e a n y oth e r m o re ch a ra cte ris tic les io n s a pp ea r. P ati en ts, of ten i n o ld er ag e gro u ps, m ay p res en t w i th ex te n si ve er os i ve les io n s , co v eri n g l ar ge s ur fa ce s o f th e to ng u e an d bu c c al m uc o s a. T he s e l es io n s h a ve a gla z ed a pp ea ra n ce a n d te n d to be se pa ra ted fr om th e a dja ce n t m uc o s a by a cl ea rly

dem a rc ate d e dg e (Fi g. 11. 4). Th es e ar ea s o f e ro si o n c an tak e m o n th s to res o lv e a n d ar e

of ten re pl ac e d b y wh i te, c o n flu e nt le si o ns , pa rtic ula rl y o n th e to n gu e. N ee dle s s to sa y, th i s ty pe o f lic h en pla n u s ca u s es a gre a t de al o f di sc o mf o rt a nd p ati en ts h a ve di ffi cu l ty w h en ea tin g.

Al l th e se c li ni c al va ri an ts o f o ra l li ch e n pl an u s can af fec t th e gi n gi vae , w ith o r w ith o ut

in vo lv em en t o f o th er o ra l s ites . In a bo u t 10 pe r c en t o f c a se s gi n giv al i nv o lve m en t a lo n e

oc c u rs an d c an ma ke th e c l in ic a l d ia gn o s is mo re d iff ic u lt. In a dd iti o n, g in g iva l a rch i tec tur e

ca n c rea te pro b le ms w h en ch o o s in g a s ite to bi o ps y a n d the re s ul tin g h is to pa tho l o gy is m o re dif fic u lt to in ter pre t, pa rtic u la rly i f th er e i s a su p eri mp o se d n on -sp ec if ic gi n gi viti s. In

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pa tien ts w ith in vo lv em en t o f th is s ite th e g in g iva e o ften h ave a re d, g la z ed ap pe a ran c e

aff ec tin g th ei r fu l l w i dth (Fi g. 11. 5 ). Th is cl in i ca l ap pe ar an c e is kn o w n a s β € des q ua m ati ve

gin g iv itis β€™ wh i ch ma y b e di ffi cu l t to c li ni c al ly di ffe ren tia te fro m i nv o lve me n t w ith o th er

mu c o cu ta ne o u s di se a se s, p a rtic u la rly pe mp h ig o id. The te rm β€ des q ua m ati ve gi n gi viti s β €™

is a cl in i ca l de sc ri ptiv e te rm a nd d o es n o t th ere fore in f er an y sp ec if ic u n de rly in g pa th o lo gy . No t al l ca s es a re du e to li ch e n pl an u s .

Th e de fi ni tive d ia gn o s is o f al l ty pe s o f l ic h en p lan us i s by bi o ps y an d h is to pa tho l o gy sh o u l d

in cl u de p eri od ic a c id S ch i ff (PA S ) s ta in in g fo r Can d id a sp ec i es (s ee Ch ap ter 4). A lth o u gh th e ap pe ara n c e o f th e l es io n s m ay g ive a n in d ic ati o n o f th e di ag n o si s in s o m e c a se s, th i s is n o t

al wa ys s o a n d th e c li ni c al fe atu re s ma y so m eti me s be co n f us e d w i th tho s e o f i mm u n o bu ll o u s les io n s o r wi th pa tc he s o f e ryth ro p la kia . Th e pre sen ce o f sk in l es io n s i s a u s ef u l d ia gn o s tic po in te r bu t, e vi de ntl y, n o t a co n s tan t on e .

Fig. 1 1 .3 Reti cu l ar a nd ero s iv e les io n s o f o ral l ic h en pl an us .

Fig. 1 1 .4 Lar ge ero s iv e le si o n o f l ic h en p la n u s wi th w h ite pl aq u e at co m m is u reys

Fig. 1 1 .5 A tr o ph ic a n d ero s iv e lic h en pla n u s af fe cti ng th e gi n gi vae .

HISTOPATHOLOGY

Th e e s se n ti a l h i sto lo g ic a l c h an g e in th es e les io n s i s the s a me a s in th o se o f th e s ki n β€” th e

pre se n ce o f a su b ep ith el ia l ba n d of ly mp h o cy tes , pre do m in a n tly o f th e T typ e, an d s om e

ma cr op h ag es ( Fi g. 11. 6)β€” bu t i n the c a se o f th e o ra l mu c o s a th e re is a m uc h w i de r ra n ge o f epi th el ia l re s po n s e, w ith a c an th o s is o r atr op h y, o rth o ke rato s is , o r p a rak era to si s. Ho w eve r,

th e m o st c o m mo n f in di n g i s o f p ar ak era to si s, th e rete p eg s be in g di sto rte d to gi ve a β€ sa w -

to o th β€™ or , mo re c o mm o n ly, fla tten e d a p pe ara n ce . Aro u n d th e ba s em en t m em b ran e th ere is o ed em a, a s so c ia ted w i th de ge ne ra tive c h an g es i n the b as a l c e lls , a n a ss o c ia tio n th at i n so m e c a se s le ad s to vi rtu al s ep ara tio n of th e ep ithel iu m fro m th e co r iu m. T h e d ire c t im mu n o flu o re s ce n ce fi n din g s in l ic h en p la n u s are n ot

hi gh l y sp ec i fic . A b an d o f f ib rin i s sh o w n u p a t the b as a l zo n e , bu t n o i mm u n o glo b u li n

dep o s its . Cyto id β € ci va tte β €™ bo di es m a y b e se en , h o w eve r, b o th in th e ep ith el iu m a n d i n

th e d erm is . Th e se a re no n -dis e as e -sp ec if ic s ph er ic al str uc tu res th a t c o nta in va ria bl e

im mu n o gl o bu li n s an d c om p lem e nt co m po n e nts . A h ig h in c id en c e o f th es e is in d ic a ti ve , if n o t dia g no s tic , o f lic h en pl an u s .

Th e p a th o g en es i s of l ic he n p la n us i s n o t fu ll y u n dersto o d bu t p ro ba b ly in vo l ves a c el l -

me dia te d i mm u n e r es po n s e to an tig en i c ch a n ge s in o ra l mu c o s a. Th i s w ou l d be c on s is ten t wi th th e p red o mi n an tl y T- lym ph o c yte in fi ltra te.

Fig. 1 1 .6 S ec tio n o f o ral mu c o s a i n li c he n p la n us .

Lichenoid reactions

In a mi no ri ty o f c as es ( bu t s til l a s u bs ta nti al n u mb er) li ch e n pl an u s is p re ci pi ta ted b y d ru gs or o th er s u bs ta n ce s. A nu m be r o f dru g s h av e b ee n imp lic a ted in l ic h en o i d d ru g eru p ti o n s

(LDE s) in c lu di n g n o ns ter o ida l an ti - in fl am ma to ry ag en ts (NS A IDs) , an ti hy pe rten s ive d ru gs (es pe ci a lly a n gio te n si o n co n ve rtin g e nz y me (A CE ) i nh ib ito rs ), an ti ma la ri al s, a n d go l d

in jec tio n s (Tab le 11. 2). With d raw a l o f th e pre c ip itati n g d ru g u su a ll y re s ul ts in re s o lu tio n o f th e LDE b u t n o t al l les i on s u n ifo rm ly re gre ss .

Loc a li ze d li ch e no i d rea cti o n s h a ve a ls o be en a s so c ia ted w ith hy pe rs en s itiv ity rea cti o n s to

me rcu ri c sa l ts r ele a se d fro m am a lg am re sto ra tio n s . Th es e c ha ra c te ri sti ca ll y o c c ur w h ere th e or al m uc o s a is in co n ta ct w ith th e of fe nd in g re sto ra tio n . Re mo v al o f th e re sto ra tio n freq u en tly res u lts i n reg re ss io n o f th e l es io n an d th is w o u ld su g ge st a type IV

hy pe rse n si tiv ity r ea cti o n , si mi la r to th a t s ee n in c o n tac t d erm a titis a s a res p on s e to m eta ls su c h a s ni ck el . A fe w ca s es o f li ch e no i d rea cti o n s ha ve , h o we ve r, a lre ad y b ee n re po rted i n

res po n s e to co m po s ite re si ns . P a tien ts u n de rgo in g bo ne m ar ro w tran s pl an ta tio n c a n de ve lo p an o ra l li ch e no i d les i on as a ma n if es tati o n o f a g ra ft-vers u s - ho s t re ac tio n .

P. 129

Table 11.2 Systemic drug groups commonly implicated in oral lichenoid drug eruptions No ns ter o ida l an ti -in fl am ma to ry dru g s (NS A IDs)

An g io ten s in - co n ve rtin g en z ym e (A CE ) i n hi bi to rs Ora l h ypo g ly ca em ic s Diu reti cs

An tim a la ria ls Go ld

Pe n ic il la mi ne

Lic he n o id dr ug e ru pti on s c a n be c au s ed b y a l arg e num b er o f dr ug s , in c lu d in g NS AIDs a n d an tih y per ten s ive s.

LDE an d id io p ath ic o ra l li ch e n pl an u s a re si mi la r bo th cl in ic a lly a n d h is to lo gi ca ll y. Mu c o sa l les io n s i n o ral l ic h en p la n u s te n d to be bi la tera l, w h e rea s LDE s h av e a te n de nc y to o cc u r un i la tera ll y a n d ma y af fec t th e pa la te. His to lo g ic al ly in LDE s , ly mp h o id fo ll ic le s ma y be

pro m in en t a n d th ere ma y be a m or e m ix ed c el l p o pu latio n in cl u di n g e o si n o ph il s an d p la sm a ce lls .

Management of lichen planus and lichenoid reactions INVESTIGATIONS In ad di tio n to s tan d ar d h i sto ry tak in g an d c li n ic al ex am in a tio n , p ati en ts s ho u l d b e as ke d in

deta il a bo u t th e ir d ru g h is to ry a n d po s si bl e in vo lvem en t o f o th er s i te s , in c lu di n g th e s kin an d oth e r mu c o u s me mb ra n es . Mi c ro bi ol o gi ca l sa m pl in g o f m u co s al s ite s ma y rev ea l c a n did a l

in fe cti on , w h i ch i t m ay be a dv is ab le to tre at pri o r to b io ps y. B i o ps y s h o u ld be u n de rtak en fo r al l p ati en ts w ith s u s pe cte d o ra l li ch e n pl an u s a n d m a y n e ed to b e fro m m o re th a n o n e aff ec ted s ite . PA S s tai n in g o f h i sto l og ic a l s a mp le s is es s en tia l.

THERAPY Trea tm en t o f sk in l ic h en p la n us i s di rec ted at co n tro ll in g th e itc h an d m o der ate ly po ten t to p ic a l s tero i ds a re pro b ab ly th e mo s t e ffe cti ve a gen t fo r th is . S ed a ti n g, s ys tem ic

an tih i sta mi n es m ay b e h e lp fu l at n igh t. S h o rt c o u rse s o f s ys tem ic s ter o ids h a ve b een

su g ges te d f o r s eve re ca s es b u t lo n g -te rm s tero id s sho u l d n o t b e u se d f o r c h ro n ic c as es . Reti no i ds a n d ci c lo s po rin h av e b ee n u se d in s o me repo rte d ca s es , w ith m ixe d su c c es s.

Th era py fo r sy mp to ma tic o ra l lic h en pla n u s is a im ed a t p ro vid in g re lie f o f d is c om fo rt, h e al in g of er o si ve le si o ns , a n d in cr ea si n g epi th el ia l th i ckne s s in a re as o f atr op h y. It u s u al ly in c lu d es th e u s e o f a n an ti se pti c mo u th wa s h (to ai d w ith p laqu e co n tro l an d re du c e se co n d ary

in fe cti on ) an d a n a na l ge si c mo u th w as h (to red uc e dis co m fo rt) β€” se e Tab le 11. 3 . Loc a l

ste ro ids a re th e ma in s tay o f tr ea tme n t an d a ss is t in th e he a lin g o f ero s io n s a nd red u cti o n o f atro p hy (Tab le 11. 3 ). Hyd ro c or tis on e l oz e n ge s ar e of li mi ted va lu e in or al li c he n p la nu s a n d tr ia mc in o lo n e , in a n a dh e si ve pa s te, i s dif fic u lt to ap pl y to wi de sp re ad er o si ve an d a tro ph ic les io n s . Th e se pre pa ra tio n s , h ow e ve r, ar e u n li ke ly to gi ve ris e to pr o ble m s a s so c ia ted w i th

sys te mi c ab so rp tio n . O the r to p ic al p rep ar ati on s (Chap ter 3 ) in c lu d e s o lu b le b eta me tha s o ne ta b lets , d is so l ved i n w ate r a n d us e d a s a mo u th w as h, s tero id s pr ay s (fo r e xa mp le ,

bec l om e th a s o ne ), p ote n t s tero id o in tm en ts (fo r exa mpl e, fl u o ci n on i de ) m ixe d w ith a n ad h es ive b as e, an d tria m ci no l o n e m o u thw a s he s . In all the s e p rep a rati o ns , s ys tem ic

ab so rp tio n c a n o cc u r a n d o ral c an d id o si s ma y oc c as ion a ll y c o m pli c ate trea tm en t. To p ic al an tif u ng a l th e rap y i s o ften in di ca ted f o r pa tie n ts wi th

sym p tom a tic o ra l l ic h en pl an u s . No t o n ly do top ic a l s ter o ids a n d o the r i mm u n o mo d ul ato rs

P. 130

pre di sp o se to ca n di do s is b u t s eve ra l s tu di es h a ve sho w n a n i nc re as ed p rev al en c e of Can d id a

sp ec ie sβ €”in b oth myc o lo g ic a l a n d h is tol o gi ca l stu die s o f o ra l lic h en pla n u s . Su p eri mp o se d or al c an d id os is c a n ex ac e rba te th e o ral s ym pto m s o f o ra l lic h en pl an u s a nd s h o u ld be

tr ea ted , in th e fi rst in s tan c e, b y ap pro p ria te top ic a l a n tif un g al a ge n ts (Ch ap ter 4 ). Nys tati n

pa sti lle s o r a m ph o teri ci n lo z e ng es a re a pp ro pri ate b ut to pi ca l ny sta tin ma y n o t b e tol era te d

by pa tie nts w i th a so re , a tr op h ic m u co s a . M ic o na z o le ge l ma y be pre fe rred a n d in s o me c as e s a s ys te mi c an tif u ng a l, s u ch a s fl uc o n az o l e, i s in dica ted . Ca re m us t b e take n w ith th e u s e o f az o le s be ca u s e o f th eir in te rac tio n s w ith o th e r d rugs , n o ta b ly wa rf ari n .

Table 11.3 Therapeutic options for oral lichen planus Ty pe

Exa mp les

An tis e ptic

Ch lo rh ex id in e gl u co n a te (m o u th wa s h )

An a lg es ic s

Be n z yda m in e h ydro c h lo ri de (m ou th w a sh )

An tif u ng a ls

Nys tati n pa s ti ll es (to pi ca l)

Lign o c ai n e rin s e

Am ph o ter ic in l o ze n ge s (to pi ca l) Mi co n a z ol e ge l (to pi ca l) Fl uc o n a zo l e ( sy ste mi c)

To pi ca l co rti co s tero i ds

Hyd ro co rti so n e h em is u cc in a te (pe ll ets )

Tria m ci n ol o n e a c eto n id e (in a dh e si ve pa s te) Fl uo c in o n id e (in ad h es ive p as te)

Be tam eth a s on e s o di um ph o s ph a te ( mo u th w as h ) Tria m ci n ol o n e m o u thw a s h

Be cl o me th as o n e di pro p io n a te (s p ray ) Bu d es o n id e (s p ray ) Sy ste mi c im mu n o mo d u la to rs

Pre dn i so l o ne Az a th io pri n e Cic lo s po ri n

Oth er rep o rted th er ap ie s

An tim a la ria ls (h yd ro xyc h lo ro q u in e) To pi ca l tac ro li m us Reti no i ds Dap so n e

In se ve re c a se s w ith m aj or ero s iv e les i on s , s h or t co u rs e s o f s ys tem ic ste ro id s ca n b e

eff ec tive to a ll ev ia te a c u te e xa ce rba tio n s o f o ral l ic he n p la n us bu t a re c o ntra i nd ic a ted in th e lo n g te rm . Hig h -co n c en tra tio n s tero i d m o u thw a s he s in s o m e c a se s ap pe a r to b e e qu a ll y

eff ec tive . A z ath io p rin e m ay be re qu i red a s a ste ro id -sp ari n g dru g . Oth e r s ys tem ic dr ug s , in cl u di n g c ic l os p or in , re ti n o id s, an ti ma la ri al s, a nd da ps o n e (Tab le 11. 3), h av e b ee n

ad vo c ate d f o r th e tr ea tme n t o f s ev ere o ra l lic h en pl an u s w ith v ary in g rep o rted res u lts . Th e re is , h ow e ve r, a pa u ci ty o f ev id en c e-ba se d trea tme n t pro to c o ls fo r th is c o nd iti o n. N ea rly a ll th e se s ys tem ic f or ms o f me di ca tio n h a ve s ig ni fi ca n t si de -eff ec ts.

OTHER ASPECTS OF MANAGEMENT If h is to pa th o lo gy in d ic ate s a si gn i fic a nt de gre e of d ys pla s ia , th en s u rg ic al (o r l a se r) e xc is io n

of th e af fec ted s ite m ay n ee d to be c on s id ere d. Liche n o id le s io n s du e to dr ug th era py m ay be dif fic u lt to ma n a ge if th e d ru g is re qu i red fo r a pa tien t's ge n era l me di ca l co n d itio n a n d an

al te rn a tive c an n o t b e s u b mi tte d. Li ch e n o id dru g erup tio n s c an o n l y b e r eli a bly c o n firm ed i f

th e y r es o lve a fter w ith dr aw a l o r retu rn a fte r re -cha ll en ge w ith th e d ru g. In m o st ca s es , th is

is di ffi c ul t o r u n eth ic a l to d o . Den ta l r es to rati o ns , p arti cu l ar ly la rge , c or ro di ng a ma l ga ms i n

po s te rio r mo l ar s, s h o u ld be c o n si de red fo r rep la ce men t a s th is m ay res u lt in res o lu ti on o f a n ad jac en t lic h en o i d r ea cti o n. S tre ss h a s be en im pli cate d a s a fa cto r in o ra l li ch e n pl an u s b ut th e re are f ew s tu di es th at ha ve p ro ve d a d ire c t relati o n sh i p. Th e c h ro ni c di sc o m fo rt

as so c ia te d w i th sy mp to ma tic o ra l lic h en pl an u s i s un d o ub ted ly go i n g to b e a stre s sf ul fa c to r an d af fe ct a p a ti en t's qu a li ty o f li fe.

Pa tie n ts s h o u ld be e n co u ra ge d to sto p s mo ki n g an d give n a dvi c e a b ou t s e ns i bl e d rin k in g.

Ora l an d de n tu re hy gi en e sh o u ld b e ad dr es se d an d a tte n tio n p ai d to the p ati en t's p eri o do n tal he al th . A ll the s e fa cto rs c a n de le teri o us ly a ffe c t o ral l ic h en p la n u s a n d ma y pre di sp o se to or al ca n di do s is .

LONG-TERM REVIEW OF ORAL LICHEN PLANUS Th e g rea t ma jo ri ty o f o ral li c he n p la nu s c a se s ru n a c om p lete ly be n ig n co u rs e an d s o me w ill go i nto rem is s io n a fter a va ria bl e n um b er o f ye ar s. H o we ve r, in a s m al l pro p o rtio n o f ca s es (0.4β €“3. 3 per c en t i n rep o rted s tu die s , o ver a fo ll o w -up pe rio d o f 0.5β €“20 ye ars ), o ra l

les io n s u n de rg o ma li gn a n t c ha n ge . F o r th is rea s o n, lo n g -te rm re vie w s of a ll c as es s h o u ld be un d erta ke n a nd re -bio p sy pe rfo rm ed i f th er e a re a ny s u sp ic io u s c li ni ca l c ha n ge s , su c h a s a no d u la r, ve rru co u s , s pe ck led , o r β € vel ve ty - red β€™ ap pe ara n c e o f th e mu c o sa . P ati en ts

sh o u ld b e a dv is ed to re po rt an y si gn i fic a n t ch a n ge s in th e ir l es io n s o r sy mp to ms . Ide a lly , a ph o to gra ph i c rec o rd o f th e pa tie n ts β €™ les i on s s h o uld be tak en a t e ac h f ol lo w -up vi s it. It

ha s be en su g ge ste d th a t i t is th e ero s iv e/a tro ph i c le si o ns o f o ra l l ic h en p la n u s tha t a re mo re lik el y to tra n s fo rm an d th a t th es e c a se s s ho u l d th eref or e u n d erg o m or e s trin g en t f o llo w -up . Th ere ar e, h o w ev er, n o c o n tro lle d stu d ie s to ref ute o r s u b sta n tia te th is cl a im .

A sm a ll pro p o rtio n o f o ral li c he n p la nu s c a se s u n der go m a lig n an t c h a n ge . Lo n g -te rm re vie w is th ere fo re es s en tia l.

Immunobullous disease Pemphigus

Pe mp h ig us i s a gro u p o f im mu n o bu l lo u s di so rd ers th at a ffe ct the s ki n an d mu c o u s

me mb ran e s. It is c h ara c teri ze d by th e d ev el o pm en t of au to a n ti bo d ie s to an ti ge ns o n th e

kera tin o c yte c ell s u rfa ce . Th e bu l la e are i ntra e pith e li al a nd th ere fo re li e a b ov e th e b as a l

la yer . Pe mp h ig u s is a di se a se o f mi dd le ag e w ith mos t p ati en ts be tw ee n the a ge s o f 40 an d

60 ye ars w i th an e ve n ge n de r di stri bu ti on . T he re is a ra c ia l fac to r i n vo lv ed in th a t th er e i s a hi gh in c id en c e o f th e co n di tio n a m on g p eo pl e o f Je wis h o rig in ,

al th o u g h pa tie n ts a re b y n o me a ns r es tric ted to th is g ro u p. Th e o ra l mu c o us m em b ran e is

in vo lv ed in a hi gh pro p o rtio n o f th e p ati en ts a n d, i n fa ct, h a lf o f a l l i n itia l le si o ns a re fo u n d

P. 131

in th e mo u th . B efo r e th e in tro d uc tio n o f s tero i ds th e pro g no s is i n th is d is ea s e w as v ery po o r. Th e i n tro du c tio n o f tr ea tme n t by s ys te m ic s tero id s , h ow e ve r, h as ma d e th e o u tlo o k

co n s id era bl y l es s gl o o my, al tho u g h pe mp h ig u s mu s t sti ll b e co n s id ere d a ver y s er io u s

co n di tio n . It i s, th e refo r e, p arti cu l arl y i m po rta nt th at th e o ra l l es io n s s h o ul d be re co g ni z ed

ea rly in o rd er th at tr ea tme n t ma y be sta rte d a t th e ea rli es t o p po rtu n ity. Th ere a re fo u r m ai n var ie ti es o f pe mp h ig u s: pe mp h ig u s vu lg ari s, veg eta ns, fo li ac eu s , a nd e ryth em a to su s .

Pe mp h ig us v u lg ari s is th e mo s t c o mm o n va ri ety an d acc o u nts f o r a bo u t 70 p er ce n t o f al l

ca se s . It u su a ll y b eg in s w ith s h a llo w e ro s io n s o n th e s ki n a nd ru p tu red bl is ters o n mu c o s al su rfa c es . In p em ph ig u s ve ge ta n s , th e ru p tu re o f bu lla e is a cc o m pa n ied b y ex ub er an t

gra n u la ti o n tis s u e, th e β€ veg eta tio n s β €™ of th e n om en c la tu re. A rar e h ere di tary fo rm i s

kn o wn as f am il ia l b en i gn c h ro n ic p em ph i gu s (o r Ha iley β€“ Hai le y d is ea s e). In thi s co n d itio n th e h is to lo g y o f th e l es io n s c lo s el y res e mb le s th a t o f pe mp h ig us p ro pe r, w ith m a rke d

ac an th o ly si s . Th e i m mu n o lo g ic al fi nd in g s are n e ga tive . Th is f o rm is , h o we ve r, a m uc h l es s ag gre ss i ve di se as e th an p em ph i gu s a n d r un s a p ro trac ted c h ro n ic c o u rse . Th e o ral l es io n s

cl os e ly res e mb le th o se o f p em p hi gu s a n d th e ra re o cc u rre nc e o f th i s va ria n t s ho u l d b e bo rn e in m in d in d if fer en tia l di ag n os i s.

Ora l mu c o sa l in vo l vem e nt in p em ph i gu s vu l ga ris i s co m mo n a n d fre qu e ntl y pre c ed es in vo lv em en t a t o th er si tes .

Clinical presentation

Th e c li n ic a l p ic tu re in pe mp h ig u s is o f w i de sp rea d bu l la e fo rma tio n on th e s ki n an d m u co u s

me mb ran e s. Th e bu ll a e, e ven of th e sk in , a re fra gi le an d b rea k do w n ra pi dl y to f or m cr us te d, ero de d le si o n s ( Fi g. 11. 7). Th e le si o ns o f mu c o u s me mb ra n es a re eve n m o re fra gi le an d

rap id ly bre a k d o wn wi th th e f o rma tio n o f irr egu l ar u lc ers , o fte n w ith a ra g ged e dg e as a

res u lt o f th e s p lit an d fra g ile ep ith el iu m . Th e o ral l es io n s ma y o cc u r i n a ny s ite w ith in th e mo u th a nd o ro ph a ryn x an d m ay be a c co m pa n ie d b y si mila r l es io n s o f o th e r m uc o s ae . Th e vu lva is o fte n in v ol ve d in th is w a y.

Immunopathology (Table 11.4)

Bu l la e a re p ro du c ed a s a r es u lt o f ac a n tho l ys is β€”th e b rea kd o wn of th e in ter ce ll ul a r

co n n ec tio n s , u su a ll y i n th e s tra tu m s p in o su m o f th e e pi th eli u m. Imm u n o pa tho l o gy sh o w s th e pre se n ce o f au to a nti bo d ie s d ir ec ted a ga in s t th e ep id erm a l a n d in terc e llu l ar ma ter ia l. Th e

au to an ti bo d y i s us u a lly a n IgG a nd co m pl em en t is a ls o fi xed a t th e si te ( Tab le 11. 4). Th e

ta rg et a n tig en s a re no w r ec o gn iz e d to be de s mo g lei n I a n d de sm o gl ei n III, tra ns m em bra n e gly co p ro tei ns b el o n gi ng to th e c ad h eri n fa mi ly o f adh e si o n mo l ec u le s.

Fig. 1 1 .7 M ul tip le ru ptu re d bu l la e o f th e la bi al m uc o s a an d to n gu e in o ra l pe mp h ig us vu lg ari s.

Management

Pa tie n ts f req u en tly p res en t w i th a se ve rel y u l ce ra ted mo u th w ith ver y l ittle n o rma l mu c o s a, betw e en th e le si o n s. B io ps y ma y pro v e to b e di ffi cul t as th e ep ith e liu m i s rea di ly de tac h ed fro m th e u n de rlyi n g m u c o sa a n d i t i s im po rta nt tha t p eri le si o n al tis s u e i s o bta in ed . Th e

fra gi lity o f th e ep ith e liu m m ay b e c o n fi rme d in s o me p ati en ts by th e po s itiv e Nik o ls ky si gn ; th e e pith e li um c a n be d eta ch e d b y la tera l pre ss u re. Im m u no l o gic a l me th o ds a re no w

em plo y ed fo r the d ia gn o s is o f pe mp h igu s , a s au to a n ti bo d ie s are fo u n d in b o th the a ffe c ted

or al (a n d s ki n ) tis s u e a n d ci rc u la tin g in th e pa tien t's se ru m. Dire ct i m mu n o flu o re sc e nc e (IMF ) o f th e

per ile s io n al o ra l ti s su e de mo n s tra te s IgG -cl as s a nti bo d ie s b in d in g to th e in terc el lu l ar

su b sta n ce s an d ce ll m em bra n es i n th e stra tu m sp in o sum o f a ffe cte d epi th el iu m. Po s iti ve

dir ec t IMF i s es s en tia l fo r d ia gn o s is . Ci rc ul a ti n g au to an ti bo d ies a re d em on s tra ted by in d ire ct im mu n o flo r es ce n ce i n ap pro x im ate ly 90 p er c e nt o f pati en ts w ith pe mp h ig u s vu lg ari s.

Table 11.4 The immunopathology of bullous diseases Diseas e

Pe mp h ig us

Dire ct I M F: siteβ€

Ep ith el ia l -

bo u n d, Ig G, C3:

In dir ect I M F * (cir cu latin g

au to an tibo dies ) Po s iti ve IgG (90%)

Des mo g le in I

Po s iti ve IgG (75%)

Bu l lo u s

In te rce ll u la r Bu l lo u s

pem p hi go i d

Lin ea r, Ig G C3: BM z o n e

Tar ge t a nt igen s

an d III

pem p hi go i d

an tig en s , B P 180 an d B P230

Mu c o u s

me mb ran e

pem p hi go i d

Lin ea r Ig A dis e as e

Derm ati tis

he rpe tif or mi s

Ep id erm o ly si s bu ll o sa

Lin ea r Ig G, IgA C3: B M z o n e

Po s iti ve IgG (75%), Ig A (50% )

BP 180 i n th e ma jo rity;

la mi ni n 5; Ξ² 4 in teg rin

Lin ea r, Ig A, C 3: BM z o n e

Po s iti ve IgA (30% of a du lts )

Gran u l ar, IgA ,

Neg ati ve fo r

der ma l pa pi ll ae

au to an ti bo d ies

C3: ti ps o f

Lin ea r IgG, C3

ac qu i si ta

epi th el ia l

Oc ca s io n a lly d erm al bin d in g IgG

A nu m be r o f

ta rg et a n tig en s

in cl u di n g B P 180 Un kn o w n

Typ e 7 co l la ge n

*T h e p res e nc e o f ci rc u la tin g au to a nti bo d y d ep en d s on sk in s u bs tra te u s ed , i. e .

mo n ke y o es o ph a gu s fo r pe mp h ig us a n d sa lt -sp li t sk in f o r pe m ph ig o id a nd li n ea r Ig A dis e as e.

β€ BM , b as em e nt me mb ra n e.

Th e tre a tm e nt o f p em ph ig u s is a m u ltid is c ip lin a ry ma tter, i n vo lv in g o ra l ph ys i ci an s ,

der ma to lo gi s ts , a n d oth e r m ed ic al s pe ci a liti es β€” as re qu i red . Th e pa rtic u la r r o le o f th e o ral

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ph ys ic ia n i s, f irs t o f al l, to e n ab le e arl y di ag n o si s by th e r ec o gn iti o n o f th e o ral l es io n s a nd , la te r, to h e lp in th e ma n a ge me nt o f th e o ften very s ev ere o ra l d is ea s e. T he p ro gn o s is h a s

bee n co m pl ete ly al tere d by th e i n tro du c tio n o f sy ste mi c ste ro id s an d ma n y pa tie nts ma y le ad rea so n a bl e liv es m ai n tai n ed o n s ub s ta n tia l do s es o f pre dn i so l o ne , o fte n w ith th e u se o f

az a thi o pri n e a s a s tero i d-sp ari n g dru g . Ve ry hi gh do s ag es a re u se d in iti al ly to s up pr es s bu ll a fo rma tio n ( o f th e o rde r o f 1 m g/k g p red n is o lo n e da il y), b u t th is m ay o fte n be s lo w ly re du c ed to a ma in te na n c e d o se o f 15 m g da il y o r th ere ab o u ts. O th er ste ro id -sp ari n g,

im mu n o su p res s ive d ru gs s u ch as cy cl o ph o s ph a mi de o r c ic l os p or in h a ve al s o be en u s ed to tr ea t pe mp h ig u sβ €”se ver e ca s es m a y re qu i re p la s ma phe res is . Th e titre o f ci rc u la tin g an tib o di es re fle c ts th e d is ea s e se ver ity an d is th erefo re a u s ef ul g ui de f or th era py .

Sy ste mi c ste ro id th era py m ay b e s u pp le me n ted by h igh -co n c en tra tio n ste ro id m o uth w a sh e s, si nc e th e o ra l mu c o s a is o fte n le ss re s po n si ve to tre atm en t th an th e s kin . J us t a s th e o ra l

mu c o sa s ee ms to b e pa rtic u la rl y p ro n e to a tta ck by th e an tib o di es e arl y i n th e co u rs e of th e

dis e as e, i t a ls o o fte n rem a in s qu i te s ev ere ly a ffe cte d w h e n th e s ki n le s io n s are i n rem is s io n . Th is m ay be th e ca s e eve n w h en th e le ve l o f th e a n tib o dy titre is u n m ea su ra b ly lo w . Lo n g -

te rm , to pi ca l ste ro id th era py m ay be re qu i red , su p pl em en te d b y s u c h me a su re s as an ti fu n ga l th e ra py an d th e us e o f a n a es the tic m o u th w a s he s as ne ce s sa ry. O ra l h ygi en e m ay pr es en t a gre at p ro bl em a n d ca re mu s t b e ta ke n if th e teeth are n o t to be l o st, a n im p or ta n t d en ta l fac to r, s in c e th e w ea ri ng o f de n tur es m ay be di ffi cu l t, if n o t im po s s ibl e.

Pemphigoid

Th is g ro up o f im mu n o bu l lo u s di so r der s is ch a ra cte riz e d by the f or ma tio n o f su b ep id erm al

bu ll ae a n d th e p res en c e of im mu n o re ac ta nts a t th e ba s em en t m em bra n e z o ne . Th e re a re tw o bro a d c l in ic a l su b typ es o f th i s co n d ito n : th e fir st, w h ic h p red o mi n an tly a ffe c ts th e s kin wi th

oc c as io n a l mu c o sa l in vo l vem en t, i s ref erre d to as bu ll o us p em ph i go i d (B P ). T he re is a f urth e r cu ta ne o u s su b gro u p o cc u rri n g i n pr eg na n c y k no w n a s pe mp h ig o id ge sta tio n i s. T he s ec o n d su b ty pe p red o mi na n tly in v ol ve s th e m u co u s m em bra n es w i th o n ly o cc a si on a l sk in

in vo lv em en t a n d is n o w re fer red to a s mu c o u s me mb rane pe mp h ig o id (M MP ) β€”th i s wa s fo rme rly re fer red to a s be n ig n mu c o u s me mb ra ne pe mph ig o id o r c ic a tr ic ia l p em p hi go i d.

Bullous pemphigoid CLINICAL PRESENTATION Th e p a ti en ts w ith p em ph i go i d ar e, i n th e m a in , el derly , mo s t b ei ng o ve r th e a ge o f 60 yea rs , al th o u g h so m e ve ry f ew m a y b e c o n s ide ra bl y y o un g er. A s i n pe mp h ig us , th e ge n de r

dis tri bu tio n is e ven . T h ere is n o r ac ia l fa cto r in vo lv ed . B u llo u s p em ph ig o id i s th e m o st co m mo n i mm u n o bu ll o u s dis e as e in We s tern E u ro p e.

Fig. 1 1 .8 Des qu a m ati ve gi n gi viti s in a p ati en t w ith b u ll o us pe mp h ig o id.

Th e co n d itio n sta rts w ith a p ru ritic , s o me tim es u rti ca ria l ra sh , o fte n o n th e lim b s bu t i t m ay al so i n vo lv e th e tru n k. Wi th in th es e er yth em ato u s area s de ve lo p ten s e bu l la e a t a n i nte rva l var yin g fro m a f ew d ay s to a few we ek s. B u l la e m a y re m ai n lo c al iz e d to th e li m bs o r m a y

bec o m e m o re ge n era li z ed . Th ey a re f lu id -fil le d b u t ma y la ter be co m e ha e mo rrh a gi c. M u c o us me mb ran e l es io n s ar e u s u al ly c on f in ed to th e m o u th an d o c cu r in u p to 20 p er ce n t o f

pa tien ts β€” in s o me c as e s th e gi n gi vae a re in vo l ve d ( Fi g. 11. 8). In tr ao ra ll y, i n co n tra s t to

pem p hi gu s v ul ga ri s, b li ste rs ma y o cc a si o na ll y be seen . T he c li n ic al co u rs e o f B P i s u su a ll y les s s ev ere th an th a t o f pe mp h ig us w i th ma n y p a ti ents h av in g a res o lu tio n wi th in 2 to 5

yea rs . Nev erth e le ss , w h ile p ati en ts w ith lo c al iz e d d is ea s e ma y b e co n tro l led a de qu a tel y w i th to p ic a l c o rtic o s te ro id cr ea ms , p ati en ts w ith m o re wi de sp re ad le s io n s w ill re qu ir e s ys tem ic im mu n o su p pre s si o n. In th i s el de rly po p u la tio n , pa tie n ts a re a t a pa rti cu la r ris k fro m th e co m pl ic ati o ns o f s ys te m ic dr ug th e rap y.

IMMUNOPATHOLOGY Th e b li ste rs in b u ll o us p em ph i go i d ar e s u be pi de rm al a nd m ay th ere fo re re ma in in tac t f or a

nu m be r o f da ys . Dir ec t im mu n o fl u o res ce n ce o n b io p sy m ate ria l sh o u ld d em o ns tra te Ig G o r C3 im mu n o rea c tan ts in a lin e ar di stri bu ti on al o ng th e ba s em en t m em br an e z o ne (Fi g. 11. 9 ).

Circ u la tin g a nti -ba se me n t m em bra n e zo n e IgG au to a n tibo d ie s (75 p er ce n t o f p ati en ts ) a n d

oc c as io n a l IgA m ay a ls o be d ete cte d by in d ire ct im mu n o fl uo r es ce n ce tec h n iq u es to b in d o n to th e b as em en t me mb ran e o f th e e pi th eli u m ( Tab le 11. 4). Lin e ar de po s itio n o f IgA an ti bo d ies

al on e , in th e c o nte xt o f a pre do m in a n tly cu ta n eo u s bl is teri n g di se a se , is m o re su g ge sti ve o f

lin e ar IgA d is ea s e. Th e m ai n ta rge t an ti ge ns i n bu llo u s pe mp h ig oi d ar e th e B P230 an d B P 180 an tig en s . Th es e a re co n s id ere d to be im po rta n t p ro te in s i n ma in ta in i ng th e str uc tu ra l

in teg rity o f th e ba s em en t me mb ra n e, th a t is , in provi di n g c o h es io n of th e de rmi s to th e epi de rm is .

MANAGEMENT Dia gn o s is is b as e d u p o n a co m bi n ati o n o f cl in i ca l si gn s (s ki n a nd o ra l) an d im mu n o flu o re s ce n t fin d in g s. T o im pro v e th e su c c es s o f

IM F te ch n iq u es , a pe ril es io n a l bio p s y i s es se n tia l. Wi th th e u s e o f sy ste mi c a nd to pi c al

ste ro ids m o s t pa tie n ts ca n b e k ep t in r ea so n a bl e c om fo rt. S te ro id s c an , i n mo s t c as e s of

bu ll o us p em p hi go i d, b e r ed uc e d to a m in i mu m o r co m pl ete ly tai le d o f f, a s mo s t p ati en ts w ill

eve n tua ll y ac h ie ve rem is s io n , tai le d o f f a fte r 3β €“6 ye ars ( un l ike p em ph i gu s ). Fo r lo n g -te rm us e o f s ter oi ds , ad di tio n a l i mm u n o su p pre ss iv e ag en ts s uc h a s a za th io p rin e s ho u l d b e ad de d as ste ro id -sp ari n g ag en ts . Dap s on e m ay be a n a lte rnati ve firs t -lin e a ge nt in B P . To pi c al ste ro id pre pa ra tio n s a nd a n al ge si c mo u th w as h es m a y b e be ne fi ci al fo r o ral l es io n s .

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Fig. 1 1 .9 Imm u n o flu o re sc en t de mo n s tra ti o n o f Ig G -gro u p an ti bo dy c o mp le xes a lo n g th e ba sa l z on e in pe mp h ig o id.

Mucous membrane pemphigoid (MMP)

Th is c o mp ris es a h e tero g en eo u s g ro up o f d is o rde rs th at ma y be di sti n gu is h ed f ro m c l as s ic al bu ll o us p em p hi go i d b y h av in g a pre di le cti o n fo r m u co s a l s i te s a nd a te nd en c y to res u lt i n fib ro si s .

CLINICAL PRESENTATION Mu c o u s me mb ran e p em ph ig o id i s a cl in i ca ll y h e tero g en eo u s d is ea s e, w h ic h m ay pr es en t to a wi de ra n ge o f sp ec i al is ts in c lu di n g o ra l ph y si ci an s, de rm ato l og is ts , o ph th al mo l o gi sts ,

gyn a ec o lo g is ts, a n d E NT s pe c ia li sts . Wh il e th e m ajori ty o f pa tie n ts h ave o ra l le si o n s, w i th

ad di ti o n al m u co s al s ite s freq u en tl y i nv o lve d, th e re are s u bg ro u ps o f p ati en ts w ith d is ea s e lim ite d to o ne s ite , fo r e xa mp le , pu re o c u la r pe mp hig o id o r p ur e o ra l pe mp h ig o id. Sk in

les io n s a re in fr equ e n t a nd a re pre s en t in 10 β€“25 pe r c en t o f c as es , u s u al ly ap pe ar in g o n the fac e , n ec k, o r sc a lp .

In tra o ra lly , in ta ct bu ll ae m a y s o me tim es b e p re se n t, fo r e xa m ple , o n th e gi ng iv ae o r s o ft pa la te. M o re o ften p a tien ts p res en t wi th u lc era ted a rea s o f mu c o sa i n vo lv in g the b u cc a l, pa la tal , o r o c ca s io n a lly li n gu a l o r l ip mu c o s a ( Fi g. 11. 10 ). Des qu a m ati ve gi ng iv itis i s a

freq u en t p res e nta tio n a n d ma y be lo c a liz e d o r ge n era li z ed . Th e dif fe ren tia l di ag n o si s o f th is in cl u de s li ch e n pl an u s a nd p em ph i gu s . In tr ao ra l sc arrin g m ay o cc u r i n M MP b u t it ma y be a si gn if ic an t fin d in g in o th er mu c o s al s ites s u c h as th e c o n ju n cti va o r i n th e s ki n . Th e te rm β€ ci ca tric ia l β €™ pe mp hi go i d ha s th u s be en f av o ur ed in th e p as t fo r th is s ub gr ou p o f pem p hi go i d a n d rem a in s th e pre fe rred te rm in th e o ph th a lm ol o gy li tera tu re.

On s et o f M MP va rie s fro m u n de r 30 y ea rs o f ag e to ov er 70, bu t i s mo re c o mm o n in la te mi dd le to o ld a ge (50 β€“ 70 ye ar ag e gro u p) . Th ere is a 2:1 pr ep on d era n c e o f fe ma le

pa tien ts . Th e fre qu e nc y an d e xten t o f bl is teri n g v ar y f ro m v irtu a ll y c o n tin u o u s an d m u ltip le , to in term itte n t an d s in g le. Th e b u ll ae , w h en e sta blis h ed , ar e i n ge n era l pa in le s s, b u t th ere ma y b e di sc o mf o rt w h en th e bu l la is f o rmi n g a n d af ter ru ptu re.

Fig. 1 1 .1 0 Ru ptu re d gi n gi val b u lla e in m u c os a l pe mp h ig o id.

Mu c o u s me mb ran e p em ph ig o id m ay pr es en t a s a de s qu a ma tive gi n gi viti s. In a co n s id era bl e pro p or ti o n o f pa tie n ts (as m u ch as 75 per c en t) th e c on ju n c tiva a re

eve n tua ll y in vo lv ed a n d, si n ce th e le si o n s i n th is s ite h ea l w ith s ca rri n g, v is io n m ay b e

aff ec ted . A co m bi n ati o n o f s u bc o n ju n cti va l f ibr o si s lea d in g to ad h es io n s o f the c o n ju nc tiv a,

sym b lep h ar o n fo rm ati o n (a dh es io n o f b ul ba r c o n ju n cti va to th e eye -lid ), a n d l o ss o f th e tea r fil m res u lts in o pa ci fic a tio n o f the c o rn ea a n d bl in dn e ss .

Al l p a ti en ts d ia gn o s ed a s ha vi n g m u c ou s m em b ran e pem ph ig o id s ho u l d b e ref erre d to a n

op h th al mo l og is t, a s ea rly trea tm en t o f o cu l ar in vo lvem en t, w h ic h ma y be a sy mp to ma tic , is es se n tia l.

Oc u la r i nv o lve m en t in mu c o u s me mb ra ne p em ph i go id i s co m mo n a n d ma y lea d to bl in d ne s s. Al l p a ti en ts s h o ul d be re ferr ed fo r op h th al mo l og ic al as s es s me n t.

Oth er mu c o u s me mb ra n es s u ch a s vu l va l, n as a l, p h aryng ea l , la ryn g ea l, o es o p ha g ea l, a n d an o ge n ita l m a y b e af fec ted . P ati en ts sh o u ld th e refore b e as ke d a b ou t sy mp tom s (s u ch as ho a rs en e ss , dy sp h ag ia , a nd g en i ta l di sc o m fo rt) th a t m igh t in di ca te in vo l vem e nt o f o th er mu c o us m em b ran e s.

IMMUNOPATHOLOGY As i n bu l lo u s pe mp h ig o id, th e o ra l bl is ters a re s ubep ith e lia l. Dire ct i m mu n o flu o re sc e nc e o n un f ixe d, f res h b io ps y ma ter ia l s h o w s IgG, Ig A , or C3 in a l in ea r d is trib u tio n a lo n g th e ba se me n t me mb ra n e z o n e. No t a ll pa tie nts w i th the cli n ic al d is ea se h a ve po s iti ve im mu n o flu o re s ce n ce .

Im m un o p ath o lo g ic a l f in di n gs c o n ce rn in g th e de tec tion o f ci rc ul a ti n g an ti -ba se me n t

me mb ran e z o n e a u to an ti bo d ies i n M MP ha ve sh o w n w id e va ria tio n i n th e p a st an d in m a n y pre vio u s

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stu di es a u to an tib o di es w e re in fr eq ue n tly de tec ted . Ho w eve r, w ith th e u s e o f a ba ttery o f sk in su b stra tes an d , in pa rtic u la r, w ith th e u se o f sa lt-sp li t sk in s u bs tra te, d ete cti on of b oth IgG

an d IgA c ir cu la tin g a n tibo d ie s in M M P h as c o n si de rabl y i nc re as e d ( se e Tab le 11. 4 ). Mo l ec u la r te c hn i qu e s ha ve re ce n tly de mo n s trate d th at a u to an tibo di es ta rg et s pe c ifi c pro te in s in th e

ba se me n t m em bra n e. T he ma jo ri ty o f se ra rea c t to the bu ll o u s pe mp h igo i d an tig en , B P 180. Ho w eve r, ad di tio n a l s u bg ro u ps re ac t to al mi ni n 5 a nd Ξ² 4 in teg rin (o cu l ar su b gro u p ).

MANAGEMENT Trea tm en t o f or al in v ol ve me n t is i n itia ll y o n a lo cal b as is , u s in g to pi ca l ste ro id s in a

mo u th wa s h , sp ra y o r in c or po ra ted in a p a ste . Sy mp toma tic tre atm en t f o r th e rel ie f o f o ral ero s io n s ca u s ed by th e pe rfo ra tio n o f th e b u ll ae m ay a ls o be n ec e ss a ry. P a ti en ts w ith

rec u rren t mu l tip le bu l la e m a y n e ed lo n g -te rm s tero id m o u thw a s he s . Of te n , h o we ve r, c on tro l , eve n w ith h i gh c o n ce n trati o ns o f lo c a l s tero i ds , is i n ad eq u ate . Dap s o ne h a s be en ad vo c ate d as a s ec o n d-lin e tre atm en t f or mi ld to m o de rate m u co s a l p em ph i go id . It is , in ge ne ra l, a

we ll -to l era ted d ru g, b u t ma y ca u s e a h a em o ly ti c a naem i a. Th er efo re , c are fu l mo n i to ri n g of th e p ati en t's bl o o d c o u n t is r equ i red . In th e mo s t s ev ere c as e s o f m uc o s al d is ea se , fo r

exa m ple , in the p res en c e o f a c ti ve co n ju n c tiva l in fl am m ati on an d s ca rri n g, s ys tem ic s tero i ds co m bi ne d w ith a ste ro id -sp ari n g ag en t s u ch as a z athi op ri ne o r cy cl op h o sp h am id e ma y be req ui red .

Th e c li n ic a l c o u rse o f M MP i s va ria bl e bu t fo r th e ma jo ri ty o f pa tie nts i t is a c h ro n ic d is o rde r th a t re la ps es a n d rem its o v er m a n y ye ars . F o r th o s e w i th mi ld di se a se li mi ted to th e o ral mu c o sa , th e c o n di tio n m ay β € bu rn o u tβ€™ af te r a f ew y ea rs. Am o n g th e se p ati en ts in

wh o m , fo r e xa mp le , mi n im al d es qu a ma tiv e g in g ivi tis o r bu ll ae o c cu r o nc e ev ery fe w mo n th s , or al di sc o m fo rt m ay b e re li ev ed by th e in term itte nt u se o f to p ic a l s tero i ds a n d an ti se pti c or an a lg es ic m o uth w a sh e s al o n e. F o r th e rem a in de r o f pa tie n ts atte n di ng o ra l me di ci n e c li n ic s wi th mu l tis ite di se a se o r s ev ere o ra l di se as e, lo n g-te rm da p so n e ma y be re qu ire d w ith

oc c as io n a l ad di tio n al s h o rt c ou rs e s of sy ste mi c s tero id s . It is i mp o rtan t to a p pre ci ate th a t

so m e o f th es e pa tie nts m a y req u ire m u ltid is c ip lin a ry ma n ag em en t to ac h ie ve o pti ma l ca re .

Oral immunobullous diseases: summary

It sh o u ld p erh a ps b e ad de d th at th e c o nc e pt o f rig id c la s si fic a tio n s o f b u llo u s d is ea s es ,

cl ea rly de fi ne d o n c lin i ca l an d h is to lo g ic al g ro un ds , is to s o me e xten t g iv in g wa y to th e i de a th a t o ve rla p c on d iti on s m a y o c cu r. T h is re co g ni tio n b y d er ma to lo gi sts o f a sp ec tru m o f

pre se n tatio n of b ul lo u s s kin di se as e is p ara l lel ed b y s im il ar o bs erv ati o n s in th e ca s e o f o ra l les io n s . A lth o u gh th e gr ea t m ajo ri ty o f the s e ma y be c la ss if ie d wi th s om e de gre e o f

co n fi de nc e in to o n e o r o th er o f th e ac c ep ted di s ea se p atte rn s, th e re r em ai n s om e le si o n s th a t b o th cl in ic a ll y a n d h is to lo gi ca l ly se em e qu iv oca l.

Dermatitis herpetiformis

Derm ati tis h erp eti fo rm is i s an u nc o m mo n b u llo u s s kin di se as e , in va ria b ly as s o ci ate d wi th glu te n en te ro pa thy . Th e cu ta n eo u s pre s en ta ti o n is of gro u p s o f i tch y ve si cl es th a t

ch a rac te ris tic al ly a ffe ct th e kn e es , el bo w s , an d b utto c ks . Tw o typ es o f o ra l le si o n h av e b ee n des c rib ed β€” on e c o ns i sti ng o f fra gi le ve si c les a n d the s ec o n d o f k era to tic p atc h es c li ni c al ly so m ew h at res em b lin g li c he n p la n us . Ho w e ver , sk in and o ra l les i on s s h ar e th e s am e

ch a rac te ris tic im m u no p ath o l og ic a l f ea tu res β€” of gran u la r l gA d ep o si ts lo c al iz ed to th e a pe x of th e pa pi lla e o f th e la m in a pro p ria ( Tab le 11. 4).

Linear IgA disease

A me n ti o n s ho u l d b e ma de o f li ne a r IgA d is ea s e, a ra re bu l lo u s di se a se o f sk in th a t cl in i ca ll y

ov erl ap s w ith de rm ati ti s h erp eti fo rm is a nd b u llo u s p em ph i go id . Th er e a re tw o typ es o f lin e ar Ig A di s ea se : o n e a ffe cti ng c h il dre n (c h ro ni c bu l lo us di se a se o f ch il dh o o d ) a n d th e o th er

ad u lts (a du lt lin e ar IgA d is ea s e). S k in l es io n s a re c l as s ic al ly se en as te n se b u lla e o n th e

tr un k , li mb s, o r sc a lp , so m eti me s ar ran g ed in gro u ps kn o w n a s β € ro se tte s β€™. In m a n y

pa tien ts th e cl in i ca l pi ctu re is di sti n gu is h a ble f ro m th a t o f bu ll o u s pe mp h igo i d. O ra l les i on s

ap pe ar to be u n co m mo n b u t a re si mi la r to th o s e s e en i n pe mp h ig o id. As th e n am e su g ge sts , th e ch a ra cte ris tic i mm u n o hi s to l o gi ca l fea tu re is o f li ne a r de po s its o f IgA a t th e ba s em en t

zo n e (Tab le 11. 4). Th e IgA a n tibo d ie s ar e di rec ted a t a n u mb er o f ta rge t a n tig en s , in c lu di n g th e b ul lo u s pe m ph ig o id an ti ge n, BP 180. Th i s fin d in g em ph a s iz es th e bro a d o ve rla p be tw e en th e su b ep id erm a l b li ste rin g di s ea se s. Dap so n e is the s ys te m ic dr ug o f ch o i ce fo r the tr ea tme n t o f l in ea r Ig A d is ea se .

Epidermolysis bullosa

In th e se g ro u ps o f di so rd ers th ere is s ki n a nd m u cos a l f rag il ity w ith b lis ter in g fo ll o wi n g me ch a n ic al tra um a β€” he n ce th ey a re so m eti me s ref erre d to as β € me ch a n o bu ll o us

dis e as es β€™.

Inherited forms

In h e rite d f o rms o f ep id erm o ly si s bu ll o sa (E B ) co m pri s e a ra re bu t s eri o u s gro u p o f b li ste rin g dis o rd ers , o f w h i ch o v er 20 typ es h a ve be en rep o rted . Th ey a re, h o w ev er, hi gh l y u n li ke ly to

be di ag n o se d on the b as is o f o ral s ym pto m s al o n e b ut m ay be o f gre at si gn if ic a nc e to de n tal tr ea tme n t.

Th e v ari o u s type s o f E B h a ve be en di vid ed in to th ree m ai n su b gro u p s, b a se d on the hi sto l o gic a l lev el o f b u ll a fo rm ati on , th e mo l ec u lar b as is o f th e de fec t, a nd m o de o f in h eri tan c e (Tab le 11. 5).

Th e p red o mi n ati n g f ea tu re is ex trem e fra gi li ty o f th e ep ith e liu m , th e r es u lt o f bu l la e

fo rma tio n o c c u rrin g ei th er sp o nta n eo u s ly o r a s a res p on s e to mi n im al d eg ree s o f tra u ma . In

th e β€ si mp le xβ €™ fo rm o f th e di se a se th ere i s les s s ev ere bu l la fo rm a tio n a n d th e m uc o u s me mb ran e s an d tee th ar e r are ly in vo l ve d. M a ny p ati en ts o nl y ha ve b lis te rs o n the s o le s o f

th e ir fe et. In th e le tha l ju n cti on a l an d dy str op h ic typ e o f E B , th ere i s exte n si ve in vo l ve me nt of m uc o u s me m bra n e an d th e o ra l mu c o sa i s ex trem el y f rag il e. B u ll a e bre a k d o wn to fo rm

pa in fu l ero s io n s a n d th e re is s ca r f o rma tio n . In the ju n cti on a l type o f E B th e a ff ec ted c hi ld a t bir th pre se n ts wi th a n ex tr em e de gre e o f fr ag il ity of th e sk in a n d mu c o us m e mb ran e s th at i s in co m pa tib le w ith li fe, an d de ath is

co m mo n i n in f an c y. In th e n o nl eth a l j un c tio n a l ty pe, the re is e xten s iv e s ki n a n d m u c os a l bli s te rin g b ut sc a rrin g i s mu c h le ss s e vere th a n in r ec es s ive d ys tr o ph ic E B .

Table 11.5 Subgroups of epidermolysis bullosa Clin ic al featu r es

Site o f b lis ter

In h er itan ce

Si mp le x e pi de rmo l ys is b ul lo s a Sk in b li ste rs at bir th, m a in ly in d u ce d by fric tio n .

Ba s al c el ls

Ora l in vo l vem en t ab se n t o r m il d. T eeth

Ma in l y

au to so m a l do mi n an t

no rm a l

Ju nc tio n a l ep id erm o ly si s bu ll o sa Herl itz (le th al ) fo r m r es u lts in e xte n si ve

Lam in a lu c id a

Al l a u to so m a l

P. 135

ski n a n d m u c os a l i n vo lv em en t, de n tal

rec es s ive

ab n or ma li ties a n do f ten w ith dea th i n

in fa n cy . Th e no n l eth a l fo rm p ro du c es

wi de sp rea d s kin an d va ria b le mu c o sa l in vo lv em en t

Dy stro p h ic ep id erm o ly si s bu ll o sa Dom in a n t fo rm is o fte n m ild . Re ce ss i ve

Im m ed ia tely b elo w

Dom in a n t

bli s te rs an d s ca rri n g o f s kin , l o ss o f

th e b as em en t

fo rms

fo rm is ve ry se ve re wi th ex ten s ive

na il s , se ve re o ra l mu c o s al bl is teri n ga n d sc ar rin g , an d h yp o pla s tic tee th

th e la m in a de n sa o f me mb ran e

an d rec e ss iv e

In rec e ss iv e d ys tro ph i c EB , b u ll ae a re se en a t, o r s o o n a fter, bir th. T h e l es io n s ar e p ro du c ed in re sp o n se to th e mo s t m in o r d eg ree s o f tra u m a an d ev en tu al ly h ea l w ith s ca r fo rm ati on , th i s le ad in g ev en tu al ly to gr os s tis s u e d ef o rmi ty, pa rtic u la rl y o f th e e xtre mi ties . Th e o ra l mu c o us m em b ran e is e qu a ll y s u s ce pti ble to da m ag e and , as a re s ul t o f the re pe ate d sc a r

fo rma tio n , o p en in g o f the m o u th ma y be co m e gre atl y re s tric ted a nd th e to ng u e bo u n d do w n . Th e e s se n ti a l p ath o lo g y in th i s fo rm o f th e di s ea se i s d er ma l; th e f rag il ity o f th e tis su e s is

du e to de fi ci en t c o ll ag en f o rma tio n i n th e su b epi thel ia l s tru c tur es . Th is s itu a tio n i s pa ra ll ele d in th e teeth , th e re bei n g ab n or ma li tie s i n d en tin e fo rm ati o n th at le ad to h yp o pl as i a a n d a hi gh s u s ce pti bi lity to c ari es . S in c e co n s erv ati ve de n tal trea tm en t o r eve n ef fe cti ve o ral

hy gi en e me as u re s ma y b e al mo s t im po s s ibl e in th e se pa tie n ts, th e re su l ti n g gro s sl y c a rio u s te eth , a s so c ia ted w ith a re stri c te d ac c es s an d e xtrem e mu c o s al fra g ili ty, p res en t a m a jo r

pro b lem to th e d en ta l su rg eo n . It h a s al so be en re po rte d th a t th e o ral s c ars h a ve , in so m e

pa tien ts , be en fo ll o we d by th e o n s et o f le u ko pl ak ia a n d, f in a lly , ca rc in o m a, b u t th is c a nn o t be reg a rde d a s a c h ara c teri sti c of th e di se a se .

In ep id em o lys is b u ll o sa th e o ral m u co s a an d te eth may be af fe cte d.

Epidermolysis bullosa acquisita

Ep id erm o ly si s bu ll o sa a c qu i si ta i s a ra re au to i mm u ne s u be pi de rma l bu l lo u s di so rd er in vo l vin g th e s kin an d m uc o u s me mb ra n es . Th er e a re tw o bro a d c lin i ca l su b gro u p sβ €”an i n fla m ma to ry ty pe rem in i sc e nt o f B P a n d a m ec h a no b u ll ou s s u bty pe in w h i ch b u ll ae o c cu r in re sp o n se to

tr au m a an d le s io n s are th er efo re m o st pro m in en t on the k ne es e lb o ws , h a n ds , an d fe et. O ra l les io n s , w h ere p res en t, m ay va ry in s e veri ty f ro m mi ld de sq u am a tive g in gi vi tis to s eve re

gen e ra liz e d ul ce ra tive a n d of ten s c arr in g mu c o s al in v ol ve me n t. Dire ct i m mu n o flu o re sc e nc e is po s itiv e f o r li n ea r b as e me n t me mb ra n e Ig G a n d C3. On s al t -sp li t sk in , c irc u la tin g IgG an tib o di es b in d to th e ba s e o f th e s p lit co rr es po n di n g wi th th e ta rg et a n tig en , typ e 7

co ll a ge n. Tre atm en t i s o ften very u n s atis fa c to ry d es pi te sy ste mi c im mu n o s up pr es si o n , an d pa tien ts f req ue n tly de ve lo p pro m in e nt mu c o sa l an d skin sc a rrin g .

Erythema multiforme

Ery th em a mu l ti fo rm e (E M) is a n a cu te ves ic u lo b u llo us d is ea s e o f s kin a n d mu c o u s

me mb ran e s w ith a w id e ran g e of cl in i ca l pre se n tati ons β €”he n ce th e term β€ mu lti fo rm e β€™.

It ma y be pre c ipi tate d by a ra n ge o f f ac to rs , in c ludi n g in fe cti on s (p a rtic u la rly vi ral ), d ru gs o f var io u s ki nd s , ne o pl as i a, a n d pre gn a n cy (Tab le 11. 6). In le ss th a n h al f of th e ca s es n o s u ch

in du c in g fa c to r is f ou n d . In th e m o s t fu ll y de ve lo pe d fo rm (a lte rn ati vel y k n ow n a s th e

Ste ven s β€“ Jo hn s o n s yn dr om e) , the re is w id es pre ad i nvo lve m en t o f the s ki n an d m u co u s

me mb ran e s bu t, i n the m o re u su a l res tri cte d f o rm, th e o ra l m u c ou s m em br an e is m a in ly

in vo lv ed , w ith n o m o re tha n mi n o r le s io n s in o th er s ites . Th e p ati en ts ar e p red o mi n an tl y yo u ng a du l ts, m a les be in g a ffe cte d m o re fre qu e n tl y th a n fe ma l es .

Ery th em a mu l tifo rm e is a n a cu te ve si cu l o bu ll o u s dise as e o f s ki n an d m u co u s me mb ra n es . Th e m a in f ea tur e o f a n atta c k i s th e su d de n de ve lo pme nt o f w id es p rea d ero s io n s o f th e o ra l

mu c o sa , c ha ra c te ri sti ca ll y in vo l vin g th e li ps . Th e ero s io n s a re pro d uc e d b y th e d is in teg ra tio n of su b ep id erm a l b u ll ae , le si o ns th a t o n ly ra rel y l as t l o ng e n o ug h to b ec o me a di ag n o sti c

fea tu re. T he e ro si o n s on the l ip s (es pe ci a lly th e lo w er lip ) a re ac c o mp an i ed b y c ru sti n g a n d

ble ed in g a nd a re , if n ot ab so l u tely d ia gn o s ti c , stron g p oi n ters to th e n atu re o f the c o n di ti o n (Fi g. 11. 11). Th ere i s o ften a c e rvic a l l ym ph a de n itis w i th

P. 136

pyre xi a an d th e pa tie n t fe els u n w el l. T he a c co m pa n yin g s ki n le si o ns , w h en pre se n t, ma y h av e a c h a rac ter is tic β€ ta rg etβ €™ (o r β € iris - lik eβ €™) ap pe ar an c e tha t i s di ag n o sti c. O c u la r

in vo lv em en t, i f p res en t, m a y re su l t in co n ju n cti va l d a ma ge bu t th i s is u n u su a l. A n a ttac k

gra du a ll y s u bs id es a fte r s o me 10 d ay s, bu t th er e is a c o n si de rab le l ike li ho o d o f rec u rren c e o f EM a fte r a p eri o d v ary in g fro m n o mo re th a n a fe w week s to a ye ar o r s o . Th is i s mo re li ke ly if EM i s a ss o ci ate d w ith re cu rr en t h er pe s si mp le x viru s (HS V ) i n fe cti on .

Table 11.6 Some precipitating factors in erythema multiforme In f ec tio n s

H erp es s im pl ex vi ra l (HS V ) i n fec tio n s M y co p la sm a pn e u mo n ia (ra rel y)

Dru gs

S u lf o n am id es

A n tic o n vu ls a n ts

Pre gn a n cy

Fig. 1 1 .1 1 Cru s ted le s io n s o f th e li ps i n ery th em a mu l tifo rm e.

Rec u rren t ery th em a mu lti fo rm e is a ss o ci a te d pa rtic ula rly w ith H S V in fe cti o n an d p ati en ts ma y r equ i re lo n g -te rm pr op h yl ac tic a c ic lo vi r.

Th e in i ti a l d ia gn o s is i s en tire ly c lin i ca l, th e im por ta n t d iff ere n tia l d ia gn o s is be in g fro m a

pri ma ry he rp etic s to ma titi s. In th e c as e o f a r ec u rren t a ttac k, h o we ve r, h erp eti c sto m ati tis

ma y b e co n fi de n tly exc l ud ed s in c e th i s is a n is o la ted e ven t i n im m un o c o mp ete n t in d ivi du a ls . Si mi la rly , a h is tor y o f rec u rre n t h erp es s im pl ex rend er s th e d ia gn o s is o f h e rpe tic s to ma titi s un l ike ly . Th e in vo l ve me nt o f th e li ps i s a s tro n g in d ic ati on o f th e di ag n o si s o f e ryth em a

mu lti fo rm e a n d th e p res e nc e o f β € ta rg et β€™ les i on s o f th e s ki n c an b e tak en a s al mo st

co n c lu s ive e vid en c e fo r th e di ag n o si s. B i o ps y o f the o ra l le si o n s sh o w s a ra the r n o n -sp ec if ic hi sto l o gic a l pic tu re w ith d eg en er ati ve ch a n ge s in th e ep ith el iu m a n d s u be pi th eli a l b u lla

fo rma tio n . B e ca u se o f th e a c ute c li n ic al fe atu re s, h o w ev er, th e di a gn o si s do e s n ot o ften

dep en d o n th e h is tol o gi ca l ap pe a ran c e o f th e le si o ns . O th er im mu n o bu l lo u s di se as e s ma y ne ed to b e exc lu d ed by im m un o f lu o res c en c e s tu di es .

Trea tm en t o f th e c as e s res tri cte d to th e m o uth dep en d s o n th e u s e o f l o ca l o r s ys tem ic

ste ro ids , to w hi c h th ere is u s u al ly a ra pi d re s po n se. A s tero i d m o u th wa s h is l ike ly to g ive sym p tom a tic re lie f an d ef fec tiv el y re ve rse th e pro ces s i n a few da ys . In th e mo re s ev ere ca se s (p arti cu l arl y wh e n o th er m u c ou s m em br an e s are i nv o lve d) , wh e n th e s ki n o r o ra l

les io n s a re se ve re o r w h en th e ey es a re af fec ted , a s h or t co u rs e o f s ys tem ic s ter oi ds m a y b e ne c es sa ry to s ho rte n th e atta ck . Th e us e o f sy ste mic s te ro id s fo r E M r em ai n s co n tro ve rsi a l. Ho w eve r, a c o ur se o f 40 mg pre dn i so l o ne d ai ly fo r 1β€“ 4 w ee ks , rap id ly red u c in g ov er th e ne xt f ew w e eks , ma y be be n ef ic ia l fo r s ym pto m ati c rel ie f. A to pi ca l o r s ys tem ic a n tifu n g al ag en t ma y al so be re qu ire d. Su c h pa tie n ts ma y be s uf fic i en tly il l to req ui re h o sp ita l

ad mi ss io n , p ar ti c ul ar ly if th ey be co m e deh y dra ted . P ati en ts w ith re cu rr en t E M req u ire l on g te rm pr op h yl ac tic a c ic lo vi r.

So m e au th o riti es h av e s u gg es ted th a t E M an d S tev en sβ€“ Jo hn s o n s yn dr om e ar e d is tin c t

cl in ic a l di so rd er s, o n th e ba s is o f the ir cl in i ca l fea tu res a n d sp ec if ic c au s es . H ow e ve r, o the rs

ha ve c h al le ng ed th is c o n c ept as s o m e p ati en ts w ith E M a n d mi ld sy mp to ms ma y su b se qu e n tl y dev el o p s e vere a ttac ks , n ec e ss ita tin g h o sp ita l ad m is si o n .

Idiopathic oral blood blisters (angina bullosa haemorrhagica)

So m e pa tien ts d ev elo p sp o n tan e o us b lo o d -fil le d b u llae ( β€ blo o d b lis ter s β€™) o f th e o ral

mu c o sa fr o m tim e to tim e. Th e se h a ve be en d es c rib ed u n de r th e ra th e r u n fo rtu n a te a n d

in ap pr op ri ate titl e o f β€ an g in a bu l lo s a h a em o rrh a gi ca β€™. T he u s u al p atter n is th at th e

pa tien t fee ls a s h arp p ric ki n g s e ns a tio n i n th e m o uth (m os t u s u al ly o n th e p a la te ) a n d fi nd s th a t a b lo o d-fil le d b li ste r h a s su d de n ly de vel o pe d. Th i s mo s t c o mm o n ly o cc u rs w h en th e

pa tien t is ea tin g . Th e bu ll a e m a y b e qu ite la rg e (up to 2β €“3 cm i n di a me ter) an d th e p a tien t

ma y b e in c o n si de ra ble f ea r o f o bs tru cti o n . Th e b lis te r e ve ntu a ll y ru p tu res o r is pe rfo ra ted b y th e p ati en t a n d h ea li n g o c c ur s un e ve ntf ul ly .

Th es e pa tien ts h a ve n o de mo n s tra ble a bn o rm al ity o f th e bl o o d-cl otti n g m ec h a n is m, a lth o u gh pa tien ts w ith th ro mb o cy top en i a ma y al so d ev el o p b loo d bl is ters . B o th m al e a n d fe ma le

pa tien ts h av e b ee n d es cri be d, o v er a wi de a ge ra n ge. Th e me tho d o f fo rm ati on o f th e bl is ters is n o t kn o w n . It is s pe c ul a te d tha t th e ba s ic m ec h an i sm i s o f b le ed in g fro m th e ca pi ll ary b ed bel o w a ba s al z o n e th a t i s fo r s o me re as o n w ea ke n ed. Th is ma y in de ed b e th e m ec h an i sm , bu t th e rea s on s f o r it are fa r fro m cl ea r.

Th e p a ti en t's h i sto ry is o fte n s u gge s tive o f the diag n o si s, b u t it is i mp o rtan t to e xc lu d e a n im mu n o bu l lo u s co n d itio n a n d al so to ca rry o u t a fu ll b lo o d co u n t a n d cl o tti n g sc ree n .

Pe rfo ra tio n o f a l ar ge, in tac t, b lis ter to re le as e the c o n ten ts ma y be n ec es s ary a n d an ti se pti c or an a lg es ic m o u th wa s h es c an be pre s cri be d. O fte n , h ow e ver , o nl y a ru ptu re d b u ll a is

pre se n ted fo r e xa mi n ati o n , the p ati en t h a vin g p erf or ate d i t a s a fi rst me as u re. N o pr eve n tive tr ea tme n t is kn o w n .

Connective tissue diseases

Th is is a c o m pe nd iu m ter m u se d to de sc ri be a n u mb er of d is ea se s w ith a s im il ar , bu t b y n o me an s id en ti ca l im mu n o lo g ic a l b ac kg ro u n d. T he y are n o t s kin di se as e s, b u t th ere a re sk in

les io n s in a n u m be r o f the m, an d it is c o mm o n pra c tic e to gro u p th em w ith th e s kin di se a se s fo r d es cr ip ti ve pu rp o se s . Th e gro u p in c lu d es S jΓ¶g re n' s sy nd ro m e a n d rh eu m ato i d a rth ri ti s ,

wh i ch d o n o t h a ve sp ec if ic s ki n le si o n s. T he s e a re di sc u s se d in Ch ap ters 8 an d 15 . A pa rt fr om th e se tw o c o n diti o n s, l u pu s er yth em ato s u s h as i mp o rtan t o ra l ma n if es tati o ns a s d o mi xe d co n n ec tiv e ti ss u e di se as e an d s ys tem ic s c ler os i s, al th o ug h th es e la s t two are re la tiv e rari tie s.

P. 137

Lupus erythematosus

Th e g ro u p of d is ea se s in c lu d ed u n de r th is h ea d in g pre se n ts w ith a w id e ran g e o f s ym pto ms , bu t a ll res u lt fro m ab n o rma l itie s o f th e co n n e cti ve tis su e s bro u g ht ab o u t by a n au to im m u ne

pro c es s. Tw o m ai n c lin i ca l en titi es a re rec o gn i z ed , al tho u g h th ere a re ma n y v ar ia ti o n s. Th es e are s ys tem ic l up u s ery the m ato s us ( SLE ) an d d is co i d l u pu s e ryth em ato s u s (DLE ).

Systemic lupus erythematosus

SLE te n ds to o c cu r in a d ul t li fe a n d fe ma le s ar e a ff ec ted m uc h m o re tha n m al es . In th is

co n di tio n , th ere a re w id es pre ad c h an g es i n th e c o n nec tive ti ss u es w ith se co n d ary e ffe cts i n

th e ca rd io va s cu l ar, mu s cu l o sk ele tal , a nd o th er s ys te m s, a s w el l a s in th e s ki n. Cu ta ne o u s LE cl as s ic al ly pre s en ts as a p h o tos e ns i ti ve er up tio n of th e f ac e (bu tte rfly -pa tte rn ) a n d h an d s. Th e c o u rs e o f th e d is e as e va rie s fro m a rel ati ve ly mi ld c hr on i c co n d itio n to a rap id ly fa tal

pro c es s, an d a n eq u al ly w id e ra n ge o f sk in r ea cti o ns m a y o c cu r. Th es e are p ara l lel ed b y a n equ a ll y di ve rse ra n ge o f or al s ym pto ms , th e mo s t c omm o n ly de sc ri be d b ei n g s u pe rfi ci a l

ero s io n s an d e ryth em a to u s p atc h es o n th e mu c o s a. It w o u ld s ee m ve ry u n li ke ly th at th e

in iti al di ag n o si s o f th e di se a se w o u ld be m ad e o n th e g ro u n ds o f or al le si o n s al on e , bu t th e po s si bi lity o f s u c h an ae tio lo g y f o r u n rec o g ni z ed ora l le si o n s sh o u ld b e b o rn e i n m in d. In pa rtic u la r, it sh o u ld b e r em em be red th at mo s t c li n ic al d es cri pti o ns o f th e or al le s io n s o f lu pu s va ri an ts re se mb le th o se o f l ic h en p la n u s. H isto lo gi c al ly, a ls o , th er e i s a cl o se

res em bl an c e be twe en th es e co n di tio n s . Th e fin a l diag no s is o f S LE is l ike ly to b e m a de a s a res u lt o f th e i mm u n e ab n or ma li ties p re se n tβ €”in p arti cu l ar, a wi de (a n d var ia bl e) ran g e o f

an tin u c le ar au to a n ti bo d ie s ma y be fo u n d in th e se rum. In SLE , c ir cu l atin g an ti bo d ies to DNA are a lm o st al wa ys p res e nt an d th is i s the m o st si gnif ic an t im mu n o lo g ic a l s c ree n in g tes t. If th e re are s ki n le si o n s, th e h is to lo gy a n d im mu n o fl uo res ce n t fi n di ng s o n bi o ps y are a s in dis c o id lu p u s eryth e ma to su s (be lo w ).

Ju st a s a li c he n o id rea c tio n m ay o c cu r as a re sp o n se to dr ug s , SLE ma y b e pre c ipi tate d in th e sa me w a y a n d by an eq ua l ly wi de ra n ge o f d ru gs . Hy dra ll az i n e, u s ed in th e m an a ge me n t o f

refr ac to ry h y pe rten s io n , is th e m o s t qu o ted e xa mp le, bu t o th er dr ug s in c lu d e b eta - blo c ke rs , an tic o n vu ls a n ts , an d q ui n id in e.

SLE m a y b e pre ci pi tate d b y dru g s. Trea tm en t o f SLE is es s en tia ll y wi th s te ro id s , of ten re qu i red i n h ig h do s es , a n d w i th th e

ad di ti o n o f s te ro id- sp ari n g dru g s su c h a s az a thi o pri n e. Th e o ral l es io n s m ay be b o th ver y

pa in fu l an d d iff ic u lt to tre a t. Hig h -co n c en tra tio n ste ro id β €“an tib io tic m o u th wa s h es m ay be us e fu l, to ge th er wi th s uc h s u pp o rtin g me a su re s as an al ge s ic m ou th w a sh e s.

Discoid lupus erythematosus (DLE)

Th is is a m u c h mo re re stri c te d fo rm o f th e di se a se , w h ic h pre s en ts as a s ki n di s or de r a nd wi th ou t th e w id es pr ea d ge n era li z ed a bn o rma l itie s fou n d in th e sy ste mi c fo rm . Th e sk in

les io n s , w h ic h res u lt fro m de ge n era tiv e c h a ng es i n th e s u b epi th el ia l c o n n ec tive ti ss u es ,

pre se n t as s c al y red pa tc h es th at la ter he al w ith sc a r fo rm a tio n . Th e fa ce is th e a re a mo s t co m mo n ly a ffe c te d, a n d ci rc u ms cr ib ed le si o n s oc c u r b ila ter al ly. A lo pe c ia c an o c c u r if th e

sc al p is i nv o lve d. F o ll ic u la r p lu g gi ng o f the s ki n is an im po rta n t c uta n eo u s fe atu re to e li ci t th e d ia gn o s is . Th e pa tie nts a re pre do m in a n tly fem a le (fe ma l e:m al e, 2: 1), th e a ge o f

in ci de n ce b ei ng w id el y di stri bu te d, b u t h av in g a peak in th e fo u rth d ec a de o f l if e. Th e fi rs t ap pe ara n c e o f th e l es io n s m ay fo l lo w s o me fo rm o f tra um a (s u ch as a n u n u su a l de gre e o f

exp o su re to s u n lig h t) a n d la ter e xa c erb ati o ns m a y fol lo w re pe a te d tra um a o f th i s kin d . Or al les io n s a re fo u n d in a c o n si de rab le p ro po rtio n o f pa tie n ts wi th DLE , al th o ug h th e es tim ate d

in ci de n ce va ri es ve ry w ide ly fro m 3 to 50 p er c e nt ac c o rdi n g to th e s o ur ce qu o te d. A lth o u gh les io n s m ay b e f o un d o n a n y pa rt o f th e o ra l mu c o s a, th e ch a ra cte ris tic s ite i s on the l ip s.

Th e l es io n sta rts a s an are a o f e ryth em a th at de velo ps to a th ic ke n ed , ra the r c ru s ted , le si o n wi th a w hi te ma rgi n . Th e h is tol o gi ca l ap pe a ran c es are o f e pi th eli a l atr op h y a t th e ce n tre o f th e le s io n w ith h y pe rke rato s is a t th e ma rgi n s w ith a cl o se re s em bl an c e to the c h an g es i n

lic h en pla n u s. Th e f u nd a me n ta l di ffe ren c e be tw een the h is to lo g ic al f in di n gs in li ch e n pl an u s an d in LE (an d o th er co n n e cti ve tis su e di s ea se s w ith s ki n an d m u co s al l es io n s ) is th a t th e su b epi th el ia l b a nd o f lym p ho c yte s, r ela tiv el y e ve nly d is tri bu ted i n li ch e n pl an u s , h as a

te n de n cy to a fo l lic u la r d is tri bu tio n i n DLE . Dire ct im mu n o fl u o res ce n ce i n LE g ive s va ria bl e

res u lts w ith h o m o gen e o u s or gr an u la r de po s its o f IgG, s o m etim es w i th IgM a n d co m pl em en t co m po n en ts , a t th e de rm o -epi de rm al ju n cti o n o r b el ow th e b as a l z o n e. C irc u la tin g

au to an ti bo d ies a re fo u n d in a pp ro xi ma tel y o n e -th i rd o f p ati en ts w ith s ki n le si o n s o f DLE . Trea tm en t o f DLE is o fte n s ym pto ma tic w i th the u s e of p o te n t to pi ca l ste ro id s to s up pr es s the les io n s . P ar en tera l trea tme n t, o dd ly en o u gh , i s wi th th e a n tim al a ria l d ru gs , s u ch as

hy dro x yc hl o ro qu i ne , w h ic h ma y co m pl ete ly su p pre s s th e s ym pto m s bu t w h ic h m ay a ls o

in tro du c e a w i de ra n ge o f s i de -eff ec ts, so m e mi n o r a n d s o me m o re se rio u s , fo r exa m pl e, th e pro d uc tio n o f c o rn ea l de po s its a n d r etin o p ath y. T h e m o s t si gn i fic a n t co n s id era tio n , s o fa r a s

th e o ra l le si o n s are c o n ce rn ed , is th e po s si bi li ty o f ma li gn a n t c ha n ge . It is d if fic u lt to a ss es s th e in c id en c e o f th is f ro m the p u bli s he d fig u res , bu t th ere is n o do u b t th at c a se s in vo l vi ng

ma li gn a nt tran s fo rm a ti o n in lip l es io n s h av e bee n do c u me n ted . It is , th ere fo re , n ec es sa ry to ob s erve th e le si o n s on a lo n g-te rm ba s is .

Morphoea and systemic sclerosis

Mo rp h o ea is a p u rel y c u tan e o u s d is e as e, i n w hi c h there is a s po n ta n eo u s ap pe a ran c e o f a sc ar -lik e b a nd o r pl aq u e. S ys tem ic sc l ero s is i s a mu lti sy ste m di se as e in w h i ch th e re is

wi de sp rea d fi bro s is o f th e sk in a n d gu t, to ge th er wi th o th er o rg a ns . Th e re ma y al so be o th er ele me n ts o f co n n e cti ve tis su e di s ea se p res en t, s u ch as S LE a n d oc c as io n a ll y S j Γ¶g re n' s

syn d ro me (s e e Ch ap ter 8). Fe m al es a re mo re c o mm o n ly af fe cte d a n d th e e ar lie s t fe atu re is us u a lly Ra yn a u d's p h en o me n o n . Inv o lve me n t o f th e perio ra l tis su e s ca n l ea d to res tri cte d

mo u th o pe n in g an d d iff ic u ltie s w ith o ra l hy gi en e and d en tal tre atm en t. P a tien ts m ay d eve lo p an e xp res s io n le ss β € ma sk -lik e β€™ fa ci al ex pre s si o n.

P. 138 In sy ste mi c s cl ero s is th er e m a y b e res tric ted m o uth op en i n g d u e to in vo l vem en t of th e per io ra l ti ss u es .

Wid en in g o f th e p eri o do n tal me mb ra n e s p ac e, p a rtic ula rly in p o s te rio r tee th is th e

ch a rac te ris tic ra di ol o gi ca l de n ta l fi nd in g . A va rian t o f sy ste mi c sc le ro s is h a s bee n n a me d th e CRS T o r CRE S T s yn d ro me (C , ca lc i fic a ti o n ; R, R ay na ud' s ph en o m en o n ; E , o es o ph a ge al

dys fu n c tio n ; S , s cl ero d a ctyl y; T, tel an g ie cta s ia ). M os t p a ti en ts w ith s ys tem ic s c ler os i s ha ve hi gh ti tres o f an ti n uc le a r a n ti bo d y ( AN A), us u a lly o f th e s pe ck led v ari ety, al tho u g h o the r ty pe s ca n a ls o b e f o un d . Tre atm en t o f sy ste mi c s cl ero s is i s es se n tia ll y s ym pto m ati c.

Wid en in g o f th e p eri o do n tal m em bra n e sp a ce is a c h arac teri s ti c ra di ol o gi ca l fea tu re o f sc le ro de rma .

Mixed connective tissue disease

Mi xed c o n n ec tive tis su e d is ea s e i s an ov erl ap c o n diti o n in wh i ch a n u m be r o f th e

ch a rac te ris tic s o f o th er di s ea se s in th e gr ou p a re fo u n d i n a s in gl e pa tie nt. P a tie nts a re

pre do m in a ntl y f em al e an d m ay h av e f ea tu res o f S LE, s ys te mi c sc le ro si s , de rm ato m yo si tis ,

an d po l ym yo si tis . It i s a r are c o n di ti o n , bu t o f inter es t in th e p res e nt co n tex t s in c e th e re are

a n u m be r o f o ral d ia gn o s tic in d ic ato rs o f th e d is eas e. T he f irs t is a β € lic h en pla n u s -lik e β€™

les io n of th e o ral m u co s a, bu t w i th a h is tol o gi ca l ap pe ar an c e res em bl in g th at of LE . Th e

se co n d is th e in vo l ve me nt o f th e trig em in a l n e rve in the n e ur ol o gi ca l ch a n ge s tha t m a y o c cu r

in m ixe d co n n ec tiv e ti s su e di se a se . Th is m a y l ea d to a co m pl ai n t o f f ac ia l an a es th es ia , d u e to a tr ige m in al n e ur op a th y . In ad di tio n , m ixe d co n n ec tiv e tis su e d is ea s e m a y b e a s so c ia ted w i th se co n d ary S jΓ¶g re n' s sy nd ro m e ( Ch ap ter 8). Th e mo s t im p or ta n t i mm u n ol o gi ca l in d ic ato r in th i s co n d itio n is th e pre se n ce o f an tin u c le ar an ti bo d ies o f th e s p ec kl ed typ e a n d h ig h le vel s of a s pe ci fic a n tib o dy to RNA a se -se n si ti ve , ex trac ta ble n u c le ar rib o n uc l eo pr ote in ( RNP ) an tig en .


Wh at i s th e m o s t li kel y dia g no s is o f th is l ad y's o ra l les i on s ?

Q2

Are h e r s kin sy mp tom s rel ate d?

Q3

Wh at in ve s ti ga tio n s w o u ld yo u c a rry ou t?

Q4

Wh at a d vic e an d tre atm en t w o u ld yo u giv e to thi s la dy ?

Th e m o s t li kel y d ia g no s is o f th is la dy 's o ra l c o n ditio n is l ic h en p la n u s in i ts n on -ero s ive ,

retic u la r f o rm. Sh e sh o u l d b e sp ec if ic al ly a sk ed abo u t m edi c ati on , a s li c he n o id eru p tio n s c an be dru g -in du c ed , a lth o ug h th es e ma y be u n il ate ral . F em al e pa tien ts m ay b e r elu c ta nt to

rep or t ge n ita l i n vo lv em en t, if p res en t. Th e fl ex or s ur fa ce s o f th e w ris ts are cl a ss ic a l s ite s fo r ski n l ic he n p la n us . In c is io n a l b io p sy o f th e o ra l mu co s a is ad vi sa b le to c on f irm th e cl in ic a l

dia g no s is . Th i s la dy s ho u l d b e rea s su re d ab o ut th e d ia gn o s is b u t ad vi se d ab o u t th e n e ed fo r

lo n g-te rm fo l lo w -up . Th e o ra l le si o n s are a sy mp to matic a n d, th e refo r e, n o tre atm en t i s req ui red . A to pi c al ste ro id p rep ar atio n ca n b e p resc rib ed fo r h er sk in .


Ho w w o ul d yo u in v es tig ate a nd m a na g e th is g en tl eman ?

Th e h i sto ry a nd c li n ic al e xa mi na tio n a re s u gg es tive o f an i m mu n o bu l lo u s co n d itio n wi th

in vo lv em en t o f th e s ki n a n d o ra l mu c o sa . O ra l b io p sy , w ith d ire ct im mu n o fl u o res ce n ce o f per ile s io n al tis s u e s h o u ld be a rra n ge d a n d bl o od take n fo r in d ire ct im mu n o flu o re s ce n ce . Bi o ps y o f th e or al m uc o s a ma y be di ffi cu l t be c au s e o f th e ex trem e ti s su e fra gi li ty a n d exte n si ve in vo l vem e nt of th e o ral m u co u s me mb ra n e.

Pe mp h ig us v u lg ari s is th e mo s t l ike ly di ag n o si s in th is c as e an d th ere i s a hi gh e r i nc i de nc e am o n gs t th e J ew is h ra c e. Th e p ati en t's ag e an d h is to ry of o ra l les i on s , p rec ed in g

in vo lv em en t o f th e s ki n , to ge th e r w ith a p os i ti ve Ni ko ls ky s ign are m o re su g ge sti ve o f

pem p hi gu s th a n bu l lo u s pe mp h ig o id. Mu c o u s me mb ra ne p em ph i go id te nd s to a ffe ct an o l de r ag e g ro u p, pa rtic u la rl y w o m en , a nd d o es n o t c o mm o n ly in vo l ve the s ki n . Th e pro tra cte d hi sto ry o f or al u lc er atio n an d c lin i ca l ap pe a ran c e o f s ki n le si o ns i s n o t co n s is ten t w i th

eryth e ma m u ltif o rme , w h ic h ten d s to be ep is o di c a nd m an i fe st a s tar get le si o ns o n th e s kin . Th e c li n ic a l d ia gn o s is o f pe mp h ig u s vu lg ari s is c o nfirm e d b y th e p res en c e o f a u toa n tib o di es dir ec ted ag a in s t ep id erm a l i n te rc el lu la r s u bs ta n ce o f th e e pith e li um . Ro u tin e h is to lo g y in

pem p hi gu s re ve al s bu ll o u s fo rm ati on wi th a ca n th ol ysis . P o si tive d ire ct IM F w ill d em o n stra te

in terc el lu l ar de po s itio n of IgG a nd u s u al ly C3 i n th e ep ith el iu m (th e so -ca ll ed β€ fis h n et β€™

or β€ ch ic ke n w ire β€™ ap pe ara n c e). Ur gen t tr ea tme n t is requ i red a n d th e pa tie n t is

ma n ag ed in co n ju n cti o n w ith a de rm ato lo g is t. A dm is si o n to h o s pita l is u s u al ly req u ire d.

In i tia ll y h ig h d o se s o f s ter oi ds a re gi ve n, wi th the l ate r a dd iti o n o f a s tero i d -sp ari n g dru g , if ne c es sa ry. An tif u ng a l m ed ic a tio n i s us u a lly re qu ir ed . An a lg es ic m o u thw a s he s , co n ta in i ng

lig n o ca in e , pro v id e s ym pto m ati c rel ie f a n d he lp th e pa tie n t ma in ta in fl ui d an d n u tritio n a l

in tak e. S ki ll ed n u rsi n g c a re is e ss en ti al if th ere i s w id es pre ad s ki n in vo l ve me nt. A tte nti o n

sh o u ld b e p ai d to lo n g- te rm m ai nte n an c e o f o ra l h ygi en e . Den ta l tre a tm e nt is o fte n di ffi cu l t.


Wh at a re th e or od en ta l ma n ife s tatio n s o f th is c ond iti on ? Dis c us s th e di ffi cu l ti es y o u mi gh t e nc o u n ter wh e n pr ov id in g de n ta l trea tme n t fo r thi s bo y .

Ora l mu c o sa l in vo l ve me nt o cc u rs in th e d ys tr op h ic an d jun c tio n a l ty pe s o f e pi de rmo l ys is bu ll o sa (E B ). In c h il dre n w ith th e ju n cti o na l typ e, th ere a re o ften se ve re de n tal

ab n or ma li ties . H ypo p la sti c tee th h av e b ee n de s cri bed in p ati en ts w ith dy stro p h ic E B . M in im al tr au m a fro m to o th b ru s hi n g a n d ea tin g c au s es o ra l bull ae an d s ca rri n g, a s do e s su c kl in g in ne o n ate s. A s a re su l t o f r epe a ted sc a rrin g , mo u th op en in g b ec o me s res tric ted a n d th e

to n g u e a n d lip s m ay be co m e im mo b il e. C rac ki n g a t the c or ne rs o f th e m o uth freq u en tly oc c u rs.

Ora l h ygi en e is d if fic u lt f o r th es e ch i ld ren a n d ina de qu a te p la qu e co n tro l , su p eri mp o se d o n def ec tiv e te eth a n d a res tric ted d ie t o ften res u lts in ra mp an t c a rie s. Ph ys i ca l ac c es s fo r

den ta l trea tme n t be co m es d if fic u lt be ca u se o f s c arrin g. Du e to e xc es s ive tis s u e f rag il ity aff ec tin g a ll mu c o u s me mb ra ne s , lo c al a n d gen e ra l an ae s the s ia a re pro b lem a tic al a n d

extra c tio n s mu s t b e c a rrie d o u t w ith e xtrem e ca re . P are n ts of a ffe c te d ch i ld ren n e ed to b e

giv en p rev en tiv e ad vi ce c o n ce rn in g di et, o ra l hy gi en e , an d fl u or ide s u pp le me n tatio n be fo re th e de ci du o u s tee th eru p t. Re gu la r d en ta l revi ew s an d e m pa the tic s u pp o rt fo r pa ren ts ar e es se n tia l. M o st ca s es a re trea ted b y a s pe ci al is t pa ed o do n tis t.

Project

P. 139

1.

Fi nd o u t a bo u t th e s ho rtan d lo n g -te rm ef fe cts of sy ste mi c ste ro id s an d h o w th es e ma y

be pre ve n te d an d /o r ma n a ge d. Di sc u ss th e im p lic a tion s o f p ro vi din g o ra l an d de n tal ca re fo r pa tie n ts wh o a re ta kin g s ys tem ic ste ro id s.

Au th o rs : Title:



Co py rig ht Β © 2003 Ox fo rd U ni ve rsi ty P re ss > T ab le o f Co nt e nt s > 1 2 - Ga st r oint e s t inal d is e as e

12

Gastrointestinal disease Problem case Case 12.1

A 26-yea r-ol d fem a le pa tie n t vi si ts yo u r de n tal pr ac tic e fo r th e fi rst tim e s in c e mo v in g in to th e lo c a li ty . S he c o mp la in s o f s o ren e ss o f th e mo u th a n d u l ce ra ti o n . Her m ed ic al h is to ry reve a ls th at sh e h as b ee n di ag n o se d by h er ge ne ra l p ra cti tio n er (GP ) a s s uf fe rin g fro m

irri tab le bo w el s yn d ro me (IB S ), fo r wh i ch sh e tak es a n tis pa s mo di c s, to n o eff ec t. S he h a s

rec en tly n o tic ed i nc re as in g b ou ts o f bl o od y dia rrh oe a an d s to m a ch p a in . Yo u n o te th at s h e is pa le a nd h a s ce rvi ca l lym p ha d en o pa th y. Th e a n gle s of th e m o uth a re cr ac ke d. T he re is

ma rke d i rre gu la ri ty o f bo th b u cc a l m u co s a e w i th as so ci ate d li n ea r u lc e rati o n. T h ere a re no si gn s o f a p hth o u s s tom a titis b u t yo u n o te sm a ll tissu e ta gs i n th e re tro mo l ar reg io n . Th e

pa tien t is ex trem el y a n xi o us a n d h as n o en e rgy a t wo rk. He r a pp eti te i s po o r an d s he h a s

lo st o ver a sto n e in we ig h t o ver th e la st c o u pl e of m o nth s . He r GP h a s to ld h er th at h e r g u t

pro b lem s a re str es s -rel ate d a n d pre sc ri be d s o m e v ita mi n s. T he l ad y w a n ts to kn o w w h y s h e ha s rec e ntl y d ev el op ed u l ce rati o n o f h e r m o uth . Q1

Co u ld th is la d y's o ra l c o n di tio n b e r el ate d to h er g u t sy mp to ms ?

Q2

Wh at a re th e sy mp to ms o f IB S?

Q3

Ho w w o ul d yo u m an a ge th is l ad y?

Introduction

Ora l le si o ns m a y o c c ur in a n u m be r o f ga s tro in tes tin a l tr ac t d is ea se s . In a fe w, th e se a re

pri ma ry or al le s io n s res e mb li ng th o s e o f th e l o we r g u t, bu t, i n al mo s t a ll , se c on d ar y l es io n s ma y b e in d uc e d b y fa cto rs s u ch a s m al ab s or ptio n . Not on l y th e di s ea se p ro ce ss , b u t al s o su rg ic al re se c ti o n o f p a rts o f th e gu t m a y re su l t in th e se s ec o n da ry ch a n ge s.

So m e dis e as es o f th e g a stro i nte s ti n al tra ct are o f pa rtic u la r s i gn if ic an c e in th e fi el d o f o ra l me dic i ne a n d are d is cu s s ed be lo w .

Coeliac disease (gluten-sensitive enteropathy)

Co el ia c di se as e is a p erm a ne n t in to le ra nc e to gl ia di n β€” th e p ro te in co m po n en t of w h ea t. It is a l if el on g in fl am m ato ry c on d iti on o f th e ga s tr oi n tes tin a l tra c t th at af fec ts th e s m al l in tes tin e in g en eti ca ll y su s ce pti bl e i n di vid u al s. Co el ia c di se as e is ch a ra cte riz e d by ma la bs o rp tio n d ue

to mo rp h ol o gi ca l ab n o rma li tie s in th e s ma ll in tes tin a l mu c o sa . Th e se c h an g es a re rev ers ib le on wi th dra w al o f gl ute n fro m th e di et. Th e fi rs t detai le d d es c rip tio n o f co e li ac d is ea s e i n

ch il dr en w a s gi ven i n 1887. Cl as s ic al ly, co e lia c d is ea se i s ch a ra cte riz ed b y th e de ve lo p me nt of di ar rh oe a , lo s s of a pp eti te , a n d w a sti n g w h e n a w ea n in g di et c o n tai n in g gl ute n is

in tro du c ed , to ge th e r w ith se co n d ary e ffe cts o f ma lab so rp tio n s u ch a s a n ae mi a. Co el ia c dis e as e is a ls o kn o w n a s β€ co e lia c s pru e β€™ an d it is in te res tin g th at th e n a me

β€ sp ru e β€™ wa s d eri ved fr om th e Du tch wo rd f or a ph th ous u l ce r, th is in d ic ati n g th e h ig h

pro p or ti o n o f s u ff ere rs w ith o ra l ul c era tio n . Co el ia c di se a se is a p erm a n en t i nto l era n ce to g li adin . It w as n o t u n til th e mi dd le o f th e tw en tie th ce n tu ry th a t th e of fe nd in g d ieta ry pro d u ct in co e lia c d is ea se w a s fo u n d to b e w he a t fl ou r. D am ag e to th e i n tes tin a l mu c o s a i s c au s ed

pre do m in a ntl y b y th e g lia d in fr ac tio n o f gl u ten . Glute n is pre s en t i n w he a t, rye, an d ba rl ey (an d po s s ib ly o ats ).

In ad u lt co e li ac d is ea s e, d ia rro h ea , w ei gh t l o ss , an d w e ak n es s are th e cl a ss ic s ig n s an d sym p tom s .

Si n ce th e 1980s i t h a s be co m e a p pa ren t th at th e c lini ca l pa ttern o f c o el ia c di se as e is

ch a n gin g a n d th a t i t is fre qu e ntl y u n d erd ia gn o s ed . It is b ec o mi n g in cr ea si n gl y a pp rec i ate d th a t i t ma y be co m e ap pa re nt o r b e d ia gn o s ed i n ch i ld ren o r ad u lts lo n g a fter th e i n itia l in tro du c tio n o f gl ute n in to th e di et. In th e la te 1990s thi s le d to the c o n ce pt o f th e

β€ co e lia c ic e be rg β€™ (Fi g. 12. 1 ). A t th e ti p a re th ose w ith c li n ic al ly o ve rt di se a se fo l lo w ed

by, i n o rd er, s il en t c o el ia c di se a se , la ten t c o el iac di se a se , an d h ea lth y in d ivi du a ls w h o m ay ha ve th e su s c ep tibi li ty g en e s fo r th e di se as e .

Th e i n ci de n ce o f bio p sy -pro ve n c o eli a c d is a se i n the U K i s 1 in 2000, wh e rea s th e in c ide n ce wh e n sc re en i ng fo r th e d is ea s e us i ng s u ch ma rke rs as a n tien do m ys ia l an tib o di es ac tu a lly ap pro a ch e s 1:300. Th is l a tte r g ro u p o f c o eli ac p a tien ts m ay b e th e po p u la tio n th at ge ts

ap h th o u s u lc er s, d es pi te no t ha vi n g a n y o f th e mo re ge n era li z ed ga s tro in tes tin a l sy mp tom s (se e Ch ap ter 5). A pp ro xim a tely 5β €“10 pe r c en t o f pa tie n ts wi th c o eli ac d is e as e ha ve a n aff ec ted fi rst -deg re e re la tiv e. Th e re is a ls o a re co g n iz ed

as so c ia tio n of c o eli a c dis e as e w ith o th er au to im m u ne d is ea s es s u ch a s in s u li n - dep en d en t

P. 144

dia b etes m el li tu s. A pp ro xim a tely 5β €“10 pe r c en t o f pe o pl e w i th th is s o rt o f d ia be tes w i ll al so ha ve c o el ia c di se as e.

Fig. 1 2 .1 Th e co e li ac i ce be rg an d sp ec tru m o f g lu te n se n si tiv ity.

Defi n itiv e d ia g no s is o f c oe li ac d is ea s e req ui re s the d em o ns tra tio n o f ch a ra cte ris tic m u c os a l

ab n or ma li ties i n b io ps ie s o f th e s ma ll b ow e l ( us u a lly o bta in e d v ia e nd o sc o py ). Th e ea rl y

les io n co m pri se s in c rea s ed ly mp h o cy tic in fi ltra tio n i n to th e e pi the li u m an d th en th e la m in a

pro p ria . Cry pt h yp er pl as ia o cc u rs n ex t a nd i s fo ll owe d by th e a p pea ra n ce o f vil lo u s a tro ph y.

Th e a c tua l ap pe a ran c e o f to ta l o r su b tota l vi llo u s a tro ph y is n o t vi ll o us a tro ph y a t al l bu t i s, in fa c t, du e to c ryp t h yp erp la si a fil li n g i n th e spac e s betw e en th e vi lli (s ee Fi g. 12. 2 ). IgA

an ti- gli ad in , Ig A an ti -retic u li n , an d IgA en do m ys ial au to a n ti bo d ie s are b lo o d tes ts tha t c a n be us e d a s a fi rst- lin e in v es tig ati on in th o s e p eo p le s us p ec ted o f h a vi n g c o el ia c di se a se o r a s a sc ree n in g to o l. Ho w e ver, by de fin i tio n , th e f in a l dia gn o s is res ts o n th e mi cro s c o pic

ap pe ara n c e o f th e s m al l bo w el bi o ps y. T ho s e pa tie n ts i n w ho m th e bl o o d te sts a re po s iti ve bu t th e bi op s y i s es se n tia ll y n o rm al a re de fi ne d as h a vi ng l ate n t co e li ac d is ea s e. It i s

un c erta in wh a t p ro po rti on o f th es e wi ll pr og re ss to m ee t th e fu ll di a gn o sti c cr iteri a o f c o el ia c dis e as e.

Fig. 1 2 .2 Hi sto lo g ic a l a pp ea ra n ce o f vi llo u s a tro pl y (je ju n um) in a p ati en t w ith co e lia c

dis e as e.

Sm a ll bo w el b io p sy s am pl in g is e ss en ti al fo r the d ia gn o s is fo r co e li ac d is ea s e. Dia gn o s is sh o u ld n o t be b as e d o n s ym p to m s o r s er ol o gi ca l tes ts al o n e.

Pa tie n ts w i th co e li ac d is ea s e w i ll u n de rgo cl in i ca l rem is s io n o n a g lu ten -free d ie t bu t th e y ma y i n itia l ly ne ed i ro n an d fo la te su p pl em en ts to co rrec t m ic ro c ytic /m ac ro c ytic a n a em ia s ca u se d by ma la b so rp tio n . Der ma titi s he rpe tif or mi s, a s ki n di s or de r c h ara c teri ze d by

bli s te rin g , (s ee Ch ap ter 11), is th e cl as s ic a l n o n-ga stro i n te s ti n al m an i fes ta tio n o f co e li ac dis e as e, a n d bo th th e in tes tin a l le si o n a nd ra s h res p on d to a g lu ten -free d ie t. Derm ati tis

he rpe tif or mi s is m o re pre va le n t in th e a du lt po p ul ati o n w ith c o el ia c di se as e a nd i s ha rd ly eve r s ee n in c h il dre n .

Derm ati tis h erp eti fo rm is i s th e c la s si ca l n on - ga stro i n te s ti n al ma n if es tati o n o f c o el ia c dis e as e.

Oral manifestations of coeliac disease

Th e as s o ci ati o n o f re c ur ren t a ph th o u s sto m a ti tis (RAS ) wi th c oe li ac d is ea s e is w el l

es tab li sh e d b u t in i tia l s tu di es o f thi s rel a ti o n sh ip i mp li ed th at the p ro po rti on o f R AS -aff ec ted in di vid u al s w ith c o el ia c di se as e w as o f th e o rd er of 25 pe r ce n t. Rec e nt stu d ie s in di ca te tha t

th i s fig u re is m u ch l o w er a n d li kel y to be le ss th an 5 p er ce n t. Ad u lts w ith u n di a gn o se d co e li ac d is ea s e m a y p res e nt wi th re cu rre n t ap h th ou s s to ma titi s. Ma la bs o rp tio n , as a r es u lt o f co e li ac d is ea s e, m a y re su l t in a h a em a tin ic d ef ic ie nc y,

pa rtic u la rly o f i ro n a n d fo li c a ci d. P a ti en ts w ith c o el ia c di s ea se m ay th ere fo re pr es en t w i th

an g ul ar ch e il itis , g lo s si tis , o r RA S . It i s, th e refo re , e ss en ti al to u n de rtak e a f u ll bl o o d c o u n t an d h ae ma tin i c as s ay fo r pa tie nts p res e nti n g w i th the s e o ra l co n d itio n s . S ero l o gic a l te s ti n g fo r a n tibo d ie s is i nd ic a ted if th ere a re gu t sy mp toms s u gg es tiv e o f c oe li ac d is ea s e or

evi de n ce o f ma la bs o rp ti o n . Wh eth er o r n o t se ro lo g ica l te sti ng s h o u ld be re co m me n de d fo r

pa tien ts w ith RAS , i n th e ab s en c e o f th es e fea tu res, i s a ma tter fo r d eb ate b ut in s o me c as e s ap h th o u s u lc ers m a y b e th e o n ly m an if es tati o n o f the di s ea se . Th e se n si tivi ty a n d sp ec i fic ity of th es e tes ts are n o t, h o w eve r, 100 p er ce n t a nd sma l l b o w el bi o ps y is re qu i red fo r a

dia g no s is o f c oe li ac d is ea s e. In te res tin gl y, d en tists m ay be al erte d to th e p o ss ib il ity o f

co e lia c d is ea se b y f in di n g d en ta l en a me l d ef ec ts (th at is , h ypo p la s ia ) o n th e pe rma n en t te eth . Th e p res en c e o f d en ta l e n am el h ypo p la s ia is th o u gh t to in di c ate th at th e c o eli a c

dis e as e h as b ee n pre s en t a t le as t i n th e f irs t 2 y ea rs o f lif e e ve n th o ug h i t mi gh t h a ve be en cl in ic a lly si le n t.

Den tis ts ma y su s pe ct co e li ac d is ea s e b ec a u se o f e n am e l d ef ec ts on pe rm an e nt te eth β €”pa rtic u la rly lo w e r in c is o rs .

Inflammatory bowel disease (IBD)

Th e i n fla m ma to ry bo w el d is ea s es a re lif el o ng c o n di ti o n s res u lti ng f ro m a b erra n t i nf la mm a tio n of th e m u c os a l l in i ng o f th e

P. 145

ga stro i n te s ti n al tra c t. Th e tw o ma in ca teg o rie s are C ro hn ' s dis e as e, w h i ch m a y a ff ec t th e g u t an yw h er e f ro m mo u th to a n u s, an d u lc era tiv e c o li tis, w h ic h is p red o mi n an tl y w ith i n th e co lo n .

Oral manifestat io ns of co eliac d isease Ora l u lc era tio n β€” RAS Glo s si tis An g ul a r c h eil iti s En a me l h ypo p la si a

Ora l in vo l vem en t ha s fo r so m e tim e b ee n re co g ni z ed in pa tie nts s u ffe ri ng fro m b oth Cro h n 's dis e as e (CD) an d , to a le ss e r ex ten t, u lc er ati ve col itis ( UC) . Or al le s io n s ma y pre ce de o r ac co m pa n y ga stro i n tes tin a l d is ea s e a n d ca n b e th e on ly si te o f in v ol ve me n t.

Cro h n 's di se a se a nd u l ce ra ti ve c o liti s are th e tw o ma jo r f o rms o f n o n- sp ec if ic in fl am m ato ry bo w el di se a se (IB D).

Crohn's disease

Cro h n 's di se as e w as f irs t d es cr ibe d in 1932 as re gio n al il ei tis , th at i s, in fl am ma tio n o f th e ile u m. T he o ri gi na l pa pe r i n cl u de d a d es c rip tio n o f mu c o sa l in fl am m ati on an d o c ca s io n al

ul ce ra tio n o f th e a ffe cte d g u t, ly mp h n o de h yp erp lasi a le ad in g to o bs tru c tive o ed em a , an d

th e p ro du c tio n o f gra n u lo ma to u s le si o ns . S h o rtly a fter the f irs t de s cri pti o n o f th e d is ea s e, i t bec a me c le ar th at i t w a s no t of n ec e ss ity c on f in ed to th e il eu m , an d l es io n s h av e s i nc e be en des c rib ed th ro u gh o u t th e wh o l e o f th e ga s tro in tes tin a l tr ac t. Th e cl in i ca l pro g res s o f th e

dis e as e is ve ry va ria bl e w ith i na c tive p h as es . Ho w ever , in th e mo re a gg res s ive s ta ges o f th e dis e as e, th ere m ay be a bd o mi n al pa i n, d ia rrh o e a, a nd ma la is e an d i nf la mm a tio n o f the

aff ec ted g ut ma y pro g res s to th e pro d uc tio n o f fi stu l ae . Th ere m a y b e ar thr op a th i es a n d sk in

gra n u lo ma s a ss o c ia ted w ith th e di se as e , as w e ll as pro b le ms a ri si ng f ro m ma la bs o rp ti o n . Th e ae tio lo g y o f th e d is ea s e is n o t kn o w n an d th e ep o nymo u s d es cr ipti o n h as , th ere fo re , be en ma in ta in ed . Tre atm en t is me di ca l , us i ng s tero i ds , aza th io p rin e (a n d o th e r

im mu n o su p pre s si ve ag en ts ), am in o s a lic yl ate s , di eta ry tre atm en t stra teg ie s, a n d rep la c em en t th e ra py to co r rec t m al ab s or ptio n . Di eta ry i n terv en tio n a lo n e h as b ee n d em on s tra ted to be

eff ec tive a n d is es p ec ia ll y f av ou re d in c h il dre n a s it m ay a vo id th e u se o f ste ro id s. In so m e ca se s w h ere th e dis e as e is re si sta n t to m ed ic a l th erap y, su rg er y i s ne c es sa ry .

Table 12.1 Clinical features of orofacial granulomatosisβ€”oral Crohn's disease Sw e lli n g o f li ps a n d fa ce *

Mu c o sa l tag s o r β € co b ble s ton i n g β€™ Ora l u lc era tio n ( RA S an d n o n -RAS ) β€ An g ul a r ch e il itis Lip fis s u res

Pe rs is ten t ly mp h ad en o p ath y

Pe rio ra l ery th em a an d s ca li n g o f s kin β€ Fu ll -wi dth β €™ gi n gi viti s

*F i ss u red to n gu e (l in gu a p li ca ta) a nd f ac ia l pa ls y ar e o th e r m an i fes ta ti o n s of th e Me lke rs o n β€“Ro se n tha l sy n dro m e.

β€ RAS , R ec u rren t a p hth o u s sto m a ti tis .

Oral Crohn's disease

Ora l le si o ns , i n pa tie n ts su f fer in g fro m CD, w h ic h h i sto lo g ic a lly re se mb le d tho s e el se w he re in th e g ut w ere fi rs t de sc ri be d i n 1969. Th e fi rst ca ses i n wh i ch o ral le s io n s o f C D w ere

des c rib ed i n th e a bs e nc e o f g a stro i nte sti n al le s io ns w ere re po rted i n 1973. Th e o ro fa ci al fea tu res o f Cro h n 's d is ea se a re de sc ri be d b el o w an d s u mm a riz ed i n Tab le 12. 1.

Orofacial granulomatosis

Ove r th e la s t de c ad e, th er e h a s be en in cr ea si n g atte n tio n p ai d to no n -in fe cti ou s

gra n u lo ma to u s di so rd ers o f th e o r of ac ia l reg io n , w hic h i nc lu d e: o ral C ro hn 's d is e as e an d o ra l sa rc o id, as w el l as cl in i ca l en titi es , kn o w n a s th e β€ Me lke rs so n β€“ Ro se n tha l sy n dro m eβ €™

an d β€ Mi es ch e n er's c h ei li tis g ran u lo m a tos a β€™ (gra n u lo m ato u s c he il iti s).

Th e te rm β€ or of ac ia l gra n u lo m ato s is β€™ (OF G) w as i ntr od u ce d to en c o mp as s th es e

dis o rd ers a n d to de sc rib e a cl in i ca l sy nd ro m e p res enti n g w i th sw e ll in g o f th e fa ce , li ps , o r or al tis s ue s in a s so c ia tio n wi th h is to lo gi ca l ev id en c e o f n o nc a se a ti n g gra n u lo ma to u s in fl am ma tio n w i thi n th es e tis su e s.

Clinical manifestations of orofacial granulomatosisβ€”oral Crohn's disease (Table 12.1)

Oro fa c ia l sw e lli n g, p ar ti c ul a rly in vo l vin g th e li ps, is th e mo s t c o n si ste n t fe atu re o f O F G a n d th e m o st c o m mo n rea s o n fo r p a tien ts p res en ti ng f or in v es tiga tio n an d trea tm en t. Th e

sw el li n g o f th e l ip s is p ai n les s , h as a f irm β € rub be ry β€™ co n s is ten c y; a n d ca n i n vo lv e b o th

up pe r a n d lo w er lip s , in d ivi du a ll y o r to g eth e r. It c an b e u ni la tera l o r bi la tera l. T h e l ip s ca n

be mi ld ly sw o l len o r gr o ss ly en l arg ed l ea di n g to s ever e co s m etic d is fi gu re me n t ( Fi g. 12. 3).

Th e f lo o r o f th e m o u th is o c ca s io n a lly in v o lve d. F aci al s w el lin g c an af fec t o n e o r b oth si de s o f th e fa ce a n d ma y i n vo lv e th e p eri or bi ta l tis su e s and c h in .

Lip fis s u res a n d an g ul a r c h eil iti s are c o mm o n ly se en i n OF G. Pa tie n ts o f ten g ive a h is to ry o f re c ur ren t o r pe rs is ten t s w el lin g o f th e s u bm an d ib u la r o f

ce rvi ca l no d es . P ers is te nt ver ti c al fi ss u rin g , ch a pp in g o f th e l ips , a n d an g ul ar c he il iti s a re

al so s e en i n OF G. Th er e m a y a ls o b e p er si ste n t ery the m a a n d sc a li n g o f th e p eri o ra l ti ss u es . Th e bu c c al m uc o s a is th ic ke ne d an d fo l de d, g iv in g a co rru g ate d or c ha ra c te ri stic

β€ co b ble s ton e β €™ ap pe ara n c e ( Fi g. 12. 4). Mu c o s al ta gs m ay be p res en t, p arti cu l ar ly in th e retro m o la r re gi on s .

Pa tie n ts ma y pre se n t w ith re c ur ren t a ph th o u s s to m a ti tis (R AS ), b u t th is c o n diti o n is co m m on in th e po p ul ati o n ge n era ll y

an d n o t sp ec i fic to O F G (s e e Ch ap ter 5). Pe rs is ten t l in ea r ul c ers (n o n -RAS ) ten d to o cc u r a t th e b as e o f h yp erp la s tic tis s u e f o lds , p arti cu l ar ly in th e bu c ca l a nd la bi al s u lc i, a n d ca n b e pa in fu l , pa rtic u la rly w h en e ati n g. Th i ck en e d b u cc a l mu c o s a c a n a ls o be c om e tra um a tiz ed

al on g th e o cc lu s a l ri dg e, re s ul tin g in u lc era tio n . Th e re is fr eq ue n t gi n gi va l i n vo lv em en t i n OF G, w h ic h is q u ite di sti n ct f ro m th at s e en i n a n on - sp ec if ic in f la mm ato ry g in gi viti s. Th e

gin g iv ae ma y ap pe ar h yp erp la sti c an d o ed em a tou s a n d the a bn o rm al ti ss u e us u a lly e xten d s fro m th e g in g iva l ma rg in s o n to th e no n -kera tin i z ed mu c o s a o f th e su lc i β €”a β € fu ll

th i ck n es sβ €™ pa ttern ( Fi g. 12. 5).

Fig. 1 2 .3 Gro s s sw e ll in g of l ow e r l ip in pa tie n t w ith e sta blis h ed Cro h n 's d is ea s e aff ec tin g lo w e r gu t.

P. 146

Fig. 1 2 .4 Co rru g ate d ( co b bl es to ne d) ap pe a ran c e o f th e bu c ca l mu c o s a in o ro fa c ia l

gra n u lo ma to s is (O FG ).

His to lo g ic al ly, n o nc a se a ti n g an d ep ith el io i d g ra n ulo ma s , w ith o r w i tho u t m u lti n uc le a ted gi an t ce lls , a re se en in a bo u t 90 pe r c en t o f ca s es . Gra nu lo m as a re n o t al w ay s pre se n t an d th ei r ab se n ce d oe s n o t ex cl u de th e c l in ic a l d ia gn o s is o f OF G.

OFG as a predictor of Crohn's disease

Th e i n terre la tio n s hi p be twe en o ral C ro hn 's d is e as e an d O FG is a m atte r fo r de ba te. In on e

stu dy , 10 p er ce n t o f 60 OF G p a tien ts h a d d ef in ite e vid en c e o f Cr o hn 's d is ea s e af fec tin g th e in tes tin e bu t a ll s ix pa tie n ts ha d re po rted g as tro in te sti n al s ym pto ms . A n o the r stu d y

dem o n str ate d a s ym pto m ati c i n tes tin a l i n vo lv em en t i n 37 p er ce n t w ho pre s en ted s o le ly wi th

fea tu res o f OF G. T h e p rev al en c e o f a sy mp to ma tic i n te s ti n al di se a se in pa tie nts w i th OF G h a s bee n re po rte d a s b etw ee n 10 a n d 50 pe r c en t i n va rio u s s eri es . In i nd iv id ua l ca s es o f OF G it is di ffi c ul t to p red ic t w h eth er th ere w il l b e su b sequ e n t de vel o pm en t o f CD el se wh e re in th e gu t.

Fig. 1 2 .5 Gin g iv al a pp ea ran c e in a c h il d w ith O FG .

OFG and oral sarcoid

Pa tie n ts s u ff eri ng f ro m sa rc o id o si s ca n p res en t wi th O FG ma n if es tin g as re gi o n al

lym ph a de n o pa thy , o ro fa ci al s w el lin g , gi n gi va l e n la rge me n t, o r tis s u e ta gs . O ra l l es io n s c an al so o cc u r a s m ul tip le o r s o li tary , pa in l es s mu c o sal le si o n s th a t a pp ea r a s re d n o du le s

aff ec tin g th e g in g iva , l ip s, p al a te , a n d b u cc a l m u cos a . In vo lv em en t o f th e m a jo r s al iva ry

gla n ds m a y o c c as io n a ll y p res en t a s a p ai n les s p ers is ten t en l arg em en t o f th e gla n d res u lti n g in a re du c tio n i n sa li va ry flo w . S a rco i d c a n th ere fo re pre s en t a s a β€ SjΓ ¶g re n' s -ty pe β€™

co n di tio n . In vo lv em en t o f th e to ng u e wi th s arc o id is rar e. P ar oti d gl an d in vo l ve me n t, to g eth e r w ith u ve iti s an d l ow -gra de fe ve r, is c h ar ac teri s ti c o f H ee rfo rd t' s sy n dro m e.

Ch ara c teri sti c sk in , o p hth a lm ic , a n d u p pe r re sp ira to ry trac t l es io n s o f sa rc o id ma y be pr es en t an d co n f irm th e di ag n os is .

OFG: Melkerssonβ€“Rosenthal syndrome (MRS) and cheilitis granulomatosa Th e te rm OF G en c o mp as s es th e Me lk ers s on β €“Ro se n tha l sy n dro m e (MR S) , bo th in i ts

cl as s ic al p res en ta tio n a n d th e o li go s ym pto m ati c an d m on o s ym pto m ati c va ria n ts. Th e c l as si c al pre se n tatio n of M RS i s as re cu rr en t o ro fa c ia l o e de ma , rel ap s in g fa ci al p ara l ys is , an d fi ss u re d

to n g u e. Th e se s ym pto m s ma y o c c u r a t th e sa m e ti me or at i n terv al s o f m o n th s o r yea rs . Th is rare s yn d ro me a ffe cts b o th se xe s eq u al ly an d m o st co m mo n ly o c cu rs i n th e s ec o n d de ca de o f lif e. M o s t rev ie ws o f M RS c as es i n di ca te tha t p ati en ts s el do m rep o rt a ll th e co m po n en ts o f th e s yn dr om e . Th e i n co m pl ete ex pre s si o n o f M RS h a s be en r ec o gn iz e d a s h a vin g

mo n o sy mp to ma tic a n d ol ig o sy mp to ma tic fo rm s . Or of ac ial s we ll in g , pa rtic u la rly o f th e l ip s, i s th e m o st i mp o rta nt an d c o ns is te nt sy mp tom o f M RS a nd i s de sc ri be d as a p a in le ss , n o n pru ri ti c , fir m o e de ma .

P. 147 Th e c la s si c pr es en ta ti o n o f M RS i s o f r ec u rren t o rof ac ia l o ed em a (o ften af fe cti ng th e li ps ), rel ap si n g f ac ia l pa ls y, an d fi ss u red to ng u e.

Th e r ec u rren t p eri ph e ral f ac ia l pa ra lys is se en in MRS i s ch a ra cte ris tic a lly s u dd en i n o n se t a nd in di sti n gu is h a ble f ro m B el l's p al sy . Th e pa ls y is usu a ll y u n i la tera l an d in th e m ajo ri ty o f

pa tien ts cl ea rs s po n ta ne o u sl y. Th e fi ss u re d to n g ue (l in g ua p li ca ta ) a pp ea rs to b e th e le as t co m mo n a n d le as t i mp o rtan t f ea tur e o f M RS . A n u mb er of a ss o c ia ted di so rd er s ha ve b ee n

rep or ted in M RS a n d in c lu de o th er n eu ro lo g ic a l ma n if es tati o ns a n d o ph th al mi c fi nd in g s, s u c h as ke ra titis , re tro bu lb a r n eu ri tis , di pl o pia , a n d pa ral ys is o f th e m ed ia l rec tu s mu s c le.

Mi es ch e r in 1945 de sc rib ed a c h ar ac teri sti c di ffu s e sw e lli n g of o n e or bo th l ip s, w h i ch ma y be fo ll ow e d by pe rma n en t e n la rge m en t. M ie sh c er' s gra n ulo ma to u s ch e il itis i s ch a ra cte riz e d

hi sto l o gic a ll y b y a g gre ga tes o f n on c a se ati n g g ra n ulo ma s a nd i s co n s id ere d by s om e to be a n ol ig o sy mp to ma tic (o r mo n o s ymp to ma tic ) f o rm o f th e Melk ers s o n β €“Ro se n tha l sy n dro m e. Ch ei liti s gra n u lo m ato s a is th e mo s t fr equ e n t si gn of O FG .

Th e te rm OF G the re fo re en c o mp as s es M RS a n d i ts o li go s ym pto ma tic o r mo n o s ym pto ma tic

var ia n ts , s u ch as c h ei liti s gra n u lo m ato s a, al l o f wh ic h a re ch a ra cte riz e d h i sto l og ic a lly b y th e pre se n ce o f no n c a se ati ng gra n u lo m ata i n o ro fa ci al bio p sy s pec i me n s.

OFG: aetiology and other associations

Th e a e ti o lo g y o f O F G u n re la te d to s ys te m ic di s ea se re ma in s u n c le ar. H o we ve r, a lle rgy ,

in fe cti on s , a n d h e red ita ry ca u se s h av e al l b ee n im pl ic a ted, to g eth e r w ith in fe c ti o u s ag en ts

su c h a s Sa c ch a ro my ce s ce re vis ia e, M yc ob a cte riu m pa ra tu be rcul o si s , an d β€ Cam p ylo b ac ter lik eβ €™ ba c teri a.

Cli n ic al a top y is m or e p re val en t i n O FG . Rec en t stud ie s ha ve i n ves tig ate d the a s so c ia tio n o f OF G w i th β€ in to ler an c e β€™ to sp ec i fic f oo d s , fo o d ad di ti ve s, fla vo u rin g s , an d th e

co n s ti tu en ts o f to o th p as tes . C in n am o n al de h yde a n d so d iu m be n zo a tes h a ve be en pa rtic u la rly im pli c ate d i n th is re sp ec t a n d, i n s o me s eri es , th ere w as a c l in ic a l re s po n se to sp ec i fic

eli mi n ati o n di ets . It re ma in s u n kn o w n w h eth er se n sitivi ty to fo o d ad di tiv es is th e pr im ary fac to r f o r s o me pa tie n ts w ith O FG o r a s ec o n da ry aggr av ati ng f ac to r to s o me u n de rly in g pro c es s.

Management of OFG

Pa tie n ts w i th OF G mu s t be ap pr op ri ate ly in ve sti ga ted , n o t o n ly to co n fi rm th e d ia g no s is b u t to id en tif y an y pr ov o kin g fa c to rs an d s ig ns a n d sy mp to ms , s u gg es tive o f an u n d erl yin g

sys te mi c co n di tio n , s u ch a s CD o r s ar co id o s is . A full ra n ge o f ha e ma to lo gi c al an d b io c he m ic al in ve sti ga tio n s, in c lu di n g i n fla m ma to ry m a rke rs, sh ou ld b e u n de rtak en , to ge the r w i th an

es tim ati on o f th e se ru m an g io ten s in -co n ve rtin g en z ym e (A CE ) a n d a ch e st r ad io g rap h . B io ps y of a n a ffe cte d s i te (u s u al ly th e la bi al o r bu c ca l mu c o sa a n d oc c as i on a ll y th e g in gi va e) sh o u ld be ca rri ed o u t b y an exp er ien c ed o p era to r. Gra n u lo mata m ay o n ly be p res en t i n th e

un d erl yin g mu s c le an d i t is th er efo re a dv is ab le to e xten d th e bi op s y d ee pe r b eyo n d th e su p erfi c ia l ti ss u e.

So m e au th o riti es re ga rd co m pl ete ga s tro in tes tin a l eva lu a tio n b y

oe s op h ag o ga s tro du o de n o sc o py , il eo c ol o n os c o py , an d sm a ll bo w e l ra d io gr ap h s as m an d ato ry in a ll pa tie n ts wi th O FG. Oth er s arg u e th a t th es e po ten tia l ly un p le as a n t in ve s ti ga tio n s a re un w a rra nte d fo r a ll c as e s of OF G, u n l es s the h i sto ry, cl in ic a l e xa mi n ati o n, or pr eli mi n ar y

in ve sti ga tio n s ar e su g ge sti ve o f l o w er g a stro i n te s ti n al i nv o lve me n t. Th e au th o rs β €™ op in i o n is th at al l pa tie nts w i th OF G sh o u ld b e s ee n b y a gas tro en te ro lo gi s t, wh o c a n de c ide o n th e ap pro p ria ten e ss o f fu tu re ga stro i n tes tin a l i n ves tiga ti o n s.

Wh eth er o r n o t a ll pa tie n ts wi th O FG sh o u ld b e pa tch -te s te d to id en tif y po s s ibl e al le rgi es to fo o ds o r f o o d a dd iti ve s i s de ba ta ble . To d a te, th ere i s n o to tal ly co n vi n ci n g e vi de nc e o f a cl in ic a l re s po n s e to e li mi n ati o n di ets , bu t th ere may be a th era p eu tic ro l e fo r d ie ta ry ma n ip ul ati o n in s o m e pa tie n ts.

Pa tie n ts w i th OF G o ften s e ek trea tme n t fo r lip s w ellin g , w h ic h ca u s es di stre s s an d em ba rra ss m en t.

Th e o u tlo o k fo r pa tie n ts wi th OF G is va ri ab le a n d tre atm en t is o fte n u nr ew a rdi ng . P ati en ts ,

an d th eir pa re n ts , u s u al ly se ek trea tm en t b ec au s e of u n s ig h tl y l ip -sw el li n g, w h ic h ca n c a us e co n s id era bl e e m ba rra ss m en t a n d d is tre ss to c h il dre n a nd te en a ge rs. Oc c as i on a l, s h o rt

co u rs es o f sy ste mi c ste ro id s ca n b e e ff ec tive a t redu c in g th e sw e ll in g an d th e do s e c a n be

gra du a ll y re du c ed o ve r a n u m be r o f we ek s, d ep en d in g o n th e s e ver ity o f th e ep is od e. Lon g te rm s ys tem ic s tero id s a re, h o w ev er, co n tra in d ic ated, p a rtic u la rly in c h il dre n . In tral es io n a l ste ro ids c a n be in je cte d i n to th e l ip s o r fa c e b u t lo c a l a n a es th etic b lo c ks s h o ul d be gi ve n

bef o reh a n d, to m a ke the p ro ce du re m o re ac c ep ta b le to pa tie n ts. T h es e i n jec tio n s ma y n ee d to be rep ea ted . A l arg e n um be r o f s ys te m ic dr ug s h av e be en tri ed fo r OF G wi th m ixe d

su c ce s s a n d in c lu d e a z ath i o pri ne , c lo fa z im in e , h ydro xyc h lo ro q ui n e, d an a z o l, c ic lo s po ri n , su la z o su l fa py rid in e, th a li do m id e, a n d an ti mi cro b ia ls s u c h as m etro n i da z o le an d c o tr im ox az o l e.

Trea tm en t o f OF G i s u n sa tis fa c to ry a n d r es po n s e to dru g -th e ra py is di sa p po in ti ng . To pi ca l ste ro id s an d a nti se pti c an d an a lg es ic m o u thwa s h es c an be h el pfu l fo r ma n ag in g th e or al u lc era tio n , as s o ci ate d wi th O FG. An g u la r c h ei liti s an d li p fis s u res fr eq ue n tly be co m e

se co n d ari ly in fe c te d bu t i n m an y ca s es c an be im pr ov ed b y th e ap pl ic a ti o n o f an an ti fu n ga l or a nti bi o ti c c rea m (fo r exa m pl e, n ystati n , fu s id ic a ci d, mi c on a z o le ), as

P. 148

in di ca ted b y m ic ro b io lo g y. To p ic al s tero i d c re am s may a ls o be req u ire d bu t h ig h -po ten c y

pre pa ra ti o n s are b es t a vo id ed , pa rtic u la rl y i n th e lo n g term . Dry, ch a pp ed li ps w i ll u su a ll y

ben e fi t fro m re gu la r ap pl ic ati o n o f a n em o ll ie nt. Su rg ic al re du c tio n o f the l ip s we ll in g in O F G ha s n o t be en rel ia bl y ef fec tiv e a n d is n o t u s u al ly rec o mm e nd ed .

Table 12.2 Summary of OFG OF G is a c li n ic o pa tho l o gi ca l en tity de sc ri bi ng o ro fac ia l le si o ns w i th n o nc a se a ti n g gra n u lo ma s . A s a un i fyi n g c o n c ept it in c lu d es :

Pa tie n ts w i th es tab li sh e d CD a n d tho s e w ho su b s equ en tly de vel o p CD Pa tie n ts w i th o ral s a rco i d

Pa tie n ts d ia g no s ed a s h av in g M RS o r c h ei liti s gra n ulo m ato s a

A sm a ll, h e tero g en eo u s g ro u p o f pa tie n ts wi th o ro faci a l ma n if es tati o ns o f del a yed -ty pe h yp ers en s iti vity re ac tio n s to fo o d o r fo o d ad di tiv es

Th e m a n ag em en t o f OF G n ec es si tate s a mu l tidi s ci pl ina ry ap pr oa c h . Ch il dre n w ith O FG a nd th e ir p a ren ts req u ire a g rea t d ea l o f tim e an d s u pp or t fro m a c lin i ci a n wh o i s fu ll y c o g ni z an t o f th e c on d iti on . O F G i s a co m pl ex co n d itio n an d its m an a ge m en t

ne c es si tate s a mu l tid is ci pl in a ry a pp ro a ch . P er si sten t s we ll in g o f th e li ps a n d f ac e a nd p ai n fu l or al u lc era tio n ca n c au s e co n s id era bl e em ba rra ss m ent a n d di stre ss to c h il dre n an d a du l t pa tien ts ; b o th req u ire s ym pa th etic m a n ag em en t.

Ulcerative colitis

Ul ce rati ve c ol iti s is a di s ea se , u su a ll y b eg in n in g i n yo u n g ad u lt l ife , in wh i ch i n fla m ma to ry

ch a n ge s i n th e co l o ni c mu c o sa a n d su b mu c o s a lea d to w id es pre a d u l ce ra tio n . Th is u l ce ra ti o n

ma y b e co m pl ic a te d by h ae mo rr ha g e, p erf or ati on , a n d, o c c as io n a ll y, b y th e ev en tu al o n s et o f ma li gn a nc y. Pa in , d ia rrh o ea , a n d g en e ra liz e d a b do m in a l d is c om fo rt are th e pre do m in a n t

sym p tom s . Th e ae tio lo g y o f u lc er ativ e co l itis i s n ot cl ea r, a lth o u gh it s e em s to be o n e of th e β€ dis e as es o f c ivi li za tio n β€™. T he tre atm en t o f u lc era tiv e c o li tis i s by the u s e of

su lf as a la z in e an d s tero i ds , u se d eith e r lo c al ly (a s pe ss a rie s o r e ne ma s ) o r sy ste mi ca ll y. Su rg ery m ay be n e ce ss a ry i f me di ca l trea tm en t fa il s.

Cro h n 's di se as e c an a ff ec t a n y p art o f th e ga s tro intes tin a l tra c t. Ul ce ra tive c o li ti s o n ly aff ec ts th e l ar ge bo w el .

Stomatitis and inflammatory bowel disease Pyostomatitis vegetans

Py os to m ati s v eg eta n s is a ra re o ral d is o rde r th a t is c o n si ste n tly as s o ci ate d wi th ch ro n i c

in fl am ma to ry bo w el di se a se a nd c o n si de red to b e a hi gh l y s pe c ifi c ma rke r f o r IB D. Th e bo w el

sym p tom s o fte n pre c ede o ra l in vo l vem en t by se ve ral m o nth s o r yea rs . P yo s to m a tis ve ge ta n s ha s a ge n de r p red il ec tio n w ith a m a le :fe ma le ra tio o f 3:1 a n d ca n af fe ct a l l a ge g ro u ps . It is

th e o ra l e qu i va le nt o f p yo de rm a veg eta n s. Ora l an d cu ta n eo u s le s io n s are s im il ar a nd m ay b e se en c o n cu rr en tly o r i n is o la tio n .

Py os to m ati ti s ve ge ta n s is a n i mp o rtan t o ra l ma rk er f o r i nf la mm a tor y bo w el d is ea s e. Py os to m ati ti s ve ge ta n s h a s a di sti n ct c l in ic a l a pp ea ra n ce w ith mi lia ry ab s ce ss e s an d pu s tu la r les io n s af fe cti ng th e o ral m u co s a a nd g in gi va , w h ich be c om e th ic ken e d, e rth em ato u s , a nd

ma y e xh ib it veg eta tio n s o r β € co b ble s ton i n g β€™. P u s tul ar le si o n s o ften r up tu re, whi c h le ad s

to ero s io n s a nd u l ce rati o n , wi th fi ss u rin g , in a p atte rn d es c rib ed as β € sn a il -tr ac k β€™

ul ce ra tio n . Th e o ral l es io n s pr edo m in a n tly af fe ct th e la bi al a n d bu c ca l mu c o sa a n d th e l ab ia latta ch e d g in g iva e (Fi g. 12. 6), al th ou g h th e ha rd a nd s o ft p a la te, ve s tibu l e, a n d to n s il la r reg io n ca n a ls o b e af fec ted . Th e h is to lo gi ca l fe ature s o f p yo s to m a tis ve ge tan s a re o ften ch a rac te ris tic , al th o ug h n o t p ath o gn o m o ni c , sh o w in g in tra ep ith e lia l an d su b ep ith el ia l mi cro a bs c es s es c o nta in i n g l ar ge n u mb ers o f eo s in o phi ls .

Fig. 1 2 .6 P yo sto m a ti tis ve ge tan s af fe cti ng l ab ia l mu c o sa a n d gi ng iv ae in a pa tie nt wi th

ul ce ra tive co l iti s.

To pi ca l ste ro id th era py h as b ee n s u cc es s fu l fo r th e trea tm en t o f pyo s to ma titi s ve geta n s bu t

in ma n y ca s es s ys tem ic tre atm en t, w ith o r w ith o ut az a th io pi n e or su l fa me th ox yp yrid a zi n e, i s req ui red . M an a ge m en t o f the a ss o c ia ted IB D m ay a ls o res u lt in i mp ro ve me nt o f th e o ral

les io n s . In vi ew o f th e cl os e a ss o ci a ti o n o f py o stom ati ti s ve ge tan s w ith IB D, i t h as b ee n

su g ges te d th a t a ll pa tie n ts sh o u ld be i nv es tig ate d fo r b o we l dis e as e, eve n in th e a bs en c e o f ga stro i n te s ti n al sy mp to ms .

Pyostomatitis gangrenosum

Oth er fo rm s o f s to ma titi s h av e b ee n re po rte dly a ss oc ia ted w ith IB D a n d in c lu de i rreg u la r,

dee p, fo u l -sm el li ng u lc e rs o f v ary in g si z e, w ith rolle d ma rg in s a nd a g rey is h , fi bri no u s b as e.

Gastro-oesophageal reflux disorder (GORD)

Hea lth y in d ivi du a ls e xpe rie n ce ga s tro -oe s op h ag ea l ref lu x af ter a m ea l a nd th is i s du e to th e rel ax atio n of th e lo w er o es o ph a ge al s ph i nc te r. In pa tie n ts wi th ga s tro - oe s op h ag ea l ref lu x

dis o rd er (GO RD), th e re i s in c rea s ed fre qu e n cy a nd du rati o n o f re fl u x a n d d a ma ge i s ca u se d

to the o e so p ha g ea l m u c os a b y re gu rg ita tio n o f ga s tric c o nte n ts. A s a re su l t th ere c an b e

refl u x o e so p h ag iti s, u l ce rati o n , stri ctu re , o r e pi the li al me tap la s ia (B a rrett's o e s op h ag u s) .

Sy mp tom s o f GOR D in c lu d e he ar t-bu rn , ep ig as tric p a in , a nd re gu rg ita tio n . Ho w e ver, so m e

pa tien ts , th e β€ si len t ref lu xe rs β€™, h a ve n o s ym pto m s. Dru g th era p y i s u su a ll y s u cc e ss fu l fo r GO RD an d co n s is ts o f s im p le an ta c ids ( or c o verin g ag en ts ), H 2 rec e pto r bl o ck ers (f o r

exa m ple , c im ete din e ) th a t

in h ib it g as tric a c id se c reti on , o r pro to n -pu mp i n hibi to rs (fo r e xa m ple , o m epr az o le ) th a t

P. 149

in h ib it a c id pro d u cti o n. Su rg ery is r are ly in d ic ated bu t p ati en ts w h o de ve lo p o es o ph a ge al

stri ctu re s ma y ne ed p eri o dic d il ata tio n . P a tien ts with G OR D ma y de ve lo p de nta l ero s io n ( se e Ch ap ter 18), pa rtic u la rl y o f the p al ata l as p ec ts o f th e tee th , a n d, in p ati en ts w h o ar e

as ym pto m ati c, th i s ero s io n m ay b e th e o n l y i nd ic a tor o f p a tho l o gi ca l GOR D. Pa tie n ts w ho reg ul a rly ch e w a nta c id pre pa ra tio n s m ay sh o w s ig n s o f to o th w ea r a n d s o m e a l gin a te-

co n tai n in g a nta c id s h av e a h i gh s u ga r c o n ten t th a t c an pre di sp o se to c ar ies . O c ca s io n al ly , pa tien ts pre s en t w ith o ro f ac ia l ma n if es tati o ns o f ir on de fic ie n cy ( Ch ap ter 13 ), if th ere h as bee n b lee di n g fro m o es o ph a ge al u lc e rati o n.

Pa tie n ts pre s en tin g w ith p al ata l de n ta l ero s io n sh ou ld b e a s se ss e d f o r g as tro -oe s op h ag ea l refl u x ( GOR D).

Discussion of problem case Case 12.1 Discussion Q1

Co u ld th is la d y's o ral c o n di ti o n be re la ted to h er g ut sy mp tom s ?

Th is la d y's o ro fa c ia l s i gn s a re s u gg es tiv e of O FG , w hi ch ca n b e a ma n if es tati o n o f C ro hn ' s

dis e as e. T h e g as tro in te sti n al s ym pto ms , p rev io u sl y di ag n o se d as IB S , ma y al so be in d ic ati ve of in f la mm a to ry b o we l dis e as e (IB D), pa rtic u la rl y Cro hn 's d is e as e. Q2

Wh at a re th e sy mp tom s o f IB S ?

IB S i s a fu n cti o na l bo w e l d is ea s e, i n w h ic h pa tie n ts r ep or t va ria b le de gre es o f a bdo m in a l pa in , d is ten s io n , an d b lo a ti n g. T he p ai n is c la s si cal ly in th e l ef t il ia c fo s sa a n d us u a lly rel iev ed b y d efe c ati on o r pa s sa g e o f w in d . P ati en ts o ften co m pl ai n o f c o n sti pa tio n o r

dia rrh o e a an d ma n y rep o rt fre qu e n t bo w el a c ti o n s in th e mo rn i n g. Wo m en a re a ffe cte d mo re th a n m en . P s yc ho l o gi ca l fa cto rs a re im po rta n t a n d stre ss o fte n e xac e rba tes s ym pto m s. Q3

Ho w w o ul d yo u m an a ge th is la dy ?

Th e p rio ri ty, i n th is pa rti cu l ar ca s e, i s fo r th i s la dy to be re fe rred a s so o n a s p os s ib le fo r fu ll in ve sti ga tio n s by a ga s tro en tero l o gi st, to e xc lu d e IB D, p ar ti c ul ar ly Cro h n 's di se a se . It is

im po rta nt tha t y o u li ai se w ith h e r GP a n d di sc u s s yo u r s u sp ic io n s ab o u t th is l ad y's c o n di ti o n . Refe rra l to a ga s tr oe n tero l og is t i s pre fe rab ly m ad e by h er GP . Th e si tu atio n sh o u ld b e

exp la in e d to th e p ati en t, w h o sh o u ld b e giv en rea s su ra n c e a n d ap pro p ria te to pic a l th e ra py

fo r h er a ng u la r c h ei li tis a nd o ra l u lc era tio n . Re ferra l to a n o ra l ph ys ic i an s h o u ld be m ad e fo r co n fi rma tio n o f yo u r d ia gn o s is a n d pro vi si o n o f defin iti ve trea tm en t. A lter na tiv el y, th e

pa tien t ca n b e ref erre d dir ec tly to an o ral m ed ic ine u ni t a n d th e y w i ll ar ran g e f o r ap pr op ri ate in ve sti ga tio n s, in c o n jun c tio n w i th a de pa rtme n t o f ga s tro en tero l o gy. It is im p or tan t,

ho w e ver, th a t th e pa tie n t' s GP is k ep t ap pr ai se d o f th e si tu ati on , a s th is l ad y's g en e ral co n di tio n m ay d eter io ra te, w hi ls t s h e is a wa iti n g ap po in tm en ts .

Project 1.

In te sti n al po l yp s oc c u r in a nu m be r o f s yn dro m es th a t h av e or of ac i al ma n if es tati o ns .

Dis cu s s th e f ea tu res o f the s e a n d co m me n t o n th ei r p ro pe n si ty fo r m a li gn a nt ch a n ge .

Au th o rs : Title:



Co py rig ht Β © 2003 Ox fo rd U ni ve rsi ty P re ss > T ab le o f Co nt e nt s > 1 3 - Blo od and nut r itio n, e nd o c rin e d is tu rb a nc e s , and re n al d ise as e

13 Blood and nutrition, endocrine disturbances, and renal disease Problem cases Case 13.1

A 40-yea r-ol d la dy rep o rts to h er de n ti s t w ith a 2 -mo n th h is to ry o f s po n tan e o u s ble ed in g

fro m h er gu m s, w h ic h a ls o b le ed ex ce ss i vel y w h e n too th b ru sh i ng . He r m o u th h as b ec o me

very s o re ov er the p rev io u s 2β€“3 w ee ks . Sh e c o mp lain s o f fee li n g ti red a n d l o o ks ve ry p a le . On c lo s er qu e sti o ni n g th e p ati en t re po rts fr eq ue n t a tta c ks o f si nu s iti s an d c h es t in fe c ti o n . In ad di ti o n , sh e h a s no ti ce d th a t s h e te n ds to b ru is e ea s ily . S he i s n o t tak in g an y m edi c ati on . Q1

Wh at d ia g no s is m u st be ex cl u de d i n th is l ad y's c ase ? Wh at o th e r o ral m an i fe sta tio n s

Q2

Ho w w o ul d yo u m an a ge th is l a dy?

of th is c o n di ti o n m igh t yo u fi n d o n ex am in a tio n ?

Case 13.2

A 45-yea r-ol d ma le p ati en t w ith a h i sto ry o f c h ro n ic re n al fa il u re pre se n ts wi th s eve re to o th a ch e . He is n o t a re gu l ar de n tal a tte n de r. Th is g en tl em an u n d erg o es re gu l ar

ha em o di a lys is a n d h as a n in d we ll in g ar teri ov en o u s sh u n t. He l oo k s ver y ti red a n d h a s co m e

dir ec tly fro m th e r en al d ia ly si s un i t, w he re h e h a d be en g iv en s o me a n al ge si c s to tak e f o r h is den ta l pa in . O n ex am in a tio n th e re is a gr os s ly ca rio us m a xil la ry fir st m o la r th at r eq ui res

extra c tio n . Th er e i s n o as s o ci ate d so ft- ti ss u e sw e ll in g bu t y o u n o ti c e w h ite p la qu e s on the to n g u e a n d bu c ca l mu c o sa e . Th es e do n o t w i pe o ff and th e p a ti en t r ep or ts th a t β € th e y

co m e a n d go β €™. Q1

Ho w w o ul d yo u m an a ge th e de nta l ex tr ac tio n o f th is m ed ic al ly co m pr o mi se d p a tien t?

Q2

Wh at i s th e m o s t li kel y d ia g no s is o f th e w h i te p la qu e s o n the pa tie n t's o ral m u co s a?

Disorders of the blood

It is w el l k n o wn th a t l es io n s o f th e o ra l m u c os a may o cc u r i n pa tie n ts wi th a bn o rm al itie s o f th e b lo o d. In p a rtic u la r, th e ap pe a ran c e o f g lo s si tis o r a n gu l ar ch e il itis i n a n ae mi c pa tie nts ha s o fte n be en de sc ri be d. H ow e ve r, it h a s mo r e re c en tly be co m e rec o gn i z ed th at s u c h o ra l sym p tom s m ay be th e res u lt o f re la tive ly mi n o r c h ang es i n the c o n di ti o n o f th e bl o o d a n d th a t th ey m ay o cc u r e a rly in th e di s ea se p ro ce ss , even be fo re ab n o rma l itie s ca n b e

dem o n str ate d b y a s i mp le bl o o d e xa mi n ati o n. Th u s, an e arl y di ag n o si s o f th e bl o od d is o rde r

ma y d ep en d o n a re co g n itio n o f th e s i gn if ic an c e o f th e o ra l s ym pto m s. Th e g re at m a jo rity o f

th e se p ati en ts a re s u ff eri n g f ro m an a em ia s o f va riou s ki n ds a n d, h en c e, th e m ajo r in tere s t is ce n tr ed o n th is gro u p o f co n di tio n s , bu t i t m us t b e bo rn e in m in d th at ab n o rma li tie s of th e wh i te c el l an d pl ate le t c o mp o ne n ts o f th e bl oo d m ay a ls o b e re fl ec ted in or al ch a n ge s.

Anaemias

Th e c h a rac ter is tic fe atu re o f an a em ia i s a red u cti on in th e le vel o f h ae mo g lo b in , w h ic h is

us u a lly a cc o m pa n ied b y a d ec re as ed n u mb er o f e ryth ro c yte s. Th e red c el ls (e ryth ro c ytes ) a re th e c irc u la tin g c ell s pre do m in a n tly co n c ern e d w ith th e tran s po rt o f o xy ge n to th e ti s su e s by

me an s o f the i ro n- co n tai n in g su b s ta n c e h a em o gl o bi n w ith in th e m. T he y are n o rm al ly reg u la r, bic o n c ave d is c s bu t, i f d is tu rba n ce s o f f o rma tio n oc c ur , the y ma y b ec o m e q u ite irr egu l ar in si ze an d s h ap e. S u c h irr eg ul ar ity is o fte n a si gn of im pa i red fu n c tio n . Th e fo rm ati o n o f th e

eryth ro c yte s wi th in th e bo n e ma rro w i s sti mu l ate d by a nu m be r o f n u tr itio n a l f ac to rs , th e tw o of g rea tes t s ig ni fi ca n ce b ei n g v ita mi n B 1 2 an d fo l ic a ci d. B o th th e se s u bs tan c es a re ab s o rbe d

fro m th e g u t an d m u st b e pre s en t i n ba la n ce d qu a n titie s fo r n o rm a l re d ce ll pr od u c ti o n to

ta ke p la ce , ev en w h e n su f fic ie n t ir on is a va ila b le fo r th e s yn th es is o f h ae mo g lo b in . Iro n , fo li c ac id , an d vi tam in B 1 2 ar e k n ow n a s h ae ma tin i cs a n d are e ss en ti al fo r n orma l ery th ro p o ie si s. Ab so rp tio n d ep en d s o n n o rma l mu c o sa i n th e sm al l intes tin e a nd , in pa rtic u la r, o n th e

pre se n ce o f in tri ns i c f ac to r, w h ic h i s syn th e si z ed in th e ga s tric m u co s a a nd w h ic h m u st be pre se n t be fo re a bs o rpti on o f vi tam i n B 1 2 ca n ta ke pl a ce . If th e re a re ab n or ma li ties th a t lea d

to fa ilu re o f in trin s ic f ac to r s yn th es is , v ita mi n B 1 2 ca n n o t be a bs o rb ed fro m th e gu t a n d mu s t be rep la c ed pa re nte ra lly . La ck o f e ith er vi tam in B 1 2 , fo li c ac id , o r i n tr in s ic fa cto r w ill

th e ref o re a ff ec t re d ce ll pro d u cti o n in th e bo n e m arro w . T he ery th ro cy te s fo rm ed u n de r th es e co n di tio n s a re l a rge r th a n n or ma l (ma c ro cy tic ) an d th ei r f un c tio n i s se ve rel y d is tu rbe d. T he res u lta nt cl in ic a l co n di tio n s a re kn o w n a s me ga lo b la sti c an a em ia s .

P. 154

Megaloblastic anaemias

Vi tam in B 1 2 o r fo li c a ci d d ef ic ie n cy ar e th e m os t c o m mo n c a us es o f a n ae mi as w i th

ma cr oc yto s is . P ern ic i ou s a n ae m ia is a n a u toi mm u n e co n d itio n c a u si ng a tro ph y o f th e ga s tric mu c o sa (a tro ph i c ga str itis ) a n d co n s eq u en t f ai lu re to s ec rete in tri n si c fa cto r du e to a n a nti in trin s ic fa c to r a n ti bo d y. Th e ref o re, i n pa tie n ts wi th th is co n d itio n an tib o di es to i n tr in s ic

fac to r m a y b e de te c te d in th e bl o o d. M o re co m pl ex mal ab s or ptio n s yn d ro me s ma y al so o cc u r, in vo lv in g fa il u re to ab so rb n o t o n ly vita m in B 1 2 , bu t a ls o fo l ic a ci d an d ir on co m po u n ds . Th e te rm me ga lo b la s ti c a na e mi a refe rs to th e ch a n ge in si z e a n d stru c tu re of th e ba si c ma rro w

ce ll fro m w h ic h th e eryth ro c yte s are d eri ve dβ€” th e la rg e ci rcu l ati n g ery th ro cy te s fo rm ed fr om th e se a bn o rm al s tem c el ls a re ma c ro cy tes . S im ila r la rge ci rc u la tin g red c el ls m ay be f ou n d in oth e r a n ae mi as th a t a re no t de pe nd en t o n ab n o rma l itie s o f th e vi ta m in B 1 2 /fo li c ac id

me ta b o lis m (fo r in s tan c e, in s o m e i ro n de fi ci en c y an a em ia s) a nd s u ch ma c ro cy ti c an a em ia s fo rm a se pa ra te gro u p th at, e vi de ntl y, w il l no t respo n d to tre atm en t w ith vita mi n B 1 2 o r fo l ic ac id . Oth e r c au s es o f vi tam in B 1 2 an d fo l ate d efi ci en c y in c lu d e m a la bs o rpti o n an d d ieta ry

def ic ie n cy . Fo l ate d efi ci en c y c a n be d u e to dru g s (fo r exa m pl e, p he n yto in ) o r th e res u lt o f th e in c re as ed p h ys io lo g ic al de ma n d du ri ng p reg n an c y.

Th e s itu a tio n i s co m pl ic a te d by th e fa ct tha t, i n mu l ti pl e de fic ie n ci es , th e ten de n cy to mi cro c yto s is a s a res u lt o f iro n d ef ic ie n cy ma y be c o un te rac te d b y a te n de n cy to

ma cr oc yto s is c a u se d, s a y, by fo l ate de fi ci en c y. Th e re su l t ma y be a n o rma l me a n co rp u sc u la r vo lu m e ( MC V) in a p a ti en t w i th bo th d efi c ien c ie s pres en t. In th es e ci rc u ms tan c es a ro u ti ne

blo o d c o un t wi ll be re tu rn ed as n o rm a l. It i s al so cl ea rly e sta bl is h ed th at pa tie n ts wi th fo la te or B 1 2 de fi ci en c ies m a y w e ll de ve lo p o ral s ig n s an d s ym pto m s be fo re the e ryth ro c ytes a re

aff ec ted a n d b ef o re an a em ia d eve lo p s. A ga in , th is is an arg u me n t fo r th e n ec es s ity fo r a f u ll ha em a to lo gi ca l ex am in a tio n i n th es e p a tien ts . It m ay w el l b e tha t th e pa tie n ts de vel o pi ng

or al si gn s a t a n ea rl y s tag e o f a h a em a to l o gi ca l abn or ma li ty re pr es en t a s el ec ted gro u p w ith an u n u s ua l ly se n si tive m u co s al re sp o n se to th e ch a nge s.

Iron deficiency anaemia

A mu c h mo r e c o mm o n c au s e o f a n ae m ia th an fa il ur e to ab so rb vi tam i n B 1 2 o r fo l ic a c id is i ro n def ic ie n cy , w hi ch le ad s to in a de qu a te ha e mo g lo bi n syn th es is . Th e de fic i en c y m ay b e d u e

eith e r to in a de qu a te i n tak e o f iro n or to ex ce s si ve bl o od l o ss a s in m e ns tru a l a b no rm a li ty o r ga stro i n te s ti n al b le ed in g. In i ro n d efi ci en c y an a emia , th e e s se n tia l f ea tu re o bs erv ed is a red uc tio n in th e h ae mo g lo bi n c o nc e ntr ati on wi th in th e ery thr oc yte s (h yp o ch ro m ic ). Th e y

ap pe ar pa le o n m ic ro s co p ic ex am in a tio n , a n d th e re ma y be va ria tio n s in si z e a n d sh a pe . In iro n d efi ci en c y th e e ryth ro cy tes a re u su a ll y sm a ll (m ic ro cy tic ). Ho w e ver, th e n u mb er o f

eryth ro c yte s pe r u n it v o lu me m a y n o t va ry gre atl y fro m its n o rma l va lu e. Th e e ryth ro c yte co u n t is n o t c o n si de red to be a p arti cu l arl y i m po rta nt di ag n os tic te s t in m o st ca s es .

Of th e to ta l i ro n c o n te n t o f th e b lo o d, b y f ar th e g re ate r p ro po rtio n i s c om b in ed in th e fo rm

of h a em o glo b in w i thi n th e red bl o o d c e lls . A s m al l f rac tio n is pre s en t i n th e p la s ma , bo u n d to a s p ec if ic pr ote in , tra n sf erri n , an d re pre s en ts th e tra n s po rt i ro n m ad e a va i la bl e f ro m th e bo dy re se rve s to rep la c e h a em o gl o bi n lo s se s . If th e sto re s be co m e ex ha u s ted, th e re is a per io d o f l ate n t iro n de fi ci en c y i n w h ic h th e h a em ogl o bi n co n c en tra tio n i s wi th in n o rm al

lim its a n d th e e ryth ro c ytes a re o f no rm a l s i ze a n d f o rm, b u t th e se ru m iro n c o n ce n trati o n is

red uc e d. Th i s is s id ero p en ia , a n ir on de fic ie n cy that ma y af fec t th e tis su e s an d i s ca pa b le o f

ca u si n g o ra l s ymp to ms , b ut w hi ch do es n o t p ro du c e an a em ia si n ce th e h ae mo g lo b in re ma in s un a ff ec ted . Wh en th e s eru m iro n is de pl ete d i n th is w ay , th e d eg ree o f sa tu ra tio n o f th e

tr an s fe rrin b y th e ir o n w ill e vid en tly be re du c ed . Th is f or ms th e ba s is o f a v al u ab le d ia gn o s ti c te s t. In mo re c o mp le x co n d itio n s th a n iro n d ef ic ie ncy , the re ma y al so be a red u c ti o n in th e ci rcu l ati n g tra n s ferr in a n d so th e de gr ee o f s a tur ati on ma y rem ai n h ig h in sp ite o f a l o w

se ru m iro n v al ue . A s w as p o in ted o u t in Ch ap ter 2 , th e te st f o r s eru m fe rri tin l eve ls i s no w reg ard ed a s th e b es t g en e ral in d ic ato r of b od y iro n s to res .

Th e s ta ge s of i ro n de fic ie n cy ar e s u m ma riz e d i n Table 13. 1 . Th e s e ru m ferr itin lev el s, as a

me as u re o f th e o ver al l b o dy iro n sta tu s, w o u ld b e exp ec ted to fa ll o ve r th es e th re e sta ge s . Ora l sy mp to ms m ay a pp ea r i n th e s e co n d an d th ird o f th es e sta g es .

Haemolytic anaemias

Th ere is a f u rthe r g ro u p of a n ae mi as , th e h ae mo l ytic an a em ia s , in w h i ch th e es s en tia l

ab n or ma li ty i s an i n cr ea se i n the ra te o f ery th ro cyte d es tru cti o n . Un d er n o rm a l c o n di tio n s,

th e re d c e lls l a st f o r a bo u t 100 d ay s, b u t in h a em oly tic a n ae mi as th e ir l if e m a y b e red uc e d to on l y a f ew da ys . Ha em o ly tic a n ae mi as m a y b e du e to an in trin s ic d ef ec t o r m a y b e

ac qu i red β€”an i mp o rtan t, a lth o u gh rel ati vel y u n c o mmo n , ca u s e o f th e a c qu i red fo rm b ein g

th e e ffe ct of s o me dr ug s (Tab le 13. 2). Ho w ev er, th e m o s t im po rta n t h ae m ol yti c a n a em ia i n

te rm s of d en ta l p ra cti ce is s ic kl e- ce ll an a em ia , a lth o u gh th i s co n d itio n i s so m ew h a t di ff ere nt fro m th e o th er s un d er co n s id era tio n i n th at its m ajo r si gn i fic a nc e to th e de nti st is n o t in the pro d uc tio n o f o ra l l es io n s .

SICKLE-CELL DISEASES Th e s ic kl e- ce ll di se a se s are a gro u p o f gen e tic al ly de term in e d co n d itio n s in wh i ch th e re d

ce lls c o n tai n a n ab n o rma l h ae mo g lo b in , Hb S . Wh en H bS l o se s o xyg en i t u n de rgo e s ch a n ge s th a t

pro d uc e di sto rti o n o f th e ce ll s, th e s ic kl e e ffe c t. HbS is in h e rited a s a n au to s o ma l rec e ss iv e

P. 155

co n di tio n p rev al en t i n th o se w ith B l ac k Af ric a n/ Carib be an a n c es try a n d in s o m e f am il ie s fro m th e M id dl e a n d Fa r Ea s tern c o u n trie s. T he c a rrie r state (in wh i ch th e p ati en t i nh e rits th e co n di tio n f ro m o n l y o n e pa re nt) is k no w n a s th e s ickl e- ce ll trai t a n d i s by fa r th e m or e

co m mo n c o n di tio n . In th is tra it th e pro p o rtio n o f Hb S in th e red c el ls i s lo w a n d s ic kl in g do e s no t ta ke p la ce u n de r n o rma l ci rc um s tan c es . Ho w e ver, si ck lin g m ay o c cu r in c o n di ti o n s of l ow

ox yg en ten s io n a n d, i f th i s ch a n ge do e s tak e p la c e, th e o xyg en - ca rryi n g ca pa c ity o f th e bl o o d is gr ea tly red u ce d wi th th e c o n s equ e n t po s s ib ili ty o f a no x ia in th e pa tie n t. Th e fu ll y

dev el o pe d s ic kl e-ce ll an a em ia i s th e r es u lt o f the p res en c e o f tw o H bS g en es , o n e fro m ea c h pa ren t. In th is c a se , th e p ro po r ti o n o f H bS i s h ig h an d s ic kl in g o cc u rs u n de r n o rm al bo d y co n di tio n s . In th es e p a tien ts , th e o xyg en -ca rryi n g ca pa c ity o f th e bl oo d i s po o r, th ere i s

im pa irm en t o f va s cu l ar fl ow , a n d a h ae mo l ytic a n ae mi a res u lts f ro m th e s h or ten ed l ife o f the

ab n or ma l ery th ro cy te s . Th is i s a se ver e c o n di tio n an d th e s ym pto m s o f g en era l il l h e al th ar e so m a rke d a s to m ak e i t ve ry u nl ik ely th a t a pa tie nt w ou l d pre se n t fo r de n tal trea tm en t

un d ia gn o s ed . Ho w ev er, th e re i s n o s uc h g u ara n tee i n the c a se o f th e s ic kl e -ce ll trai t a n d th e on l y w a y to ide n tify th es e pa tie n ts i s to c arr y o u t a tes t fo r the p res en c e o f H bS .

Fo rtu n ate ly , the i n itia l sc re en in g tes t f o r th is c ond iti o n is re la tive ly s im pl e a n d is e as il y

per fo rm ed in th e la b or ato ry. It is a ls o tru e th at mo d ern te ch n i qu es o f a na e sth es i a, d es ig n ed

to av oi d ev en tra ns ie n t a n d m in o r de gre es o f hy po x ia , are m u ch les s l ike ly to c au s e pro b lem s in th es e pa tie n ts th a n p rev io u sl y w a s th e c a se .

Table 13.1 Stages of iron deficiency 1. 2. 3.

Pre -la te n t ir on de fi ci en c y, in wh i ch th e b o dy store s o f i ro n ar e d ep le ted , bu t

th e ci rc u la tin g h ae mo g lo bi n a n d s er um f erri tin r em ai n w ith in n o rm a l li mi ts

Late nt iro n d ef ic ie nc y, i n w h ic h th e bo dy s to res a re ex ha u s ted an d th e se ru m

ferr itin r ed uc e d. Th e h a em o glo b in co n c en tra tio n re mai ns u n a ffe c ted

Late nt iro n d ef ic ie nc y, i n w hi c h th e b o dy s to re s a re e xh a u ste d a n d th e s e ru m

ferr itin red u ce d. T h e h a em o gl o bi n co n c en tra tio n re ma i ns u n af fec ted

Table 13.2 Oral signs and symptoms of haematinic deficiencies Glo s si tis

S m o o th, d ep a pil la ted to ng u e (iro n d ef ic ie n cy ) R aw , b ee fy ton g u e (vi tam in B 1 2 an d fo l ate d efi ci en c ie s)

Ora l ca n di do s is (in c lu d in g an g u la r c h ei liti s) Ex ac er ba tio n o f RA S

Pl u mm er β€“Vi n so n (P a tters o n β €“Kel ly) sy n dro m e ( iro n de fi ci en c y)

Oro fa c ia l ma n ife s tati on s o f si c kle -ce ll an a em ia h a ve b een rep o rted a nd in c lu d e p ro mi n en c e of th e m ax ill a a nd m an d ib le a nd o ro fa c ia l pa in . Th e se o c cu r as a r es u lt o f ma rro w h yp er pla s ia an d ex pa n si o n o f th e ma rro w s p ac e du e to a lo n gs ta ndin g h a em o lyti c an a em ia . De stru c tive

os teo m ye li ti s is a re c og n iz e d co m pl ic a tio n o f de ntal in fe cti o n in s i ckl e ce ll di s ea se . P eri ph e ral ne u ro pa th ie s as s o ci ate d wi th s ic kle c el l cri si s h ave a l so b ee n re po rte d, i nc lu d in g m en tal an a es th es ia .

Normocytic anaemias

No rmo c yti c an a em ia s ma y be s ec o n da ry to sy ste mi c dise a se s, s u c h as s c u rvy (s ee be lo w ) o r as so c ia te d w i th pri ma ry o r s ec o n da ry bo n e ma rro w a pla si a an d n eo p la si a . Ap la s tic a na e mi as ca n be c a us e d b y a nu m be r o f c yto to xi c dru g s.

Oral signs and symptoms in anaemias

A wi de ra n ge o f or al s ig ns a n d sy mp to ms ma y ap pe ar in an a em ic p ati en ts bu t th e se a re du e to the u n de rly in g de fi ci en c y o f iro n , v ita mi n B 1 2 , or fo l ic a ci dβ €”fo r m u co s a l p al lo r to be

no ti ce ab le in the m o u th the p ati en t's h a em o gl ob in ne ed s to be l ow (< 8 g /dl ). Th e o ral s ig n s an d sy mp to ms a re a res u lt o f b as ic c h a ng es i n th e me ta b o lis m o f th e o ra l ep ith el ia l ce ll s, wh i ch a re p arti cu l arl y s u s ce pti bl e to m in o r va ria tio n s in the q u al ity o f th e bl oo d s u pp ly. Th es e ch a n ge s giv e ris e in th ei r tu rn to a bn o rm al itie s o f c e ll stru c tu re an d o f th e

kera tin i z ati on pa ttern o f th e o ral e pi the li u m, th e en d re su l t o ften b ei n g atro p hy . Th is a tro ph y se em s pa rtic u la rl y to a ff ec t th e co m pl ex fil if o rm pa pi lla e o f th e to n gu e, wh i ch ma y b e al mo s t co m pl etel y l o st. H o we ve r, th e ch a n ge s are b y n o m ean s res tri cte d to th e to ng u e an d

ul ce ra tio n o r g en e ra liz e d s o re n es s ma y oc c u r o ve r th e w ho l e of th e o ral m u co s a . Ap a rt fro m th i s type o f sy mp to m, th e pa tie n t w ith a n ae mi a o r late nt iro n d ef ic ie n cy is p ar ti c ul ar ly

su s ce pti bl e to i n fe cti on by Can d id a al bi ca n s an d a n gu la r ch e ili tis o r th ru s h m ay o c cu r. In

ad di ti o n to th es e ch a n ge s, th e p ati en ts ma y co m pl ain o f dis tu rb an c es o f tas te se n sa tio n . It ha s be en su g ge ste d th a t th is i s du e to a tr o ph y of th e to n g u e ep ith el iu m w ith r es u ltin g dis tu rba n c e o f th e un d erl yi ng n e rve en d in gs , b ut suc h a di stu rb an c e o f ta ste h a s be en

ob s erve d in p a ti en ts w ith a p pa ren tly c li ni c al ly n o rma l ton g u e e pi th el iu m. A nu m be r o f rep o rts ha ve d es cr ibe d th e re s ul ts o f th e in ve s ti ga tio n o f o ra l l es io n s i n an a em ic p ati en ts a n d i t h a s bec o m e e vi de nt tha t th ere is n o c l ea r c o rrel ati o n be tw ee n the o ra l sy mp to ms a n d th e b as ic ae tio lo g y. S or e to n g ue , ta ste di stu rb a nc e , ge ne ra lize d sto m ati tis , ca n di do s is , a n gu la r

ch e ili tis (Fi g. 13. 1 ), gi n giv iti s, a n d an e xa c erb ati o n o f rec u rre n t ap h th ou s s to ma titi s (RA S ) ma y o c c ur in an y o f th es e pa tie n ts (se e Tab le 13. 2).

Th e s a me c o n si de rati o ns ap pl y in re la tio n to f o la te a n d B 1 2 de fi ci en c y. It h a s be en p o in ted

ou t a b o ve tha t v ery ea rl y d efi c ien c ie s o f e ith er o f th e se fa c to rs ma y res u lt i n o ral m u co s a l

ch a n ge s. T he s e a re c erta in ly n o t d ue to s ec o n da ry an a em ia s in c e n o ne m ay b e p res e nt. T h e pre ci se re as o n fo r th es e c h a n ges i s n o t kn o w n .

In th e P lu m me rβ€“ Vi n so n (P a tters o n β€“ Kel ly) sy n dro me, pa tie n ts pre se n t w ith d ys ph a gi a, ca u se d by an oe s o ph a gea l w eb , iro n

def ic ie n cy an a em ia ( he n ce th e term s id ero p en i c dys pha g ia ), a nd g lo s si tis . Th ey m ay a ls o dev el o p o ra l ca n di do s is a n d are p red is po s ed to th e de ve lo p me nt o f p o stc ric o id an d o ra l ca rc in o ma ( se e a l so Ch ap ter 10 ).

P. 156

Fig. 1 3 .1 B ila te ral a n gu la r ch e il itis a n d a ge n era li ze d sto mati tis i n u nc o n tro ll ed per ni c io u s an a em ia .

Th e rel ati o n sh i p b etw e en RA S a n d co e lia c d is ea se w as di sc u ss e d i n Ch ap ter 5. It is g en er al ly ac ce pte d tha t RA S i s n ot di rec tly as s o ci ate d wi th ir on de fic ie n cy (a s ma y o cc u r i n c oe li ac

dis e as e) , al tho u g h pre -exi sti n g R AS m a y w e ll be e xace rba ted b y i t. It is l es s ea s y to d ef in e th e ro le o f fo la te or B 1 2 de fi ci en c ies β€”so m e d ef ic ie n t pa tie n ts w i th RA S sho w a n i mm ed ia te

res po n s e to su p pl em en tati o n w h ils t, q ui te cl ea rly , th os e w ith co e li ac d is ea s e r es po n d pri ma ril y to th e g lu ten -free d ie t. Th is , h o we ve r, res u lts i n th e c o rre cti o n o f th e

ma la bs o rp ti o n pr oc e ss a n d th e re sto ra tio n o f de fi cien t l eve ls . It i s, th e refo r e, d iff ic u lt to te ll pre ci se ly w h at p a rt ea c h co m po n e n t pl ay s in th e revers a l o f th e R AS .

Fu rth er u n ce rtai n ty i s in tro du c ed b y th e fa ct tha t s o me p ati en ts w ith a dv an c ed

ha em a to lo gi ca l di stu rb an c es d o n o t s h o w o ral c h an g es . S o me pa tie n ts, f o r ex am pl e, th o s e wi th ad va n ce d me ga lo b la s ti c a na em i a, th e res u lt of fo l ate de fi ci en c y, m ay h av e no or al

co m pl ai n ts a n d th e m u co s a m ay a ppe a r to b e w i thi n no rma l cl in ic a l lim its . It w o u ld s ee m rea so n a bl e to rev is e th e lo n g -he ld vi ew th at c l ea r -cu t o ra l si gn s a n d s ym p to m s m ay be

as cr ib ed to s pe ci fic b lo o d de fi ci en c ies , a n d to ad opt th e a lter na tiv e vie w po i n t th at a w i de ran g e o f o ra l ch a n ge s ma y ari s e f ro m an y o f th e c ond iti on s u n de r d is c u ss io n .

Management of patients with anaemias and haematinic deficiencies In th e m ajo ri ty o f th e pa tien ts d es c rib ed , trea tme nt o f th e u n de rly in g h ae ma to lo g ic al

def ic ie n cy le ad s to a ra pi d res o lu ti on o r im p ro vem en t in th e o ral s ym pto m s. In p a ti en ts w ith lo n gs tan d in g la ten t i ro n de fi ci en c y, h o w ev er, th e res p on s e ma y be a s lo w o n e. S u c h

refr ac to ry be h av io u r i s we ll re co g n iz ed a n d m a y i n di ca te po o r d ru g co m pl ia n ce o r i mp a ire d pa tien t ab so rp tio n o f rep la c em en t th er ap y.

Leukaemia

Leu ka em ia re pr es en ts a ma l ign a n t pro l ife ra tio n o f wh i te c e lls , re pl ac in g th ei r n o rm al

dev el o pm en t i n th e b o n e ma rro w . Th i s pro c es s m ay a ffe ct a n y o f th e w hi te ce ll str ai ns , b u t th e m o st u su a l fo rm s are l ymp h o cy tic , mo n o c ytic , a nd m ye lo i d, d ep en di n g on wh e th er

lym ph o c ytes , m o n oc yte s , or gr an u lo c yte s are i nv o lved. E a c h o f th e se fo rm s o f le u ka em ia pre se n t ei the r i n a n ac u te o r a c h ro ni c fo rm (s ee Tab le 13. 3).

It is n o t u n co m m on fo r o ra l si gn s a n d s ym pto m s to be th e fir st in di ca tio n o f th e pre se n ce o f

leu k ae mi a, p a rtic u la rly o f o n e o f th e a c u te typ es (se e Tab le 13. 4). Th e gi ng iv ae a re

freq u en tly in v o lve d wi th a h yp erp la sti c gi n giv itis . Th e h yp erp la s tic gi n gi viti s res u lts i n fra gi le red s po n gy gi n gi va e th a t b lee d sp o n tan e ou s ly o r f o llo w i ng sl ig h t in ju ry . In a few ca s es , a

mi ld gi n giv al h yp erp la s ia m ay be th e fi rst in d ic atio n o f a n a cu te le u ka em ia ( Fi g. 13. 2). In

ac u te mo n o b la sti c le uk ae mi a (a s ub gr ou p o f ac u te mye lo b la s ti c le u ka em ia ), th e mo n o c ytes are kn o w n to i n fil trate s ite s of in f la mm a tio n s u ch a s th e gin g iv a. In mo re a c ute c as e s, th e hy pe rpl as tic n a tu re of th e gi ng iv itis m a y n o t b e evi de n t an d th e co n d itio n m a y s h o w a s

sp o nta n eo u s h a em o rrh ag e fro m th e g in g iva l ma rg in s (Fi g. 13. 3 ). Th e gi ng iv ae a re h igh l y su s ce pti bl e to in fe c ti o n a nd a s ec o n da ry ac u te ul c era tive g in g ivi tis is c o m mo n . Th i s

su s ce pti bi lity to i nf ec tio n o c cu rs a ls o i n m or e c h ro n ic c a se s wh e n th e or al sy mp to ms m a y

co n s is t o f r ec u rren t a tta cks o f a cu te u lc era tiv e gin g iv itis . Th e se ma y o cc u r w ith o u t a n y o f th e hy pe rpl as tic c h a ng es m en ti on e d ab o ve an d s o m ay ap pe a r to b e n o t o f gr ea t s ign i fi ca n ce . Un ex pl ai n ed o r re pe ate d rec u rren c e o f ac u te u lc era tiv e g in g ivi tis s h o ul d be trea ted w ith

su s pi ci o n an d b lo o d ex am in a tio n a rra n ge d i n o rd er to eli mi n ate th e po s si bi li ty o f the b lo o d dys c ras i a. T he o ro fa c ia l ma n ife s ta tio n s o f ch ro n i c

P. 157

leu k ae mi as a re s im ila r to th os e o f a c u te l eu ka e mi as b u t ar e o f te n l es s flo ri d an d m ay pre s en t in a n in s id io u s ma n n er. In ch ro n ic l ym ph o c ytic l eu ka e mi a th e re is i nv ari a bly l ymp h n o de

in vo lv em en t. Le uk ae m ic in f iltra tio n o f th e sa li va ry a n d la c rim al g la n ds is o cc a si o n al ly se en .

Table 13.3 Classification of leukaemias Acu te

Ch ro n ic

Lymp h o bl as tic *

Lymp h o cy tic

No nl ym ph o b la sti c (my el ob la s tic )β €

My elo i d

*M o s t c o mm o n ac u te le uk ae m ia in c h il dre n . β€ Mo s t c o mm o n a cu te le u ka em ia in ad u lts

Table 13.4 Orofacial manifestations of acute leukaemia* Sp o n ta n eo u s h a em o rrh ag e fo r g in g iva e (re du c ti o n in pl ate le ts) Ora l pu rp u ra an d pe tec h ia e

Gin gi va l sw e lli n g (le u ka em ic i nf il trati o n)

Ora l u lc era tio n (l eu k ae mi c de po s its , in fe c ti o n s, h aem a tin ic d ef ic ien c ie s )

Mu c o sa l sw e ll in g an d lo o s en in g (e xfo l ia tio n ) o f teeth (le u ka em ic de po s its ) Op po rtu n is tic i nf ec tio n s (e .g . h erp es , Can d id a ) Lymp h n o de e nl a rge me n t

*N .B . C h em oth e ra py to trea t l eu ka em ia m a y a ls o p ro du c e o ral s id e -eff ec ts.

Fig. 1 3 .2 M ild g in gi va l h ype rp la si a β€” th e fi rs t in d ic ati o n of a c ute m ye lo bl as tic

leu k ae mi a.

Fig. 1 3 .3 S po n tan e o us h ae mo rr ha g e f ro m the g in gi va l ma rgi n in ac u te le u ka em ia .

In mo re ad va n ce d ca s es o f le uk ae m ia , o ral u lc e rati on is ve ry co m mo n . T he u lc e rs, pro d u ce d

by the b rea kd o wn o f th e tis su e s o ver lyi n g d ep o si ts o f l eu ka em ic c e lls , ma y be la rg e, p ai n fu l, an d di ffi cu l t to tre at. Th e m ai n ten a nc e o f o ra l h ygi en e ma y be o f gre at h e lp in red u ci n g th e dis tre ss in g o ra l s ym p to m s in th e se pa tie n ts. C ov erin g ag en ts a nd a n tis ep tic m ou th w a sh e s are a ls o h el pf ul i n ea s in g th e p a in fu l sy mp to ms d u rin g th e l ate s tag es o f th e d is ea s e.

Leukopenia

Leu ko p en ia re pr es en ts a fa ll in th e w h ite ce ll c o n te n t o f th e b lo o d. T hi s ma y be a

sp o nta n eo u s ly ar is in g co n d itio n , b u t ma y o cc u r a ls o a s a res p on s e to dr ug th er ap y.

Car ba ma z ep in e h as b ee n a ss o c ia ted w ith s ev ere h a em ato lo g ic a l e ffe c ts , i n cl ud in g le u ko p en ia an d ap la s tic a n ae mi a. Leu ko p en i a ma y a l so o c c u r a s a tr an s ie n t sta ge i n th e d ev el o pm en t o f leu k ae mi a an d o th er di se as e s af fec tin g th e bo n e ma rro w . It m ay a ls o res u lt fro m

au to im mu n e di so rd er s su c h a s sy ste mi c lu pu s e ryth ema tos u s (S LE ) a n d vira l in fe c ti o n s; pa rtic u la rly HIV /E B V in fe c ti o n s.

Al tho u g h it is n o t a pa rtic u la rl y c o mm o n c o nd iti o n, th e m o st us u a l c li n ic al p res en ta tio n i s o f ag ran u l oc yto s is . Th i s rep res en ts a re du c tio n i n th e n u m be r o f gra n u lo c ytes fo rm ed in the

ma rro w a n d c ir cu l ati ng i n th e b lo o d . Th e eff ec t o f th i s is to in c rea s e th e s u sc ep tib il ity o f th e pa tien t to in fe cti o ns o f va ri ou s k in ds . In th e c a se o f the o ra l mu c o sa th is m a y l ea d to

wi de sp rea d in f ec tio n a n d u l ce ra tio n o f a ll p arts o f th e m u c os a . Th es e c ha n ge s ma y n ot be dis s im il ar to th o se o cc u rri ng i n le u ka em ia . Th e ae tio lo g ic a l p ro c es s is s im il ar in th a t th e

pro tec tiv e fu n cti on of th e w hi te c e ll co m po n e n t o f th e bl o o d i s red u ce d, i n th e o n e c a se b y th e e xc es si ve pro d u cti o n o f a bn o rm a l c el ls a n d, i n th e oth e r c as e , by a n in a de qu a te pro d uc tio n o f n o rma l c ell s.

In cy cl ic n eu tro pe n ia , a ra re co n d itio n i n w h ic h n eu tro p hi l pro d uc tio n i s in te rmi tten tly

def ic ie n t, gi ng iv itis a n d RA S- lik e u l ce ra tio n m ay oc cu r d u rin g th e ne u tro pe n ic ep is o de s (s ee Ch ap ter 5).

Myelodysplastic syndromes

Th is is a g ro u p of s tem ce ll d is o rde rs in w h i ch th ere is a s up pr es si o n o f o n e o r m o re ce ll li n es in th e bo n e ma rro w . In s o me c a se s thi s le ad s to bo ne m arr ow fa ilu re a n d i n o th ers to

leu k ae mi a. M y elo d ys pl as tic s yn d ro me s ar e m o re co m m on in m al es o v er 60 ye ars o f ag e an d ma y b e de tec ted in th e o ra l m e dic i ne c li n ic w h en p ati en ts u n de rgo ro u tin e h ae ma to lo g ic al sc ree n in g . An ab n or ma l bl oo d c o u nt in a n e lde rly p ati en t sh o u l d th e refo r e s u gg es t th e

po s si bi lity o f a m ye lo d ys pl as tic s yn d ro me . Or al u lcera tio n a n d gi n giv al i nf il trati o n h av e b ee n rep or ted in a f ew ca s es .

Platelet abnormalities

Wh en th e fu n cti o n o r th e n um b er of th e pl ate lets in th e c irc u la tin g bl o od i s red u ce d, th e re is a te n de n cy fo r s p o nta n eo u s h ae mo rr ha g e to o c c u r w ith in the ti ss u es . Th is m a y w e ll sh o w

in iti al ly in th e fo rm o f pe te c hi a l h a em o rrh a ge s o n th e o ra l mu c o sa a n d th e se a re, in fa c t, a we ll -kn o wn si gn o f ea rl y i mm u n e th ro m bo c yto pe n ia . In le u ka em ia s o f v ari o u s ki nd s , bo th

pla tel et fu n cti on an d n u mb ers a re gr ea tly red u ce d, a n d h ae mo rrh a ge s o f m u co s a a n d s ki n ma y th e ref or e b e an e a rly w arn i n g s ig n . It is a d visa bl e to ca rry o u t a fu l l b lo o d sc re en ,

in cl u di n g a p la tel et c o u n t, o n an y pa tie n t w ith o the rw is e u ne xp la in e d a re as o f h ae mo rr ha g e aff ec tin g th e o r al mu c o s a. It s h o u ld be re me mb ere d, ho w e ver , th a t tra n si en t h a em o rrh a ge s

of th is k in d ma y oc c u r o n th e s o ft p a la te i n pa tie nts wi th a s eve re co l d. Id io p ath ic o ra l bl o o d

bli s te rs (w ith o u t a ny a ss o c ia ted h ae m ato lo g ic a l d efec t) w ere d es c rib ed in Ch ap ter 11. Si mi la r blo o d -fil le d b li ste rs ma y al s o be pr o du c ed in th ro mbo c yto pe n ia , pa rti cu l arl y o n th e p al ate , al th o u g h th ey ma y oc c u r el se w h ere o n th e or al m uc o sa.

The selection of patients for haematological examination

Pa tie n ts w i th th e sy mp to ms d es c rib ed in Tab le 13. 5 fo rm a c o ns i de rab le pro p o rtio n o f th os e refe rre d to th e o ra l me di ci ne c li n ic fo r in ve sti ga tio n a n d, as a c o n s equ e n ce , a fu ll

ha em a to lo gi ca l ex am in a tio n m u st be c arri ed o u t i n al l th es e ca se s . Th e ac c ep te d ro u tin e

β€ blo o d s cre en β€™, c o n si s ti n g of a fu l l b lo o d ce ll c ou nt (h ae mo g lo b in (H b), d if fer en tia l

wh i te c el l co u n t (WC C), a n d pl ate le t es tim a ti o n ) a nd th e ex am in a tio n o f a bl o o d f il m, i s so m etim e in s u ffi ci en t to de mo n s tra te a n ae mi a s i n thei r e arl y or la ten t s ta ge s bu t i t is

im po rta nt to ex ten d the i nv es tig ati o n fu rth er in s elec te d p ati en ts ( Tab le 13. 5). Th e rati o n al e fo r th is h a s al re ad y b ee n di sc u s se d in Ch ap ter 2. Wh en a n e xten d ed in v es tig atio n is de c id ed up o n , a rea s o na b le s ch e me o f i n ve sti ga tio n is th a t s h o wn in Tab le 13. 6.

It is e vid en t th a t fu rth e r, mo re s pe ci a liz e d tes ts ma y be n ec es s ar y fo r a fu ll d ia gn o s is o f so m e o f th e pa tien ts i n vo lve d. A n u mb er of th es e w ere di s cu s se d in Ch ap ter 2.

It is e ss en ti al th at th e p res en c e o f s o me h a em ato l og ic a l a b no rm a li ty sh o u l d b e fo ll o we d u p

wi th ap pr op ri ate in ve s tiga tio n s a c co rd in g to th e c lin ic a l ci rc u ms tan c es . S im pl e rep la ce m en t th e ra py fo r a d ef ic ie nc y is u n ac c ep tab le u n les s a ttem pts a re ma de

to de fin e th e un d erl yi ng ca u s e. It m u st be s ai d, h ow ev er, th a t, ev en a fte r c arr yin g o u t fu l l in ve sti ga tio n s, a sm al l nu m be r o f pa tie n ts pre se n tin g wi th o ra l s ym p to m s attri bu te d to def ic ie n ci es re ma in a di ag n os tic p u z zl e in te rms o f th e a e ti o lo g y o f th e de fi ci en c y.

P. 158

Table 13.5 Oral medicine patients who should be considered for an extended haematological examination Pa tie n ts w i th rec u rre n t ap h th ou s s to ma titi s (RA S )

Pa tie n ts w i th a pe rs is ten tly so r e a n d/o r dry mo u th

Pa tie n ts w i th o ral l es io n s w ith a n a typi c al h is to ry o r u n us u a lly re si sta n t to tr ea tme n t

Pa tie n ts c o m pl ai ni n g of a s o re o r b u rn in g mo u th o r to n g ue , o r a bn o rm a l ta s te se n sa tio n , ev en th o u gh n o mu c o s al c ha n g es c an b e seen Al l p a ti en ts w ith p er si ste n t o ral an d o ro fa c ia l ca ndi do s is

Pa tie n ts s h o w in g ab n o rma li tie s fo ll o wi n g an in iti al sc re en i ng

Table 13.6 Haematological investigations for oral medicine patients

Fu ll b lo o d co u n t a n d fil m exa m in a tio n . Th is i s th e r ou ti ne s c ree n pro c ed u re, an d fro m i t ev ide n t a n ae mi as a re de mo n s tra te d by varia tio n s in red c el l

mo rp ho l o gy an d l ow e red h ae mo g lo b in v al ue s . Ab n o rma litie s o f th e w hi te ce ll s an d pl ate le ts ar e a ls o s h o w n

Es tim a tio n o f se ru m ferr itin as a n in d ic a to r o f fu ll -bo dy ir on sta tu s. T hi s h as

rep la ce d the p rev io u sl y us e d s er um iro n /i ro n bi n di ng c a pa ci ty/s a tu ra tio n te s ts wh e n u se d fo r th is pu rp o se . Th is l atte r g ro u p o f tes ts , h ow e ver , is s til l u s ed in th e in ve s ti ga tio n o f c o mp le x i ro n de fic i en c y s tate s

Se ru m B12, se ru m fo la te , an d re d c el l fo la te es tim ati o ns . Th e n ec e ss ity fo r

As a n a dd itio n a l tes t a n ery thr oc yte se di me n tati o n ra te (E S R) me as u re me nt is

ca rryi n g ou t b o th fo l ate es tim a tio n s h as b ee n di sc us se d in Ch ap ter 2

us e fu l a s a n o n -sp ec if ic gu i de to u n de rly in g pa th olo gi ca l pro c es s es s u ch as

ch ro n ic in fl am m ato ry c on d iti on s o r n eo pl as i a β€” al tern a tive ly, me as u rem en t o f C-rea cti ve pro te in ( CRP ) m a y b e u s ed a s a ma rk er f or pre -exi sti n g di se a se

Disorders of nutrition

Th e i n teg rity o f th e o ral mu c o u s me mb ra n e i s ma in tain e d b y a w i de -ran g in g co m pl ex o f

fac to rs , in c lu d in g th os e de pe n de n t o n ad eq u ate n u triti o n. Th e s ig n if ic an c e o f i ro n me tab o li sm an d as s o ci ate d fa cto rs w a s pre vio u s ly di s cu s se d, a s w a s th e re la tio n s hi p be twe en ga stro i n te s ti n al d is ea s e a n d n utri tio n .

Nutritional deficiencies

A wi de ra n ge o f co n di tio n s th at de pe nd o n th e ab s enc e o r red u cti o n o f c er ta i n sp ec if ic nu tri tio n al fa c to rs, p a rtic u la rly vi ta m in s , h as b een de s cri be d in th e pa st. W ith a fe w

exc ep tio n s th es e sp ec if ic co n d itio n s a re n o w rar ely s ee n u n de r E u ro pe an co n di tio n s , al th ou g h th i s ma y ce rta in ly be f ar fro m th e ca se i n o th er s itu a tio n s . It s ho u l d b e rem em b ere d th a t a

nu tri tio n al d efi c ien c y ma y o c c u r in thr ee w ay s: (1) a s a re su lt o f re du c ed in ta ke; (2) as a res u lt o f fa u lty ab so rp tio n o r me tab o li sm ; a n d (3) a s a res u lt o f i n cre a se d e xc re ti o n . Th e

rel ati on s h ip o f iro n d efi c ien c y an a em ia to th e se three fa c tor s is a go o d a n d s im p le ex am pl e. Mo s t p atie n ts se en in th e o ra l m ed ic in e c li ni c w ith f ol ate d efi c ien c y ha ve th is a s a re su l t o f

so m e fo rm o f ma la bs o rp tio n ra th er th a n p oo r in ta ke. It sh o u ld b e r em em be red , h o we ve r, th at a h i gh a lc o h o l in tak e m a y r es u lt i n lo w f ol ate l evels , a s ma y so m e dru g s β €”ph en y to i n in pa rtic u la r. O nl y rare ly , an d th en u s u a lly in stri ct veg a ns , is v ita mi n B 1 2 de fi ci en c y a re su l t o f po o r d ie tary in ta ke . On th e w ho l e, th o s e p a tien ts wh o a do p t u n us u a l d ie ts do s o o n a rea so n a bl y i n fo rm ed ba s is a n d a re fo u n d to h av e s a tis fa cto ry h ae m ato lo g ic a l i n dic e s.

Th ere h a s r ec en tly b een co n s id era bl e in tere s t in the ro le o f o th e r n u tr itio n a l e le me n ts in th e in teg rity o f th e o ra l m u co s a β€”th e B c o mp le x o f vi ta m in s a n d a n u m be r o f tr ac e el em en ts (pa rtic u la rly z in c ) h a ve be en th e su b jec t o f in ve s ti ga tio n s . S eru m z in c le ve ls h a ve be en

in ve sti ga ted in pa tien ts w ith b ur ni n g mo u th s yn dr ome (B M S) an d β € geo g ra ph ic to n g ue β€™,

bu t th ere i s n o co n v in c in g evi de n ce th a t z in c de fi ci en c y i s in vo l ved i n th e p a th o g en es i s of th e se c o n di ti o n s.

It wa s p rev io u sl y p o in ted o u t th a t, in pa tie n ts wi th n u tr itio n a l d ef ic ie n ci es , s ec o nd a ry e ff ec ts ma y f o llo w . P red o mi na n t a mo n g st th e se i s the s u pp res s io n o f the n o rm al i mm u ne re s po n se . Th is w as d es c rib ed in Ch ap ter 4 in th e c as e o f n u triti o n al ly de fi ci en t c h il dre n af fe cte d b y

ca n cru m o ri s. N u tr itio n a l d ef ic ie n ci es d ep en de n t o n fa u lty di et a re ra rel y s im p le o ne s a nd th e pa tien t su ff eri n g f ro m an y sp ec i fic d ef ic ie nc y sh o uld b e c o n s ide re d a c a n did a te f or a m or e co m pl ete in ve s ti ga tio n o f n u tritio n a l sta n da rds .

A sp ec ia l ca s e i s th at o f th e a no re xi c pa tie nt β€” mos t c om m o nl y, b u t n o t ex cl u si vel y, y ou n g

an d fe ma le . S uc h p ati en ts m ay c om e to th e or al m edic in e cl in ic a s a re su l t ei the r o f ge n era l

nu tri tio n al d efi c ien c ie s , wh i ch ma y r es u lt i n sto m ati tis o r so m e o th e r m u co s al pro b le m, o r o f

th e p aro ti d s w el li ng th a t is a f ea tur e i n s o me pa tie n ts. T h e ac i d -in du c ed e ro si o n o f th e tee th th a t m ay o c cu r in p ati en ts w ith th e bu l im ic fo rm o f an o re xia i s di sc u s se d i n Ch ap ter 17.

Scurvy

Sc u rvy (a sc o rb ic a ci d def ic ie n cy ) i s no w a n u n c o mm on di se a se in Eu ro p e, bu t i s n o t by a ny me an s u n kn o w n , be in g the m o st co m mo n l y re co g n iz ed co n di tio n as s o ci ate d wi th a s in gl e

vita mi n d efi ci en c y. A lth o u gh the d is ea s e o c c ur s mo re o ften in o ld a n d ne gl ec ted p ati en ts ,

th e re are o c ca s io n a l c a se s o f m u ch yo u n ge r in d ivi dua ls w h o a do p t su c h a re stri c te d fo rm o f die t th a t cl in i ca l si gn s o f as c o rbi c ac id d ef ic ie ncy ap pe ar .

Th e p red o mi n an t o r al s ym pto m in a s e vere v ita mi n C d ef ic ie nc y is a h yp erp la s tic g in gi viti s, th e g in gi va e be co m in g s w ol le n a nd f ria bl e a n d pu rp le - red in co lo u r. T h ere is m a rke d f al se

po c keti n g a n d thi s , tog eth e r w ith g en e ral l ac k of tis s u e r es is tan c e, m a y l ea d to se c o nd a ry

in fe cti on . T h ere m ay be , th ere fo re, a su p eri mp o se d ac u te g in g ivi tis . Gen e ra liz e d sy mp tom s

in cl u de tire dn e ss a n d ma la i se . Ca pi lla ry fra gi li ty i s a fe atu re o f th i s co n d itio n a n d ma y lea d

to the a pp ea ra n ce o f sp o nta n eo u s h ae mo rr ha g e a n d w ide s pre ad b ru is in g, pa rtic u la rly a ro u nd th e jo in t ar ea s.

In v es tig ati on o f s u ch a p a tien t mu s t in c lu d e a b lo od ex am in a tio n i n o rde r to e li mi n ate th e po s si bi lity o f a b lo o d di s ea se , s in c e th e mo s t i mp ortan t d if fer en tia l di ag n os i s i s fro m

leu k ae mi a. T h e m a in b lo o d ch a n ge in s c u rvy is th e pre se n ce o f a se c on d ar y a n ae mi a . Th e po s itiv e d ia gn o s is i s by la bo ra to ry tes ts fo r l eu koc yte o r p la sm a a sc o rb ic a ci d lev el s.

Ho w eve r, a sa tis fa c to ry c li n ic al d ia gn o s tic tes t i s de riv ed fro m th e res po n s e of th e sy mp to ms to the ra pe uti c do s es o f vi ta m in C , a res po n s e tha t o c c ur s wi th in a fe w da ys a n d tha t i s ac co m pa n ie d by a dra m ati c rev ers a l o f al l th e sy mp to ms .

Th e tre a tm e nt o f s cu rv y i s a ge n era l me di ca l an d , so m eti me s , so c ia l pro b le m. A lth o u gh the ad mi n is trati o n o f a h ig h d o se o f as c o rbi c ac id (1 g da il y) f o r a fe w da ys m a y i mp ro ve th e co n di tio n o f th e pa tie n t rem a rka bl y, th e fu rth er man ag em e nt mu s t be a m atte r f or th e

P. 159

gen e ra l me di ca l pra c titio n e r. No t o n ly th e de fic ie ncy s tate its el f, b u t al s o th e co n d itio n s

lea d in g to its a pp ea ra nc e m us t b e co rre c te d. It sh ou ld a ls o b e rem em b ere d th a t, a lth o ug h

th e s ym pto m s pre se n tin g ma y be o f a s co rb ic a c id de fi ci en c y, th ere i s eve ry lik el ih o o d th a t, in fac t, m u ltip le d ieta ry de fe cts ex is t.

Sc u rvy (vi tam in C d efi c ien c y) is ra re . In ad va n ce d ca s es th er e m a y b e a ma rk ed gi n gi viti s wi th sw e ll in g o r b lee di n g.

Endocrine disturbances

In ge ne ra l, c h an g es i n th e o ral m u co s a di cta ted b y e n do c rin e a bn o rm al itie s a re n o t c o m mo n .

Pe rh ap s th e m o s t fre qu e ntl y c ite d ch a n ge s are th o s e d u e to the e nd o c rin e di stu rb an c es f o un d in n o rma l li fe β€”es pe ci al ly du ri n g p re gn a nc y, a n d at the m en o p au s e. H ow e ve r, a fe w w el l es tab li sh e d o ra l ch a n ge s o cc u r i n s om e en d o cri n e p ath o l o gie s a nd th es e w il l be o u tli ne d bel o w . It sh o u l d b e rem em be red th a t en d o cri n e dis o rd ers a re h ig hl y co m pl ex a n d o f te n

in vo lv e a n u m be r o f sy ste ms b ec a u se o f th e fe ed ba c k m ec h a ni sm s th at c o n tro l th e e nd o cr in e sys te m a s a w h o le . It is , th ere fo re , o ften d if fic u lt to de term in e th e exa c t ef fe ct o f a s in gl e en do c rin e a bn o rm al ity o n an y str uc tu re. En d o cri n e dis o rd ers a ffe c tin g bo n e an d tee th (gi ga n ti s m, a c ro me ga ly , an d h yp erp a rath yr oi di sm ) are d is cu s s ed in Ch ap ter 18.

Hyp o pa rath y ro idi s m res u ltin g fr om p ar ath yro i d or thyro id s u rge ry h as n o pa rtic u la r e ff ec ts o n th e o ro fa c ia l ti ss u es b u t re su l ts i n a lo w s e ru m c alc iu m . Te ta n y is th e cl in ic a l m a n ife sta tio n of re du c ed s eru m c al ci u m. T eta n y m ay a ls o be e nc o u ntere d in a n xio u s d en tal p ati en ts w h o hy pe rve nti la teβ €”th i s res u lts i n al ka lo s is a n d a red u ce d p la s ma i o ni z ed c al ci u m.

Normal endocrine changes Pregnancy

Dur in g pre gn a n cy , the h o rm o na l c ha n ge s th at o cc u r ma y h a ve th e e ff ec t o f e xa c erb ati ng a pre vio u s ly ex is tin g ch ro n i c gi n gi viti s th a t m ay h a ve be en p rev io u s ly sy mp to m -free a n d

un re co g n iz ed . Th e res u lti n g g in g ivi tis i s es se n tia lly h yp erp la s tic , al th ou g h th ere i s mi ni ma l pro li fe rati o n o f f ib ro bl as ts. Th e m o s t ma rk ed pro l ife ra tio n i s of c ap il la rie s an d th is l ea ds to

th e typ ic a lly p urp le c o lo ra tio n o f th e g in g iva l pa pi ll ae (Fi g. 13. 4 ). Th es e pa pi ll ae ten d to b e fra gi le an d m ay bl ee d a t th e le as t i n ju ry. B ec a u se o f th e p res en c e o f fa l se p oc ke tin g a nd ble ed in g , sta gn a tio n a n d se co n d ar y i nf ec tio n m a y o ccu r, an d ma y le ad to h al ito s is .

Fig. 1 3 .4 P reg n an c y gi n gi viti s, i n cl u din g th e fo rm ati o n o f a p reg n an c y ep u lis .

Oc ca s io n a lly , a si n gl e p a pi lla m a y b ec o me c o n si de rab ly en l ar ged a n d pre se n t a s an e pu l is . Th is is th e so -ca ll ed β € pre gn a nc y ep u lis β €™ (Ch ap ter 9). Th e cl in i ca l ch a racte ris tic s a nd ti mi n g of th e oc c u rren c e o f th e se pr eg na n c y l es io n s ar e s u ff ic ie nt to gi ve a stro n g

pre su m pti ve di ag n os i s. H o we ve r, s ho u l d a n y do u bt o cc u r a s to th e n atu re o f th e c on d iti on , fu ll in ve s ti ga tio n i s es s en tia l, in c lu d in g exc i si o na l bi o ps y o f a ny d ou b tfu l ov erg ro w th .

Pre gn a n cy is , i n its el f, n o c on tra in d ic a ti o n to s u ch a b io p sy , bu t i t s h ou l d b e rem em b ere d

th a t th e le si o n is l ike ly to b e e xtre me ly va sc u la r a n d pro f u se bl ee di n g i s to be ex pe cte d. O n ba la n ce , it i s o ften be tte r, i f a c o n fid en t c li n ic al d ia gn o s is h a s be en m a de , to a vo id bi o ps y,

si nc e th e c o n di tio n i s lik el y to r eg res s co n s id era bly, i f n o t c o m pl etel y, a fte r pr eg na n c y. Th e rel ati on s h ip o f RA S to pre gn a n cy a n d th e m en s tr ua l c yc le h as n o t y et b ee n es ta bli s he d.

Trea tm en t d ur in g pre gn a n cy s ho u l d c o n s is t o f th e a pp lic a tio n o f stri ct o ral h yg ie n e m e as u res . Th is , in i tse lf , is o fte n s uf fic i en t to h a lt th e prog re ss o f th e gi n gi viti s. H o we ve r, o ra l h y gie n e me as u res a lo n e a re un l ike ly to le a d to th e c o m ple te res o lu tio n o f a di s cre te β € epu l is -

lik eβ €™ ma ss a n d ev en tu al ex ci si o n ma y be n ec e ss ar y.

Menopause

Th ere is n o s u bs ta n ti ve ev id en c e th a t th e h o rmo n a l c h a ng es o c c u rrin g du ri n g a n d af ter the

me no p au s e di rec tly a ffe ct the o ral m u co s a . Th e qu estio n o f o ra l s ym pto m s o f v ari o u s kin d s

wi th n o id en tif ia bl e ph ys i ca l ca u se o r ab n o rma li ty i s fu rth er ad dre s se d in Ch ap ter 17. Th es e ha ve a ten d en c y to oc c u r i n me n o pa u sa l w o me n.

Adrenocortical diseases Addison's disease

Ad di so n 's d is ea s e i s th e r es u lt o f la ck o f fu n cti o n o f th e a d ren a l c o rtex , u su a ll y th e re su lt o f an a u to im mu n e di so rd er, bu t w ith o th e r po s s ibl e ae tio lo g ie s. A s a re su l t o f th i s de stru c tio n , th e

fee db ac k me c ha n is m be tw ee n th e a dr en a ls a nd th e pi tu ita ry i s di stu rb ed a nd a w i de - ran g in g se rie s o f e nd o c rin e ch a n ge s res u lts . Th e o ra l c h an ge of s ig n ifi ca n c e i n A dd is o n' s di se as e is

me la n oti c pi gm en ta tio n o f th e o ra l mu c o sa , w h ic h m ay in c lu d e th e bu c c al m uc o s a, g in g iva e ,

an d pa la te. It sh o u ld b e r em em be red th a t th is fo rm o f pi gm en ta tio n is b y no me an s s pe c ifi c, bu t n o n e th e le s s th e ap pe a ran c e o f s u ch si gn s in a pa tie nt kn o w n pre vi ou s ly to b e f ree o f pig m en tati o n sh o u l d a lw a ys b e c o n s ide re d a s o f si gnif ic an c e. Th e m ec h a n is m of me la n in pro d uc tio n i n th is w a y i s n ot cl ea rly kn o w n . A lth o ugh a me la n in -stim u la tin g h o rm on e is se cre ted b y th e pi tui tar y g la n d, i t s eem s th a t th e a c tua l o n se t o f p ig m en tati o n ma y be as so c ia te d w i th va ria bl e ad re no c o rtic o tro ph i c ho rm on e (A CTH) le ve ls . In mo re f ul ly

dev el o pe d A d di so n 's di se a se , o ral c an d id o si s ma y also oc c u r. Th e as s o ci ati o n o f o ra l

me la n oti c pi gm en ta tio n w ith c a n di do s is is e vi de ntl y a f u rthe r, m o re ma rke d in di ca tio n f o r

en do c rin e s tu die s . Inv es tig ati o n o f a p ati en t w i th su s pe cte d Ad di so n 's d is ea s e is s h o wn in Tab le 13. 7.

Al tho u g h A dd is o n' s di se as e is a w el l-kn o wn ca u se o f o ral m u co s al p ig me n ta tio n , i t is , i n fa ct, very u n u su a l fo r th e di se as e to b e re c og n iz e d i n this w a y. Th e re are m an y mu c h m o re co m mo n c a u se s o f m el an i n pi gm en ta tio n , w h ic h w ere co ns i der ed in Ch ap ter 9.

Cushing's syndrome

Th is is d u e to h ype rfu n c tio n o f th e a dre n al c o rtex , u su a ll y a s a re su l t o f a n A CTH -se cre to ry

P. 160

pitu i tary a de no m a . Co rtic o s te ro id th era py , pa rtic u la rl y i f p ro lo n ge d, c a n h av e ph ys io l o gic a l eff ec ts si mi la r to th os e o f C u sh i ng 's s yn dr om e (s ee Ch ap ter 2 ).

Phaeochromocytoma and the adrenal medulla

Ph a eo c h ro mo c yto m a is a tu mo u r o f th e ad ren a l me du lla, wh i ch se cre tes ca te ch o la m in es . It ca n be a s so c ia ted w ith n e u ro fib ro ma to s is a n d th e m ul tip le en d o cri n e a d en o ma s yn d ro me (type III). Ne u ro fib ro m as o f the o ra l mu c o sa o r li ps sh o u l d a le rt th e cl in i ci an to the ra re po s si bi lity o f a ph a eo c h ro mo c yto m a.

Thyroid disease Hyperthyroidism

Ex ce ss i ve pro d u cti on o f th yro i d h o rmo n e s (h ype rth yro id is m ) d o es n o t a pp ea r to h a ve a ny dir ec t ef fec t o n th e o ra l mu c o s a b u t m ay c au s e pro blem s o f de nta l ma n a gem e nt. De n tal

pra cti tio n ers m a y b e th e f irs t to o b se rve p atie n ts w ith e xo ph th a lm os w h o m a y a ls o a pp ea r hy pe rex ci tab le a nd re po rt we ig h t lo s s β€”th e y m a y a ls o h a ve a trem o r a n d tac h yc a rdi a. Trea tm en t is u s u a lly by d ru gs s u ch a s c ar bim a z ol e or u s e o f ra di oa c tive i od in e . P arti al th y ro id ec tom y is ra re ly req u ire d u n l es s the th yro i d is c a us in g c o mp res s io n s ym pto m s.

Table 13.7 Investigation of patient with melanotic pigmentation due to possible Addison's disease Im p ai red a dre n al re sp o n se to s yn th eti c an a lo gu e o f AC TH (S yn a cth en Β ® te s t) * Me as u rem e nt o f s eru m el ec tro ly te s (Na β†“ , K β†‘ )β€

Me as u rem e nt o f p la sm a c o rtis o l ( β†“) an d A CTH( β†‘ )β€

Bl o o d p res s u re me as u rem e nt (β†“ hy po ten s io n β €”es pe cial ly po s tu ral ) *De fi n itiv e tes t fo r A ddi s on ' s dis e as e. β€ Ma y be n o rma l.

Hypothyroidism

Hyp o th y ro id is m in a du l ts i s o ften au to i mm u ne b u t may o cc u r a s a c on s eq u en c e of e xc es si ve rem o va l o f th e th yr o id gl an d to tre at hy pe rth yro id is m . Ac q ui red h yp o th yro id is m

(my xo ed em a) m an i fes ts a s w ei gh t g ai n , in a bil ity to to le rate c o ld , dry s kin , l o ss o f h ai r, a n d a sl ow i n g do w n o f ac tiv ity an d m en tal p ro ce s se s. H yp oth y ro idi s m is a ss o ci a te d w ith a n im pa ire d im mu n e me ch a n is m an d o ra l ca n di do s is m ay be th e res u lt. T he re is o fte n a

rel ati on s h ip b etw ee n pe rn ic io u s a n ae mi a an d h yp o th yro id is mβ €”po s si bl y i n th e fa mi ly if n o t in th e in di vi du a l.

Co n ge ni tal h yp o thy ro id is m (c reti ni s m) is c h ara c terize d by dw a rfi sm a n d me n ta l reta rd ati on . Oro fa c ia l si gn s in c lu d e e n la rge me n t o f th e to n gu e (s ee Ch ap ter 6), de fe cti ve fa ci al dev el o pm en t, a n d d el ay ed er up tio n o f th e tee th.

Diabetes mellitus

Dia be te s m ell itu s is a c o m mo n e n do c rin e di so rd er that o cc u rs a s a res u lt o f a de fi ci en c y o f in su l in o r res is ta nc e to in s u li n . Tw o cl in i ca l types a re r ec o gn iz e d: ju ven i le o n se t (i ns u li n dep en d en t, typ e 1) a n d ma tu rity o n se t d ia be tes (type 2).

Dia be te s m ell itu s h a s no sp ec i fic o ra l si gn s o r s ymp to m s . Ho w ev er, p o ss ib ly be c au s e of th e

gen e ra l l ac k o f re s is tan c e to in fe cti o n o f th e di abeti c pa tie n t, pe rio do n ta l dis e as e pro c es s es ma y b ec o m e e xa gg era ted (Tab le 13. 8 ). It is n o t u n com mo n to f in d th at an u n d ia gn o s ed

dia b etic p res e nts w i th ad va n ce d pe rio d on ta l di se a se. Th e pri n ci pl es o f tr ea tme n t o f th is a re

si mp ly th os e o f p eri o do n ta l trea tme n t in the n o n di ab eti c pa tie nt. H o we ve r, in th i s an d in al l

oth e r trea tme n t, th e d en ta l s u rg eo n m u st al wa ys r emem be r th a t th e d ia be tic p ati en t i s mo re th a n n o rm al ly su s c ep ti bl e to in fe cti o n . Th e f req u ent o c c ur ren c e o f o ra l ca n di do s is i n di a be ti c

pa tien ts w a s di sc u ss e d i n Ch ap ter 4. Pa tie n ts wi th u n di ag n os e d o r po o rl y c o n tr o lle d di ab ete s ma y r epo r t xe ro sto m ia , w h ic h is d u e to d eh y dra tio n , s ec o n da ry to po ly u ria . O ral hy po g lyc a em ic d ru gs m ay c au s e lic h en o i d d ru g

rea cti o n s a n d , oc c as i on a ll y, pa tie n ts pre se n t w ith s w el lin g o f th e s al iv ary g la nd s (s ia lo s is ).

P. 161

Glo s so d yn ia (bu rn i n g m o u th s yn dro m e, B M S ) m ay b e a n e arl y ma n ife s ta tio n o f u n di ag n o se d dia b etes ( Ch ap ter 17).

Table 13.8 Oral features of diabetes mellitus Dry m o u th

Co mp ro mi s ed pe rio d o nta l h ea lth * Ora l ca n di do s is

Glo s so d yn ia β €”BM S

Lic he n o id dr ug re ac tio n s (o ra l h y po gl yc a em ic dr ug s ) *De pe n ds o n o ra l h ygi en e .

Th e p a ti en t w i th u nd ia g no s ed o r in a deq u ate ly trea ted d ia be tes m ay h a ve a ge n era li z ed sto m ati ti s a nd , in pa rtic u la r, a s o re to n g ue . Th i s is pr ob a bl y a t le as t p ar tl y du e to

deh y dra tio n a n d pa rtly du e to c an d id al i nf ec tio n . Th e po s s ibi li ty o f la ten t d ia be tes s h o u ld be co n s id ere d, a mo n g st oth e rs, in th e pa tie n t w ith n o n -sp ec if ic gl o ss i ti s o r a c an d id o si s. A

fam il y hi s to ry o f di ab ete s is o f pa rtic u la r s ig n ific an c e. Ju s t a s in th e c as e o f th e s or e to n g ue du e to an a em ia , a s o re ton g u e r es u ltin g fro m d ia be tes m ay o c cu r ea rly in th e di se a se

pro c es s, be fo re su b sta n tia l am o u nts o f gl u co s e are p as s ed in to th e u rin e . In o rde r to p ro vi de a s u ff ic ie nt sc re en in g p ro ce du re , it i s n ec es s ary in th es e pa tien ts to c a rry ou t a b lo o d

glu c o s e e sti ma tio n , p ref era bl y f as tin g , ra the r th a n a s im ple u ri n e s c ree n (s ee Ch ap ter 2).

Renal disease

Ren a l d is ea s e is o f gre at s i gn if ic an c e in o ra l me dici n e a n d ge n era l de n tal pr ac tic e. O ra l

ch a n ge s o c c u r n o t o n ly as a r es u lt o f ch ro n i c ren a l fa il ur e b u t a ls o a s a co n s eq ue n ce o f th e

me dic a l ma n ag em en t o f re na l di se a se . Th e de n ta l ma na ge me n t o f p ati en ts w ith re n al d is ea s e ma y a ls o n e ed to b e mo d if ied to p rev en t c o m pli c ation s .

Chronic renal failure

Ch ro n ic re na l fa il ur e ( CRF ) i s irre ve rsi bl e de teri orati o n in r en a l f un c tio n . Wh e n pl as m a

cre ati n in e pe rsi s te n tly ex ce ed s 300 Β µmo l/ l (n o rm a l ra n g e 80 β€“120 Βµ mo l/ l), th er e i s pro g res si ve de teri o ra ti o n in ren a l fu n cti o n, irre spec tiv e o f a etio l o gy. Wh en th e pl as m a

cre ati n in e rea c he s 1000 Β µmo l/ l, d ia lys is w i ll be re qu i red . Th e gl om er ul a r fi ltra tio n ra te

(GF T; n o rma l, 120 ml /mi n ) m ea s u res re n al fu n c tio n . P ro gre ss iv e im pa irm en t i n fu n c tio n o f th e ki dn e ys le ad s to th e de vel o pm en t o f th e c l in ic al s yn d ro me o f u rae m ia .

Orofacial manifestations of chronic renal failure

Ch ro n ic re na l fa il ur e an d i ts r es u lta nt u rae mi a ca us e a n u m be r o f ph ys io l o gi ca l ch a n ge s th a t ca n c au s e o ral s ym pto m s ( Tab le 13. 9). In ad di tio n , re stri cte d flu i d in ta ke, as p art o f th e me dic a l ma n ag em en t o f th is c o n diti o n , ca n c au s e o ral dry n es s an d , in s o m e p a ti en ts ,

in fe cti on s o f th e p a ro ti d gl an d s. In c h ro n ic ren a l fa ilu re , u ra em ic s to ma titi s oc c u rs ei the r i n th e fo rm o f w id es pre ad a n d pa in f ul o ra l ul c era tio n s (u lc er ati ve u rae mi c sto m a ti tis ) o r as

wh i te p la qu e s af fe cti ng th e o ral m u co s a . Th e la tte r a re pa in -free a n d tr an s ie n t. On c e th e un d erl yin g m eta bo l ic d ysf u n cti on is tre ate d, th e o ral m u co s a rev erts b ac k to n o rma l. In

pa tien ts w ith an a em ia , pa l lo r o f mu c o sa m a y b e evi de n t a nd p u rpu ra o r h ae mo rr ha g e c a n oc c u r a s a res u lt o f pl a te le t de fi ci en c ie s. O ra l can di do s is i s fre qu e ntl y a p ro bl em a n d co n trib u tes to o ra l sy mp to ms i n pa tie n ts w i th ch ro ni c ren a l fa ilu re .

Table 13.9 Orofacial manifestations of chronic renal failure Dry m o u th

Mu c o sa l u lc era tio n

Ba c teri al a nd f un g al p la qu e s

Pa ll o r o f the m u co s a (a na e mi a) Ora l pu rp u ra

Wh ite pl aq u es (u ra em ic sto m a ti tis )

Gia n t c ell l es io n s β€” os teo l ytic l es io n s i n ja ws

Gia n t ce ll l es io n s o f th e ja ws m a y o c c ur as a r es u lt o f h ype rp ara th yro id is m s ec o n da ry to

ren a l f ai lu re (o r p ro lo n g ed di al ys is ) a n d gi ve ris e to o ra l l es io n s o r o ste o lyti c le si o ns i n th e bo n e. P a tie nts w ith CRF a re n ow tr ea ted w ith p o ten t al ph a -hy dro x yla ted vi tam in D su p ple m en ts to pre ve n t se c o nd a ry h ype rp ath yro i di sm .

Grea t c a re m u s t be ta ken wh e n pr es cri bi n g dru g s fo r p a ti en ts w ith r en a l f ai lu re a nd th e

sa fe st d ru gs to us e a re th o s e th a t ar e m eta b ol iz e d p rim a ril y b y th e li ve r. Do sa g es o f oth e r dru gs n e ed to b e ad ju s te d to c om pe n sa te fo r d ec re ase d re n al e xc reti on .

Renal patients undergoing dialysis (Table 13.10)

Wh en p ati en ts re ac h a s tag e o f re na l fa il u re i n w hic h th e k id ne y fu n cti on is in a de qu a te, th e y are a ss e ss ed fo r di al ys is . Ha em o di al ys is i s a te c hni qu e w h ere by the p ati en t's b lo o d is

deto x ifi ed b y pa s si n g i t th ro u gh a m a ch i ne . A cc e ss to th e pa tie n t's ci rc u la ti o n is a c h iev ed b y fas h io n i ng a n a rteri o ve no u s s h un t, u s u al ly o n th e fo rea rm . P ati en ts u nd er go in g

ha em o di a lys is a re h ep ar in iz e d a n d de n ta l ex trac tio ns a re be st d el a yed u n til a t le as t 12 ho u rs afte rw ar ds . Th e o ptim u m tim e fo r d en ta l p ro c edu re s is 12β €“24 h o ur s po s tha em o di a lys is .

Im m ed ia tely a fter di al ys is , p ati en ts al s o ten d to be ex trem el y ti red . In dw el li ng a rter io ve n ou s sh u n ts ma y be co m e i n fe cte d a n d ma n y au th o ri ti es c o nsi de r th a t a nti bi o ti c pro p h yla xi s is

ad vis a bl e f o r o ro de n tal pro c ed u res l ik ely to p ro du ce a ba cte ra em ia in dia l ys ed pa tie n ts. A s fo r th o se w ith c h ro n ic re na l fa il ur e, c a re m u s t be ta ken wh e n pr es cri bi n g dru gs f o r th es e

pa tien ts . Ha em o di al ys ed p ati en ts h av e b ee n re po rte d a s h a vin g a n in c rea s ed ca rri ag e ra te o f he pa titi s B an d C. Ho w eve r, fo l lo w in g fa tal o u tbre aks o f h ep ati tis B a mo n g st s ta ff an d

pa tien ts i n ren a l u ni ts, d ia ly se d pa tie n ts a re reg ula rly s cr een e d fo r h e pa titis B . U n ive rs al

pre ca u tio n s fo r cro s s -in fe cti on co n tro l s ho u l d, h o wev er, b e in sti tu te d fo r a ll d en ta l p a ti en ts , reg ard le ss o f th eir m edi c al o r β € ris k β€™ sta tu s.

Table 13.10 Dental management of haemodialysed patients: some important considerations Im p ai red ex cr etio n o f dr ug s by ki dn e y Bl ee di ng ten d en c y

Hep ar in iz a tio n p rio r to di al ys is

Arte rio v en o u s sh u n ts su s c ep tibl e to in fe c ti o n An a em ia

In c re as ed c ar ria ge o f h ep ati tis B a n d C Hyp erte ns i on

P. 162 Th e o p tim u m ti me f or ren a l pa tie n ts to un d erg o d en tal p ro ce du re s is 12β €“24 h o ur s po s th a em o di a lys is .

An a l te rn a ti ve fo rm o f di al ys is is C AP D (co n tin u o u s a mb u la tor y p eri to ne al d ia ly si s), wh i ch do es n o t req u ire h o s pita li z ati o n. In d iv idu a ls o n C AP D sh o u ld b e tre a te d as h ae mo d ia ly se d pa tien ts b u t do n o t h a ve th e pro b le ms a ss o c ia ted w ith ar teri ov en o u s sh u n ts a nd he pa ra n iz a ti o n . CA P D pa tie n ts are , h o we ve r, a t ris k fro m p eri ton i tis .

Renal transplant patients

Ren a l tra n s pl an ta tio n , ei the r f ro m a tis su e -ma tch e d ca d av er or c lo s e re la tiv e, o ff ers th e be st ho p e of a n o rm al li fe . Ho w eve r, i t is n o t w ith o u t pro b le ms , m ai n ly du e to th e s i de -eff ec ts o f im mu n o su p pre s si ve reg im en s g ive n to p rev en t re jec tio n o f th e tr an s pl an te d k id ne y ( Tab le

13. 11). In th e p a st l a rge do s es o f c o rtic o ste ro id s ga ve ris e to c on s id er ab le s ho rt - an d lo n g te rm pr ob le ms ( se e Ch ap ter 3 ). Ren a l tran s pl an t pa tie nts p o se a n u mb er o f d en ta l a n d me dic a l ma n a ge me nt pro b le ms .

Table 13.11 Oral complications of renal transplantation Dru g-in du c ed g in gi va l o v erg ro w th (h yp erp la si a )

Ba c teri al a nd f un g al p la qu e s

In c re as ed i nc i den c e o f o ra l ma li gn a n cy (re po rte d) Ora l ca n di do s is

Herp es s im p lex in fe c ti o n s (s ec o n da ry)

Cic lo s po ri n A i s w i de ly u se d in a ll br an c h es o f tran s pl an t su rg ery ye t, pa ra do xi c al ly, th i s is a ne ph ro to xi c dru g . Gin g iv al o ve rgro w th i s a w el l -reco gn iz e d co m pl ic ati o n o f c ic lo s p or in ( Fi g. 13. 5), bu t c a n a ls o o cc u r w i th ca lc i um c h a nn e l b lo cke rs , su c h a s ni fe di pi ne , w h ic h ma y be

pre sc rib ed f or c on c o m itan t hy pe rten s io n i n ren a l patie n ts ( se e Ch ap ter 14). Rec e nt stu d ies

ha ve s h o wn th a t p retra n s pla n t gi n gi val h yp er pla s ia a pp ea rs to b e p o ten tia ted b y c ic l os p or in th e ra py, r es u ltin g in s e vere p o sttra n sp la n t o ve rgr ow th .

Fig. 1 3 .5 Gin g iv al h ype rp la si a in d uc e d b y c i cl o sp o rin i n a ren a l tran s pl a nt pa tie n t.

Im m un o s u pp res s ive s , gi ven to pre ve nt tis su e re jec tio n , m ay ca u s e a n u mb er of o ra l

co m pl ic ati o ns i n cl u di ng th e de ve lo pm en t o f b ac teri al (o r ca n di da l) pl a qu es , w id es pr ea d or al ca n di do s is , h erp es s im pl ex vi ru s in fe cti o n s (s ec o n da ry), s u s ce pti bil ity to de n tal i nf ec tio n s ,

an d , les s c o mm o n ly, β€ ha ir y le u ko pl ak ia β€™. Le si o n s pre se n tin g as s u pe rfici al p la qu e s on

th e o ra l m u c os a m ay res e mb le a cu te ps eu d o me mb ra no u s c a nd id o si s (th ru sh ) an d c an b e

wi pe d of f w h e n fi rml y s c rap ed . Ho w e ver, it i s no w rec o gn iz e d th a t th e in fe cti ve o rga n is m

ma y b e ba c teri al (fo r ex am pl e, s ta ph yl o co c ci , s treptoc o c ci , co l if or ms ) a n d wi ll n o t res p on d to an tifu n ga l th e rap y. T he s e p la qu e s gro w i n vi rtu al ly pu re c u ltu re an d in a d he ren t pla q u e-lik e les io n s . Tre atm en t of th es e le si o n s d ep en d s o n cu l tu re an d s en s iti vity o f p u tati ve ba c te ria . Th ere is a ls o a re po rte d in c rea s ed in c id en c e o f o ral c a rc in o ma i n ren a l pa tie n ts ta ki n g

im mu n o su p pre s si ve dru g s. Ca rc in o ma a ri si ng i n c ic los po ri n -in du c ed g in gi va l o v erg ro w th h as al so b ee n rep o rted .

Co n tr o vers y sti ll ex is ts as to w h e th e r re n al tra ns pla nt pa tie n ts s h o u ld be g ive n a n ti bi o tic co ve r f o r o ro de n tal pr o ce du re s li kel y to ge ne ra te a b a cte rae mi a . It is e ss en ti al th at the

den ti st lia is e s wi th th e p a ti en t's re n al tra n sp la n t tea m , ma n y o f w h o m ad vis e pro p h yla c tic an tib io tic co v er. A n tim ic ro b ia ls a n d o th er dr ug s that are p o te n tia ll y n e ph ro to xic to th e

tr an s pl an te d k id ne y sh o u ld b e a vo i de d. E ve ry atte mpt mu s t b e m a de to ke ep th es e pa tie n ts den ta ll y f it a n d fre e f ro m in fe cti o ns .


Wh at d ia g no s is mu s t b e e xc lu d ed in th i s la dy 's cas e? Wh a t o th er o ral m an i fes ta tio n s of th is c o nd iti o n mi gh t y o u fin d o n e xa mi n ati on ?

Th e m o s t li kel y d ia g no s is i n th is l ad y's c a se is a n a c ute fo rm o f le u ka em ia . If s h e pre se n ted on me di ca tio n , it w ou l d b e im po rta n t to c o n si de r a dru g -in du c ed a gra n u lo c yto s is . Pa l lo r a n d

fati gu e a re bo th m an i fes ta ti o n s of i ro n de fi ci en c y a n d a hi sto ry o f su s ce pti bi li ty to re cu rr en t in fe cti on s w o u ld b e co n s is ten t w i th le u ka em ia . A sec o nd a ry p la tel et d ef ic ie nc y

(thr om b oc yto p en ia ) w ill p red is po s e to s po n tan e o us bru is in g o f the s ki n an d b lee di n g fro m th e gin g iv ae . Sp o n tan e o us g in g iva l h ae mo rr ha g e, a s rep orted b y th is l ad y, i s u nu s u a l ev en i n th o s e pa tie nts w i th po o r o ra l h ygi en e a n d re in f or ces the c li n ic al su s p ic io n o f leu k ae mi a. Oth er o ra l s ig n s to ch e ck fo r in c lu de : o ra l pu rp u ra an d pe tec h ia e, g in g iva l sw e ll in g o r

in fe cti on , o ra l u lc era tio n , m u co s a l s w el li ng , an d evid en c e o f o p po rtu n is tic i nf ec tio n s , su c h a s ca n di do s is (Tab le 13. 4 ). Th ere m ay a ls o b e c e rvic a l l ym ph a de n o pa thy , ei th er as a r es u lt o f a lym ph a de n itis s e co n da ry to o ra l i n fec tio n or (le s s co m mo n l y) le u ka em ic in f iltr atio n . Q2

Ho w w o ul d yo u m an a ge th is l ad y?

Th is p atie n t mu s t b e i mm e dia te ly refe rre d fo r fu l l b lo o d c o un t. If th is is c o n si s te n t w ith yo u r cl in ic a l d ia gn o s is o f le u kae m ia , th en th e pa tie n t mu s t b e r efe rred to a h ae ma to lo g is t fo r fu rth er

in ve sti ga tio n a n d a p pro p ria te trea tme n t. A s an i n teri m me as u re thi s pa tie n t s ho u l d b e gi ven

P. 163

ap pro p ria te th era py fo r he r o ra l co n d itio n . T hi s lady h a s rep o rted s or en es s o f th e m o uth ,

wh i ch m a y b e he lp ed b y u s in g a n an a lg es ic o r an ti septi c m o u th wa s h , pa rtic u la rly if pl aq u e co n tro l is po o r. C an d id al i nf ec tio n s h o u ld be tre ate d b y ap pro p ria te to pi ca l an tif u ng a l th e ra py. S y ste mi c an tib io ti cs a re pro b ab ly b es t a void ed a t th is s tag e a s th es e mi gh t

co m pl ic ate th is l ad y's m ed ic a l m a na g em en t. Th e h ae ma to lo g is t s h ou l d, h o w ev er, b e in fo rm ed

if th e re is e vid en c e o f o ro d en tal in fe c ti o n th at may n e ed tre ati ng b ef or e d ef in iti ve th era py fo r th e le u ka em ia i s co m m en c ed . If a n y e xtra cti o n s are n ee de d, th e se m u st be ca rri ed o u t in a ho s pi tal e nv iro n m en t b ec a us e o f p o ten tia ll y s e rio u s c om p lic a tio n s, su c h a s ex ce ss i ve po s te xtra c tio n b lee di n g.


Ho w w o ul d yo u ma n a ge th e d en ta l extra c tio n o f this me di ca l ly co m pro m is ed pa tie n t?

Arr an g em en ts s ho u l d b e ma de fo r th is pa tie n t to h a ve h is ex tra cti on ca rri ed o u t at le as t 12 ho u rs po s th ae mo d ia ly si s, w h e n po te nti al b lee di n g pro b lem s a ss o c ia ted w ith h ep ari n iz a tio n

are li ke ly to be l es s (th e h a lf -lif e o f h ep ar in i s 4β€“ 6 h o ur s). A rec e nt fu ll b lo o d co u n t s h ou l d be ch e ck ed to e xc lu de th ro m bo c yto pe n ia . If th ere is n o e s ta b lis h ed l o ca l pro to c o l f or

pre op er ati ve an ti bio ti c pro p h yla xi s in h a em o di al ys ed p ati en ts , the n th e pa tie nt' s ren a l u n i t sh o u ld b e c o n tac ted fo r ad vi ce . Ro u tin e lo c a l a n ae sth e si a s ho u l d b e em pl o yed fo r th is

pa tien t. A fte r th e ex trac tio n i t is a d vis a bl e to s utu re the s o ck et a n d/ o r in s er t a h ae mo s tati c ga u ze a n d c h ec k th at th e re is n o p os to pe ra tive b leedi n g. T hi s pre ve n ti ve m ea su re is

im po rta nt as th e pa tie n t m ay h av e a te n de nc y to bl ee d af te r th e ex trac tio n . Th e pa tie n t

sh o u ld b e re vi ew ed a n d arra n ge m en ts ma d e f o r re gu lar d en ta l ca re . Q2

Wh at i s th e m o s t li kel y dia g no s is o f th e w h i te p la qu e s o n the p ati en t's o ra l mu c o s a?

Th es e tr an s ie n t w hi te pla q ue s a re li kel y to rep res en t ur ae mi c sto m ati tis a n d r equ i re n o tr ea tme n t. If th e pl aq u es ru b o ff th e y m a y b e du e to o ral c a nd id o si s, in w h ic h c as e th e pa tien t req ui re s a sy ste mi c a nti fu n ga l ag en t (f o r exa mp le , flu c o n az o le ) o r a ba c te ria l

in fe cti on . Cu ltu re a n d s e ns i ti vi ty o f pu ta ti ve m ic ro o rga n is ms w i ll in d ic ate th e the ra py o f ch o ic e.

Project

Wh at d ru gs s h o u ld be a vo i ded f or pa tie n ts un d erg o ing h ae mo d ia ly si s?

Au th o rs : Title:



Co py rig ht Β © 2003 Ox fo rd U ni ve rsi ty P re ss > T ab le o f Co nt e nt s > 1 4 - Im m u nod e ficie n c y , hy p e r se ns itiv it y , aut o im m unit y , and or al r e ac tio ns t o d ru g

th e ra p y

14

Immunodeficiency, hypersensitivity, autoimmunity, and oral reactions to drug therapy Problem cases Case 14.1

A 30-yea r-ol d fo rm er n u rs e atte n ds fo r ro u tin e de n ta l ca re a n d r epo rts th a t s h e h a s rec e ntl y bee n d ia gn o s ed by a n al le rgi s t as h a vi ng n a tu ral rub be r la tex (NR L) a ll erg y. A s a res u lt o f

th i s sh e is u n a bl e to c o n tin u e n ur si ng a n d is n o w em pl o ye d i n a n ad m in is tra tive p os t. Th is la dy al so h as a h is to ry o f h a y f eve r a n d ch i ld ho o d ec z em a . S he i s al le rgi c to ki wi f ru it a n d ba n an a s. Q1

Wh at i s th e s ig n if ic an c e o f th is l ad y's a to pi c h is to ry a n d he r pre vi o us o c c up a tio n ?

Q2

Wh y is th e rep o rted a ll erg y to f ru it o f re lev an c e?

Q3

Wh at s p ec ia l pre ca u tio n s n ee d to be tak en w he n p ro vid in g de n tal tre atm en t f o r th is la dy?

Case 14.2

A 55-yea r-ol d la dy , wi th a h is to ry o f s tab le a ng in a a tten ds f o r ro u tin e de n tal c are . S h e i s co n c ern e dβ€” on e o f he r f rie n ds h a s rea d in a m ag a zin e ar tic le th at m e dic a tio n u s ed to co n tro l an g in a c an c a u se pr o ble ms i n th e mo u th an d m ay a ffe ct the te eth . Q1

Wh at i n fo rm ati o n do y o u n ee d to an s w er th i s la dy 's en q u iry?

Immunodeficiency

Im m un o d ef ic ie nc ie s m ay be c la ss i fie d as p rim a ry o r se co n d ar y. P rim ar y im mu n o d efi ci en c ie s

rep res en t a n i n tr in s ic fa il u re in d eve lo p me n t o f o ne o r m o re pa rts o f th e im m u ne s ys tem a n d

are o fte n ge n etic a ll y b as e d. Th e y c a n be pre do m in a ntly B - or T -ce ll de fe cts , a c om b in ati o n o f bo th , o r d ue to a s el ec tiv e im mu n o d efi ci en c y ( fo r exa mp le , IgA d ef ic ie nc y) . A fe w in di vi du a ls are b or n w ith gro s s pr im ary im m u no d ef ic ie nc i es . Th es e ma y res u lt i n li fe -th re a te n in g

co n di tio n s a nd th e af fe cte d i n di vid u al s ten d to di e yo u n g, o fte n a s a r es u lt o f rec u rre n t

in fe cti on s . Can d id a in fe c ti o n s are o fte n pre do m in a n t in th e se p ati en ts (s ee Ch ap ter 4) w h o

su ff er fro m a ph th o u s -lik e u l ce ra tio n a n d h e rpe s in fe c ti o n s. T he y are a ls o m o re su s c ep tibl e to per io do n ta l d is e as e.

Ig A de fi ci en c y i s rel ati vel y mo re c om m o n th an o th er pr im ary im mu n o d efi ci en c ie s β€” lev el s of Ig A a re de cre as e d i n bo th s e ru m an d sa l iva .

Se co n d ary im m u no d ef ic ie nc i es a re th e res u lt of th e mo d ifi ca tio n an d s up pr es si o n o f s o me

pa rt o f th e i mm u ne d ef en c e me c ha n is m a s a res u lt o f a n extr in si c in fl u en c e. T hi s ma y be th e

res u lt o f so m e di se a se pr oc e ss . E n do c rin e d is ea se s s uc h a s di ab ete s me ll itu s a nd

im mu n o su p pre s si ve dru g re gi me ns ( fo r e xa mp le , ci c los po ri n the ra py ) a re co m mo n l y in vo l ve d (se e Tab le 14. 1).

Se co n d ary im mu n e s up pr es si o n m ay o cc u r i n m al ig na n cy (p a rtic u la rly in l eu ka e mi as ) a n d in

pa tien ts s u ffe rin g fr om m a ln u triti on , a s in ca n cr um o ri s. In the l atte r, it i s su g ge ste d tha t a n exte n si o n o f a c ute u lc e rati ve gi n giv iti s i n s u sc ep tib le pa tie n ts ma y o c c u r fo l lo w in g a vi ral in fe cti on , u s u al ly m ea sl es (s e e Ch ap ter 4). Iatro g en ic c a u se s o f s ec o n da ry im mu n e su p pre ss io n ar e m o s t co m m on l y a s so c ia ted w ith i m mu no su p pre s si ve dru g re gi me s,

pa rtic u la rly in th e pr eve n tio n o f reje cti o n in tra ns pl an t p ati en ts . Gro s s o ral in f ec tio n s (o fte n by Can d id a , bu t a ls o b y o th er o rga n is m s) o ften fo ll o w ed th e ma ss iv e do s es o f ste ro id s us e d un ti l re c en tly in th e m an a ge me n t o f reje c tio n . Ho w ev er, w i th the i ntro d u cti o n o f m o re se le cti ve dru g re gim e ns , th is h as b ec o me s o me w h at les s o f a p ro bl em . Lo c al

im mu n o su p pre s si ve th era py m ay al s o pro d u ce m o re restric ted e ffe cts . F o r e xa mp le , the u s e of in h a le d ste ro id pre pa ra tio n s fo r th e tre atm en t of a sth m a or th e u s e o f s tero i d-co n tai n in g mo u th wa s h es o c ca s io n al ly re su lt in o ra l o r o ro ph a ryn g ea l ca n di do s is .

Vi ral i nf ec tio n s m ay re su lt in s ec o n da ry im mu n e de fi ci en c ie s of va ry in g de gre es o f si gn if ic an c e. Th e d o mi n ati n g i n tere st i n th is

fie ld , at th e p res en t ti me , is in th e pr of o u nd i mm un os u pp re ss io n th a t m ay a cc o mp a ny HIV

P. 168

in fe cti on , a s so c ia ted w ith th e ac q ui red i mm u n e d ef ic ie nc y sy n dro m e (AIDS ). T h e o ra l ma n ife sta tio n s o f A ID S are d is cu s s ed in Ch ap ter 4.

Table 14.1 Examples of secondary immunodeficiencies Diso r der

Exa mp le

En d o cri n e di so rd er s


Ma li gn a n cy

Ac u te le u ka em ia

Vi ral i nf ec tio n

HIV in fe c ti o n

Ba c teri al in fe c ti o n

Tu be rcu l o si s

Au to im m un e d is ea se

Rh eu m ato id ar thr itis

Nu tr itio n a l d ef ic ie n ci es

Ma ln u triti o n

Dru g-in du c ed (ia tro ge n ic )

Sy ste mi c co rti co s tero i d th era py

Hypersensitivity

In co n tra st to the d is ea s es c au s ed by im m un e d efi cien c ie s, th e re are o th er s th a t d ep en d o n

an o v era cti ve o r ex ag ge ra te d res po n s e o f s o me a sp ect o f th e i mm u n e sy ste m. T he c o n diti o n s kn o wn as h y pe rse n si tivi ty r ea cti o ns a re o f th i s type , a n d th e y d ep en d o n a n en h a nc e d

res po n s e o f ei th er th e h u mo ra l o r th e ce ll -me dia te d m ec h a ni s ms , c on s eq u en t u p o n a se c on d

co n tac t w i th an an tig en to wh i ch th e h o s t h as p rev io u s ly be en se n si tiz e d. Th e se re ac tio n s in cl u de s o me in wh i ch a s e ver e to xi c ef fec t m a y b e pro d u ce d in th e h o st. Th e ter m

β€ al ler gy β€™ is n o w u se d to de sc ri be a dve rs e rea c tio ns th at r es u lt f ro m a p a tien t b ei n g

im mu n o lo g ic al ly h ype rs en s itiv e to an ex og en o u s a ge nt, th a t i s, a n a ll erg en .

Hyp ers en s iti vity rea c tio n s w ere c la ss i ca ll y d ef in ed i nto fo u r g ro up s by Co o m bs a n d Gel (Tab le 14. 2). It is , h o w eve r, c o n ven i en t to c o n si de r tw o mai n typ es : i mm ed ia te an d de la ye d

rea cti o n s. T ype I h y per se n si tivi ty re a cti on s a re imme di ate a n d me dia te d b y Ig E a n ti bo d ie s. Cli n ic al m an i fes ta ti o n s o f th is ty pe o f re a cti on ran g e fro m s ys te mi c a na p hy la xi s to

an g io ed em a , as th ma , a ll erg ic rh in i ti s , an d ge n era liz ed o r l o ca li z ed u rtic a ria . Typ e IV

rea cti o n s a re d ela ye d h ype rs en s iti vity rea c tio n s and m ed ia ted by s en s iti ze d T l ym ph o cy tes . Th e cl in i ca l ma n if es tati on s u s u al ly o cc u r a fte r 48 h o u rs , a nd e xa mp le s in c lu de co n ta ct der ma titi s du e to n ic ke l a ll erg y a n d o rga n tra n sp lan t reje c tio n .

Th e s ig n if ic an c e o f th es e h yp ers en s iti vity rea c tio ns in o ra l me di ci n e is th at the o ra l mu c o sa ma y b e di rec tly in vo l ve d o r th e o ra l tis su e s af fe cte d a s p art of a ge n era l iz ed re ac tio n . A n

al ler gi c an g io e de ma (ty pe I re ac tio n ) freq u en tly p res en ts a s sw e ll in g o f th e li ps (s ee be lo w ). A lo c al iz ed c o n tac t s to ma titis ( ty pe IV re ac tio n ) ca n o c cu r on the o ra l mu c o s a a s a re sp o n se to a wi de ra n ge o f al le rge ns , i n cl ud in g to o th pa ste s, to pi ca l an a es th etic s , a nd re si n s in co m po s ite ma ter ia ls .

Th ere ar e a n u m be r o f co n d itio n s en c o u n tere d i n o ral m e dic i ne i n w hi c h β€ al ler gyβ €™ h as

bee n i mp lic a ted bu t co n vi n ci n g e vi de nc e fo r th e se as so c ia tio n s h as y et to be pro d u ce d. T h es e in cl u de re cu rre n t a ph th o u s s to m ati ti s (RA S ), o ro fa cia l gra n u lo m ato s is (O F G), e ryth em a mu lti fo rm e, l ic h en p la n u s, a n d pl as ma c e ll gi n gi viti s.

Table 14.2 Hypersensitivity reactions Ty pe

Descr ipt ion

I

An a ph yl a cti c ty pe

II

Cyto to xic r ea cti o ns

III

Se ru m si ck n es s

IV

Dela ye d h ype rs en s iti vity

Angioedema

An g io ed em a (s o ft-ti ss u e sw e ll in g) c an b e al le rgi c or n o n -al ler gi c in a eti ol o gy a nd m ay o c cu r

wi th o r w ith o u t u rtic a ria (ra is ed w h ea ls o r pl aq u es o n th e sk in , a s in β€ ne ttle ra sh β €™ an d

of ten ref erre d to a s β€ hi ve sβ €™). M o s t c as es o f a ng io e de ma (a n d u rtic ar ia ) a re n o n -al ler gi c

an d id io p ath ic .

Ac u te a n gi o ed em a ca n , h o w eve r, o cc u r a s a n al le rgi c res p on s e to a w id e r an g e o f s ti mu l ae ,

in cl u di n g f o od s tu ffs (fo r exa m pl e, p ea n uts ), d ru gs (f o r e xa mp le , pe ni c ill in ), i n se ct bi te s (fo r exa m ple , w as p s tin gs ), a n d na tu ra l r ub be r l ate x. A ttac ks o f a cu te an g io e de ma c an res u lt in wi de sp rea d a nd o fte n d an g ero u s s of t-ti ss u e sw e ll in g tha t m a y a ff ec t th e f ac ia l , or al , an d

ph a ryn ge al re gi o ns a n d co m pr om is e th e a i rw ay. Urti ca ria ca n a c co m pa n y s o ft -ti ss u e sw e ll in g.

Th es e ma n ife s ta tio n s a re the re s ul t o f a typ e I hy pe rs en s itiv ity rea cti o n th at is IgE -

me dia te d. De gra n u la tio n o f ma s t ce ll s le ad s to th e l ib era tio n o f h is tam i ne a n d β € hi sta m in e lik eβ €™ su b sta n ce s th at c a u se a n in c rea s e in v as cu lar pe rm ea bi lity a n d re s ul t i n so f t- ti ss u e sw el li n gs . Re sp ira to ry o c c lu s io n i s lif e-th re a te n ing a nd p ati en ts mu s t b e giv en ad re na l in e

(ep in ep h rin e ), s up pl em en te d b y ste ro id s an d an ti h istam in e s as ap pr op ri ate (s ee Ch ap ter 19, β€ Me di ca l em erg en c ie s in de n tis tryβ €™).

Al le rgi c an g io ed em a a ffe cti n g th e fa c e a n d n ec k c a n be li fe -th re a te n in g . So m e pa tien ts p res en t wi th an g io e de ma (w i th o r w itho u t u rtic a ria ) th a t is n o n -al ler gi c an d

in term itte nt in n atu re . A ng io e de ma i s a we ll d oc u m en ted a dv ers e si de -eff ec t o f an g io te ns in co n ve rtin g en z ym e (A CE ) i n hi bi to rs an d c an oc c u r for the f irs t tim e ev en a fte r p ro lo n ge d tr ea tme n t. Sy mp tom a tic e pis o d es o f a n gi o ed em a ma y al so fo ll o w th e i n ge sti o n o f a

no n s tero i da l a n ti- in fl am ma to ry dru g (NS A ID), s u ch as ib u pro fe n o r a s pi rin . P a ti en ts w ith a hi sto ry s u gg es tive o f n on -al ler gi c an g io e de ma s h o uld be c he c ked f or C1 e s tera se i n hi bi to r def ic ie n cy (s ee b elo w ). Or al a n ti h is tam in e s are th e ma in s ta y o f trea tme n t fo r no n -al ler gi c an g io ed em a (a nd u rti ca ri a). R ela tiv el y n o n -se da tin g pre pa ra tio n s a re n o w av ai la bl e.

An g io ed em a is a w e ll do c u me n ted ad ve rs e, s id e-eff ect o f A C E in h ib ito rs an d c an o cc u r fo r th e fi rs t tim e eve n a fter pro l o ng ed trea tme n t.

C1 esterase inhibitor deficiency

C1 e ste ras e in h ib ito r de fic ie n cy c an be h ere di tary o r a cq u ire d an d pr es en ts a s an g io ed em a . Here di tary a n gi oe de ma i s a ra re au to s o ma l do m in a nt ge n etic d is ea s e. A s a re su l t o f C 1 es tera se i n hi bi to r de fi ci en c y, th e ac tio n o f th e c om ple m en t s ys tem is u n c o n tr o lle d an d

ac tiva tio n o f k in in s l ea ds to i n cre a se d ca pi ll ary perm ea bi li ty. In its f ul ly de ve lo p ed fo rm it i s a s e rio u s a nd l ife -th re a te n in g c on d iti on tha t m ay be p re ci pi ta ted b y mi n im al l oc a l tr au m a, su c h a s den ta l trea tme n t. C1 e ste ra se in h i bito r an d co m pl em en t co m po n en ts C3 an d C4 lev el s ca n b e

me as u red . P ro ph yl ax is a n d ma n a ge me nt o f h e red ita ry a n gi o ed em a is b y u s e of a s yn th eti c

P. 169

an d ro ge n (fo r ex am pl e, s ta na z o le ) o r, i n a n em erg enc y, f res h fr oz e n pl as m a.

C1 es tera s e in h ib ito r d ef ic ie nc y ca n b e he red ita ry o r a cq u ire d an d pr es en ts a s an g io e de ma .

Autoimmunity

In an a u to im mu n e rea c tio n th e im mu n e res p o ns e is d ire cte d ag ai n st the h o st's o w n ti ss u es , wh i ch , f or s om e rea s o n , ha ve b ec o me a n tige n ic a lly acti ve. B o th the c el l -me dia te d a n d the hu m o ra l re s po n se s ma y be in v o lve d in th e pro c es s . It is n o t k n ow n h o w th e fa ci li ty to rec o gn iz e β€ se lf β€™ is de s tro yed , a nd m an y di ffe ren t th e o rie s have b ee n pr op o se d.

A nu m be r o f di se a se s o f ac c ep ted a uto i mm u n e o ri gi n a ffe ct the o ra l ti s su e s se co n d ari ly .

Th us , i n pe rn ic io u s a n ae mi a (Ch ap ter 13), al th ou g h th e pri ma ry au to i mm u ne p ro ce s s dir ec tly aff ec ts th e g a stri c pa rie tal c el ls , th e h ae ma to lo g ic al c h an g es i nd u ce d by th e r es u ltin g in ab il ity to a bs o rb vita mi n B 1 2 ma y ca u s e m a rke d a bn o rm a liti es i n th e o ra l mu c o s a. T he s e

are th e res u lt of i ns ta bi lity o f th e ep ith e liu m i n du c ed by th e def ic ie n cy . A pa rt fro m th es e an d si mi la r s ec o n da ry eff ec ts, ho w e ver, a nu m be r o f di se a se s o f es ta bli s he d o r s u sp ec ted

au to im mu n e o rig in a ff ec t th e o ral tis s u es d ire ctl y, pro d u ci n g o ra l le si o n s as a p rim a ry sym p tom . Th e se i nc lu d e: im mu n o b ul lo u s s kin di se a se s ( Ch ap ter 11) a nd S jΓ ¶g re n' s syn d ro me (Ch ap ter 8 ). Th e de tec tio n o f au to a nti bo d ies i n p ati en ts w ith su s p ec ted au to im mu n e di s ea se i s dis c u ss ed i n Ch ap ter 2.

Oral reactions to drug therapy Spectrum of adverse reactions

Th ere is a l ar ge n u mb er of d ru gs th a t ca n c a us e ad ve rs e e ffe c ts o n th e o ra l mu c o sa a n d th e se i nc l ud e o ral u lc e rati o n (Ch ap ter 5) a nd l ic h en o id d ru g e ru pti o ns ( Ch ap ter 11).

Loc a li ze d rea c tio n s to to pic a l th e ra py, i n cl u di ng irrita n t su b s ta n c es , ca n a ls o o c cu r (Tab le

14. 3) a nd th e o ra l tis su e s ma y be a ffe cte d as a m ani fe sta tio n o f a sy ste mi ca ll y i n du c ed d ru g rea cti o n (Tab le 14. 4). On e o f th e c o mm o n a dve rs e r ea cti o ns o f th e o ra l m u co s a to d ru gs is

th a t o f ch e mi ca l trau m a fo l lo w in g co n ta ct wi th as p iri n o r a n a sp iri n -co n tai n in g tab le t o f so m e kin d . Pa tie n ts fre qu en tl y u s e th is fo rm o f th era py fo r th e re li ef o f to o th ac h e an d th e

as so c ia tio n of a ca ri o us to o th c a us in g to o th ac h e and a n ac u tel y p res e nti n g w h i te p atc h o f the ad jo in in g bu c c al m u co s a sh o u ld b rin g th is p o ss ib il ity stro n g ly to mi n d ( Fi g. 14. 1). Th e

ap pe ara n c e o f th e a ff ec ted a rea m a y b e q u ite sp ec tacu l ar an d o n ly th e h i sto ry w il l p ro vi de th e d ia gn o s is . Th e co n d itio n i s, qu i te e vi den tl y, sel f -lim iti n g a n d req u ire s n o trea tme n t

exc ep t fo r th e to o th a c he . X ero g en i c me di ca tio n i s di sc u ss e d f u lly in Ch ap ter 8 an d d ru gs ca u si n g d is c o lo u ra ti o n o f th e o ra l m u c os a a re di sc uss e d i n Ch ap ter 9. Ora l rea c tio n s to

an tib io tic s a n d s te ro ids a re di sc u s se d in th is c h a pter , tog eth e r w ith d ru g -in du c ed g in gi va l ov erg ro w th an d fi xed d ru g e ru pti o ns .

Table 14.3 Topical therapy: localized oral reactions Th er apy

Exa mp le

As pi rin

β€”

β€ To o th a c he β€™ so l u tio n s

Oi l o f cl o ve s

Ir rita n t ch e mi ca l s

Hyd ro ge n pe ro xi de u se d as ro o t c a n al ir rig an t

To pi ca l an ti bi oti cs

Ch lo rte tr ac yc li n e

To pi ca l ste ro id s

Be tam eth a s on e rin s e

Table 14.4 Systemically induced drug reactions with oral manifestations Dr ug rea ctio n

Exa mp le o f dr ug

Lic he n o id er up tio n s

NSA IDs

Ery th em a mu l ti fo rm e (S teve n sβ €“Jo hn s o n s yn dr om e)

Su l fo n am id es

Lup u s ery th em ato s u s

Hyd ral la z in e

Typ e I hy pe rs en s itiv ity rea cti o n s

Pe n ic il lin s

Fi xed d ru g eru p tio n s

Sa li cy la tes

Dru g-in du c ed b on e m ar ro w su p pre ss i on

Me tho tre xa te

Im m un o s u pp res s ive d ru gs

Cic lo s po ri n

Sa li va ry gla n d h ypo f u nc tio n

Tric yl ic an ti de pre ss a n ts

Fig. 1 4 .1 A sp iri n bu rn of th e b u cc a l m u c os a .

Oral reactions to antibiotics

It is w el l k n o wn th a t h ype rs en s iti vity rea c tio n s may o cc u r d u rin g an ti bi oti c th era py . Th es e

are co m m on l y g en e ral iz e d r ea cti o ns i n vo lv in g th e w ho le m eta bo l is m. T he ir se ve rity ma y va ry fro m a mi ld a n d tra n s ien t ras h to th e ex trem el y s evere re ac tio n o f an g io n eu ro ti c o ed em a, i n

wh i ch o e de ma a n d sw e lli n g of th e tis su e s o f th e h ead an d n ec k ma y exte n d to the to n gu e a n d la ryn x an d res u lt in d an g ero u s re sp ira to ry o bs tru cti o n . Su c h a c o nd iti o n rep res e nts a n extre me m ed ic a l e me rge n cy a n d m u s t be tre ate d a s s uch .

Hyp ers en s iti vity re ac tio n s m ay o cc u r o cc a si o n al ly fo ll o wi n g th e re pe ate d u s e o f te trac yc li n e mo u th wa s h es . Th e rea c tio n m ay o c cu r

eith e r e arl y i n th e in iti al co u rs e o r a fte r m an y trea tme n ts. T he re a cti on of ten ta ke s the f or m

P. 170

of a lo c al iz e d a n gi o n eu ro tic o e de ma w ith s w el li n g of th e e ye li ds a n d th e fa c ia l tis su e s in

gen e ra l. If thi s rea c tio n o c cu rs , i t in d ic ate s tha t th e pa tie n t h as ac q ui re d a h yp er se n si tivi ty to the te tr ac yc li n e a n d mu s t b e w a rn ed th a t fu tu re u se o f the d ru g ma y b e da n ge ro u s. T he

po s si bi lity o f th i s type o f rea c tio n is ye t an o th er a rgu m en t a ga in s t th e in d is cri mi n ate u s e o f

an tib io tic th e rap y. Oc c as i on a ll y, a l oc a li ze d fo rm o f hy pe rse n si tiv ity rea cti o n ma y be s ee n in

th e o ral ti ss u es f ol lo w in g tetra c yc li n e m o u thw a s h th era py . Th is i s rel ati vel y li mi ted in n a tu re an d le ad s to m ul tip le ve si cl e fo rm ati on . A g ai n , the a pp ea ra n ce o f th e se s ym pto m s sh o u ld lea d to th e im me di ate c es sa tio n o f th e u s e o f th e an ti bi oti c, to g eth e r w ith w a rn in g s as to fu rth er u s e. Th e i n ci de n ce o f h ype rs en s iti vity rea ctio n s du e to th e us e o f te tra cy cl in e

mo u th wa s h es i s, h o we ve r, rem a rka bl y l o w . Trea tm en t o f h ype rs en s itiv ity rea c tio n s de pe nd s

on th e se ve rity a nd a cu te n es s o f th e sy mp to ms . In a m il d re a cti o n it m a y o n ly b e n ec es s ary to di sc o n tin u e th e u s e o f th e d ru g an d to o b se rve th e p a tien t ca re fu ll y. In o th er c as es , th e

us e o f a n tih is ta mi n es a lo n e wi ll b e s u ffi c ien t to su p pre s s th e s ym pto m s. In a f u lly d eve lo p ed an g io n eu ro tic o e de ma , im m ed ia te trea tme n t w ith i n tram u sc u la r ad ren a li n e (e pi n ep h rin e) of ten c o mb in e d w i th h ydr o co rti so n e ma y be n ec es s ar y (s ee Ch ap ter 19).

Ap ar t fro m th es e ma n if es tati o ns o f h ype rs en s iti vity, th e re ar e a n u m be r o f mi no r rea c tio n s to an tib io tic s th a t ma y be l oc a li ze d to th e m o u th. Bl ac k h ai ry to n g u e o c c as io n a ll y fo l lo w s

tr ea tme n t w ith e ith er w ide - or n arr o w -sp ec tru m an tib io ti cs . A s th e e ss e nti al f ea tur e o f bl ac k ha ir y to n gu e is e lo n g ati on of th e fil if or m pa pi lla e a nd th is i s as s o ci ate d o n ly wi th s ec o n da ry ba cte ria l ch a n ge s, i t is d if fic u lt to u nd er sta n d w hy an ti bi oti c th era py s ho u l d i n du c e th i s

pa rtic u la r fo r m o f re ac tio n . F o llo w i ng th e ce s sa tion o f th e th e rap y th e to n gu e m ay retu rn to no rm a l e ith e r qu i ck ly o r, o cc a si o n al ly, v ery s lo w ly. Th is fo rm o f re ac tio n m a y o c cu r af ter

eith e r s ys tem ic o r l oc a l an tib io ti c th e ra py, a lth o ug h mo s t c as e s of b la ck h ai ry ton g u e a re o f un k no w n a eti o lo gy .

It mi gh t w e ll be th o ug h t th at the u s e of an ti bi oti c mo u th w as h es to tr ea t o ra l l es io n s w o u ld

lea d to s ym pto ms a ri si n g f ro m th e w i de sp rea d lo c al o ve rgr o wth o f re si sta n t o rga n is m s an d , in p arti cu l ar, Can d id a sp p, bu t, in fac t, th is i s n ot o ften s o . P rec i se ly as g en e ra l p h ys ic ia n s ha ve fo u n d it un n e ce ss a ry to in c or po ra te a n tif un g al an ti bi oti cs w i th w id e- sp ec tru m

an tib io tic s i n o rde r to a vo i d g a stro i nte sti n al in fes ta tio n s b y y ea sts , s o it ha s b ee n fo u n d un n e ce ss a ry to co m bi n e a n tifu n g al an ti bi oti cs w i th w id e-sp ec tru m an tib a cte ria ls i n

mo u th wa s h th era py fo r o ral u lc era tio n . Th i s do e s not me an , h o w ev er, th a t s uc h a n

ov erg ro w th is n o t p o ss ib le an d i t mu s t a lw a ys be reme m be red th at an y lo c al iz e d u s e o f a n an tib io tic m a y le av e b eh i n d r es is tan t s tra in s o f orga n is ms , e ven i f no cl in ic a l o v erg ro w th

oc c u rs. It is , th ere fo re, wo rth r eite ra tin g tha t the u se o f an ti bio ti c mo u th w as h es s h o u ld be res erv ed fo r si tua tio n s i n w hi c h th ere a re p o s itiv e in d ic ati o ns .

Oral reactions to steroids

Th ere a re no w m a n y p ati en ts tak in g sy ste mi c s te ro ids o n a lo n g -te rm ba s is , a nd o fte n th is tr ea tme n t res u lts i n su s c ep tibi li ty to i n fec tio n and ge n era l lo s s o f tis s u e r es is tan c e. In th e

mo u th , th is ta kes th e fo rm o f ac u te eryth e ma to u s can di do s is i n mo s t c as e s, a lth o u gh ac u te

or c hr on i c ps eu d o me mb ra no u s c an d id o si s ma y al so oc cu r. A lth o u gh th e pa la te is m o st of ten aff ec ted , th e w h o le o f th e o ra l an d p ha ryn g ea l mu c osa m a y b ec o me in vo l ve d, a s ma y the

mu c o sa o f the l ary n x. S ec o n da ry in fe cti o n by Can d id a al bi ca n s is a lm o st in va ria b ly pre se n t, an d trea tm en t o f th e se p ati en ts by lo c al a n tifu n g al th era p y i s of ten s a tis fa cto ry in r ed uc i ng

sym p tom s . In s eve re ca s es a s ys tem ic a n tifu n g al a gent ma y be req u ire d. T hi s , h o we ve r, c an be n o mo re th a n a tem po ra ry so l u tio n s in c e th e se p ati en ts ar e, i n ge n era l, d es tin e d to ma in ta in s tero i d th e ra py fo r a n i nd ef in ite p eri o d. It m a y, th ere fo re, be n ec es s ary to

rep ea ted ly trea t th is c a nd id al i nf ec tio n . In th es e pe rs is ten t c as e s of c an d id al i nf ec tio n th e lo n g-te rm u se o f a to pic a l an tif u ng a l pre pa ra tio n is req u ire d.

Ste ro id m ou th w a sh e s a n d o the r to p ic al pre pa ra tio n s a re a d vo c ate d f o r a n u mb er o f

co n di tio n s in or al m edi c in e an d m ay o bv ia te th e n eed f o r s ys tem ic s tero i ds . Th is f o rm o f

th e ra py mi gh t b e exp ec ted to be a p ro li fic s o u rce of ca n di do s is b u t, in f ac t, th is c o mp li ca tio n is rel ati ve ly u nu s u al . If i t d o es o c cu r, l o ng -te rm a nti fu n ga l me as u re s mu s t ac c o mp an y th e

an tib io tic β €“ste ro id th era py . Mi c on a z o le ge l is o fte n ve ry h e lp fu l in th e se c irc u ms ta n ce s, pa rtic u la rly if th e pa tien t ha s a s o re m o u th a nd c ann o t to le rate n ys tati n pa s till es o r

am ph o ter ic in l o ze n ge s. P a tie nts w h o re gu l arl y u s e a ste ro id i nh a le r f or res p ira to ry d is ea s e,

su c h a s as th ma , a re pre di sp o se d to de ve lo pi n g o r al c an d ido s is , p ar ti c ul ar ly o f th e pa la te an d or op h ar yn x. Th e y sh o u l d a lw a ys ri n se th ei r m o u th afte r u s e o f th e ste ro id a n d, i n so m e ca se s , a sp a ce r d ev ic e m a y b e in di ca ted ( Fi g. 14. 2).

Drug therapy and the periodontal tissues

Dru g-in du c ed g in gi va l h y pe rpl as ia (o ve rgro w th ) i s a w ell re co g n iz ed c o mp li ca tio n a n d th e

dru gs m o s t c om m o nl y im pl ic a te d are p h en yto in , c i cl osp o rin , a n d c a lc iu m -ch a n ne l bl oc ke rs (n ife di pi ne , ve rap a mi l, d ilti az e m). Ora l co n tra c epti ve s ha ve b ee n as s o ci ate d wi th a

hy pe rpl as tic o e de ma to u s gin g iv itis b u t it is li ke ly tha t th is r es po n s e i s a se c o nd a ry re a cti on to pl aq u e a n d is th ere fo re d epe n de n t o n th e s ta n da rd o f pl aq u e co n tro l.

Fig. 1 4 .2 S te ro id i nh a le r w ith a s pa c er dev ic e.

P. 171

Gingival overgrowth and sodium phenytoin (Epanutin)

So d iu m ph e n yto in i s th e m o s t co m m on l y u s ed d ru g f o r th e trea tme n t o f e pi le ps y a n d , in

ap pro xi m ate ly 50 p er c e nt o f th e pa tie n ts ta ki n g i t, th ere is a m a rke d c h ro n ic h yp er pla s tic

gin g iv itis . Th e h yp erp la sti c rea c tio n is ty pi ca ll y p a pil la ry an d th e in terd en ta l pa pi ll ae b ec o me sw o ll en , s o me tim es g ro ss ly s o . Th is i s es se n tia ll y a fi bro b la sti c rea c ti o n a nd th e tis su e i s

gen e ra lly fi rm a nd m u ch l es s h ae mo rr ha g ic th an in th e ca s e of p reg na n c y g in g ivi tis . In vi ew

of th e ma rke d fa ls e po c ke ti n g th a t m ay o c cu r, th e re are g rea t d iff ic u ltie s in m a in tai n in g o ra l hy gi en e an d s ec o n da ry in fl am ma to ry ch a n ge s are a lm ost in va ria b ly pre se n t. Th e n atu re o f

th e re ac tio n to th e d ru g i s n o t cl ea r s in c e th ere is e vi de nc e th at th e h ype rp la si a rep res e nts an e xa gg era ted f or m o f c h ro ni c gi n gi viti s, w h ic h d oes n o t o cc u r i f i mm a cu l ate s tan d ard s o f

or al h yg ien e a re ma in ta in e d. A lth o u gh w ith dra w a l o f th e d ru g is , in i tse lf , su f fic ie n t to h a lt

th e p ro gre ss o f th e co n d itio n , th e di ffi c ul tie s of s tab il iz a ti o n in an e pi le pti c p a tien t ma y m a ke it im pra c tic al to s u gg es t i ts w i thd ra w al a s a trea tme n t fo r th e o ra l c o n di tio n . S tr in ge n t o ra l

hy gi en e an d re gu la r sc a lin g , pre c ed ed , if n eed b e, b y gi n gi vec to my , mu s t b e c a rrie d o ut. In

se ver e c a se s, th e g in gi ve cto m y s h o u ld be pe rfo rm ed u n de r h o s pi tal c on d iti on s . A lth o u gh th e pro li fe rati o n is e ss e nti al ly fi bro b la sti c, th e s ec on da ry i n fec tiv e pro c es s es o fte n res u lt in th e pro d uc tio n o f v ery va s cu la r tis su e , an d b lo o d lo s s m a y b e co n s id era bl e if su rg ery i s

un d erta ke n du ri n g th i s ph a se . Hi sto lo g ic a lly , n o ne o f th e d ru g-in du c ed g in gi va l ch a n ge s sh o w

tr ue h yp erp la s ia , bu t th e re is a n in c rea s e in c o ll ag en a n d gro u n d su b s ta n c e.

Gingival overgrowth and ciclosporin

Cic lo s po ri n is a n i mm u n os u p pre ss iv e dru g th a t is n ow w id el y p res c rib ed i n al l b ra n ch e s o f

tr an s pl an t s u rg ery an d in c rea s in gl y us e d f o r s ys temic d is ea se s s uc h a s rh eu m ato i d arth ri tis . Cic lo s po ri n -in du c ed g in gi va l o v erg ro w th (Fi g. 13. 5 ) in ren a l pa tie nts w a s di sc u s se d in Ch ap ter 13 .

Gingival overgrowth and calcium-channel blockers

Th es e dru g s a re u s ed ex ten s ive ly fo r th e m an a ge me n t o f c a rdi o va sc u la r d is o rde rs , in c lu d in g

an g in a, h ype rten s io n , a n d ca rd ia c arr hy thm i as . Ni fed ep in e , on e o f the d ih yd ro py rid in es , h as

bee n re po rte d a s c au s in g g in gi va l o v erg ro w th in 10 to 15 pe r c en t of d en tate pa tie n ts. Re n al pa tien ts m ay b e ta ki n g c ic l os p or in to p rev en t re jectio n o f th ei r tra ns p la nte d kid n ey a nd a ca lc iu m -ch a n ne l bl oc ke r to c o n tro l h ype rten s io n .

Drug s co mmo nly imp licat ed in d rug- ind uced gingival o verg ro wt h

Ph e ny to in

Cic lo s po ri n

Cal c iu m -ch a n ne l bl oc ki n g dru g s

Fixed drug eruptions

Th e i mm u n o lo gi c al m ec h an is m in v o lve d in fi xe d dru g eru p tio n s h as y et to b e el u ci da ted bu t it wo u ld a pp ea r th a t th es e rep re se n t a typ e o f de la ye d h y pe rse n si tiv ity r ea cti o n. Th e o ra l

mu c o sa i s rare ly a ffe cte d an d m o st les i on s o c cu r o n th e s ki n . Fi xe d d ru g rea c tio n s o cc u r a t

th e s am e si te ea c h tim e th e p rec ip ita tin g dru g is ad mi ni s te re d. O ra l l es io n s c an oc c u r o n th e pa la te, l ip s, o r ton g u e a n d th ey fre qu en tl y s tar t as v es ic le s o r b u lla e , wh i ch ra pi dly b rea k

do w n to fo rm u l ce rs. Dru gs c o mm o n ly im pl ic ate d in fi xe d d ru g eru p tio n s in c lu d e: sa li cy la tes , da ps o n e, tetr ac yc li n es , an d s u lfo n a mi de s. B arb itu rates h a ve be en rep o rted as c a u si n g o ra l les io n s i n th e a b se n ce o f s ki n in vo l ve me nt.


Wh at i s th e si gn i fic a n ce o f th i s la dy 's ato p ic h is to ry an d h er pre vi o us o cc u pa tio n ?

Ato p y (th a t is , a h is to ry o f as th m a, e cz e ma , h a y f eve r) i s a ris k fa cto r f o r d eve lo p in g NRL

al ler gy. Oth er pa tie n ts ide n tifi ed a s β€ at r is k β€™ in c lu de th o se w ith lo n g -te rm ex po s u re to

NRL-co n tai n in g ite ms , th at is , h ea lth -ca re w or ker s ( la tex gl o ves ) a n d pa tie n ts w ith s pi n a bif id a (u rin a ry c a th eter s). Q2

Wh y i s the re po rte d a l ler gy to fru i t o f rel ev an c e?

Th ere is a re po rte d cr o ss -se n si ti vi ty b etw ee n NRL an d c erta in f ru its (f or ex am pl e, b a na n a s, kiw i fru i ts , a vo c ad o s) . Q3

Wh at s p ec ia l pre c au tio n s n ee d to be ta ke n w he n p ro vid in g de n tal tre atm en t f or th is la dy?

Th is la d y n e ed s to be trea ted i n a la tex -co n tro ll ed, de n ta l en v iro n me n t. No n -NRL g lo ve s mu s t be w or n by a ll sta ff di rec tly in v ol ve d i n h er c are a n d i tem s su c h a s NRL da ms a n d po li s hi n g cu p s mu s t n o t be u s ed . No n -NRL d en ta l a n d me di ca l eq u ipm e nt ca n b e s u bs titu te d. Lo c al

an a es th etic c a rtrid ge s w ith s yn th etic ( no n -NRL) b u ngs a re av ai la bl e to av oi d the po te nti al

ris k o f c on ta mi n ati o n o f th e lo c a l a n ae s th e tic s o lutio n . E m erg en c y eq u ip me nt w ith n o n -NRL mu s t a ls o be a va il ab le (s ee lo c a l a n d n ati on a l gu idel in e s fo r f u rth er a d vic e ).


Wh at i n fo rm ati o n do yo u n ee d to an s w er th i s la dy 's en q ui ry?

Yo u w ill o b vio u s ly req u ire a li s t o f th is l a dy' s curre n t m edi c ati on , w hi c h ca n th en be c he c ked

ag ai n st a n a tio n a l d ru g fo rm u la ry a n d ma n u fa ctu re rsβ€™ dru g d ata s h ee ts. B o th l is t po te nti al si de -eff ec ts o f th e dru g s.

Sta bl e an g in a u su a ll y re s ul ts fro m a the ro s cl ero ti c pla q ue s in th e co r on a ry arte rie s . Mo s t

an g in a pa tie n ts w i ll h av e a n i trate (g ly ce ryl tr in itra te (GTN ) o r i s os o rb id e n i tr ate ) fo r u se du rin g a n a cu te atta c k, ei th er as a ta bl et o r a s pra y fo r s u bl in g ua l u se . GTN

is a ls o a vai la bl e as a fa st -rel ea se ta bl et th a t is p la c ed be tw een the u p pe r li p an d la b ia l

P. 172

mu c o sa a n d le ft to d is s o lve . M an y pa tie n ts w i th an gin a m ay req u ire re gu l ar dru g th era py a n d

wi ll h av e bee n p res c rib ed pr op h yl ac tic a sp ir in . M ain te n an c e dru g s fo r th e trea tme n t o f an g in a in cl u de b eta -blo c ke rs , ca lc iu m -ch a n ne l bl oc ke rs (for ex am pl e, n i fed ip in e ), an d n ic o ra n di l (a po tas s iu m -ch a n ne l bl oc ke r).

Th e p o ten tia l o ral (d en ta l) si de -eff ec ts o f th es e dru g s in c lu d e th e fo ll o w in g.

Glyc e ryltr in itra teβ €”rap id re le as e β € bu c ca l pre pa ra tio n β€™ th a t co n ta in s la c to se ; th is

Be ta bl oc ke rs β€”xero s to mi a (s al iva f lo w re du c ed β€”se e Ch ap ter 8).

Cal c iu m- ch a n ne l bl oc ke rs (n if ed ep in e) β€”gin g iv al o ver gro w th .

Nic o ran d il β€”or al u lc era tio n .

ca u se s a pro n o u n ce d dro p in pH, wh i ch p re di sp o se s to de n tal c ari es .

Yo ur pa tie n t s ho u l d b e rea s su re d tha t th e p o ten tia l o ral a n d d en ta l si de -eff ec ts o f m ed ic ati o n fo r a n gi na a re m in o r an d c an be m an a ge d. It i s im porta n t th at thi s la dy a dh er es to m ed ic al ad vic e co n c er ni n g h e r m ed ic a ti o n a nd th e c or rec t d os a ge . M os t p ati en ts s u ffe rin g fro m

an g in a rec e ive d en tal tre atm en t i n ge n era l pra c tic e bu t i t is e ss en ti al th at ap pr op ri ate dr ug s an d eq u ip me n t ar e a va il ab le i f th ey d eve lo p a n an g in a a tta c k. It is a ls o i mp o rtan t th a t s taf f are a de qu a tel y tra in e d to dea l w ith a ll m ed ic al em erg en c ie s (s ee Ch ap ter 19).

Projects

Den tis ts an d d oc to rs a re req u ire d to rep o rt an y a dve rs e r ea cti o n s th a t th ey s u sp ec t a re du e to dru g s th a t th e pa tie n t h as be en p re sc rib ed . 1.

Wh at me c ha n is m s are i n pl ac e fo r th e re po rtin g o f ad ve rse d ru g rea c tio n s?



Copyri gh t Β©2003 Oxfo rd Un ive rsity P ress > Tab le of Cont e nts > 15 - Facial p ain and ne ur olog ical d ist urb anc e s

15 Facial pain and neurological disturbances Problem cases Case 15.1

A di stress ed 60 -year-old patie nt w ith fac ial pai n presen ts to the ora l med ici ne cli ni c. After

ta kin g a his tory a nd carryi ng ou t an exa min atio n, yo u excl ude a d ental ca us e fo r the pai n a nd

make a cli ni cal dia gn os is of trig emin al ne ura lgia . Yo u dis cu ss the medic al an d s urgi cal op ti on s for th e ma na geme nt o f thi s c on ditio n β€”in th e fi rst in stan ce the p atien t requ ires so me medic atio n . Q1

Wh at is the us ua l dru g o f c ho ic e fo r thi s pa ti en t a nd wh at startin g do se wo u ld yo u

Q2

Wh at w arn ing s a nd in forma tio n w ou ld you gi ve to th e patie nt?

Q3

Wh at in vestig atio ns do you n eed to c arry o ut be fore startin g o n th is drug ?

Q4

If you r pa ti ent ha d pres en te d takin g w arfa rin , w ould you en vis age an y ma na gemen t

presc ribe?

prob lems ?

Case 15.2

A 25-year -old , h ea lthy f emal e pa ti en t i s rec eivi ng tre atmen t in you r su rgery. After

admi ni stratio n o f a lo ca l an ae sthe ti c ma nd ibu la r nerve blo ck (for cro wn prep aratio n of th e

man dibu la r firs t perma nen t mo lar), yo ur p atien t repo rts th at th e lef t s ide of he r fac e fee ls

stran ge. Sh e i s u na ble to c los e h er le ft eye o r sm ile. Yo u ha ve prep ared less tha n ha lf o f the cro wn . Q1

Ho w wo ul d yo u man age this situ atio n?

Facial pain: an overview

Patie nts with a c om pla int o f fa cia l p ain are see n da ily in th e d enta l pra ctitio ner' s s urge ry .

Th ey are o ften dia gno se d an d tre ated w ith re lative eas e, bu t th ere re main s ome few patie nts in wh om th e ori gin of th e p ain s ymptom s rem ain s o bscu re an d w h o a re refe rre d to spec ia lis t cli nic s o f va rio us kin ds for diag no si s. Ap art from thes e pa tien ts wi th s ympto ms of pai n, very few p atien ts p re sen t wi th o th er n eu rol ogi ca l di sturba nc es aro un d th e m ou th a nd fac e. The

diag no si s o f the se m ay b e a c o mplex matter an d o u tside th e p rovi nc e o f den tistry, bu t it is impo rtan t th at the den ta l p ra ctitio n er is abl e to re co gn ize sig ni fica nt c ha ng es a nd in itiate

furth er ac ti on . Ta ble 15. 1 gives so me of th e c on di ti on s th at mig ht gi ve ris e to fac ial pai n. Th e m atte r may be fu rther co mpli cated by th e diffi cu lties tha t may b e met by th e p atien t in

desc ribi ng his o r he r symp to ms acc u ra tely. On the wh ol e, ho we ver, th e terms u sed by pa ti ents to des cri be pai nβ €”β € stabb ing β€™, β€ th rob bin g β€™, β € dul l ac he β €™, an d so o n β€”are fairl y co ns ta nt a nd rela te d to th e ae ti ol og y of th e p ain .

Th e reac ti on o f the patie nt to pain de pen ds o n two fac tors th at a re in dep end ent o f th e

streng th o f the pai n s timu lu s. The se a re the pai n th resh o ld fo r the patie nt (th e d egree o f

stimu latio n ne ces sa ry fo r th e p atien t to pe rceiv e pa in) an d the in divid ua l's sen si ti vity to th e

pain w hen pe rcei ved. The se tw o f acto rs va ry greatly from patie nt to patien t an d, in the cas e of th e in di vidu al sen si ti vity, may v ary fro m time to tim e in the sam e pa ti ent, dep end in g o n

gen eral hea lth a nd oth er ps ych ol ogi ca l fa ctors . Pain c an no t be sepa rated from an emo tion al re spo n se.

Table 15.1 Conditions that may cause facial pain Den tal pain

Pu lp itis, cra cke d too th, perira dic ul ar pa th o log y, peric oro ni ti s, dry so cket

Te mpo rom an dibu la r pai n d iso rders Ne uro path ic pain dis orde rs

Trige min al neu ral gia, glo ss op ha ry nge al n eu ralg ia, po sth erpetic n eura lgi a

Path ol ogy in ass o cia te d lo ca l s tru ctu res (i .e. s alivary g lan ds, pa ran as al sin u ses , eyes, c ervic al s pin e d iso rders , n as o pha rynx, ears ) Vas cu la r dis orde rs

Hea dac hes : migra in e, clu ster h ea dac he, ten sio n -typ e hea dac h e Gia nt ce ll a rte ritis

Psyc h oge nic fac ial pai n Atypi ca l fa cia l pai n

In tra cra ni al les io ns Neo pla sms

Mu ltipl e sc lero sis

Re ferred p ain fro m a remo te site An gi na pec to ris

Facial pain

P. 176

Pai n i s a co mmo n an d freq uen t o ccu rren ce experie nc ed b y mos t patie nts. P ain ma y be acu te

or c hro ni c. Pa in no t o nly ha s c on se que nc es to th e pa tien t an d hi s o r h er qu al ity of life , b ut it can al so h ave a n impa ct o n th e p atien t's s oc ial ci rc le an d o n th e so c iety an d e co no my o f a nati on . P ai n i s respo n sib le fo r a larg e pro po rti on o f mis se d da ys o f w ork.

Th e majo rity o f pa ti ents with fac ia l pa in are s uf ferin g fro m s om e eas il y detec ta ble

patho lo gi cal pro ces s i n th e teeth, th eir s upp ortin g tis su es, o r as so ci ated struc tures . Wh ateve r th e n ature of th e p ain an d h o weve r un u su al i ts prese ntatio n , th e fi rs t step mus t be th e

elimi na tion o f the pai ns o f lo ca l ori gin β€”su ch as abn o rmal pul ps, bu ried te eth a nd roo ts,

peria pic al l esi on s, o r acu te pe riod on tal lesi on s. Od on tog eni c pa in may be rea dil y dia gn os ed b y cli nic al an d rad io graph ic exam in atio n. Un fo rtu na tely, every den ta l pra ctitio ne r kno w s th at th is is n o t a lw ays so strai gh tfo rwa rd. Fa cia l pa in o f den tal o rig in may b e diffi cu lt to re co gn ize an d re ferred or pro jec te d pa in may m ake l oc ali za ti on diffi cu lt. Clin ica l a nd radi ogra ph ic

exami na ti on ma y be i nc on cl us ive. V itali ty te stin g of the te eth a nd th e u se of dia gno sti c lo ca l

ana es th etic in je ctio ns ma y be u sef ul aid s. Pu lpi ti s ma y be diff icu lt to loc al ize , e spe cia lly i n a

hea vily resto red qu ad ran tβ€”so metimes res to ratio n s h ave to be remo ved a nd te eth d ress ed in a sy stema ti c ma nn er. Thi s i s fru stratin g f or bo th th e c lin ic ian an d th e patie nt. In pa rti cu lar,

th e pa in as so cia ted w ith tem poro ma ndi bu lar jo in t dysf un ctio n may b e very p uz zl in g an d m ay simu la te a n u mber o f o ther co nd ition s .

Th e d iag no si s o f fa cia l pa in ca n b e di ffic ult d ue to the mu ltifa ctori al n atu re o f the prob lem.

The nerve supply to the face

Th e s en so ry n erve s upp ly to the fac e a nd ora l tiss ue s i s s ha red a mon g a nu mb er of ne rves: th e trige min al nerve ; the g lo ss oph aryn gea l n erve; and the b ran ch es o f th e c ervica l p lexu s

(s ee Fig. 15.1 ). Ho we ver, th e gre at majo rity o f pa in symp to ms in th e f ace are f elt in the area co vered b y the trig emin al ne rve. There is us ua lly a cl ear -cu t d istin ctio n betwe en th e z o nes

su ppli ed by th e va rio us te rmin al b ran ch es o f th e trigem in al n erve, w ith v ery little ove rl ap, bu t th ere is often co n sid erabl e o verla p betw een the trig emin al an d c utan eo us bra nc hes o f th e

cervic al plex us wh ere th ese are a djac en t. Ap art from are as of overl ap th ere is als o a co mp lex

serie s o f in terco n nec tion s betwe en th e trige min al, fa cia l, an d glo ss op ha ry nge al n erves an d th e nerve s a ri sin g f rom th e a uto no mic ne rvou s sys te m, in partic ula r, th e s ympa th etic fibre s

ass oc ia ted wi th bl oo d ve sse ls servi ng th e a rea. Th ese symp athetic fi bres m ay p lay s o me pa rt in th e tra ns mis sio n of deep imp uls es. It ha s b een sh o wn th at s uc h pain c an be p rodu ce d by th e s ti mul atio n of th e s upe rior cervi cal sym pathe tic g an glio n .

Th e g reat ma jority o f p atien ts c omp lai nin g o f p ain in an d a bo ut th e fac e are s uf ferin g fro m

so me fo rm o f to otha ch e. Ho weve r, th ere are man y o ther po ssi ble cau se s o f s uc h p ain ( Ta ble 15. 1). Th e s tru ctu res related to th e mo uth tha t migh t give rise to pa in sym ptoms are so

co mple x, a nd th e i nn ervatio n of th es e s tru ctu res s o interrel ated, tha t errors in di agn os is are

eas ily m ade. Th e ma in sen so ry n erve o f th e are a, the trig emin al ne rve, even tu al ly di vide s in to a la rge n um ber o f s mall termin al bran ch es su ppl ying the skin o f a larg e pa rt o f th e fa ce a nd sca lp a s w ell as the majo rity of the o ral ti ss ues an d m an y deep er stru cture s. Al th ou gh the

su perfic ial li mits o f th e tri gemi na l n erve c an be acc ura te ly determ ine d w ith l ittl e o verla p, th e

deepe r limi ts are mu ch les s w ell defi ned an d u nd ersto od. It is often di ffic ul t to determin e th e poi nt a t w hi ch a f aci al pai n be co mes a h ea dac he (for e xamp le, in th e temp ora l reg ion ) a nd

th ere m ay be co ns equ en t diffi cu lties in co mmu n ica tion betwe en th e pa tien t and the cli nic ia n.

Fig. 15 .1 Sen so ry derma to mes o f the tri gemi na l an d u ppe r cervi ca l nerve s.

An oth er po ss ible so urc e o f co n fus io n wh en diag no si ng oro fac ia l pa in is th at o f pro jected an d re ferred pain .

Projected and referred pain

If a pa in path wa y is su bjected to stimu la ti on at s om e po in t alo ng its c o urse , i t is pos si ble f or pain to be fe lt in th e p eriph eral dis tri bu ti on o f the nerve . Th is is pro je cted p ain . An exa mple of th is is th e fa cia l p ain tha t may o cc u r in patients w ith in tra cran ia l neo pl asm s.

If th e s ou rce of a pa in an d the si te of th e p ain (th e l oc atio n) a re o ne an d the sa me, the p ain is c on si dered to b e prim ary pa in . Thi s

mus t be di stin gu ish ed from referred pai n i n w hi ch the pain i s fel t in an area dis tan t f rom th at in wh ich the ca us ative p atho lo gy i s l oc ated. P ain referral is a c om mon o cc urren ce in the oro fac ial regio n an d c lin ic ian s m us t app re cia te this ph en om eno n β€”othe rwis e erro n eou s

diag no se s a nd m an age men t m ay ens ue . In referre d pa in th e p ra ctitio n er mu st be abl e to

iden tify th e true so urc e o f the pai n f ro m th e s ite (lo ca ti on ) o f the pai n. In th e de ntal fiel d,

perha ps th e m os t co mmo n e xamp le o f refe rred pa in is th at i n w hi ch pai n is f elt, for ins tan ce, in th e ma xill ary teeth du e to a le sio n in a m an dibula r to o th . In th is ca se th e pa in imp uls es

orig ina te in the dis eas ed to oth an d, if th e pa th wa y from th is too th i s b loc ked, fo r exa mple , b y

P. 177

a lo ca l a na esth etic, the n th e referred pai n w ill cea se . Th us , in ou r den tal examp le,

ana es th es ia o f th e man dib ula r bra nc h o f th e trigem ina l n erve w il l reli eve th e pa in felt i n th e area su ppli ed b y the m axil lary bran ch . Th is is the bas is o f a mo st u sef ul te st fo r th e inve stiga ti on o f s us pected referred pai n.

Alth ou gh pai n i s frequ en tl y referred betwe en diffe rin g bran c hes o f the tri gemin al ne rve, pai n

re ferred to the fac e fro m a dis ta nt a rea i s rel ativel y un c omm on . The o n ly referred pai n o f th is typ e se en with an y deg ree o f freq uen cy is th at in coro na ry in su ffic ien cy, wh en pa in may ra dia te ov er the left s ide o f th e ma ndi ble. A ng ina pec to ris ha s a lso be en des cribe d as

prese ntin g a s a pai n o f th e so ft pa late, bu t th is is un u su al. Th is a cc om pan ies o th er w ell -

re co gn ize d sy mptom s o f the co nd ition , bu t a very few ins tan ces ha ve be en repo rte d in wh ic h pain o ver the left ma nd ible ha s b een the f irst c omplai nt o f a patien t wh o su bs equ ently devel ope d sym ptom s o f myo ca rdia l in fa rc tio n.

Th e m ech an is m o f proje cted p ain is fai rly c lear, but tha t o f re ferred p ain is mo re c on tentio u s. Th ere is s till co ns ide ra ble deba te as to th e the orie s o f pa in referra l a nd th e site a t wh ic h th e β€ cro ss -over β€™ o r ne ural co n vergen ce oc cu rs l eadi ng to th e sen so ry mi sin terpretatio n of

th e im pu lses tran smi tted from the d ise ase d site.

Referred pain

Pai n o f d enta l o rigin c an be ref erred to the op po sin g d ental arc h.

The evaluation of facial pain

Pai n i s e xtrem ely su bjectiv e an d i n m an y in stan ces the re are few cli nic al sig ns . P ai n n eed s to be ass es sed on two di men sio ns β€”as a di sea se, u sin g a med ica l mo del , a nd as an ill nes s th at ine vitabl y ha s a n effec t on the qua lity o f l ife. The res pon se of patie nts to pa in is e xtre mely

varia ble a nd it is thi s tha t make s a sse ss men t o f pai n s o diffi cu lt. S o me pa in ac ts as an ea rly warn in g s ystem a nd so is cru ci al for su rvival , w he reas oth er pa in s h ave ou tlived thei r

us eful ne ss. M an y of th e cli ni cal co nd itio ns se en b y the den ta l s urge on se em to fal l in to a

categ ory i n wh ich mi no r (o r even qu ite u nrec o gni za ble) patho lo gi cal ch an ges eli cit a qu ite disp ropo rtio na te resp on se lea din g to great di stres s in the in divi dua l co nc ern ed.

Th e a ss ess men t an d di agn os is of oro fac ia l pa in require th e cl in ici an to h ave kno wl edge an d un ders ta ndi ng of th e a na to my, phy sio lo gy, an d pa tholo gy o f the he ad a nd ne ck a nd, i n

partic ula r, of th e oro fac ia l stru cture s. The cli ni cia n sh ou ld b e ab le to un derta ke a system atic

cli nic al as ses smen t as sh o wn in Ta ble 15. 2. Th e in vestig atio n o f a patie nt c omp lai nin g o f f aci al pain ma y be th o ugh t of as ha vin g two sta ges. In th e fi rst sta ge a n a ss ess men t of th e pa in is made rela ti ve to the den tal a ppa ratus (teeth an d suppo rtin g tiss ue s) a nd th e c lo sel y rela te d struc tu res , s uc h as th e ma xill ary a ntra an d the te mpo roma nd ibu lar jo in ts. If, after fu ll exami na ti on , no ab no rmal ities are fou n d in thes e a reas , th e in vestig ator i s ju stifi ed in

co ns ideri ng oth er po ss ibil ities . If th e es sen tial first s tep o f el imin atin g d ental ca us es of th e

pain is o mitted, co nf us io n i s bo un d to fol lo w i n many ca ses . Wh en all thes e lo c ali zed ca us es o f pain a re elim in ated by ca re ful in vesti gatio n, the re rema in s a nu mbe r of co nd ition s of les s evide nt o rigin fo r co n sid eratio n in a po ss ibl e di agn os is .

History

A de ta iled pai n his tory i s c ruc ial fo r patie nts w ith fac ial pai n a nd in 70β€“80 pe r cen t of cas es

a di agn os is is made on h isto ry al on e. The cli ni cia n c an ga in valu ab le i nfo rma ti on by o bs ervin g th e patie nt wh en h e o r sh e is des cri bin g the featu res of th e pa in . It is al so impo rtan t th at the patien t is in itial ly al lo wed to d esc ribe th e pa in in hi s o r her ow n w o rd sβ€”with ou t pro mptin g from th e d entis t. A pa in his tory s ho u ld b e un dertake n i n a sys tematic fa sh io n a nd all the

featu res l isted in Ta ble 15. 3 sh ou ld be exp lo red. So me f orm of m ore obje ctive ass es smen t o f

pain is im portan t in o rder to meas ure no t jus t in ten s ity bu t th e dis abi lity it i s c au sin g. The us e of a li nea r pai n sca le a nd the McGi ll pa in que stio nn ai re ma y be h elp ful . Th e h is to ry s ho u ld inc lu de th e eff ects th at the pai n h as o n th e li festyle of th e p atien t. S oc ia l, co gn itive,

emo ti on al, a nd beh avio u ral f acto rs b eco me v ery imp ortan t in ch ron ic pa in an d thes e a re

disc us se d fu rth er i n Cha pte r 17 . Th e s oc ial an d fa mil y hi stori es a re c ru ci al as a pa tien t's pa in impa cts o n th o se with wh om they are livi ng.

Examination

Th e e xami na ti on o f the oro fac ia l tiss ue s sh ou ld inclu de a gro ss sen so ry a nd moto r eva lua tio n

of th e c rani al nerve s (lis te d in Ta ble 15. 4). A bn orma l fu nc tio nin g o f th e cran ia l n erves sh ou ld ind ica te to th e cl in ici an tha t re ferral to a ne uro lo gis t or o the r appro pri ate sp eci ali st is

re qui red. The fifth an d s even th n erve n ee d to be examin ed with ca re an d a lteratio n s in

sen so ry fu nc ti on a re an aes thes ia , para esth esi a, dysa esth esi a, an d h eig hten ed s en sitivi ty to pain (h ypera lges ia). Mo to r dys fu nc ti on is evid en t from paral ysi s, gro ss mus cl e we akn ess , mus cu lar atrop hy, o r spa stic ity.

Neuropathic pain

Acc ord in g to th e Intern atio n al As so cia ti on fo r the Stu dy o f Pa in , th e de fin itio n o f n eu roge ni c pain (a lso kn ow n as neu ro path ic

pain ) is pain in itia te d o r ca us ed by a pri mary les io n, dys fun ctio n , o r tran sito ry pertu batio n in

th e pe riph eral or c en tra l nervo u s s ystem. Th is defi nitio n is all -inc lu siv e an d en co mp ass es nerve -inju ry -re lated co nd itio ns .

Table 15.2 The assessment and management of facial pain Hi sto ry

Comp lai nt

Pai n hi story

On se t, peri od ici ty, lo ca tio n a nd radi atio n, du ratio n, freq uen cy , c ha racter,

and in te ns ity o f pai n

Mo dif yin g fa ctors (in itiatin g, agg ra vatin g, an d a llev iatin g fa cto rs) E ffect on li festyle

E ffect o n mo od β€”depres si on , an xiety

Ou tco me o f pre vio us in vestig atio ns an d the rapie s (e. g. med ica tion )

Co ns ider u s e of a vis u al a na lo gue lin ea r pai n s ca le a nd a pa in qu estio n na ire

Den tal his tory

Medi cal hi sto ry (in clu din g a lc oh ol an d dru g us e) So cia l hi story

Exa min atio n

Odo nto gen ic struc tures

Re lated struc tures

Cra ni al nerve fu nc ti on

Denta l sta tu s, pu lp vitali ty te sts, sta ti c a nd dyna mic o ccl us al relatio n sh ips ; Mu sc les o f mas tica ti on , te mpo roma nd ibu lar jo in t, s al ivary g lan ds , s in us es Gros s tes t of sel ected or a ll cran ia l n erves Se ns ory d efic its

In ves ti gatio ns

Radi og raph y an d o the r ima gin g tech n iqu es

P. 178

Hae mato log y an d c lin ic al ch emis try (eg E SR, vitam in B 1 2 ) Vi rol ogy

Diag no stic oc clu sa l app lian c ey B iop sy

Ps ych o metric que stio nn ai res Nerve c on du ctio n te sts

Dia gn os tic, lo ca l a na esth etic n erve blo ck

Th is m ay h elp defi ne th e di stribu tio n of pai n, an d c an eli min ate o r co n firm

re ferred pain

Re que st furth er in fo rmatio n

Fro m g ene ral prac ti ti on er, sp ecia lis ts, ho sp ital cas en otes

Man age men t

Es ta blis h patie nts' trea tmen t g oa ls an d lik ely a dh erenc e to thera py

Eli min atio n of o do n to gen ic pai n

Medi cal tre atmen t

Re fer to appro pria te spe cia lis t

Inve stiga te or d ress a re store d too th, oc clu sa l a djus tmen t, extrac tio n Drug th erap y (ca rbama ze pin e, an ti depres sa nts ) Fu rther inv estiga tion s (e.g . to neu ro lo gis t f or n erve c o ndu ctio n te sts)

Table 15.3 Pain characteristics to be evaluated when taking a pain history Du ratio n Lo ca ti on

Ra dia ti on

Pro voki ng fac to rs

Ass oc ia te d fac to rs Perio di city Cha ra cter Severi ty

Re lievi ng fac to rs

Table 15.4 The cranial nerves Nu mber

Name

Nu mber

Name

I

Olfa ctory

VII

Fac ial

II

Optic

VIII

Aco u stic vestib ul ar (ves ti bu loc o ch lear)

III

Ocu lo mo to r

IX

Gl os so ph aryn geal

IV

Tro ch le ar

X

Vag us

VI

Abdu ce ns

XI

Acc es so ry

V

Tri gemi na l

XII

Hy pog lo ssa l

So me useful neuro logical terms Alo gen ic All odyn ia

Cau sin g pa in Pai n f rom a sti mul us tha t d oes n ot n orm ally pro voke pai n, e. g. ligh t

to uc h

An alge sia

Abs enc e o f a pai n re spo ns e to a p ain fu l stim ul us

An aes th es ia

Comp lete lo s s o f sen sa ti on

Dys aes th es ia

Altered un ple as ant s en satio n ; may be s po ntan eo us o r in respo n se to a

stimu lu s

Hy peral ges ia

An in crea sed respo n se to a stimu lu s th at is no rma lly pai nfu l

Ne uro path ic

Pai n i ni ti ated o r ca us ed by a prima ry les io n, dys fun cti on , o r tra ns itory

pain

prob lem i n th e pe riph eral or c en tra l n ervo us sys te m

Th e term neu ro path ic pai n w ill be u se d in thi s c ha pter. Chro ni c n eu ropa thic pa in in clu des

diso rders su ch as trigemi na l a nd glo ss oph aryn ge al neu ralg ia, po sth erpetic ne ura lgia , dia betic neu rop athy, a nd perip hera l n erve d ama ge du e to HIV in fec tio n a nd alc oh o l. Trau ma o r

inf lamm atory ch an ge a ffec ti ng th e ex tra - or i ntrac ran ial co urs e o f the ne rv e, f or e xampl e, spin al ne rve in jury or m ultip le s cle ros is, c an als o resu lt in n euro pa th ic pai n. B eni gn or

mali gn ant n eo pla sms eith er co mpres sin g o r in fi ltra ting a n erve can gi ve ris e to neu ro path ic pain .

Th is ch ap te r dis cu ss es n eu rop athi c pa in tha t i s l ikely to be en co un tered in th e o ral medi cin e

P. 179

cli nic . It ma y pres en t with or with ou t oth er sym ptoms o f neu ro lo gial dis turba nc es (su ch as parae sthe sia , hype ralg esia , all ody nia , an d moto r n erve dys fu nc ti on ).

Ne uro path ic pain do es n ot u su all y respo n d to co nve ntio na l a na lges ic s s uc h a s a spi rin,

parac etamo l, a nd no ns teroi dal an ti-inf lamm atory drug s (NSA IDs ). Th e m edic al man ag emen t of th is pai n i s be st ac hi eved us ing oth er ph arma co lo gic al age nts s uc h as an ti co nvu ls an ts an d

so metimes an tidep ress an ts . Treatmen t sh ou ld be i ni ti ated, wh ere p os si ble, us in g si ng le dru g th erap y. Th is wi ll l imit th e si de -effec ts .

Ne uro path ic pain i s pa in in itiated or c au se d by a prim ary le sio n , dysf un ctio n, o r tran sito ry perturba tion in th e peri phe ral o r c entral ne rvou s sys te m.

Trigeminal neuralgia Tri gemi na l n eura lgi a: s udd en, u su al ly u nil ateral , severe , b rief, stabb in g, re cu rrent p ain s i n th e di stribu tio n o f o ne or more bran ch es of th e fifth cran ia l n erve.

Tri gemi na l n eura lgi a is a dis eas e o f su ch c ha racteri stic in ten si ty th at i t h as be en k no wn an d desc ribe d ove r a lo n g pe ri od of hi story. In spi te of th is , th e true na tu re o f th is extremel y

pain fu l c on ditio n is no t kno w n. In s om e pa ti ents there is a va scu la r abn o rm ali ty (veno u s o r arterial ) tha t c au ses c omp ress io n o f th e trigem ina l roo tβ€”adjac en t to the po ns in the

pos terio r cran ia l fo ss a. Th is re su lts i n area s o f de myeli na ti on an d a bn orma l c on du ctio n. Eli min atio n of th is co mpres si on ha s l ed to lo ng -term pai n re lief in mo st patie nts. Th e

ass oc ia ti on betw een mul ti ple sc lero sis (MS ) an d trige min al neu ral gia is wel l kn ow n a nd is disc us se d later i n th is ch apter.

A s mall pro po rti on (app roxi mately 2 per cen t) of patie nts w ith po sterio r fo ss a tum ou rs (f or examp le, ac ou stic n euro ma s or b eni gn tu mo urs ) pres en t w ith typ ica l trige min al neu ral gia . Pai nfu l s ympto ms may p rece de o r ac co mpa ny s en so ry or moto r def ici ts in thes e c as es.

Th e p atho ph ysi ol ogy of tri gemin al n eura lgia h as yet to be elu ci dated but on e th eory is th at the paro xysms o f pa in in th is co n ditio n m ay rep rese nt dis ch arges in se lec te d n euro n es w ho s e

th resh o ld fo r rep etitive fi rin g h as b een altered , pos sib ly by foc al ne rv e in jury. S uc h fi rin g ma y

th en be i ni ti ated b y in no cu ou s ta ctile stim uli (this wo u ld e xpla in β€ trig ger z on es β€™). Cros s exci ta ti on o f n eigh bo uri ng nerve s c ou ld th en resu lt in th e recru itmen t o f su ffic ien t dis ch argi ng cell s fo r a no ci cep ti ve si gn al to be perce ived as pain . How ever, n on e o f the cu rren t th eo ri es ful ly exp lai n th e cl in ica l fe atures o f tri gemi na l neura lgi a.

Pretrigeminal neuralgia

Th is co n ditio n is d esc ribed as a du ll ac he, s imil ar to to o th ac he , a nd th e p atien ts s ubs equ en tl y devel op tri gemi na l n eura lgi a. It i s tho u gh t to o ccur in u p to 20 pe r cen t o f patie nts wh o

devel op trig emin al ne ura lgia . Thera peu ti c o ptio ns fo r trigem ina l n eu ralgi a are e ffec ti ve fo r this co ndi ti on . The cli ni cia n m us t first, ho w ever, en su re tha t th ere i s n o pai n o f d enta l o rigin

re spo n sib le fo r the se s ympto ms. It is at this ti me th at the patien ts a re mo st o ften see n by th e den ta l s urge on fo r the in vestig atio n o f w ha t may b e h igh ly pe rplexi ng pai n s ympto ms. Pretrige min al neu ral gia is a di agn o sis mad e in retro spec t.

Clinical presentation of trigeminal neuralgia

Th e in cide nc e o f trigem in al n eu ralg ia i s a rou n d 150 pe r 1 mill io n a nd more patie nts are f emal e (f emal e:mal e rati o a ppro xima te ly 2:1). Th e pre vale nc e o f the co nd ition i s 1 in 1000. The p eak on set i s in the fifth to s even th d eca des an d symp toms us ua lly oc cu r after th e fo urth dec ade . Th e p ain dis tri butio n is alm os t alw ays un il ateral in th e fi rst in stan c e, w ith on ly 3 p er cen t of

cas es su bse que ntly d evelo pi ng bila te ral i nvo lve men t. P ain ma y aff ect on e, two , o r eve n th ree

divis io ns . The f irst di visi on o n its o wn is o nly rarely affe cted. Th e pa in is o f g reat in tens ity, is

desc ribe d as sh arp an d sta bbin g i n na tu re (l an cin atin g), an d la sts for o n ly a few sec on ds . Th is tra ns ien t atta ck may b e repe ated i n a ma tter o f m inu tes or h ou rs. Th ere m ay be no ap pare nt preci pitatin g fa cto r, b ut ma ny p atien ts h ave a β€ trig ger z on eβ €™, a po in t on th e fa ce or i n

th e mo u th (o ften ou ts ide the a rea of pain di stribu tio n) w he re the ligh test c on tac t m ay i nitia te an atta ck. S ti mul i ma y in cl ude tou ch in g, wa sh ing , drau gh ts o f co ld air, too thb rus hin g, ea ti ng,

or s mili ng . B etwe en th e pa roxy sms of pai n th ere ma y be a du ll bac kgro un d a ch e in the area or no pa in at all . Thes e attacks rare ly o cc ur a t n ig ht. The so -cal led β€ froz en fac eβ€™ is an

atte mpt by th e p atien t to de fer attac ks b y lim itin g mo veme nt, hen ce avo idi ng th e trig gerin g of th e pa in . S po nta neo u s rem iss io n fo r a matter o f w eeks or m on ths may oc cu r, but th is is ra rely

perman en t. P atien ts i n s evere pai n l os e w eigh t, a re u na ble to so cia liz e, an d o ften bec om e depres sed .

Des cri pto rs of pai n i n trige min al neu ral gia :

sh arp

stabb ing

β€ elec tri c sh oc k -like β€™

Investigation of patients with trigeminal neuralgia

Tri gemi na l n eura lgi a is di agn os ed p rin cipa lly from th e p ain h isto ry. There is no sp eci fic

diag no stic test, bu t a fu ll cli nic al exam ina tio n, in clu di ng ass es smen t o f the cran ia l n erves ,

sh ou ld b e ca rried ou t to ex clu de o the r ca us es of pai n. Ve ry few patie nts h av e su btle sen so ry ch an ges, a nd trig ger po in ts c an us ua lly b e el ici te d. Th e d enta l s urgeo n ha s a pi votal rol e in

th e di agn o sis o f patie nts p resen tin g w ith trige min al neu ral gia . He /s h e mu st al wa ys ex clu de a den ta l c au se fo r th e pa ti ent's pai nfu l s ympto ms.

Patie nts with tri gemi na l n eura lgi a ma y as crib e the pai n to too tha ch e in wh at m ay a ppea r to b e an entire ly s ou nd too th . Fo llo w ing the extractio n of th is too th th e pa in may th en be desc ribe d

as c o min g fro m a nea rby to oth an d extrac tio n o f th is m ay a lso be de man ded by the pati ent. It is n o t ra re to fin d pa ti en ts mad e virtu all y un ila terall y ede ntul ou s in th is wa y. Man y eve ntu al diffi cu lties ca n b e avo id ed if i t i s rec o gni zed that a ny patie nt pres en ti ng with po ss ible trig emin al neu ral gia symp to ms

sh ou ld b e co n sid ered a s w o rth y of ful l in ves ti gation befo re fu rther extractio n s a re co n sid ered.

Trigeminal neuralgiaβ€”key features

Brief paro xys ms o f s ha rp, stabb in g pa in

Tri gger z o ne (s )

Pro voki ng fac to rs i nc lu de ea tin g, ta lkin g, sh avi ng, wa sh ing fac e

Pai n u su al ly c on fin ed to o ne divi sio n of tri gemin al ne rve

Usu all y un ila teral

Usu all y pa in -free du rin g sle ep

Dru g the rapy w ith ca rb ama ze pin e is the medi cal treatmen t of ch oi ce

Perip hera l o r cen tral s urg ica l treatme nts are ma na geme nt o ptio ns

May hav e exten ded remis sio n wi th co mp letely p ain -free perio ds

Th e d enta l su rgeo n ha s a piv otal rol e in the dia gnos is o f p atien ts prese ntin g w ith trig emin al neu ral gia β€”a den tal cau s e fo r th e p atien t's p ain ful symp to ms mu st al way s be excl ud ed.

Con verse ly, th e situ atio n m ay a rise in wh ic h p ain cl os ely re sem blin g trig emin al neu ral gia may aris e fro m so me qui te o rd in ary de ntal path ol ogy su ch as a re ta in ed ro ot o r a crac ked to o th o r even from un sa tisfa cto ry den tures . If th ere i s a ny abn orm ality of th is kin d pre sen t, i t s ho u ld be treated as th e e sse ntia l fi rst step in th e man age men t of th e pa tien t.

Yo u nge r patie nts (< 50 years o f age ) pres entin g w ith tri gemi na l neu ral gia , p articu la rly if

th ere i s bi latera l in vo lvem ent, sh ou ld be ref erred to a n eu rolo gi st fo r app ropri ate di agn o stic tes ting to e xclu de mul ti ple sc lero sis .

P. 180

As ha s be en poi nted ou t, trig emin al neu ral gia te nd s to oc cu r in la ter life . Th e o ns et o f

sympto ms of tri gemin al neu ral gia in a p atien t un der th e ag e o f 40 years sh ou ld be trea te d

with so me su spi cio n as a p os sib le fi rst wa rni ng of th e o ns et o f mu ltipl e sc lero si s (s ee nex t

sec ti on ). In thi s c on ditio n the n eu ral gia -like pains are o ften the f irst symp to ms . It h as been su gges te d tha t patien ts o f th is a ge g rou p s ho u ld poss ibl y be ref erred fo r a spe cia lis t

neu rol og ica l a sse ss men t. Whe n a ss ess in g the ne ed for a n euro lo gic al op in ion o n a pa ti en t w ith su spec ted trige min al neu ral gia, i t sh ou ld alw ays be re membe red th at there are n o dia gno s ti c ind ica to rs oth er tha n th e p ain itse lf. An y o ther a ss oc iated ne uro lo gic al f eatu res, su ch a s

mus cle wea kne ss , bl urri ng of v isi on , diz zi nes s, pa raes th es ia, o r th e p os sib le in vol vemen t of othe r cra nia l n erves , s h ou ld b e taken a s a bso lu te in dic atio ns fo r a fu ll neu ro lo gic al ass ess men t an d c erebra l ima gin g.

Central ne rvou s s ystem ima gin g s ho ul d be un dertake n i f the re is sen so ry lo ss.

Table 15.5 The differential diagnosis of trigeminal neuralgia Den tal pain , esp eci all y β€ crac ked to oth syn dro meβ€™

Sin us pa in

Po sthe rpetic neu ral gia

Tri gemi na l n eura lgi a s eco nd ary to ne rve in jury, tumou r, or o the r path ol ogi cal co ndi ti on

Clus ter he ada ch e

Atypic al fac ial pai n

Te mpo rom an dibu la r pai n d ysfu nc tion syn dro me

Th ere are a nu mbe r of co nd itio ns to be co n side red in th e dif feren ti al dia gno si s o f trige min al neu ral gia (Ta ble 15. 5) but i n m an y of th es e the pain s ympto ms re ported by the patie nt a re no t ch arac teristic o f tri gemi na l n eura lgi a (tha t is, bri ef, sh oo tin g, paro xysm al p ain ).

Tri gemi na l neu ral gia may be the firs t man ifes tatio n i n a sm all nu mbe r of patien ts w ith MS.

Th es e are yo un ger th an th e trig emin al ne ural gia po pul atio n as a w ho le a nd thei r neu ral gia is often bil ateral . Con verse ly, tri gemi na l n eura lgi a is di agn os ed i n 1β€“5 p er ce nt o f pa ti ents with MS .

Medical management of trigeminal neuralgia

Ph arma co lo gica l th erapy is still co ns ide re d the mo st ap prop riate man age men t for m os t

patien ts w ith trig emin al ne ura lgia , but rec en t re sults fro m larg e ne uro su rgic al seri es w ou ld

ju stif y su rgic al interve ntio n early on , partic ul arly i n th e yo un ger patien t. A ll patie nts s h ou ld be gi ven a dis pas sio n ate a cco u nt o f the medi ca l an d s urg ica l o ptio ns ava ila ble.

Th e m edic al tre atmen t of tri gemi na l n eura lgi a is prim aril y wi th the us e o f

Carbam az epin e i s c urren tly th e dru g o f ch o ice

Th e i ni ti al d os e o f c arbam az epin e i s 100 mg, tw ic e dai ly; thi s i s in cre ase d gra dua lly, by

anti co nvu ls an ts

100 mg e ach da y, un til th e pa in is co ntro lled

A ma ximu m da ily do se o f 800β€“1000 mg s ho u ld n ot b e exc eeded

Ph arma co lo gica l trea tm ent o f trige min al n eu ral gia depen ds la rgely u po n th e u se of

anti co nvu ls an ts an d th e dru g o f ch o ice ha s trad itiona lly b een carb ama zep in e (Tegra to l).

Re spo n se to ca rbama ze pin e is repo rtedly dia gno sti c fo r tri gemi na l n eura lgi a; h ow ever, it is

wo rth men tio ni ng th at c arba maz epi ne c an oc ca sio n ally redu ce (o r a bol ish ) pa in of od on toge ni c orig in. Den ta l c au ses o f fac ial pai n s ho u ld, there fore , h ave been el imin ated prio r to th e co mmen cem ent o f drug thera py. Othe r an ti co nvu ls an t drug s a re o cca si on all y u sed, bu t

carb ama zep ine rema ins the β € gol d sta nda rd β€™ the ra peu tic agen t fo r tri gemi na l neu ral gia

and ha s b een eva lua ted in ra ndo miz ed pla cebo -co ntrol led tri als . Th e in itial do sag e o f

carb ama zep ine is kept lo w an d sta rted at 100 m g, twic e da ily, an d gra dua lly inc reas ed in dail y inc remen ts o f 100 mg u ntil co ntro l o f pa in is attain ed. A maxi mum do se o f 800β€“1000 m g dail y sh o uld no t be excee ded. Th e medi catio n sh ou ld be tak en abo ut

30 mi nu te s b efo re mea ls, thu s maxi miz in g pa in co ntro l fo r ma stica tion . Ab ou t 70 pe r cen t of

P. 181

patien ts w ith tri gemi na l n eura lgi a are estim ated to ben efi t f rom carb ama zep in e. A ll patie nts wil l h ave m in or s ide -effec ts , wh ich va ry an d a re dose -depen den t. So me pa tien ts experi enc e drow si nes s o r di zz in ess , wh ereas so me repo rt g as troin te stin al reac ti on s. In a very few

patien ts, ho w ever, the m uc h m ore seri ou s s ide -effect of agra nu lo cyto sis ma y oc cu r. The

elderl y are partic ul arly s us cep ti ble to sid e-effec ts . The wh ite ce ll c ou n t may b e mo ni to red by re pea ted blo o d tests , b ut it wo u ld a ppea r th at, wh en thi s rea ctio n oc cu rs, it ma y do so

su dden ly a nd with ou t prio r w arni ng . O th er, les s s erio u s, ha ema to lo gic al effec ts h ave been

re co rded inc lu din g a na emia , n eu trope nia , th ro mbo cytop en ia, an d h ypo na traemi a. Fo r thes e

re aso n s i t i s g ene rall y reco mme nde d tha t ha emato lo gy a nd blo od ch emis try te stin g is ca rried ou t at the co mmen ce men t o f trea tm ent, an d a t 1 mon th an d 3 mo nth s, an d the reafter

depen di ng o n ci rcu mstan ce s. It i s i mpo rta nt to rei te ra te, ho we ver, th at a su dden , po te ntia lly fatal, fa ll i n th e w hi te cel l co un t may oc cu r as a ve ry rare e ven t, an d m ay be en ti rely m iss ed

by lo ng -term blo od -tes ting regi mes. It is als o gen erall y rec omm end ed th at live r fu nc ti on te sts sh ou ld b e pa rt o f th e dru g mo n itori ng prog ramme , a s c arba maz epi ne is acti ve in li ver en zym e ind uc ti on . Hypo na tra emia (a re duc ed s eru m lev el o f so diu m) h as als o been repo rted w ith carb ama zep ine . Wh en pres crib ing carb ama zep in e the cli ni cia n s ho u ld a lw ays be a ware of

pos si ble d rug in tera ctio ns β€”so me dru gs may beco me in effec ti ve be cau se thera peu tic s eru m

level s a re no t ac hi eved or m ain tain ed d ue to the increa sed rate o f me ta bol is m. Wa rfari n i s o ne su ch drug .

Monit oring for patients o n carbamazepine therapy

Fu ll blo od co un t, liver fun ctio n te sts, an d e lec trolytes sh ou ld be mon ito red:

prio r to th era py

at 1 mon th

at 3 mon ths

A fu rther sid e-effec t of c arb ama zep ine , w hi ch o ccu rs in appro xim ately 7 p er ce nt o f patie nts,

is th e o cc urren ce of a s evere ra sh . Jus t a s i n th e c ase of agra nu lo cyto sis , th is may oc cu r qu ite un expec tedly after a lo ng perio d o f tre atmen t with the d rug.

As a res po ns e to th e pro bl ems ass o cia te d wi th c arbamaz epi ne th erap y, a rela ted dru g,

oxc arba zep in e, was in tro du ced . Th is ha s e sse ntia lly th e s ame prop erti es as carb ama zep in e bu t

is repu te d to b e be tter to lerated . Th is is no t, h o weve r, a lw ays th e c as e. Oxca rbaz ep ine ha s ye t to es ta bli sh its p lac e a s a su bstitu te fo r ca rb ama ze pin e as up to a qu arter o f patie nts experie nc e c ro s s -sen sitiv ity.

Th ere are o ther drug s tha t may be u sed in the trea tmen t o f trig emin al neu ral gia fo r patien ts wh o c an no t tol erate c arba maz epi ne. Th ese in clu de phe nyto in , l amo trogi ne , ba cl ofe n, an d

clo na ze pam . No ne of th es e dru gs are a s effec tive a s c arba maz epi ne in th e c on trol of

trig emin al ne ura lgia , alth ou gh phe nyto in in co nju n ctio n w ith carb ama zep in e ma y be va lu abl e in so me pa tien ts un resp on si ve to carb ama zep in e al one. The startin g do s e fo r ph enyto in is us ua lly 300 mg/d ay (in di vided do ses ) al th o ugh thi s d rug a lo ne is rarely su cc ess ful in

co ntrol lin g p ain . Ph eny to in h as a n umb er o f si de -effec ts in cl udi ng a s evere rash . Con si derin g th e mo rbid ity an d p revale nc e o f trigem in al n eu ralg ia, very few co ntro lled stu dies ha ve b een

carri ed o ut o n drug s a vail abl e fo r tre atmen t. A recen tly i ntrod uc ed d rug, ga bap entin , ha s b een evalu ated fo r us e in po sth erpetic neu ral gia an d di abetic n euro pa th y a nd may b e u sefu l f or

trig emin al ne ura lgia . It app ears to be co mpa ratively free of s erio u s s ide -effec ts an d c an be us ed in co mbin atio n wi th c arbam az epin e.

Side- effect s o f carbamazepine therapy

Dro ws in ess , d iz zin es s, co nfu s ion

Vertigo

Na us ea

Skin rea ctio n β€”ra sh

Leu ko pen ia (an aemi a, ne utro pen ia, thro mbo cy to pen ia , a nd apl astic an ae mia oc cu r

He patic mic ros oma l e nz yme i ndu cti on (an d c on se que nt dru g in terac tion s)

Hy pon atrae mia

ra rely)

Surgical treatment of trigeminal neuralgia

A mi no rity o f pa ti ents are un su itab le fo r dru g th erapy, ei th er be ca us e they are β€”or h ave

beco me β€”un resp on siv e to i t or c an no t tole rate the si de -effec ts . Others are keen to exp lo re th e s urgi ca l o ption s in th e h o pe o f a perma nen t cu re. The re a nu mber of diffe rent s urgi ca l

appro ac he s a vail abl e fo r th e trea tm ent o f trige min al neu ral gia an d the se v ary in c omp lexi ty from relativ ely s impl e pe ri ph eral proc edu res (for examp le, cryo the ra py) to hi gh -frequ enc y

sele ctive co agu la ti on o f th e n erve fibres in the regio n of th e g an glio n an d o pen in tra cra ni al proc edu res in th e p os te rior fos sa fo r mic rova sc ula r dec om press io n of th e trige min al nerve

(Ta ble 15. 6). F ul l deta ils of th es e pro ced ures are , ho we ver, ou tside the sco pe of th is bo ok

(s ee Pro je ct 3 at th e e nd of th is ch apter). Whe n c on si derin g a su rgic al app ro ac h to treatme nt, th e fol lo win g f acto rs mu s t b e co n sid ered: p os to perative pa in reli ef; rec urren ce rates; g en eral hea lth; ri sks ; an d ad verse effec ts.

Mic rova sc ula r dec om press io n of th e trige min al nerve is no w c o ns idered th e s urg ica l treatme nt of c ho ic e by a n um ber o f c entres , p rovi ded th at the patie nt is in reas o na ble h ea lth a nd

evide nc e o f vas cu lar deco mp re ss ion h as been iden tifie d, as it is th e o n ly pro ce dure th at i s

no n -destru ctive. A spe cif ic ima gin g tech n iqu e, mag netic res on an ce to mo grap hic an gi ogra ph y (M RTA), is

P. 182

us ed to op ti mall y ima ge th e trige min al n erve an d bloo d ves se ls i n i ts vici ni ty.

Table 15.6 Surgical treatments in current use for trigeminal neuralgia Su rgical th erapies

Pro cedur e

Mor bidity

Recu rr ence after

sur gery

(average) Perip hera l Cry othe rapy,

Th e p eriph eral nerve

Lo w an d lo ca l,

alc oh ol in jectio n s

direc tl y expo se d o r

sen so ry lo ss

neu rec to my,

bran ch is eith er

injec ted u nd er lo ca l

main ly m ild

Up to a year

ana es th es ia Ga ss eria n ga ng lio n Ra dio freq uen cy,

th ermo co ag ula tion

Sh ort -actin g,

Sen so ry

ana es th es ia u se d to

trig emin al nerve ,

intermi tten t gen era l pen etra te fo ramen

ova le. The Gas seria n

gan gli on is su bjec te d to cyc les of th erma l lesi on in g

(tem peratu re vary ing from 60 Β ° to 80 Β°C) Gl ycero l in je ctio n

impa irmen t to th e ana es th es ia

dol oro sa (rare), co rnea l

ana es th es ia (w ith

su bseq uen t ris k of keratitis ).

Mo rta lity rate low .

Lo ca l a na esth esia

Dys aes th es ia

us ed. Mec kel's ca ve

trig emin al area

(Β ± seda tion ) c an be is f ille d wi th gl ycero l

with in th e

Mic roc omp ress io n

Fu ll ge ne ra l

Dys aes th es ia can

co mpres sio n )

Th e Ga ss erian

Mo to r co mpo n en t

(b all oo n

ana es th es ia u se d. gan gli on is

co mpres sed by a

3β€“ 5 ye ars

be pro ble matic .

Up to 3 years

3β€“ 5 ye ars

often in vol ved

ball oo n for a fe w sec on ds Po sterio r fo ss a Mic rova sc ula r

deco mpre ss ion

Fu ll gen eral

8th cran ia l n erve

An y ves sels lyi ng on

oc cu r. Mo rta lity

ana es th es ia u se d.

th e trige min al nerve

at the p oi nt o f en try to the cran iu m a re move d as ide or re mo ved

dama ge m ay

ra tes o f u p to

0. 7% repo rte d.

Serio u s mo rbi dity, 10%

8 ye ars

Stereo ta ctic

ra dio su rgery

Lo ca l a na esth esia

Lo ng -term effec ts

us ed. Usi ng

un kno wn . Se ns ory

(Β ± seda tion ) c an be stereo ta ctic

tec hn iq ues the

of ra dia ti on

Mean 15 mon ths

los s o f s lo w o n set

pos terio r fos sa is iden tified an d a gamm a kn ife is direc te d at th e

proxi mal tri gemi na l ro o t nea r the pon s

*A dap ted wi th permi ssi on fro m T. J. Nu rmikk o, Pa in Rese arch Ins titute, Un ivers ity of Li verpo ol , a nd P. R. E ld ri ge, Th e Walto n Centre f or Neu ro log y an d Neuro su rgery NHS Tru st, Liverpo o l, UK.

Su rgica l trea tm ent o f trige min al n eu ral gia β€”facto rs to be co ns idere d:

pain relie f

re cu rrenc e ra tes an d th eir man age men t

co mpli catio n s β€”morb idity a nd morta lity

Glossopharyngeal neuralgia

Gl os so ph aryn geal n eura lgia is simi lar to n eura lgi a a ffec ti ng th e trige min al nerve , b ut mu ch

less co mm on . The pai n i s o f the sa me n ature as that o f trig emin al neu ral gia an d is un ila terall y felt in the oro ph aryn x, so metime s w ith p ain refe rred to th e ips ila te ra l e ar. Alth ou gh the main co mpo nen t o f the pain is de scri bed (a s i n tri gemi nal n eura lgi a) as sta bbin g o r sh o otin g i n

natu re, th ere may a ls o b e an app re cia ble resi dua l ach e tha t may l as t f or s om e time after the paro xysma l attack. It is les s s evere th an trigem inal n eu ralgi a. Th e pa in is o ften preci pitated by sw al low in g, ch ew ing , o r c ou gh in g, and there may be a tri gger zo ne . Ca rdia c a rrhyth mias and syn co pe ha ve be en d esc ribed du rin g attac ks. Glos so ph aryn gea l n eura lgi a ma y be sec on dary to a tu mo ur of th e thro at, po sterio r crania l fo ss a, o r jugu la r fo ra men .

Gl os so ph aryn geal n eura lgia is a ra re c on ditio n ch arac teriz ed by a paro xysm al, s ha rp ,

lan ci nati ng p ain tha t affe cts o n e si de o f th e thro at a nd bas e of th e to ngu e. It is us ua lly trig gered by s wal lo win g.

Tre atmen t is th e s ame as for trig emin al ne ura lgia . Spo nta neo u s rem iss io n of th is co nd itio n i s pos si ble b ut fi rst-lin e

trea tmen t i s u su al ly w ith a n an ti co nvu ls an t an d ca rbam az epin e i s s uc ces sfu l i n m os t cas es. A

P. 183

pos itive respo n se to thi s h elp s c on firm th e di agn o sis . A s urgi ca l ap proa ch ma y be requ ired i f drug th era py bec om es ine ffec ti ve o r pro blema tica l.

Postherpetic neuralgia

He rpes zo ster (s hi ng les) is th e rea ctiva tion o f a laten t in fecti on wi th the vari cell a z o ster viru s

(s ee Cha pte r 4). Th is viru s is u su all y reac tivated on ce in a life ti me, wi th few er th an 5 per ce nt

of p atien ts h avi ng a s eco nd attack . He rp es zo ster is mo re l ikel y to o cc ur i n later l ife a nd in patien ts w ho h ave T-cell immu n os upp ress io n. Pa in may oc cu r bef ore, du rin g, or a fter an

atta ck o f f aci al herp es z o ster. The firs t w arn in g of su ch a n a tta ck m ay be a s evere burn in g

pain oc cu rrin g in the area of th e e ruptio n , u p to 2 days bef ore th e ves ic les app ear. The pai n i n herpe s z o ster is mu ch mo re se vere th an th at i n h erpe s s impl ex, in wh ich th e sym ptoms are

large ly du e to the s ec on dary inf ectio n of ruptu red vesi cle s ( Cha pte r 4). Th e pa in as so cia ted with he rp es zo ster u su al ly s ubs ide s w ithi n w eeks . In so me patie nts a n eura lgi a -like pain

persi sts f or mo n th s or e ven years . Th e p ain h as an in te ns e bu rni ng co mpo ne nt an d may b e acc om pan ied by s ta bbin g s en sa ti on s β€”th ere i s typi cal ly h ypera lges ia or para esth esi a an d allo dyn ia . Th e d istrib utio n of p ain fo llo w s the distri bu ti on o f th e o rigi na l in fec tion . Thi s

neu ral gia te nds to b e a persi sten t as opp os ed to a paro xysm al p ain . In a dditio n , th e pa in ha s a mo re va riab le c ha racter a nd seve rity than trige min al neu ral gia . Th e in c iden ce of tri gemin al pos therp eti c neu ral gia is un certa in, bu t o lder p atien ts w ith h erpe s z os ter are partic ula rly

su sce ptible to p os th erpe ti c n eu ra lgi a β€”60 pe r cen t of patie nts o ver 60 are affe cted b y this

distres si ng c o ndi ti on . It i s rel ativel y un co mmo n in tho se un der th e a ge o f 50 yea rs . The risk o f pos therp eti c n eu ralg ia i s a lso th ou gh t to be greater i f pa ti en ts ha ve s evere pain o r ras h d urin g th e a cu te ph ase an d a lso if th e p atien ts exp erien ce pai n p rior to the app eara nc e o f the rash .

Tre atmen t of estab lis hed po sthe rpetic neu ral gia ca n b e very diffi cu ltβ€”it is often res istan t to trea tmen t. It doe s n o t a ppea r to re spo n d to carb amazep in e an d a na lges ic s ma y al so be ine ffec ti ve. Stero ids ha ve be en advo ca ted bu t there is no co n firma ti on o f thei r val ue in

preven ti ng po sthe rpetic neu ral gia . Tri cyc lic an ti depre ssa nts ha ve be en ass oc ia te d wi th pa in re lief in so me p atien ts an d g aba pen ti n h as be en s h ow n, in cl in ica l tria ls, to be ef fective for

pos therp eti c n eu ralg ia. A ntivira l trea tme nt c omb in ed w ith a ntid epres sa nts i n th e e lderl y in the acu te ph as e h as been fo un d to redu ce th e d ura ti on an d preva len ce o f p os th erpe ti c n eu ralgi a, by as mu ch as 50 p er ce nt. The us e o f tra ns cu tan eous elec tri cal stim ula tio n i s tho u gh t to be effec ti ve fo r so me patie nts.

To s um mariz e, pro ph ylaxi s o ffers the bes t h o pe o f preven tin g the dis tres si ng pai n o f

pos therp eti c n eu ralg ia a ffec ting the tri gemi na l n erve. Al l pa ti ents wi th h erpes zo ster sh ou ld b e vigo rou sl y treated with sys temic an tivira l ag ents su ch as ac ic lov ir. Thes e s ho ul d be

co mmen ced as so on as po ss ibl e af te r th e o n set o f the rash . Mu ltic entre c lin ic al tri als ha ve

estab lis hed th at s uc ces sfu l trea tmen t o f th e pri mary i nfe ctio n c on si dera bly redu ces the risk o f th e s ubs equ en t devel op men t o f n eu ralg ia.

Pai n m ay pre ced e, a cc om pan y, an d pers is t a fter the ac ute ve sic ul ar ph as e o f h erpes

Pai n p ersi stin g af ter herp es z o ster is ca lle d po stherp etic n eu ral gia

Th e m ain ris k fa ctor i s in crea sin g a ge

zo ster

Neuropathic pain secondary to other conditions

Fac ial pai n a nd hea da ch e ma y be s ympto ms o f a wi de va riety o f in tra- and extrac ran ial les io ns

su ch as ne opl as ms o r va scu la r abn o rmali ti es. F aci al pain ma y ari se th ro ugh in vo lveme nt ei th er by pres su re o r by m alig na nt in fi ltra tio n o f th e trigemi na l ga ngl io n o f th e peri ph eral bran ch es of th e n erve. Pri mary a nd sec on da ry in tra cran ia l n eo pla sms , n as op ha ryng eal tu mo urs ,

ane urys ms, a nd cereb ral e pide rmoi d c ysts a re am on g th e mo st c omm on ly re co rded l esi on s o f th is typ e ca us in g fa cia l p ain . Sim ila rl y, th e l esion s l eft fo llo wi ng brain da mag e o f an y kin d may b e the so urc e o f fa cia l pa in . In ca ses o f this type, fa cia l pa in is on ly ra rely th e o nl y

sympto m, alth ou gh it ma y we ll b e the earl iest. Involve men t of oth er cra ni al n erves an d th e

prese nc e o f un u su al sympto ms su ch as an aes thes ia or p arae sthe sia sh ou ld at o nc e rai se th e su spic io n of th e po s sibi lity o f a les io n o f th is kind .

An oth er ca us e o f fa cia l pa in aris in g fro m co mpres sio n o f the trig emin al ne rve o cc urs in Pa get's

dise as e aff ectin g th e ba se of th e s kul l (s ee Cha pter 18). S om e deg ree o f cl os ure of th e

fora min a i s c omm on , an d the co ns equ en t strictu re o f the nerve may lea d to a varie ty of p ain sympto ms. A fu rth er c au se o f trau ma to th e trigem ina l n erve i n Pa get's d ise ase may be the defo rmatio n of th e s kul l a s a wh ol e, cau s ing co mpres sio n o f the s en so ry ro ot o f the ne rv e

wh ere it c ros ses the petrou s bon e. The pai n o ften sim ul ates trig emin al ne ura lgia , alth ou gh

ach in g pa in s m ay so metime s be repo rte d. A s imi lar com press io n of th e a udi to ry n erve m ay give ri se to de afn ess , an d ch an ges in the cervi ca l sp in e ma y als o lea d to p ain in the area served by th e se ns ory n erves o f the cervi cal ple xus.

Migraine

Th ere are m an y varia nts of migra in e an d a ful l de scri ptio n o f th e dif feren t typ es i s o u ts ide th e sco pe of ora l med ici ne. The den ti st sh o ul d, h o weve r, b e fa mili ar w ith th e sy mptom s o f

migra ine . P atie nts with mig rain e a nd clu ster h ea dache (migra in ou s n eu ralg ia) o cc as io na lly

prese nt in th e o ral medi cin e c lin ic . Alth ou gh mig rain e is es sen tial ly a hea dac h e, i t may als o hav e a f aci al co mpo ne nt, us ua lly o ver th e ma xill a, an d th is m ay c au se co nfu s ion . In mo s t cas es, h ow ever, the patie nts co mpla in of an in te ns e h ead ach e, in term itte nt in n ature an d

us ua lly (b ut n ot in vari abl y) un il ateral β€”hen ce th e te rm β€ hem icra nia β€™. Ea ch attack of

hea dac he may las t for s evera l h ou rs or even a

nu mber o f d ays , a nd is often as so ci ated w ith vario us s ymptom s s uc h a s na us ea a nd vis ua l

P. 184

distu rban ce . Th e s ystemi c u ps et is co ns idera ble an d ma ny patie nts n eed to tak e bed rest i n a

qui et dark ro o m u ntil th e a tta ck s ub sid es. In c las sic mi grain e p atien ts h ave an au ra w here th e hea dac he is prece ded b y neu ro lo gic al s ympto ms a nd defi cits β€” visu al , s en so ry, s pee ch , o r

moto r dis tu rba nc es m ay o cc ur a nd us ua lly las t aro und 15 min utes . M igra in e wi th o ut a ura is,

ho weve r, w ell reco gn iz ed. Th e h isto ry of th e c on di ti on wi th its as so cia ted dis tu rba nc es o ften gives a s tron g c lue to i ts n ature, a s do es the frequ ent prese nc e o f a fami ly h isto ry. So me

patien ts rec o gni ze a pre cipi tatin g fac to r β€”so metimes o f stres s, so metim es o f a fo od stuf f. Th e on set o f mi grai ne may o c cu r at an y a ge, bu t i s m os t co mmo n d urin g a do les cen ce . In migra in e th ere are n o symp to ms betw een attacks .

Th e c au se of migra in e h as gen eral ly bee n a ttri buted to in s ta bili ty of th e c ran ial arterie s. The

re spo n se of migra in e to d rug th erap y is varia ble . Simp le a na lges ic s, an ti -emetic s, prop ran ol ol, and ergo tami ne c an be su cc ess ful . F irs t-lin e dru g treatme nt fo r the acu te ph as e s ho ul d be a

simp le a na lges ic su ch as pa race ta mo l, asp irin , or a NSAID. An an ti -emetic may be h elp ful in so me pa tien ts wh en vo mitin g is a prob lem β€”th e a dmin is tra ti on o f dru gs by s upp os itory i s ben efic ial fo r su ch patie nts. 5 -hydro xytryptami ne (5-HT 1 ; sero ton in ) ago n ists (for exam ple,

su matriptan o r n ewer an alge sic s) m ay be give n b y the o ral , i ntran as al , or s ub cu ta ne ou s ro u te .

Hi sto rica lly, the mo st wi dely us ed gro u p o f dru gs ha ve been de ri ved f ro m e rgo t, the se a re n ow best a voi ded i n view of th eir pow erfu l sid e-effec ts.

Migra in e pro ph ylaxi s m ay be val uab le f or patie nts who h ave tw o or m ore seve re attac ks ea ch mon th. B eta -blo ckers , su ch as prop ran ol ol, a nd 5-HT 1 an ta go ni sts (P iz otife n) m ay be

ben efic ial . Meth ysergi de i s u sed o nly oc ca sio na lly bec au se o f th e ris k o f un wa nted sid e-

effec ts , inc lu din g th e very s erio u s o ne of retro pe riton eal fibro si s. Meth ysergi de s ho u ld o n ly be admi ni stered u n der h os pital su pervi sio n . S o diu m valp roa te an d tricy cli c a ntide press an ts ma y be c on sid ered. Clo n idin e, a drug th at h as been u sed in la rger do se s fo r the tre atmen t of

hype rten s ion a nd wh ich ha s a vas cu lar stabi liz in g eff ect, ha s b een us ed fo r th e co n tro l o f

migra ino u s attacks . This dru g h as co ns ide rable si de-effec ts , it may aggra vate d epres sio n an d prod uc e in so mn ia, a nd is n o t n o w re co mmen ded rou tine ly fo r pro ph ylaxi s o f migra in e.

Th e d iag no si s o f mi grain e i s ma de o n bas is of th e cli ni cal hi sto ry , b ut a phys ic al e xami na ti on of th e pa ti en t a nd a c aref ul ass es smen t o f the his tory i s req ui re d to excl ude oth er c au ses of

hea dac he . Th ere is no evid en ce th at o ccl us al prob lems ca us e mi grai ne. Th e u se o f a n oc tu rna l acryl ic oc clu sa l s plin t, wh ich s ign ific an tly in crease s the vertic al o cc lu sa l dime ns io n, ha s b een advo ca te d by s o me to hel p pa ti en ts wh o wa ke u p in the morn in g w ith migra ine or devel op

migra ine sh ortly after w akin g b ut the evid enc e is no t bas ed o n ran dom iz ed c on troll ed tria ls.

Pai n o f a mig rain ou s typ e ma y al so be a ss oc iated with Mel kerss on Ro sen tha l s ynd ro me (see Cha pte r 12 ).

Cluster headache (periodic migrainous neuralgia/migrainous neuralgia)

Th is co n ditio n, clo se ly a sso c iated with mig ra in e, may als o a ppe ar as a diag no stic pro ble m in

th e ora l med ici ne cli ni c o r in the den ta l s urge ry. Th e a etio lo gy o f thi s c on ditio n is th ou gh t to

be va scu la r ch an ges at the bas e o f th e sku ll . Wh ilst man y fea tures of th e p ain ma y be s imi lar

to tho se of cla ss ica l mi grai ne, th ere are diff eren ces ( Ta ble 15. 7). Th e pa tien ts are muc h more co mmo nl y you n g mal es a nd th ey m ay e xperien c e 1β€“8 a tta cks per da y. Alc o ho l a nd

vaso di lato rs may p reci pitate a tta cks , b ut the y als o oc cu r sp on ta ne ou sl y. C lus ter he ada ch e is

less co mm on tha n m igrai ne . Du ring an attac k there are re peated epis o des of pain , us ua lly o f abo ut 30 min utes in du ratio n, w ith a vari abl e in terval betwe en. Th e pai n i s u su al ly lo ca te d

beh ind an d a rou nd th e e ye, with exten sio n s to the maxi lla an d to th e temp ora l a re a. The pai n las ts fo r 30β€“120 min utes an d i s us ua lly a cc om pan ied by va sc ula r ch an ges . Au to n omi c

sympto ms are o ften pres ent, su ch as we epin g o f th e eye on the affe cted s ide an d co n ges ti on

of th e n as al muc o us memb ran es. The attac ks o ften occu r at reg ula r times at n igh t an d di sturb slee p β€” hen ce th e p seu do nym β€ ala rm cl oc k pa in β€™. Spo n ta neo u s rem iss io n c an oc cur

after s everal mo nth s o f attac ks. Th e ch ara cteris tic g rou pin g

of a tta cks of pai n o ver a sh ort p erio d fo llo we d by lon g p erio ds of re mis sio n ha s l ed to th e

term β€ clu ster h ea dac he β€™. So me p atien ts do n ot h ave th ese epi so dic oc cu rrenc es an d th ey a re the n di agn o sed as ha vin g ch ro nic cl us ter hea dac h es.

Table 15.7 Comparative features of migraine and cluster headaches Migraine

Clu ster h eadach es

Age a nd gen der Sli ghtly more co mmo n in fema les , c an oc cu r at an y a ge, mai nl y

Mai nly you n g ma les, > 20 y ears

2n d β€“3rd deca des Site Usu all y u nil ateral te mpo ral, o cc upi ta l, or fron tal m us cle s

Uni latera l, orbi ta l reg io n

Na tu re o f p ain an d i nten si ty dura tion Severe thro bbin g, pu ls atin g co ntin u ou s,

Severe bo ring epi so dic 30 β€“180 min ,

ti me. Up to 12 attacks per mon th

per da y.

ho urs β€“ days (4β€“ 72 h ) us ua lly day

often at n igh t. Ca n be s everal attac ks

P. 185

Prec ipita ti ng f acto rs Stress , foo ds tu ffs fo r so me p atien ts

Alc oh o l fo r so me patien ts

Pro drom al s ympto ms Au ra -pha se. Co mmo n neu ro log ica l

sympto ms (e.g . vi su al dis tu rba nc es,

Au ra pha se is un co mmo n

spee ch pro blem s, nu mbn es s) Ass oc ia te d fea tu res

Fam ily h is to ry c omm on . May be s en sitive

Fam ily h is to ry un co mmo n . V as cu lar

co mmo n

(l ac rimatio n /na sal co ng estio n)

to li ght, sm ells , no is e. vomi ting is

ch an ges on aff ected sid e

Tre atmen t Avo ida nc e o f preci pitatin g f acto rs

Avo ida nc e o f prec ipi ta ti ng fac to rs.

NS AIDs, 5HT 1 ago n ists.

Su matriptan , NSAIDs

Th e pai n o ften resp on ds to a ntimi grai ne d rugs in the sa me w ay a s m igrai ne itsel f bu t

su bcu tan eou s inje ctio n o f s um atriptan is tho ug ht to be th e treatmen t of ch oi ce duri ng an

atta ck. Th ere a re very c lea r dia gno s ti c c riteria for th is co nd ition , wh ich h as led to h igh -qua lity mul ti cen tre ran do miz ed c on trol led tria ls of th era py.

Charact eristics o f clust er headaches

Mal e to f emal e ratio , 9:1

Predi lec ti on fo r yo un g ma les

Epi so dic sev ere pa in of 30 mi n to 3 ho u r du ra tio n th at can w ake a pa ti en t f rom slee p

Centred ove r orbi ta l reg io n

Acc om pan ied by l acri matio n an d na sal co n gestio n

Alc oh o l ma y pro voke pai n

Tension-type headache

Te ns io n -typ e he ada ch e is n ow the te rm reco mme nde d to des cribe a va riety o f po o rly de fin ed co ndi ti on s, s uc h a s β€ ten si on h ead ach e β€™, β€ stress he ada ch e β€™, an d β€ mus cle

co ntrac ti on h ead ach e β€™, th at a re very co mmo n. Ten si on -typ e he ada ch es are c ha racteri zed

by pa in th at is mi ld to mod erate i n s everi ty, a nd th e di stribu tio n i s bi latera l. The co mpla in t is on e o f a fe elin g o f tig htne ss , c on stric tion , or press ure . Th e pa in may affe ct the fron tal, oc cip ital, an d tem pora l mu sc les . The h ea dac he may be ep iso dic o r ch ron ic an d is n ot

acc om pan ied by s ystemi c di sturb an ce o r n eu ro lo gi cal sig ns . Th e c au se s o f ten sio n -typ e he ada ch e a re po orl y un ders to o d, but m us cu lar ten si on an d s tress are th ou gh t to be impo rtan t. Overw ork, fa ti gue , a n emo ti on al cris is , a nd men strua tion a re

co mmo n preci pitatin g f acto rs. It is w orth n otin g tha t te ns io n -typ e he ada ch es do no t us ua lly wake the patien t fro m sl eep. S imil ar s ymptom s ma y be experi enc ed b y patie nts w ho h ave

drug -re lated hea da ch es, h yperten sio n, o r h yperthyroid ism. A min ori ty of p atien ts ma y s uffe r from both tens io n -typ e he ada ch e a nd migra in e.

Tre atmen t of te ns ion -typ e he ada ch es inv olv es avo idanc e o f trigge r fac to rs su ch as al co ho l, impro vin g s leep pattern s, ta kin g more exerc ise , o r u sin g re laxa ti on tec hn iqu es or o the r

stress -co pin g s tra te gies . Dru g trea tme nts co ns ist of th e in termittent u se of sim ple an alge sic s su ch as asp irin , para cetam ol, o r NSA IDs . Ca ffei ne -co ntai nin g d rugs sh o ul d be avo ided . Mo st

patien ts w ith epis od ic ten si on -typ e he ada ch es ha ve sh o rt-las ti ng atta cks an d res pon d w ell to simp le a na lges ic s. How ever, a ro u nd 2β€“3 p er ce nt of su fferers ha ve c hro n ic ten si on -typ e

hea dac he s w ith i ntrac table sym pto ms tha t are resi stan t to s impl e an al gesi cs . S o me o f the se

are po ten ti ated b y ove rus e o f an alg esic s. Th e u se of tric ycl ic a ntid epres sa nts may b e h elpfu l but, ide all y, sh ou ld be u sed in co n ju n ctio n w ith cogn itive a nd beh avio u ral app ro ac h es.

Facial pain: miscellaneous conditions

Tw o co nd itio ns th at s ho u ld b e men tio ned bec au se th ey m ay ve ry oc ca sio n all y ca us e fa cia l pa in are temp ora l arteri ti s a nd au ricu lo tempo ral syn dro me (F re y's s ynd rom e). It is imp ortan t tha t th e de ntis t is aw are o f the fo rmer le sio n bec au se early d iag no si s c an preven t se ri ou s co mpli catio n s.

Giant cell arteritis (temporal arteritis/cranial arteritis)

Gi an t cell arteritis is an in fla mmato ry co n ditio n th at typic all y af fects th e w al ls o f m ediu msiz ed arteries . The lum en of th e ves se l bec o mes co mpro mis ed a nd, a s a co ns equ en ce,

isc ha emia o f the ti ssu es su ppl ied by the artery ma y o ccu r. Gian t cel l a rteri ti s can a ffec t m an y parts o f th e bo dy (p oly myalg ia rh eu mati ca), bu t th e h ea d is often in vo lved . Wh en th e tem po ra l a rte ry is in volv ed it i s c all ed temp ora l or cran ia l a rteri ti s.

Temporal arteritis

In tempo ral arteritis the pain i s lo ca liz ed to the te mpo ral a nd fro ntal regio n s β€”th is

co rre spo n ds to the area su ppli ed b y the su perfi cia l tempo ral artery. The pai n is d esc ribed as a severe th ro bbin g a ch e, but p aro xysma l pa in is als o oc ca sio n ally des cribe d. In be tw een attac ks of p ain th e a ffec te d are a ma y be very ten der to th e to uc h a nd the tem pora l a rte ry may be

to rtuo us , pron o un ced . Th e p ain ma y be i ni ti ated b y eatin g a nd , a s a co n sequ en ce , wei ght l os s may e ns ue. Th e ma stic atory pain is du e to isc h aemi a o f the mu scl es an d sh o ul d be

diffe re ntia te d fro m tempo ro man dib ul ar pa in dis orde rs. The patie nts, mo st o f w ho m a re in old er ag e gro up s a nd predo min an tly fem ale , a lso co mpla in o f gen eral mal ais e a nd diffu se

mus cu lar an d joi nt pa in s. The re ma y al so be de gen eratio n of visi on be cau se of in vol vemen t of

th e retin al artery a nd su bse que nt is ch aem ia of th e o ptic nerve . Th e e sse ntia l pa th o lo gy o f thi s co ndi ti on is gen era liz ed i nfl amma tio n w ith a n in filtrate o f g ian t cel ls. Th is co nd ition c an aff ect othe r vess els in the he ad a nd ne ck (fo r exa mple , l in gua l arteritis ).

It is imp ortan t tha t th is co nd itio n i s rec og niz ed an d treated as early as po ssi ble, be cau s e it may o therw is e pro gres s to ca us e irrevers ibl e da mage to th e o ptic n erve. P atien ts s ho ul d

th erefo re b e as ses sed on an emerg enc y ba sis . Di agn os is is mad e o n c lin ic al g rou nd s, la rg ely depen den t o n th e e viden tly en la rged a nd pai nfu l artery an d o n eleva ted eryth roc yte

sedi men ta tion ra te (ES R). Th e E SR i s ma rkedl y rais ed (o ver 100 mm i n 1 h ou r) an d C-re acti ve protei n (CRP ) leve ls m ay a lso be elev ated. A no rmal ES R virtua lly rule s o u t tem pora l a rteri ti s. Abs ol ute di agn o sis dep end s o n th e fi nd ing of a typ ica l gi an t-cell in fla mmato ry pro ces s i n a

bio psy of th e a ffec ted ves sel , wh ich s ho ul d o nl y be d on e i n p atien ts w ho h ave a rai sed ES R.

Tre atmen t is with hi gh dos es of sys tem ic stero ids, o f the ord er o f 60 m g o f pred nis o lon e d ail y,

un ti l sympto ms dis appe ar. Pa ti ents are m ain tain ed on a l ow er do s e o f stero ids fo r up to 2 yea rs.

P. 186

Charact eristics o f tempo ral (giant cell) art erit is

A va scu la r dis eas e pri mari ly affe ctin g el derly p eople β€”mean ag e 70 years

Su dden -on set pa in o r hea dac h e tha t m ay b e in itiated or exac erbated by eatin g

Bli ndn es s i s a reco gn iz ed co mpli ca ti on β€”it is th erefo re i mpo rta nt to pro mptly di agn o se

Th e E SR is a bn orma lly hi gh; a rterial bio psy may demo ns trate gra nu lo mato us arteritis

Dif feren ti al dia gno se s: temp oro man di bul ar jo int d iso rders : trigemi na l neu ral gia

Tre at wi th h igh -dos e s ystemi c stero ids

and treat thi s co nd ition

Auriculotemporal syndrome (Frey's syndrome)

Th e au ricu lo tempo ral syn dro me is ca us ed b y da mage to th e au ric ulo temp oral n erve as it

pas ses thro ug h th e su bs ta nc e o f the paro tid g lan d in th e ea rly pa rt of i ts co urs e. It i s a rare co ndi ti on tha t may be the resu lt o f a wid e ran ge of path ol ogi ca l pro ces se s w ithin th e gla nd , both in fla mmato ry an d n eo pla stic , o r o f su rgic al trau ma (po stgu stato ry ne ural gia ). Th e

sympto ms are o f eryth ema an d sw eatin g in th e cu tan eou s dis tri butio n of th e a uri cu lo te mpo ral nerve . Th e symp to ms are u s ual ly i n res po ns e to gu statory stimu li. In app ro xim ately 10 pe r

cen t of cas es th e c on di ti on is ac co mpa nie d by p ain in the dis tri bu ti on o f the au ricu lo tempo ral

nerve . Th e pa in is of ten de sc ribed as β€ burn in g β€™ in n ature . Betw een attack s o f pa in th ere

may b e an ae sthe sia or parae sthe sia o f the skin in th e a ffec te d are a. The te rm β€ gus ta tory

neu ral gia β€™ h as been pro po sed for th is pai n. No effec ti ve treatme nt h as been id entif ied f or th is co nd itio n.

Frey's syn dro me i s a reco gn iz ed c om plic atio n of su rgica l trau ma (for exam ple, pa rotid gla nd su rg ery an d tem poro ma ndi bu lar jo in t su rgery), bu t it ca n als o be du e to ac cid enta l trau ma,

loc al in fectio n , s ympa th etic dys fun ctio n , a nd paro tid pa tho lo gy. It i s e viden t, th erefo re, tha t, on ce th e c on ditio n is reco gn iz ed, co nf irmatio n of the d iag no si s a nd su bseq uen t treatmen t depen d o n a ful l in ves ti gatio n of th e i nvo lve d pa rotid glan d. Th e top ica l a ppli catio n of an anti persp iran t may b e h elpf ul in co ntro llin g th e s wea ti ng .

Neurological disturbances Facial nerve deficits

Def ici ts o f the seven th cran ia l n erve (fa cia l n erve) may re su lt fro m a c en tra l o r peri phe ral cau se an d it i s es sen tial to d istin gu is h betwe en these two types of neu ro log ica l l esi on .

Charact eristics o f Frey's syndro me

Flu sh in g a nd s we atin g in the cu ta ne ou s distrib utio n of th e a uri cu lo tem po ra l n erve

Pai n i s u nc o mmo n (l ess tha n 10 per c en t of cas es )

Tra um a, lo ca l in fec ti on , lo cal su rgery, symp athe ti c dys fu nc ti on , an d ce ntral ne rvou s

fol low in g a gu stato ry stimu lu s

system dis ord ers a re po ss ibl e aetio lo gic al fac to rs

1. Upper moto r ne uro ne les io ns are c en tra l le sio n s tha t affe ct the nerv e fib re s res po ns ibl e

for m ove men t i n th e l ow er fa cia l mu sc les . In su ch a les io n, the mus cl es o ver th e ma xill a and man dib le a re we aken ed, bu t tho se of th e fo reh ead are u n affe cted. Th is i s bec au se

th e mo tor n erve s to th e m us cle s o f the fo re hea d a re regu la te d by b oth cereb ral

hem isph eres , with free cro ssi ng of nerve fib re s. In c on tras t, the mus cl es o f th e lo we r

part o f the fac e are in nerva te d o nl y by n erves co n tro lle d in th e o ppo s ite h emis phe re. In

th ese les io ns the emo ti on al mo vemen ts o f the wh ol e o f the fac e ma y be u n affe cted s in ce th ese are ini ti ated b y u pper mo tor neu ro nes se para te d fro m the pyram ida l mo tor fi bres th at s erve the fac ial mus cl es f or n o rm al fun ctio n .

2. Lo we r mo to r n euro n e les io ns affe ct bo th th e u pper a nd lo wer f aci al m us cle s. The

foreh ea d an d f ace are e qu ally affe cted. In th ese cas es th e p atho lo gic al pro ces s i s

periph era l to th e fa cia l m oto r nu cl eus in th e p on s. Th e mo st c omm on ca us e o f s uc h a paral ysi s is B ell's pa lsy (dis cu sse d belo w) but a wi de va riety o f le sio ns af fectin g th e

faci al nerve bel ow the b as e of th e s kul l (in cl udi ng so me i n th e pa rotid gla nd) may b e re spo n sib le fo r symp to ms . E mo tion al mo vemen ts a re al so lo st in th e area si nc e the lesi on respo n sib le a ffec ts the co mmo n path of all th e p eriph eral fibres .

Perip hera l pa ral ysis is su bdi vided in to in tra petrosal an d extrap etro s al l esi on s. Whe n th e le sio n affec ts th e fac ia l nerve in the intra petros al tra ct, th e c ho rda tym pan i a nd stape diu s n erve are als o i nvo lve d. There may, the refo re, b e a hea rin g def ici t (h ype racu si a) a nd los s of ta ste o n

th e a nterio r two -th irds o f the to ng ue on the affe cted s ide . S in ce th e c ho rda ty mpan i i s g iven

off with in th e f aci al can al at the bas e o f the sku ll, le sio n s o cc urrin g b elo w th is wil l n ot a ffec t th e s ens e o f ta ste.

Th e n erves res po ns ible fo r moto r fu nc ti on to th e face are s ho w n i n Ta ble 15. 8.

Anaesthesia and paraesthesia

Th e o n set o f a na esthe sia or p arae sthe sia , e ither slo wl y o r su dden ly, in the dis tri bu ti on o f a

nerve may ha ve lo ca liz ed or g ene rali zed sig ni fica nce; Ta ble 15. 9 lis ts po ss ibl e co n ditio ns tha t may c au se oro fac ial an aes thes ia or para esth esi a.

Lo ca l c au ses of an aes thes ia in bran ch es of th e tri gemi na l n erve in cl ud e dire ct trau ma to the nerve (as in frac tu res , trau ma ti c

extra ctio n s, sa gital spl it os teoto mie s), press u re on th e ne rve by a fore ign bo dy (s uc h a s a

P. 187

to oth ro ot o r roo t ca na l me dic amen t), i nfl amm atory ch an ges in rela ti on to th e n erve

(e spe cia lly o s te omye litis ), a nd ne opl as ms enc roa ch in g o n th e n erve. Mo re g ene rali zed

neu rol og ica l c on ditio n s th at may affe ct the tri gemin al n erve in thi s w ay i nc lu de mu ltip le

scl eros is an d the neu ro path y of mixe d co n nec tive tis su e di sea se (Cha pte r 11). S imil ar c ha ng es may o cc ur as th e res ul t of a va ri ety o f in tra cra nial spa ce -fill ing les io ns su ch as an a co us ti c neu rom a ( Fig. 15. 2)

Table 15.8 The nerves responsible for motor control of the head and neck Nu mber

Cr anial ner ve

Muscles

V

Tri gemi na l

Mas ti ca to ry mus cl es

VII

Fac ial

Mus cl es o f f aci al expres sio n

IX

Gl os so ph aryn geal

Uvul a, so ft pa late, bas e o f ton gu e, ph aryn x

XI

Acc es so ry

Tra pez iu s

XII

Hy pog lo ssa l

To n gu e

Charact eristics o f upper mo to r neuro ne disease

A c entral les io n th at c au ses partia l pa ralys is of th e fa ci al m us cle s

Th e lo wer f aci al m us cle s, co n tra latera l to th e le sio n , a re pa ralys ed

Th e o rbi cu laris o cu li a nd fro ntal is m us cle s, wh ic h recei ve bi latera l c ortic al f ibres , h av e

Th e b lin k refl ex an d m ove men t o f f oreh ea d mu sc les un aff ected

limi te d fu nc ti on

Charact eristics o f lo wer mo to r neuro ne disease

A le sio n affe ctin g the fac ial nerve al on g its in tra - or e xtra petros al co urs e

Th ere is p aral ysi s o f the fac ial mu sc les aro un d th e eye, fo reh ead , a nd mou th, the bli nk

Th ere may b e tas te an d h earin g d efic its i f the les io n i s s itua te d with in the i ntrap etros al

re flex is l os t, a nd droo li ng is co mmo n tra ct

Causes o f facial palsy In tra cra ni al cau s es

Extrac ran ial ca us es (inc lu din g in tra petros al les ion s )

Cerebro vas cu lar acc ide nts

In co rrect p lac emen t of lo cal an aes th etic

Cerebral tumo u rs

Ia tro gen ic (fo llo wi ng re sec tion o f tum ou r)

Ne uro lo gic al d ise ase s

Paro tid tu mo ur

Mul ti ple sc lero sis

He erfo rdt's s ynd ro me

He ad in ju ries

Melke rss on β€“Ro se nth al s yn drom e (Cha pte r 12 )

Ra mse y Hun t syn dro me (Cha pte r 4)

Table 15.9 Conditions that may cause orofacial anaesthesia or paraesthesia

Lo cal

System ic

Lo ca l an aes th etic

Mul ti ple sc lero sis

Tra um a

In tra cra ni al les io ns

Re so rptio n o f th e al veol ar ri dge expo si ng men ta l

Hy perven tila ti on /te ta ny

fora men

Medi catio n

Psyc h oge nic

In fec ti on (o steo myeli ti s, h erpes zo ster) Mal ign an cie s

Fo llo wi ng a c erebro vas cu lar a cc iden t there may be a ran ge o f neu ro lo gic al a bn orma litie s affec ting the fac e an d mo u th . Amo n g the mo re diffi cu lt to d eal wi th are th e c ha ng es i n

sen sa ti on , prop rioc eptio n , a nd mus cu la r co ntro l, whic h ma y ma ke de ntu re -wea ring very diffi cu lt fo r th e a ffec te d patie nt.

Fo llo wi ng a c erebro vas cu lar a cc iden t there may be a ran ge o f neu ro lo gic al a bn orma litie s affec ting the fac e an d mou th.

Bell's palsy

Bel l's pals y is the na me gi ven to a n a cu te pa ralys is of th e fa ci al n erve. It is prac ti ca lly a lw ays un ila te ral a nd no o bvio u s c au se i s fou n d in the vast majo rity of ca ses . It is tho ug ht tha t so me cas es

may h ave a viral aetio lo gy; p os sib ly h erpes si mple x viru s. Be ll's pa lsy is th e mo st c om mon

P. 188

low er mo tor neu ron e l esi on an d, dep en din g u pon th e se verity o f the co nd ition , so me o r al l o f th e mu sc les o f expre ssi on o n th e a ffec ted si de ma y be wea ken ed o r para lyse d. The extent o f th e pa raly sis is eas ily seen if the p atien t is as ked to smi le, to c lo se th e ey es, an d to fu rro w

th e bro w. Th ere m ay o r may no t be l os s of ta ste s ens atio n on the an terio r latera l pa rt of th e to ng ue on the affe cted sid e, depen di ng on wh eth er or no t th e c ho rda ty mpan i n erve is

invo lve d. App roxi mately ha lf the patie nts co mpla in of sev ere pa in in th e a rea of th e paro tid glan d o r the ear β€”sprea din g do w n th e ma ndi ble β€”alth ou gh th is gen era lly prece des th e

paral ysi s o r o ccu rs in th e ea rly s ta ges o f the co ndi ti on . The fac ial app eara nc e is ch ara cteris ti c. Th e i mpa ired f un ctio n of th e s even th cran ia l n erve c an be demo ns trated by as king the patien t to sm ile, c lo se th eir eyes , a nd attempt to purs e their li ps. Th e af fected sid e w ill be u na ble to perfo rm the se a ctio ns .

Fig. 15 .2 Mag netic reso na nc e s ca n s ho wi ng a ma ss in the cereb ello po n ti ne an gle.

(P atie nt prese nted with β€ faci al nu mbn es s β€™)

Pro mpt dia gn os is an d treatmen t of Be ll's pal sy m ay pre vent f un ctio na l an d ae sthe ti c impa irmen t.

One cu rren tl y reco mme nde d treatmen t regi me fo r B ell's pa lsy is a co mb ina tion o f s ystemi c

steroi ds an d ac ic lov ir. Th e a ntiv iral is a dvo ca te d bec au se of th e po s sib le in vol vemen t of th e herpe s s impl ex vi ru s . A cic lo vir i s gi ven at a dos e o f 400 m g, fou r time s da ily. S te roid s a re

admi ni stered i n h ig h d os age β€”60 mg predn is ol on e dai ly fo r 5 day s, th en redu ci ng th e d os e over th e s ame perio d o f time , i s a reas on ab le reg ime. Th e ratio n ale beh ind this ap pro ach is

un prov en, bu t it is th o ugh t tha t steroi ds may b e advan ta geo us by re du cin g o ede ma a rou nd the faci al nerve . S o me w orkers h ave s ug ges te d tha t it nee ds to be in stituted with in th e first

24 β€“ 72 h ou rs o f o n set o f the Bel l's pal sy. Oth ers s ug gest th at it s ho ul d be a do pte d o nl y if

spo nta neo u s res ol utio n do es n o t ta ke pl ace . Th ere is n o co nc lu sive evid enc e fro m ra ndo mi zed co ntrol led tri als tha t s teroi ds or a ntivi ral agen ts, gi ven alo ne or in co mbin atio n , p rovi de a ny

lon g -term ben efit fo r patie nts w ith Bel l's pal sy. Non ethe les s, it is the au th o rs β€™ vie w th at, un les s th ere a re s ign ific an t co ntra ind ica ti on s to stero id th era py, it sh o uld be s tarted with a system ic a ntiv iral agen t at th e ea rl ies t opp ortu nity a fter di agn os is . Thi s is in an a ttemp t to

avo id th e po ss ibi lity o f lo ng -term co sme ti c d efo rm ity th at a cc omp an ies an un res ol ved fa cia l

pals y. It i s im po rta nt to protec t an eye th at rem ain s p artial ly o pen , eith er by a n e ye pa d o r sh ade.

Management o f Bell's palsy

In itia l h igh -dos e pre dni so lo ne, red uc in g after 5β€“7 days

System ic an ti viral th era py

En su re ad equ ate eye protec tion

Mo st patie nts with Be ll's pal sy rec ov er wi th in a few weeks , bu t f or th e ch ro nic ca se s

su ppo rti ve o r co rrective tre atmen t may b e n ece ssa ry. A s impl e sp lin t or a mo dif ica ti on to an exis ti ng den tu re ma y h elp s u ppo rt th e s oft tis su es an d imp rove th e fa ci al prof ile of th e

affec ted s ide. Refe rra l to ph ysio the rapy d epartme nts for elec tri ca l sti mul atio n o f th e pa ralys ed faci al mus cl es m ay a lso be he lpfu l. Galva ni c s ti mulatio n i s c on si dered to b e wo rthw hi le

beca us e it s timu lates mu sc le co ntrac tion , po ss ibly prom otes mo to r en d-plate fun ctio n , a nd is of p syc ho lo gic al ben efit. Han d -hel d, patien t-ope rated s mal l n erve s ti mul ato rs are no w

avai lab le for th is pu rp os e. Su rgic al trea tmen t m ay b e co n sid ered i n s om e pa ti ents fo r co sme ti c re aso n s, bu t th ere i s l ittl e evi den ce th at thi s a ppro ac h i s s uc ces sfu l. Fac ia l pa lsy as a

man ifes tatio n o f th e Me lkerss o n β€“Ro se nth al s yn drome is fu lly dis cu ss ed in Cha pte r 12 .

Multiple sclerosis

Mul ti ple sc lero sis (dis se min ated sc lero sis ) is a d isea se featu rin g pro gres sive demy elin atio n of nervo u s tis su e with epi so des of rela pse an d remi ssion . Thi s res ul ts in perm an ent a nd

inc reas in g dis abi lity. The co ndi tion a ffec ts mai nl y yo un g a dul ts , it is sli ghtly mo re preva len t i n femal es, a nd th e u su al age of on se t i s 20β€“40 yea rs. The dis eas e can pre sen t wi th a wid e

ra ng e o f sym ptoms ( Ta ble 15. 10) depe ndi ng upo n the sites of les io ns in the b rain . Di agn o sis

of th e c on ditio n depe nds u pon c lin ica l fe ature s, mag netic res on an ce ima gin g (to demo ns trate th e cen tral a reas o f dem yelin atio n ), a nd excl us io n o f o the r neu ro lo gica l defi cits.

Patie nts with mu ltiple sc lero sis (MS ) ma y pres ent with pa in re sem blin g trig emin al neu ral gia . Th is ca n oc cu r at an y s ta ge o f th e dis eas e an d i t can be a p re sen tin g fea tu re. If trigem ina l

neu ral gia is th e fi rst man ife statio n o f M S, the patie nts a re o ften you n ger tha n th e trige min al neu ral gia pop ul atio n a s a wh o le a nd th eir neu ral gia is often bila teral.

Th e c lin ic ian s ho ul d, ho we ver, co ns ider th e po ssi bili ty of mul ti ple sc lero sis wh en a patien t

you ng er tha n 50 yea rs o ld prese nts with sym ptoms sugge stive of trig emin al ne ura lgia . It is

impo rtan t, ho we ver, th at thi s s us pic io n i s n o t v oiced to the patie nt u ntil th ey h ave been ful ly ass ess ed b y a n eu rol og ist. Mu ltipl e sc lero si s d oes n ot n ece ssa rily give rise to s ympto ms th at

mimic c las sic al tri gemi na l n eura lgi a β€” it may g ive ri se to persi sten t pai n, wi th n o iden tifia ble trig ger a reas . P ara esth esi a an d a llo dyn ia may a ls o b e fea tu res of th e c o ndi ti on . Mu ltiple neu rol og ica l de fic its a re us ua lly p rese nt su ch a s mu sc le wea kne ss, vi su al d istu rban ces , and sen so ry lo s s. The man age men t for th e fa cia l pa in is si mila r to th at fo r tri gemi na l neu ral gia.

Table 15.10 Common symptoms of multiple sclerosis Type of sym ptom

Descr iptio n

Vis ua l

Lo ss o f vis ua l ac u ity an d c olo u r vis io n a nd eye p ain (du e to

We akn ess of th e

Ti ng lin g o r para esth esi a m ay be prese nt

limb s

Vertigo

optic n euri ti s); di plo pia

P. 189

Ataxia

Mul ti ple sc lero sis (MS ) is dia gn os ed in 2 β€“4 p er ce nt o f pa ti ents wi th tri gemi na l n eura lgi a.

Extrapyramidal syndromes

Extrapy ra mid al s yn drom es a re n euro lo gic al dis ord ers th at a ffec t p athw ays o th er th an th e

prin cip al o n es c on ce rned wi th vo lu ntary mov emen t. Th ey a re ch ara cteriz ed by ab no rmal ity o f mus cu lar actio n wi th tremo rs. Pa rk ins o nis m is the bes t kno wn co n ditio n of th is kin d.

It h as recen tly b eco me e viden t, h o weve r, th at sy mptom s o f a sim ila r kin d ma y oc cu r fol lo win g th e u se of certai n d ru gs , e spe cia lly in elderl y pa ti ents . In partic ul ar, sed atives an d

tra nqu il liz ers o f th e ph en oth iaz in e gro u p h ave b een impl ica ted. The se p atien ts ma y

oc cas io na lly co me to th e d enta l su rgeo n fo r dia gno si s s in ce th e dys kin etic symp to ms are often mos t evide nt in th e mu sc les of th e m astic ato ry app aratu s. Thi s c an ma ke den tal trea tmen t diffi cu lt. Re petitive mov emen ts o r tremo r of th e tongu e o r ma nd ible , w hi ch ma y be o f a

biz arre n atu re, sh ou ld be s us pec ted as ha vin g a drug -ind uc ed o rigi n i n p atien ts o f thi s o lde r age g rou p. Treatme nt is by w ithd raw al o f th e o ffen din g dru g, al th o ugh res ol utio n may ta ke so me time .

Extrapy ra mid al s yn drom es a re n euro lo gic al dis ord ers th at aff ect pa th w ays oth er tha n th e prin cip al o n es c on ce rned wi th vo lu ntary mov emen t.

Discussion of problem cases Case 15.1 Q1

Wh at is the us ua l dru g o f c ho ic e fo r thi s patie nt an d w ha t startin g do s e wo u ld yo u presc ribe?

Carbam az epin e i s th e firs t drug of ch oi ce. A reas onabl e regi me w ou ld be to co mme nc e the patien t on 100 m g twi ce dai ly, in crea sin g by 100 mg every two to th ree days un til th e

paro xysms o f pa in are c on trol led. Ca utio n is re qui red in el derly patien ts a s th ey ma y be very su sce ptible to th e si de -effec ts . A pa in dia ry is usefu l to mo ni to r the effec ts of th e d ru g. Q2

Wh at w arn ing s a nd in forma tio n w ou ld you gi ve to th e pa tien t?

Th e p atien t sh ou ld be w arn ed a bo ut the fo llo win g p os sib le s ide -effec ts : ti redn ess ; diz z ine ss ;

ataxia ; do ub le vi sio n ; an d in ab ility to co nc en tra te β€”patien ts rep ort tha t th ey f eel β € spa ced ou tβ€™ o r like a β€ zo mbie β€™.

Th ey sh ou ld b e in stru cted:

no t to dri ve wh il st experi enc in g the si de -effec ts li sted;

to be cau tio us wh en drin kin g a lco h ol ;

to co n ta ct the ho sp ital o r me dic al p ractitio n er if a ras h devel ops .

Th e p atien t sh ou ld be to ld th at the drug is an an tico nv uls an t an d n ot a co nve ntio na l a na lges ic . It s ho ul d, th erefo re, be ta ken regu larl y, no t β€ on dema nd β €™ li ke o th er a na lges ic s a nd,

prefera bly, 30 m in utes befo re a mea l. The patie nt sh o ul d be to ld th at the y ne ed to be

re view ed reg ul arly, tha t th e d os e o f an ti co nvu ls an t may n eed to b e in crea sed , a nd tha t b lo od tes ts w ill be req ui red. Pa in relie f sh ou ld oc cu r, for th e ma jority o f p atien ts, wi th in 24 h o urs , alth ou gh th e f ull thera peu tic b ene fit ma y no t be a tta in ed fo r 2 β€“ 3 wee ks. Q3

Wh at in vestig atio ns do you n eed to c arry o ut be fore startin g o n th is drug ?

Th e p atien t sh ou ld ha ve bl oo d take n so tha t s o me basel in e in dic ato rs c an be o btai ned (ful l blo od co un t, l iver fun ctio n tests, a nd elec tro lytes; see text). Q4

If you r pa ti ent h ad prese nted ta kin g w arfari n, wou ld yo u env isa ge a ny m an age men t prob lems ?

Carbam az epin e i s a he patic mic ros om al e nz yme ind uc er. There fore , i f ta ken co nc u rre ntly w ith

any oth er dru g tha t is metabo li zed by th e li ver, a pha rmac ok ine ti c d ru g i ntera ctio n w ill o ccu r. In thi s c ase wa rfarin w ill be metabo li zed mo re qu ickly a nd, c on seq uen tly, the seru m w arfa ri n level s w ill fall . Thi s w ill inv aria bly red uc e the effi cac y o f w arfari n a nd th e p atien t's

proth romb in ti me w ill be a ltered β€”th e in tern atio nal n orma liz ed ratio (INR) w ill drop . Th is

co uld in crea se a pa ti en t's su sc eptib ility to thro mbo embo li c s equ ela e. The dos e o f w arfa rin wil l, th erefo re, pro bab ly n eed to be inc reas ed a nd mon itored w hi lst th e pa ti ent i s taki ng

carb ama zep ine an d fo r a sh ort time after ces sa ti on o f the an ti co nvu ls an t. P atien ts o n warf arin carry a wa rni ng ca rd an d th is wi ll g ive i nfo rma ti on ab ou t th eir dos e, INR, a nd a co n ta ct

nu mber f or th eir an ti co agu la ti on cl in ic. Th is cli nic w ill adju st the patie nt's warfa rin do se if nec ess ary.

Case 15.2 Q1

Ho w wo ul d yo u man age this situ atio n?

Th e p atien t is exh ibitin g symp to ms of a l ow er mo tor n eu ron e pa ls y. Yo u ne ed to ch eck th at th e s ign s a re un ila teral an d tha t th e pa tien t ha s not rec en tl y bee n ex perien ci ng fac ial

wea knes s or s ympto ms o f a ne uro lo gic al defic it, su ch as para esth esi a. The left -side d fa cia l mus cu latu re s ho ul d be ch ecke d fo r fu nc ti on to ens ure th at th is is a lo we r mo to r n euro n e

prob lem a nd that th e pa ti en t i s n ot s uf ferin g fro m a n upp er mo tor n eu ron e l esio n su ch as a cereb ro va scu la r ac cid ent; a ltho u gh th is wo ul d be unlike ly in th is p articu la r ca se. Th e mo st likel y dia gn os is is a tra ns ien t fac ial ne rve pa lsy resu ltin g fro m the dep os itio n o f l oc al

ana es th etic aro un d th e se venth cra ni al n erve (us ua lly i nto or n ea r the p aro ti d gl an d). Fac ia l

nerve para lysi s i s ev iden t with in min u tes o f the inje ctio n a nd the dura tion o f fa cia l w eakn es s

is u su al ly le ss th an 12 ho u rs. If a l on ger -actin g lo cal an aes thetic so lu tio n s uc h as bup ivac ai ne has be en u s ed the n re so lu ti on wil l be dela yed. It is, h ow ever, u nl ikely th at b upi vac ain e w ou ld be ro utin ely us ed fo r res to rativ e pro ced ures . Th e f ol low in g acti on s a re ad vise d.

P. 190

Re ass u re the patien t; expl ain tha t it is an un co mmon bu t wel l reco gn iz ed c omp lic atio n of

th e a na esth eti c tec hn iqu e th at w as u s ed.

Use an eye p atch o ver the affe cted e ye to p reven t co rnea l d ama ge (the patie nt s ho ul d

Ass es s the gen eral co n ditio n o f th e patie nt an d c ontin ue trea tmen t if s he i s h ap py to do

be w earin g p rotec ti ve gl ass es duri ng th e tre atmen t).

so . If th e pa ti ent app ears dis tre sse d, th e to oth shou ld be temp ori zed (furth er

prepa ra tio n ma y be requ ired f or th is) to en su re that th e p atien t wi ll n o t e xperie nc e an y pos tope rative d isc om fort o r pa in from th e m ol ar tooth.

Re view ne xt day to en su re tha t th e p aral ysis h as reso lved . A telep ho n e cal l, if p os sib le,

Wri te th e d eta ils o f the in cid ent c lea rl y in the case n o te s s ta ti ng wh at in fo rmatio n was

later th at day is goo d p ractic e.

given to th e pa tien t an d the detail s f or pa tien t fol lo w up.

Projects 1.

Des cri be h ow yo u w o uld as ses s th e fu n ctio n of th e c ran ial nerve s.

2.

Des cri be th e i ntrac ran ial co urs e o f the trigemi nal a nd fac ial nerve s.

3.

Wh at in fo rmatio n is ava ilab le f or pa tien ts w ith trige min al neu ral gia : (a) in the fo rm o f

4.

leaf lets; (b) o n th e in terne t?

Acu te s inu si ti s c an be mis ta ken fo r pai n o f de ntal ori gin ; wh at i s the evid enc e fo r the

effic ac y of an ti mic robi al th erap y in this co nd ition?



Copyri gh t Β©2003 Oxfo rd Un ive rsity P ress > Tab le of Cont e nts > 16 - Te m p oro man dib ular d is ord e r s

16 Temporomandibular disorders Problem cases Case 16.1

A 20-year -old fema le p atien t attend s yo u r su rgery fe elin g f everis h a nd co mpl ain in g o f a pai n over th e le ft sid e o f h er jaw. S he is v ery wo rried bec au se sh e ca nn o t o pe n h er mo u th fu lly. Th es e s ymptom s ha ve de velo ped over th e pa st 24 ho u rs. Exa min atio n sh ow s a ma ximu m ope nin g o f app ro xim ately 12 mm. Q1

Wh at a re the like ly ca us es for th is patie nt's trismu s?

Q2

Li st the pos si ble a etio lo gies fo r ac ute an d ch ro ni c li mitatio n of mou th ope nin g.

Case 16.2

A 70-year -old eden tul ou s p atien t pres ents to yo ur prac ti ce w ith bila teral pain l oc ated o ver th e

tem po ro ma nd ibu lar jo in t an d in the pre -auri cu lar regio n. Th ere i s n o his tory o f l oc kin g. He h as been wea rin g h is c om plete den tu res sati sfac tori ly fo r 30 y ears . Th e pa tien t repo rts th at th e

pain h as been ge ttin g g ra du all y wo rse ove r the p as t 3 ye ars a nd thin ks th at i t m ay be du e to

β€ arthritis β €™. He is no w getting β€ a bi t f ed -up β€™ wi th th e p ain . His d au gh ter wo nd ers i f he m igh t nee d n ew den tu res. S he co mmen ts, β€ his mo uth se ems to h ave c ave d in an d h e

doe sn β€™t lo ok as i f h e h as an y te eth β€”he i s a lso fi nd ing it dif ficu lt to eat. β€™ B ila tera l

crepi tu s is a ud ible ove r th e tem poro ma ndi bul ar jo ints an d th ere is ten dern ess of the m as seter and te mpo rali s mu sc les o n p alpa tio n. Ma ximu m o pen ing is in exc ess o f 40 m m. The patie nt is gros sly ove rclo se d du e to exce ssi ve o cc lu sal we ar on th e den tu res . Q1

Wh at c on ditio n s may b e res po ns ible fo r th is patient's pai n a nd wh at co u ld yo u do , i n

Q2

Th ere are s evera l ca us es of pai n th at ma y prese nt in o r ad ja cen t to th e

th e first i ns ta nc e, to h elp yo u rea ch a defin itive dia gno s is?

tem po ro ma nd ibu lar jo in ts. Ma ke a l is t o f th e po ssi ble diffe rentia l di agn o ses (you w ill nee d to u se in form atio n f rom oth er ch ap te rs to an swe r this fu lly).

Introduction

Patie nts with symp to ms fro m the te mpo roma nd ibu lar jo in t (TMJ) a re frequ en tly s een an d

man age d by d enta l prac ti ti on ers . S om e pa ti ents are, h ow ever, referre d fo r sp ecia lis t ca re, eithe r to o ral medi cin e, maxi llo fa cia l, or re stora ti ve de ntis try depa rtm ents or to

mul ti dis cip lin ary c lin ic s th at are d edic ated to di so rders of th e TM J. Th e to pic of TMJ pro ble ms has ge nera te d a pleth ora of litera tu re, bu t a grea t dea l o f co nfu s ion s ti ll e xists as to th e

term ino lo gy, ae ti ol ogy , a nd man ag emen t of TMJ co nd ition s . U nfo rtun ately, th ere is very l ittl e objec tive s ci enc e to su bstan tiate mos t of th e p ubl ica tion s .

Wh en an al ysin g s ympto ms aris in g fro m the TMJ i t s hou ld b e bo rne in min d th at there are tw o distin c t so urc es of s uc h symp to ms . Th e fi rst is from the mus cl es, jo int s tru ctu res, a nd oth er

ass oc ia ted tiss ue s a s a resu lt o f ab no rmal ph ysic al ac ti vity wi th in the join t (dys fun ctio n ). Th e

sec on d is fro m pa tho lo gic al c h ang es in th e jo in t itself an d, in su ch ci rcu mstan ce s, the jo in t

dama ge m ay be as so cia ted w ith a sys temic abn o rm ali tyβ €”rh eu mato id a rthritis is an exa mple of th is situ atio n. Di fferen tiatio n betwe en th es e may be ve ry diff icu lt w hen the patie nt fi rst

prese nts. Th e ma jo rity o f c as es o f pa in an d o ther symp to ms aris in g fro m the TMJ resu lt fro m a dysfu n ctio n s yn drom e rath er tha n a ny prima ry path olog y of th e join t. Te mpo rom an dibu la r join t pro blem s ma y be du e to: (1)

a dys fu nc ti on syn dro me

(2)

patho lo gi cal ch an ges in clu din g: in terna l deran gem ent

ap las ia, h ypo pla sia , hyp erpla sia

arth ritic c o ndi ti on sβ €”tra uma ti c arthri ti s, in fectiv e arth ritis, rh eum atoi d arth ritis,

os te oa rth ros is , an kylo sin g s po nd ylitis , c rystal -induc ed a rth ritis (go ut), pso ria ti c arthro path y

ma xillo fa cia l trau ma β€” man dibu la r co nd ylar frac tu res dis lo ca ti on an kylo si s

n eop las ia

Investigation of the stomatognathic system History

A h isto ry sh o ul d be ta ken as previo u sly des cribe d (Cha pte r 15). Th is mus t in clu de th e ch arac te r, in ten s ity, f re que nc y, dura ti on ,

site, an d ra dia ti on o f an y pai n. Ex ace rbatin g, relievin g, an d as so ci ated f acto rs s ho u ld a lso be

P. 194

iden tified . In di so rders of th e TM J th e f ol low in g fo u r featu res a re very impo rtan t in hel pin g th e cli nic ia n a rrive a t a di agn os is .

1. Pai n hi story . P ain ma y o rigin ate p rima ri ly w ithi n th e TMJ or fro m th e mus cl es o f

mas ti catio n β€”both arthra lgi c a nd myo gen ic p ain ma y be prese nt. Pa in may b e fel t as a dul l ac he ov er the area of th e jo in t, the ear, o ver the te mpo ral f os sa , o r o ver the maxil la. Th e pa in may be bi latera l o r un il ateral and is us u all y des crib ed a s be ing

co ns ta nt, bu t with ac ute ex ace rbatio ns . It is duri ng th es e ac ute e xac erbatio n s tha t the ra dia ti on o f the pai n f rom th e join t o fte n o cc u rs . In s ome in stan ces as so ci ated p ain in th e n eck , u ppe r arm, oc cip ital area, o r al on g th e li ngu al ne rve ma y be re ported . Th e severe attack s of pai n o cc ur p redo min an tly in the earl y mo rnin g i n s o me pa ti en ts ,

wh ereas in oth er pa ti ents th ey a re mo re c om mon a t th e en d o f th e da y. A cu te epi so des may als o be pre cip itated a fter a m eal, a t th e w ide ope ni ng of th e mo u th , or d urin g th e nig ht w hen lyi ng he avil y on o ne si de o f the fac e.

Mus cu la r pai n a ss oc iated wi th tempo ro man dib ula r pa in may c au se a hea dac h e. It is

pos si ble th at so me prac ti ti on ers may co nfu se th e d iag no si s with mig rain e. Si mila rly,

pain a ri si ng w ith in th e jo in ts the msel ves may b e attri bu te d to e arac he . M an y pa ti ents fou nd to h ave pai nfu l jo in ts w ill ha ve ha d an ea r exa min atio n w ith neg ative resu lts.

2. Jo in t so u nds . Th e pa tien t mos t co mmo nl y co mpla in s o f a cl ick. Th is cli ck rep resen ts th e

move men t of o n e co mp on en t o f th e jo in t ove r th e o thers , an d ca n mean tha t th e d isc is

sli ppin g o ut o f pla ce, stic kin g, or malf un ctio ni ng . Jo in t so un ds are q ui te co mmo n an d are no t alw ays si gni fic an t. Th e pres en ce o f a cl ick does n o t, o n i ts o wn , i nd ica te th at

trea tmen t i s req ui red. Clic ks m ay be qu ite lo u d an d rea dil y au dib le, an d th is may ca us e

so cia l em barras sm ent w he n e atin g. In o th er c as es, how ever, a stetho sc o pe is requ ired to hea r the s o un d. Al th ou gh an a cu te epis od e o f pa in an d cl ick ing may be pre cip itated b y move men t of th e TM J, pa tien ts ma y fin d th at pa in is as so cia ted w ith p erio ds in wh ich

cli ckin g i s mi ni mal. Th ere m ay be a cl ick o n bo th open in g a nd c lo si ng β €”th is is referred to as a re cip roc al cli ck. Ap art fro m the sin gle lo ud cli ck, o th er s ou n ds m ay be he ard i n

th e join t o n s te th o sc opi c ex amin atio n (au sc ul ta ti on). Crepitu s (a gratin g o r grave l-like

so un d) is mo st c omm on ly h ea rd in o steo arthro si s. Care m us t be ta ken in the us e o f the stetho sc op e to e limi na te the crac klin g s ou n d pro du ced by la yin g the bel l o f the

ins tru me nt o ver h air. Th e res ultin g c rac klin g s ou nd can ea si ly be mis ta ken fo r join t crepi ta ti on .

3. Re strictio n of op eni ng . Th e pa tien t may repo rt d iffi cu lty o n w ide ope ni ng, o ften

ass oc ia ted wi th the imm ine nt o ns et of a lo u d c lic k. In oth er in sta nc es th e di ffic ul ty m ay

be in a pplyi ng press ure on clo si ng th e m ou th. The in abi lity to op en th e mo u th w ide, du e

to refl ex mus cu la r spa sm (co ntrac ti on ) o f the mas tica to ry mu sc les , is c al led tris mu s a nd is u su al ly a te mpo rary ra th er th an perma ne nt co n ditio n. P atien ts w ith TM J prob lems

often co mpl ain th at thei r jaw lo cks . Th is may mean tha t th e ja w can n ot o pen n orma lly.

It s ti cks or β€ loc ks β€™ an d th is restric ts o pen ing . Thi s is tho u ght to be d ue to the dis c bein g β€ squ as hed an d bu n ch ed u pβ€™ an te riorl y, preven ting fu rth er o pen in g. This is

us ua lly terme d a β€ clo se d lo ck β€™ si tu atio n an d i s ma in ly du e to an terio r dis c

disp lac emen t wi th o ut redu ctio n a nd may fol lo w d isc la xity an d s tre tc hi ng or p os sib ly

tea rin g o r perfo ratio n in th e p os te ri or b ila min ar zo ne a rea of th e di sc . A ltern ativel y, th e term β€ loc kin g β€™ refe rs to th e ma nd ibl e tempo raril y bec omin g β€ stuc kβ€™ in an

ope n p os itio n β€” th e pa tien t is n o t able to c lo se o r o pen the jaw furth er. This co mp lai nt

is n o t th e s ame as dis loc atio n . Dis lo cati on refers to th e d isp lac emen t of th e h ea d o f the co ndyl e o ut o f the gle no id f os sa to a po sitio n an terior to the artic ula r emi nen ce .

4. Swe lli ng . P atien ts w ith TMJ di sturba nc es oc ca sio n ally co mplain o f sw ell ing ov er the

maxil la, bu t there is no cl ear reas on fo r thi s. A slig ht de gree o f s oft-ti ssu e s wel lin g m ay

be o cc asi on al ly no ted o n exami na ti on . In a few oth er in stan c es p atien ts ma y co mpl ain o f ten dern es s a nd sw elli ng in th e a rea o f th e pa rotid , pre su mab ly an ef fect brou gh t abo u t

by the clo se pro ximity o f p art of th is gla nd to th e TMJ. In th es e ca ses it ma y be a ma tte r of s o me di ffic ul ty to dis tin gui sh betw een paro tid invo lvem ent i n jo in t dis tu rban ce or jo in t in vol vemen t in a p aro ti d path ol ogy.

Tri smu s is th e in ab ility to op en th e mo u th w ide d ue to refl ex mu sc ul ar s pas m

(c o ntrac ti on ) o f the mas ti ca to ry mu sc les an d i s u suall y a tem pora ry rath er tha n perman en t co nd ition .

Dis lo ca ti on o f the TMJ ref ers to the dis pla ceme nt of the he ad o f th e co n dyle ou t of th e glen oi d fo ss a to a p os itio n an te rior to the artic ula r emi nen ce .

Th o rou gh den tal, med ica l, an d s oc ial hi sto ries are req ui red. An y h isto ry of tra uma to th e TM J sh ou ld b e exp lore d an d th e o utco me s o f an y pre vio us tre atmen ts , if a ny, sh o ul d be

asc ertain ed. It is als o impo rtan t to ide ntify if the p atien t su ffers from oth er pa in syn dro mes , and if th ey h ave hi gh level s o f emo ti on al stres s. (Cha pte r 17 des cribe s th e as ses sm ent o f psyc ho lo gic al fac to rs in so me de ta il. )

Examination

Th e c lin ic ian s ho ul d h ave a s ou nd kno wl edge of th e f un ctio na l a na to my o f th e TMJ an d

ass oc ia ted stru ctures pri or to u nd erta kin g a n e xamina ti on o f the patien t. E xa min atio n o f th e TM J an d mas ti ca to ry mu sc les sh o uld begi n b y o bserving th e d egree of symm etry of th e

man dibl e an d f ace , a nd by o bs ervin g the path of excursi on o f the man dib le o n op eni ng an d clo si ng. It is hel pfu l to foc us o n a sp eci fic lan dma rk (su ch a s the mes ial in cis al edge of a

man dibu la r cen tral inc is or) wh ils t ask ing the p atien t to o pen a nd clo se th ei r mou th β€”in th is

P. 195

way an y latera l de viatio n wil l be no ted. The amo u nt o f ma nd ibu lar o pen in g s ho u ld be

re co rded. A n i nter -inc is al o pen in g o f a ppro xima te ly 35β€“45 mm is with in th e no rmal ran ge.

Lo ud joi nt s ou nd s ma y be hea rd du rin g ma nd ibu lar fun ctio n . In orde r to ex amin e th e jo int b y palp atio n th e exa min er s ho ul d be in fron t o f the patie nt so th at mo vemen t of th e m an dibl e

may b e rela te d to th o se p alp ated i n th e co nd ylar h ea ds. A sin gl e fin ger i s p lac ed o ver e ach co ndyl ar h ead wh ile the man dib ula r mo veme nts a re c arried ou t. A bn orm al ten dern es s

ass oc ia ted wi th the latera l as pec t of th e jo in ts is detec te d by l igh t pres su re o ver the co nd yle

and th e i mmedi ate p re -auri cu lar re gio n. E xami na ti on o f the po sterio r joi nt c an be un dertake n by in tra -auri cu lar (i ntra -meatal ) pal patio n β€” th e li ttl e fin gers are po sitio ne d in the extern al aud itory meatu s fo ll ow ed b y the g en tl e ap plic atio n of forw ard press ure . F ain t joi nt s ou nd s may b e h eard b y u sin g a stetho s co pe pl ac ed ove r th e c on dyl e h ead wh ile man dib ula r move men ts are perfo rmed.

Th e c lin ic ian s ho ul d rec ord th e a mo un t of man dib ular o pen ing . A n in ter -inc is al o pen in g o f appro xim ately 35 β€“ 45 mm is with in th e n o rm al ran ge.

Mus cu la r te nd ernes s ass oc ia ted wi th jo in t dis tu rba nc e ma y be d etected b y pa lpatio n o f the

mas seter a nd te mpo rali s mu sc les . The reade r mus t be c og ni za nt o f the ori gin an d in s erti on s o f th e ma stic atory mus cl es. Cli ni cal exam in atio n o f the ma sse te rs is ca rried ou t by a skin g th e patien t to c len ch the te eth f irmly to geth er an d p alpa ting a m us cle man u ally . Wh en the mas seters are c on trac te d, th e exa min in g fi nge r is run u p the an te ri or mus cl e bo rder

intrao ral ly, co un terpres su re be ing exerted from th e e xte rna l su rfac e. Wh en the exam ini ng

fin ger rea ch es th e z ygo matic o ri gin o f the mas seter, te nde rnes s b eco mes evi den t a nd is sh ow n by the patien t's re actio n . A s imil ar te st sh o uld be c arrie d o ut o n th e o ppo si te si de. The

tem po ra l o rigi n o f th e te mpo rali s c an be a ss ess ed extra ora lly w h en th e pa tien t is c len ch in g, but th e in sertio n of th e ten do n i s fe lt in tra ora lly b y run n ing the little fi nge r up the an te rior

bord er of the m an dibu la r as cen din g ra mu s. It i s n ot p ra ctic al to direc tl y pa lpa te th e m edia l and la tera l p teryg oi d mu sc les . Th e l ateral pterygo id mus cl es c an , ho we ver, be a sse ss ed by

appl yin g la te ral p ress ure to the man dib le an d as king th e pa ti ent to resi st the forc e a ppli ed. Spa sm o f th is mus cl e wi ll elic it pa in or ten dern es s in th e pre -auri cu lar re gio n.

Mus cu la r te nd ernes s ass oc ia ted wi th jo in t d istu rbance may b e de tec ted by pal patio n of th e mas seter a nd te mpo rali s mu sc les .

Dentition Fo llo wi ng th e exa min atio n of th e TM J an d ass o cia te d mu sc les , a ca refu l de ntal exam in atio n

sh ou ld b e u nde rta ken . Th e cli ni cia n s ho u ld i den ti fy an d trea t oro den tal p atho lo gy th at ma y be a so u rce o f pai n. The oc cl us al re latio n sh ip o f th e teeth, bo th static an d dyn am ic, is reco rded . Centric o ccl us io n (CO ) an d c entric rela tion (CR) s ho ul d be iden tifi ed an d the ir rela tion sh ip

no te d. In the majo rity o f pa ti ents thes e po si ti on s are no t co in cid ent. Th ere s ho ul d be a s mall (1 m m) an terio r sli de fro m CR to CO. La rge s lid es sho u ld b e n oted, pa rti cu larl y if th e s lide is

in a la te ral direc ti on . Oc clu sa l in terferen ce s s ho uld be ide ntifi ed du rin g ma nd ibu lar excu rsi on s and the prese nc e o f fac etin g, frac tu red re stora tions , a nd to o th w ear no ted. So ft-ti ssu e

evide nc e su gge stive of b ruxi sm i s rid gin g o f the bucc al muc o sa an d la te ra l b orde rs o f the to ng ue. S tu dy cas ts s ho ul d be ta ken as a bas eli ne re co rd.

Features suggest ive o f bruxism

To o th -wea r fac ets th at matc h i n m an dibu la r bo rd er (para fun ctio n al) m ove men ts

Cre na te d (pie -crus t) latera l bo rder o f th e ton gu e

Ri dgin g o f th e buc ca l mu co s a

Mas seteri c h ypertro phy

Hi sto ry of repea te d fra cture of resto ratio ns

Imaging

Th e c o mplex an atom y of the TM J h as led to the develop men t o f m an y radi og ra ph ic view s. The

main va lue of pla in film radi og ra ph y is to id entif y gro ss an atom ica l o r fu nc ti on al ch an ges th at wo uld in dic ate a n un derl ying org an ic cau se . E arl y bo ne path olo gy is n o t us ua lly d etected an d eros io ns mu st be qui te adva nc ed to be seen o n pla in films . A s ta nd ard p an ora mic radi ogra ph

may b e h elpfu l to exc lu de de ntal dis eas e bu t this is n ot a goo d v iew for sh ow in g the articu la r

su rf ace s o f th e TMJ. An β€ ope n m ou th β€™ den tal pan tomo gra ph wil l s ho w th e c ondyla r ne cks

and la tera l vi ew o f th e c on dyla r he ads (Fig. 16.1 ). Th e rel atio n o f th e co n dyle hea ds to the

disc an d fo ss ae a re n ot, ho w ever, wel l di spl ayed . Tra ns ph aryn geal view s a ls o g ive th e bo ny

ou tl in e o f the co nd yle a nd th e l ateral as pect o f the artic ul ar s urfa ce. Tran scra ni al view s, wi th

th e mo u th o pen an d c lo sed , d emo ns tra te co nd ylar an atomy an d the ran ge o f mo vem ent in th e jo in t, i nc lu din g the siz e o f th e jo int s pac e (Fig. 16.2).

Fig. 16 .1 Pa no rami c vi ew w ith mou th ope n sh ow in g c on dyle heads an d n eck.

Fig. 16 .2 Tran scra ni al view s o f te mpo roma nd ibu lar jo in t wi th mo u th o pen , tee th in oc clu si on , an d at rest.

P. 196

Comp uteriz ed to mo grap hy (CT) is u se ful fo r ima gin g the ha rd -ti ssu e d etail . M agn etic

re so na nc e ima gin g (MRI) vis u aliz es bo th s oft-ti ssu e d etail an d bo ny ou tlin e ( Fig. 16.3) as wel l as b ein g u sef ul in determin in g th e po si ti on , fun ctio n , a nd fo rm o f the disc w hen the mo uth is ope n a nd clo se d. Thi s is requ ired i n th e pre ope rative a ss ess men t prio r to d isc su rgery.

Arthro grap hy i nvo lve s th e in jectio n of a ra dio -opa qu e co n tra st ag ent in to the joi nt sp ac e

(u su al ly th e lo we r on e) to deli ne ate artic ul ar s urfa ces an d th e d isc . Th is tech n iqu e rema in s

th e o nl y truly dyn amic s tu dy o f th e joi nt a nd th e mos t sen sitiv e exa min atio n f or i den ti fyin g disc perf ora ti on s. Arth rog ra ph y is ind ica te d in c hro ni c temp oro man di bul ar pa in dys fun ctio n

syn drom e tha t ha s n ot respo n ded to in itia l treatme nt mo dal ities an d a lso fo r pers is te nt lo cki ng and limi te d mo uth o pen in g o f un kn ow n aetio lo gy. The te ch ni que is no t, h o weve r, alw ays we ll to lera te d by p atien ts a nd carri es a rela tively hi gh ra dia ti on do se . It h as no w larg ely b een re pla ced by MRI.

Fig. 16 .3 Sa gital magn etic reso n an ce sc an sh ow in g de nto -alveo la r co mpl ex, in clu din g

th e temp oro man dib ul ar join t.

An β€ ope n m ou th β€™ den tal pan tomo gra ph wil l s ho w th e c ondyla r ne cks an d la teral

view of th e c on dyl ar hea ds , b ut the rela ti on o f the co ndy le h ead s to th e d isc an d fo s sae are n ot wel l di spl ayed.

Tra ns ph aryn gea l vie ws give th e bo ny o u tl ine of th e c o ndyl e an d th e la teral asp ect o f the

Tra ns cra ni al vi ews , with the mou th op en a nd cl os ed, dem on strate c on dyl ar an ato my a nd

articu la r su rfac e.

th e ra nge of mov emen t in th e join t, inc lu din g th e si ze of th e jo in t spa ce.

Arthroscopy

Min i -arthro sc opy may be a n i nves tiga ti ve or th erapeutic pro ced ure. Th is min ima lly i nva sive tec hn iq ue all ow s fo r the direc t exam ina tion o f the up per jo in t s pa ce. Low er jo in t s pac e

arthro sc opy is no t us ua lly perfo rmed due to th e sm all siz e o f th e sp ac e betw een th e d isc an d th e c on dyla r h ead in co mpari so n to the siz e o f th e in stru men ts u sed for min i -arthro sc opy .

Lys is of th e u ppe r join t sp ace , l ava ge, ca psu la r dis te ns ion , remo val of i ntraa rticu la r adh esi on s and lo os e bo di es, di sc relea se, an d th e pla ceme nt of co rtic os te ro id prepa ratio ns ma y be perfo rmed w ith arthro sc op ica l su rgery. On rare oc cas io ns mo re exten si ve in traa rtic u lar

su rg ery ma y be un dertake n, su ch a s bi op sy a nd dis c rep os itio nin g. TMJ arthro cen tesi s ma ybe carri ed o ut a s a n i so lated pro cedu re, to p rovi de a rti cu lar l ysis an d l ava ge, bu t with ou t the nec ess ity o f u sin g e xpen sive arthro sc o pic al equ ipment.

Temporomandibular pain dysfunction syndrome (TMPDS)

Th ere are m an y syn on yms u sed to des cribe this co n ditio n β€” myof aci al pain dys fu nc ti on , fac ial arthro myal gia , fac ial pai n d ysfu n ctio n, ma stica tory m us cle dis orde r. Th e term

tem po ro ma nd ibu lar pai n d ysfu nc tion s ynd rome (TMP DS ) wi ll b e u sed in th is text. Th is

co ndi ti on is th e m os t preval ent d iso rder o f th e TMJ an d aff ects predo min an tly fe male patie nts. Epi demi olo gi cal da ta su gges ts th at 40β€“80 pe r cen t of patie nts w ith dis orde rs o f

th e TM J are f emal e. In TMP DS th e pred omi na nt c omp lai nt is o f pa in, wh ich ma y take an y of

P. 197

th e fo rms previo u sly des cribe d. This pa in may be ass o cia te d wi th l imitatio n of op eni ng or w ith jo in t so un ds , a ls o a s p revio us ly ou tlin ed. Th e pa tients qu ite o fte n a dmit to a h is to ry o f psyc ho lo gic al stress , alth ou gh ov ert psyc hi atric abn orm ali ty is u n us ua l.

Th ere may be a hi story of previo u s jo in t c lic kin g, lim itatio n o f o pen in g, trau ma, o r recu rren t

disl oc atio n. Q ues tion in g ma y el ici t th e pre sen ce of a h abi t th at alters man di bul ar po si ti on in g or a ctio n. S ome patie nts may h o ld th e ma ndi ble in a pa rti cu la r pos itio n, u su al ly a protru sio n

or l ateral pos itio n, w he n en ga ged in so me m ental acti vity. There may be a hi story of bitin g o n so me fo reig n b ody , su ch as a p en or p enc il. Th e pa ti en t m ay be aw are o f g rind in g thei r teeth, but a hi story o f b ruxi sm i s o ften su ppli ed b y so me o the r memb er o f the patien t's fami ly o r a

partne r. E xam ina tion ma y revea l o n e o r seve ral o f th e fo llo wi ng : limi ta tion o f o pen in g o f the mou th; d evia ti on o f the man dib le o n ope ni ng; cli ckin g h ea rd o r felt i n th e joi nt; gro ss

malo cc lu si on lea din g to abn o rmal join t mo vemen ts, or min or degree s o f ma lo cc lus io n with abn orm al c us pal gu ida nc e of clo su re; gro ss o cc lus al attrition ; o cc lu sal in te rferen ces ; un sa ti sfa ctory den tu res ; te nde rnes s o f th e mu sc les o f mas tica ti on .

Signs and sympto ms asso ciated with T MPDS

Pre -auri cu lar pain th at may radia te to o the r sites

Te nd ernes s of th e jo in t o n pal patio n

Li mited ja w mo vem ent o r dev iatio n of man dib le o n ope ni ng an d cl os in g

Te nd ernes s of mas ti ca to ry mu sc les on pa lpa tion

Jo in t so u nds (cli cks , c repitu s)

He ada ch e

Clin ica lly, th ere is te nde rnes s o r pa in of th e TM J an d m us cles o f ma stic atio nβ€”th is may be bi or un ila teral. Limi ta tion o f ma nd ibu lar o pe nin g a nd joi nt so u nd s a re of te n p re sen t. In mo st

patien ts w ith ch ro n ic symp to ms , ra dio grap hs of th e jo in ts reve al n o abn o rm ali ty of struc tu re, alth ou gh limi tatio n o r in cre ase in joi nt mo veme nt ma y be s een .

Th e a etio lo gy o f TMP DS is u nc lea r. M an y theo rie s h ave been po stu lated , i nc lu din g sk eletal jaw re latio n sh ips , o cc lu sa l di sh armo ni es, lac k o f po sterio r teeth , a nd un il ateral to o th lo ss , but th ere i s n o cle ar evide nc e to su ppo rt th es e. In terestin gly , rel ativel y few pa ti ents with

co mple te den tu res app ear to pres ent w ith TMPDS . P ara fun ctio n al c len ch in g a nd grin din g a nd abn orm al p os tu rin g o f the jaw ha ve be en impl ica te d as in itia ti ng or p erpetua ting fac to rs in

so me pa tien ts β€”it is hypo the siz ed th at rep eti ti ou s adve rs e l oa din g cau s es m icro trau ma o f th e mas ti cato ry sys tem. Ps ych os o cia l fa ctors su ch a s anxiety a nd depres si on ma y predi spo se certai n pa tien ts to tem po roma nd ibu lar dis orde rs a nd may a lso s erve to perpe tu ate th e

sympto ms. In so me p atien ts the re is a h isto ry o f trau ma. Bo th yawn in g a nd den ta l trea tm ent

hav e al so been impl ica ted. Care m us t be take n i n d iffere ntia ti ng th e pa in du e to

tem po ro ma nd ibu lar d istu rban ce s fro m th at aris in g fro m de ntal ca us es o r fro m fac ial pai n o f th e types des cri bed i n Cha pte rs 15 an d 17. In partic ul ar, th e d iffere ntia l di agn os is betwe en

faci al pai n o f ps ych o gen ic orig in an d tha t cau s ed by ch ron ic te mpo roma nd ibu lar dysf un ctio n in patien ts u nd ergo in g emo tio na l s tres s i s d iffic ul t. In fac t, th e tw o co nd ition s oc ca sio n all y see m to merg e in a patien t wi th ph ysi ca l si gn s o f TMJ dys fu nc ti on , but w ith th e d emo ns tra ti ve,

anx iou s , o bs ess ive o u tl oo k typic al of th e p atien t co nve rti ng h id den an xietie s in to fac ial pai n sympto ms. S om e patie nts w ith TMP DS ha ve a hi sto ry of gen eral pai n d iso rders . Ne edles s to

say, in su ch ca ses , th e i nitia l p re su mptive dia gno si s s ho u ld be of joi nt dys fu nc ti on . On ly w hen all phy sic al sign s of TMPDS ha ve be en addre ss ed by trea tmen t, s ho ul d th e d iagn o sis o f

psyc ho gen ic pai n b e mo re fi rmly e ntertain ed. It is in ob servi ng th e rea ctio n to trea tme nt tha t th e di fferen tial dia gn os is is p erha ps best m ade. Irres pec tive o f th e aeti olo gy of c hro n ic tem po ro ma nd ibu lar p ain , th e psyc ho lo gic al resp on se to pa in ca nn ot b e ign o red i n th e man age men t o f th is co nd ition β€”Cha pte r 17 explo res this fu rth er.

Dis c disp lac emen t h as been imp lic ated a s b ein g im porta nt in the aetio lo gy o f TM PDS.

Ho w ever, abn o rmal ities in th e l oc atio n o f th e di sc ha ve be en no ted in ma ny patie nts w ith an d with ou t TMP DS .

Management

A fu ll dis cu ssi on o f the man ag emen t of te mpo roma nd ibu lar dis orde rs is o utsi de th e sc op e o f th is bo ok a nd this is o nly a brief ove rv iew . In view o f the fac t th at the aetio lo gy i s p oo rly un ders to o d, i t is no t su rpris ing that th e ma na geme nt o f thi s c on ditio n is co nten tiou s. Th e ra ng e o f ma na gemen t o pti on s us ed fo r TMP DS i s li sted in Ta ble 16. 1. M an y patie nts with

TM PDS are s uc ce ssf ull y ma na ged i n p ractic e by off erin g an exp lan atio n fo r th e s ympto ms a nd

re ass u ra nc e, toge th er w ith oc clu sa l app lian c es i f in dic ated. Ini ti al man age men t s h ou ld a lw ays be c on serva tive, an d prefera bly i t sh ou ld be n on -inva si ve an d re versi ble. Th ese requ iremen ts are impo rtan t for th e ma na geme nt o f TMJ d iso rders bec au se a s uc ces sfu l o u tc om e can n ot be

ens ure d. Th e va lu e o f rea ss uran ce an d c ou ns ell in g ca nn o t be un deres tima ted . P atie nts s h ou ld be tol d a bou t the na tu re a nd prog no si s o f thi s co ndition . An in fo rmatio n lea flet is h elpfu l to re in forc e thi s. A min ority o f patie nts w ill be wo rried th at the y ha ve a seri ou s, eve n l ife th reaten in g, co nd ition a nd inf orma tion c an all ay s uc h fears . Th ere may a ls o b e a s tron g plac ebo res po ns e to th e di fferen t tre atmen t mo dali ties advo ca te d fo r TMP DS .

Th e s uc ce ssf ul man age men t of TM PDS ca nn ot be gu aranteed. Th erefo re the in itial tre atmen t of TM PDS sh ou ld be:

no n -inva si ve

re versi ble

Man y pa ti en ts resp on d to reas su ran ce (Β ± a remo va ble o cc lu sa l ap pli anc e)

Table 16.1 Spectrum of management strategies for temporomandibular disorders Initial ( con servative) m anagemen t Re ass u ra nc e

Fur ther (specialist) m anagemen t Psyc h olo gi cal in te rven ti on

Edu ca ti on

Occ lu sal adju stmen t

Ha bit m ana gem ent (e. g. jaw

Occ lu sal reh abi litatio n (e.g . re stora ti ve,

exerci ses , awa ren ess of daytim e jaw clen ch in g)

ortho do nti c, orth ogn ath ic su rgery)

Mo dific atio n of fun ctio n (e. g. ch ewi ng,

An ti depres sa nts

Re st

In tra artic ul ar stero ids

An ti -inf lamm atory agen ts a nd

Man ipu la ti on un der g ene ral a na esth esi a

Mus cl e relax an ts (e. g. dia zep am)

Su rgery (e. g. arthro ce ntes is,

yawn in g, si ngi ng )

ana lge sic s (l oc al an d sys temic )

Psyc h othe rapy

arthro sc opy , o r o pen artic ul ar su rgery at arthro to my)

Occ lu sal sp lin ts (remo vabl e)

Psyc h iatric lia iso n cl ini c/pa in cli ni c

Ph ysio the ra py

Th e u se of oc cl us al a ppl ian ce s, or s pli nts as th ey a re c omm on ly c all ed, is frequ ently he lpfu l

P. 198

for s om e pa ti ents , a nd stu dies ha ve d emo ns tra te d tha t th ere i s a si gni fic an t plac ebo eff ect.

Occ lu sal app lia nc es sh ou ld b e remo va ble an d gi ve full o c clu sa l c overa ge. Th ere is n o p lac e fo r th e ro utin e u se of spl ints tha t o n ly gi ve pa rti al oc clu sa l c ove ra ge β€”su ch spl in ts may ca us e un wa nted to oth mo veme nt. Ta ble 16. 2 hi ghl igh ts s ome impo rtan t poi nts co nc erni ng th e

provi sio n of oc cl us al a ppl ian ces , wh ils t Ta ble 16. 3 gives deta ils of th e th ree s pli nts m os t co mmo nl y us ed for TMJ di so rders.

Ja w exerci ses an d va rio us fo rms o f phy sio thera py may al l pl ay a part i n trea tm ent. Ex ercis es to co rrec t fau lty patterns o f ac tivity β€”su ch as devi atio n o n op eni ng an d cl os ure β€”may b e hel pfu l. Thera py w ith a na lge sic o r relax an t d rugs ma y ha ve a lim ited u se, bu t on ly o ver a

re stric ted peri od of tim e. Non stero ida l an ti -inf lamm atory drug s (NS AIDs) a re the an alg esi cs of ch oic e, pro vided tha t th ere a re n o co ntrai ndi ca ti ons to the ir us e. The us e o f be nz od iaz epi ne s for th eir m us cle relax an t p rope rti es m ay b e he lpfu l i n a cu te ca ses w here th ere i s tris mu s. In addi ti on , th eir hypn o ti c a ctio n can be ben efic ia l in en su rin g sl eep. It is impe rative th at o nly

sh ort co u rses of ben zo di az epin es are u se d, idea lly 2 w eeks or l ess , beca us e o f the po te ntia l ri sk o f d epen den ce . B en zo dia ze pin es are, th erefo re, o nl y of valu e d urin g th e a cu te pha se of TM PDS .

Table 16.2 Points to remember when providing removable occlusal appliances in the management of temporomandibular disorders

Prio r to c o ns tru ctio n of oc clu sa l spl ints :

Un dertake a p re tre atmen t an alys is of th e s toma togn ath ic system Ma ke c omp rehe ns ive written reco rds

Arc hi ve s tu dy m ode ls with an oc clu sa l rec o rd prio r to spl int th erapy

All oc cl us al app lian c es:

h ave a pla ce bo effec t

al te r oc clu s al co ntac ts

dec reas e o cc lu sal fo rces tran smitted to teeth

Do no t us e pa rti al c o verage spl in ts (ris k of un co n tro lle d too th m ove men t)

Re in forc e the ne ed fo r go o d o ra l a nd app lian c e hy gien e

Th ere is n o evid enc e th at perma ne nt o cc lu sal reh abil itatio n is o f va lu e for TMJ di so rders an d

su ch tre atmen t sh ou ld on ly b e un de rta ken with sp ecia lis t advi ce. In a s mall nu mbe r of ca ses ortho do nti c treatme nt o r a c om bin ed a ppro ac h us in g o rth og na th ic su rgery i s i ndi ca te d. Mo st maxil lo fac ial su rgeo ns w ou ld o n ly perfo rm o rth o gna thic s urge ry for a pa ti en t w ith a

tem po ro ma nd ibu lar d iso rder i f, in add ition to th e arti cu lar sig ns an d s ymptom s, the patien t was h avin g p ro bl ems from th e m alo cc lu sio n an d u nd erlyin g s kele ta l rel atio ns hi ps d ue to ina dequ ate fun ctio n or for a esth etic reas on s. In a few c ase s o f c hro ni c TM J dis orde rs,

anti depres sa nt dru gs , w ith or w itho u t p syc ho thera py, may hel p. TMJ s urge ry sh ou ld be

re serve d fo r patie nts with a c lea rly id enti fiab le jo in t a bn orm ality, su ch as a d isp lac ed o r

dama ged dis c. The re are othe r prereq uis ites for su rgery. As a g ene ra l ru le th e c on ditio n te nd s to be sel f-limi ti ng an d is no t tho ug ht to prog ress to a deg ene rative jo in t co nd itio n i n th e

majo rity of ca ses . TM J su rgery sh ou ld no t be c arried o ut u ntil th e fo ll ow in g c ri te ria h av e bee n met. 1. 2. 3.

A me ch an ica l in traartic ul ar join t pro blem sh ou ld ha ve be en prove n b y the us e o f

spec ia liz ed i magi ng , s uc h as MRI.

All exh au stive n o ns urgi ca l reve rs ibl e tre atmen t meas ure s h ave fail ed to co ntro l th e

prese ntin g s ign s a nd symp to ms .

Th e p atien ts' s ympto ms d eleteri ou sly affe ct thei r day to da y ac tivity to th e e xte nt tha t

th ey h ave a po o r qu ali ty of life .

Chro ni c TM PDS th at is resi stan t to c on se rvative manage men t may re qui re the mo dali ti es us ed to tre at ch ron ic pa in of psyc ho ge nic o rigin .

Internal derangement In terna l de ran gemen t is a c om mon dis orde r an d is due to th e p erman en t

disp lac emen t o f the articu la r dis c, wh ic h ha s a n a bn orma l rel atio ns hi p to both the glen oi d fo ss a a nd artic ula r emin en ce.

In terna l de ran gemen t is a c om mon di so rder a nd is due to a n a bn orma l rel atio ns hi p o f the dis c to the co nd yle, gle no id fos sa , an d artic ul ar em ine nc e. Thi s c on ditio n is diffe rent fro m TM PDS

beca us e th e articu la r dis c i s pe rman en tl y, n o t interm itte ntly d isp lac ed, as in TMP DS . The cli ck in tru e a rti cu lar d eran geme nt is co n ti nu ou s β€”no t interm itte nt. Pa in is no t alw ays a featu re, espe cia lly

in th e ea rly s ta ges o f this co nd ition . Disc dis pla cem ent ma y be divi ded i nto two

P. 199

prese ntatio n s β€”eac h h as a diffe rent trea tm ent mo dality.

Table 16.3 Characteristics of commonly used occlusal appliances in disorders of the TMJ So ft vac uu m -forme d sp lin ts Usef ul mai nly for m us cu lar (myof aci al) s ign s an d symp to ms

So ft ful l c overa ge s pli nts, be tter to lera te d in the lo wer a rch Qui ck to mak e β€”impres si on requ ired of on ly o n e arch

Do n ot c on fo rm to a sp eci fic oc clu sa l pre sc ri ptio n, bu t ca n be ma de i n d iffe re nt th ic knes se s

No t ame na ble to o cc lus al adju stmen t Wo rn at ni gh t ti me

Go o d fo r ac ute TM PDS; can ma ke bruxi sm wo rse i n s o me pa ti en ts La st ap proxi matel y 6 mon ths Stabi liz atio n spl int

Ha rd a cryli c, fu ll c ove rage spl int

Te ch ni cal ly dema nd ing an d time -co ns umi ng to ma ke. Requ ires imp re ss io ns of both arch es an d a reco rd o f c entric rela ti on . A f ace bo w i s requ ire d in diffi cu lt o ccl us al cas es.

Maxi lla ry or man dibu la r app lia nc e bu t maxi lla ry is often ea sie r to a djus t Wo rn at ni gh t ti me, for lo ng -term us e

Des ign ed to pro vide an id eal oc clu si on at res t an d in fun ctio n (i. e. cen tric

oc clu si on β€”maxim um sim ulta neo u s o cc lu sal co ntac ts β€” idea lly th is sh ou ld equ ate to cen tri c rel atio n. Th ere sh ou ld be in c isa l an d c an ine gu ida nc e w ith n o po sterio r interfe renc es)

An teri or re pos itio ni ng spl int Man dib ul ar o r maxi lla ry ha rd acry lic , fu ll oc cl us al c ov erage

Re qui re im press io ns o f bo th arch es an d a n o cc lu sa l rec ord with the man dibl e protru ded

In dic ated for d isc dis pla cem ent w ith redu ctio n (i .e. cli ck d isa ppea rs w he n p atien t is aske d to ope n a nd clo s e fro m a protru siv e man di bul ar po s ition )

Fo r fun ctio n al us e, as muc h as po ssi ble; idea lly w orn 24 h/d ay fo r ab ou t 12 w eeks Avo id i n ado les ce nts to preve nt it a ctin g as a fun ctio n al o rtho do ntic app lian c e Objec ti ve is to a llo w th e d isc to repo sitio n

Disc displacement with reduct io n

Re prod uc ibl e reci pro cal cl icki ng

Dis c dis pla ceme nt s ho wn by i mag ing an d a bsen c e o f dege ne ra tive b on e dis eas e

In ad ditio n, the re ma y be p ain , devia tion o f jaw move men ts , no li mitatio n of ope nin g.

Disc displacement without reductio n

Pers isten t lim itatio n o f mo u th o pen in g ( β‰¤ 35 mm) with hi story o f s ud den o ns et

Dis c disp lac emen t sh o wn by im agi ng a nd abs en ce of degen era ti ve bo ne dis eas e

In ad ditio n th ere m ay be pai n a nd cl icki ng.

Disc displacement with reduction

In thi s c on ditio n th e d isc is dis pla ced du ri ng op eni ng an d cl os in g. Du rin g fu n ctio n th e

mala lig ned dis c β€ re duc es β€™ o r impro ves its struc tu ral rela ti on sh ip with the c o ndyl ar hea d.

In dis c disp lac emen t wi th red uc ti on the re is a rec ipro ca l c lic k, no te d duri ng op eni ng an d

clo si ng, tha t is no t alw ays pa inf ul , a nd jaw devia ti on o n op eni ng an d cl os in g is co mmo n . Th e re cip roc al c lic k is u su all y eli min ated w h en th e p atien t op ens an d c lo ses fro m a protru ded man dibu la r po sitio n , o ften wi th th e i nc iso rs in an edge to e dge p os itio n c on firm ing the

prese nc e o f an terio r dis c di spl ac emen t with redu ctio n . Th is co nd itio n d oes n ot al wa ys m erit trea tmen t, es pec ial ly if th e o n ly pres en ti ng prob lem is th at o f a cli ckin g join t. The

man age men t o f th is co ndi ti on ma y ran ge from co un se llin g to the prov isi on o f a s tabil iz atio n

spli nt a nd phys io thera py. Mu sc le rel axa nts are oc casio n ally us ed a nd , i n a min o rity of ca ses , su rg ery ma y be co ns idere d. Th e co nd ition ma y de teriora te wi th the dis c be co min g

prog re ss ively mo re dis pla ced an d po ss ibl y in te rferin g w ith o pen in g o f th e ma ndi ble, le adi ng to disc dis pla ceme nt w itho ut red uc ti on an d a cl os ed l oc k si tu atio n .

Disc displacement without reduction

In thi s c on ditio n th e d isc is dis pla ced du ri ng op eni ng an d cl os in g. The mal alig ne d dis c d oe s n ot impro ve i ts stru ctura l

re latio n sh ip w ith th e c on dyla r hea d du rin g fu n ctio nβ€”th e di sc is perma nen tly di spl ac ed,

P. 200

us ua lly i n a n a nteri or or a ntero med ial direc tion . The re is a h is to ry o f the jaw lo ckin g, bec au se

th e mis ali gne d di sc mech an ic all y o bstruc ts th e co n dyle duri ng ja w o pe nin g. Th ere is us ua lly n o evide nc e o f an y artic ul ar c lic kin g o f a re cip roc al n atu re, alth ou gh a previo u s h is to ry o f su ch cli ckin g β€”fol low ed by lo cki ng β€”may b e giv en. Th e di sc dis pla ceme nt ma y, on o cca si on , be re duc ed from a pro trude d ma ndi bu lar p os itio n, alth ou gh this is n ot al wa ys th e ca se. Th e

impl ica ti on is tha t th e pro trud ed man dib le h as pla ced the c o ndyl e in a po sitio n wh ere i t i s i n a

no rmal fun cti on al relati on sh ip w ith th e d isc β€”as opp os ed to co mpres si ng th e p os te ri or p art o f th e dis c (bi lam ina r z on e). In o ther c as es, the cl osed l oc k is no t redu ci ble w ith an te rior

man dibu la r repo si ti on in g (pro trus ion ) a nd, c on seq uently, the dis c dis pla ceme nt is perm an ent. Cou ns ell ing , p hys io th era py, an d mu sc le rel axa nts can be o f va lu e in thes e c as es a nd an

anteri or re pos itio ni ng spl int m ay a lso be in dica ted. Su rgery m ay b e su itab le fo r a min ori ty of patien ts.

Dis c disp lac emen t ca n so metimes be redu ced from a protru ded man dibu la r pos itio n.

Rheumatoid arthritis

Rh eu mato id a rthritis is a c om mo n mul ti sys te m, au to imm un e, in fla mmato ry dis eas e. It i s n o w acc epted th at the TMJ is invo lve d to so me ex ten t in a large pro portio n of patie nts w ith gen erali zed rheu ma to id dis eas e. It i s, ho we ver, un us ua l fo r the patie nt to pres ent,

un dia gno se d, with prim ary s ymptom s fo r the TMJ. When p atien ts w ith rh eu mato id arthri ti s do

seek trea tmen t for TM J pro blem s th e ma jo r c omp lai nt is o f limi ta tion o f o pen in g a nd c repi tu s, stiffn es s, an d pa in fo llo wed by a ch in g. Sig ni fica nt p ain as a s ympto m is rare . Crep itus is the mos t co mmo n cli nic al sig n on exa min atio n β€”jo in t tend erne ss an d fu nc ti on al abn o rmali ti es

may a lso be see n. Symp to ms are us ua lly b ila tera l. Radi og ra ph ic evide nc e o f ch an ges h as been fou nd in a h igh pro po rti on o f the se p atien ts, man ife stin g as ero si on s, pro lif eratio ns , an d

flatten ing of the c o ndyl ar h ead . A s i n othe r joi nts, the dis eas e pro ce ss may oc cu r in a p ha sic

man ne r, a cu te exac erba ti on s b ein g fo ll ow ed by eith er a h ea lin g pha se or a se co nd ary c hro ni c pha se. A nkyl os is of th e TM J ca n o cc ur. Rare ly, it is pos si ble f or th e pa ti ent to deve lo p an

anteri or o pen bi te defo rmity c au se d by des tru ctio n of th e c on dyl es a nd th e l os s o f c on dyl ar and po sterio r an d o c clu sa l fac e h eigh t.

Imm un ol og ica l tes ts fo r rhe uma to id arthri ti s w ere ou tlin ed i n Cha pte r 2. A rai sed erythro cyte

sedi men ta tion rate (E SR) a nd h ype rgamm agl obu li na emia are u s ua lly p re sen t, to geth er wi th a n eleva te d titre o f rh eum atoi d fa ctor a nd an tinu cl ear a nd oth er an tibo die s. The se te sts m ay

prove po sitive befo re s ystemi c c ha ng es h ave been n otic ed. It is als o wo rth rem emberi ng th at

a si gn ific an t prop ortio n of patien ts w ith rheu ma to id arthri ti s h ave Sj Γ¶gre n's syn dro me. Th ese ind ividu al s m ay de velo p s ali vary gl an d path ol ogy that pre sen ts a s fa cia l pa in . SjΓ¶gre n's syn drom e is des crib ed i n fu ll in Cha pte r 8.

No n stero idal an ti -inf lamm atory an alge sic s a re the mai ns ta y o f treatmen t fo r rhe uma toid arthritis . How ever, mo re s evere c as es a re treated by di sea se -mod ifyin g d rugs su ch as

metho trexate o r az ath io prin e. Su rgery o f th e TMJ m ay b e in dic ated f or an kylo sis an d c as es o f severe co nd ylar d estru ctio n. In so me pa tien ts pro sth eti c join t repl ace men t or a uto gen ou s

(c o stoc ho n dral) graftin g ma y be requ ired to dec re ase pa in, im prov e ap peara nc e, or re store fun ctio n .

Osteoarthrosis (osteoarthritis)

Cha nge s ma y take pla ce i n th e TMJ as part o f a gen erali zed , d egen era ti ve arth ritic c o ndi ti on . Osteo arth ro s is i s a metab ol ic d efec t of a rticu la r cartil age an d is u su all y asy mptom atic in th e

TM Js a nd is see n a s a ch an ce fin din g o n radio gra phs. Radio gra ph ic c ha ng es m ay n o t a lw ays be evide nt bu t, w he n p resen t, th ey a re varia ble an d incl ude a re duc tion in jo in t s pa ce a nd eros io ns of the a rticu la ti ng su rfac es o f th e co n dyle an d o f the fos sa . O steo ph yte s ma y oc cas io na lly be s een at the an te ri or edge of th e c on dyle .

Mo st patie nts with os te oa rth ro sis are fema le a nd over th e a ge o f 50 years . Pai n i s ra re an d th e hi story i s u su al ly o f jo in t s ou n ds a nd grad ua lly i nc reas in g stif fne ss. Th e jo in t may b e ten der to pres su re a nd crepi ta tio ns are o ften he ard a nd felt w he n th e join t is mo ved.

Mo vemen t may als o b e res tri cted. Whe n p ain is pres en t i t te nds to be lo ca liz ed to the p re-

auri cu lar re gio n β€”in co ntras t to the myo fac ial pain dis tri butio n of TMP DS . Th e rh eu mato id facto r is ne gative an d the ES R no rma l in th es e pa tients . S ympto matic an ti-inf lamm atory ana lge sic s a re u su all y effe ctive for os te oa rth ros is an d mu sc le re laxa nts are o cc as io na lly ind ica te d. Su rgery i s rarely app ro pri ate.

Masseteric hypertrophy

Uni latera l en la rg emen t of the m us cle s o f mas ti ca ti on , an d o f the mas seter i n p articu la r, oc cas io na lly oc cu rs a s a respo n se to se rio us dera nge men t o f th e o cc lus io n lead in g to

un ila te ral mas ti ca ti on . It ma y al so , h o weve r, o cc u r with little or n o oc clu sa l d ish armo n y an d, in fac t, ma y be b ila te ral ra th er th an un il ateral . In th e a ffec ted pa ti en ts th e c om pla int i s

us ua lly o n ly o f in crea si ng fac ial as ymmetry. On exam in atio n th e mas seter (o r ma ss eters) i s fou nd to b e en larg ed a s a wh ol e, of ten w ith a ma rked in crea se o verly ing the man dibu la r

ins ertio n. Whe n th e ma ss ete r is defi ned fo r exam ination by as king the patien t to c len ch the

tee th , th e m us cle is eas ily pal pated, the lo wer part o fte n s tan din g o ut a nd resem blin g a so ftti ssu e m ass . Pla in radi og raph y (po sterio r β€“anteri or m an dibl e) o r a de ntal pan o rami c vie w

may sh ow a m arked co nc avi ty at the i ns ertio n o f th e ma sse te r, a t th e p eriph ery o f wh ich th ere is l ippi ng of th e b on e. If elec tromy ogra ph ic f aci lities are ava ilab le, it is ma y be p os sib le to

demo ns tra te atypi ca l mu sc le a ctivi ty i n all th e m uscle s o f ma stic atio n. Ma gn etic re so na nc e

imag in g is us efu l in de fin in g no rmal an ato my an d h ype rtro ph y of th e m us cle an d in id en ti fyin g any oth er path ol ogy (Fig. 16.4).

Man ag emen t of mas seteric hyp ertro ph y w ith i njec ti on o f bo tul inu m to xin ha s b een repo rte d, but th ere is n o co nvi nc in g evi den ce o f i ts lo ng -term ben efit.

P. 201

Fig. 16 .4 Axia l m agn etic reso na nc e s can sh ow in g pterygo id and ma ss eter mus cl es.

Fig. 16 .5 An o steo ma o f th e ne ck o f th e co nd yle.

Tumours

Pse udo tum ou rs, su ch a s s yno via l ch on dro mato sis , an d true tu mo urs o f the TMJ are rare.

Osteo mas ( Fig. 16.5) an d ch o ndro ma s fo rm th e ma jority o f th ese an d are ben ign an d res trict man dibu la r op eni ng . Th ey m ay a lso res ul t i n o c clu sal prob lems wi th o cc as io nal ske letal an d ja w d efo rmities . Mal ign an t os te os arco ma s are e xcep ti on all y rare in th e TM J, a ltho u gh

mali gn ant tu mo urs of nea rby struc tures , s u ch as th e p aro ti d gla nd , m ay i nvo lve th e TM J area by se co nd ary in vasi on .


Wh at a re the like ly ca us es for th is patie nt's trismu s?

Tri smu s is th e red uc ed a bili ty to o pen the man dib le du e to refle x mus cu la r spa sm (c o ntrac ti on ) o f the mas ti ca to ry mu sc les . Th e

term is, ho we ver, often u sed mo re bro adl y to ref er to a n ina bil ity to o pen th e mo uth ful ly, fo r

P. 202

examp le, du e to an kylo sis o f the TMJ fo llo w ing tra um a o r fib ros is of th e m astic ato ry mu scl es fol low in g ex pos ure to ex tern al bea m the rapeu tic radi atio n.

Th e m os t likel y ca us e o f thi s 20 yea r ol d's re cen t hi story o f p ain an d tris mu s i s a den ta l

inf ectio n. A t her a ge th is may b e ass o cia te d wi th an i mpa cted ma nd ibu la r th ird mol ar du e to

peric oro ni ti s. Qu estio ni ng th e p atien t abo u t h er d enta l h isto ry wi ll b e h elpfu l. If the co nd ition

is p erico ro ni ti s, it is po ss ible th at th e pa ti en t is a wa re tha t s he ha s a n e ruptin g to oth an d ma y hav e expe rien ced sim ila r symp to ms in the pas t. A radi ogra ph is ess entia l to co n firm th e

pos itio n o f a pa rti all y erup te d too th. The patien t ma y ha ve h ad an in ferio r den tal n erve blo ck for re cen t tre atmen t an d th is oc ca sio n all y res ults in dam age to the mu scl es due to inf lamm atio n o r ha emato ma form atio n. Q2

Li st the pos si ble aetio lo gie s fo r ac ute an d ch ro nic li mitatio n of mou th op eni ng.

Th e m os t co mmo n c au se s o f trism us are i nfe ctio n (f or ex ampl e, peric oro n itis, s iala den itis ), acu te TMP DS, an d tra um a. The tra uma ma y be i atrog eni c (fo ll ow in g remo va l o f ma ndi bul ar

th ird mol ars or i nfe rio r den ta l n erve b lo cks ) or a res ult o f vi ole nc e o r an ac ci den t (fac ial an d

co ndyl ar fra ctu re s, dam age to th e mu sc les of mas ti ca ti on ). Th e cau s es o f tris mus are lis ted in Ta ble 16. 4.


Wh at c on ditio n s may b e res po ns ible fo r th is patient's pai n a nd wh at co u ld yo u do , i n th e first i ns ta nc e, to h elp yo u rea ch a defin itive dia gno si s?

Th is ede ntu lou s 70 -year-old patie nt ma y be s uf fering fro m TM PDS, des pite th e fa ct tha t it is less co mm on in eden tul ou s a nd elde rly pa ti en ts . A n ol der pa tien t w ith crep itus may be

su fferin g from os teoa rth ro sis . The p ain h isto ry is dif feren t for th ese tw o co nd itio ns . A pla in film of th e TM Js, su ch as an o pen jaw den tal p an to mo grap h m ay s ho w irregu la ri ties of th e co ndyl ar s urfa ce su ch as ero sio n s o r os teo phytes in os te oa rth ro sis .

Table 16.4 Causes of trismus Extra -ar ticular

In fec ti on /in fla mmatio n in rela te d

Ha ema to ma , i nfl amm atio n, o r in fectio n

Tra um a (po st -ope rative o ede ma, fac ial

TM PDS

struc tu res (e.g . p eric oro ni ti s, si ala den itis) fol low in g ma nd ibu lar tra uma ) Te ta ny

Intr a-ar ticular

In fec ti ve arth ritis Osteo arth ro s is

Rh eu mato id a rthritis An kylo sis o f TMJ

In tra -cap su lar co nd ylar

Dis lo ca ti on

fractu re bl oc k in jectio n s

Te ta nu s

Fibro si s d ue to bu rn s o r radi atio n

System ic scl eros is an d s ubm uc ou s fibro si s Ne opl as m

Table 16.5 Causes of pain in or adjacent to the temporomandibular joints

TM PDS

In terna l de ran gemen t (dis c dis pla ceme nt) Arthritis

Osteo arth ro s is

Odo nto gen ic in fectio n Tra um a β€”fractu res Atypic al fac ial pai n

Ia tro gen ic β €”inf lamm atio n f oll ow in g ma nd ibu lar b lo ck i njec ti on or re mova l o f

Sal ivary glan d d ise ase (e.g . m ump s, au toim mu ne s ia lad eni ti s, mal ign an ci es)

man dibu la r third mo lars

Gi an t cell arteritis (isc ha emi c pa in ) Mal ign an cie s

Ear prob lems

Th is pa ti ent h as two pro blem s, pai n a nd ov erclo su re w ith hi s den tu res β€”th ese may or m ay

no t be rel ated. In th e fi rst in stan ce , o n e o f the den tu res co ul d be fitted w ith a so ft o cc lus al

spli nt a nd th e p atien t review ed ove r a 6-week period to see if th e pa in ha s re duc ed. Th e sp lin t wil l in crea se th e o cc lu sa l vertic al dimen s ion a nd th is may be be nef ici al i n re duc in g the pai n. Th is is a re versib le p roc edu re an d a he lpfu l o n e prio r to co ns tru c ti ng new den tu res fo r th is

patien t. NS AIDs ma y be pres cribe d (pro vidi ng th ere are n o co ntrai ndi ca ti on s). If sy mptom s do no t reso lve after the co n struc ti on o f n ew den tu res , p hys io th erap y ma y be c on si dered . Th is patien t may nee d refe rra l to a spe cia lis t cli nic if a ra dio graph ic as ses sm ent h as no t bee n un dertaken o r if th e pai n fa ils to re spo n d to th e ma na geme nt o utli ned abo ve. Q2

Th ere are s evera l ca us es of pai n th at ma y prese nt in o r ad ja cen t to th e

tem po ro ma nd ibu lar jo in ts. Ma ke a l is t o f th e po ssi ble diffe rentia l di agn o ses .

Ta ble 16. 5 lis ts ca us es of pai n i n o r ad jace nt to th e te mpo roma nd ibu lar jo in ts.

Projects 1.

Des cri be the fea tu res of th e diffe rent a na to mi cal zo n es o f th e artic ul ar d isc an d li st the

orig in an d in sertio n of a ll th e mu s cles o f ma stica tio n. Ex plai n w ith th e a id o f d iag ra ms

th e di fferen ce s be tw een di sc dis plac eme nt w ith red uc ti on an d d isc di spla ce men t with ou t re duc tio n. 2.

Wh at a re the su rgic al tre atmen ts ava ila ble fo r TMPDS ?

3.

Wh at ro le d oes th e p hys io th erap ist p lay i n th e mana geme nt o f TMP DS ?



Copyri gh t Β©2003 Oxfo rd Un ive rsity P ress > Tab le of Cont e nts > 17 - Psy ch og en ic or ofac ial p rob le m s

17

Psychogenic orofacial problems Problem cases Case 17.1

A 55-year -old fema le p atien t pres ents with a 3 -mon th h is to ry o f a β€ burn in g a nd ti ngl in g sen sa ti on o n th e tip o f th e to n gue β€™. The patien t rela te s th e o ns et of symp to ms to th e provi sio n of ne w repla cem ent c om plete d entu res. Q1

Ho w wo ul d yo u i nve stiga te this la dy's symp to ms and w ha t are th e po ss ible cau s es?

Case 17.2

A n ew patien t pres en ts to yo u r prac tice an d giv es a 3-year h is to ry o f to o th ac he . O n exami na ti on the patie nt h as a h eavi ly res tored den tition . Q1

Dis cu ss h ow yo u m igh t diffe re ntia te betwe en p ain o f de ntal ori gin an d a typ ica l odo n ta lgi a in thi s patie nt.

Case 17.3

A 60-year -old eden tul ou s f emal e is se en by a co lleagu e in yo ur d en ta l prac ti ce. Th is lady ha s

a 3 -mon th h is to ry o f fa cia l p ain , wh ich is in crea sing in in ten s ity. Th e e xamin in g d entis t th in ks th at the patie nt h as eith er atypi cal fac ial pai n o r trige min al n eu ral gia. Rad io graph s do no t

sh ow an y si gn ific an t abn o rm ali ty an d the den tu res are s atis fac to ry. You a re as ked to giv e yo ur opi ni on . Q1

Wh at in fo rmatio n from th e p ain h isto ry an d c lin ical e xami na ti on w ou ld yo u us e to

diffe re ntia te betwe en th es e two co n ditio ns ? (Yo u wil l a lso n eed to read Cha pte r 15 to ans we r th is que stio n. )

Introduction

A s ubs tan ti al n u mber o f o rof aci al prob lems ma y oc cur as a man ifes tatio n o f p sych o so matic dise as e. Th es e in cl ud e atypic al fac ial pai n, ten sion hea dac h es (s ee Cha pte r 15 ), ora l

dysa esth esi a (in cl ud ing bu rnin g m ou th s ynd rome ), a nd dis tu rba nc es i n ta ste an d sa liv atio n.

Psyc h olo gi cal sym ptoms ma y agg ravate or i nitia te disea se. Ma ny co nd ition s th at h ave org ani c cau se s c an ha ve s ign ific an t psy ch os oma tic co mpo ne nts, s uc h a s a sthm a a nd migra in e. It h as been es ti mated th at app ro xim ately on e-th ird o f a ll c o ns ulta ti on s b etwee n p atien ts an d th eir medic al prac ti ti on er a re es sen tial ly ab ou t psy ch ologi cal pro blem s. Diagn o sis o f a

psyc ho so ma ti c s ympto m do es no t nec es sari ly im ply that the patie nt h as an un derl ying

psyc hi atric illn es s. A tra ns ien t emo ti on al dis orde r, s uc h as an an xiety s ta te or stress fu l li fe

even ts , can freq uen tly g ive ris e to oro fa cia l symp to ms . P atien ts w ho a re emo tio na lly d istu rbed may p resen t wi th ph ysi ca l symp to ms an d the re is an u nfo rtun ate ten den cy to di smi ss th es e as bein g β€ imag in aryβ€™. It i s im porta nt to appre cia te th at e motio n al d istu rban ce s c an ha ve

an effec t on the ho rmo na l, vas cu lar, a nd mus cu lar sys tem s, an d ma y pro du ce phy sic al

sympto ms su ch as xero stom ia or fa ci al p ain . The denti st, ho wev er, m us t alw ays be v ery

caref ul to el imin ate an y org an ic c au se for patien ts' s ympto ms befo re di agn o sin g th em a s be ing due to a n u n derlyi ng emo ti on al or p syc ho lo gic al prob lem.

Emo tio na l di sturba nc es (an xiety, depre ssi on , an d s tres s) c an exa cerb ate or c au se phy sic al sympto ms.

It is al so imp ortan t to be ar in mi nd th at pa tien ts wi th o rof aci al pain o f ps ych o gen ic o rig in may still devel op pai n o f d ental o ri gin . It c an be a ll to o eas y fo r a c lin ic ian to be l ess

disc rimi na to ry in a sse ss ing β€ new pa ins β€™. A th oro ug h p ain h isto ry an d den ta l examin atio n are a lwa ys re qui red w he n th e pa in hi story a lters. Patien ts with a h is to ry o f ps ych o lo gic al prob lems frequ en tly fee l tha t th ey h ave been β€ labe lled β€™ an d a re preju dge d by th e

medic al an d de ntal pro fess io n. Th e cli ni cia n n eed s to keep an β€ ope n m ind β€™. The patien t sh ou ld b e ab le to reco gn iz e tha t his o r he r co mpla in t is bein g take n s erio us ly. It is alw ays wo rth rememb erin g tha t a pa tien t may h ave an u ndi agn o sed, li fe-th reaten in g co nd ition .

Examples o f psychoso matic diseases

Oral dysa esth esi a

Te mpo rom an dibu la r dysf un ctio n

Dis rup ti ve ga ggi ng

Dry m ou th

An orexi a nervo s a

Atypic al fac ial pai n

Te ns io n h ea dac hes

Chro ni c fatigu e s yn drom e

Pan ic attacks

Th e b us y den tal su rgery i s n ot th e ide al p lac e to eli cit a so cia l h is to ry a nd patien ts m ay be

re lu ctan t to di scu s s pe rso na l pro ble ms i n s uc h an en viron me nt. How ever, ma ny patie nts b ui ld up a rela tio ns hip of tru st w ith th eir d en ti st, p artic ula rly i f th ey h ave been u nd er h is o r h er

care for a w hil e. Takin g a so ci al an d psyc ho lo gic al hi story is often time-co ns umi ng but c an be inva lu abl e if it prov ides an in si ght i nto oro fac ia l sy mptom s w ith n o demo n strabl e o rgan ic

cau se . A p atien t may a ppe ar to be c opi ng brave ly w ith a co n ditio n u n ti l qu es ti on ed abo u t

lifes tyle a nd emo ti on al we ll -bein g. Ta ble 17. 1 su gges ts s everal qu estio n s tha t may be u sef ul in ass ess in g h ow a patien t is co pin g w ith a ch ro nic c on ditio n su ch as atypi cal fac ia l pa in or

burn in g mo u th syn dro me. The us e o f ps ych om etric q ues tio nn aire s c an be h elp ful . Ho w ever, man y requ ire s kill ful in te rpretatio n. Th e Ho spi ta l A nxi ety an d Depres si on (HAD) s ca le is a

re lative ly s tra igh tforw ard i nd ica to r o f a patien t's a nx iety an d d epres sio n . It c an as sis t th e

cli nic ia n i n i den ti fyin g em otio na l pro bl ems in patien ts. Th e sc ale was de vise d fo r us e in n on psyc hi atric ho spi ta l de partmen ts a nd ca n b e fill ed i n b y th e p atien t ove r a fe w m inu tes, a nd qui ckly sc ored by the cli ni cia n.

Patie nt c on fide ntia lity is o f the utmo st im po rta nc e an d mu st be mai ntai ned in al l as pec ts o f cli nic al den ti stry. Thi s po in t mu st be stress ed to al l me mbers of th e d enta l team.

Th e su cc ess ful man ag emen t of ch ron ic pai n c o ndi ti ons re qui res th at ps ych o log ica l iss ue s a re addre sse d.

Te rmin ol og y ca n s om etimes be co nfu s ing to th e u nin itia te d. So matiz atio n is a term u s ed to

desc ribe th e proc es s w here by an in di vidu al' s ps ych olog ica l a nd so cia l di stres s a re man ife sted as b od ily s ympto ms th at c an no t be w ho ll y attribu te d to o rga ni c pa tho lo gy. So mato fo rm

diso rders are th eref ore psyc ho gen ic co n ditio ns . Ho wever, there are strict c riteria th at s ho u ld

P. 206

be fu lfi lled befo re a dia gn os is of s o matof orm dis orde r is made . So matiz atio n is a term u se d to des cribe the p roc ess wh ereb y an in div idu al's psyc ho lo gic al an d so c ial dis tres s are m an ifes te d as bo dil y sym ptoms tha t can n ot be wh ol ly a ttribu ted to o rg an ic path ol ogy.

Table 17.1 Useful questions when taking a history of pain with suspected psychogenic origin Ho w are yo u sl eepin g?

Ha s y ou r ap petite be en a ffec ted by th is co nd ition ?

Do es th e c om pla int s to p y ou en joyi ng you rsel f (e. g. so cia liz in g w ith frien ds)? Ho w do you r fa mily /partne r/frien ds reac t to yo ur c ondi ti on ?

Wh at do yo u th in k (o r do you h ave an y idea wh at) h as ca us ed th e pa in? Do yo u d o a ny th in g to hel p yo u co pe w ith you r pa in /dis co mfo rt?

Do es an yth in g take yo ur mi nd off th e p ain (e. g. exerc ise , re laxa ti on , drin kin g alc oh ol )?

Chronic orofacial pain Atypical facial pain Clinical features

Th is is a re lative ly co mm on ca us e o f n on -odo n to gen ic fac ial pai n, w hic h is e nc ou n tere d

partic ula rly i n mi ddl e -aged wo men . Atypic al fac ial pai n i s c ha rac teri ze d by a co n ti nu ou s dul l ach e. If the pai n i s n ot co ntin u ou s, it is pres en t for m os t of th e time . Th e d esc riptors o f the

pain c an be va riab le a nd are o ften emo tive. The pain f requ ently affe cts th e ma xill a a nd can be bila te ral o r u ni latera l. How ever, it is n ot u nu su al fo r it to b e po orl y lo cal iz ed. The pai n i s n ot us ua lly p ro vo ked b y fac ia l mo veme nts, a nd gen erall y fail s to resp on d to si mple an alg esi cs.

Cha ra cteris tica lly, it a ffec ts the no n -mus cu lar sites of th e f ace an d a n e piso de may las t for

ho urs or days , alth ou gh it ca n be in termitten t. Th e pa in is frequ en tl y ag grava ted by fa tigu e, wo rry , o r emo tio na l u pse t.

Atypic al fac ial pai n i s c ha racteri zed by a co ntin u ou s dul l ac he tha t c an be bi latera l o r un ila te ral b ut freq uen tly a ffec ts the maxi lla .

Severa l s pec ial ist o pin io ns ma y ha ve b een so ug ht and n u mero us in vestig atio ns un derta ken

prio r to th e pa ti ent pre sen ting to th e o ral medi cine c lin ic . Ty pica lly , p atien ts h ave b een se en

by (o r alrea dy h ave app oin tmen ts fo r) E NT spec ia lists , p hys ic ian s, ma xill ofa cia l s urge on s, a nd neu rol og ists . M an y ha ve s ou gh t tre atmen t from prac ti ti on ers of altern ative medi cin e. Ta ble 17. 2 su mma ri ze s the fea tu res of atypic al fac ial pain.

A n um ber o f stu dies h ave su gges te d tha t atypic al fac ial pai n i s o ften pro voke d by s om e fo rm

of d enta l treatmen t, su ch as extractio n s o r res to rative pro cedu res , b ut it ma y a lso be ini tiated or exac erba te d by s tre ss ful life even ts s uc h a s b ereav emen t, di vorc e, or illn es s i n th e fa mil y. Th e p atien ts fre que ntly c om pla in of pain el sew he re in the bo dy, wh ich ma y h ave b een

diag no se d as irritab le b ow el s ynd rome , ten si on h eadach es , o r dysm eno rrh oea (a mo ng man y o th er p os sib ilitie s), so tha t th ere is f requ ently a co mple x (an d pe rha ps

P. 207

ina cc ura te ) hi story o f c hro n ic ill hea lth. It ha s been estim ated th at up to 80 per c en t of

patien ts w ith psyc ho gen ic fac ia l pa in ha ve oth er chro nic pa in co nd ition s (see Ta ble 17. 3). Th ere is als o co ns idera ble ove rl ap of s ympto ms betwee n a typic al f aci al pain a nd ch ron ic tem po ro ma nd ibu lar p ain dys fu nc ti on . So me a uth orities b elie ve tha t th e two gro u ps are

ind istin gu is hab le. It is c ertain ly th e c as e tha t some pa tien ts wi th a typ ica l fac ial pai n m ay gi ve a h isto ry o f in te rmittent temp oro man di bul ar jo in t (TM J) dysf un ctio n, a nd othe rs rep ort sympto ms of ora l dys aes th es ia.

Table 17.2 Features of atypical facial pain Du ll, n agg ing na ture b ut pa in des cripto rs m ay n ot be co n sis te nt Emo tive a djec ti ves m ay be us ed to de scri be th e pa in Pai n i nten si ty may va ry

Uni latera l o r bila teral dis trib utio n of pai n b ut loca ti on ma y ch an ge with ti me Pai n i s n o t u su al ly rel ated to an ato mic al d istrib utio n of a nerve Simp le a na lges ic s a re u su all y in effec tive

Pai n exac erba te d by s tre ss an d/o r den ta l treatme nt Hi sto ry of oth er ch ron ic pai n d iso rders is co mmo n No o bvio u s u nd erlyin g o rga nic sig ns

Hi sto ry of exten sive resto rative a nd /or s urg ica l treatme nt to res ol ve the pai n i s co mmo n

Frequ en tl y pa ti ent h as bee n s een by seve ral s pec ialists fo r th is co nd ition

Table 17.3 Chronic medical conditions frequently associated with atypical facial pain Irrita ble b ow el syn dro me Dys peps ia

He ada ch es

Dys men orrh oe a

Ne ck a nd/o r bac k pa in Fibro mya lgi a

Chro ni c fa ti gu e syn dro me

Atypical odontalgia

Atypic al odo n to la gia (idi opa thic od on ta lgi a) is a varia nt in w hi ch th e p ain is lo ca liz ed to o ne to oth (o r a nu mber of tee th ) tha t app ears to be cl ini ca lly a nd radi ol ogi cal ly s ou n d. This

situ atio n i s ve ry diffi cu lt to d iag no se . Th e s ymptoms c an res emble tho se of pul pitis an d

perio do ntitis an d rep eated repla cem ent res tora ti on s f ail to res ol ve th e p ain . Opera ti ve

interve ntio n can o ften aggra vate th e co n ditio n. Cl ass ic all y, th e o ffen di ng to o th is roo t-fill ed in an atte mpt to elim ina te pa in of pul pal ori gin . P erira dic ula r su rgery may th en be c arrie d o ut.

Ultima te ly, th e to oth is extracted . Th e p ain is then freq uen tly tran sf erred to an ad ja cen t too th or te eth. On o cca si on , th is ca n re su lt in an ag grieved pati ent bl ami ng th e d entis t for ta kin g th e w ron g to oth ou t. In the abs enc e o f teeth the p ain ma y the n persi st in the alv eol us .

Expl ora to ry s urge ry an d rid ge -smo oth in g pro ced ures ma y be tried befo re a dia gn os is of atypic al f aci al pai n i s ma de.

A prov isi on al dia gno si s o f a typ ica l fa ci al p ain ca n often be made after l isten in g to the patie nt's his tory, bu t it is ess en ti al to eli min ate a ll o the r demo n strabl e ca us es of fac ial pai n,

partic ula rly th os e du e to den ta l c on di ti on s. Pa ti ents wh o give a h isto ry su gge stive o f s en so ry distu rban ce , o r h ave evide nc e of a c ran ial nerve defi cit, mu st be referred for spec ia lis t

evalu atio n an d ima gin g, to el imin ate th e po s sibi lity o f a laten t ne op las m o r gen eral iz ed neu rol og ica l di so rder.

Patie nts with un expl ain ed sen so ry de fici ts o f cra nial n erve s s ho ul d be referred for furth er

inve stiga ti on to excl ude sp ace -oc cu pyin g le sio n s a nd un dia gn os ed n eu rol ogi cal co n ditio ns . Th e re su lts of on e s tu dy h ave sh ow n th at a bo ut h alf th e p atien ts pres en ti ng with sym ptoms o f atypic al f aci al pai n w ere s uff erin g fro m a n u nd erlyin g p syc hi atric d istu rban ce , mos t co mmo n ly a dep ress ive i lln es s o r n euro si s. Ad verse life events are frequ en tl y revea led on cl os er

que stio ni ng o f p atien ts w ith a typic al f aci al pain , an d in cl ude mari ta l di ffic ul ti es a nd ch ron ic il l

hea lth i n th e fa mil y. Ch ro nic an xiety a nd bou ts o f de pres sio n are a lso i mpo rta nt fa ctors in thi s typ e o f fac ial pai n. Un fo rtu n ately, so me patien ts with atypic al fac ial pai n d o n o t readi ly a cc ept th e im po rta nc e o f u nde rlyin g ps ych o log ica l fa cto rs a nd su ch in divid ua ls are fre que ntly d iffi cu lt to ma na ge. The y of te n p re fer to purs ue nu mero us fu rth er i nve stiga ti on s o r exp lo ratory

su rg ery, wh ich , as men tio ned previo u sly, ma y exa cerb ate the situ atio n. It is ess en ti al n o t to impl y to th e patie nt tha t th ere i s a nyth in g β€ imag in aryβ€™ in vo lved in th eir p ain a s thi s

may b e met w ith d isb elie f an d, in so me cas es , a ng er. Des pite g oi ng to grea t len gths to a vo id

patien ts mi sin terpretin g yo u r dia gno s is, the y are frequ ently of th e o pin io n th at yo u thin k th eir

pain o r co n ditio n i s β€ all in th eir min dβ€™. Thi s be lief may b e s o fi rmly h eld tha t th e pa tien t

wil l refu te yo ur d iag no sis an d dec lin e y ou r adv ice. Th e a utho rs fin d it h elp ful , w hi le d isc us si ng th e situ atio n w ith th e p atien t, to co mpa re the pai n to tha t of a β€ stress -ind uc ed β€™

hea dac he , w hi ch may oc cu r as a re su lt of cra mped mus cl es o r blo od ves sels . The co nc ept o f a

paral lel with a β€ stress he ada ch eβ €™ is gen eral ly w ell a cc epted by pati ents , w ho a re ab le to appre cia te th at th is is n o t gen eral ly du e to an y u nde rlyin g pa tho lo gy.

Patie nts with atypic al fac ial pa in often wa nt a ph ysi cal so lu ti on to th eir co ndi ti on . Th ere are s evera l o ptio ns ava ilab le to the cli nic ian i n th e ma na geme nt o f fac ia l pa in .

Cou ns ell ing an d reas su ran ce m ay be al l tha t is re qui red by so me p atien ts, bu t o the rs ma y

re qui re psyc ho trop ic m edic atio n, w ith o r w itho u t p syc ho thera py. The se o ptio ns are ou tlin ed a t th e en d o f th is sec ti on .

Oral dysaesthesia

Oral dysa esth esi a is a te rm us ed to den ote d istu rban ce s o f o ral s en sa ti on . It in cl ud es

co ndi ti on s su ch as bu rn in g mou th syn drom e, but a lso en c omp ass es symp to ms of abn o rmal ta ste (dys gu esi a) o r xero stom ia i n c as es w h ere the re are no cl in ica l s ign s o r di sce rnib le

cau se . A nu mber of diffe ring fea tu res of ora l dys aes th es ia may o cc ur in th e s ame patie nt.

Burning mouth syndrome

Bu rnin g m ou th s ynd rome (BM S) (s to ma to dyn ia in an older termi no lo gy) is a co nd ition in w hi ch th e pa tien t prese nts w ith a co mpla in t of a ge nera liz ed s o re ne ss or bu rn in g se ns atio n i n th e mou th. Th e to n gue is frequ en tl y aff ected (g lo ss opyro si s o r gl os so dyn ia) b ut the bu rnin g

sen sa ti on ca n affec t all pa rts of th e o ral mu co sa an d in s ome in stan ces is lo cal iz ed to a s mal l disc rete a re a. So me patien ts w ith th es e sym ptoms may ha ve, on exa min atio n, s ome read ily re co gn iza ble abn o rmali ty , s uc h a s g eo graph ic

to ng ue th at i s res po ns ible fo r th e s ympto ms. Oth ers h av e a c o mpletel y n orma l a ppea ran ce of

P. 208

th e o ral muc os a a nd it is this gro up wh o are i nc lu ded i n th e di agn o stic categ ory o f B MS . A fu ll

ass ess men t of th es e pa ti en ts is ess en ti al to eli minate an y ide ntifi abl e ca us e, su ch as sys temic dise as e o r cli ni cal ly u nd iagn o sed ora l can di dos is . S cree ni ng tes ts s ho u ld be un dertak en

ro u ti nel y to excl ude ha ema ti nic defi cien c ies or e ndo cri ne prob lems su ch a s di abe tes (se e

Ta ble s 17. 4 an d 17. 5). A nu mbe r of patie nts w h o c ompla in of a bu rni ng mo uth als o ha ve a dry mou th a nd sh ou ld be i nves tiga te d to e limi na te co nd ition s su ch as Sj Γ¶gre n's syn dro me (Cha pte r 8).

Den ture -ind uc ed pro bl ems sh ou ld be el imin ated as far a s p os sib le, bu t it mus t be a pprec ia ted th at s ome in divi dua ls can n ot to le ra te den tures , ho we ver we ll c on s tru cted. A very s mal l

prop ortio n of p atien ts with BM S w il l h ave h ad th ei r prob lem asc ribe d to a n all ergy to den tu re materia ls, a nd it may be extrem ely di ffic ul t to di ssu ad e pa ti ents fro m thi s attra ctive , b ut

un like ly ca us e. Al lergy to de nture materi als ca n o cc ur but i s very u nc om mon . Irri ta tion du e to leac hi ng ou t of exce ss ac ry lic mo no mer h as al so been repo rted, bu t sh ou ld no t oc cu r if th e

den tu res ha ve bee n p roc ess ed co rrectly. In on e s urvey of patien ts p resen tin g wi th a bu rnin g mou th, the patie nts w ere p redo min an tl y (bu t no t excl us ivel y) fema le w ith a me an age of arou n d 60 years an d w eari ng c o mple te den tu res . Th e ma jori ty co mpla in ed o f a burn in g

sen sa ti on in the to n gue an d u pper den tu re -beari ng area. Th e n ext mo st co mmo n site w as the

muc os a o f th e li ps a nd , th en, o ther sites of th e o ral muc o sa. F rom th e res ul ts o f thi s s urvey it wo uld ap pear th at thre e gro up s o f patie nts e merged : tho se with a d emo ns tra ble so urc e o f

loc al irritatio n (50 pe r cen t); th o se w ith an ide ntifi abl e sys temic ab no rmal ity (30 p er ce nt); and tho se with a p syc ho gen ic bac kgro un d (20 per c ent). S ystemic fa ctors in cl ude d hae mato lo gic al d efic ien ci es a nd un dia gn os ed d iabe tes, bu t othe r fema le e ndo c ri ne abn orm alitie s w ere n o t p romi ne nt.

Features o f burning mo uth syndrome

Predo mi nan tly m iddl e-aged fema les (7 fema les :1 mal e)

Ge nera liz ed or l oc ali zed bu rnin g o f th e ora l mu co sa

Oral muc os a l oo ks no rmal

Table 17.4 Burning mouth syndrome: underlying conditions Dia betes Ha ema ti ni c defi cien c ies β€”vitamin B 1 2 , i ro n , f ol ate*

Sal ivary glan d hyp ofu n ctio n. Can dido si s

Para fun c ti on al ha bits (c hro n ic tra um a)

Ga stro -oes op ha geal refl ux d ise ase (GO RD) Dep ress io n

All ergy to resto rative o r de ntu re materia ls *V itami n B 1 , B 2 , B 6 defi cien c ies ha ve al so bee n i mpli cated bu t th ere is little s ci entifi c

evide nc e to s up po rt th is ass o cia ti on .

Table 17.5 Burning mouth syndrome: investigations Deta iled cli ni cal hi sto ry

Fu ll cli ni cal exa min atio n Blo o d tests

Fu ll blo od co un t Se rum B 1 2 + fol ate

Red b lo od cel l fol ate Se rum ferritin B loo d glu co se

Mic robi ol ogy β€”qua ntitativ e as ses sme nt o f c arria ge o f ora l Can dida *

Ass es smen t of sa liva ry gla nd fun ctio n

*Iso la ti on o f Can dida spe cies is no t in dic ative o f a ca nd ida l in fec ti on . There fore , a

ro u ti ne sw ab i s of limi te d u se i f the re are no cl inica l s ign s. Qu an ti ta tive ass es smen ts are o nl y u nde rta ken in spe cia lis t cen tres.

Th e re su lt of on e s tu dy ha s s ugg ested th at s in gle or c omb in ed defi cien c ies of vitami ns B 1 , B 2 ,

or B 6 may b e prese nt in a nu mbe r of patien ts w ith BM S, an d th at vitami n th erap y may ben efit

su ch in divid ua ls. Ho we ver, o the r stu dies ha ve fa iled to sh ow an y res pon s e to th es e vitam in B su pplem en ts . S eru m le vels of zi nc ha ve be en inve stiga ted in patie nts w ith BM S, bu t th ere i s

no co n vinc in g evi den ce th at z in c d efic ien cy i s in vol ved in th e pa th og en esis o f thi s c on ditio n . A n um ber o f patie nts d iag no sed as su fferi ng from BMS h ave no id entif iabl e u nd erlyin g c au se

for th eir c om plai nt. The se i dio pa th ic ca ses frequ entl y h ave a n un derlyi ng psyc ho ge nic ca us e,

su ch as ch ron ic an xiety o r dep ress io n. Ma ny ha ve a can ce r pho bi a a nd kno w o f s om eo ne w h o has s uffe red fro m o ral ca nc er. It is there fore hel pfu l to explo re th e pa ti en ts ' bel iefs ab ou t

th eir c on di ti on . This ma y in vol ve ask ing them directly if th ey th in k they migh t ha ve c an cer. So me pa tien ts wi th th is co n ditio n re spo nd we ll to reas su ran ce, s upp leme nted by an

inf orma ti on lea flet ab ou t BM S. Ho weve r, o thers ma y requ ire p syc hia tric i nterven tio n, in the

form of an ti depres sa nt me dic atio n w ith or w itho u t psyc ho thera py (s ee n ext sec ti on ). An overvi ew of man age men t o ptio n s fo r B MS is g iven in Ta ble 17. 6.

Management of chronic orofacial pain

Th e m an agem ent o f c hro n ic o ro fac ial pai n i s u su al ly u nde rta ken in a h o spi ta l en viro nm ent wh ere the patie nt c an be ful ly in ves tigated

and the respo n se to med ica ti on an d ps ych oth erap y monito red. On rec eipt o f ref erral f rom a

P. 209

den ti st it is o fte n advi sab le to wri te to th e pa ti ent's medi ca l pra ctitio ner to en qu ire i f there is any releva nt m edic al, ps ych o log ica l, an d ps ych ia tric h is to ry. Thi s a llo ws in valu ab le

inf orma ti on c on cern in g pre vio us thera pies an d trea tm ent to be co lla te d an d a ss ess ed.

Simi larl y, it is impo rtan t f or th e o ral p hys ici an to k eep th e m edic al prac ti ti on er in fo rmed abo ut patien t man ag emen t.

Table 17.6 Management of burning mouth syndrome Eli min ate

Sy stemic dis eas e

Loc al cau s es (e. g. can di dos is )

Cou ns ell ing

Cogn itive beh avi ou ral th erap y β€” re fer to c lin ic al psyc ho lo gis t for a ss ess men t An ti depres sa nt th era py

A nu mber of den ta l s ch oo ls in the U K an d el sew he re no w ha ve ps ych ia tri c li ais on cli ni cs th at can pro vide an id eal envi ron men t fo r th e p atien t to be in tro du ced to ps ych ia tri c a dvic e.

Patie nts can fi nd th e di agn o stic lab elli ng of a c onditio n very rea ss urin g. A sma ll p ropo rtio n o f patien ts a re extremel y an xio us o r agi ta te d bec au se th ey b elie ve tha t th ey h av e a s erio u s o r life -th reaten in g c on ditio n su ch as ca nc er. Reas su ran ce m ay b e al l tha t is nee ded for so me patien ts. So me patien ts c an dem on strate re marka ble i ns igh t in to the ir co nd ition , an d co un sel lin g o r a psyc ho lo gic al app ro ac h to th erapy may b e hel pfu l.

Antidepressant medication

Tri cyc lic an tidep re ss an ts , su ch as do su lepi n hydro ch lo ride (do th iep in ) or n o rtryp tyl ine , h av e

been us ed su cc ess ful ly in o ther forms o f ch ro nic pain , s uc h as bac k pai n, an d th eir an alg esic

actio n app ears to be in depe nde nt o f the an ti depre ssa nt a ctio n. Th ese drug s h ave been s ho wn to be effec tive i n a typ ica l fa ci al p ain an d B MS bu t th ey do ha ve s ome sid e-effec ts , su ch as

drow si nes s a nd dry mo u th . It i s h elpf ul to make it cl ear to the p atien t tha t th e a ntide press an t is b ein g gi ven for its pa in co ntro l pro perties an d th at it i s n ot li kely to le ad to an y fo rm o f addi ctio n. Pa ti ents wil l o ften app re cia te the pain -re liev ing pro perties of an ti depre ssa nts if

th ey a re ma de a ware of th ei r us e in o th er c hro n ic pain co n ditio ns s uc h a s arthri ti s, ch ron ic

bac k pai n, an d p os th erpe ti c n eu ralg ia. It is a ls o help ful to in form patien ts th at ch ro nic pa in often ca us es a rea ctio na ry dep ress io n. Dro ws in ess has alrea dy be en men ti on ed as a s ide-

effec t of tric ycl ic an ti depres sa nts an d thi s ma y be prob lema tica l. An al te rna ti ve wo u ld b e to

us e on e o f th e no n sed atin g se lec ti ve se roto nin reu ptake in hi bitors (SS RIs) s uc h a s sertral ine .

Ho w ever, if a pa ti ent w ith ch ron ic oro fac ial pa in is h av ing diffi cu lty sl eepi ng , th e se datin g prop erti es of tric ycl ics ma y be h elp ful . Xero gen ic si de -effec ts li mit thei r us e i n o ral dysa esth esi a.

Benefits from t herapy with tricyclic ant id epressants

An alge sic eff ects

Imp rove d sl eep

Mo od eleva tion

Cognitive behavioural therapy

Th ere are s evera l types of psy ch olo gi cal app ro ac h to th e man ag emen t of psyc h oge nic

prob lems in clu di ng ch ron ic pai n. Cog nitiv e beh avi oural th era py (CB T) is o n e tech n iqu e tha t may b e u sed by cli ni cal psy ch ol ogi sts. In ess enc e, CBT explo res an in divi dua l's emo tion s , th ou gh ts, attitude s, an d b elie fs. The co ns equ en ces tha t th es e h ave o n beh avi ou r are a lso

ass ess ed. F re que ntly th e th erapi st wi ll d isc ov er tha t a pers on 's ass u mptio ns an d be lief s ma y

be u nh elp ful an d irra ti on al . Th is is of ten th e ca se in patie nts w h o h ave β € can ce r ph obi a β€™.

Th ei r beli efs agg ra vate th eir a nxi ety. The goa l o f CB T is to rep lac e dys fu nc ti on al co gn itive

struc tu res with mo re reali stic fu nc ti on al on es β€”cogni ti ve res tru ctu rin g. Ta ble 17. 7 sh ow s th e stage s in vo lved in CBT. A cas e h is to ry o f o ne patien t wi th BM S i s gi ven in th e text bo x.

Side- effect s o f antidepressants Tricyclic ant id epressants

Th es e c omm on ly h ave th e fol lo win g s ide -effec ts :

seda tion ;

dry mo uth ;

co ns ti patio n;

blu rre d visi on ;

urin ary re te ntio n.

Th es e eff ects are d ue to the an ti ch ol in ergic (mo re sp eci fica lly, an ti mus ca rin ic) a ctivi ty of

tric ycl ic an ti depres sa nts. Th e in di vidu al drug s with in the tric ycl ics do vary in th eir prop ens ity to ca us e s ide -effec ts (fo r exam ple, the re are tri cycl ic s tha t ha ve in cre ase d se dative

prop erti es). S ome memb ers h ave add ition al si de -effec ts . Tole ran ce to si de -effec ts do es oc cu r

in man y pa ti en ts . Th e s ide -effec ts are redu ced by comm enc in g th erapy on lo w d os es an d the n inc reas in g the do se g radu al ly. Thera py fo r el derly p atien ts s ho u ld alw ays be i ni ti ated a t lo w dos es bec aus e th e h ypo ten si ve pro pertie s o f tri cyc lic s pre dis pos e to diz zi ne ss an d po ss ible syn co pe.

Selective serot onin re -uptake inhibit ors (SS RIs)

Th es e h ave fewe r an ti mus ca rin ic s ide -effec ts tha n tric ycl ics . The typ ica l s ide -effec ts are :

gas tro in te stin al dis tu rban ce s s uc h a s n au s ea, dys peps ia, vo mitin g, abd omi na l pa in ,

hea dac he ;

sexu al dysf un ctio n.

diarrh o ea, an d c on stip atio n;

Abru pt ce ssa tion o f the rapy w ith SS RIs is no t reco mme nde d as it c an be a ss oc iated with

hea dac he s, para esth esi a, diz zi nes s, a nd a nx iety.

Hy pon atrae mia is a po ss ibl e sid e-effec t of a ll an tidepres sa nts in th e e lderl y an d ma y be

man ifes t as drow si nes s, c on fu sio n, o r co n vul sio n s. Comp lete pai n rel ief is n o t alw ays fea sibl e a nd patien ts s ho u ld be mad e aw are th at CB T can

hel p the patie nt u nde rstan d the ir co n ditio n an d symptom s an d h o pefu lly β€ tu rn dow n β€™ the pain vo lu me. Pa in dia ries ha ve prov ed to be a us eful to ol in hel pin g pa tien ts u nde rstan d tha t th eir e nvi ron men t can i nfl uen ce pai n.

Table 17.7 Major components of cognitive behaviour therapy with chronic pain patients* Step 1 A sse ss men t Pai n level s

Inten si ty

Fre que nc y Dura ti on Qu al ity

Si gn al of impe ndi ng pai n ep iso de or a tten u atio n

Emo tio na l rea ctio ns to pai n Worry

An xie ty

Depres sio n

An ger an d h os ti lity

Cogn itio n s an d beli efs

Se lf -effic ac y (i. e. β€ Can I co pe w ith th e p ain ?β€™ o r β € Can I do wh at the

cli nic ia n i s exp ectin g m e to do ?β €™) Loc us o f co ntro l

E xpecta ti on s o f p ain (o ver a ti me co urs e, or i n c ertain situ atio ns )

Beh avi ou r

Me dic atio n (pre sc ri bed an d n on -presc ribed ) Ac tivity level s

Av oid an ce o f p ain fu l are a (w hen ea ting , po sitio ni ng to n gu e) E nc ou ragi ng symp athy an d su ppo rt from oth ers

Step 2 D erive formu la ti on (see Fig. 17.1) Step 3 Co gn itive restru cturi ng Re laxa tion

Dis tractio n : us ed i ntermi tten tly duri ng critic al mom en ts (e. g. peak level s o f pa in inten si ty)

Bel iefs : ch an ge n ega tive a nd cata strop hic thi nki ng (e.g . β€ Th is co n ti nu ou s pai n mean s I m us t hav e can c erβ€™) Step 4 B eh avio ura l c ha nge s

Activ ity: in crea se p hys ica l a nd so cia l be ha vio ur

Atte ntio n: decre ase attentio n from oth ers in re spo nse to pa in beh avio u r

Avo ida nc e: redu ce th e de gree o f a voi din g a re as of mou th with in crea sed sen si ti vity

Medi catio n : revie w the us e o f exc es sive medi ca ti on an d ratio n ali ze to eff ective level s * A dap te d, with permi ss io n, fro m Hu mph ris, G. M. , Long man , L. P ., an d F ield , E . A. (1996). Co gni ti ve -beh avio u ra l th erapy for idio pa th ic bu rni ng mou th s yn drom e: a re po rt o f two c as es. Britis h Den tal Jou rna l 181 , 204 β€“ 8.

P. 210

Disturbances in taste and salivation

Patie nts wh o co mpla in o f an u npl eas an t ta ste i n w hom n o abn o rmali ty of an y kin d is detec ted

are very diffi cu lt to m an age . P atien ts c om mon ly re po rt a so u r, m eta lli c, o r bitter tas te an d are typ ica lly m iddl e -aged fema les . Th es e in divi du als ofte n bec ome qu ite o bses si on al abo u t th eir

co ndi ti on an d a re freq ue ntly c on ce rned tha t th ey h ave ha lito sis as w ell a s a ba d tas te . O th ers co mpla in of a dry mou th, des pite the fac t th at th e o ral m uc os a a ppea rs mo is t an d s ali vary

flo w is n orma l. Symp to ms of BM S s om etimes co exis t an d s ome des crib e del us io na l s ymptom s su ch as san d o r gri t in th e mo u th o r an exc ess ive dis ch arge of m uc us . Th e c om pla int c an be ass oc ia ted wi th s ign ific an t detrime ntal ch an ges in die t a nd life style.

Cognitive behavio ural therapy: a case histo ry

An exam ple o f th is beh avio u ral a ppro ac h i s i llu strated in Fig. 17.1. Th is flo w d iag ra m

re prese nts a h ypo th es is or β€ formu la ti on β €™ fo r a p atien t wi th B MS wh o prese nted to the

auth ors ' cl in ic. It su mma rize s th e imp ortan t fea tu res of hi s s ymptom s a nd th e fa cto rs th at

may i nfl uen ce the co ndi ti on . Thi s 38-year -old mal e n otic ed h is ora l dys aes thes ia sh ortly after majo r life even ts β€”re dun da nc y, marita l brea kdo wn , restric te d ac ces s to h is c hi ldren , an d

movi ng bac k to re sid e in hi s pa ren ts ' h ome . Th e p atien t ha d n o i den ti fia ble p hys ica l c au se for

his B MS an d w as re ferred for p syc ho lo gic al ass ess men t. Th e p atien t attend ed thre e 50-min ute

ses sio n s, wh ic h a llo we d the cli ni cal ps ych ol ogi st to co ns tru ct th e form ula tio n i n Fig. 17.1 . Th e psyc ho lo gis t hyp oth esiz ed th at th e ma jo r li fe ev ents prod uc ed a nxi ety. Th e p atien t pres ented with symp to ms of seve re pai n, an xie ty, pa ni c a tta cks , a nd depres si on . His an xiety w as

exac erbated by th e bel ief th at h is c o ndi ti on w as due to c an cer. Th e pa ti ent w as so cia lly

iso la ted an d h ad no fo rmal struc ture to h is day. The pa ti ent w as amen ab le to ps ych ol ogi ca l

interve ntio n an d atten ded th ree fu rther ses sio n s for th era py. The patien t wa s e nc ou rage d to devel op a mo re s tru ctu red l ifes tyl e (regu lar s lee p, eatin g pro perl y), s oc ia l ac tivities w ere

inc reas ed, an d th e beli ef th at he ha d o ral can ce r wa s refu ted. Rela xatio n te ch ni que s w ere demo ns tra ted to th e p atien t an d me th od s to he lp th e pa ti ent c op e wi th th e pai nβ€”co pin g

strategie s β€”were su gges te d. The patien t wa s p ain -free a t a 6-mon th re view app oi ntmen t. Th is ca se his tory i s a dap te d, with permi ssi on , from Hum phri s, G.M ., Lon gm an, L. P. , a nd Fi eld, E. A. (1996). Cog nitiv e-beh avio u ra l th erapy for idiopa th ic bu rnin g m ou th s ynd rome : a rep ort of tw o cas es . Britis h D en tal Jo u rna l 181 , 204β€“8.

Th ere are seve ral c on di ti on s th at ma y ca us e an a lteratio n in ta ste a nd ha lito sis (see Cha pte r 6) an d the se s ho u ld be exc lud ed o r treated in th e first i ns ta nc e. Ma ny p atien ts h ave a

co mbin atio n of symp to ms des crib ed a bove , wh ich c an be m an ifes ta tion s of an un derl yin g

co gni ti ve di so rder. Fi na nc ial wo rries , b ereave men t, a nd can ce r ph obi a a re frequ en tly reve aled . Patie nts may respo n d to reas su ran ce b ut a nu mber wil l requ ire psyc ho lo gic al man age men t.

Fig. 17 .1 Ps ych ol ogi ca l form ula tio n fo r a patie nt wi th bu rn ing mo uth syn dro me. The majo r fac to rs are s ho w n w ith h ypo the siz ed a ss oc ia tion s (a rrow he ads in dic ate the

direc ti on o f in flu en ce). (Th is c as e h isto ry is ada pted, wi th pe rmis sio n , from Hum phri s,

G. M. , Lon gma n, L.P ., a nd Fiel d, E. A. (1996). Cog nitiv e-beh avio u ra l th erapy for idio pa th ic burn in g mo u th sy ndro me: a repo rt o f tw o c as es. Britis h D en tal Jo u rna l 181 , 204 β€“8. )

P. 211

Delusional symptoms

A s mall nu mb er of patie nts ha ve delu si on al symp to ms in vol vin g the ora l c avity. A delu si on is

an abn orm al b eli ef, from wh ic h th e in divi du al c an not be diss u aded an d w hic h is no t in keepi ng

with hi s o r he r cu ltura l ba ckg ro u nd . Th es e pa ti ents may h av e a h is tory o f ps ych ia tri c ill nes s s o it is prud ent to lia ise wi th the medi ca l pra ctitio ner. On e g rou p o f patie nts th at ma y be

enc ou n te re d by th e de ntal su rgeo n are th os e w ho su ffer from delu si on al ha lito sis . De spite inten si ve o ral h ygi ene regi men s a nd nu mero us

inve stiga ti on s, th ese patien ts i ns ist th at thei r brea th sm ells w hen it do es n o t. S uc h p atien ts

P. 212

are very diffi cu lt to m an age an d freq uen tly h ave no oth er ma nif estatio n of a ps ych ia tri c

diso rder. Delu si on al ha lito sis is a f orm of a mo n osympto matic hyp oc ho n driac al psyc ho s is.

An oth er pres enta ti on o f thi s co nd ition i s the β€ pha nto m bi te syn dro meβ€™ in w hic h patien ts

ins is t th at the y ha ve an a bno rma l bi te , w hi le o thers h ave a fa lse beli ef tha t th ere are l ump s o r

seed s u nd er the ora l mu co sa . It is imp ortan t tha t th e de ntis t iden tifie s the patie nt wh o is

delu ded abo u t th eir o cc lu sio n . Irreve rs ibl e o cc lus al reha bil itatio n i n th ese patie nts is to be

avo ided . P atien ts w ith delu si on al symp to ms do no t read ily a cc ept ps ych ia tric as si stan ce or a

psyc ho lo gic all y ba sed expla na tion o f the ir s ymptom s. It i s a dvis abl e fo r the cli nic ia n to in fo rm

th e gen eral medi cal prac titio ner a bo ut h is or h er con ce rns . O rgan ic ca us es o f d enta l an d fa cia l pain s ho ul d be excl ud ed be fore dia gno si ng a d elu sion al s tate. Ea rly reco gn itio n o f d elu sio n al

sympto ms may p rotec t th e pa tien t from un dergo in g protra cted i nves tiga ti on s a nd un n eces sa ry trea tmen t.

A de lus io n is an abn o rm al beli ef, from wh ic h th e indivi du al c an no t be diss u aded an d w hic h is no t in keepi ng with hi s o r h er cu ltu ral bac kgro un d.

Dysmorphophobia

Th e term dysm orph o pho bi a is rath er mi slea din g b eca us e it i s n ot a pho bi a. The patie nt w ith

dysmo rph op ho bia h as a s erio us preo cc up atio n w ith an asp ect o f th eir ph ysi ca l ap peara nc e th at th ey fe el i s de fectiv e. Th e p hys ica l abn o rm ali ty may b e a min or p hys ica l pro bl em o r it ma y be

imag in ary. The su fferer ha s a feel in g o f β€ ugl ine ss β€™ an d a des ire f or c orrec tive trea tm ent.

Wh ils t an y part o f th e bo dy ma y be ta rgeted, a fac ial fea tu re is mo st c om mon ly in vol ved, fo r examp le, the te eth o r fac ia l pro fil e. The e ffec ts of thi s di so rder on the su fferer are va riab le

but th e pa ti ent ma y bec o me dys fu nc ti on al an d requ ire h os pita liz atio n. S uic ide attempts can be a fea ture. P atien ts w ith dysm orph o pho bi a ma y be relen tles s in the ir pu rsu it fo r den tal or

su rg ica l tre atmen t to rec ti fy the ir perc eive d def ormi ty. S uc h th erap ies may s erve to enh an ce

th e pa tien t's mo rbid preo cc upa tion . Diss atis fac ti on, an ger, an d litiga tio n ma y en su e. Cli nic ia ns sh ou ld c aref ull y expl ore a pa tien t' s requ es ts an d exp ectatio n s be fore emba rkin g u pon

co smetic pro ce dures an d b e w ary o f patie nts w ho th ink s tha t tre atmen t is a ma gic al c u re fo r

th eir p robl ems . Th e patie nt wi th dy smo rph oph o bia is no t alw ays ea sy to dia gno s e. Th e b orde rs betwee n an ac cep ta ble an d ab no rmal app eara nc e are op en to su bjec tive i nterpreta ti on . Wh en an un derl ying ps ych ol ogi ca l pro blem is su spe cted a s th e ma in reas on fo r the patie nt's

diss atis fac ti on w ith th eir a ppea ran ce, it ma y be advi sa ble to se ek a spe cia lis t opi ni on bef ore co mmen ci ng trea tmen t.

Cha nge s in the cla ss ific atio n of psyc hi atric dis ease h ave re defin ed dysm orph o pho bi a in to no n delu si on al an d del us io na l va rian ts. The delu si on al ty pe is a psyc ho tic dis orde r an d the no n -

delu si on al ha s be en na med β€ bod y dys morp hic sy ndro me β€™, but f or th e pu rpo ses of th is

ch apter n o dis ti nc ti on h as been ma de be tw een the se tw o varia nts .

Self-injurious behaviour

Th ere are s om e pa ti ents wh o cau se sel f -harm to th eir oro fac ia l tiss ue s β€”th is may or ma y n ot be in ten ti on al. S elf -inju rio us beh avi ou r is wel l rec ogn iz ed in severa l gro u ps o f p atien ts w ith devel opm enta l, phys ic al, or l earn in g di sab ili ti es. E xampl es of th ese co nd itio ns are epil epsy ,

profo u nd neu ro dis abi lity, cereb ral p als y, au ti sm, Lesc h β€“Nyh an s ynd ro me , a nd Riley β€“Da y syn drom e (co n gen ital ind iffere nc e to p ain ). Trau ma to the lip, c hee k, a nd ton gu e are

co mmo nl y see n i n th ese gro ups o f pa ti ents . Ta ble 17. 8 su gges ts s ome man ag emen t op ti on s th at ma y be us ed, bu t trea tm ent i s ra rely sim ple a nd no t al wa ys s uc ces sfu l.

A fac ti ti ou s d iso rder is a ps ych ol og ica l o r ps ych iatric dis ord er ch ara cteriz ed by th e

co mpu lsi ve, vol un tary pro du ctio n of sign s an d sy mptom s o f a dis eas e fo r the so le p urpo se of ass um ing a β€ patien t's ro le β€™ an d i n th e ab sen ce of oth er sec onda ry gai n.

Patie nts wh o d elib erately cau s e sel f -mutil atio n p resen t a d iffi cu lt as ses sme nt an d

man age men t p robl em. Se lf -harm is co mmo nl y as so cia ted w ith d epres sive dis ord ers. The

oro fac ial les io ns ca n b e varia ble in prese ntatio n an d s ite. Ging ivitis artefa cta, w hic h co ns ists of des tru ctive les io ns to th e gin gi vae, is we ll reco gni zed in the literatu re. Often su ch les io ns are c au sed with a f ing er n ail . S imi lar trau ma ti c i nju ries , s uc h a s tra um atic ulc eratio n , m ay oc cu r on the ora l mu co sa ( β€ facti ti ou s s toma ti ti sβ€™) an d o n th e s kin ( β€ dermati ti s

artefac ta β€™). The diag no si s c an be d iffic u lt bu t th e l esi on is ne arly a lw ays in an area

acc ess ibl e to th e pa tien t an d the cli ni cal fea tu res are o ften in co ns is te nt wi th th e hi sto ry an d

othe r oro fa cia l co nd ition s . P atien ts ra rely a dmit to ca us in g su ch le sio n s a nd it ca n b e diffi cu lt to pers ua de th e

patien t tha t th ey a re in n eed of spec ia lis t h el p. Beh avi ou ral th era py ca n be be nef ici al.

P. 213

Ho w ever, so me patie nts m ay req ui re ps ych otro pic medi catio n .

Table 17.8 Management of self-injurious behaviour in patients with disabilities Lo ca l

To pi cal an ae sthetic s To pi cal an ti sep ti cs

Su tu res to c lo se w ou n d Bite -ra is ing app lian c es

Occ lu sal gu ards an d l ip bu mpe rs to dis pla ce s of t tiss ues Re stora ti on o f bro ken teeth Den tal extractio n s

Occ lu sal adju stme nt to te eth System ic

An alge sic s

An ti bio ti cs fo r sprea din g in fectio n s

Eating disorders

An orexi a n ervo sa an d bu lim ia nervo sa are c om mon c onditio n s i n th e Western w orld . E atin g

diso rders pres en t an eno rmo us ch all eng e to so cie ty an d the hea lth -care prof ess io ns . E atin g diso rders o cc ur i n bo th fem ales an d m ale s, bu t a re mo re c om mon in fe male s w ith a ratio o f 10:1. Bro adl y sp eaki ng, a no rexic s a voi d fo od an d c on se que ntly l os e w eigh t, i n c o ntras t to

bul imic s w h o h ave epis o des of b in ge ea ting an d pu rgin g. P urgi ng may i nvo lve th e a bu se o f

laxa ti ves, en ema s, diu retic s, an d ex ercis e; s elf -ind uc ed vo mitin g is als o c o mmo n. Thi s m ay be co ns idere d an o versi mpli fic atio n a s th e dif feren ces betwe en th e two c on ditio n s a re so metim es no t so cl ear -cu t. An o rexic s c an al so su ffer fro m s elf -ind uc ed vo mitin g.

Eating disord ers

An orexi a nervo s a: fo od avo ida nc e; u nd erwei gh t; d isto rte d bo dy i mage

Bu limi a n ervo sa : bin ge e atin g an d p urgi ng; us ua lly no rmal bo dy w eigh t

An orexi a n ervo sa is les s c omm on tha n bul imia an d the med ian a ge o f o ns et is 17 ye ars, bu t it has been repo rted i n p atien ts a s yo un g a s 8 ye ars old . Co mo rbid ity wi th o th er psyc hi atric an d

perso n ali ty d iso rders is we ll rec o gni zed β €”depres sion , s o cia l pho bi a, an d o bse ss ive c om pul sive diso rder are c om mon . Med ica l c on ditio n s ass o cia te d wi th ma ln utritio n oc cu r, s u ch as rena l fail ure, li ver dys fu nc ti on , amen o rrho ea, a nd deh ydratio n.

Bu limi a n ervo sa is more co mmo n th an a no rexia an d is tho u ght to aff ect ap prox imatel y 2 per

cen t of you ng adu lt wo men . Unl ike a no rexi cs, bu li mic s a re us ua lly of no rmal bo dy w eigh t, b ut man y ha ve a hi story o f a no rexi a o r ob esi ty. A s w ith a no rexi a th ere are hi gh level s o f

ass oc ia ted ps ych iatric o r psyc ho lo gic al pro blems . Sys te mic dis eas e c an als o acc o mpan y

bul imia . Oes oph ag eal eros io ns are co mmo n an d frequ en t vom itin g an d mis us e o f la xatives ca n cau se po ta ss ium dep letio n. Th e res ulta nt h ypo kala emia predi spo se s the patie nt to myo card ial ins tabil ity an d f atal a rrhyth mia s c an oc cu r.

An orexi a a nd bul imi a c an bo th h ave a si gn ific an t impa ct on den tal ha rd tiss ue s. Ac id e ros io n of d enta l h ard tis su es is desc ribe d in Cha pte r 18. S elf -ind uc ed vo mitin g a nd a h igh in take of aci dic drin ks a nd fo ods are the p rime c au se s o f tooth wea r in patie nts with eatin g di so rders .

Paro tid g lan d en la rgeme nt ca n als o be a featu re o f ea ti ng dis orde rs, an d mu co sa l l esio n s h ave been repo rted in pa tien ts with bu lim ia. Th is i s b eca us e the fin gers or o bjec ts u sed to i ndu ce

vomi ti ng ca n tra um atiz e the muc os a, in pa rti cu lar th e s oft p ala te . S toma ti ti s m ay a lso o cc ur

due to n utritio n al o r h aem atin ic d efic ien ci es. Th e den tist th erefo re n ot o nl y ha s a rol e in the

preven ti on a nd man age men t o f o ral dis eas e in th ese patien ts, but a lso i n th e id entifi ca ti on o f

eatin g di so rders. Th e de ntal prac tition er m ay be a ble to en co u ra ge th e pa ti en t to ac kno w ledg e th e n eed for p rofe ss ion al he lp an d li ais e w ith th e pa ti ent's gen eral med ica l pra ctitio ne r.

Drugs and alcohol

Dru g de pen den cy, in cl udi ng alc oh o lis m, may p resen t pro ble ms i n th e o ral m edic in e c on te xt, a s in man y oth ers. Pe rha ps th e mo st s ign ifi can t dru g-re lated prob lem affec tin g den tistry i s th e

intrav eno u s dru g β€“HIV β€“hep atitis relatio n sh ip. It is q ui te cle ar tha t man y drug -depen den t

patien ts ma y h ave prob lems o f nu tritio na l an d i mmu ne abn o rm ali ty, resu ltin g in o ral ch an ges su ch as th o se d isc us se d in ea rlier c ha pters.

Th e term β € alc oh ol ism β €™ is us ed as a ge neri c term, ap prove d by th e Wo rld Heal th

Organ is atio n, to des cribe all types of alc oh o l -re lated prob lems . It ha s be en estim ated th at 1 in 10 mem bers of th e p opu la ti on h as a s erio us al co ho l prob lem. P hys ica l p ro bl ems th at ma y re su lt fro m exc ess ive a lc oh ol in take a re wid esp re ad a nd aff ect al l s ystems . A s umm ary is

given in Ta ble 17. 9. M an y of the e ffec ts o f al co ho l a bu se c an imp ac t u po n th e ma na gem ent of th e de ntal patie nt, bu t o f p articu la r releva nc e to ora l me dic ine are nu trition al

defic ien ci es β€”often fo late defic ien cy β€”th at ma y res ult i n stom atitis an d o th er p ro bl ems , a s detail ed i n Cha pte r 13 . Ps ych ol ogi ca l pro ble ms m ay in c lud e dep ress io n a nd may man ifes t

th ems elves as oro fac ia l pro ble ms, su ch a s the bu rnin g m ou th s ynd rome or atypic al fac ial pai n. Occ as ion al ly, patie nts with ch ron ic pa in co nd ition s may re so rt to al co ho l o r oth er dru gs in th e ho pe th at

P. 214

th ey w ill he lp c on trol th eir pai n. The rol e o f a hig h i ntake of alc o ho l to gethe r with toba cc o us e in th e aetio lo gy o f o ral mal ign an cy w as dis cu ss ed in Cha pte r 10.

Table 17.9 Effects of alcohol abuse on general health System affected Cardio vas cu la r system

Imm un e s ystem

Effect Hy pertens io n

Cardio myo pa th ies Dys arrythm ias

In crea sed su sc eptibi lity to in fec tio n

In crea sed su sc eptibi lity to mal ign an cie s

Ha ema to lo gy

Def ici enc y o f h aema tini cs an d c lottin g agen ts

Metabo li sm

Hy pog lyca emi a, elec troly te defi cien c ies, mal nu tri tion

Ga stro intes tina l

Ga stritis , u lc eratio n , ma la bso rptio n, hep atitis , c irrh os is,

Ne uro mu scu la r

Myo path ies, neu ro path ies , d emen ti a

Psyc h iatric

Dep ress io n, an xie ti es, su ic ida l id eatio n, psyc ho s es

Orod enta l tiss ue s

To o th w ear, sa liva ry gl an d sw ell in g, aptho u s stom atitis

Fetus

Fetal alc oh o l syn dro me

system

system

pan crea ti ti s

Ha ema to lo gi cal sc reen in g in an al co ho l -depen den t pati ent ma y s ho w a mac roc ytos is i n th e

prese nc e o f no rma l fo la te level s. Th e reas on fo r thi s i s n ot cle ar. Li ver fu nc tio n tes ts ma y very well be no rmal as th es e tests sh ow grea t in divid ua l va ri atio n.

It s ho ul d be appre cia ted tha t alc oh o l an d dru g a ddic ti on s are w ell reco gn iz ed i n th e med ica l o r den ta l pro fes sio n al an d thi s i nc lud es th e s tu den t po pu latio n (see Pro je ct 1 at th e e nd of th is ch apter).


Ho w wo ul d yo u in vestig ate thi s la dy's sym ptoms and w ha t are th e po ss ibl e cau s es?

A fu ll his tory s ho u ld b e taken . It is impo rtan t to kn ow if th e s ymptom s w ere a tten u ated o r abs ent w he n th e den tures were left o ut. An in trao ral e xami na ti on is requ ired to i den ti fy if

th ere i s a ny muc o sal abn o rmali ty pres ent. The patient rel ates th e o ns et o f sym ptoms to th e

provi sio n of ne w rep lac emen t co mpl ete den tures . The den tu res, th erefo re, sh ou ld be a ss ess ed for l ac k of freew ay s pac e. The ne w de ntu re s ma y h ave been ma de w ith an un ac cep ta ble

inc reas e in th e o ccl us al vertica l di men sio n . A lternativel y, th e den tu re teeth ma y no t ha ve

been pla ced in the neu tral z o ne . It is alw ays h elpfu l to exa min e th e pa ti ent's ol d de nture s (if sh e stil l h as th em) to co mp are th e den tu re d esig ns in rela tion to a pa tien t's c omp lai nts.

Table 17.10 Characteristics of pain in dental conditions Pu lpitis Vari abl e pres en ta ti on fro m mi ld to sev ere pa in . Reversi ble pul pitis may pres ent a s

sh arp pa in wi th h ot, co ld, a nd sw eet stim uli (os mo ti c o r the rmal ch an ges in

press ure ). P ain ma y be lo cal iz ed to affe cted to oth or adjac en t te eth , bu t can be re ferred to a ffec ted si de o f fa ce (u n likel y to c ro ss midl ine ).

Def ective resto ratio ns , c arie s, o r oc clu sa l trau ma are p os sib le c au ses . An alg esi cs can be hel pfu l bu t no t an tibi otic s. Chro n ic p ul pitis ca n als o prese nt as a poo rly loc al ize d du ll ach e Cra cked too th Vag ue or in co ns isten t hi story of pain du rin g ea ting; may be s ens itive (sh arp pai n) to th erma l stim ul i. There may be a n i ntermi tten t du ll a ch e. Pa in is n o t alw ays eli cited by the sam e sti mul i, an d it ma y be diffi cu lt to e lic it pa in cli nic al ly.

Pai n u su al ly l oc ali zed bu t can be referred (si mila r man n er to p ul pitis ). A ffec te d too th is us ua lly re store d, crac k(s) m ay be vis ible bu t a re n ot n ec ess aril y path ol ogi ca l.

Re lief is obta ine d w hen o cc lus al fo rces no lo n ger stress the frac tu re l ine (e. g. piec e of c us p fra ctu re s off )

Perira dic ul ar perio do ntitis Th ro bbin g p ain , mild to s evere. P ain sh o uld be w ell lo ca liz ed. Th e fo llo wi ng may be prese nt: a sin us , a lo c ali zed alve ol ar s wel lin g, a pe rio don tal po cket.

An ti bio ti cs an d a na lges ics ma y ha ve b een effec tive i n redu cin g o r el imin atin g th e pain . The affe cted to oth is us ua lly resto red o r ca rio us ; altern ative ly, th ere is a

his tory o f trau ma . Relie f sh o uld be o btai ned wh en the so urc e o f in fec ti on is remo ved (e .g . a ne cro ti c pu lp ) Peric o ro n itis Du ll ach e w ith ac ute ex ace rbatio ns . Pai n ten ds to be we ll l oc ali ze d. Mo st co mmo nl y, th e ma nd ibu lar th ird m ol ar is in vo lved. P ain ca n radia te acro s s the affe cted s ide of th e jaw . Pa in wo rse o n eatin g. V ery rarely is slee p di sturb ed.

Th e p ain fu l too th is u s ual ly p artly erup te d o r ha s a n o perc ul um with an as so ci ated

perio do ntal stagn atio n area . An ti bio tics an d a na lges ics ma y h ave b een he lpfu l. Relie f is o btai ned wh en : (1) th e to oth is ta ken ou t o f oc cl us io n (preven ting trau ma to the ope rcu lum ); (2) th e o perc ul um i s re move d; (3) th e too th is e xtra cted; or (4) the to oth eru pts ful ly

It is pos si ble th at the patie nt h as devel op ed pa rafu nc tio na l h abits , s uc h as rubb in g the to n gue aga ins t th e d entu re, sin ce the den tu res were fitted. Pa ti ents are o ften un aw are of th is . If th e den tu re to oth pos itio n is th ou gh t to be sati sfac tory, a so ft oc cl us al s pli nt ma y be hel pfu l in β€ breaki ng β€™ the ha bit.

All ergy to den tu re -bas e ma teri als is exc eptio na lly rare, an d a mu co sa l pro ble m is mo re lik ely

to be a res ul t of a n exce ss of un cu red f ree mo n ome r. If th is is th e c ase , th e sy mptom s s ho ul d re so lve i f the den tu res are n o t wo rn. In a ddi ti on , th e mo n om er sh o uld cea se to le ac h o ut o f th e den tu re w ith ti me. So me p atien ts s eem u n abl e to to lera te den tures an d a fu ll den tu re

his tory i s es se ntia l. It is n ece ssa ry to enq uire if th e pa ti ent h as su cc ess ful ly w orn den ture s befo re (in cl udi ng partia l de nture s).

If th e pa tien t's de nture s a re fo un d to be a dequ ate a nd the ora l mu co sa app ears no rmal , th en

th e pa tien t sh ou ld b e fu lly inv estiga ted fo r B MS as ou tlin ed i n Ta ble 17. 5. Ta ble 17. 4 lis ts the un derlyi ng co nd itio ns th at c an ca us e B MS .


Dis cu ss h ow yo u m igh t diffe re ntia te betwe en pai n o f de ntal ori gin an d a typ ica l odo n ta lgi a in thi s p atien t.

Wh en a to oth ha s h ad man y de ntal in te rventi on s o r has a la rge resto ratio n p resen t, i t is often all to o eas y to fin d an im perfec tion pre sen t th at ma y be th o ugh t to b e res pon s ible for th e

sympto ms. Wh en a la rge f illi ng or a c row n i s p re sent, i t is re aso n abl e to co ns ider th at a pul p co uld be i nfl ame d or i nfe cted o r tha t a pa in fu l ro ot-fill ed too th co ul d requ ire re -trea tmen t o r perira dicu la r su rgery. In cli nic al prac ti ce, the refo re, it ca n o ften be diffi cu lt to e xclu de a den ta l c au se i n a too th w ith ch ron ic

sympto ms. It i s a lwa ys e asi er to make a di agn os is of atypic al od on talgi a (a vari ant o f atypic al

P. 215

faci al pai n) w he n a su cc ess io n o f i nterven tio ns h ave b een ca rri ed o u t with ou t su cc ess . It i s

th erefo re e sse ntia l tha t detai led d enta l an d p ain hi stori es a re taken . A p atien t wi th atypi ca l odo n ta lgi a ma y ha ve a hi sto ry of repea te d res to ratio n s, fo llo wed by ro ot trea tm ent, cro wn plac emen t, an d an api cec to my. It is no t un us ua l to fin d o ut th at ad ja cen t teeth ha ve h ad

simi lar tre atmen ts fo r ch ron ic pa in. Tee th ma y al so ha ve b een extracted . Th e c lin ic ian nee ds to es ta bli sh if a ny trea tmen t w as ben efi cia l. The effe ct of an y medi catio n s th at ha ve

been us ed for th e pa in sh ou ld be a ss ess ed. In a typ ica l o do nta lgia it is un li kely th at s impl e

ana lge sic s a nd an ti bio tics wi ll h ave reli eved th e pa in . Des pite th is, pa ti en ts ma y still h ave a hig h d ail y in ta ke o f a na lges ics .

Table 17.11 Trigeminal neuralgia and atypical facial pain: differential diagnosis Trigemin al n eur algia

Aty pical facial pain

Age (years ) 50+

30 β€“60

Pai n i nten si ty Re marka bly s evere

In tens ity ma y vary from mil d to severe , b ut u su all y a

β € bac kgrou n d β€™, lo w -inten si ty pai n

is p resen t Pai n des cripto rs Stabb ing , s ha rp, lan ci na ti ng pai n o f a few sec on ds dura tion . Pa roxys ms may f ol low

Du ll, thro bbi ng , g na win g, na ggin g, pul lin g, oc ca sio n all y sh arp

P. 216

in qui ck s uc ce ssi on

Flo rid a nd emo tive adjec tives are u se d Des cri pto rs may b e in co ns isten t

Lo ca ti on /dis tribu ti on /radi atio n of pai n Man dib le o r ma xill a

Maxi lla is th e m os t c o mmo n s ite

We ll l oc ali zed

No t al way s w ell lo cal iz ed a nd site m ay

Uni latera l

Uni - or bila teral

Fo llo ws an ato mic al dis tri butio n of on e

Do es no t us ua lly fol lo w a na to mic al

(o c cas io na lly more th an o ne divi sio n is

Can be bi latera l a nd can c ros s mi dlin e

sen so ry di visi on o f the tri gemin al n erve invo lve d)

ch an ge

distrib utio n of nerve s

Can radi ate o ver wh ol e o f fac e

Du ratio n o f pai n Brief β€” us ua lly o n ly sec on ds

Con ti nu o us

Tri gger z o nes fo r pa in Ye s, we ll d efin ed

No t us u ally prese nt

Perio di city o f pai n In termittent

Con ti nu o us or i ntermi tten t

Prec ipita ti ng f acto rs Mo ving or to u ch ing a trig ger z on e. Mi ld stimu li m ay prov oke an attack

Stress , life even ts

Re lievi ng fac to rs Carbam az epin e β€”re spo n se to tre atmen t

An ti depres sa nts β €”re spo n se va riab le

Perip hera l n erve b lo ck w ith l oc al

often fa ils to rel ieve pai n

us ua lly g oo d

ana es th etic in je ctio n tem po ra rily relie ves pain

Comm on ly u se d an alg esic s are n ot

Lo ca l a na esth eti c in je ctio n i nto area

An alge sic s a re oc ca sio n ally so me h elp , but n ot a lwa ys

Pai n m ay n ot b e prob lema tic d urin g

ben efic ial

slee p

Pai n b eha vio ur A β€ froz en fac eβ€™ ap peara nc e

Vari abl e prese ntatio n

if the as so cia ted fa cia l mo vem ents ca us e

e.g . to uc hi ng or pic kin g the affe cted

Spee ch an d fa cia l a ppea ran ce are altered pain

Avo ids to u ch in g trigge r zo ne

Patie nt ma y h ave d evelo ped ha bits, site

Hi sto ry Other c hro n ic pai n s ynd rome s n ot u su al ly prese nt

Frequ en tl y a lo ngs tan din g h isto ry o f pain

Frequ en t resto rative a nd su rgic al interve ntio n is c om mo n

Hi gh preva len ce of c hro n ic ill hea lth, espe cia lly othe r ch ron ic pai n syn drom es

Th e n atu re o f the pai n i s a lso im porta nt. Pa ti ents with atypi cal od on ta lgi a/fa cia l pa in may als o give a h isto ry of pai n a nd al tere d se ns atio ns th at a re vari abl e. It is n ot u nu su al fo r a pa tien t with atypic al od on talgi a to ha ve a defi nite v iew of wh at sta rted the pain . De ntal ca us es for

co ns idera ti on in th e d iffere ntia l di agn os is are g iven in Ta ble 17. 10 β€”co mpare th es e w ith th e featu res o f a typ ica l fa ci al p ain li sted i n Ta ble 17. 2.


Wh at in fo rmatio n from th e p ain h isto ry an d c lin ical e xami na ti on w ou ld yo u us e to diffe re ntia te betwe en th es e two co n ditio ns ?

To di stin gu ish be tw een trigem ina l n eu ralg ia a nd atypic al fac ial pai n th e cli ni cia n w ill n eed to lis ten c arefu ll y to th e pa in his tory a nd no tic e the patie nt's d emea no u r an d ma nn eris ms. Th e

desc ripto rs th at the p atien t us es to des cribe the pain a re of te n ve ry imp ortan t. A si mple pai n

que stio nn aire su ch as th e M cGil l pa in qu estio nn ai reca n b e very hel pfu l. Ta ble 17. 11, w hi lst n ot exha us tive, is of valu e i n d iffere ntia ti ng betwee n tri gemi na l n eura lgi a an d a typic al f aci al pain .

Projects 1.

Th e B ritis h De ntal As so cia tion h ave prod uc ed a publi catio n en ti tl ed Dru gs a nd alc oh o l :

addi ctio n i n th e den ta l pro fes si on , prob lems an d s ol utio n s. Wh at is the ro le of th e

β€ den ti sts' h ea lth s up port p ro gra mme β€™ in rela tio n to den tists with al co ho l an d

addi ctio n p rob lems ? 2.

Th e Ho s pital An xiety an d Depres si on (HAD) s cal e can be u sef ul wh en as ses sin g a pa ti ent

with ch ron ic pai n. Obta in a c opy of th is ps ych ome tri c qu es ti on n aire an d fin d o u t h o w to evalu ate a c omp leted f orm.

Au th o rs : Title:



Co py rig ht Β © 2003 Ox fo rd U ni ve rsi ty P re ss > T ab le o f Co nt e nt s > 1 8 - Dis or d e r s of t he te e th and b on e

18 Disorders of the teeth and bone Problem cases Case 18.1

A 14-yea r-ol d bo y is u n h ap py w ith th e ap pe a ran c e o f h is f ro n t tee th . He do e s no t lik e th e ir

β€ blo tc h y a p pea ra n ce β€™. Cl in ic a l e xa mi n ati o n rev ea ls th e pre se n ce o f sm a ll w h ite fle c ks on th e e na m el su rf ac e s of m o st o f th e te eth . Th e e na m el s ur fa ce s are n o t p itted . Th e tee th ha ve n o re sto ra tio n s p res en t a n d ar e c a rie s- free . Th e di sc o lo ra tio n is in tri ns i c. Q1

Wh at a re th e di ffe ren ti al d ia gn o s es fo r thi s bo y's de n ta l co n d itio n ?

Q2

Wh at a d di ti o n al in f or ma tio n d o yo u n e ed to h el p yo u ma ke a d efi n iti ve di ag n os i s?

Case 18.2

An 18- yea r-ol d gi rl p re se n ts to yo u r su rg er y b ec a use sh e i s un h a pp y a b ou t th e a pp ea ra nc e o f he r f ro nt tee th an d co m pl a in s th at h e r f ro nt tee th are g ettin g th in n er . Sh e re po rts th a t h er

te eth ar e s o m etim es s e ns i ti ve to h o t a n d c o ld . E xa mi n ati on rev ea ls e xpo s ed d en tin e o n th e

pa la tal s u rfa ce s o f th e ma xi ll ary i nc is o rs a n d c a n in es . Th e re is a ls o ex po s ed de n tin e o n th e oc c lu s al s u rfa ce s o f th e m axi ll ar y fi rs t pe rm an e nt mo l ar tee th. Q1

Wh at a re th e po s s ibl e ca u s es o f to o th w e ar in th is c a se ?

Q2

Wh at c o n di tio n w o u ld yo u s u s pe ct i f th e pa tie n t al s o pre s en ted w ith s w o ll en p ar oti d gla n ds a n d ap pe a red to h a ve ge n era li z ed s kel eta l mus cl e wa s tag e?

Disorders of the teeth

Th e te eth a re po o r in di ca to rs o f ge ne ra li ze d di se ase. Fo l lo w in g th ei r c al c ifi ca tio n , m eta bo l ic pro c es se s h av e littl e e ff ec t o n th e str uc tu re o f the tee th. Stru c tu ral a bn o rm al iti es a lm o st

in va ria bl y refl ec t ch a n ge s o cc u rri ng i n th e p er io d du ri ng w h ic h th e tee th we re be in g fo rm ed . Ap ar t fro m s tru ctu ra l va ria tio n , a bn o rm al itie s in th e n u mb ers , s iz e , an d s h ap e o f th e tee th oc c as io n a ll y o c c ur , in c o n ju nc tio n wi th ab n o rma l itie s o f th e bo n e s or o f th e s ki n a nd o th er epi de rm al ly de riv ed s tr uc tu res . Wh e n n u mb ers o f mi ssi n g tee th, su p ern u me ra ry te eth , o r

ab n or ma ll y s h ap ed te eth a re o bs erv ed , it i s as w el l to c o n si de r th e po s s ibi li ty o f so m e su c h co m pl ex as s o ci ati o n. It mu s t al w ay s be re me mb ere d tha t te eth m is s in g fro m th e a rc h m ay

eith e r b e c o n ge n ita lly ab s en t, h av e be en e xtra cte d, o r ma y be u n eru p te d. T h e m a jo r po s teru pti o n ch a n ge s th at o c c u r a re th e l os s es o f to oth su b sta n ce c au s ed b y c a rie s, attri tio n ,

ero s io n , a nd a br as io n . Th i s ch a pte r w il l dis c u ss s ome o f th e di so rd er s me nti o n ed. Th e l is t i s no t, h o w ev er, e xh a us tiv e an d th e r ea de r i s ref erre d to a tex tb o o k o f o ra l p ath o l og y. Tab le

18. 1 h ig hl ig h ts so m e d en ta l an o m al ie s th a t a re as s oci ate d wi th ge n eti c or s ys te m ic di se a se .

Table 18.1 Dental anomalies and associated diseases

Den tal an o m aly

Ass oc iated s ys tem ic/gen etic d iseas e

Di so rd er s of to o th de ve lo pm en t An o d on ti a, o li go do n ti a

Hyp o hi dro ti c ec to de rma l dys p la si a,

Su p ern u m era ry tee th

Cle id o cra n ia l dys p la si a, Gar dn er' s

Ma c ro do n tia , m ic ro do n tia ,

Dow n 's sy n dro m e, Kl in e fel ter' s

Den tin o ge n es is i mp erf ec ta,

Os teo g en es is i mp er fec ta , co n g en ita l

ta u ro do n ti sm , di la c era tio n , ge m in ati o n

am el o ge ne s is im pe rfe cta , en a me l

hy po p la si a, d en ti ne d ys pl as ia , fl u o ro si s

Dow n 's s yn d ro me

syn d ro me

syn d ro me

syp h il is , ric ke ts, h ypo p h o sp h ata s ia

Di so rd er s o f e ru pti o n an d s h ed din g Pre ma tu re eru p tio n

Hyp erth yro i di sm

Dela ye d eru p tio n

Creti n is m, r ic kets , cl ei do c ra n ia l dys pl a si a, h e red ita ry gin g iv al

fib ro ma to si s , Do wn 's sy n dro m e Im p ac tio n o f tee th

Cle id o cra n ia l dy sp la s ia

Hypodontia

Hyp o do n tia (o l igo d o n tia ), th e c o n ge n ita l ab s en c e o f tee th , rep res en te d by the l o ss o f o ne o r tw o te eth w i th n o ap pa re n t as s o ci ate d ab n o rma li tie s is n o t u n co m mo n . Th e m o st co m mo n te eth to be mi s si ng ar e th e la s t in ea ch s e rie s. M os t s ur vey s, h o w ev er, sh o w th at o ne o r mo re th ird mo l ar s are m is si n g in a pp ro xi ma tel y o n e -qu a rter o f th e po pu l ati o n. A stu d y

ca rri ed o u t in a n E n gl is h p op u la tio n a n d exc l ud in g th e th i rd mo l ars h a s s ho w n th at th e tee th mo s t li ke ly to be m is si n g a re th e lo w er se co n d pr emo la rs

(40.9 pe r ce n t) fo l lo w ed by th e up pe r l ate ra l i n ci so rs ( 23.5 pe r c en t) a n d by th e u p pe r

se co n d pre m ol a rs (20. 9 pe r c en t). T he p atte rn o f miss in g tee th do e s, ho w e ver , va ry fro m po pu l ati o n to po p u la tio n . A c om m o n fin d in g in hy po don ti a is th e pre s en c e o f s ma ll a nd

co n ic a ll y s h ap ed tee th re pl ac in g n o rma l u n its o f the de n titio n ( Fi g. 18. 1). Hyp o do n tia i n th e pri ma ry de nti tio n is a re la tiv ely ra re o cc u rre nc e .

P. 220

Fig. 1 8 .1 A c on i ca ll y sh a pe d to oth in th e po s iti on of a n u pper la tera l in c is o r.

Th e c o n ge n ita l ab s en c e o f tee th a ss o ci ate d w ith a bno rm al itie s o f th e bo n e o r e c to d erm a l ap pe nd a ge s is re la tive ly ra re. T he d ys pl as ia i n vo lved ma y be a ttr ib ute d to ec to de rma l ly

der ive d stru c tu res o r to mo re c o mp le x s yn d ro me s in w h ic h th e re a re bo th d erm a l a n d bo n y ab n or ma li ties .

Variation in eruption

Th e w i de n o rma l ra ng e ma ke s it di ffi cu l t to s pe ci fy ac c u rate ly th e d a te s o f e ru pti o n o f e ith er th e d ec id uo u s o r th e p erm a n en t te eth . S eve ra l f ac tors h av e be en f ou n d to a ffe ct the d ate o f eru pti o n , in c lu di n g r ac ia l o rig in a n d su c h u n li kel y i n flu e n ce s as th e so c io - ec o no m ic

en vi ro nm e nt. In g en e ra l, e arl ie r b od il y d ev el o pm en t i s ref lec ted in e a rly eru p tio n o f the te eth .

Ma rke dl y p rem a tur e e ru pti o n o f th e p erm a n en t te eth is v ery ra re. It h as b ee n su g ge ste d tha t it m ay o c cu r in ca s es o f h ype rs ec reti o n o f th o s e horm o n es th a t in fl u en c e dev el o pm en t. It i s so m ew h at mo re c o mm o n to n o te pre ma tu re eru p ti o n o f th e de c id uo u s te eth . Fr eq ue n tly, no sys te mi c fa cto r i s fo u n d to ac c o un t fo r th is . It s ho u ld pe rh ap s be m en tio n e d th a t tee th

pre se n t at bi rth in a f ew c h il dre n (th e n eo n ata l tee th ) do no t r ep res en t p re ma tu re e ru pti o n . Th es e are s u pe rn um e rary tee th a n d p ar t o f a se pa ra te pre de ci du o u s de n titio n .

Dela ye d eru p tio n o f th e de ci du o u s tee th m ay o cc u r in en d o cri n e def ic ie n cy s tate s an d it ha s

bee n s h o wn th at, i n Do w n' s sy nd ro m e, n o t o n ly a re th e e ru ptio n d a te s s om e wh a t re tard ed i n

gen e ra l, b ut al so the re is o fte n a n u nu s u a l s eq u en ce of e ru ptio n . It i s ve ry di ffi cu l t to a sc ri be a c h a rac ter is tic d en tal p ic tu re to ma n y o f th e e n doc rin e ab n o rma l itie s si n ce , in a n u m be r o f ca se s , va ryi ng a n d co n tra di cto ry ef fec ts h av e b ee n d es cri be d. In ma n y s u c h pa tie nts th e

mo s t o bv io u s ab n o rma li ty i s di sp ro po rti o n in th e s iz es o f th e te eth a n d the ja w s, th i s in tu rn lea d in g to gro s s ir reg u la rity o f th e o cc lu s io n . Th e p res en c e o f s u pe rn u me rar y o r u n e ru pte d

te eth m a y d el ay o r p rev en t e ru pti o n. C le id oc ra n ia l dys p la si a, a l th o u g h ra re, i s a w ell k no w n syn d ro me i n w h ic h th ere a re mu lti pl e a d di ti o n al teeth a ss o ci a te d w ith u n eru p ted tee th ( Fi g. 18. 2).

Fig. 1 8 .2 M ul tip le u n eru pte d tee th in c le id o cra n ia l dy sp la s ia .

Variation in the size of teeth

Th e s iz e o f the te eth o f a n y i n di vid u al i s de te rm ined l arg el y b y in h eri ted fa c tor s. E xtre m e

var ia tio n in s i ze , ei th er i n th e di rec tio n o f sm a ll tee th (m ic ro do n tis m) o r i n th e di rec tio n o f la rge tee th (m ac ro d on ti sm ) m a y b e ac c om p an ie d by n o o th er gro w th ab n o rma l ity. Co n vers e ly, e n do c rin e g ro wth d is o rd ers l ea di ng to gig an ti sm o r dw a rfi sm m a y b e

ac co m pa n ie d by n o c or res po n d in g va ria tio n i n to o th si z e. Th e si z e of th e teeth al on e b ea rs n o rel ati on s h ip to m eta bo l ic fa c tor s in th e va st ma jo ri ty o f in s ta n c es .

Non-carious tooth surface loss

Attri tio n , ero s io n , a n d ab ras i on are c o mm o n ly se en in d en ta l p ati en ts . Th e cl in ic a l

ap pe ara n c e o f th e te eth m a y b e su g ge sti ve o f a s pe cifi c ca u s e f o r to o th w ea r b u t ca u tio n

sh o u ld b e e xe rci se d in th e id en tif ic ati o n o f a s in gle a etio l o gi ca l ag en t. To o th w ea r is u s ua ll y a mu l tifa c tor ia l p ro c es s. T h e p res e nc e o f a ttriti o n o r e ro si o n ma y be o f in tere s t to th e o r al ph ys ic ia n . We ar fa ce ts in o p po s in g a rch e s tha t m a tch i n pa ra fu n cti o n al po s iti o ns o f th e

ma n di ble a re in d ic ati ve o f b ru xi sm . Th is m a y b e si gn i fic a n t in th e a etio l o gy o f a h e ad ac h e th a t i s pre se n t o n w ak in g o r i n my o fa ci al p ai n .

In an y in d ivi du a l, to o th w ea r us u a lly h a s a mu lti facto ri al ae tio l og y. Ero s io n is th e l o ss o f de n tal h a rd tis su e b y a c h emic a l p ro c es s tha t d o es n o t i nv o lve b ac teri a (Fi g. 18. 3). Th e ca u se s o f e ro s io n a re li ste d i n Tab le 18. 2. Th e a c tive s u bs tan c es m a y b e

en do g en o u s o r e xo ge n o us . A c idi c fo o ds tu ffs a n d be verag es a re c om m o nl y i mp li ca ted . Th e se in cl u de c itru s fru i t a nd ju ic e, a n d ma n y s o ft dri nks in c lu di n g fru it sq u as h es a n d co rd ia ls ,

mi xer- ty pe dri n ks , an d m an y o th ers . A h ig h ly si gn i fic a nt ca u s e o f ero s io n is th e se lf -in du c ed

vo mi tin g o f b u lim ia n e rvo s a, wh i ch m a y r es u lt i n w id es pre ad l o ss o f en a me l, p a rtic u la rly fro m th e p al ata l su rf ac es o f th e

up pe r a n teri o r tee th a n d th e cu s p s o f p erm an e n t mo lars . P a ro tid en l arg em en t a n d a n o n-

P. 221

sp ec if ic m uc o s itis m a y al s o be s ee n in th e bu l im ic p ati en t. P ati en ts w ith g as tri c co n di tio n s th a t res u lt in c h ro n ic a ci d reg u rgi ta tio n wi ll pr esen t w ith th i s type o f to oth w ea r.

Fig. 1 8 .3 A ci d e ro s io n w ith ce rvi c al le si o n s an d lo s s o f n o rm a l e n am el co n to u r.

Table 18.2 Causes of dental erosion Ex trin s ic a ci ds Be ve rag es (e . g. f res h fr ui t ju ic e s, c o rdi a ls , ca rb ona ted dri n ks , w in e, f ru it te as ) Fo o ds ( e. g. c i tr us f ru it, p ic kle d fo o ds )

In d u stri al p ro ce ss e s (e. g . wi n e ta s tin g, b a tte ry man u fa ctu re , me ta l p la tin g ) In tri n si c ac i ds Gas tro -oe s op h ag ea l ref lu x di se a se (GO RD) * Ea tin g di s or de rβ€” bu li mi a n erv os a Mo rn i ng s ic kn e ss i n pr egn a n c y

Ru mi na tio n (vo lu n ta ry reg u rgi tati o n) *T h is m ay be s ec o n da ry to o the r co n d itio n s , e. g . alc oh o l is m.

An o re xia n e rvo s a ca n b e as s o ci ate d wi th de n tal e ro si o n be ca u s e o f a h ig h in ta ke o f

Bu l im ia n er vo sa i s as s o ci ate d wi th de n tal ero s io n bec a u se tee th a re exp o se d to

ac id d rin ks a n d fru i t

in trin s ic (g as tri c) ac i d

Discoloration of the teeth

Wid es pre a d c o lo ra tio n o f th e te eth o c c ur s in a fe w di se a se s in w h ic h a bn o rm al b lo o d

pig m en ts ci rc ul a te . O f th e se , in fa n til e ja un d ic e is th e mo s t c o mm o n. In thi s co n d itio n th e dec i du o u s teeth ma y b e co l o u red bl u e -gre en d u e to th e la yi n g do w n o f a p ig me n t in th e

im me di ate po s tn ata l de nti n e z o n e. A le ss c o mm o n , and n o w vi rtu al ly el im in a ted, ca u se o f

to o th d is c ol o ra ti o n is h a em o ly ti c a na em i a o f th e new bo rn c a u se d by rh es u s in c o mp ati bi li ty .

Fo ll o w in g the h a em o ly si s, p ig me n ts ma y b e de po s ite d i n th e sk in an d i n th e te eth , w h ic h m ay ta ke o n c o lo ra tio n th a t va rie s fro m g rey to gre en - gre y a n d to bro w n . Co l or ati on o f th e tee th

al so o c c u rs in s o me o th er co n s id era b ly rar er s i tu a tio n s in wh i ch ab n or ma l pi gm en ts c irc u la te, fo r e xa mp le , in p o rph y ria . Ho w ev er, a f ar mo re c o mmo n c au s e o f to o th di s co l or ati on is

te tra cy cl in e sta in i n g. It n e ed h ard ly b e s a id , h ow ever , tha t th e n u mb er o f y ou n g pa tie n ts

wi th tetra c yc lin e s tai n in g is ra pi dl y re du c in g w ith th e a lm os t to ta l w ith dra w a l o f th is g ro up o f an tib io tic s fr om β € at -ris kβ €™ gro u p s.

Disturbances of the structure of enamel and dentine

Wh en th e n o rma l se qu e nc e o f en a me l ma trix fo rm a ti o n a nd c a lc ifi c ati on is di s tu rb ed , a s eri es of ab n o rma l itie s ma y be pr od u ce d. Th es e ma y b e di sti ng u is h ed a s h ypo p la s ia , w he n th e

qu a nti ty o f en a me l is re du c ed , o r a s hy po c a lc ifi ca tio n , i n w h ic h th e d eg ree o f c a lc ifi c ati on is un s a ti s fa cto ry. Th e tw o c o n di tio n s ma y be c om b in ed a n d va rio u s c li ni c al c on d iti on s m a y b e dif fe ren tia ted . A p ara ll el ra n ge o f di stu rb an c es i n th e fo rm ati on of d en tin e m ay al s o o cc u r,

bu t th es e ar e n o t s o w el l d iff ere n tia ted a s in e na mel . Th e us e o f th e ter m β€ hy po p la si a β €™

ca n be co n f us i ng a n d r eq ui res e xp la n ati o n, si nc e it is u s ed bo th i n th e s tri ctl y sc i en tif ic s en s e as m en tio n e d a b ov e an d a ls o in a c l in ic a l s en s e to de sc ri be a ge n era li z ed di stu rb an c e o f en am e l s tru c tu re c a us e d b y s o m e f o rm o f s ys tem ic distu rb an c e.

Th is g ro up o f co n d itio n s c an be c on v en ie n tly di vid ed a s fo l lo w s: 1.

en am e l o r de nti n e d ev el o pm en ta l d ef ec ts res u ltin g fr om l o ca li z ed di stu rb an c es ;

2.

en am e l o r de nti n e d ev el o pm en ta l de fe cts re su l tin g fro m ge n era li z ed di s tu rb a nc e s;

3.

gen e tic al ly de term in e d de fec ts o f e n am el f or ma tion o r ge ne tic al ly de term in e d d ef ec ts o f

den ti n e f o rma tio n .

Defects due to local causes

Wh en a n in f ec tio n o c cu rs in a s so c ia tio n w i th a de cidu o u s to o th the p erm a ne n t s u cc es s io n al to o th de ve lo pi n g b el o w it ma y u n d erg o a di s tu rb a nc e o f d ev el op m en t. In s u ch c a se s th e

en am e l i s u su a ll y d is to rted a nd p itted . Th i s co n di tio n i s ea si ly re co g ni z ed b y its re stri c ti o n to a s i ng le s u cc es s io n a l to o th .

Defects due to generalized causes

Wid es pre a d in fe c ti o n s o r n u tritio n a l di stu rb an c es du rin g th e to o th e na m el de ve lo p me nt

per io d ma y ad ve rse ly a ffe ct th e l ayi n g do w n o f e n ame l. S u ch co n di tio n s a ffe ct al l the te eth

dev el o pin g a t th e ti m e a n d it i s o ften p o ss i bl e to ti me a cc u ra tel y th e o n s et o f th e dis tu rb an c e by the p o si tio n o f th e d efe c t o n th e te eth . In ge n era l, th e de fi ci en t e n am el fo rm s a ba n d

aro u n d the to o th c o rres p on d in g to th e pe rio d o f distu rb an c e (Fi g. 18. 4 ). Th e ba n d m a y b e wi de o r n arr ow an d , in s o me c irc u m sta n ce s , the b an din g ma y be in c o mp le te. It h a s be en

su g ges te d th a t th e en a me l o p ac iti es s ee n in so m e patie nts m a y re pre s en t a m in o r fo rm o f th i s co n d itio n . U n le ss th e di stu rb an c e o f to o th fo rm a ti o n is u n u s ua l ly se ve re, th e tee th do no t s e em to b e u n d ul y su s ce pti bl e to atta ck by c ar ies . Hi sto lo g ic a lly , th e d en ti ne th a t dev el o pe d a t th e sa m e

P. 222

ti me a s th e a ff ec ted en a m el ma y sh o w s li gh t d ef ic ienc i es , bu t th i s do es n o t re s ul t i n cl in i ca l ab n or ma li ty.

Fig. 1 8 .4 E n am el h yp o pla s ia as a r es u lt o f a ge n era l dis tu rba n c e d u rin g de ve lo pm en t.

Pre n ata l sy ph i lis m a y p ro du c e de fec ts in to o th d ev el op me n t. Th e sp iro c h ae te ma y lo dg e in th e e na m el o rga n a n d in terf ere d ire ctl y w ith th e form a tio n o f th e e na m el . Th e ef fec ts a re

gen e ra lly c o nf in e d to th e a n te rio r pe rm an e nt teeth an d to th e firs t pe rm an e nt mo l ar. T he ty pi ca l mo l ar fo rm o f th e s yp hi li tic to o th is th e mu l be rry mo l ar in w h i ch th e s ha p e o f th e

to o th i s we ll e xpr es se d by its n a me ; th es e tee th are a ls o te rme d Mo o n 's m o la rs . Th e typi ca l

var ia tio n i n the a n teri o r tee th ta kes th e f o rm o f a c o n ic al , sc re w -dri ver sh a pe w ith a no tc h ed in ci s al ed ge c al le d a Hu tc hi n so n 's i n ci so r (s ee Ch ap ter 4).

If th e d ev el op m en t o f the te eth o c cu rs w h en la rge amo u n ts o f f lu o rid es a re in g es ted ,

mo ttli n g o f th e e n am el m ay o c cu r. T h e e ffe c t o f f luo ro si s ma y be rec o gn i z ed by th e pre se n ce of o pa q ue w h ite pa tc h es in the e n am el , o ften arra n ge d in a b a nd -lik e f o rma tio n . U n li ke the

te eth i n o th er fo rm s of h yp o pl as ia , th e tee th af fecte d b y f lu o ro s is a re su s c epti bl e to a bro w n dis c o lo ra tio n (Fi g. 18. 5 ) th at ma y res em bl e am el o gene s is im p erfe c ta . S im il ar id io p ath ic mo ttli n g m a y o c cu r in te eth o f pa tie n ts fro m n o n- flu o ri de ar ea s, b u t th is is r are .

Hyp o pla s ia o r h y po c al ci fi ca tio n o f th e e n am el ma y occ u r i n pa tie n ts su ff eri n g f ro m

gen e ra liz e d ec tod er ma l dis e as e (s uc h a s ep id erm o ly si s bu ll o sa ) or fro m d is tur ba n ce s o f

ca lc iu m m eta bo l is m su c h a s h ypo p a rath yr oi di sm . Th e se c o n di ti o n s cl ea rly re pre se n t th e f in a l

res u lt o f ab n o rma l to o th ge rm fo rm ati on an d a bn o rm al c al ci fi ca tio n , re sp ec tiv el y. M an y o the r dis e as es m a y p ro du c e de nta l ab n o rma li tie s of a s im il ar type . Th e se c h an g es ar e, h o w ev er,

rare . It i s un l ike ly th at th e g en er al iz ed d is ea s e pro c es s wi ll h av e g o n e u n re co g n iz ed b y th e ti me th a t th e to o th a bn o rm al ity h as b ec o m e e vi den t in th e m ajo ri ty o f th es e p a tien ts ( Fi g. 18. 6).

Fig. 1 8 .5 Fl u o ro si s.

Fig. 1 8 .6 Hyp o pl as ia i n a pa tie n t w ith d is tu rbe d c a lc iu m me tab o li sm .

It wi ll b e ev id en t th a t the d ia gn o s is o f the a bn o rma liti es o f too th s tru c tur e d es c rib ed ab o ve dep en d s la rge ly o n th e rec o gn i ti o n o f th e c li ni c al a pp ea ran c e o f th e tee th. Rad io g rap h y

ap art, th e re is ve ry littl e i n th e wa y o f s u pp le me nta ry tes ts o r i nv es tig ati o ns th a t w il l a d d to a c a ref u lly c ar rie d o u t c li n ic al e xa mi n atio n o f th e pa tie n t, an exa m in a ti o n th at sh o u ld

evi de n tl y in cl u de a c are fu l me di ca l an d fa m il y h is to ry. In virtu a ll y a ll c as e s la bo ra to ry te sts pro ve u n pro d uc tiv e.

Defects due to genetic causes AMELOGENESIS IMPERFECTA Am el o ge ne s is im p erfe c ta i s a h ere di tary de ve lo p me n ta l de fe ct o f en a m el. Th e c o n di tio n m ay sh o w a s ei th e r a h ypo p la s ia o f th e e na m el o r a s a hyp oc a lc if ic ati o n. If a n ea rl y p h as e o f en am e l fo rm ati o n is d is tu rbe d, th e am o u n t o f m a trix la id d ow n i s red u c ed, bu t th e

ca lc if ic ati o n is c o mp le te. We th u s h av e a th i n an d i rreg u la r l ay er o f h ar d en a me l. T hi s is th e hy po p la sti c type (Fi g. 18. 7 ). S eve re attri tio n m ay o c cu r ea rly in lif e. In th e h yp o ca lc if ie d ty pe

a l a te r sta g e o f en a me l fo rm ati on is d is tur bed a n d we h a ve a n o rma ll y th i ck la ye r o f po o rl y

ca lc if ie d e n am el . In th is c a se , th e w h o le o f th e e na m el is s o ft a n d ero d ed w ith l o ss o f m uc h

P. 223

en am e l b y a ttriti o n a nd e xpo s u re o f th e de n tin e (Fig. 18. 8). In bo th f o rms th e de ci du o u s a nd per ma n en t d en titi o n s ma y be a ffe cte d. T he d en tin e re tai n s i ts n o rma l stru c tu re in b o th ca se s .

Fig. 1 8 .7 Hyp o pl as tic en a me l. T he e n am el is i mp er fec t, b ut is h ard .

Fig. 1 8 .8 Hyp o c al ci fie d en a me l. T he e n am el is s o ft an d ero d ed .

DENTINOGENESIS IMPERFECTA In th i s co n d itio n th e re is fa il ur e o f de ve lo pm e nt of th e de n ti n e wi th n o rma l en a me l

dev el o pm en t. It i s a h ere di tary c o nd iti o n an d a ffe cts b o th th e d ec id u o u s an d pe rm an e nt

den ti tio n s. Th e tee th eru p t u su a ll y w i th n or ma l mo rp h ol o gy , bu t h a ve a gre y or br ow n c o lo u r. Th ey sh o w a ra th er iri de sc en t co l or ati on , w h ic h l eads to th e ter m β € he red ita ry o pa le sc e nt

den ti n eβ€™. T he p u lp ch a m be rs are o fte n re du c ed in si ze a n d ma y b e o bl iter ate d. A lth o u gh th e e na m el is o f n o rma l stru c tu re, i t re ad il y b rea ks a w ay , le av in g th e d en tin e e xpo s ed . Oc ca s io n a lly , th is c o nd iti o n o cc u rs a s pa rt o f a gene ra li ze d co n d itio n o f o s te o ge n es is

im per fec ta in wh i ch th e i mp erf ec t c al c ifi ca tio n o f th e bo n es l ea ds to f req u en t f rac tu res . O ften in su c h c a se s the re is a d efi c ien c y in th e sc l era of the e ye le ad in g to a bl u e co l o rati o n .

Disorders of bone

Dis ea se s o f b o n e h a ve tra di tio n al ly be en d iv id ed into th re e g ro u ps : ge n etic , i nf la mm a to ry, an d me tab o li c. In rea li ty, th is c la s si fic a tio n i s rath e r si m pli s ti c . Fo r exa m pl e, th e

cl as s ifi ca tio n o f P ag et's d is ea s e is pr o ble m atic beca u s e th e a etio l o gy o f th is c o n di tio n i s un c erta in . N o ne th ele s s, th e cl a ss if ic ati o n rem a in s a u se fu l o n e f o r m an y co n d itio n s .

Rel ati vel y f ew di so rd er s of b on e p res en t w i th an y fre qu e nc y fo r i n ve sti ga tio n in the o ra l

me dic i ne c li n ic . Fi br ou s d ys pl as ia a n d Pa g et's d is ea s e a re s een o nl y o cc a si o n al ly. H o we ve r,

so m e g en e tic al ly de term in e d dis e as es h a ve a n im pa c t o n de n tis try i n ge n era l, bec a u se th ey are p art of g en er al iz ed s yn dr o me s in vo lv in g bo n e a nd e pi de rma l ap pe n da ge s (in c lu d in g

te eth ). In th e se c o mp le x s yn d ro me s th e pre do m in a n t o ra l c o n di tio n is v ar ia tio n in the s iz e , nu m be r, m o rph o lo g y, a n d, so m eti me s , stru c tu re of the teeth . C le ido c ra n ia l d ys pl a si a an d os teo g en e si s im pe rfe cta a re ex am pl es o f su c h s yn d rome s.

Table 18.3 Blood chemistry in diseases of bone Levels in blo o d o f Calciu m

Ph o sp ha te

Alkalin e

ph o sph at ase

No rma l

2. 2 β€“ 2. 7

0. 8 β€“ 1. 4

Va ria b le β €

Pa ge t's di s ea se

N

N

++

Mo n o s to ti c fib ro u s dy sp la si a

N

N

N

Po l yo sto ti c fib ro u s dys p la si a

+

N

+

Pri ma ry h yp erp ara th yro i di sm

+

N/ β€“

+

wi th bo n e le si o n s

mm o l/l

mm o l/l

* N, No rm a l; + , a mo d era te ris e β €™ ++ , a m a rked ri se ; β €“, a m o de ra te f al l. β€

Al ka li ne p h os p ha ta se va lu e s: n o rma l le ve ls fo r the a ge g ro up s h o u ld be

dete rm in ed fr om th e s pe ci fic l ab o ra tor y. U su a l a d u lt va l ue s u p to 125 IU /l.

In f la mm a to ry b on e d is ea s e i s ma rg in a lly w ith in the fi el d of o ra l me di ci n e, a lth o u gh th e cl as s ic pr es en ta tio n o f os te om ye li tis o f th e ja w s is n o w ve ry r are . In th is c h ap ter,

cl eid o c ran i al dy sp la s ia a n d f ib ro u s dys p la si a are res p ec tive ly c la ss if ie d a s β € in h eri ted β €™

an d β€ dev el o pm en ta l β€™ co n di tio n s . Th e me tab o li c bo n e dise as es d is c u ss ed i n th is c ha p ter

are g iga n tis m , ac ro m eg al y, o ste o po ro s is , a nd o s teo mal ac ia . H ype rpa ra th yro id is m is

es se n tia lly a n en d o cri n e di se a se bu t ha s b on y m an ifes tati o n s in th e jaw s th a t a re o f o bv io u s im po rta nc e .

Th e s c ree n in g pro c ed u re tha t i s li kel y to pro vi de th e f irs t e vid en c e o f a m eta bo l ic bo n e

ab n or ma li ty i s an e s ti ma tio n of s eru m ca l ci u m, p h osp ho r us , a n d a l kal in e ph o s ph a tas e . Tab le

18. 3 gi ve s deta il s o f th e le ve ls in the c o n di ti o n s me n ti o n ed in th i s ch a pte r. Wh en in ter pre tin g th e re su l ts fo r a lk al in e ph o s ph a tas e it i s im po rta nt to be a w ar e th at th e re ca n b e w i de var ia tio n s de pe n din g u po n th e a ge o f th e p ati en t a nd th e ac tiv ity o f th e di se a se .

Inherited and developmental disturbances Cleidocranial dysplasia

In cl ei do c ran i al d ys pla s ia (dy so s to si s ) th ere i s an a b no rm a li ty o f me mb ra ne b o n e f or ma tio n . Th e c h a ng es o bs erv ed a re la ck o f ca lc if ic ati o n o f th e c la vic le , fl atte n in g o f th e fro n ta l bo n e , an d th e p res e nc e o f a n u m be r o f su p ern u m era ry tee th. T he s e te eth a re o ften o f c o m ple x

fo rm, res em bl in g u n its o f th e n o rma l de n titio n , a n d freq u en tly re ma in u n eru pte d ( Fi g. 18. 2).

It ma y ap pe ar th at th e pa tie n t is su f fer in g fro m h yp o do n tia b ec au s e o f th e fa il ur e to e ru pt o f la rge n u mb ers o f tee th w ith in th e ja ws . Ra d io gra p hs o f th e sk ul l (sh o w in g w o rmi a n bo n es )

an d cl a vic le s (d em on s tra tin g cl av ic u la r a pl as i a or h yp op la s ia ) s h o u ld co n fi rm th e di ag n o si s. Th e a b se n ce o f cl av ic le s en a ble s pa tie n ts to bri n g th e ir sh o u ld ers

fo rw ard to ap pr ox im ate i n th e m id li n e. A f la tte n ed n a sa l bri dg e a n d a h igh -arc h ed n a rro w

P. 224

pa la te m a y a ls o b e pre se n t.

Fibrous dysplasia

Fi bro u s dy sp la s ia i s a f ib ro -os s eo u s le si o n o f th e bo n e a nd m ay in v o lve o n e (mo n o s to ti c ) o r se ver al (p o lyo s to tic ) b on e s in th e bo d y. Th e c au s e o f th e co n d itio n i s u n kn o wn an d it is

gen e ra lly re ga rde d as a de ve lo pm en ta l di so rd er. Di agn o si s is b as e d u p o n cl in i ca l sy mp to ms , rad io l og ic a l ap pe a ran c e, b io c h em ic a l i n ves tig a ti o n s, a n d po s si bl y h i sto p ath o lo g y.

Fibro us d ysp lasia

Mo n o s to ti c fib ro u s dy sp la si a is e ss e nti al ly a s in gle les io n af fec tin g th e ja w s w ith o u t a ny

Po l yo sto ti c fib ro u s dys p la si a is a g en er al iz ed c o n di ti o n th at m a y a ff ec t th e jaw b o n es .

oth e r s ke leta l o r o th er ge ne ra li ze d ab n o rma li ty.

MONOSTOTIC FIBROUS DYSPLASIA Th is is a c o n di tio n th a t ma y ari s e i n ei th er ma le or f em al e pa tie nts a n d is a ss o c ia ted w ith very li ttle o the r d is tu rba n c e o f bo n e o r a n y o th e r tis s u e. It i s mo re c o mm o n th an th e

po ly o sto tic f o rm. T he l es io n s o cc u r m o re o ften in th e ma xi ll a tha n i n th e ma n di bl e. T he

es se n tia l c h a ng e in th is c o n di tio n i s the re pl ac em ent o f th e n o rma l bo n e ar ch ite c tu re b y a pa rtia ll y c a lc ifi ed fi bro u s m as s w ith a h i sto l og y su g ge sti n g a n a c ce le rati o n o f th e n o rma l

bo n e me tab o lis m o f os teo c la s is a n d o ste o ge ne s is . The de gre e of o s si fic a tio n o f the l es io n i s wi de ly va ria bl e. Tis s u e r em o ved fr o m a l es io n m a y vary fr om a v ery s of t a nd h a em o rrh a gic

sp ec im en to a re la ti ve ly h ard a n d we ll o s si fie d tissu e . Th is p ro ce s s is n o t a ss o ci a te d w ith a n y gen e ra liz e d ch a n ge . In pa rtic u la r, th e ac c ep ted bl oo d c h em is try de term in a n ts (ca lc i um ,

ph o sp h o ru s, a n d al ka li ne p h o sp h ata se ) r em ai n u n ch a nge d. In th e c a se o f w ell -dem a rc ate d

les io n s o f fib ro u s dy sp la s ia , the re is a g rea t de al o f di sc u ss i on as to w h e th e r th es e re pre se n t ben i gn , n e op la s tic c ha n g es . Th is q u es tio n h a s be en , to s o me e xten t, s id es tep pe d by the

ad o ptio n o f th e te rm β€ fib ro -os s eo u s le si o n β €™. Th e c h a rac ter is tic fe atu re o f fi bro u s dy sp la s ia is o f a n o the rw is e sy mp to ml es s sw e ll in g o f th e

ma n di ble o r ma xi ll a ( Fi g. 18. 9). In th e c a se o f the m a xil la , th e sw e ll in g ma y en c ro ac h o n th e an tra l c a vi ty (Fi g. 18. 10 ). Th er e i s vir tu a ll y n o oth e r c o mp la in t a n d al l in ve sti ga tio n s o f a

bio c h em ic a l n atu re p ro ve to be u n pro d u cti ve. Th e o nl y u s ef ul i nv es tig ati o n is ra di o gra p hy , al th o u g h th e a pp ea ra n ce o f fib ro u s dy sp la s ia m ay be ve ry va ria bl e. In g en e ra l, th e b a si c

pa tho l o gi ca l pro c es s o f d ec a lc if ic ati o n an d re ca lc if ic a ti o n is ref le cte d i n a m o ttl ed a pp ea ra nc e of th e bo n e on rad io g ra ph y. T hi s , ho w e ver , de pe n ds o n th e sta ge a n d th e ra pi di ty o f th e

pro c es s. In tho s e pa tie n ts i n w h o m th e p ro c es s is slo w a n d in cl u de s a si gn i fic a nt el em en t o f

ca lc if ic ati o n, th e mo ttl in g wi ll b e m in i ma l an d th e exp an d ed b on e w il l ha ve a n a lm o st n o rm a l ap pe ara n c e. If the d ec a lc ifi ca tio n o f b on e is p re dom in an t at th e tim e o f ra di og ra ph y, th e n th e ra di o gra ph i c ap pe ar an c e wi ll re fle ct thi s fa ct.

Fig. 1 8 .9 Fi br ou s dy sp la s ia . A mo n o s toti c le si o n o f th e rig ht ma xi ll a.

Fig. 1 8 .1 0 Fi br ou s dy sp la s ia . Ra dio g ra ph o f le si o n in th e le ft ma xi ll a.

Ma n ag em en t of mo n o s to ti c fi bro u s dy sp la s ia s h ou l d be h ig hl y co n s erv ati ve. T h ere is n o

me dic a l trea tme n t a va il ab le a nd , s in c e th e le si o n s pro g res s to a s el f -lim iti n g s ta tic ph a se

ov er a few yea rs , it is a lm os t a lw a ys b etter to a w ai t ev en ts be fo re ca rry in g o u t su rg er y. Th e exte n t o f th is s u rg ery s ho u l d b e de term in ed e n tire ly by c o sm eti c fa cto rs . S in c e n e o pl as tic ch a n ge is v irtu a lly u n kn o w n in th i s co n d itio n , th e re i s n o n ee d to atte mp t to re mo ve th e wh o le o f th e l es io n a n d, i n fa ct, th i s is o fte n an al mo s t im p os s ib le ta sk . It is g en er al ly

ac ce pte d, th ere fo re, th a t s im pl e co s me tic c o n tou ri ng of th e fa ci al s ke leto n i s be st ca rri ed o u t afte r g ro w th is c o mp le te.

POLYOSTOTIC FIBROUS DYSPLASIA Th is c o nd iti o n is ve ry mu c h l es s co m mo n th a n m o no s to tic fi br ou s d ys pl as ia . It i s vir tu a ll y

al wa ys as s o ci ate d wi th w id es pre a d c h a ng es th ro u g ho ut th e s kel eto n a n d in o th er sy ste ms o f th e b od y

th a t a re co l le cti vel y k n o wn as A lb rig h t's s yn dro m e. In th i s co n d itio n ar ea s o f b o ne

th ro u g h ou t th e b od y are re pl ac ed b y f ib ro u s ti ss u e w ith w id el y di ffe rin g a mo u n ts o f n e w os s ifi c ati on in c lu de d wi th in th em . Th i s o ften l ea ds to mu l tip le fra c tu re s a nd to g ro ss

dis to rtio n o f th e s ke le to n . In Al bri gh t's sy n dro m e, th e bo n y le si o n s are a ss o c ia ted w ith

pa tch y me la n o tic s kin pi gm en ta tio n (c a f Γ© -au -la it spo ts ) a n d, i n th e ca s e o f fe ma le s, se xu a l

P. 225

pre co c ity. Th is c o nd iti o n is a fa r mo re a cti ve o n e th a n th at of m o no s to tic fi bro u s dy sp la s ia an d th is is r efl ec ted in the b lo o d ch e mi stry c h an g es . In po l yo s to ti c fib ro u s dy sp la si a th e

se ru m al ka lin e p ho s p ha ta se is o f ten g rea tly el eva ted, a s in th e s eru m c al ci u m. T hi s is i n co n tra st to mo n o s to ti c fib ro u s dy sp la s ia w h en n o s uc h ch a n ge s ca n b e s h o w n .

Albrig ht's synd ro me

Po l yo sto ti c fib ro u s dys p la si a

Me la n o ti c sk in p ig me n tati o n

Pre c oc io u s p u be rty i n fe ma le s

Metabolic and endocrine disorders Gigantism and acromegaly

Hyp ers ec re tio n o f gro w th h o rmo n e is u s u al ly as s o ci ate d wi th a n ad en o m a o f th e an te rio r

pitu i tary gl a nd . Th e cl in ic a l ma n ife s ta tio n s o f the c o n di ti o n a re de pen d en t u p o n th e ti me o f on s et o f th e h ype rs ec reti o n. In ch i ld ren w i th o pen ep ip hy se s , gi ga n ti s m o c c u rs, le ad in g to

gen e ra liz e d ov erg ro w th o f th e s kel eto n , o rga n s , an d s o ft ti ss u es . Wh en th e e pip h ys es h a ve

fu se d, o v erp ro du c tio n o f gro w th h o rm on e c au s es ac rom eg al y β€”on l y b o n es w ith th e po te n ti a l fo r g ro wth wi ll en l ar ge. Ac ro m eg al y is a c co m pa n ie d b y ren ew e d gro w th o f th e m an d ib ul a r

co n dy le , h an d s, a n d fee t w ith o ver gro w th o f so m e soft tis su e s. C o nd yl ar gro w th re su l ts in ma n di bu la r pro g n ath i sm . A s a co n s eq u en c e o f th is , tee th, w h e n pre s en t, be c om e sp a ce d. Ch an g es o c cu r in th e fa ci a l ti ss u es β€” th e li ps a n d n os e be c om e th ic ken e d, res u lti n g i n a co a rs en in g o f th e fa c e. Th e to n gu e m ay al s o en la rg e (m ac ro gl o ss ia ). A c ro me ga l y i s al so ac co m pa n ie d by s eri ou s s ys tem ic c o mp li ca tio n s su c h a s , rai se d in tra cr an ia l pre s su re ,

he ad a ch e s, b li n dn es s , h yp erte ns io n , ca rd io m yo pa th y, a n d dia b etes m e lli tu s. T he o ro fa c ia l fea tu res o f ac ro m eg al y a re su m m ari z ed in Tab le 18. 4. An en la rg ed to n gu e is a c o mm o n ly

rep or ted fe atu re o f a c ro me ga ly . Th e de n ti s t ma y there fo re h ave a n i mp o rtan t ro l e to pl ay i n th e d ia gn o s is o f th e d is ea se (s ee Ch ap ter 6). Ra rely, p ati en ts w ith a cr om e ga ly ma y pre se n t wi th fa ci a l p ai n .

Table 18.4 Acromegaly: orofacial features Ma c ro glo s s ia Fa c ia l pa ls y

Ma n di bu la r gro w th Fa c ia l p ai n

Tem po ro m an d ib u la r jo in t pa in Ma lo c c lu si o n

P ro c li n ati on o f a n teri or tee th In c rea s ed i nte rde n tal sp a ce s

P o s teri o r a n d a n teri o r cr o ss - bite s

Hyp erc em en to s is

Co ars e ni n g of fa c ia l fe atu re s Sk in h yp erh i dro s is , ac n e

Trea tm en t o f ac ro m eg al y d ep en d s u p o n th e c a u se o f the c o n di ti o n . Ide al ly , gro w th h o rm on e

lev el s ne ed to re ve rt to n o rm al . Tre atm en ts in c lu d e su rg er y (u s u al ly tra ns p he n o id al re se cti o n of th e tu m o u r) a n d rad io th er ap y to th e tu mo u r. Dru g th era p ies ( fo r e xa mp le , bro m o cr ipti n e

or s om a tos ta tin a n al o gu es su c h a s o ctre o tid e) ma y al so b e u se d. S u rg ery ma y al so be req ui red to c o rre ct so m e o f th e s kel eta l a n d de n tal c on s eq u en c es o f th e d is ea s e.

Hyperparathyroidism

Hyp erp ar ath yro i di sm i s ca u se d by o ver se cr etio n o f pa ra th o rmo n e (P TH). Pa ra tho r mo n e mo bi li z es c al ci u m fro m bo n e to in c rea s e th e s eru m cal ci u m le vel s. Th is is a c h ie ved b y

in cr ea se d in tes tin a l ab s o rptio n of c al ci u m an d in c re as ed re ab s or ptio n of c al ci u m by th e re n al tu b u le s, b u t mo s tly by i nc re as ed o s teo c la s ti c bo n e res o rp tio n .

Pri ma ry h yp erp ara th yro i di sm i s us u a lly d ue to a n a de n om a o f th e pa ra th yro id g la n ds .

Se co n d ary h yp erp a rath yr oi di sm i s in c rea s in g in p reval en c e an d i s a co n s eq ue n ce o f ch ro n i c dep re ss ed p la sm a c al ci u m le vel s. T h is is m o s t fre que ntl y d u e to ch ro n ic r en a l f ai lu re

(Ch ap ter 13) o r lo n gs ta n din g m al ab s o rpti on (fo r e xamp le , c oe li ac d is ea s e; se e Ch ap ter 12 ). Th e m a n ag em en t o f pri ma ry h yp erp ara th yro i di sm i s u su a ll y s u rg ic al . Th e trea tme n t o f se co n d ary h yp erp ar ath yro i di sm i s de pe nd en t u po n treati n g th e c au s e.

PRIMARY HYPERPARATHYROIDISM Pri ma ry h ype rp ara th yro i dis m m ay be m il d an d as ym p tom a tic bu t in s ev ere c as es m a y b e li fe th re a te n in g du e to u n c on tro l le d h y per ca lc a em ia a n d r en a l f ai lu re . Th e co n d itio n is fre qu e n tl y dia g no s ed i n ci de nta ll y wh e n c al ci u m i s me a su re d as p art o f a b io c h em is try p ro fi le β €”th i s is gen e ra lly b efo re th er e i s ex te n si ve de stru c tio n o f th e b o n e. C ys t-lik e, o s teo ly tic s w ell in g s (bro w n tu m ou rs ) of th e ja ws c a n de ve lo p; th es e are h is to lo g ic al ly in d is tin gu i sh a bl e fro m

gia n t c el l gra n u lo m as o f th e ja w s . Ra dio g ra ph ic a ll y, th ey a re w e ll de fi ne d ra dio l u ce n ci es an d ma y b e mu l ti lo c u la r. Th e y o c cu r mo re f req ue n tly in th e m an d ibl e an d th e ma xi ll a. In

hy pe rpa ra thy ro id is m th ere m ay be l os s o f th e l am in a d ur a an d ge n era li z ed ra ref ac tio n o f th e jaw b o n es .

Th e d ia gn o s is s h o u ld be c o n firm ed w i th in ve sti ga tion o f bl o od c h em is try, w h ic h re ve al s ra is ed pla s ma ca l ci u m a n d ra is ed P TH. T he p la sm a ph o s ph a te le vel is o f ten l o w bu t m ay b e n o rma l,

pa rtic u la rly if th ere i s an e le me n t o f r en a l f ai lu re . Pl as m a al ka li ne ph o s ph a tas e is o n ly ra is ed wh e n th ere is b o n e i n vo lv em en t.

P. 226

Hypoparathyroidism

Th is c o nd iti o n is u s u al ly ia tro ge n ic , fo ll o w in g remo va l o f th e pa ra th y ro id gl an d s. Ea rl y -on s et

hy po p ara th yro i dis m c an af fec t c a lc ifi ed tis s u es a n d re s ul t i n h ypo p la s ti c en a me l, s h o rt ro o ts , an d in c o mp le te h y po m in era li z ati o n o f d en tin e (s ee earl ie r se c tio n o n te eth ). A ll o f th es e ch a n ge s a re d ue to h ypo c a lc ae m ia .

Osteoporosis

Os teo p or os i s is a c o nd iti o n in w h ic h th e sk el eta l bo n e s tru c tu re u n d erg o es d eg rad ati o n bo th of b on e m atri x a n d ca lc i um , th at i s , a red u cti on in b o ne m a ss p er un i t vo l um e . Se ru m

bio c h em is try in di c es ar e n o rm a l. Th e re is s kel eta l rar efa c tio n a n d l o w trau m a fra c tur es o fte n oc c u r, in c lu d in g ver ta b ral fr ac tu res . O ste o po ro s is i s pro b ab ly th e m o st co m mo n dis e as e o f bo n e. W om en are m o re lik el y to b e af fec te d th a n m en. In th e ed en tu lo u s pa tie n t w ith

os teo p o ro si s th e r es id ua l al ve o la r ri dg es m ay re so rb m o re rap id ly. Os te op o ro si s ma y li mi t

th e s ite s ava i la bl e f o r im p la nt pl ac em en t an d a dve rs ely af fe ct th e p ro gn o si s o f e n do s se o u s im pla n ts . Co n ver se ly , th e p res en c e o f fu n c ti o n in g im pl an ts m ay re du c e th e ra te o f pro g res si o n o f o ste o po ro s is .

Th ere a re ma n y ri s k fa c to rs th at h a ve be en id en tif ie d: in c rea si n g ag e, p o stm en o p au s e

(es pe ci a lly ea rl y m en o p au s e); s te ro id th era py ( Ch apter 3); h yp erth y ro idi s m a n d pri ma ry hy pe rpa ra thy ro id is m; a nd i mm o bi li za tio n . Th e d is ea se o c cu rs m o s t c om m on l y i n

po s tm e no p au s a l f em al es , a n d h o rm o n e r ep la ce me n t th era py c an be h el pfu l in pre ve n ti o n a nd

tr ea tme n t. Bi ph o s ph o n ate th er ap y i s w ide ly u se d an d h a s be en sh o w n to i n cre a se b on e den s ity a nd re du c e fra ctu re ri sk .

A w el l d o cu m en ted i atro g en i c ca u se o f o ste o po ro s is is s ys tem ic s te ro id th era py a nd 50 p er ce n t o f p ati en ts o n p red ni s ol o n e ( 5 mg /d ay o r m o re) fo r m o re th an 3 mo n th s o f e a ch y ea r

are li ke ly to de ve lo p o ste o po ro s is . Th e pro p h yla xi s o f o ste o po ro s is fo r pa tie n ts o n lo n g -te rm ste ro id th era py is f u lly d is cu s se d in Ch ap ter 3.

Rickets and osteomalacia

Ric ke ts i s a c on d iti on th a t o c cu rs i n c h ild re n as a res u lt of d efi ci en t c a lc if ic ati o n o f th e bo n e s an d (u n u su a ll y) th e tee th . It is e ss en ti al ly du e to la c k o f vi tam in D , be ca u se o f ei the r

nu tri tio n al de fi ci en c y, m al ab s or ptio n , o r im pa ire d me tab o lic p ro c es se s . In thi s c on d iti on th e bo n es a re po o rl y f or me d a n d, as a re su l t, ba dl y s h ape d. It i s sa id th a t th e tee th ar e n o t

aff ec ted i n thi s c on d iti on , b u t in fa ct, s o me p ati en ts w ith a h is to ry o f ric ke ts h av e s ig n s o f

hy po p la si a o f th e tee th (Fi g. 18. 11). Os teo m a la ci a is th e ad u lt e qu i va len t of th is c o n di ti o n , th a t i s, de fe cti ve bo n e mi n era li z ati on in b o ne th a t h as s to pp ed g ro wi n g. O s teo ma l ac ia m a y oc c u r in pre gn a n cy (p arti cu l arl y i n th o se o f As i an o ri gi n ), in m a la bs o rpti o n (s u ch a s in

co e lia c d is ea se ), o r i n re n al di se a se . Th er e i s a fa il ur e o f mi n era li z ati o n du ri ng n o rm al b o ne tu rn o ve r. Ca l ci u m p la s ma l eve ls te n d to b e lo w , p hos ph a te le vel s ca n b e no rm a l o r lo w , a nd

al ka lin e p ho s ph a ta se le ve ls a re ra is ed . Os teo m a la cia is ra re ly dia g n os e d i n th e o r al m edi c in e cl in ic .

Fig. 1 8 .1 1 Den ta l h ypo p la s ia in a pa tie nt wi th a h is to ry o f ric ke ts.

Disorders of unknown aetiology: Paget's disease

Pa ge t's di s ea se (o s tei tis de fo rm a ns ) is a w id es pre ad c o n di tio n o f ol d ag e an d is f o u nd i n a

la rge pr o po rtio n o f o ld er pa tie nts a t a u to ps y. Ra diog ra ph i c si gn s o f P a ge t's di s ea se m ay be

se en i n a bo u t 3 p er ce n t o f th e po p u la tio n a ge d o v er 40 ye ars β €”mo s t w il l b e as ym pto m ati c. Pa ge t's di s ea se i s si m ila r to fib ro u s dys p la si a, in th a t bo th re pr es en t a n im b al an c e of th e

os teo g en i c an d o ste o lyti c pro c es s es o c cu rr in g in b on e f o rma tio n . A c ti vi ty i s no t rel ate d to

ph ys io l og ic a l r eq ui rem e nts a n d th e fi n al re su l t is b o n e g ro w th. P ag et's d is ea s e c a n a ffe ct an y

bo n e in th e bo d yβ€” th o s e mo s t c o mm o n ly af fec ted ar e th e pe lv is , sp in e , s ac ru m, fem u r,

ti bi a, a n d sk u ll . It o ften o c c u rs in i ti a lly in th e s ku ll a n d f ac i al bo n e s. T he c la s si c co m pl ai n t o f a p a tien t i s th at h i s o r h er h at ha s b ec o me to o tigh t, bu t a n e qu a lly c o mm o n c om p la in t i s of den tu re s tha t a re be c om in g β € to o sm al l β€™. T he b o ne g ro w th in P a ge t's di se as e

pa rtic u la rly a ffe cts th e va u lt o f th e s ku l l a n d ma xi ll a, a lth o u gh th e m an d ib le ma y al so be

in vo lv ed (Fi g. 18. 12 ). Th e exp a ns i on of th e bo n e o f th e ba s e of th e sk ul l le ad s to cl o su re o f th e fo ra m in a a nd re s ul tan t n eu ro lo g ic a l c h a ng es s u ch as d ea fn e ss . Ne rve c om p res s io n m ay

al so l ea d to β€ ne u ral gi a -lik eβ €™ sy mp tom s in the tri ge mi n al n erv e. Th i s sh o u ld a lw a ys b e co n s id ere d a s a po s s ib le di ag n o si s in o l de r p ati en ts, pa rtic u la rly w h en th e re ar e o th e r

sym p tom s s u ch a s de a fn es s . Ap a rt fro m n e ur al gi a-like s ym p to m s th e pa tie n t ma y co m pl ai n o f pa in w ith i n th e b o n e i tse lf . Th is i s a co m mo n s ym p to m kn o w n a s bo n e pa in . Ra di o gra p hi ca l ly, th e b on e i s r ep ute d to h av e th e a pp ea ran c e o f c o tton w oo l . Th er e i s lo s s o f n o rm al b o ne

tr ab ec u la tio n a n d, i n la ter sta ge s , ar ea s o f s c ler os i s ca n b e s ee n β€”giv in g ris e to th e co tto n

wo o l ap pe a ran c e. S in c e c em e ntu m i s e s se n tia lly b on e, th is is a l so a ff ec ted b y th e ch a n ge s o f Pa ge t's di s ea se , a nd h yp erc e me n to s is i s a co m m on fin d in g in th e se p ati en ts . M al ig na n t ch a n ge in th e a ffe cte d bo n e is a re pu ted c o mp li ca tio n , a lth o u gh th e in c id en c e o f

os teo s a rco m a is l o w an d u s ua l ly o cc u rs in p a ti en ts w ith p o ly os to tic P ag et's d is ea s e. C ard ia c

fai lu re b ec au s e o f h ig h o u tpu t i n to th e e xp an d ed blo o d sp a ce s o f th e bo n e is a w e ll -rec o rde d, bu t ra re co m pl ic a tio n .

Fig. 1 8 .1 2 P ag et's d is ea s e. Ra di og ra ph s h o w in g ex pa n si o n o f th e sk u ll an d β€ co tto n wo o l β€™ ap pe ara n c e o f th e bo n e .

Th e c a u se o f P ag et's d is ea s e i s u n kn o wn . V i ral a n d g en e tic fa c tor s ha ve b ee n im pl ic a te d.

P. 227

Dia gn o s is o f P a ge t's di se a se in the f irs t in s tan c e is c li n ic al , co n f irm ed b y im ag in g

(rad io g ra ph y an d a ra di oi s oto p e b o n e sc a n) an d b y blo o d ch e mi s tr y, th e ch a ra cte ris tic

fin d in g be in g an in c rea se d (s om e ti me s gr ea tly in c rea s ed) s eru m al ka li n e p h o sp h ata s e l ev el. Oth er bo n e ma rk ers a re o ften ab n or ma l in P a get' s dise a se . Fo r ex am pl e, u ri n ary

deo x ypy rid in o li n e is el ev ate d i n a cti ve P ag et's di se a se a nd f al ls i n res po n s e to trea tme n t.

Pa tie n ts w i th su s pe c ted P ag et's di se a se a re us u a lly re fer red to a s pe ci al is t i n me tab o li c bo n e

dis e as e. B is ph o s ph o n ate s (fo r exa m pl e, p am id ro n ate o r r is ed ro na te) a re very e ffe cti ve in th e tr ea tme n t o f P a ge t's di se as e be c au s e o f th e in h ib ito ry eff ec ts thi s c la ss o f dru g h a s o n

os teo c la s ts. T h e n e t res u lt is a re du c tio n in bo n e tur no v er an d , co n s eq u en tly , in p ro gr es si o n of th e dis e as e.

Th ere a re a n u mb er of i mp or tan t d en ta l c o n si de ra tion s w he n tre ati ng p ati en ts w ith P a ge t's

dis e as e. E x trac tio n o f tee th ma y be di ff ic ul t b ec aus e o f h yp er ce me n tos i s a n d th ere m ay be pro fu s e po s to per ati ve h ae mo rrh a ge b ec a us e o f th e irr eg ul a r a nd in c rea s ed bl o o d s u p ply to

th e n ew bo n e. P a ge t's di se as e s ho u l d, i f p o ss ib le , al s o be re n der ed bi o ch e mi ca ll y in ac tiv e by bis p ho s ph o n a tes p rio r to su rg ery , as th is w il l r educ e th e v as c u la rity o f th e af fe cte d b o n e. Pa tie n ts w i th th is co n d itio n are a ls o m o re s u s ce ptibl e to i n fe cti on fo ll o wi n g a n y fo rm o f

in terv en tio n a n d an y ex trac tio n o r o ral s u rge ry proc ed u re s h o u ld be c o ve red b y an ti bi oti c

pro p hy la xi s. T he s e p ro bl em s o f h a em o rrh a ge a nd i nf ec tio n l ea d to gre a t ca u tio n i n ta kin g bio p si es i n P ag et's d is ea s e. S i nc e th is is a c o n di tio n th at c a n be d ia gn o s ed by n o n -

in terv en tiv e m ea n s , th e q u es tio n o f co n fi rm ato ry bio ps y s h o u ld be a pp ro a ch e d w i th gre at

res erv e. A s u mm a ry of th e sa li en t cl in i ca l fea tu res o f P ag et's d is ea s e is g ive n in Tab le 18. 5. Pri o r to e xo do n ti a P a ge t's di s ea se s h o ul d be re nd ere d ch e mi ca l ly in a cti ve by bis p ho s ph o n a tes β€” th i s wi ll re du c e th e va s cu l ari ty o f th e a ff ec ted bo n e .

Table 18.5 Summary of clinical features in Paget's disease Pre do m in a n tly a dis e as e o f l ate r li fe, rare u n de r 40 yea rs , o fte n as ym pto m a ti c Res u lts in erra tic in c rea s ed bo n e g ro wth , b o ne d en sity, a n d de fo rm ati o n Sk ul l fre qu e ntl y a ff ec ted

H at si z e b ec o me s to o sm a ll

C ra ni al n e rve de fic i ts p o s si bl e

Se ru m al ka li n e p h o sp h ata s e m a y b e e le va ted Rad io g rap h ic fe atu re s

β€ Co tton wo o l ap pe a ran c e β €™

β€ Hyp erc em en to s is , po s s ib le ro o t res o rp tio n β€™

Fu rth er de n tal co n s id era tio n s

A p pe ara n c e o f di a ste ma s be twe en tee th, oc c lu s al d era n ge me n t, lip in c o mp ete nc e De n tu res b ec o me to o tig h t

P o s si bi li ty o f di ffi cu l t ex trac tio n s

R is k o f po s tex trac tio n b le ed in g an d /o r i n fec tio n C an ca u se f ac ia l pa in o r pa ra lys i s


Wh at a re th e di ffe ren ti al d ia gn o s es fo r thi s bo y's de n ta l co n d itio n ?

Th e o p ac i ti es a re pre s en t o n mo s t te eth β €”th e ref o re i t i s un l ike ly th at th i s is c h ro n ol o gi ca l

hy po p la si a. T h e mo s t l ike ly di ag n o si s o f th i s 14 -yea r -ol d' s c o n di tio n i s de nta l flu o ro s is , b ut th e d en tis t n ee ds to c o n s id er g en e tic c au s es s u c h as a m elo g en e si s im pe rfe cta . Q2

Wh at a d di ti o n al in fo rm a tio n d o yo u n e ed to h el p yo u ma ke a d efi n iti ve di ag n o si s?

On ra re o c ca s io n s, it m ay n o t be p os s ib le to d iff ere n ti a te b etw ee n fl u o ro si s an d a me lo ge n es is im per fec ta . Ho w ev er, th e in fo rm a tio n g ai n ed by a sk in g th e q u es tio n s be lo w u s u al ly al lo w s a def in iti ve di ag n o si s to be m ad e. 1.

Was th e pr im ary de n titio n af fec ted ?

2.

Do an y o th er m e mb ers o f th e f am il y h a ve a s im ila rl y af fe cte d d en ti ti o n ?

3.

Has th e pa tie n t li ve d i n a n ar ea w h ere th e wa te r s up pl y wa s fl u or id ate d o r h ad a

4.

na tu ra lly h ig h fl u or id e le vel ?

Were fl u o rid e s u pp le me n ts u se d d u rin g th e p eri o d of c al c ifi ca tio n o f th e c ro w n s o f th e

te eth ?

Th e p res e nc e o f d is c o lo ra tio n i n the p rim a ry d en titi o n w o ul d su p po rt ge n etic i n flu e n ce s,

al th o u g h th e p rim a ry d en ti ti o n is n o t i n va ria bl y a ff ec ted . A h ere di tary c o nd iti o n is a ls o

su p po rted b y th e o cc u rre n ce o f th e c o n diti o n in a fa mi ly me m be r w h o be lo n gs to a dif fe ren t gen e ra ti o n o r l ive s in a dif fe ren t ge o gra ph i ca l ar ea . Th e pre se n ce o f en a me l op a ci ties i n si bl in gs i s n ot a he lp fu l di sc ri mi n ato r.

Den tal fl u o ro si s is a c o mm o n en a m el de fe ct, re s ul tin g fro m a n in c rea s e in c o n ce n tr ati on o f

P. 228

flu o ri de in th e m ic ro en vi ro n me n t o f a m elo b la s ts du rin g e na m el fo rm ati o n . Fl u o ro si s th ere fo re res u lts fro m s ys tem ic i n ta ke o f flu o ri de d ur in g en ame l fo rm ati o n. In v es tig ati o n o f th e

flu o ri de h is to ry i s es s en tia l. Le ve ls o f fl uo ri de in th e w a ter su p pl y c an be o bta in e d f ro m the ap pro p ria te wa ter su p pl ie r. It is a ls o p o ss i ble th at fl uo ro s is c o u ld b e d u e to flu o ri de su p ple m en ts (to o th p a ste ; fl u o rid e d ro ps , ta bl ets , or mo u th w as h es ).


Wh at a re th e po s s ibl e ca u s es o f to o th w e ar in th is c a se ?

To o th we a r u s ua ll y ha s a m ul tif ac to ria l ae tio lo g y. Th e c li ni ca l s ign s de cr ib ed in c a se 18. 2, ho w e ver, are h ig h ly su g ge sti ve o f d en ta l ero s io n . Th i s do e s n ot ne ga te th e p o ss i bil ity o f

nu tri tio n a n d a b ras io n bei n g im po rta n t co -des tru c tive a eti ol o gi es . A fu l l m ed ic a l a n d de n ta l

hi sto ry , in c lu d in g a di eta ry an a lys i s wi ll be re qu ired to try to id en tif y i f th e a ci d is di eta ry o r ga stri c in o ri gi n . Th e in tak e of fo o d a nd b ev era ge s w ith e ro si ve po te nti al n ee ds to be as se s se d. A h i sto ry o f a c id re flu x a nd v om iti n g s h ou ld a ls o be e xpl o red . It s ho u l d b e

rem em be red th a t ga s tric re flu x m ay be s il en t (a s ym pto ma tic ) an d th at p a tien ts m ay n o t ad mi t to s el f-in du c ed vo m iti n g. Q2

Wh at c o n di tio n w o u ld yo u s u s pe ct i f th e pa tie n t al s o pre s en ted w ith s w o ll en p ar oti d gla n ds a n d ap pe a red to h a ve ge n era li z ed s kel eta l mus cl e wa s tag e?

A pa tie n t w h o pre s en ts w ith d en tal e ro si o n , en la rg ed pa ro tid g la n ds , a nd ge n era li z ed mu s c le wa s tag e i s li ke ly to h av e a n e ati ng d is o rde r, a n d bu li mi a n erv os a s h o ul d be s us p ec ted .

Projects 1.

Lis t th e d is o rde rs o f te eth th at y o u a re l ik ely to s ee i n pa tie n ts . Di vid e the s e d is o rde rs

in to : (a ) th o s e w ith a p ren a tal a n d p o stn a tal a eti ol o gy ; (b ) th o s e th a t a re co n ge n ita l o r ac qu i red c o nd iti o ns . 2.

Id en ti fy th e d iff ere n t ca u s es a n d c l in ic a l p res e nta tio n s o f to o th d is c ol o ra ti o n .

3.

Un de rta ke a li te ra tur e s e arc h u s in g ke y w o rd s β€ Pa ge t's di se a se β €™ an d

β€ den ti stry β €™. W ha t a re th e d if fer en t w a ys in w h i ch p a ti en ts pre s en t w ith

un d ia gn o s ed s ym pto ma tic P a ge t's di se a se to d en ta l, o ra l m e dic i ne , o r m ax il lo fa c ia l

cl in ic s ? Wh at a re th e pro b le ms i n ma n ag in g a d en tal p ati en t w ho h as a d ia gn o s is o f Pa ge t's di se a se ?

Au th o rs : Title:



Co py rig ht Β © 2003 Ox fo rd U ni ve rsi ty P re ss > T ab le o f Co nt e nt s > 1 9 - Me d ic a l e m e r g e nc ie s in d e nt ist r y

19 Medical emergencies in dentistry Life -th re a te n in g em er ge nc ie s a re r are b u t ca n o c cu r at an y tim e in de n tal pr ac tic e. De n tis ts mu s t be a bl e to pro vi de a cu te ly il l p a ti en ts w ith li fe -sa vi ng m ea s ur es p rio r to th e a rriv al o f

sp ec ia li st he lp . It i s e s se n tia l, th ere fo re, th a t th ey a re trai n ed in th e m an a ge me n t o f m e dic a l em erg en c ie s th a t th ey mi gh t p rec ip ita te o r en c o u n ter.

Medical emerg encies that may be enco untered in denta l p ract ice a nd that require pro mp t manag ement

Va s ov ag a l atta c k

Se iz u res

An g in a

As th ma a ttac k

Hyp o gly ca e mi a

My oc a rdi al in fa rc tio n

An a ph yl a xis

Car di ac a rre st

The prevention of medical emergencies

Me di ca l em erg en c ie s ar e u su a ll y u n e xpe cte d bu t ra rely o cc u r w ith o u t w a rn in g. Th e d ec l in e in a p a tien t's h ea lth w h il st in th e de n tal c h ai r is l ik el y to b e pre ce de d by si gn s a n d s ym p to m s an d ea rl y re co g n itio n o f a n u n we ll o r d ete rio ra tin g pa tie n t c an s o m etim es a b o rt an ac u te pro b lem .

Den tis ts ex am in e a nd tre at a po pu l ati o n o f pa tie n ts wi th va ria b le h ea lth s tatu s . A dv an c es i n me dic a l ca re an d a tre nd f or lo n ge vi ty m ea n s th at an i n cre a si ng n u m be r o f me di ca ll y

co m pro m is ed p ati en ts pre s en t fo r de n tal c are . It is i mp o rta nt tha t th e ris ks a s so c ia ted w ith th e ma n a ge me nt o f th es e pa tie n ts are a s se ss ed . A d eta ile d me di ca l h is to ry i s o f pa ra mo u n t

im po rta nc e . Wh en a s se s si ng th e s ign i fic a n ce o f a patie nt' s m e dic a l pro b le ms , i t is h el pf u l to co n s id er th e fo l lo w in g qu e sti o ns .

Wh at a re th e eff ec ts, i f an y, o f th e me di c al c on d iti on ( s) o n the p ro po s ed tre atm en t?

Wh at e ff ec t w il l th e pro p o se d trea tme n t h av e on the d is ea s e?

Are a dv ers e dru g re ac tio n s o r i n tera cti o n s an ti ci pated ?

It is n o t p os s ib le to be f am il ia r w ith al l si gn if ica nt dru g in te rac tio n s a n d th e c li ni ci a n sh o u ld ha ve i mm ed ia te a c ce s s to a re gu la rly u pd a ted ref eren ce te xt, su c h a s th e Bri tis h Na tio n al

Fo rm u la ry. An o th er in va lu a bl e so u rc e o f a dv ic e, i n th e U K, is th e Dr ug In fo rm a ti o n S erv ic e,

wh i ch h a s a u n it d ed ic ate d to th e m a na g em en t o f drug -rel ate d p ro bl em s a ss o ci ate d wi th

den ta l trea tme n t. Den tal pa tie n ts ma y b ec o m e s ev ere ly a nx io u s a nd e xperi en c e a c u te s o ma tic s ym pto m s pri o r to , o r du ri n g d en ta l tre a tme nt. A pa n ic a tta c k i s an extre m e a c ute m an i fes ta tio n o f th i s. Ph ys i ol o gi ca l an d p sy ch o lo g ic al s tres s o rs ma y pre sen t a s eri o us ri s k to a me di ca l ly

co m pro m is ed p ati en t. M eth o ds o f pa in an d a nx ie ty c on tro l a re o f pa ra mo u n t i mp o rtan c e in th e m an a ge me n t o f m e dic a ll y c o mp ro m is ed pa tie n ts. Fo r ex am pl e, i n a de n tal p ho b ic w h o

su ff ers f ro m hy pe rten s io n a n d an g in a, it i s im po rtan t to a me li o rate a p ati en t's ph y si o lo gi ca l res po n s e to an xi ety -pro vo k in g pro c ed u res β €”th e u s e o f s ed ati on ca n b e b en e fic ia l in th i s si tu a tio n .

An a w a ren e ss o f po ten ti al p ro ble m s or c om p lic a tio n s as s o ci ate d wi th me di ca l c on d iti on s c a n so m etim e s pre ven t a c ri tic al i nc id en t. F o r e xa mp le , h ypo g ly ca em ia i s an ac u te co m pl ic ati o n of b oth ty pe 1 a n d 2 di ab ete s me ll itu s . Th e tim in g o f d en ta l a p po in tm en ts s ho u l d b e dis c u ss ed w ith th e p ati en t s o th at th ey are c o mp a tibl e wi th th e p a tien t's d ia be tic

ma n ag em en t, a ll o wi n g a d he re nc e to u su a l ea tin g s ch edu le s. If a d en tal p ro ce du re (f or

exa m ple , m ul tip le ex trac tio n s ) i s lik el y to r es u lt i n di eta ry res tric tio n s , co n s u lta tio n w ith th e

pa tien t's p h ys ic ia n o r d ie titia n m a y, o n o c ca s io n s, be ad vi sa b le. H o w eve r, in ma n y dia b etic s , it i s th e p ati en t w h o h a s th e re qu ir ed ex pe rt kn o w le dg e.

Den tis ts u se lo c a l a n a es th etic s o n a da i ly ba si s and th es e ag en ts h a ve an exc e lle n t s af ety

rec o rd. It is , h ow e ve r, im po rta n t th at cl in i ci an s do n o t be c om e co m pl a ce n t ab o u t th ei r s id e eff ec ts an d to xic i ty . Th e ma xi mu m re co m m en de d do s age o f a l oc a l an a es th etic m u s t n o t be

exc ee de d an d ca u tio n s h o u ld be e xer ci se d wh e n u si n g mo re th an on e p rep ar atio n . It s h o u ld

al so be re me mb ere d th at to p ic al a n ae sth e tic s su c h as li do c ai n e g el s o r m o uth w a sh e s wi ll be ab so rb ed a n d w i ll , the ref o re, c o n trib u te to d ru g level s in th e s ys tem ic ci rc u la tio n . Th e

ma xi mu m s af e d o se o f lo c al a n ae sth e ti c s sh o u ld b e sig n if ic an tly red u ce d in p ati en ts w h o ha ve

co n c ur ren t d is ea s e, a re o n ce rta in m ed ic a ti o n s, o r ar e v ery yo u n g o r el de rly .

P. 232

Wh en c o n si de rin g th e m a xim u m do s e o f l o ca l an a es th etic c o n s ide r th e f o llo w i ng . Pa tie n t fa cto rs Age

W ei gh t

V a s cu la ri ty o f th e tis s ue s be in g a na e sth eti z ed C o n cu rre n t di se a se

Th e l o ca l an a es th eti c A n a es th etic ag en t C o n ce n tr ati on

M a n u fa ctu re r's in s tru cti o ns V a s oc o n s tr ic tor

Circ u la to ry co l la ps e ha s b een rep o rted in pa tie nts u n de rg oi n g su rg ic al pro c ed u res a n d th is ha s ra rel y b ee n a ttrib ute d to ad ren a l in s u ffi ci en c y th a t h a s res u lte d fro m ste ro id th era p y. So m e au th o rs ad vo c a te th a t pa tie n ts wh o a re tak in g ste ro id s reg u la rly o r h a ve do n e s o

wi th in th e pa st 6 m on th s s h o ul d rec e ive a p retre atmen t d o se o f ste ro id o r d o u bl e th e ir d a ily do s e pe rio p era tiv el y. Th e u se o f ste ro id c o ver is a c o n te n tio u s is s ue a n d pre trea tm en t

pro to co l s ar e n o t u n iv ers a ll y a c ce pte d. It i s the au th or s' o pi ni o n th at th e ro le o f ste ro id

pro p hy la xi s fo r d en ta l p ro ce du re s h as , i n th e p as t, be en o v ers tate d. E v id en c e-ba se d res ea rc h is re qu ire d to se ttle th is de ba te.

Th e r ec o gn iti o n o f th e u n we ll pa tie n t a n d th e as s ess me n t o f h i s or h er su i tab il ity f o r

tr ea tme n t ar e i mp o rtan t s ki ll s fo r a cl in i ci an to de vel o p. De n tis ts wi ll o n ly en c o u nte r a lim ite d nu m be r o f em erg en c ie s a nd a ll o f th e se c a n res u lt i n th e co l la ps e o f th e

ca rdi o va sc u la r sy ste m. Th ere fo re , it i s es se n tia l th a t d en tis ts reg u la rly u pd a te th e ir

kn o wl ed ge o n th e pre ve nti o n an d m an a ge m en t o f rel eva n t m ed ic al e me rge n ci es . Th e be st

wa y o f a dd res s in g th is is b y ha vi n g r eg ul ar s ce n ario tra in in g w ith a ll o f th e d en ta l te a m. Wh ils t re h ea rs al o f ba si c li fe su p po rt us in g m an i kin s i s ma n da to ry fo r a n y c l in ic ia n ,

res pi rato ry a n d c a rdi ac a rre st sh o u ld n o t b e th e o nly em erg en c ie s tha t a re th e s u bj ec t o f

sc en a rio tr ai n in g. It sh o u ld a ls o b e rem em b ere d th at c o ll ap se m ay o c cu r an yw h e re o n th e pre mi se s an d m ay a ffe ct a me mb er o f s ta ff or a c om pan i on of a p ati en t. It is n o t a lw a ys po s si bl e, th ere fo re, to h av e i mm ed ia te ac c es s to the m e dic a l h i sto ry o f th e ca s u al ty.

Den tis ts h av e a n e thi c al a nd p ro fe ss io n a l d u ty to be co g n iz a nt o f ap pr op ri ate gu i de lin e s fo r th e m an a ge me n t o f a c u te m ed ic a l p ro bl em s th at mi gh t o cc u r β€”th e y a ls o n e ed to h a ve the ski ll s an d re so u rc es to f o ll ow th i s gu i da n ce .

Wh en a s se ss in g th e ris ks th a t ar e as s o ci ate d wi th trea tin g a p ati en t i t ca n b e he lp fu l to

gra de th e le vel o f sy ste mi c di se a se th at a pa tie nt h as i n term s o f h o w th a t c on d iti on lim i ts th e da il y a c tivi tie s o f a p ati en t. Th e A me ric a n So cie ty o f A n es th es io lo g is ts (A SA )

cl as s ifi ca tio n f o r fi tn es s fo r a n a es the s ia c an b e hel pfu l in as s es si n g th e si gn i fic a n ce o f a

pa tien t's m ed ic a l c o n di tio n (Tab le 19. 1). Th is s ys tem i s al so us e d b y den ta l se da tio n is ts fo r pa tien t as s es s me n t. It is u n li kel y th a t a p ati en t wh o is A S A I o r II w ill re qu i re a n y

mo di fi ca tio n s to d en ta l tre atm en t wh i ls t a pa tie n t w h o is A S A III ma y h av e a si gn i fic a n t dru g hi sto ry a n d m a y b e su s ce pti bl e to po ten tia l dru g inter ac tio n s .

Clinical risk management

Cli n ic al ri sk ma n a ge me n t is a s ys tem ati c pro c es s for th e i de n ti fi ca tio n , a n al ys is , a nd c o n tro l of a dve rs e eve n ts or po te nti al ri sk s. C li ni c ia n s s ho u ld a lw a ys :

ta ke a fu l l d o c um en te d me di ca l hi s to ry ;

be aw a re o f p o ss ib le a dv ers e rea c tio n s (if u n su re see k ex pe rt a dvi ce );

kn o w the p rin c ip le s in th e pre ve n ti o n a nd m a na g em ent o f me di ca l em erg en c ie s;

reg ul a rly reh e ars e a nd d ev elo p th e ma n ag em en t of e me rge n ci es w ith th e de n tal tea m

be fa mi li ar w ith th ei r w o rki n g e n vir o nm en t;

en s ure th a t re gu la r c h ec ks a re c arr ied o u t o n e me rge n cy e qu ip me n t a n d th e e xp iry

an d rec o rd a ll trai n in g se s si o ns ;

da tes o f em erg en c y d ru gs ; th e se c h ec ks s h o ul d be rec o rd ed .

Table 19.1 Classifications of the American Society of Anesthesiologists (ASA) Class ificatio n

Descr ipt ion

I

Fi t an d w el l

II

Mi ld s ys te m ic di s ea se th a t do e s no t in terf ere w ith d a y to da y

III

Mo d era te to se ve re sy ste mi c di se a se th at is li mi ting bu t n o t

ac tivi ty ( e. g. w e ll c on tro l le d a s thm a )

in ca p ac ita tin g . Day to d ay a cti vity ma y be a lter ed (e. g . w el l -

co n tro ll ed in s u li n - dep en d en t d ia be tes me ll itu s , an gin a pe c to r is ,

ch ro n ic b ro n ch i tis ). Th e me di ca l s ta tu s ma y be u ps et by trea tm en t

IV

Se ve re s ys tem i c d is e as e tha t i s a co n s tan t th re at to li fe; th e dis e as e is s ev ere ly li mi ti n g an d in c ap a ci tati ng (e .g. u n sta bl e

an g in a, se ver e h a em o ph i lia ). T he m ed ic a l c o n di tio n is u n sta bl e an d u n pre di cta bl e V

Mo ri bu n d, n o t e xp ec ted to s u rvi ve 24 h o ur s

Administration of drugs

Th ere a re se ver al ro u tes av ai la bl e fo r th e a dm in is tra tio n o f em erg en c y d ru gs a n d the s e a re exp an d ed u po n in Tab le 19. 2. It is w o rth n o tin g th at s o m e r ou te s are mo re u s ef ul th a n

oth e rs an d it is th e fir st f o u r tec h n iq u es th at ha ve m o st rel ev an c e to de n ta l pra c tic e. Dr ug s

ca n be a dm in i ste red v ia a n en ter al o r pa re nte ra l rou te . E nte ra l ro u te s req ui re th at a d ru g is ab so rb ed fr om th e ga s tro in tes tin a l tr ac t. Th is i n clu de s dru g s th a t a re de li vere d by bu c c al , or al , o r re cta l ad mi n is tra ti o n .

Table 19.2 Routes of drug administration Ro u te

O ns et o f

Exa mp le

actio n

Ora l

30 β€“120 mi n

As pi rin , g lu c o se

In h a la tio n

1β€“ 5 mi n

Oxy ge n , n itro u s o xid e, s a lb u ta m o l

Su b lin g u al /bu c c al

1β€“ 2m in

GTN, g lu c o se g el

In tra m us c u la r

5β€“ 15 mi n

Ad ren a li n e ( epi n ep h rin e; fo r an a ph y la xis )

Su b cu ta n eo u s

15 β€“20 mi n

Hyd ro co rti so n e

In tra ve n o us

20 β€“30 s

Ad ren a li n e ( epi n ep h rin e; fo r ca rd ia c

Glu c ag o n

arre s t)

Dia ze pa m Rec tal

6β€“ 20 mi n

Dia ze pa m su p po s ito ri es

In tra tra ch e al

1β€“ 5 mi n

Ad ren a li n e (ep in e ph ri ne )

In tra o s se o us

Up to 5 m in

Ad ren a li n e (ep in e ph ri ne )

P. 233

Routes of drug administration Oral administration

Th e o n s et o f a cti o n o f a d ru g is u su a ll y s l ow fo ll ow in g o ral a dm in i stra tio n a n d ca n b e

ad ver se ly af fe cte d b y th e p res en c e o f fo o d an d th e lo w p H. O ral a dm in i stra tio n i s o nl y

su ita b le fo r th e c on s c io u s pa tie n t an d i s no t ap prop ria te fo r th e ma jo ri ty o f dru g s us e d i n me dic a l em erg en c ie s . Th ere a re, h o w e ver, tw o a c u te s itu a tio n s w h en th e u se o f an o ra l ly

ad mi n is tere d d ru g ma y pre ve nt se rio u s s eq u ela e . As piri n sh o u ld b e us e d a s s o on as p o s si bl e in th e po s t-myo c a rdi al i nf arc tio n pa tien t an d gl u cos e sh o u ld b e gi ve n in th e c on s c io u s hy po g lyc a em ic p ati en t.

Inhalation administration

Th e o n s et o f a cti o n fo ll o w in g the a dm in i stra tio n o f a n in h a led d ru g is ve ry rap id . Ox yg en a n d bro n c ho d il ato rs s u ch as s a lbu ta mo l a re u se d s u c ce s sfu l ly by th is m eth o d. W h ere as in a

co n s ci o us p a ti en t s u pp le me n tary o xy ge n is u s u al ly gi ven at a f lo w ra te o f 2β €“6 l/m in , i n res u sc ita tio n p ro c ed ur es a m in im u m flo w r ate o f 8β €“12 l/m in is u se d.

Sublingual and buccal administration

Dru gs gi ve n by th e bu c ca l o r su b li n gu a l ro u te s are a bs o rbe d ra pid ly a cro s s th e mu c o sa w h ic h is h ig h ly va sc u la ri ze d. Th es e ro ute s ar e p ar tic ul a rly u se fu l wh e n a s ig ni fi ca n t pr o po rtio n o f th e d ru g i s me tab o li ze d by th e l iv er wh e n it i n iti al ly en ters th e h ep a ti c ci rc u la tio n . Th is i s refe rre d to as th e β€ firs t -pa ss β €™ eff ec t. Gly ce ryl trin i tr ate (GT N) h a s a si gn i fic a nt

β€ firs t -pa ss β €™ eff ec t a n d i s in e ffe cti ve w he n s w al low ed β€” ne ar ly 100 pe r c en t of it is

im me di ate ly me tab o liz e d by the li ver a fter ab s or ptio n f ro m the a li me n tary tra ct. It is

es se n tia l, th er efo re , th at p a tien ts d o n o t s wa ll o w tab le ts tha t a re in te nd ed f or a bs o rpti on ac ro s s th e o ral m u co s a .

Intramuscular (IM) administration

Th e i n tram u sc u la r ro u te h a s a s lo w er rate o f on s et o f a c ti o n th an in tra ven o u s (IV )

ad mi n is trati o n an d i s gre atl y i n flu e nc e d b y th e l oca l ti ss u e pe rfu s io n . Th e mi d -del to id re gio n of th e up pe r a rm is a g o o d si te f o r a n IM in je cti on be ca u se i t is e as il y ac c es s ib le an d is w e ll per fu se d. T h e to n g ue i s of ten c ite d as a c o n ven i en t p la c e fo r th e in tra m us c u la r

ad mi n is trati o n o f e me rge n cy d ru gs . Ho w ev er, l o ca l bl ee din g an d p o ss ib le s we ll in g c ou l d

exa c erb ate a irw a y p ro bl em s a nd , a s a co n s eq ue n ce , th is tec h n iq u e i s n ot ad vo c ate d by the au th o rs.

In tra m us c u la r ad m in is tra tio n is th e ro u te of c h o ic e f o r a dre n al in e (ep in e ph ri ne ) i n

an a ph yl ax is . Hy dro c o rtis o n e, g lu c ag o n, an d c hl o rph en ira mi n e m a y b e giv en b y th e IM ro u te i f IV a c ce s s is n o t po s s ib le . Th e m a xim u m vo l um e th at c a n be g ive n a t an y o n e in jec tio n s ite i s us u a lly 5 m l. H o we ve r, th is i s un l ike ly to b e re le va n t to de n tis ts in a n e me rge n cy s itu ati o n.

Subcutaneous (SC) administration

Th e s u bc u ta ne o u s ro u te i n vo lv es p la ci n g a d ru g in th e ad ip o se ti ss u e be ne a th the d erm is .

Th e o n se t o f a c tio n o f dru g s gi ven su b cu ta n eo u sl y is mu c h s lo w er th an fo r d ru gs gi ve n by th e IM r ou te , be ca u s e a d ipo s e tis su e i s us u a lly p oo rl y pe rfu s ed .

Intravenous (IV) administration

In tra ve n o us a c ce ss a l lo w s a dru g to b e ad m in is tere d qu i ck ly an d rel ia bl y. It i s th e p re ferr ed ro ute o f dru g ad m in is tra tio n in ad va n ce d lif e su p port. Th e m os t f req u en tly u se d me th od o f

ac h ie vin g s ec u re ven o u s a cc es s i s w i th a pl as tic c an nu l a mo u n ted o ve r a n ee dl e. Th e n ee dle

is re mo ve d o nc e th e c a n nu l a is c o rrec tly lo c a ted . Th e s i ze o f ca n n u la de pe n ds u po n wh e the r

it i s to be u se d fo r r ap id fl u id ad m in is tra ti o n (w hen a la rg e d ia me ter is i nd ic a ted ) o r f o r d ru g ad mi n is trati o n al o n e (i n w h ic h c as e sm a ll er-ga u ge ca n n u la e ca n b e u s ed ). In a n em e rge n cy si tu a tio n th e la rg es t ve in an d c an n u la a va il a ble s ho u ld be u s ed . A me tal b utte rfl y n e ed le is no t rec o m me n ded , a s ven o u s a cc e ss i s no t se cu re .

Th e s u pe rfi ci al ve in s in th e a rm ar e m o st fre qu en tly u s ed to s ite a n IV ca n n u la . In trav en o u s dru g ad m in is tra tio n re qu ir es s pe ci al is t s ki lls a n d reg u la r p rac tic e . Ma n y de n tis ts do n o t

un d erta ke IV c an n u la tio n o n a r ou ti ne b as i s a n d w o ul d be u n lik el y to g ai n va s cu l ar ac c es s in an e me rg en c y s itu a tio n w h en th e ve n ou s ci rc u la tio n m ay be c o mp ro m is ed .

Rectal administration

Th e a b so rp tio n o f dru g s fro m th e r ec tum i s si m ila r to th a t o b tai ne d fo ll o wi n g th e

in tram u s cu l ar ad mi n is trati o n o f a d ru g. Th is ro u te c an b e be n efi ci al w h en a d ru g is b ro ke n do w n by g as tric a n d in tes tin a l en z ym es o r is p H -se nsi ti ve , an d a ls o w h en a p a ti en t c a n no t ta ke me di ca tio n or al ly. In po o rly c o ntro l le d e pi le pti cs , es p ec ia ll y p a ti en ts w ith le arn i n g dis a bi liti es , d ia ze pa m a dm in is te red re cta ll y m ay b e u s ed to c o n tro l se iz u re s.

Tracheal administration

It is n o t a lw a ys po s s ib le to o bta in i n trav en o u s ac ces s in a c o ll a ps ed pa tie n t. Th is c a n be pa rtic u la rly pr ob le ma tic i n in tra ve no u s

P. 234

dru g ab u se rs a n d p a ti en ts w h o h av e se ve re h y po vo l aemi a. Ad ren a li n e (ep in e ph ri ne ),

atro p in e, a n d li do c ai n e c a n be s u cc es s fu ll y a d mi n istere d b y th e in tra trac h ea l ro u te. It i s

im pli c it i n th is tec h n iq ue th a t th e p a tien t ha s b een in tu ba ted w ith an e n do tra ch e al tu be . A la ryn ge a l m a sk ai rw ay w il l no t fac i lita te th e i n tratra c h ea l a d mi ni s tr ati on o f d ru gs .

Table 19.3 Emergency drugs: indications for use and mechanisms of action* Dr ug

Oxy ge n

In dica tio n s (do s e)

An y me di ca l

Mech an is m o f actio n

To s up pl em en t

Co mm en ts

Oxy ge n c an b e u se d

P. 235

em erg en c y

ox yg en i nta ke a n d pre ven t c e reb ra l

in m o st em erg en c ie s

hy po xi a Ad ren a li n e β €

An a ph yl a cti c

Th is d ire ctl y a cti n g

IM r ou te is m u c h

(1mg /ml )

rep ea ted a t 5

am in e h as Ξ± - an d

th e S C ro u te

(IM ) 1:1000

sh o c k (0. 5m g, mi n in terv al s if req u ire d)

sym p ath o m im etic Ξ² -ad ren e rgi c

mo re ef fec tiv e th a n

ac tivi ty. Th e Ξ± ag o ni st ac tiv ity reve rs es

per ip he ra l

vas o d ila ta ti o n a n d pre se rve s bl oo d

flo w to es s en tia l or ga n s. T he Ξ² ac tivi ty r ela xe s

bro n c hi al s mo o th

mu s cl e (di la tes th e ai rw ay s),

in cr ea se s co r on a ry blo o d fl o w an d th e

fo rc e o f m yo c ar di al co n tra cti o n, a n d su p pre ss es

hi sta m in e an d

leu k otr ien e rel ea s e Ad ren a li n e β €

Car di ac a rre st

Th is d ire ctl y a cti n g

(1mg /10ml )

rep ea ted

am in e h as Ξ± - an d

(IV ) 1:10 000

(1 m g

eve ry 3 mi n )

sym p ath o m im etic

IV r o ute pre fe rred

Ξ² -ad ren e rgi c ac tivi ty. Th e

ob jec tiv e is to

in cr ea se c ere br al an d co r on a ry per fu si o n Glu c ag o n (IM )

Dia be ti c

Th is p ol yp ep tide

IV o r S C ro u tes c a n

(u nc o n sc i ou s )

se ru m glu c o s e b y

few d en ti sts a re

hy po g lyc a em ia (1mg )

ho rm o n e in cr ea se s mo bi li z in g

gly co g en s to re s

be u se d, b u t ve ry

ski ll ed in IV ac c es s an d ab s o rptio n is

del a yed w ith th e S C ro ute

Sa lb u tam o l (in h al er)

As th ma (100 Β µg)

Ξ² 2 -ad ren e rgi c

ag o ni st ac tiv ity

rel ax es bro n c h ia l

sm o o th mu s cl e Glyc e ryl

Car di ac /c h es t

Va s od il ata tio n o f

(su b li ng u al )

me te re d d o se )

arte rie s o cc u rs

tr in itra te sp ra y

Glu c o se (o ra l)

Dia ze pa m (IV )

pa in (400 Βµ g

th e c o ro n ary

Th e c o n sc i ou s

Rap id a bs o rpti o n

Glu c o se ge ls f o r

pa tien t

glu c o s e l ev els

ab so rp tio n a re

hy po g lyc a em ic

ele va tes s eru m

Be n z od ia z ep in e

Cau ti o n: s lo w IV

mg )

ac tio n o f Ξ ³-

res pi rato ry

epi le pti cu s (10

th a t fa c il ita tes th e am in o bu ty ric a ci d ne u ro tran s m itter)

(IV)

(IV)

in jec tio n , ri sk o f

dep re ss io n . O th er

an tic o n vu ls a n ts m a y be req u ire d

Ad ju n cti ve

Hel ps rev ers e

Sl o w IV in je cti on ,

an a ph yl ax is

me dia te d

a s e co n d - lin e dr ug

tr ea tme n t fo r (10 β€“20 mg )

Hyd ro co rti so n e

ava i la bl e

Sta tu s

(an i n h ib ito ry

Ch lo rp he n am in e

rap id b uc c al

hi sta m in e -

vas o d ila ta ti o n

Ad ju n cti ve

Hel ps re du c e la te

an a ph yl ax is

an a ph yl ax is b y

tr ea tme n t fo r Ad ren a l sh o c k (200 mg )

se qu el a e o f

ca n gi ve IM . Th is i s

Can gi ve IM

red uc i ng ca p ill a ry per me ab il ity,

red uc i ng l eu ko c yte an d ma c ro ph a ge mi gra tio n an d in h ib itin g th e me dia to rs o f

in fl am ma tio n As pi rin (o ral )

My oc a rdi al

An ti -pla tel et ac tio n

If g ive n , in fo rm

(150 β€“300

ag gre ga tio n ;

sta ff

in fa rc ti o n mg )

dec re as e s pla tel et an tith ro m bo tic eff ec t re du c es

mo rta li ty a fter

ca rdi a c in fa rc tio n So l ven ts (e . g. wa ter)

So l ven ts m ay b e req ui red to

dis s o lve d ru gs th a t are p res en te d a s

pa ram e dic s /h o sp ita l

po w de r (e . g. glu c a go n ,

hy dro c o rtis o n e) * Dru g pro to c ol s a re c o n s ta n tly be in g u pd a te d an d mo di fie d as n e w sc ie n tifi c

in fo rm ati o n be co m es a va il ab le . It i s th e d u ty o f the c li n ic ia n to ke ep u p to d ate wi th cu rr en t g u ida n c e. On l y a d ul t d os e s are g ive n in thi s tab le . β€

Ad ren a li n e i s al s o kn o w n a s ep in e ph rin e .

Intraosseous administration

Th is ro u te is o fte n u se d in p ae di atri c res u s ci tati on . It ca n b e u s ef u l i n a du lts w h en no oth e r me th o d o f ac c es s is po s s ibl e. A sp ec ia l ca n n u la is u s u al ly in s erte d in to th e me du ll ar y c a vity of th e tib ia β €”as pi ra ti o n o f b o n e m a rro w in d ic a te s c or rec t p os i ti o n in g. Ad mi n is trati o n o f dru gs m u s t be fo l lo w ed b y a f lu s h o f f lu id .

Emergency drugs and equipment

Th ere a re of ten l o ca l, reg io n a l, a n d na tio n a l va riatio n s in th e gu i da n ce i ss u ed re la tin g to

wh i ch em erg en c y dru g s sh o u ld b e k ep t in a d en ta l surg ery . Cl in ic ia n s sh o u l d e xe rci se th eir pro fe ss i on a l ju dg em en t i n th e l ig h t o f c u rre nt pra ctic e a n d th e re le va n t co n te mp o ran e o us

gu id el in es a n d rec o mm e nd a ti o n s fro m a uth o ri tati ve bo d ies . P rac titi o ne rs n ee d to as s es s th e

dru gs m o s t a pp ro pri ate to th ei r n ee ds a n d th is w ill b e i n fl ue n ce d by th e ty pe o f p ra cti ce th ey ha ve . F or ex am pl e, a d di ti o n al dru g s w ill b e r eq ui red i f i n trav en o u s se da tio n o r ge n era l an a es th es ia i s u n de rtak en o n th e pre mi s es .

Th e c li n ic ia n n ee ds to b e fa mi li ar w ith th e pre pa ratio n a n d a d mi ni s tr ati on o f d ru gs i n an

em erg en c y s itu a tio n . A si n gl e d ru g is o fte n a va ila ble in s ev era l pre s en tati o n sβ €”fo r e xa mp le , hy dro c o rtis o n e i s av ai la bl e as a p o wd er fo r rec o n sti tu ti o n w ith w a te r o r as a li qu id i n a gl as s am po u le o r a pre lo a de d s yri n ge . S om e em er ge nc y dru gs are av ai la bl e in p rel o ad ed s yri ng es

an d th es e ha ve s ev era l ad va n ta g es . Th ey re qu ir e th e m in i mu m o f pre pa ra tio n (th is c a n s ave val u ab le tim e ), ca n re du c e the po s s ibi li ty o f o pe rato r erro r, an d s im pli fy tra in in g pr oto c o ls . It is p ru den t to en s u re tha t a ll e me rge n cy dru g s are c a pa bl e of a n a tu ra l ru bb er la tex - free

del iv ery . Th e freq u en tly re co m me n de d dru g s fo r u s e in th e de n ta l su rg er y i n an em erg en c y

are g ive n in Tab le 19. 3. Th e m e ch a n is ms o f ac tio n of th e dru g s are a ls o b rie fly s u mm ar iz ed .

Th ere is m er it i n de n tis ts on l y ca rry in g dru g s th at a re es s en tia l fo r th e fi rs t-lin e m an a ge me n t of a cu te m edi c al pro b le ms . It i s the a u tho rs ' o pin io n th at th e fo l lo w in g dr ug s a re th e m os t

us e fu l, fi rs t-lin e dr ug s fo r the i mm ed ia te ma n ag em en t o f a n em erg en c y: o xyg en , ad re na l in e

(1mg /ml ), s al bu ta mo l in h a le r, GTN , gl uc o s e f o r o ra l ad mi n is trati o n, an d g lu c ag o n. Tab le 19. 4

lis ts em er ge nc y eq u ipm e nt tha t sh o u l d b e pre se n t in a d en ta l su rg ery . Th is l is t is a g u id e a n d is n o t in te nd ed to be e xh a us tiv e.

Th is c h ap ter ha s n o t ta ken in to a cc o u n t dru g s th at a re u s ed in s e da tio n . If s e da tio n w ith

ben z o d ia ze pi n es i s ca rri ed o u t, th e n fl u ma z en il s h oul d be re ad il y a va il ab le in ca s e th e pa tie n t ha s be en o ver se da ted . It s h ou l d al so b e rem em b ere d th a t a c o mb in a tio n o f 50 pe r c en t

ni tro u s ox id e w i th 50 p er c e nt o xyg en m a y b e a us e fu l a n a lg es ic a n d a n xi o lyti c in a p a ti en t, po s t my o ca rd ia l i n fa rcti o n.

Table 19.4 Emergency equipment required in the dental surgery* Po c ke t ma s k β €

Se lf -in fl ati ng b ag , va lv e, a n d ma s k w i th res er vo ir β € Oro p h ary n gea l a irw a ys β € ΅

Nas o ph a ryn g ea l ai rw ay s β € ΅ Oxy ge n th era py m as ks

Tu bi ng a n d ap pr op ri ate c on n e cto rs to a ttac h o x yge n c yli n de rs to o xyg en ma s ks Sy rin g es a nd n e ed les to de liv er dru g s by a pa ren te ral ro u te in a n e me rge n cy IV c a n n ul ae a n d ad h es ive ta pe

In d ep en d en tly po w er ed po rta bl e su c tio n a pp a ratu s w ith w i de -bo re as p ira tio n tip s. Bl o o d p res s u re mo n ito r Β §

* E qu ip m en t s h ou l d be fre e fro m n atu ra l ru bb er la tex . β€

Th es e wi ll a llo w th e pr o vis io n o f i n term itten t po sitiv e p re ss u re ven ti la tio n to th e

lu n gs .

β€ ΅ Β§

A ran g e of si z es s h o ul d be a va ila b le.

Th is h a s n o t u su a ll y b ee n rec o m me n ded a s a n es s en tia l item . Ho w e ver , it m a y b e

he lp fu l in th e as s es s me n t o f th e u n we ll pa tie n t.

It is th e res p on s ib il ity o f th e de n tis t to en s u re th at r egu l ar c he c ks a re u n d erta ke n o n al l em erg en c y e qu i pm en t a n d em erg en c y d ru gs . Th e se c h ecks s h o ul d be rec o rd ed .

Management of emergencies

Tab le 19. 5 de tai ls th e ma n a ge me nt o f m ed ic a l e me rgen ci es th a t m ay o cc u r i n d en tal

pra cti ce . F ai n tin g (va s o vag a l a ttac k, sy nc o pe ) i s th e m o s t fre qu e ntl y e n co u n tere d ca u s e o f

co ll a ps e in d en ta l pra cti ce . Th e br ief l os s o f co n sci o us n es s th a t o cc u rs in fa in tin g is du e to a n ab ru pt f al l i n c ar dia c o u tpu t th a t le a ds to a re du ctio n i n ce reb ra l bl o o d f lo w . Th e de n ta l tea m sh o u ld re co g n iz e th e c h ara c teri sti c si gn s th a t pre ced e c o ll ap s e a n d tak e a pp ro pr ia te

ac tio n β€” so m etim e s th i s ma y pre ven t lo s s o f c o n sc i ou s n es s . It sh o u l d b e rem em be red th a t, oc c as io n a ll y, s yn c o pe ma y n ot h av e a b en i gn c a u se and c a n be a ss o c ia ted w ith s eri o u s ca rdi a c dys rh yth m ia s o r a tra ns ie n t is c h ae mi c atta ck (m in i -stro ke ).

Al go ri thm s fo r ca rd io p ul mo n a ry res u sc i ta tio n a re c ons ta n tl y be in g rev is ed a nd th e cl in i ci an ne ed s to be a w are o f cu rr en t p ro toc o ls . Tab le 19. 6 o u tl in e s the b as ic p ro c ed ur e f o r th e

as se s sm en t a n d ma n a ge me nt o f th e co l la ps ed pa tie n t. Wh en a p ati en t h a s lo s t c o ns c io u s n es s th e c li ni c ia n n ee ds to c o n sta n tly as s es s a nd m o n itor th e pa tie n t's ai rw ay , bre a th i n g, a n d

ci rcu l ati o n. T h is se qu e n ce o f ac tio n s is o f ten a bb rev ia ted to β € AB C β€™, a n d thi s ac ts a s an

ai de -mΓ ©mo ir e . Th is e na b les th e c li ni ci a n to qu i ck ly as c erta in if the p ati en t i s bre ath i ng a n d

stil l h as a c ar dia c o u tpu t. If the pa tie n t h as s u ffere d a re s pi rato ry o r c a rdi ac a rre st, th e n sp ec ia li st he lp i s u rge n tly req u ire d. In th e me a ntime th e de n ta l tea m mu s t p erf or m

ca rdi o pu l mo n a ry res u s ci tati on to en s u re so m e ce reb ra l b lo o d fl ow . T hi s w ill h o pe fu l ly pre ven t irre ver si bl e h y po xi c bra in

P. 236 P. 237

da ma ge . In th e eve n t o f a ca rd ia c ar res t, a s uc c es sfu l o u tc o me is g re atl y i nf lu e nc e d b y th e

P. 238

ea rly ap pl ic a tio n o f ad va n ce d l if e s u pp o rt s ki lls . Th e m ajo ri ty o f pr im ary a du lt ca rd ia c ar res ts pre se n t in v en tri cu la r fib ril la tio n a n d pa tie nt su rv iva l is de pe n de nt u po n e arl y d ef ib ril la tio n .

Table 19.5 Medical emergencies and their management Cau ses

Sig ns

Man ag em ent

Tra ns ie n t

Wea kn es s , di z zi n es s ,

Pl ac e pa tie n t in a s u pi n e p o s itio n

ce reb ra l i sc h a em ia

na u s ea , co n fu s io n ,

lev el o f th e h ea rt to i mp ro ve

Fa in t

hy po ten s io n a n d Pre di sp o si n g

fac to rs in c lu d e

hy po g lyc a em ia ,

an xi ety, fea r, p ai n , an d fa tig ue

pa llo r , sw e ati ng ,

ta c h yc ard ia f ol lo w ed by a bra dy ca rd ia , lo ss o f

co n s ci o us n es s .

Mi n or c on v ul s io n s o r in co n ti ne n ce c a n oc c u r.

wi th th e l eg s el eva ted ab o ve th e ce reb ra l f lo w

A pa tie nt w ho is s ittin g m ay

lo w er th e ir h ea d b y pl ac in g it

betw e en th e ir kn ee s , thi s is n o t as e ffe cti ve as l yi ng a p ati en t do w n .

Lay a pre gn a n t p atie n t o n h er si de .

Ad mi n is ter ox yg en .

Rea ss u re th e rec o ve rin g pa tie n t, a g lu c o se -ric h dr in k m a y b e he lp fu l.

Wh en a me mb er o f th e de n tal

te a m r ec o gn iz e s tha t a p ati en t i s lik el y to fa in t th e pa tie n t s ho u l d be pl ac ed i n a s up in e

po s itio n β €” th i s ma y pre ve n t lo s s of c o ns c io u s ne s s.

If th e pa tie nt fa ils to reg ai n

co n s ci o us n es s p ro mp tly o th e r

ca u se s o f l o ss o f co n s c io u sn e ss mu s t be co n s id ere d. Hyp og ly ca em ia An xi ety , in fe c ti o n

Co ld a nd c la m my

Co n sc io u s : gl u co s e dri n k, ta bl ets

Ir rita bi lity ,

Un c on s c io u s : in tra m us c u la r

ski n , trem bl in g . co n fu s ed ,

ag gre ss i on , a n d un c o o pe rati ve beh a vi o ur .

Dro ws in e ss a n d dis o ri en tati o n .

or ge l

glu c a go n (1 mg /ml ) o r an IV

glu c o s e i n fu si o n (25 m l o f 50% so lu ti o n) .

Ad mi n is ter o xyg en

Al w ays m o n ito r p a tien t an d ma in ta in a irw a y.

Tra ns fe r to h o sp ita l.

Ep il ep tic se iz u re Kno w n e pi le ptic

Los s o f

Pro te ct fro m in ju ry (re mo ve

no n - co m pl ia n ce

Mu s cl e rig id ity

pil lo w s a ro un d p ati en t i f th es e

Po o rl y co n tro l led o r wi th dru g re gi me Stre ss ,

hy po g lyc a em ia ;

ma y a c co m pa n y a

fai n t. Ov erd o se o f lo ca l a na e sth eti c

ma y c a u se s ei zu re s .

co n s ci o us n es s

fo ll ow e d by jerk in g mo ve me n ts;

in co n ti ne n ce m ay oc c u r.

Co n fu si o n m ay be pre se n t du ri n g rec o ver y

po ten tia l ly h arm fu l o bje c ts , u s e ai d th e ir pro te cti o n) . Ad m in is ter ox yg en a n d ma in ta in a irw a y if po s si bl e. If th e p a ti en t c a n be dis c h arg ed h o m e, e ns u re th at th e y a re ac c o mp an i ed β€” th e y

mi gh t h a ve po s t-ic ta l c on f us i on . Sta tu s ep il ep ti c us i s pro b a ble i f

se iz u re co n tin u e s in e xc es s o f 7 mi nu te s; th ere fo re em erg en c y se rvi ce s sh o u ld b e c a ll ed . If

sta tus e pi le pti cu s is d ia gn o s ed

dia z ep a m ( up to 10 mg ) b y s lo w IV i n jec tio n m a y b e gi ve n; it is no t a l wa ys e ffi c ac io u s As th ma Pre -exi sti n g di se a se

Bre a thl es s ne s s wi th

Sa lb u tam o l i n h al er o r n eb u liz e r

co n tro ll ed , an x iety ,

exp ira tio n . If

a c o m fo rta ble p o si tio n . If th e re is

th a t i s po o rly

in fe cti on , e xe rci s e, exp o su re to a n an tig en

wh e ez in g o n

un tre ate d, b rea th in g ma y b ec o m e

in cr ea si n gl y d iff ic u lt

an d o xyg en . P la c e th e p ati en t i n no im pro ve m en t s u mm o n em erg en c y se rvi c es

Hyd ro co rti so n e IV o r IM m ay b e giv en

Sta tu s as th m atic u s is a l if e th re a te n in g c on d iti on Ch es t p ai n An g in a , my oc a rdi al in fa rc ti o n

Us u al ly a cr us h in g

Su b lin g u al GTN, ox yg en , P la ce

irre gu la r pu ls e, ma y

po s itio n β €” co n s ul t w ith th e

retro s tern a l p a in , exp eri en c e

bre ath le s sn e ss ,

na u s ea , o r vo m iti n g

pa tien t in a c o mf or tab le

pa tien t. In a p a ti en t w i th a

kn o wn hi s to ry o f an g in a a sk if

th e sy mp to ms a re typ ic al . Ca ll

em erg en c y s er vic es i f pa in d o es no t su b s ide i n 3 m in u tes

(po s si bi li ty o f a my oc a rdi al in fa rc ti o n (M I)). P os s ib le

ad mi n is trati o n o f o ra l as pi rin

(300 mg ) i f MI s u s pe cte d; n itro u s ox id e a n d o xyg en , i f a va il ab le ,

ca n be h e lp fu l to red u ce pa in an d

an xi ety. Mo n ito r. If lo s s o f

co n s ci o us n es s fo l lo w th e pro to c o l fo r c ar di op u lm o n ary res u sc ita tio n . Hyp erve n til ati on Stre ss , p ai n , o r

Rap id b rea th in g,

Rea ss u re

Th is is o f ten a

tr em bl in g, d iz z y,

Sto p trea tm en t

exp ec tati o n o f p a in . res po n s e to

un f oc u s ed fe ars .

Can be a ss o ci a te d wi th ch ro n i c

gen e ra liz e d an xi ety dis o rd er

ta c h yc ard ia ,

fai n t, sw e ati n g

Pa ra es th es ia , mu s c le

En s u re co m fo rta bl e po s iti o n Reb rea th e e xp ire d ai r

pa in /s tiff n es s

Can le ad to teta n y. Pa tie n ts c a n

co m pl ai n o f c h es t pa in

An a ph yl ax is Ex po s u re to an

In i tia l f lu s h in g o f th e

Lay fl at, e le va te l eg s

th e p ati en t h a s

fo ll ow e d by o ed em a

ox yg en

an tig en to wh i ch bee n se n si tiz e d,

co m mo n ly d ru gs (mo s t n o tab ly pen i ci ll in s ) o r

na tu ra l ru bb er

la te x. A n a ph yl ax is

to lo c al a na e sth e ti c is ex trem el y ra re

ski n m ay o c cu r

of th e he a d a n d ne c k. A lter ed

se n sa tio n s s uc h a s

pa rae s th e si a a ro un d mo u th a nd f in ge rs .

Pa ll o r, c yan o s is w i ll ac co m pa n y ac u te

Ma in ta in a ir wa y an d a dm in i ste r Cal l exp er t as s is tan c e

Im m ed ia te ad re na l in e (0. 5 m l o f 1: 1000) IM ; re pe at if n ec es s ary Hyd ro co rti so n e a nd

ch lo rp h en i ram in e a re 2nd -lin e dru gs

bre ath in g d iff ic u ltie s wi th bro n c h o sp as m an d /o r se ve re

hy po ten s io n . Lo s s o f co n s ci o us n es s a n d ca rdi a c arre s t ca n oc c u r Cere br ov as c u la r ac c id en t Is c h ae mi a,

A stro k e (p a rtia l o r

Lay fl at a n d ad mi n is ter o xyg en ,

em bo li sm i n a

on e s id e o f th e

Su m mo n e xp ert he lp .

ha em o rrh a ge , o r ce reb ra l a rter y

to ta l we a kn es s o n

bo dy ), dy sa rth ri a,

ap h as ia , h em ip le gia , an d po s s ibl e lo s s o f co n s ci o us n es s

ma in ta in a irw a y, mo n i tor

Loc a l a n a es th etic to xi c ity Ove rd os e

Ligh th e ad ed ne s s;

Ad mi n is ter ox yg en , m ai n ta i n a

dis tu rba n c es .

Cal l em erg en c y s e rvic e s

vis u al o r h ea rin g

Ag ita tio n , c on f u si on , se iz u res , re sp ira to ry

co m fo rta ble po s iti o n Mo n ito r pa tie n t

dis tre ss . Lo s s of co n s ci o us n es s ,

res pi rato ry a n d ca rdi a c ar res t. Ad ren a l sh o c k ( Ad di so n ia n c ri si s) Stre ss i n a pa tie n t

Pa ll o r, ra pi d w e ak

Lay pa tie n t fl at.

su p pre ss io n ( e. g.

blo o d p res su re , lo s s

hy dro c o rtis o n e ( IV is th e

wh o h a s a dre na l

in du c ed b y d is ea s e

or lo n g term s tero i d

pu ls e, ra pi dly fa ll in g of c o ns c io u s ne s s

th e ra py)

Ad mi n is ter ox yg en , 200 m g pre fer red ro u te bu t IM c an be us e d)

Su m mo n e xp ert he lp .

Th is is r are β€” co n s id er o th e r ca u se s

Res pi ra to ry ar res t Sta tu s as th ma tic u s, ai rw ay o bs tru cti o n

No br ea thi n g;

Fo ll o w ba s ic l ife s u pp o rt

(in iti al ly )

If u n trea ted , w il l b ec o me a

ce n tr al pu l se p res en t

al go ri th m fo r r es cu e b rea th in g. ca rdi a c ar res t

Car di ac ar res t My oc a rdi al in fa rc ti o n

Circ u la to ry co l la ps e An a ph yl a xis Hyp o xia

Res pi ra to ry a rre st

Un c on s c io u s ; n o ce n tr al pu l se

Fo ll o w ba s ic li fe s u ppo r t

al go ri th m ; th i s wi ll in v ol ve

as se s sm en t o f res p on s iv en e ss ,

ai rw ay , bre ath i ng , a nd c ir cu la tio n (AB C ). E xpe rt as s is tan c e sh o u ld

be su m m on e d as s o o n as p o s si bl e to en s ur e e a rly

def ib ril la tio n ar res t (i f i n di ca ted )

an d ad m in is tra tio n o f em erg en c y dru g (a dva n c ed li fe s up po rt al go ri th m s )

So m e au th o riti es s u gg es t th at an IV b en z o di a ze pi n e s h o u ld be g ive n to th e pa tie nt wh o h a s s eiz u re s fo ll o wi n g ov erd o se o f lo c al an a es th eti c. T h is is n o t a dv is ed

bec a u se i t ca rri es th e ris k o f res p ira to ry de pre s sio n

Table 19.6 Assessment and management of the collapsed adult patient. Ass ess Is th e pa tie n t co n s ci o us ?

Actio n If ye s , is ex pe rt h el p req u ire d? If n o , ca ll fo r h el p f ro m sta ff

Airw a y

Es ta bli s h an d m ai n tai n ai rw ay *

B rea th in g (lo o k ,

If b rea th in g, i s ex pe rt h el p

lis ten , a n d fee l)

Co mm en ts En s u re tha t i t is sa fe to ap pro a ch be fo re

as se s si n g th e pa tie n t Is th e re de bri s to

rem o ve fro m th e mo u th ?

req ui red ?

If n o t b rea th in g, c a ll

em erg en c y s er vic es β € an d sta rt arti fic ia l ve nti la tio n β € ΅

Circ u la tio n

If ye s , co n tin u e w ith ve n til ati on If n o , s ta rt e xte rn al c ard ia c co m pre ss i on s

Pa lp ate a m ajo r pu l se (e. g. c a ro tid pu l se )

* A n en d otr ac h ea l tu b e o r la ryn g ea l ma s k a irw a y sh ou ld o n ly be u s ed if th e op era to r i s sk il le d i n th ei r u s e. β€

If a p ers o n is a va il ab le s en d th em to m ee t th e e merg en c y te am a t th e en tra nc e

to the b u ild in g o r cl in i c. β€ ΅

Po s iti ve pre ss u re ve n ti la tio n s sh o u l d i de al ly be un de rta ken w i th a se lf -in fl ati ng

ba g c o n n ec ted to o xyg en . A s in g le re sc u er ma y pre fer to u s e e xp ire d ai r w ith a po c ket ma s k a tta ch e d to a n o xy ge n su p pl y.

Projects 1.

Pa tie n ts w h o re qu i re sp ec ia li s t ca re fo l lo w in g thei r m ed ic al em e rge n cy w ill n e ed to be

tr an s fe rred to a n a pp ro pri ate u n it. In th e UK pa ra me di cs w i ll tra ns fe r th e pa tie n ts. Sp ec if y w h a t in fo rm a tio n yo u wo u ld n e ed to g ive in the β € ha n d -ov er β€™ of th e pa tien t to the e me rge n cy /s pec i al is t c are te am .

2.

Id en ti fy th e e xpe rt c o mm itte es /pa n el s th at i s su e a dvi ce o n th e e m erg en c y d ru gs a n d

equ i pm en t th a t s h ou l d be h el d a t y ou r de n tal pra c tic e. A s ce rta in h o w fr equ e n tly the dru gs a n d eq u ip me nt are c h ec ke d in th e cl in i ca l en vi ro n me n t in w h ic h y o u w o rk.

Au th o rs : Title:



Co py rig ht Β © 2003 Ox fo rd U ni ve rsi ty P re ss > B ac k o f B oo k > Re s ou rc e s

Resources

Appendix Th es e mo u th wa s h es s h o ul d be ke pt i n th e refr ig era tor. Th e sh e lf- lif e o f mo u th w as h es i s ap pro xi m ate ly 2 w ee ks .

Chlortetracycline mouthwash Fo rm u la ry. To m ak e 200 ml (10 m l tds ), u s e:

ch lo rte tra cy cl in e, 4 g

mu c ila g e o f tra ga ca n th , 50 m l

dis til le d wa ter to 200 m l Mo n ito r pa tie n t fo r or al c an d id os is .

Triamcinolone (plain) mouthwash Fo rm u la ry. To m ak e 200 m l ( 10 ml td s) u se :

tr ia mc in o lo n e fo r the c o n ce n trati o n req u ire d (se e fo ll o wi n g tab le )

mu c ila g e o f tra ga ca n th , 50 m l

dis til le d wa ter to 200 m l

Co nc en tr atio n

Am ou n t o f st ero id

0. 25 mg

5 mg tri am c in o lo n e

0. 5 mg


0. 75 mg


1. 0 mg


2. 0 mg


Mo n ito r the p ati en t f or o ra l c a nd id o si s . Sy ste mi c ab s or ptio n of th is mo u th w as h i s li kel y, pa rtic u la rly in ca se s o f w i de sp rea d o ra l u l ce ra ti o n a nd a t h ig h co n c en tra tio n o f ste ro id .

Triamcinolone with 2 per cent chlortetracycline mouthwash Fo rm u la ry. To m ak e 200 ml (10 m l tds ), fo l lo w th e in s tru cti ons f o r tri am ci n o lo n e pl ai n bu t

ad d 4 g ch l or te tra cy cl in e. Mo n ito r fo r o ra l ca n di do s is . S ys tem ic a bs o rp ti o n is l ik ely , pa rtic u la rl y in c a se s o f w i de sp rea d or al u lc era tio n an d at h igh co n c en tra tio n o f ste ro id .

Suggestions for further reading and reference sources

P. 240

Bo o ks

Ora l P ath o lo g y: J V S oa m es a n d J C S o u th am , 3 r d E di ti o n , Ox fo rd Un i ve rsi ty P res s

(1988).

Me di ca l Pr ob le ms i n De nti stry : C S cu l ly, R A Ca ws o n , 4 t h E di ti o n , Wri gh t (1998).

Ora l Dis ea s es : C S cu l ly, S Fl in t, S R P o rter , M Du nitz , 2 n d E di ti o n (1996).

Co lo u r A tla s o f Ora l Dis ea s es : G Las ka ri s Th ie me 2 n d E di ti o n (1994).

Co lo u r A tla s o f Ora l Me di c in e: Wil li am R Tyl de sl ey , 2 n d E di ti o n , Mo s b y -Wo lfe (1994).

Es s en tia ls o f M ic ro bi o lo gy fo r Den ta l S tu de n ts: T Ba gg , TW M ac F arl an e , IR P o xto n , CH

Tex tb o o k o f Der ma to lo gy : A Ro o k , DS Wil ki ns o n , F GJ E b lin g (E d ito r). 5 t h E di ti o n (4

Mi ll er, A J S m ith , O xfo rd U n ive rs ity Pr es s (1999).

vo lu m es ) B la c kw el l Sc ie n tifi c P ub li ca tio n s , O xfo rd (1992).

(NB P le as e co n s u lt th e mo s t u p -to -da te e di tio n . ) Jo u r n als:

Ora l Dis ea s es

Ora l On c o lo g y

Ora l M ed ic in e an d P ath o lo g y

Ora l Me di ci n e, O ra l S ur ge ry, O ra l P a tho l o gy an d O ral Ra d io lo g y

Criti ca l Rev ie ws i n O ral B io l o gy an d O ra l M e dic i ne

Usefu l W ebsit e Add res ses:

Co ch ra n e Co ll ab o ra ti o n β€“ http ://w w w .c o c hr an e .o rg

Co ch ra n e Ora l He al th Gro u p co n ta in s li n k to O HG a bstrac ts w ith o u t se a rch i n g th e w h ol e

Cli n ic al E vid en c e β€“ http ://w w w .c li n ic al ev id en c e. c om

Co ch ra n e Lib rar y β €“ http ://w w w .c o c hr an e -or al .m a n. ac .u k

Tyldesley_s Oral Medicine 5th Ed - Field

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