DM Thappa - Essentials in Dermatology, 2nd Edition

Essentials in Dermatology (with Multiple Choice Questions)

Essentials in Dermatology (with Multiple Choice Questions)

Second Edition

Devinder M Thappa

MD, DHA, MNAMS, FIMSA

Professor and Head Department of Dermatology and STD Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER) Pondicherry, India

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Essentials in Dermatology (with Multiple Choice Questions) © 2009, Jaypee Brothers Medical Publishers All rights reserved. No part of this publication should be reproduced, stored in a retrieval system, or transmitted in any form or by any means: electronic, mechanical, photocopying, recording, or otherwise, without the prior written permission of the author and the publisher. This book has been published in good faith that the material provided by author is original. Every effort is made to ensure accuracy of material, but the publisher, printer and author will not be held responsible for any inadvertent error(s). In case of any dispute, all legal matters are to be settled under Delhi jurisdiction only.

First Edition: 2003 Second Edition: 2009 ISBN 978-81-8448-558-5

Typeset at JPBMP typesetting unit Printed at Ajanta

Preface to the Second Edition The second edition of Essentials in Dermatology (with Multiple Choice Questions) is being published 6 years after the appearance of the first edition. The encouraging response to the first edition prompted me to revise the book, keeping in view the comments received, and changing trends in the field of dermatology. This new edition incorporates differential diagnosis for each entity or group of entities to further understand the subject critically. Three new chapters—Skin in Systemic Diseases, Skin Changes of Pregnancy and Old Age, and Antiretroviral Therapy (ART) have been added. The existing chapters have been updated and treatment guidelines revised. Newer entities have been included under various chapters, but not at the cost of brevity and conciseness. For better understanding of the text, better photographs and clinical illustrations have been incorporated. The section on multiple choice questions has been considerably expanded, and this section has been divided into two—for PG entrance examinations and for postgraduates in dermatology. Additional mnemonics have been included in the useful medical mnemonics section. The objective of this edition remains the same—to serve as an aid for beginners in dermatology and those aspiring for PG entrance examinations. The making of the revised edition of this book involved a number of people besides myself. Many of the chapters were revised with inputs from my senior residents Dr Rashmi Kumari, Dr Amiya Kumar Nath and Dr Abarna Devi and junior residents Dr Nidhi Singh, Dr Abhijit Chougule, Dr Kishan Kumar Agarwal, Dr Balaji Adityan, Dr Sowmya Kaimal and Dr Sakthi Kandan. The photographs utilized in the book have been possible due to the Medical Illustration Department of the hospital, and the digital cameras of my postgraduates, making the new edition a colorful experience. My laboratory technician Mr Samsudeen deserves a mention for his skill in the staining and preparation of laboratory material for photomicrography. The final making of this edition has involved the support and cooperation of all my esteemed colleagues, patients and the forgotten names of residents (who worked for the first edition of the book). As always, this edition is open to constructive criticism and suggestions for its further improvement. Devinder M Thappa

Preface to the First Edition Dermatology, the science of the skin, was one of the many specialties, which evolved from general internal medicine during the course of the nineteenth century. In India, recognition of dermatology as a specialty distinct from internal medicine is recent; it has still not grown to its full stature in practice and teaching. In spite of having some share in the curriculum, dermatology remains a neglected subject because of its non-inclusion in the qualifying examination at MBBS level. There has been an explosion of knowledge—easily documented by the size of standard dermatology textbooks, whose length has increased from an average of 1000 pages to the most recent editions of Fitzpatrick and of Rook, which are more than 3000 and 3600 pages, respectively. Expansion has been greater on the surgical and cosmetic side of the specialty, which barely existed 50 years ago. Such vast knowledge is difficult to grasp in 3 years course of MD dermatology, venereology and leprology, sometimes may be at the cost of another. So there was need for a short textbook for postgraduates who have just joined the specialty to have the glimpse of the subject and understand the basic dermatology before venturing for detailed standard textbooks. There is lack of simple but up to date book for undergraduates who are preparing for Postgraduate Entrance Examination. Though market is flooded with a number of books, many of them are not even framed by dermatology specialty individuals and lack correct and appropriate information. This prompted me to write this book to fulfill the needs of students aspiring for entering in postgraduate courses in reputed institutes of India. The material in this book is based on the standard textbooks and latest information from specialty journals. Introduction to MCQs is a unique section in this book to guide the students. The multiple choice questions are taken from a number of sources to sensitize the student to know certain subject areas in this specialty thoroughly and accordingly the book section gives relevant points highlighted for quick revision of facts. The suggestions and healthy critical remarks will be very much appreciated to improve this book. Devinder M Thappa

Acknowledgements I would like to thank those who helped me to update chapters 1. Dr Balaji Adityan for updating • Principles of Diagnosis in Dermatology • Bacterial Infections • Viral Infections • Fungal Infections • Skin Changes in Pregnancy and Old Age 2. Dr Sakthi Kandan for • Infestations • Disorders of Hair and Nails • Metabolic and Nutritional Disorders • Skin in Systemic Diseases 3. Dr Sowmya Kaimal for • Pediatric Dermatology • Human Immunodeficiency Virus Infection (HIV) and Acquired Immunodeficiency Syndrome (AIDS) 4. Dr Amiya Kumar Nath for • Eczema • Connective Tissue Disorders • Genetics and Genodermatoses 5. Dr Abhijit Chougule for • Differential Diagnosis for Leprosy • Treatment of Leprosy 6. Dr Kishan Kumar Agarwal for • Urticaria, Angioedema and Pruritus • Disorders of Sebaceous, Eccrine and Apocrine Glands 7. Dr Nidhi Singh for • Cutaneous Tuberculosis and Atypical Mycobacterial Infections • Vesiculobullous Disorders • Pigmentary Disorders Following residents helped in framing MCQs for postgraduates 1. Dermatology Basics • Dr Abhijit Chougule • Dr Kishan Kumar Agarwal 2. Clinical Dermatology Part -I • Dr Rashmi Kumari

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Essentials in Dermatology • Dr Balaji Adityan • Dr Ajay Kumar Singh • Dr Anuradha Priyadarshini • Dr Tukaram Sori 3. Clinical Dermatology Part -II • Dr Malathi • Dr Sathyamoorthy 4. Sexually Transmitted Diseases • Dr Sowmya Kaimal • Dr Rajalakshmi 5. Leprosy • Dr Abarna Devi • Dr Sakthi Kandan

Reviews “…Most of the dermatology textbooks are too much voluminous for undergraduate students already overburdened with other heavy weight subjects. Not only undergraduates, beginners at the postgraduate level also face problem to acquire basic conception from such large books. So there is always a need for a concise book which can provide clear basic conception and up-to-date knowledge to the students….will be of immense help to the postgraduate entrance examinees….should be collected in all undergraduate medical college libraries for the benefit of the students…” Indian J Dermatol 2003; 48(4): 248. “…The stated aim of the book is to have a short textbook for new entrants to postgraduate studies in dermatology which could glimpse of the subject and understand basic dermatology before venturing for detailed standard textbooks. The second aim stated is to fulfill the needs of students aspiring for entering in postgraduate courses in reputed institutes….well written and fulfill the stated aims…An approach to attempting MCQs appears to be a very useful chapter….strongly recommend this book to the new entrants in specialty training and those preparing for admission to postgraduate courses…” Indian J Dermatol Venereol Leprol 2004; 70(6): 393.

Contents

SECTION 1: DERMATOLOGY 1. Ten Most Influential People in Medicine and Dermatology .................................................... 3 2. History of Dermatology in the World ............................................................................................ 5 3. Microanatomy of the Skin ................................................................................................................ 8 4. Physiology, Biochemistry and Immunology of the Skin ......................................................... 13 5. Principles of Diagnosis in Dermatology ..................................................................................... 16 6. Bacterial Infections .......................................................................................................................... 31 7. Viral Infections ................................................................................................................................. 43 8. Fungal Infections ............................................................................................................................. 57 9. Infestations ........................................................................................................................................ 72 10. Papulosquamous Disorders ........................................................................................................... 82 11. Eczema ................................................................................................................................................ 99 12. Vesiculobullous Disorders ........................................................................................................... 114 13. Cutaneous Tuberculosis and Atypical Mycobacterial Infections ........................................ 127 14. Connective Tissue Disorders (Collagen Vascular Disorders) ............................................... 134 15. Pigmentary Disorders ................................................................................................................... 148 16. Keratinization Disorders .............................................................................................................. 156 17. Urticaria, Angioedema and Pruritus .......................................................................................... 166 18. Drug Eruptions, Erythema Multiforme, Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis ................................................................................................. 172 19. Disorders of Sebaceous, Eccrine and Apocrine Glands ......................................................... 180 20. Disorders of Hair and Nails ......................................................................................................... 189 21. Metabolic and Nutritional Disorders ......................................................................................... 198 22. Genetics and Genodermatoses .................................................................................................... 210 23. Skin in Systemic Diseases ............................................................................................................ 222

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Essentials in Dermatology 24. Skin Changes of Pregnancy and Old Age ................................................................................. 237 25. Pediatric Dermatology .................................................................................................................. 240 26. Benign, Pre-malignant and Malignant Tumors of the Skin .................................................. 246 27. Topical Formulary and Key Systemic Drugs ............................................................................ 257 28. Dermatosurgical Procedures ........................................................................................................ 272

SECTION 2: SEXUALLY TRANSMITTED DISEASES AND HIV INFECTION 29. Historical Milestones in Sexually Transmitted Diseases ...................................................... 279 30. History Taking and Examination in Sexually Transmitted Diseases (STDs) .................... 282 31. Sexually Transmitted Diseases ................................................................................................... 288 32. Human Immunodeficiency Virus Infection (HIV) and Acquired Immunodeficiency Syndrome (AIDS) ....................................................................................... 309 33. Antiretroviral Therapy (ART) ..................................................................................................... 318

SECTION 3: LEPROSY 34. Historical Milestones in Leprosy ................................................................................................ 327 35. History Taking and Examination in Leprosy ........................................................................... 330 36. Clinical Leprosy ............................................................................................................................. 333 Multiple Choice Questions .................................................................................................................... 351 Some Useful Medical Mnemonics ....................................................................................................... 441 Terminology ............................................................................................................................................. 447 Answers ..................................................................................................................................................... 465 Index ........................................................................................................................................................... 469

Ten Most Influential People in Medicine and Dermatology

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Ten Most Influential People in Medicine and Dermatology

THE MILLENNIUM AND MEDICINE: THE TEN MOST INFLUENTIAL PERSONS 1. Louis Pasteur (1822-1895): Proposed the “germ theory”. He first associated a specific micro organism (bacillus) with a specific disease (anthrax). He developed the method of pasteurization—a heating process that kills bacteria in milk, wine and other liquids. He was also a pioneer in stereochemistry. 2. Robert Koch (1843-1910): The first to isolate the anthrax bacillus (1876). In 1883, he published a method of preventive inoculation against this disease. In 1882, he announced the discovery of tubercle bacillus and in 1883; he discovered the cause of cholera. He was awarded the nobel prize in physiology or medicine in 1905. 3. Rudolf Virchow (1821-1902): Founded Cellular pathology. His concept that the basis of disease is the cell, the essential functional and structural unit of the body, was of monumental importance as a basis for understanding the cause, the process and the results of the disease. 4. Gregor Mendel (1822-1844): Formulated the laws of heredity. Mendel’s work laid the mathematical foundation of the science of genetics.

5. Francis Crick (1916-) and James Watson (1928): Accredited with determining the molecular structure of DNA, the chemical substrate of heredity, which is regarded as the most important discovery of the 20th century in medicine and science. They were awarded the nobel prize in 1962 sharing it with Maurice Wilkins (1916). Currently, Crick is associated with the Salk Institute for biological studies in San Diego while Watson is the director of the Cold Spring Harbor lab in southeastern New York. 6. Marie Curie (1867-1934): Discovered radioactivity and was given the nobel prize in 1903. The discovery formed the basis of radiation therapy. In 1911, she was again conferred the Nobel Prize in chemistry for her discovery of radium and polonium. 7. Edward Jenner (1749-1823): Introduced the inoculation for smallpox at the end of the 18th century, which is considered one of the greatest triumphs in the history of medicine. 8. Karl Landsteiner (1868-1943): Called the “father of blood grouping” - a concept without which blood transfusion would not be possible. In 1901, he showed that there are at least three major types of blood. Landsteiner was awarded the noble prize for his work in 1930.

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Essentials in Dermatology 9. Wilhelm Rontgen (1845-1923): Discoverer of X-rays in 1895 and nobel prize winner in Physics in 1901. The value of X-rays in the diagnosis and treatment was recognised and accepted almost from the outset of their discovery. 10. Sigmund Freud (1856-1939): Considered the founder of psychoanalysis, he believed that a complex of repressed and forgotten expressions underlies all abnormal mental states and that infantile mental processes are important in later development.

TEN MOST INFLUENTIAL PERSONS IN DERMATOLOGY, VENEREOLOGY AND LEPROSY: INDIA 1. Dr. JS Pasricha: Pioneer of pulse therapy in pemphigus, contact dermatitis in India. 2. Dr. LK Bhutani: Clinical dermatology, “Bhutani’s Colour Atlas of Dermatology”, photobiology. 3. Dr. Dharmendra: “Father of leprosy” in India. 4. Dr. RV Rajam and Dr. PN Rangaiah: Monograph on donovanosis. 5. Dr. VN Sehgal: For his literary contribution in dermatology, venereology and leprosy. 6. Dr. Patrick Yesudian: Clinician par excellence, known for “Patrick Yesudian sign” for palmar freckling in neurofibromatosis type 1. 7. Dr. KC Kandhari: Established department of dermatology at AIIMS. 8. Dr. Gurmohan Singh: contribution to Indian and community dermatology. 9. Dr. Surinder Kaur: Established department of dermatology at PGIMER, Chandigarh. 10. Dr. Sardarilal: First editor of Indian Journal of sexually transmitted diseases, and for contributions in donovanosis.

TEN MOST INFLUENTIAL PERSONS IN DERMATOLOGY, VENEREOLOGY AND LEPROSY: WORLD 1. Dr. Ferdinand Ritter von Hebra– Founder of the new Vienna school of dermatology, which set the basis for modern dermatology. 2. Dr. Robert Willan– Founder of dermatology as a medical specialty. 3. Dr. Josef Jadassohn– Best remembered for his handbook of skin and venereal disease (41 volumes), pioneer in allergology, introduced patch testing. 4. Dr. Johnathan Hunter– Natural history of syphilis, role of inflammation in healing. 5. Dr. Paul Ehrlich– Developed salvarsan (magic bullet) as a treatment for syphilis, was the first to stain tubercle bacilli. 6. Dr. Thomas Bernard Fitzpatrick– Proved that melanin was produced in melanosomes, first editor of Dermatology in General Medicine (1965). 7. Dr. Arthur Rook (1918-1991), Dr. Darrell Sheldon Wilkinson and zoologist John Ebling (1918-1992): Produced their major work, Textbook of Dermatology (alias The Rook Book) in 1968. 8. Dr. Paul Gerson Unna, Dr. HKB Pinkus, Dr. A Bernard Ackerman, and Walter F. Lever: Contributions to dermatopathology. 9. Heinrich Koebner– Koebner phenomenon, founder of the dermatology clinic at the University of Breslau. 10. GHA Hansen– Identified M. leprae as the causative agent of leprosy in 1873.

History of Dermatology in the World

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HISTORY OF DERMATOLOGY IN THE WORLD • In Greek and Roman era, Hippocrates recognized and described many diseases. Some of the medical facts he observed are as true today as they were over 2000 years ago. He rescued medicine from magic and superstition, therefore rightly so called “the Father of Medicine”. • Dermatology, the science of the skin, was one of the many specialties, which evolved from general internal medicine during the course of the nineteenth century. Most diseases of skin, as ‘external diseases’, had for many centuries fallen within the province of the surgeon or of the quack. • Until the eighteenth century was welladvanced, physicians with few exceptions were little concerned with the skin, apart from the exanthematic eruptions of acute fevers. However, during the last decades of that century, many of the great physicians recorded their observations on diseases of the skin. The solid contributions of some, such as Heberden and Cullen, which have received too little attention from the historians of dermatology, laid the foundations on which the pioneer specialist

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dermatologists of the following century were able to build. Despite developments in 19th centuryvaccination against small pox, recognition of cellular pathology, Louis Pasteur’s germ theory of infection, development of anaesthesia and microscope, the treatment of most skin diseases was at best symptomatic and at worst dangerous. Amongst the first to specialize in dermatology was Ferdinand Hebra (18161880) in Vienna, who led the torch of dermatology, others followed him. The last half of 19th century saw dermatology and venereology emerge as a specialty in its own right. The 20th century brought a wealth of new scientific knowledge that can be used to help the sick. Perhaps, the most important single discovery was that of Sir Alexander Fleming, the British bacteriologist who found the first antibiotic, penicillin. During 20th century, certain turning points occurred in general sociocultural factors (welfare—public health, vaccines, hygiene, clean water, sewerage, etc; war; communications–books, photography, radio, films, television, computers; transport), general scientific developments (genetics- structure

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Essentials in Dermatology of DNA; inflammation-histamine, prostaglandins, cytokines, adhesion molecules; immunology-cell mediated and humoral immunity; tissue culture; pathogenic agentsspirochetes, viruses, prion; therapies- x-rays, antibacterial, immunosuppressive; controlled clinical trials), and strictly dermatological areas(books- Jadassohn, Pillsbury, Rothman, Rook; biologykeratinocyte, melanocyte, Langerhans cell, basement membrane; diseases- epidermolysis bullosa, pemphigus, toxic epidermal necrolysis; people- from Unna to Katz; therapies- local steroids, griseofulvin, phototherapy, retinoids, Moh’s surgery, laser, cryotherapy).

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EVOLUTION OF DERMATOLOGY • In India, recognition of dermatology as a specialty distinct from internal medicine is recent; it has still not grown to its full stature in practice and teaching. • Therapeutics of dermatoses have been known and practiced by our ancient physicians for centuries. Charaka Samhita contains one chapter on the subject. • Medical charlatans selling panaceas for cutaneous ailments and faith healers were commonly seen all over the country. With the advent of scientific dermatology, their number and importance has dwindled. • In the latter part of the 19th century, the health authorities in then British India became aware of the need to have data on prevalence of dermatoses and venereal diseases. • The first chair of dermatology was established at Grant Medical College, Jamshedji Jeejebhoy Hospital (JJ Hospital), Bombay in 1895. • The second department, at the School of Tropical Medicine in Calcutta, was started in 1923, after a gap of nearly 28 years, under

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the patronage of Dr Ganpati Panja and Colonel Acton. During the period from 1956 to 1974, the status of the specialty was further elevated and steps were taken by state governments to set up departments of dermatology and venereology in medical institutions. Dr UB Narayan Rao, a pioneer in the specialty, gets the credit for the creation of an association of dermatologists and venereologists in Bombay (July 1, 1947), and for Indian Journal of Venereology started in 1935, renamed as Indian Journal of Venereal Diseases and Dermatology in 1940, and later renamed as Indian Journal of Dermatology and Venereology in 1955, the first issue of which was edited by him. In 1962, it was decided to affiliate the association of dermatologists and venereologists with Association of Physicians of India (API). This continued until 1974, after which this affiliation was severed and association became an independent body. On January 28, 1973, the present association the Indian Association of Dermatologists, Venereologists and Leprologists (IADVL) came into existence. Since 1976 the bimonthly journal is being published under the title Indian journal of dermatology, venereology and leprology.

EVOLUTION OF VENEREOLOGY • Syphilis was first introduced into North India nearly 500 years ago. • National STD Control Programme was started in 1946. This programme continued to operate till 1991 and with the arrival of HIV infection in the country, the programme was


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brought under the purview of National AIDS Control Organization (NACO) in the year 1992. The monograph by Rajam and Rangiah on donovanosis (granuloma inguinale, granuloma venereum) is testimony to the teaching and research standards set by these two giants at institute of venereology, Chennai. Dr. CN Sowmini founded the Indian Association for the study of sexually transmitted diseases (IASSTD) in the year 1975. In the year 1980, this specialty, under the banner of IASSTD, started its own exclusive journal, the Indian Journal of Sexually Transmitted Diseases. The late Dr. Sardarilal was its founder editor and guiding force, who had already made a mark in the field of research, especially in donovanosis. Somehow venereology did not prosper as much, even though it led in front of dermatology and leprology in teaching and in the starting of its own journal in early part of 19th century. Unlike in the West, venereology in India has been combined with dermatology in most of the universities.

EVOLUTION OF LEPROLOGY • There is a great deal of speculation about the early history of leprosy. The earliest records, which give accurate descriptions of the disease, come from India and may have been written as early as 600 BC.

• In Sushrata Samhita (600 BC), one finds a reasonably good account of the clinical features and treatment of the disease. Sushrata described the different forms of leprosy, and these forms fit in fairly well with the forms of the disease as recognized at the present time. • Sushrata described the treatment of the disease with Chaulmoogra oil (hydnocarpus oil), which till 1940s was the mainstay in the treatment of the disease. • The first known asylum for leprosy patients was established in Calcutta early in the 19th century, followed by another in Varanasi. • “Leprosy in India”, a journal specific for leprosy, was started by Dr. Ernest Muir in 1929, initially in the form of quarterly notes, and later on transformed itself to a fullfledged scientific journal. Consistent with its contents and nature, Leprosy in India was renamed as the Indian Journal of Leprosy in 1984. • Dr. Dharmendra straddled the scene of leprosy in India like a giant and is known for lepromin test, Indian classification of types of leprosy and the journal “Leprosy in India”. • The National Leprosy Control Programme (NLCP) was started in 1954-55. In view of scientific advancement and availability of highly effective treatment of leprosy, the programme was redesignated as National Leprosy Eradication Programme (NLEP) in 1983. • In 1991, the World Health Organization set a target of elimination of leprosy as a public health problem by the year 2000. India achieved this target in 2005.

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Essentials in Dermatology

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Microanatomy of the Skin

Dermatology may be defined as the study of the skin and its diseases or is a branch of medical science, which deals with systematic study of skin in health and disease. Since skin conditions constitute 10 to 30% of outpatient attendance in any hospital, they are often easily noticed by others (hence a cause of great social concern to the patient) and very often, skin diseases offer diagnostic clue to many major systemic disorders, makes this subject challenging and important to study.

FUNCTIONS OF THE SKIN The skin is the largest organ of the body, accounting for 16-20% of total body weight. The skin of an average adult covers an area just under 2 m2. It not only gives shape to the body but also helps it in many ways – the important functions of the skin are: 1. Protection (Barrier function) from: a. Physical injuries b. Chemical injuries c. Infections 2. Thermoregulation 3. Sense organ: A number of sensations – touch, pressure, warmth, cold and pain are perceived by the skin. 4. Storage of electrolytes, carbohydrates, water, fat, vitamins, proteins, etc.

5. Vitamin D formation: Vitamin D 3 is essential for skeletal development. 6. Absorption: The skin surface also performs absorptive function and is the basis of topical therapy in dermatology. 7. Excretion: Some of the toxins may be excreted through the skin. 8. Immune surveillance: This immunological function is performed by Langerhan’s cells, dendritic cells (intermediate) and keratinocytes. 9. Mechanical function: The mechanical properties of the skin depend mainly on the dermis. 10. Cosmetic function: Colour of the skin and hair and nails are important for their decorative value. Hair does not perform a “vital” physiologic function but it does provide a sexually attractive ornament.

DEVELOPMENT OF SKIN Epidermis develops from ectoderm lateral to neural crest, dermis from mesenchyme and neural crest cell, subcutaneous fat from mesenchyme and melanocytes from neural crest. Foetal skin development occurs in three stagesspecification, morphogenesis and differentiation. Its specification occurs from 0 to 60 days, morphogenesis from 2 to 5 months, and differentiation from 5 to 9 months.


STRUCTURE OF THE SKIN Skin has 3 layers (Fig. 3.1) 1. Epidermis 2. Dermis 3. Subcutaneous fat (Hypodermis).

Epidermis It is approximately 0.4 mm to 1.6 mm in thickness. The majority of the cells in the epidermis are the keratinocytes. These cells are organized into five layers-stratum corneum, stratum lucidum (present only in palmar and plantar skin), stratum granulosum, stratum spinosum, and stratum basale or stratum germinatum (Fig. 3.2). Stratum corneum is the outermost layer containing flattened anucleated cells without cell organelles. The thick epidermis of palms and soles has an additional layer underneath the stratum corneum that is electron lucent and is called the stratum lucidum. The stratum granulosum is so called due to the presence of

intracellular basophilic keratohyaline granules and consists of 2-5 cells layer. The stratum spinosum contains 8-10 layers of polyhedral cells with round nuclei. The stratum basale or stratum germinativum consists of single layer of cuboidal or columnar cells. Keratin filaments are a hallmark of the keratinocytes and the process by which a keratinocyte of the basal layer ultimately changes into keratin is known as keratinization and it usually takes 4 weeks for its completion. The epidermal turnover time is about I month. The other member cells found in the epidermis are melanocytes (derived from neural crest, produce melanin), Langerhans cells (origin from bone marrow, play important role in cutaneous immune mechanisms), and Merkel cells (slow adapting type 1 mechanoreceptors).

The Dermal-epidermal Junction The dermal-epidermal junction (DEJ) is a basement membrane zone (BMZ) that welds the epidermis to underlying dermis.

Fig. 3.1: Structure of the skin

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Fig. 3.2: Diagrammatic representation of shapes of various cells in the epidermis and changes in them as they move up from basal cell layer to stratum corneum during the process of keratinization

The dermal-epidermal junction is undulated, forming dermal papillae (upward projections of the dermis into the epidermis) and rete ridges (downward projections of epidermis into the dermis). The DEJ under electron microscope consists of four components- plasma membrane of basal keratinocytes with hemidesmosomes, lamina lucida (made up of anchoring filaments and laminin 5), lamina densa ( has type IV collagen and laminin 5), and lamina fibroreticularis (containing anchoring fibrils, dermal microfibrils, and collagen fibers). This DEJ is weakest at lamina lucida.

Dermis The dermis is formed by connective tissue having fibres (collagen, elastic and reticulin) and

ground substance (made up of proteoglycans and glycosaminoglycans). It varies in thickness from about 1 mm on the face to 4 mm on the back and thigh. Collagen fibres are the major component of the dermis, accounting for 75% of dry weight of the skin. Approximately 80-90% of collagen fibres in the dermis are of type I collagen. They are responsible for the mechanical properties of the dermis. Elastic fibers constitute approximately 4% of dry weight of dermal matrix proteins. Due to their elasticity, they maintain the normal configuration of the skin. The dermis can be divided into an upperpapillary dermis that interdigitates between the rete ridges and the deeper-reticular dermis recognized by the thicker, aggregated bundles of collagen.


Epidermal Appendages Like a. Pilosebaceous unit b. Sweat glands. Pilosebaceous unit: It consists of a hair follicle containing hair and sebaceous glands opening into follicular canal of hair follicle. Sebaceous glands are lipid secreting holocrine glands. Their maximum density is in seborrhoeic areas of the body, i.e. scalp, face, upper chest, etc. They get activated at puberty under the influence of androgen hormone. Functions of sebum are: 1. Barrier function by preventing loss of water from the skin 2. Emulsification of surface eccrine sweat 3. May have mosquito repellant action 4. Protection against sunburn 5. Has a vitamin D precursor. Hair structurally consists of a cuticle, cortex and medulla. These keratinous fibres are of two types in adults-terminal hair and vellus hair. Hair grows at the rate of 0.3 mm per day and they undergo growth cycle. These recurring cycles consist of anagen (active growth phase), catagen (static growth phase) and telogen (shedding phase) phases. On the scalp, 80% to 85% of hair are in anagen phase and 15% to 20% in catagen and telogen phase. The anagen phase lasts for two to five years, a short catagen of few days and a telogen phase of three months. Functions of hair are: 1. Cosmetic 2. Hair screens entry of irritants to nose 3. Protect scalp from sunrays 4. Shields the eyes 5. Helps in perception of tactile stimuli. Sweat glands: Two types of sweat glands are eccrine and apocrine sweat glands. Eccrine sweat glands: They are tubular structures, which open on to the skin directly and have three segments–the secretory coil (consists of single layer of secretory cells–clear and dark

cells) in the deep dermis, straight intradermal (has two layers of cuboidal cells lined by eosinophilic cuticle on luminal side) part and coiled or spiral intraepidermal (consists of an inner layer of luminal cells and two to three outer layer of epithelial cells) part. Sweat glands are most abundant on the palms, soles, forehead and axillae. These glands are highly developed and responsive part of the thermoregulatory apparatus, innervated by cholinergic nerve fibers. Functions of sweat are: 1. Sweating in heat stress 2. Excretion of heavy metals and drugs. Apocrine sweat glands: These tubular glands consists of two main parts – the coiled secretary gland (consists of single layer of cuboidal or columnar cells, surrounded by a layer of myoepithelial cells) and the straight excretory duct (consists of double layer of cuboidal cells and inner eosinophilic cuticle) which opens into follicular canal just above the openings of sebaceous glands. They are distributed along the mammary line, i.e. axillae, areolae, periumbilical area, mons pubis, genital and perianal areas. Apocrine gland secretion in man serves no function. Pheromones–its role in humans is debated. Nail unit: It is yet another epidermal appendage. It consists of nail matrix just underneath the proximal nail fold which gives rise to nail plate – a keratinized structure. The distal portion of the nail matrix is visible usually in thumbnail as white crescent or half moon known as lunula. The rectangular nail plate rests on a nail bed and is bounded on two sides by lateral nail folds. The cuticle seals the space between nail folds and nail plate. The distal portion of nail juts out as a free end. The space underneath the free end of the nail plate is called subunguium. In contrast to hair, nail is a continuously growing structure, usually at a rate of 0.1 mm per day.

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Essentials in Dermatology Functions of nails are: 1. Protect terminal phalanges 2. Cosmetic function 3. Helps in appreciation of tactile stimuli 4. Scratching of skin 5. Helps in holding minute objects with finger tips. Besides the above elements, dermis contains blood vessels which form two plexuses (other than providing nutrition to the skin, blood vessels regulate temperature and blood pressure), lymphatics roughly parallel the major vascular plexuses, nerves of the skin are part of two major systems – somatic sensory and autonomic motor, smooth muscle occurs in the skin as arrectores pilorum, as the tunica dartos of the scrotum and in the areolar around the

nipples and cells – mast cells, fibroblasts, monocytes, macrophages, dendrocytes and pericytes, etc.

Subcutaneous Fat (Hypodermis) The subcutaneous fat layer is constituted by adipocytes. It is abundant over the breasts, buttocks, and abdomen, thinner over the nose and sternum and absent over the eyelids and male genitalia. It acts as shock absorber, helps in heat production and hormone conversion, facilitate mobility of skin over structures that underlie and acts as an insulator for heat. A cosmetic role is contributed by the accentuated distribution of fat in some sites in the sexes. Most importantly, it stores triglycerides, which serves as fuel for energy.

Physiology, Biochemistry and Immunology of the Skin

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Physiology, Biochemistry and Immunology of the Skin

Main functions of the skin are protection, thermoregulation, sensory, storage organ, vitamin D formation, absorption, excretion, immune surveillance, mechanical and cosmetic function. Some important physiological, biochemical, and immunological processes of the skin are summarized below.

epidermal barrier is localized to the stratum corneum. The skin has two barriers to UV radiations: a melanin barrier in the epidermis; and a protein barrier concentrated in the stratum corneum. Both function by absorbing radiation thereby minimizing absorption by DNA and other cellular constituents.

PROTECTIVE FUNCTIONS OF THE SKIN The innermost region of human skin is the subcutaneous fat layer. This layer insulates reduces heat movement into or out of the body, absorbs energy from blunt mechanical trauma and is active in general energy metabolism. Superficial to the fat layer lies the dermis, composed of collgen-glycosaminoglycan complexes which also protects the body from blunt mechanical trauma. Overlying the dermis is the epidermis which consists of several stratifying layers of nucleated keratinocytes and anucleated top layer, the stratum corneum which performs the major barrier function. The skin acts as a two way barrier to prevent the inward or outward passage of water and electrolytes. The physical barrier is largely situated in the epidermis, isolated epidermis being as impermeable as whole skin, whereas once the epidermis is removed, the residual dermis is almost completely permeable. The

PERCUTANEOUS ABSORPTION The skin is considered to be a composite membrane with three anatomically distinct layers; the stratum corneum (10 µm), the viable epidermis (100 µm), and the uppermost papillary layer of the dermis (100-200 µm), each having a different diffusion constant. Even healthy adult human skin allows some permeation of almost every substance, and rates of penetration of different materials may differ by 10,000 fold. The efficiency of the barrier differs between body sites. The scrotum is particularly permeable and the face, forehead, and dorsa of the hands may be more permeable to water than the trunk, arms, and legs. The palms are particularly impermeable to nearly all molecules except water. The barrier is affected by many other factors, such as age, environmental conditions and physical trauma, and permeability can be

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Essentials in Dermatology enhanced by various agents, permitting increased access of topically applied drugs.

KERATINIZATION Keratinization is a process of differentiation of germinative cells in the basal cell layer into the deceased cornified cells of stratum corneum. It involves: 1. Synthesis of distinctive proteins (e.g. keratins, filaggrins, and involucrin) and lamellar granules, and 2. Alterations of nuclei, cytoplasmic organelles, plasma membranes and desmosomes. Keratin function is to provide mechanical strength, cellular structure, and assistance in adhesion molecule attachment. “Soft” keratin desquamates as the result of enzymatic action but the “hard” keratin of the hair and nails does not, thus requiring periodic cutting. The epidermis is the prototype of keratinizing squamous epithelia, also present in the oesophagus, vagina and oral mucosa.

MELANOCYTES AND MELANOGENESIS Melanocytes are pigment forming cells in the epidermis. Each melanocyte supplies pigment, melanin to approximately 36 keratinocytes and thus form epidermal melanin unit. Two types of melanin are synthesized by melanocytes, eumelanins and pheomelanins. Melanin is synthesized from tyrosine under the influence of enzyme tyrosinase through the formation of various intermediates (dihydroxyphenylalanine [dopa], dopaquinone, leucodopachrome, dopachrome, 5’ 6’ dihydroxyindole, indole 5’ 6’-quinone, melanochrome).

FUNCTIONS OF THE MELANIN 1. Protect the skin from harmful effects of sunlight by scattering and absorbing ultraviolet light.

2. Melanin may also act as a biochemical neutralizer of toxic free radical oxygen derivatives, byproducts of various inflammatory processes. 3. Melanocytes situated in the matrix of anagen follicles impart to hair various colours, e.g. blond, brunette and red head.

THERMOREGULATION The maintenance of a near constant body core temperature of 37oC is a great advantage to humans, allowing a constancy to biochemical reactions which would otherwise fluctuate widely with temperature changes. The thermoreceptor cells of the skin are distributed irregularly over the skin, there being warm- and cold-sensitive thermoreceptors. Information on changes in their stimulation in response to changes in the temperature is sent to the hypothalamus leading to either to inhibition of sweating or stimulation of shivering. Skin temperature has a greater role in mediating the behavior, for example by turning on the heating or putting on extra clothing. Thermoregulation depends on several factors, including metabolism and exercise but the skin plays an important part in control through the evaporation of sweat and by direct heat loss from the surface. Heat can be lost through the skin surface in four ways: 1. Radiation 2. Convection 3. Conduction 4. Evaporation

SKIN FAILURE Skin failure is defined as a loss of normal temperature control with inability to maintain the core temperature, failure to prevent percutaneous loss of fluids, electrolytes and proteins with resulting imbalance and failure of

Physiology, Biochemistry and Immunology of the Skin mechanical barrier to penetration of foreign materials. Apart from thermal burns, skin failure can occur as a consequence of a number of dermatological diseases including Stevens Johnson syndrome, toxic epidermal necrolysis, pustular psoriasis and erythroderma of various causes.

COLLAGEN IN THE DERMIS The closely related proteins of collagen family are the main fibrillary components of the connective tissues and the major extracellular proteins of the human body. The physiological role of collagen fibers in the skin is to provide tensile properties that allow the skin to serve as a protective organ against external trauma. Collagen is the major structural protein constituting 70% to 80% of dry weight of the dermis. The main aminoacids in collagen are glycine, proline and hydroxyproline.

Elastic Fibers in the Dermis Elastic fibers of the connective tissue form a network responsible for the resilient properties of the skin. In sun protected human skin, elastin content is about 1% to 2% of the total dry weight of dermis. Ground Substance in the Dermis The ground substance of skin is largely made up of glycosaminoglycans(GAG) and provide viscosity and hydration in the dermis. Three

types of GAG are chondroitin sulphate, dermatan sulphate and hyaluronic acid.

IMMUNOLOGICAL COMPONENTS OF SKIN The immunological functions of the skin depend both upon cells in the epidermis and on dermal cellular constituents. Antimicrobial peptides (AMPs) are a diverse group of proteins that are involved as first line of immune defense by many living things. In human skin, AMPs provide a chemical barrier to potentially pathogenic microorganisms. Sebaceous lipids have been reported to possess antibacterial properties and glycophospholipids and free fatty acids of stratum corneum have bacteriostatic effect selective for pathogenic organisms. Skin associated lymphoid tissue (SALT) is langerhans cells, T lymphocytes, mast cells and keratinocytes. They are involved in various hypersensitivity reactions of the skin. Hypersensitivity is defined as inappropriate or exaggerated immune response to a foreign or self antigen resulting in tissue damage. Main types of hypersensitivity responses of skin are type I (immediate), type II (antibody-dependent cytotoxicity), type III (immune complex disease) and type IV(cell mediated or delayed). Urticaria and anaphylaxis is the example for type I hypersensitivity, transfusion reactions for type II hypersensitivity, leukocytoclastic vasculitis for type III hypersensitivity and allergic contact dermatitis for type IV hypersensitivity.

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5

Principles of Diagnosis in Dermatology

Dermatologists often prefer to examine the patient before obtaining the history and review of systems. This approach is preferred because diagnostic accuracy is higher when visual examination is approached without preconceived ideas. Moreover, some dermatologic lesions are so distinctive that history is not required to make a diagnosis. A practical and convenient way to arrive at a diagnosis may be...

Chief complaints: Limit them to just three only History of present illness: “What is your skin problem?” This allows patient to point out the lesions and the area involved. Three basic questions. 1. Onset and evolution. 2. Symptoms. 3. Treatment taken. Onset and evolution: To determine the duration of the disorder, how it evolved over time, initial site of the disease, mode of spread.

PRELIMINARY GENERAL HISTORY

Symptoms: Itching is the most common and most important symptom in dermatology. Intense itching, e.g. scabies, atopic dermatitis, lichen planus, dermatitis herpetiformis. Pain may predominate in herpes zoster, furuncles, etc. Loss of sensation points towards leprosy, or follicular mucinosis. Allodynia (production of pain by trivial stimuli) occurs in postherpetic neuralgia. Reversal of hot and cold sensation may be due to ciguatera fish poisoning. They may be just asymptomatic as in molluscum contagiosum, basal cell carcinoma, etc.

Biodata: Age, sex, income, occupation, address, marital status.

Treatment history: Skin lesions are often selfmanipulated by home remedies, or over the

Principles of Diagnosis in Dermatology counter medication, since they are easily accessible, and since disease is of chronic nature. Full detailed history of medication used should be known because 1. Disease may be caused or aggravated by medication- Fixed drug eruption, dermatitis medicamentosa. 2. Patient may have already used the drug without desired outcome, we planned to give. Avoid potential embarrassment when the patient says “I have already tried that and it didn’t work”. Detailed follow-up history: This history is taken after some diagnosis or conclusion was reached by initial history and examination, and this includes. • Past history. • Family history. • Review of systems. • Social history. • Females- menstrual/obstetric history. Past history: a. History of same disease before. b. History of prolonged illness– diabetes, hypertension. c. Drugs used for other problems (drug rash, urticaria). d. Drug allergies– avoid prescribing those drugs. e. Atopic history– asthma, hay fever, eczema. Family history: It is important for diagnosis, prognosis, treatment and genetic counseling. Family history important in: a. Infectious disorders – scabies b. Inherited disorders – atopy, psoriasis. c. Genodermatoses. Review of other systems: It is required in multisystem disorders like SLE, scleroderma, or lepromatous leprosy. Social history: Encounter with potentially sensitizing materials e.g., in patients with

industrial dermatosis, contact dermatitis. Stress and strain at work may lead to exacerbation of psoriasis, neurodermatitis, etc. Habits: Alcohol induces porphyria cutanea tarda in predisposed, influences the severity and therapeutic options in psoriasis. Smoking may be aggravating factor in palmoplantar pustulosis.

PHYSICAL EXAMINATION It has been said by Goethe “What is most difficult of all? It is what appears most simple: To see with your eyes what lies in front of your eyes” .

Requirements for the Skin to be Properly Examined Three essential requirements 1. Preferably a completely undressed patient, clothed only in an examination gown. If not possible, at least, the affected part should be properly exposed. 2. Adequate illumination: Preferably sunlight or a bright overhead fluorescent lighting. Penlight is used in side lighting- to determine if a lesion is subtly elevated and for examining the oral cavity. 3. An examining physician ready to see what is before him. A complete cutaneous examination should be made, this includes examining— • Skin from head to foot. • Mucous membrane in mouth and genitals • Hair and nails. The examination includes inspection and palpation, besides percussion and auscultation. Palpation is useful in— • Assessing the texture and consistency. • Evaluate whether a lesion is tender or not. • Reassure a patient that they do not have a contagious disease.

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Essentials in Dermatology Hand lens useful on occasions like identifying: a. Altered skin markings in tumors. b. Nail fold telangiectasia. c. Burrows in scabies. d. Wickham‘s striae- for this place a drop of mineral oil on the area, which makes the stratum corneum transparent. Subtle genital warts- ‘aceto-whitening’, gauze soaked with 5% acetic acid applied in suspected area for 3 minutes, warts turn white. Actually individual skin lesions are analogous to the letters of the alphabet, and groups of lesions can be analogous to words or phrases. Basis of morphological lesions is given in the form of table for clear understanding. Basis of morphological lesions in dermatology 1. Impalpable change- Macule 2. Palpable change• Solid change-Papule, plaque, nodule, wheal • Superficial visualized free fluid collectionVesicle, bulla • Superficial free pus collection-Pustule, abscess • Deep free fluid/semisolid material collection-Cyst 3. Loss of skin-Erosion, ulcer 4. Healing stage- Scale, crust 5. End stage- Atrophy, scar

Fig. 5.1: Macule—depigmented flat lesions of variable size and shape of vitiligo vulgaris and lip tip type

Ultimately, diagnosis may rest on recognition of lesions and their distribution and arrangement, whether they are primary, secondary or some special lesions. Describe their shape, size, color and distribution. Take the help of diagnostic tools for further details.

Primary Lesions These are the lesions, which appear first in any skin disease. They are the best clues to the diagnosis. They are: • Macule: The macule is a discrete, flat, circumscribed lesion that differs from surrounding skin because of its color (Fig. 5.1). It may be a small or a large macule. Earlier used term “patch” is now obsolete. Macule may be erythematous, hypo-

Fig. 5.2: Papule—solid elevated lesions of verruca vulgaris of less than 0.5 cm

pigmented, hyperpigmented or of any other color. • Papule: It is a discrete, circumscribed, solid elevated lesion of less than 0.5 cm in size (Figs 5.2 to 5.5). So, it is a palpable lesion. A papule may be dome shaped, verrucous, umbilicated, pedunculated, etc.


Fig. 5.3: Papule—dome shaped papule, a few of them umbilicated of molluscum contagiosum

Fig. 5.5 Violaceous colored papules of lichen planus over the genitalia and thigh

Fig. 5.4: Typical umbilicated papule of molluscum contagiosum

Fig. 5.6: Plaque—flat elevated lesions covered with silvery white micaceous scales of psoriasis vulgaris

• Plaque: A plaque is a circumscribed solid raised lesion with a flat top. It is formed due to coalescence of papules (Figs 5.6 and 5.7). It may be a lichenified plaque, eczematous plaque, psoriasiform plaque, flat smooth plaque, etc. • Nodule: A nodule is a discrete circumscribed solid elevated lesion, which is more felt than

seen from the top (Figs 5.8 and 5.9). It may develop from a papule. • Vesicles and bullae: Vesicle and bullae are two terms used for circumscribed elevated lesions containing free clear fluid, called blister. If it is less than 0.5 cm, it is called vesicle (Fig. 5.10) and if more than this, it is a bulla (Figs 5.11 and 5.12). They may be tense or flaccid.

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Fig. 5.7: Large well-defined erythematous plaques of psoriasis vulgaris

Fig. 5.8: Nodule—solid deep-seated elevated lesion due to secondaries in the skin

Fig. 5.9: Erythematous tender nodules of furuncle over the face and neck

Fig. 5.10: Vesicle—cluster of tiny blisters of herpes labialis over the lips

Fig. 5.11: Bulla—small blisters on erythematous bases of bullous pemphigoid

Fig. 5.12: Large tense bulla of bullous pemphigoid on an erythematous base

Principles of Diagnosis in Dermatology • Pustule: A pustule is a circumscribed elevated lesion containing visible pus (Fig. 5.13). It results from an epidermal or upper dermal accumulation of pus. • Cyst: A cyst is a sac that contains liquid or semisolid material. • Wheal: Wheal is a pale or erythematous edematous, transient, evanescent lesion. • Diffuse thickening of skin: It may result from edema of dermis (pitting edema or nonpitting edema) or infiltration of dermis (e.g. myxoedema, lepromatous leprosy).

Secondary or Consecutive Lesions They are due to the subsequent changes, which takes place on the primary lesions, either as a part of natural evolution or due to manipulation of the patient. • Oozing: It is due to the rupture of vesicles or bullae. • Crust: It is dried up exudate like serum, pus or blood (Fig. 5.14). It may be thick or thin, friable or adherent. It occurs in many inflammatory and infectious diseases. • Scale: Scales are thin, dry plates of heaped up desquamating epithelial cells formed as

Fig. 5.13: Pustule—numerous tiny pus filled lesions on erythematous background in a case of pustular psoriasis

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a result of either increased or abnormal keratinization (Fig. 5.15). Excoriation: An excoriation is a superficial erosion or ulcer caused by scratching. So, it will be linear or have a geometric outline (Fig. 5.16). Erosion: It is a superficial ulceration involving epidermis only which heals without scarring (Fig. 5.17). Ulcer: It is a break in continuity of epithelium, which involves epidermis, and dermis of the skin (Fig. 5.18). It has length, breadth as well as depth. It heals with scar formation. Fissure: Fissure is a linear crack in the skin, which may be superficial or deep to the dermis. Lichenification: It is characterized by thickening of the skin (becomes leather like) with increased skin markings and pigmentation. It is seen in chronic dermatitis. Scar: A scar is an evidence of destruction of the skin with fibrotic tissue replacement. It occurs wherever ulceration has taken place and reflects the pattern of healing in those areas.

Fig. 5.14: Crust—dried out oozed material over the face in impetigo contagiosa

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Fig. 5.15: Scale—face and trunk covered with moist scales of pemphigus foliaceous

Fig. 5.16: Excoriation—multiple linear scratch marks due to itching over psoriatic plaques

Fig. 5.17: Erosion—multiple superficial eroded areas in herpes genitalis

Fig. 5.18: Ulcer—single, painless, indurated ulceration of extragenital primary chancre

• Pigmentation: Pigmentation may be hyper, hypo- or depigmentation of the skin (varies according to the quantity of melanin). • Atrophy: Atrophy refers to a diminution in the size of a cell, tissue, organ or part of the body. The skin becomes thin, shiny and

wrinkled. Atrophy may be of epidermal, dermal or subcutaneous fat. • Sclerosis: Sclerosis means a circumscribed or diffuse hardening or induration in the skin. It occurs as a result of an increase in the amount of dermal collagen, expansion of the

Principles of Diagnosis in Dermatology collagen by ground substance material or altered quality of collagen.

Special Lesions These lesions are given below: • Comedone: It is a plug of keratin and sebum formed in the follicular canal of pilosebaceous unit. Comedones may be closed or open. • Burrows: These are serpentine caves of scabies mite at the level of stratum granulosum. They are visible as S-shaped brownish-black lesions, which at their distal end have a papule housing the mite. • Alopecia means loss of hair. • Telangiectasia: It refers to individually visible dilated vessels. • Poikiloderma: It is a combination of atrophy, pigmentation and telangiectasia. • Purpura: It is visible extravasated blood (Fig. 5.19). It may occur as tiny pinpoint spots

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(petechiae) or larger spots (ecchymoses). The term hematoma refers to an area of massive bleeding into the skin and underlying tissues. “Pinch purpura” hemorrhage induced by mild often subclinical trauma is a characteristic presentation of primary systemic amyloidosis of the skin. Similar periorbital hemorrhage following proctoscopy or pulmonary function testing, postproctoscopic purpura also typifies the vascular fragility induced by systemic amyloidosis. Livedo: Blue red discoloration of the skin of skin due to passive congestion of the vessels often with net-like pattern Exanthem: Abrupt appearance of diffuse or generalized similar skin lesions (usually represents viral infections or drug reactions) Enanthem: Abrupt appearance of mucosal lesions similar to exanthems. Nits: They are glistening white ovoid bodies attached to shafts of hair.

Fig. 5.19: Purpura—multiple, small, erythematous non-blanchable lesions of purpura

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DISTRIBUTION OF SKIN LESIONS IN SOME DERMATOLOGICAL DISORDERS Disease 1. Acne vulgaris 2. Atopic dermatitis 3. Dermatitis herpetiformis 4. Lichen planus 5. Neurodermatitis 6. Pityriasis rosea 7. Psoriasis vulgaris 8. Scabies

9. Seborrhoeic dermatitis

10. Tinea versicolor

Classical sites of involvement Face, upper trunk, proximal parts of upper extremities Infants -face and extensor aspects of limbs (Figs 5.20A and B) Children and adults-flexures (Figs 5.21A and B). Scalp, extensor aspects of limbs, shoulder and buttocks (Figs 5.22A and B). Flexor aspect of upper extremities (wrists), trunk (lumbosacral area), shins, glans penis (Figs 5.23A and B). Nape of neck, wrist, ankle, genitalia, and perianal area (Figs 5.24A and B). Herald patch-trunk. Daughter patches-Christmas tree pattern over the trunk (Figs 5.25A and B). Extensor aspects of limbs, scalp, lumbosacral area (Figs 5.26A and B). Infants-Face, intertriginous area of fingers, palms and soles, extensor aspect of limbs, around umbilicus, genitalia and gluteal area (Figs 5.27A and B). Children and adults-finger web spaces, wrist, elbows, axillary fold, around areola and umbilicus, genitalia, and gluteal area (Figs 5.28A and B). Infants-Cradle cap over scalp (Figs 5.29A and B). Adolescence and adults- scalp, eyebrows, nasolabial folds, presternal and interscapular area, axilla and groin (Figs 5.30A and B). Upper trunk (Figs 5.31A and B).

Figs 5.20A and B: Distribution of skin lesions in atopic dermatitis in infants

Figs 5.21A and B: Distribution of skin lesions in atopic dermatitis in children and adults


Figs 5.22A and B: Distribution of skin lesions in dermatitis herpetiformis

Figs 5.23A and B: Distribution of skin lesions in lichen planus

Figs 5.24A and B: Distribution of skin lesions in neurodermatitis (lichen simplex chronicus)

Figs 5.25A and B: Distribution of skin lesions in pityriasis rosea

Figs 5.26A and B: Distribution of skin lesions in psoriasis vulgaris

Figs 5.27A and B: Distribution of skin lesions in infants in scabies

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Figs 5.28A and B: Distribution of skin lesions in children and adults in scabies

Figs 5.29A and B: Distribution of skin lesions in seborrhoeic dermatitis in infants

Figs 5.30A and B: Distribution of skin lesions in seborrhoeic dermatitis in adolescence and adults

Figs 5.31A and B: Distribution of skin lesions in tinea versicolor

Certain phenomenon and signs are there to be seen and observed; others need to be elicited by tools.

of dermatological disorders such as psoriasis (Fig. 5.32), lichen planus, vitiligo, eczema, dermatitis herpetiformis, bullous pemphigoid, warts (Figs. 33 and 34), molluscum contagiosum (Fig. 5.35), etc. • Reverse Koebner phenomenon: Area of psoriasis clears of following injury. • Remote reverse Koebner phenomenon is the spontaneous repigmentation of vitiligo patches distant from the autologous skin graft sites.

• The Koebner phenomenon: The Koebner phenomenon is the development of morphologically identical lesion/s in the traumatized uninvolved skin of patients who have cutaneous diseases. It is also known as isomorphic phenomenon, a self-explanatory term. This phenomenon is observed in a number


Fig. 5.32: Koebner phenomenon in psoriasis vulgaris over the trunk

Fig. 5.33:Koebner phenomenon in plane warts over the wrist

Fig. 5.34: Koebner phenomenon in verruca vulgaris

Fig. 5.35: Koebner phenomenon in molluscum contagiosum

• Dermographism: It can be elicited with the help of blunt instrument like key. Firm stroking of skin may result in exaggerated triple response of Lewis, which persists for more than 5 minutes. Stroking causes histamine to be released, leading to localised redness and edema (Wheal). Dermographism can occur in urticaria.

• Darier’s sign: When the above phenomenon is limited to skin overlying a lesion (macule or papule), it is called as Darier’s sign and is diagnostic of urticaria pigmentosa (Mast cell disease). • Pseudo-Darier’s sign: Here stroking of skin produces transient induration with pilo-

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erection, seen in congenital smooth muscle hamartoma White dermographism: Stroking the skin of atopic patients produces a characteristic white line in the involved area. Grattage test: It is done on a scaly lesion to look for types of scales. Scraping of the lesion is done with a glass slide. Fine powdery scales of tinea versicolor can be made out if you examine the glass slide against light after scraping the lesion. Candle sign and last cuticle sign: In psoriasis, if the silvery-white scales are scraped off, they detach from the lesions as small flakes, similar to wax scraped from candle. With continued scraping, one can remove a coherent moist sheet from the lesion corresponding to the lowest layers of epidermis. Auspitz’s sign: This sign if present is diagnostic of psoriasis. It has three components. 1. On scraping with glass slide, initially silvery white micaceous scales come out. 2. Removal of the scales is followed by a thin membranous structure. 3. On its removal by glass slide, minute pinpoint bleeding spots are seen. Diascopy (Vitropression): It is based on the principle that vascular lesions will blanch in response to pressure with a glass slide whereas purpuric lesions, in which blood and blood pigments have leaked from the cutaneous vessels, will not blanch. Diascopy is most useful in detection of nonblanchableraised purpura, the clinical hallmark of cutaneous vasculitis. It is useful in differentiating nevus anemicus from nevus depigmentosus. Nevus anemicus (a localised area of vasoconstriction) on diascopy of adjacent skin reveals an identical color to depigmented area. By contrast diascopy of skin adjacent to nevus depigmentosus or vitiligo, the affected area still remains paler.

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Apple jelly nodules in lupus vulgaris active edge of the lesion, appear as translucent brownish color granulomatous nodules, a distinctive feature of the disease. Ollendorf’s sign: If touching of the papule of secondary syphilis with the head of pin is exquisitely tender, then Ollendorf’s sign is said to be present. Nikolsky’s sign: A frictional force is applied with a finger or thumb over the apparently normal skin, usually overlying a bone like the clavicle or perilesional skin in a patient with vesiculo-bullous lesions. If epidermis or surface of the skin breaks down or peels off leaving raw moist erosion, it is called positive Nikolsky’s sign. Various disorders, in which Nikolsky’s sign is positive, are pemphigus, staphylococcal scalded skin syndrome, toxic epidermal necrolysis, etc. This test is based on the fact that in certain diseases, even the normal looking skin has a weak cohesion between its different layers. Bulla spread sign: It can be demonstrated by marking the boundary of the bulla and then applying pressure with a finger on the edge of the bulla. In pemphigus, bulla spreads beyond the marked line showing that active process of acantholysis has weakened the cohesion between keratinocytes. Button holing sign: In neurofibromatosis, if fingertip is pressed over neurofibroma, the finger gapes in due to defect in the dermis. Dimple sign: It distinguishes dermatofibroma from malignant melanoma. Applying lateral pressure with thumb and index finger –results in formation of a dimple in dermatofibroma, whereas melanoma protrudes above its original plane. Hess test or Capillary fragility test: A blood pressure cuff is applied to upper arm between systolic and diastolic pressure for 5 minutes. The number of petechiae in predetermined area of 5 cm circle is counted.

Principles of Diagnosis in Dermatology If number is more than 5, it is abnormal capillary fragility. • Pathergy test: Inject 0.1 ml of physiologic saline intradermally with fine needle over the forearm. Read after 24-48 hours. Pustules or papules suggest the diagnosis of Behcet’s disease. Histology shows neutrophilic infiltrate or vasculitis. • Testing sensation and palpation of peripheral nerves may be required to fulfil one of the cardinal features of leprosy and thus help in its diagnosis.

LABORATORY AND SPECIAL TESTS They may be needed to confirm the diagnosis: 1. Wood’s light examination: It is useful in detecting fungal infections of the scalp (bluish-green fluorescence in tinea capitis caused by Microsporum species—Microsporum canis and Microsporum audouinii), tinea versicolor (yellow fluorescence), erythrasma (coral red fluorescence), porphyrins in patients with porphyria cutanea tarda (Urine will produce bright red fluorescence), trichomycosis axillaris (orange fluorescence), Pseudomonas infection (green fluorescence), etc. In scabies, fluorescein solution fills burrows. In pigmentary disorders, vitiligo, piebaldism, and ash leaf macules of tuberous sclerosis, the lesions become prominent. 2. KOH preparation: Indicated when infection with fungi or yeast is suspected, e.g. dermatophytosis, tinea versicolor, candidiasis, etc. 3. Tzanck test: It is used in the diagnosis of various skin disorders characterised by vesicles, pustules, bullae and erosions and in particular in viral infections like herpes simplex, herpes zoster and varicella infections.

4. Gram’s stained pus smear: Indicated for pyogenic infections, vaginal and urethral discharge. 5. Tissue smear: It is used for diagnosis of donovanosis (granuloma inguinale). 6. Dark field (ground illumination) test: Primary and secondary syphilis can be easily diagnosed by demonstrating treponemes. 7. Wet preparation: It is utilized for diagnosis of trichomonal infestation of the genital tract. 8. Slit skin smear: This test is performed on leprosy patients to demonstrate acid-fast bacilli in skin smears. 9. Lepromin test: It is useful for classification of leprosy and is strongly positive in tuberculoid leprosy and mildly positive in borderline tuberculoid leprosy. It is negative in borderline borderline, borderline lepromatous and lepromatous leprosy. 10. Dermatoscopy (Epiluminescence microscopy, dermoscopy): Method of observing superficial layers of skin using 10-100 X magnification with oil immersion. Both hand held and computer assisted instruments are available. It is used for differential diagnosis of pigmented skin lesions, melanoma, for detailed examination of nail fold capillaries, Wickham’s striae, scabies burrows (hang glider sign), surface of verrucae and the scalp surface (cadaver hairs and exclamation point hairs suggest alopecia areata, loss of follicular openings indicates scarring alopecia and follicular hyperkeratosis point towards lichen planus). 11. Biopsy: Most frequently skin biopsy is taken to confirm a clinical diagnosis or to aid in the establishment of a diagnosis where clinical diagnosis is not apparent.

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Essentials in Dermatology 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22.

VDRL HIV antibody detection test Culture and sensitivity test Patch testing Prick testing Intradermal testing LE cell phenomenon Mouse foot pad inoculation Immunofluorescence Hair examination and counts Trichogram- method for analyzing hair bulbs to identify in what stage hairs are being lost and thus to distinguish between different types of hair loss. 23. Electron microscopy

Five Thoughts for the Students in the Field Of Dermatology 1. Diagnosis is the art of recognition, not the science of cognition 2. The best diagnosticians are the ones with the best visual memories 3. The best history is taken by one who already knows the diagnosis 4. If puzzled, limit yourself to three working diagnoses. 5. A good colour atlas (a memory of 75 diseases allows you to immediately recognize 95% of all the skin lesions you will ever see) and a good dermatopathologist are your best friends.

Bacterial Infections

6


Normal human skin is colonized soon after birth by a large number of bacteria that live as commensal on the epidermis and epidermal appendages. Coagulase negative staphylococci (S. epidermidis) are inoculated during vaginal passage; coryneform bacteria take up residence on neonatal skin shortly after birth; and within several weeks after birth, the flora of neonatal skin is similar to that of adults. Staphylococcus aureus is persistent member of the microbial flora in 10 to 20% of the population. As many as 84% of healthy individuals have occasional carriage of S. aureus in their anterior nares.

PYODERMAS Pyoderma is a common purulent infection of the skin caused by staphylococcal or streptococcal organisms. They can be classified as primary pyodermas and secondary pyodermas (Table 6.1).

Primary pyodermas are further divided into non-follicular and follicular for clinical application (Table 6.2) and on the basis of organism involved (Table 6.3).

Staphylococcal Pyodermas

Impetigo Non-bullous impetigo (Impetigo contagiosa of Tilbury Fox): • It is caused by S. aureus or group A Streptococcus or both • Occurs in children of all ages, common in preschool and young school children • Commonly over the face (especially around nares) or extremities after trauma • The initial lesion is a transient vesicle or pustule that quickly evolves into a honey coloured crusted plaque (Fig. 6.1) • Surrounding erythema may be present

Table 6.1: Distinctive features of pyodermas

Primary pyodermas 1. Invasion of normal skin by bacteria 2. Single species of bacteria involved 3. Appearance of lesions is characteristic e.g., impetigo, erysipelas, furuncle 4. Treatment is clear cut – Drugs aimed at the microorganism

Secondary pyodermas 1. Develop in areas of already damaged/compromised skin 2. Mixture of organisms involved 3. Not characteristic 4. Role of antibacterial treatment less defined. Here, the aim is to treat the underlying process

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Essentials in Dermatology Table 6.2: Non-follicular and follicular pyodermas A. Non follicular pyodermas include 1. Impetigo 2. Ecthyma 3. Cellulitis 4. Erysipelas 5. SSSS B. Follicular pyodermas include 1. Folliculitis 2. Furuncle 3. Carbuncle

• Regional lymphadenopathy in up to 90% of patients with prolonged untreated infection • In severe cases, there may be fever, adenitis and constitutional symptoms • Differential diagnosis: 1.Tinea corporis – has dry, scaly, advancing edge with central clearing. 2. Ecthyma- characterized by crusted ulcers (not erosions).

Bullous Impetigo Table 6.3: Cutaneous diseases caused by staphylococci and streptococci 1. Cutaneous diseases caused by staphylococcus include: A. Direct infection of skin: Impetigo, ecthyma, folliculitis, furunculosis, carbuncle, sycosis. B. Due to effect of bacterial toxin:SSSS, TSS. 2. Cutaneous diseases caused by streptococcus: A. Direct infection of skin: Impetigo, ecthyma, erysipelas, cellulitis, vulvovaginitis, perianal infection, blistering dactylitis, necrotizing fasciitis. B. Due to effect of bacterial toxin: Scarlet fever, toxic shock like syndrome

Fig. 6.1: Impetigo contagiosa–honey colored crusted lesions over the face of a child

• Earlier bullous impetigo in neonates was popularly known as pemphigus neonatorum. • S. aureus (phage group II) is the causative agent. • Occurs commonly in the newborn and in older infants. • Bullae (flaccid) rapidly evolve from vesicles on areas of grossly normal skin (Fig. 6.2) due to local production of exfoliative toxin A and B. • Differential diagnosis: Pemphigus vulgaris-Generally occurs in young adults. Erosions show no tendency to heal, Nikolsky’s sign and bulla spread sign are positive. More importantly, mucosal erosions are more commonly associated feature.

Fig. 6.2: Bullous impetigo–large pus filled blisters over the trunk of a child

Bacterial Infections Complications of Impetigo • Post-streptococcal acute glomerulonephritis– S. pyogenes type M-49 • Scarlet fever • Urticaria • Erythema multiforme Rheumatic fever in not a complication of streptococcal skin infection (but of streptococcal sore throat).

Ecthyma • Consequence of neglected impetigo • S. aureus and/ or group A Streptococcus are causative agents • Most commonly occurs on the lower extremities of children or neglected elderly patients • Poor hygiene and neglect are key elements in pathogenesis. • Dirty grayish-yellow crust surmounts a “punched out” ulcer (Fig. 6.3).

Fig. 6.3: Ecthyma–crusted lesion over the leg with ulcerated lesions

Folliculitis Pyoderma affecting the hair follicles, classified according to depth of invasion. Superficial folliculitis • Also known as follicular or Bockhart’s impetigo • A small fragile dome shaped pustule occurs at the infundibulum of a hair follicle, often on scalps of children and in the beard area, axilla, extremities and buttocks of adults. • Differential diagnois: Miliaria pustulosa – non follicular pustules are wide spread, which occur in hot and humid conditions Deep folliculitis Sycosis barbae is a deep folliculitis with perifollicular inflammation occurring in the bearded areas of the face (Fig. 6.4) and upper lip. Differential diagnosis 1. Pseudofolliculitis– Papules/pustules are irregularly scattered at the site of ingrowing beard hairs. Neck and angle of jaw (vs. sycosis barbae– upper lip and below angles of jaw) are preferentially involved. 2. Tinea barbae– Site involved is usually submaxillary region or the chin. Hairs are

Fig. 6.4: Sycosis barbae– grouped follicular based crusted lesions in the beard area of the face

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Essentials in Dermatology broken or loosened (easy and painless pluckablity) in the affected area. Loss of hair is the norm. Suppurative or granulomatous nodules rather than pustules characterize this condition. Spores and hyphae can be demonstrated in the hair by KOH examination. 3. Herpetic sycosis is usually limited for a few days and invariably shows vesicles even in persistent lesions 4. Acne vulgaris is polymorphous condition mainly of glabrous skin of the face where comedones are the hallmark of that condition Lupoid sycosis is deep, chronic form of sycosis barbae associated with scarring usually occurring as circinate lesion. Pustules and papules surround a central scar (Fig. 6.5). Differential diagnosis: Lupus vulgaris is characterized by areas of scarring on one side and progression on the other side. There is absence of pustules in the lesions but it demonstrates apple jelly nodules on diascopy.

Furuncles and Carbuncles • A furuncle or boil is a deep-seated inflammatory nodule that develops about a hair follicle, often evolving into an abscess (Fig. 6.6). They arise in hair bearing areas,

Fig. 6.5: Lupoid sycosis–scarring alopecia in the beard area showing active pustular lesions at the periphery

particularly in regions subject to friction, occlusion, and perspiration. Differential diagnosis: FolliculitisInflammatory change is confined within the follicle without any surrounding inflammation – hence presents as a pustule whereas furuncle presents as a nodule. There is less pain and it heals without scar formation. Cystic acne- Associated with other lesions of acne – comedones, papules and pustules and acne scars and it is confined to face and trunk. • A carbuncle is a cluster of furuncles, more extensive, deeper, communicating, in filtrated lesion that develops when suppuration occurs in elastic skin. It usually involves the nape of the neck, back or thighs and usually occurs in the setting of underlying diabetes mellitus, alcoholism, malnutrition, blood dyscrasias, iatrogenic or other immunosuppression including HIV infection. Fever, malaise, prostration accompany. Differential diagnosis: Anthrax – characterized by painless, hemorrhagic crusted (blackish eschar) lesion with surrounding gelatinous edema out of proportion to the extent of the lesion.

Fig. 6.6: Furuncle–a red tender suppurated nodule in a case of erythroderma

Bacterial Infections compromise. Typically, the patient has fever and is irritable. The changes usually begin periorificially or in body folds (Figs 6.7 and 6.8). Then they spread rapidly. Nikolsky’s sign is positive even on apparently normal skin. One should culture the perineum, eyes, ears, nose and throat, looking for S. aureus as the focus of infection is located at a distant site. The bacteria can not be cultured from the skin. For differential diagnosis Table 6.4.

Staphylococcal paronychia: Clinically, skin and soft tissue of proximal and lateral nail fold are red, hot and tender, and if not treated, can evolve to abscess formation. In contrast, chronic or recurrent paronychia caused by Candida albicans is an infection of the space underneath the nail folds.

Toxin Mediated Syndromes Staphylococcal scalded skin syndrome (SSSS) (Ritter’s disease) • This is the most severe form of skin disease due to the exfoliative exotoxins (A and B) produced by S. aureus of group II, phage type 71 or 55 and is characterized by generalized bulla formation and exfoliation. Most commonly involves neonates and young children, but also in adults with renal

Toxic Shock Syndrome • TSS is a multiorgan systemic illness due to exotoxin (TSS-Toxin 1) producing strains of S. aureus. • Case definition 1. Temperature of 38.9oC or higher 2. Erythematous eruption

Fig. 6.7

Fig. 6.8

Figs 6.7 and 6.8: SSSS–typical periorificial and body fold involvement with peeling skin

Table 6.4: Differential diagnosis of SSSS from toxic epidermal necrolysis (TEN)

Staphylococcal scalded skin syndrome (SSSS) 1. 2. 3. 4. 5. 6.

Age – less than 5 years Skin shows marked tenderness Distribution – face, neck , axilla, groin Mucoca – not involved Prognosis – good Histology – subgranular split due to acantholysis

TEN 1. 2. 3. 4. 5. 6.

More than 40 years Mild to moderate tenderness No clear distribution Involved Poor Necrosis of epidermis

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Essentials in Dermatology 3. Desquamation of palms and soles 1 to 2 weeks after onset 4. Hypotension 5. Involvement of 3 or more other organ systems • About 85 to 90 percent of cases of TSS have occurred in women at the time of menstruation; almost all had been tampon users (particularly of super absorbent types). • Differential diagnosis: 1. SSSS– It has the presence of bullae, Nikolsky’s sign is positive with skin tenderness, but systemic organs are not involved and patient appears wellpreserved. 2. Scarlet fever– not usually associated with hypotension and shock. 3. Kawasaki’s syndrome– prolonged fever, cardiac involvement, generalized lymphadenopathy and absence of peripheral shock.

•

• • • • • •

of subcutaneous tissue whereas erysipelas is a bacterial infection of the dermis and upper subcutaneous tissue. Erythema, warmth, swelling and tenderness are constant features (Fig. 6.9). In erysipelas, the edge of the lesion is well defined and raised, but in cellulitis it is diffuse. In erysipelas, blistering is common. Except in mild cases, there are constitutional symptoms. The leg is the commonest site; the next most frequent site for classical streptococcal erysipelas is face. Milian’s sign – cellulitis of the face does not involve pinna, unlike erysipelas (as there is no subcutaneous tissue there). Without effective treatment, complications are common-fasciitis, myositis, subcutaneous abscesses, septicaemia, and nephritis. For presumed streptococcal infection, penicillin is the treatment of choice.

Streptococcal Pyodermas The major pathogen belongs to group A and is referred to as Streptococcus pyogenes. Streptococci colonise damaged skin, although less frequently than staphylococci. Major complications following streptococcal infection are rheumatic fever (following Streptococcal pyogenes pharyngitis), acute glomerulonephritis (both throat and skin infection), erythema nodosum and guttate psoriasis, and scleredema of Buschke (following throat infection). Impetigo and Ecthyma already discussed. Crowding, poor hygiene, and neglected minor skin trauma contribute to the spread of streptococcal impetigo in families.

Cellulitis and Erysipelas • Predominantly streptococcal disease, Staphylococcus aureus is occasionally implicated. • Cellulitis is an acute, subacute or chronic infection of loose connective tissue, mainly

Fig. 6.9: Cellulitis–lower leg showing shiny erythema and edema


Blistering Dactylitis • This is nearly always a group A streptococcal infection in children or teenagers. • A large blister containing thin seropurulent fluid forms on the distal phalanx, usually of a finger, typically on a phalangeal pad.

Perianal Streptococcal Infection • It occurs in children aged 1-10 years and is characterized by intense perianal erythema, perianal soreness, pain on defecation, faecal retention and blood-streaked stools.

Streptococcal Vulvovaginitis • Streptococcus pyogenes accounts for 10% of cases of vulvovaginitis in prepubertal girls.

Toxin Mediated Streptococcal Disease • Scarlet fever and streptococcal toxic shock like syndrome are due to toxins. • Scarlet fever is a diffuse erythematous eruption resulting from the production and subsequent circulation of pyrogenic exotoxins A, B, C (erythrogenic toxin) produced by group A streptococci usually located in pharynx. Incubation period – 2 to 5 days – starts with an acute tonsillitis. Rash appear on the 2nd day as finely punctuate erythema “Sunburn with goose-pimples”. Other features include Pastia’s lines (transverse streaks in the skin folds due to capillary fragility), pallor around mouth, red strawberry tongue, and high fever. Myocarditis may complicate this condition. • Group A streptococci cause an acute multisystemic syndrome coined toxic-shock –like syndrome (TSLS) resembling that caused by S. aureus.

• It is not only caused by group A streptococci but also due to other bacterial species (mixture of anaerobes/facultative organisms).

Diagnosis 1. Gram’s stained smear from the purulent material may demonstrate streptococci or staphylococci or both. 2. Culture and sensitivity: Swabs taken from infected sites may be sent for culture and sensitivity, so that appropriate antibiotic may be instituted to treat the condition. 3. Tzanck smear in SSSS shows acantholytic cells. 4. Histopathology: Histopathological examination is hardly required for the diagnosis. Treatment 1. General measures such as improved hygiene, loose light weight porous clothing, regular bathing, use of antiseptics in bath, antibacterial soaps, etc.

Necrotizing Fasciitis (Streptococcal gangrene) • It represents cellulitis that has progressed to gangrene of subcutaneous tissue followed by necrosis of overlying skin (Fig. 6.10).

Fig. 6.10: Necrotizing fascitis—cellulitis progressing to gangrene over the leg

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Essentials in Dermatology 2. Wet compresses: Condy’s compresses for crusted lesions of pyoderma. 3. Incision and drainage is indicated when furuncle has become localized and shows definite fluctuation. 4. Topical antibacterial agents such as gentian violet, neomycin, fusidic acid or mupirocin. 5. Systemic antibacterial agents: Systemic antibiotic therapy is indicated– i. For extensive lesions of pyoderma ii. For erysipelas, cellulitis, carbuncle, furunculosis, SSSS, etc. Penicillin is preferred for streptococcal infection whereas penicillinase resistant penicillin such as cloxacillin and cephalosporins are required for staphylococcal infections. Oral antibiotics (e.g., rifampicin 600 mg orally daily for 10 days) have been effective in eradicating S. aureus from most nasal carriers for periods of up to 12 weeks in cases having recurrent furunculosis. Intranasl application of 2% mupirocin ointment for 5 days can eliminate S. aureus nasal carriage in 70% of healthy individuals for up to 3 months.

NON-PYODERMAS Erythrasma • Causative agent-Corynebacterium minutissimum, diphtheroids bacillus, gram positive, non spore forming rod shaped organism. • Bacterial infection of the intertriginous areas like axilla, groin, gluteal cleft, inframammary folds, umbilicus and toe web spaces. • Usually manifest with asymptomatic red brown macules with sharp border (Fig. 6.11). • The best way to make the diagnosis is Wood’s light examination for coral red fluorescence. • Differential diagnosis: Hyperpigmented tinea versicolor (asymptomatic in nature) may appear like erythrasma. It predominantly affects upper trunk, individual lesions are small, but not erythematous and satellite lesions are more commonly seen than

Fig. 6.11: Erythrasma–asymptomatic brownish macular lesion with fine scaling in the axilla

erythrasma. KOH examination and culture from the lesions may clinch the diagnosis. Tinea cruris is characterized by pruritic well defined annular plaques with peripheral rim of papulopustules, and satellite lesions. • C. minutissimum can be cultured under aerobic condition • A short course of systemic erythromycin is the easiest method of treatment. Topical imidazole creams are also effective.

Trichomycosis Axillaris • Causative agent- Corynebacterium tenuis. • Collections of this bacteria forms concretions on hairs, usually in the axilla. • The axillary hairs are surrounded by whiteyellow (Fig. 6.12), red or black, difficult to remove concretions that extend for several centimetres. • Diagnosis is established by examining hair under microscope (Fig. 6.13), if necessary to culture the organism.


Fig. 6.14: Pitted keratolysis–soles demonstrating multiple pits

Fig. 6.12: Trichomycosis axillaris–yellow discoloration of axillary hairs due to concretions

Fig. 6.13: Trichomycosis axillaris–same patient's axillary hair under light microscope showing concretions over the hair shaft

• Differential diagnosis: Phthiriasis pubis can be differentiated by its associated pruritis and crawling sensation. Piedra can be differentiated by its gritty hard feeling and by doing a KOH examination. • The easiest treatment is to shave the area and treat the regrowing hairs with any topical disinfectant.

Pitted Keratolysis • Causative agent- Corynebacterium species, Streptomyces species, Dermatophilus congolensis and Micrococcus sedentarius. • Multiple pitted defects, 2-5 mm in size occur in thick horny layer of soles (Fig. 6.14). • The key factor is maceration, usually arising from hyperhidrosis, prolonged wearing of shoes and improper hygiene. • Differential diagnosis: The lesions are easily recognizable, but simple hyperhidrosis, erythrasma and tinea pedis have to be considered. • Topical erythromycin solution or benzoyl peroxide gel can be applied once or twice daily. Remove aggravating factors, if possible. Botryomycosis • Botryomycosis is a chronic suppurative, granulomatous disorder of bacterial origin. True non- filamentous aerobic and anaerobic bacteria such as Staphylococcus aureus, Pseudomonas species, Proteus vulgaris, Escherichia coli or Micrococcus cause it. • Botryomycosis needs to be differentiated from two other granulomatous diseases that

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•

Fig. 6.15: Botryomycosis–swelling of the foot with multiple nodules over it

form granules - mycetoma and actinomycosis, since clinically it has similar features (Fig. 6.15). • Effective treatment of botryomycosis depends on various factors such as the causative agent, location of the lesion and immune status of the host. Various drugs, mostly as single agents given for several weeks, have been successfully used in botryomycosis including trimethoprimsulfamethoxazole, minocycline, erythromycin and cefazolin. In addition to antibiotics, surgical excision and drainage of lesions may be useful in certain patients.

Actinomycosis • Actinomycosis is a chronic suppurative infection caused by anaerobic Actinomyces species. Actinomycetes are bacteria producing filamentous and branching hyphae. • Pathogenic organisms of these genera, namely Actinomyces israelii exists as a commensal in the oral cavity, tonsillar crypts and genital mucosa. • This organism gains entry when there is a disruption of mucosal barrier in the form of trauma or surgery. The resultant disease, actinomycosis is characterized by an early

•

• •

• •

inflammatory phase which resembles cellulitis and a more typical chronic phase, which presents as single or multiple indurated swellings (usually fibrosis). These swellings become soft and fluctuant and later suppurate, sometimes forming sinus tracts discharging yellow colour granules. These so called “sulphur granules” are lobulated masses of intertwining filaments. Human infections are categorized based on anatomical sites, namely cervicofacial (lumpy jaw), thoracic, abdominal, pelvic and primary cutaneous. Cervicofacial actinomycosis is the most common clinical presentation. It commonly follows dental extraction and presents as painful, indurated soft tissue swelling located at the angle of the jaw. Thoracic infection occurs due to aspiration and it involves lungs and pleura. Actinomycosis of gastrointestinal tract most commonly develops in ileocecal region and presents as appendicitis or slow growing mass. Pelvic actinomycosis occurs in women and is usually associated with the use of intrauterine device. Primary cutaneous actinomycosis is a rare type and probably occurs due to direct implantation of the organism. It usually occurs on the exposed skin (Fig. 6.16).

Fig. 6.16: Actinomycosis–scalp showing ulcerated indurated nodules with sulphur granules


Cutaneous Anthrax • Causative agent- Bacillus anthracis, gram positive bacillus, • The most common form of infection with Bacillus anthracis is an acute cutaneous lesion called “malignant pustule.” • Anthrax is primarily a disease of domestic and wild animals, but humans become accidentally involved through exposure to animals and their products. • Human anthrax occurs in three clinical forms: 1) cutaneous anthrax due to direct contact

with contaminated meat, carcasses, hides, hair, wool or bone, 2) inhalational anthrax (Woolsorter’s disease) due to inhalation of spores and 3) gastrointestinal anthrax due to ingestion of contaminated meat or milk. Anthrax meningitis occurs secondary to skin lesions, but it can complicate the other two forms also. • Cutaneous anthrax usually begins as a painless, pruritic papule within 3 to 10 days of inoculation. It rapidly progresses into a serous or serosanguineous vesicles, which ulcerate with a central black eschar, surrounded by a ring of vesicles within 36 hours (Figs 6.17 and 6.18). Perilesional oedema can be extensive. Toxic features occur in 50% of cases only and healing occurs in 1 to 3 weeks with variable scarring. • The following clinical features are strongly suggestive of cutaneous anthrax 1. The presence of edema out of proportion to the size of the lesion. 2. Lack of pain during the initial phases of the infection. 3. The rarity of polymorphonuclear leucocytes from vesicular fluid on gram’s stain and distinctive box car arrangement of bacillus anthracis (Fig. 6.19).

Fig. 6.17

Fig. 6.18

• A definite diagnosis of actinomycosis cannot made be on clinical grounds alone. Demonstration of sulphur granules, grams stain and culture from the appropriately obtained specimen is needed to confirm the diagnosis. Histopathology reveals granular colonies from which delicate mycelial filaments radiate. These colonies are surrounded by a chronic inflammatory infiltrate. • Actinomycosis may resemble various chronic inflammatory diseases such as tuberculosis, syphilis, etc. • Dramatic response to penicillin therapy occurs.

Figs 6.17 and 6.18: Cutaneous anthrax (Malignant pustule)–typical painless lesion with central blackish eschar surrounded by wreath of erythema, edema and vesiculation

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Essentials in Dermatology Ciprofloxacin, erythromycin, tetracycline and chloramphenicol are alternative drugs for penicillin sensitive patients.

Fig. 6.19: Anthrax–"Box car" arrangement of bacillus anthracis in gram's stained smear

• Differential diagnosis: Carbuncle Tenderness is prominent and there is presence of multiple furuncles in a group. Cow pox and sporotrichosis are other differential diagnoses. The history, rapid course, clinical appearance and lack of lymphangitis should suggest the diagnosis of anthrax which should be confirmed by bacteriological examination. • Since 20% of untreated cases of cutaneous anthrax develop bacteraemia, which leads to rapid death, cutaneous anthrax should be treated energetically with penicillin.

Bacillary Angiomatosis • Causative agent: Bartonella henselae (organism also causes Cat Scratch disease); rarely Bartonella quintana. • Infection is most common in HIV/AIDS patients, causes endothelial proliferation and produces vascular tumors. • Clinically, lesions are rapidly growing pyogenic granuloma like papules and nodules, which often ulcerate. • Purple, papular and nodular vascular lesions may resemble Kaposi’s sarcoma. • Diagnosis is based on demonstration of organism in the skin biopsy tissue section by Warthin Starry staining. Blood cultures are positive in half of the cases. • Differential diagnosis: Pyogenic granuloma and Kaposi’ sarcoma are close differential diagnosis, differentiation can be made by histopathological examination and demonstration of the organism by Warthin starry staining. • The mainstay of treatment is erythromycin. Alternatively, doxycycline or ciprofloxacin can be used.

Viral Infections

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Viral Infections

Many viral infections have cutaneous manifestations. These infections may sometimes be limited to the skin as in warts or molluscum contagiosum. In systemic viral infections, the frequency of skin lesions may range from almost always, as in varicella, to infrequently, as in cytomegalovirus. Viral infections of skin are characterized by a definite morphology and distribution. Most of the infections can be clinically diagnosed fairly and accurately without the need of sophisticated laboratory diagnostic aids. Five groups of viruses can affect the skin or adjoining mucous membrane surfaces. All except two (picorna virus group and retroviruses group) belong to DNA viruses. Viruses can be seen with the light microscope only when aggregated into inclusion bodies. Inclusion bodies are roughly spherical. Their average size is about 7 µm, the size of an erythrocyte. They are seen in three groups of viruses and are of two types. 1. Intranuclear inclusion bodies are seen in the herpes virus group and papilloma virus group. 2. Intracytoplasmic inclusion bodies are seen in the poxvirus group. In India, molluscum contagiosum is the predominant viral skin infection followed by

warts, herpes simplex, herpes zoster and chicken pox.

MOLLUSCUM CONTAGIOSUM • Molluscum contagiosum is caused by up to four closely related types of poxviruses, MCV-1 to - 4 and their variants. • The incubation period for the molluscum contagiosum (MC) has been reported to be between 14 and 50 days. • Skin to skin transmission is presumed to be the method of spread, including autoinoculation (the Koebner phenomenon), as well as contact with fomites. • It mainly affects children, sexually active adults and persons with impaired cellular immunity including HIV infection • In small children, virtually all infections are caused by MCV-1 whereas in patients infected with HIV, however, MCV-2 causes the majority of infections (60%), suggesting that HIV infection associated molluscum does not represent recrudescence of childhood molluscum. In all forms of infection, the lesions are relatively similar. • Individual lesions are smooth surfaced, firm, dome shaped pearly papules averaging 3 to 5 mm in diameter. Some

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Fig. 7.1: Molluscum contagiosum—typical umbilicated papule

Fig. 7.2: Molluscum contagiosum—lesions occurring over the face in a child

Fig. 7.3: Molluscum contagiosum—lesions occurring over genitalia in a child

“giant” lesions may be up to 1.5 cm in diameter. • A central umbilication is characteristic (Fig. 7.1) • Children more typically develop lesions on the face (Fig. 7.2), trunk and extremities, although perineal, scrotal (Fig. 7.3), perianal (Fig. 7.4), and groin lesions may be present as part of a wider distribution. • Clinically, molluscum in HIV positive persons appears to be transmitted both by sexual and nonsexual routes. The lesions are

Fig. 7.4: Molluscum contagiosum—lesions over the gluteal area and thigh in a child

more common on the face and neck (Figs 7.5 and 7.6).

Diagnosis • It is made clinically when necessary, histological examination is diagnostic.

Viral Infections

Fig. 7.5: Molluscum contagiosum—lesions around eye in a HIV patient

Fig. 7.6: Giant molluscum contagiosum just below the eye in an adult HIV patient

• Microscopic examination of a lesion crushed on a slide and left stained or unstained with Wright’s, Giemsa, Gram’s, or Papanicolaou stains demonstrates the inclusion bodies (Henderson Paterson bodies).

• Oral cimetidine or intravenous cidofovir can be used in widespread lesions.

Differential Diagnosis • Solitary MC may resemble pyogenic granuloma, keratoacanthoma or epithelioma. • Cutaneous cryptococcos is histoplasmosis, Penicillium marneffei infection, and basal cell carcinoma have been mistaken for molluscum in patients with AIDS.

• Human papillomavirus (HPV) has been associated with a broad spectrum of disease that ranges from asymptomatic latent infection to warts to squamous cell carcinoma. • This virus infects mucosal and cutaneous sites of immunocompetent as well as immunosuppressed patients. • It represents the most common mucocutaneous viral infection, and 3 to 5% of all patients have clinically evident warts. • Warts, or verrucae, are benign proliferations of the skin and mucosa that result from infection with papillomavirus. • These viruses do not produce acute signs or symptoms but induce slow-growing lesions that can remain subclinical for long periods of time. • These warts assume many clinical forms – common warts (verrucae vulgaris), filiform warts (digitate warts), flat warts (verrucae plana), plantar warts, genital warts (condylomata acuminata), oral and laryngeal

Treatment • In children, they may be left alone • Curettage– Individual lesions can be treated with curettage • Trichloroacetic acid (30-100%) application • 10% KOH application • Podophyllin – 10% to 25% • Silver nitrate • 5-FU topically • Cryosurgery • Electrodesiccation • Topical cantharidin, tretinoin (0.05% - 0.1%), cidofovir (1-3%), and imiquimod (5%) are other options

HUMAN PAPILLOMAVIRUS INFECTION (WARTS)

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papillomas and epidermodysplasia verruciformis. HPV types 1,2,3,4,7 cause first four forms of warts commonly. Genital warts are usually due to HPV types 6, 11, 16 and 18. Warts occur at any age, but are unusual in infancy and early childhood, the incidence of warts increases during the school years. Incubation period has been estimated to range between a few weeks to more than 1 year. Warts are spread by direct or indirect contact. Impairment of epithelial barrier function by moisture or trauma is predisposing factor.

• Paring shows multiple bleeding points or black dots representing thrombosed capillaries in warts. • Corns have a hard, painful translucent central core. • Lateral pressure on a wart causes pain, but corn is painful on vertical pressure. Plane warts: They are due mainly to HPV 3 and 10, and are smooth, flat or slightly elevated, skin colored or pigmented. The face (Fig. 7.8) and the backs of hand and shin are the sites of predilection. In differential diagnosis, lichen

Common warts: They are due mainly to HPV-2 and are characterized by symptomless, firm papules with a rough, horny surface, range in size from less than 1 mm to over 1 cm in diameter, and by confluence can form large masses. They are most commonly situated on the backs of hands and fingers (Fig. 7.7). They need to be differentiated from calluses. Warts do not have dermatoglyphics as opposed to calluses in which these lines are accentuated. Plantar warts: A plantar wart at first appears as a small, shining, sago grain papule, but soon assumes the typical appearance of a sharply defined, rounded lesion, with a rough keratotic surface surrounded by a smooth collar of thickened horn. Most plantar warts are beneath pressure points, the heel or the metatarsal heads. Mosaic warts are so described from the appearance presented by a plaque of closely grouped warts. Pain is a common, but variable symptom.

Differential diagnosis is corn/s. • On paring, epidermal ridges are seen to continue without interruption in corns, whereas in warts epidermal ridges are interrupted on the surface of the wart.

Fig. 7.7: Common warts—rough surfaced keratotic papules over the dorsa of hand

Fig. 7.8: Plane warts—smooth surfaced papules over the face

Viral Infections planus causes most difficulty. Lichen planus favours the flexor aspects of the forearms, is unusual on the face and is often itchy. The mucous membranes may be involved. The flat polygonal papules are lilac-pink and smooth and may show Wickham’s striae. Filiform and digitate warts: Occur commonly in the male, on the face and neck. They are irregularly distributed, often clustered. Digitate warts often in small groups also occur on the scalp in both sexes where they are occasionally confused with epidermal naevi. Anogenital warts: It has been estimated that up to 30 to 50% of sexually active adults are infected with HPV, making it the most common viral STD in some STD clinics. Most of them occur on the penis, scrotum, urethral meatus and perianal area in men and on the introitus, vulva, perineum and perianal area in women. The four morphological types are— 1. Cauliflower like (condyloma acuminata) (Fig. 7.9) 2. Papular 3. Keratotic 4. Flat topped. Bowenoid papulosis: is a clinicopathologic entity in which HPVs (HPV-16 is the most

Fig. 7.9: Genital warts—cauliflower like lesion of condyloma acuminata over the glans penis along with herpes genitalis

common that has been linked to it) have been identified. These appear as 2 to 3 mm papules often multiple over the external genitalia. Histologically, there is cellular atypia resembling Bowen’s disease. Buschke Lowenstein tumor (giant condyloma): This lesion emerges from a preexisting benign anogenital wart before developing into a verrucous carcinoma. Usually, it is associated with type 6 and 11. Clinically, it resembles a large aggregate of condyloma acuminata over the glans penis (Fig. 7.10), foreskin, vulva or anal region. Epidermodysplasia verruciformis: Usually manifests in childhood with widespread warts. It is an inherited disorder in which there is wide spread and persistent infection with HPV giving rise to a combination of plane warts, pityriasis versicolor like lesions and reddish plaques (Figs 7.11 and 7.12). Multiple warts that do not resolve spontaneously, recur after treatment, persist for years, and have an unusual morphology may suggest epidermodysplasia verruciformis. Malignant change is very common but metastasis is rare.

Fig. 7.10: Buschke Lowenstein tumor—a large verrucous growth seen involving the glans penis

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Fig. 7.11: Epidermodysplasia verruciformis— pityriasis versicolor like lesions over the forearm

Fig. 7.12: Epidermodysplasia verruciformis—verruca vulgaris like lesions aggregating over the dorsa of the foot

Diagnosis • Clinical diagnosis of warts is often sufficient but atypical, subclinical or dysplastic lesions may need laboratory confirmation of HPV infection. • Other than histopathology of lesion (Koilocytosis in the stratum granulosum and stratum spinosum), detection of virus particles by electron microscopy, immunocytochemistry using type specific antibodies, DNA hybridization on tissue extracts and polymerase chain reaction may be done.

• Electrosurgery: Electrodesiccation or electrofulguration may be done. • CO2 laser ablation. • Cryotherapy: Either using liquid nitrogen using dipstick method or spray method, or nitrous oxide cooled probes. • Keratolytic agents like 10 to 20% salicylic acid topically may be used. • Formalin soaks with 2 to 3% formalin in water every day for 15 minutes is useful for plantar warts. • Curettage or surgical excision for warts unresponsive to topical treatment. • Topical retinoic acid for flat warts. • Cantharidin causes blistering and focal destruction of the epidermis. • Cidofovir and 5-FU topically. • Intralesional bleomycin. • Injection of 0.1 ml of 1:1000 candida antigen in the base of each wart. • Topical sensitizer and systemic immunomodulators (Interferon, especially interferon

Treatment Most common warts disappear spontaneously within 2 year or with simple nonscarring treatment. Commonly employed modalities of treatment are: • Chemical cautery: Trichloroacetic acid is used for common warts, filiform warts and plantar warts.

Viral Infections alpha intralesionally or intramuscularly) may be employed depending upon the condition. • Extensive warts, even in hitherto untreatable epidermodysplasia verruciformis, have improved or cleared with oral isotretinoin or etretinate. • Treatment of genital wart is discussed separately in STD section but salient treatment options are given below. 1. Podophyllotoxin 0.5% self administered twice a day for 3 days in week for 4 weeks or podophyllin 25% in tincture benzoin, once or twice weekly. 2. Liquid nitrogen cryosurgery 3. Electrosurgery 4. Surgical excision 5. Chemical cautery 6. Topical imiquimod.

HERPES SIMPLEX INFECTION • The word herpes is derived from the Greek, meaning, “to creep”. • The human herpes simplex virus consists of two closely related viruses designated herpes simplex virus type 1 (HSV-1) and herpes simplex virus type 2 (HSV-2). The virus causes a wide variety of mucocutaneous infections and produce both primary and recurrent infections. • HSV -1 is generally associated with oral infection, and HSV-2 is associated with genital infection. However, HSV-1 genital infection and HSV-2 oral infection are becoming common, as a result of oral-genital sexual contact and as most these infections are asymptomatic • Transmission of HSV infections most frequently occurs through close contact with a person who is shedding virus at a peripheral site, mucosal surface, in genital or oral secretions. • Neonates often have maternally acquired HSV-1 antibodies, which decline to low levels by 6 months of age.

Fig. 7.13: Primary herpetic gingivostomatitis—lips also involved along with oral cavity with vesiculation, erosions and hemorrhagic crusting

• Primary herpetic infection of the mouth and pharynx (Herpetic gingivostomatitis) is a disease of children (Fig. 7.13) and young adults. The peak years of incidence occur between age 1 and 5. The usual onset is with fever, sore throat, painful vesicles and ulcerative erosions. • The clinical manifestations of genital herpes should be differentiated into first clinical episode and recurrent episodes. The first clinical episode can further be sub-divided into primary infection, occurring in a person without prior HSV 1 or 2 antibodies (true primary) or nonprimary infection, occurring in a person with prior HSV 1 or 2 antibodies. The patients with first episode non primary have lower frequencies of systemic symptoms, shorter duration of pain, fewer lesions and a shorter healing time compared with the true primary infections. • Primary genital HSV-2 and HSV-1 infections (Herpes genitalis) are characterized by frequent and prolonged systemic and local symptoms. Fever, headache, malaise and myalgia are reported in 40% of men and 70% of women with primary HSV-2 infection. Widely spaced pustular or ulcerative lesions on the external genitalia are the most frequent presenting signs. Ulcerative lesions persist for 4-15 days until crusting or re-epithelization occurs.

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Fig. 7.14: Recurrent herpes labialis—erosions seen over the angle of mouth on one side

Fig. 7.15: Recurrent herpes genitalis—closely grouped vesicles over the shaft of the penis

• Recurrences occur in 30-50% of cases of oral herpes (Fig. 7.14) but are more frequent after genital herpes infection (Fig. 7.15), developing in 95% of those with type 2 HSV compared with 50% in type 1 infection. They are usually triggered by fever, respiratory tract infection, ultraviolet radiation, trigeminal neuralgia, dental surgery or dermabrasion. Recurrent infections differ from primary infection in the smaller size of the vesicles, their close grouping and absence of systemic symptoms. • Herpes simplex virus type 2 is now the most common cause of genital ulceration among the sexually transmitted diseases. • Complications seen are eczema herpeticum or Kaposi’s varicelliform eruption (Widespread vesicular HSV lesions that arise from primary or recrudescent infection in patients with several dermatoses, particularly atopic eczema.), neurological sequelae (Aseptic meningitis, autonomic dysfunction especially of the sacral dermatomes, rarely, transverse myelitis, Bell’s palsy, Guillain Barre syndrome may follow orolabial HSV infection), extra genital lesions (extra genital lesions is a common complication of first

episode primary genital herpes and is seen over buttock, groin, or thigh, although finger and eye can also be involved.), and disseminated infection. • Atypical HSV presentation occur relatively often in HIV – infected patients, particularly severe lesions occur over back, buttocks or perianal region and these may expand to over 20 cm in diameter. Chronic HSV-2 ulcers of more than 1-month duration are an AIDS – defining illness. HSV may also cause life threatening disseminated infection, esophagitis, hepatitis and pneumonitis in AIDS – patients.

Fig. 7.16: Herpetic whitlow—grouped vesicles over the great toe

Viral Infections

Fig. 7.18: Tzanck smear showing multinucleated giant cell

Diagnosis

Fig. 7.17: Eczema herpeticum—widespread vesicular eruption over the face in a patient with allergic contact dermatitis

Clinical Entities Caused by HSV-1 1. Primary herpetic gingivo-stomatitis 2. Herpes labialis 3. Traumatic herpes—herpetic whitlow (Fig. 7.16), herpes gladiatorum 4. Eczema herpeticum (Fig. 7.17) 5. Atypical presentations—Zosteriform herpes simplex, varicella like eruption, neurologic herpes simplex 6. Ocular herpes simplex (Herpetic keratoconjunctivitis) 7. CNS involvement 8. Disseminated herpes.

• The most reliable way to make a diagnosis is by viral culture. • A valuable clinical approach for making a rapid diagnosis is Tzanck smear. Smears of the bases of deroofed HSV vesicles are stained with Giemsa or Wright’s stain and examined by light microscopy for multinucleated giant cells (Fig. 7.18). • Primary infections can be diagnosed by seroconversion or a rise in antibody titer. Differential Diagnosis 1. For herpes gingivostomatitis- Diphtheria, thrush, aphthosis, streptococcal infections, and Stevens Johnson syndrome. 2. For recurrent herpes labialis-Aphthous ulcers, erythema multiforme, and impetigo.

Treatment Acyclovir has modified the treatment outcome.

Clinical Entities Caused by HSV-2

Primary Infection

1. Genital herpes 2. Recurrent lumbosacral herpes simplex 3. HSV infections in the newborn.

Herpetic gingivo-stomatitis— • Acyclovir 5 mg/kg body weight 8 hourly for 7 to 10 days.

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Essentials in Dermatology • Others like eczema herpeticum and disseminated herpes need to be treated energetically on similar lines. • Herpetic whitlow and keratoconjunctivitis may be treated with oral acyclovir 200 mg 5 times a day for 7 to 10 days.

Recurrent Infection • Acyclovir 200 mg 5 times a day for 5 days or • Acyclovir 400 mg tds × 5 days or • Acyclovir 800 mg BD × 5 days Alternative antiviral drugs are famciclovir (125 mg, 250 mg twice daily) and valacyclovir (500 mg, 1 gm twice daily). For suppression of recurrences- Acyclovir 400mg twice a day for at least 1 year.

Treatment of Acyclovir Resistant Cases Include 1. Cidofovir: Unlike acyclovir, it is phosphorylated only by cellular enzymes, hence it is active against HSV with deficient or altered thymidine kinase enzyme. 2. Foscarnet: It is a phosphonate viral DNA polymerase inhibitor. It has been the preferred agent for patients with acyclovir resistant HSV infection.

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the sensory neurons following an earlier varicella attack. Both conditions are usually benign and selflimiting in patients with normal immunological status. One attack of varicella in childhood gives life long immunity to the patient. The incubation period is usually 14-17 days. Varicella occurs throughout the world, the highest incidence occurs in children aged 2-10 years. In tropical and semitropical countries, infection is delayed and varicella is seen more often in adults. Subclinical infection may occur. On the other hand, zoster is uncommon in childhood and the incidence increases with age. In patients with impaired immunity, both the incidence and severity of zoster are increased, and it is frequently complicated by disseminated cutaneous disease and systemic involvement, usually pneumonia, hepatitis, or encephalitis.

Vaccines: The precise role of vaccines in future management of HSV infection is yet to be determined. Modification of frequency and severity of recurrences may be achievable with vaccines. The most promising candidate vaccines are those based on HSV glycoproteins B and D.

VARICELLA-ZOSTER VIRUS INFECTION • Varicella (chicken pox) and herpes zoster are two distinct clinical entities caused by the same virus, varicella zoster virus (VZV). • Varicella is the primary exogenous VZV infection, while herpes zoster represents reactivation of an endogenous VZV infection, which persists in a latent form in

Fig. 7.19: Chicken pox—vesicular eruption over the trunk in different stages of evolution in an adult patient

Viral Infections usually by staphylococci or streptococci and may be prolonged by scarring. Other notable complications are primary varicella pneumonia, Reye’s syndrome, acute cerebellar ataxia, encephalitis, and disseminated varicella in immunosuppressed. • Maternal varicella during the early months of pregnancy can lead to ‘congenital varicella syndrome’, which manifests as extensive cutaneous scarring, muscular atrophy, limb hypoplasia, rudimentary digits, chorioretinitis and cortical atrophy. Fig. 7. 20: Chicken pox—dew drops on rose petals appearance

• In patients infected with HIV, zoster is 10 times more common than in normal population and may become disseminated and chronic.

Varicella • In older children and adults, the rash is often preceded by 2 to 3 days of fever, chills, malaise, headache, anorexia, severe backache and in some patients, sore throat and dry cough. • The rash of varicella begins on the face and scalp and spreads rapidly to the trunk (Fig. 7.19) with relative sparing of the extremities. • New lesions appear in successive crops but their distribution remains centripetal. • The lesion starts as a 2 to 4 mm red papule, which develops an irregular outline (rose petal) as a thin-walled clear vesicle appears on the surface (dew drop). This lesion, “dew drop on a rose petal” is highly characteristic (Fig. 7.20). The vesicles become umbilicated and pustular, rupture to form crusted lesions. • A distinctive feature of varicella is the simultaneous presence of skin lesions in all stages of evolution in any one area of the body. • The most common complication is the secondary bacterial infection of skin lesions

Differential Diagnosis of Varicella • Disseminated herpes simplex: Has concentration of lesions at and surrounding the site of the primary infection and there is associated marked toxicity and encephalitis. • Eczema herpeticum: Vesicles develop over the areas of active or recently healed atopic dermatitis. The lesions are particularly distributed on the face. Patients commonly have high fever and adenopathy. Zoster (Shingles) • The word ‘Shingles’ is derived from the Latin ‘singulus’ a girdle that refers to the segmental arrangement of the eruption.

Fig. 7. 21: Herpes zoster—grouped vesicles on erythematous patches in thoracic dermatome

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Fig. 7. 22: Herpes zoster—grouped vesicles on genitalia in a male

Fig. 7. 23: Herpes zoster ophthalmicus—unilateral involvement of forehead and scalp by herpes zoster

• Zoster afflicts 20% of the general population, during their life times, especially the elderly. • More than two thirds of the reported cases occur in individuals over fifty years of age and less than ten percent occur in those under the age of twenty years. • The first symptoms are usually pain and paraesthesia in the involved dermatome. This often precedes the eruption by several days and varies from superficial itching, tingling, burning or lancinating pain. • The rash is nearly always unilateral. • It begins as closely grouped maculopapules, which rapidly become vesicular in 12-24 hours (Figs 7.21 and 7.22) and then pustular in 2 to 3 days. The lymph nodes draining that area become enlarged and tender. The lesions dry up and crust in 7 to 10 days. Recovery takes about 3 to 4 weeks or longer in older patients. • The thoracic (53%), cervical (20%), trigeminal, including ophthalmic (18%) (Fig. 7.23) and lumbosacral (11%)

dermatomes are most commonly involved at all ages, but the relative frequency of ophthalmic zoster increases in old age. • Hutchinson’s sign: Vesicles on the side or on the tip of the nose that occur during episode of ophthalmic zoster are usually associated with serious ocular complications. • Complications of herpes zoster are hypopigmented or hyperpigmented scars, post herpetic neuralgia (usually defined as ‘chronic segmental pain’ appearing or persisting 3 months after cutaneous zoster healing), post herpetic anesthesia, recurrent zoster, motor neuron involvement (motor weakness usually follows the pain and eruption by a few days to weeks), ocular complications (superficial and deep keratitis, corneal ulcerations, panophthalmitis, ocular palsies, neuralgia, retinal vasculitis and optic neuritis), and rare complications include meningitis, pneumonitis, and myelitis. • Post herpetic neuralgia- Overall incidence is 8 to 15% and risk factors are old age, severe

Viral Infections • Skin biopsy of lesions also shows histological changes similar to those found in herpes simplex. • Definitive diagnosis accomplished by the isolation of virus in cell cultures and electron microscopy. • Identification of VZV antigens in cutaneous lesions and PCR for detection of nucleic acids in clinical specimen are the alternative methods of diagnosis.

Fig. 7.24: Herpes zoster—zoster occurring in multiple dermatomes

pain during acute phase, presence of prodromal pain, greater rash severity, and delay in starting treatment. • In HIV infected patients, herpes zoster ophthalmicus (HZO) is associated with several severe complications and may the initial manifestation of AIDS. Cutaneous lesions can be unidermatomal, multidermatomal (Fig. 7.24), disseminated or chronic and ulcerative. Differential diagnosis: Zosteriform herpes simplex- Vesicles vary in size in zoster, in contrast to uniformly sized vesicles in herper simplex and a history of multiple recurrences at the same site in herpes simplex.

Diagnosis • Tzanck smears of cutaneous lesions of varicella and zoster show multinucleated giant cells and epithelial cells with typical acidophilic intra-nuclear inclusions similar to those seen in herpes simplex.

Treatment • Symptomatic treatment is given in otherwise healthy patients. Rest and analgesics are advised. • Acyclovir is given in severe varicella and zoster (ophthalmic herpes zoster, Ramsay Hunt syndrome) and also in those patients who are at high risk of disseminated infection (more than 50 years of age, immunosuppressed patients). Acyclovir 800 mg 5 times a day for 7 to 10 days (in children dose is 20 mg per kg body weight four times daily). • Alternative antiviral drugs are famciclovir (500 mg three times daily), valacyclovir (1 gm three times daily) and foscarnet in acyclovir resistant cases. • Valacyclovir is contraindicated in immunosuppressed individuals. Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome have occurred in patients with AIDS, renal transplant and bone marrow transplant. • For post herpetic neuralgia-topical therapy (capsaicin, aspirin, lignocaine), nerve blocks, and systemic therapy (systemic steroids, carbamazepine, valproate, tricyclic antidepressants) may be tried. Gabapentin is the drug of choice now. Initial dose of 300 mg/day is increased over 4 weeks (up to 3600 mg/day divided into three divided doses) until efficacy obtained or side effects tolerable.

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• Live attenuated viral vaccine for varicella can be used in healthy children as a prophylaxis. Its efficacy persists for 10 years. • Varicella zoster immunoglobin therapy is indicated in those who are high risk patients, to prevent or modify varicella in them.

KAPOSI’S VARICELLIFORM ERUPTION (ECZEMA HERPETICUM) •

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It is a special form of widespread cutaneous viral infection occurring in a patient with preexisting skin disease. Atopic eczema is the most common predisposing condition. Other susceptible dermatoses include Darier’s disease, pemphigus foliaceous, ichthyosis vulgaris, mycosis fungoides and allergic contact dermatitis (Fig. 7.17). Majority of cases are due to infections with herpes simplex virus – called eczema herpeticum. Less commonly occur with other viruses like Coxsackie A 16 and vaccinia (eczema vaccinatum- no more seen). Clinically presents with clusters of umbilicated vesicles in areas where the skin has been previously abnormal. They may be associated with fever and other constitutional features. The face is usually severely affected and may be edematous.

• The evolved pustules become crusted and heal with permanent scarring in 2-6 weeks. • Multinucleated epithelial giant cells are seen in Tzanck smear. • Treated with antiviral such as acyclovir where infection is due to herpes simplex virus.

KAWASAKI DISEASE (KD) KD is an acute multisystem vasculitis of unknown etiology. It was first described in Japan by Kawasaki (1967). CDC diagnostic criteria: Fever lasting longer than 5 days plus at least four of the following: 1. Bilateral conjunctival injection. 2. Mucous membrane changes (1 or more): Red or fissured lips, red pharynx, ‘strawberry’ tongue. 3. Extremity changes (1 or more): Erythema of palms or soles, edema of hands or feet, desquamation (generalized or periungual) 4. Rash-erythematous exanthema. 5. Cervical lymphadenopathy (at least 1 node larger than 1.5 cm). • Death in KD occurs due to cardiac complications. • Treatment: aspirin, intravenous gammaglobulin.

Fungal Infections

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Fungal Infections

Fungi are aerobic organisms that form a cell wall and grow on or in organic material, forming a colony and reproducing either sexually or asexually. In contrast to plants, fungi do not manufacture chlorophyll. Without photosynthetic capabilities, they are dependent on other life forms including human beings. Fungi are also essential part of the ecologic cycle and also of considerable benefit to man as a source of antibiotics, in baking and in beer brewing. Fungal infections of skin may be divided into superficial and deep.

Superficial fungal infections DERMATOPHYTE INFECTIONS (TINEA, RINGWORM) • Causative agent: The dermatophyte (or ringworm). Three species of dermatophytes implicated are: 1. Trichophyton (affects skin, hair and nails) 2. Microsporum (affects skin and hair) 3. Epidermophyton (affects skin and nails) Humans acquire infection from three sources— soil, animal or humans • Dermatophyte fungi grow only within keratin layers, do not invade living epidermis

• Predisposing factors—Tropical climate of heat and humidity, poor nutrition and hygiene, debilitating diseases including HIV infection • It occurs at any age except tinea capitis which occurs in children mainly. • Most common dermatophyte incriminated is Trichophyton rubrum Tinea corporis—infection of the non-hairy skin of trunk and limbs • The typical lesions start as itchy erythematous macule or papules that spread outward and develop into annular (ring like) and arciform lesions with sharp, scaling or papulovesicular advancing margin and healing centers (Figs 8.1 and 8.2) • A variant of tinea corporis called tinea imbricata (imbricata is Latin for tiled) caused by Trichophyton concentricum, is characterized by large concentric rings, one inside another, which manifest commonly in childhood. • Other variants. 1. Majocchi’s granuloma–characterized by perifollicular granulomatous nodules on the scalps of children, often without pustulation 2. Agminate folliculitis- characterized by well defined erythematous plaques

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Fig. 8.1: Tinea corporis–typical annular erythematous ring like lesions of tinea corporis

studded with perifollicular pustules caused by zoophilic organism. • Differential diagnosis: A. For annular lesions are nummular eczema (does not have an advancing border, lacks central clearing), granuloma annulare (no scaling, presence of skin colored intradermal papules) and erythema annulare centrifugum (have scales at the trailing edge of the advancing border instead of over the entire border). B. For papulosquamous lesions are psoriasis (well defined erythematous plaques, scales are silvery white micacaceous and reveal bleeding points when removed positive ‘Auspitz sign’, lesions typically involve extensor extremities, scalp and sacrum), pityriasis rosea (papular or annular, oval multiple lesions have characteristic distribution pattern over the trunk, individual lesions have a collarette of scales, scale does not reach the edge of the erythematous border, this acute eruption, has self limited course). Tinea cruris-ringworm of the groin (“jock’s itch” “Dhobi itch”) • Most common in the tropics (Fig. 8.3). • Differential diagnosis are candidiasis (more often involves concavities of the flexures and produces macerated, moist, glazed, erythematous plaques with satellite pustules and it

Fig. 8.2: Tinea corporis–multiple ring like lesions over the trunk

Fig. 8.3: Tinea cruris–marked hyperpigmentation with active border showing erythematous papules

more often involves the scrotum), inverse psoriasis (tends to be asymptomatic, welldefined erythematous plaque), and erythrasma (lacks a scaling border and inflammation, asymptomatic in nature, fluoresces coral red under a wood light).

Fungal Infections

Fig. 8.4: Tinea faciei–erythematous scaly annular lesion over the face

Tinea barbae-ringworm of the beard and moustache. Tinea faciei-ringworm of the face- most often misdiagnosed (Fig. 8.4). Tinea capitis- ringworm of the scalp, transmitted from child to child, most commonly caused by Trichophyton violaceum. In the western literature, commonest causative organism recorded is T. tonsurans. Both of them cause endothrix infection (arthroconidia of dermatophytes contained within the hair shaft) of hair. Less frequently, ectothrix infection (arthroconidia of dermatophytes surrounding the hair shaft as a sheath) of hair also occurs. • Patchy hair loss and broken hairs, inflammation and scaling are characteristic of (back dot (Fig. 8.5), grey patch (Fig. 8.6), seborrheic dermatitis like) tinea capitis. • Kerion is caused by zoophilic fungi– Trichophyton verrucosum and Trichophyton mentagrophyte. Typically, it produces a boggy painful inflammatory swelling studded with exudative follicular pustules, hair fallen off or easily pluckable (Fig. 8.7). • Favus in India occurs mainly in Kashmir and is caused by Trichophyton schonleinii. On the scalp, concave sulfur-yellow crust (called scutulae) form around loose wiry hairs. It heals with thin smooth atrophic scarring.

Fig. 8.5: Tinea capitis–patchy hair loss with black dots “Black dot” type

Fig. 8.6: Tinea capitis–partial loss of hair, remaining hair in the alopecia patch area are luster less “Grey patch” type

Differential diagnosis are alopecia areata (patchy non-scarring alopecia, presence of exclamation mark hair, non-scaly condition), trichotillomania (irregular patchy hair loss from an approachable site because of hair pull trauma, so hairs are of different lengths), seborrheic dermatitis (greasy scaling is more diffuse, with erythema, changes confined to scalp and other

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Fig. 8.7: Tinea capitis–boggy inflammatory swelling over the scalp “Kerion” type

Fig. 8.8: Tinea pedis–scaly hyperkeratotic lesion on the sole of one foot, other normal for comparison

seborrheic areas of the body, generally no hair loss), and bacterial pyodermas of scalp (loss of hair is minimal, plucking of hair may be difficult and painful)

keratodermas and hyperkeratotic eczema. Contact dermatitis may also be considered although it affects the dorsum of foot than plantar aspect (tinea pedis). The vesicobullous type may be confused with pustular psoriasis, palmoplantar pustulosis, and bacterial pyodermas.

Tinea pedis- ringworm of the feet (“Athlete foot”) • Interdigital type-interdigital scaling and maceration with fissures is the most common form (Fig. 8.8). • Chronic hyperkeratotic type or Moccasin footwidespread scaling extends onto the sides of the feet. • Vesicular or bullous type due to some zoophilic fungi • Acute ulceratic type Differential diagnosis: For the interdigital type are erythrasma (very well defined lesion, it has whitish appearance usually asymptomatic and rarely causes fissures) and candidiasis (increased severity of maceration, denudation and pruritus). The hyperkeratotic type must be differentiated from palmoplantar psoriasis, palmoplantar

Tinea manuum-ringworm of the hands • Usually seen as mild erythema with hyperkeratosis and scaling, mainly over the palmar surfaces (Fig. 8.9). • Unilateral scaling with onychomycosis if present should always be scraped for fungus (Fig. 8.10). • Contact dermatitis, psoriasis, and hyperkeratotic eczema must be considered as differential diagnosis. Tinea unguium- fungal infections of the nails • Nail infection starts at the free margin or lateral borders of the nails, as yellow discoloration and progress proximally (distal and lateral subungual onychomycosis).

Fungal Infections

Fig. 8.9: Tinea manuum–unilateral scaly lesions of the palm

Fig. 8.11: Tinea unguium–thickening and dystrophy of involved nails

infection caused by dermatophytes that cause endothrix scalp infection characterized by scarred nail plate with pits and lamellar splits). • Ringworm of the nails is rarely symmetrical and it is common to find the nails of only one hand affected) • Differential diagnosis is psoriasis (coarse pitting of dorsal nail plate is never produced by fungal infection, and strongly suggests psoriasis, as does the oil drop sign from the free edge), eczema (irregularly buckled nails) and lichen planus (ridged or dystrophic nails) Tinea incognito- ringworm infections modified by corticosteroids, systemic or topical, prescribed for some pre-existing pathology or given mistakenly for the treatment of misdiagnosed tinea (Fig. 8.12). Fig. 8.10: Tinea manuum with tinea unguium

• The nails become brittle, friable, and thickened (Fig. 8.11). • Four distinct patterns of tinea unguium are distal and lateral subungual onychomycosis (most common pattern), superficial white onychomycosis, proximal subungual onychomycosis (associated with HIV/AIDS cases), and endonyx onychomycosis (nail

Diagnosis • KOH mount– definitive diagnosis is made by the microscopic examination and identification of hyphae and spores in scales (Fig. 8.13) or hair. In nails, the presence of hyphae usually means dermatophyte infection, rarely saprophytic fungi infection. • Cellophane tape examination and staining with periodic acid Schiff’s (PAS) reagent of

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Fig. 8.12: Tinea incognito–erythematous papular eruption of tinea due to immunosuppression

Fig. 8.14: Periodic Acid Schiff’s (PAS) stained smear showing septate branching hyphae–dermatophytes

2. Imidazole derivatives – miconazole (2%), clotrimazole (1%), econazole (1%), ketoconazole (2%), oxiconazole, sulconazole, eberconazole 3. Ciclopirox olamine 4. Undecylenic acid 5. Tolnaftate 1% 6. Azelaic acid 7. Castellani’s paint 8. Terbinafine (1%) Fig. 8.13: KOH mount–showing branching septate hyphae

the material taken from skin (Fig. 8.14), hair and nails can be studied. • Confirmed by culture on Sabouraud’s media. • Histological examination of nails with special stain-Periodic Acid Schiff’s stain.

Therapy • Topical therapy- indicated for infections of the body and groin and superficial involvement of the beard region, palms, and soles Topical agents used are— 1. Whitfield ointment (contains 6% benzoic acid and 3% salicylic acid)

Duration of therapy is 4 to 6 weeks or 2 weeks more after clearance of lesions. • Systemic therapy– required in case of hair and nail infection and in those cases where tinea is extensive, widespread and non-responsive to topical therapy. Griseofulvin (10-20 mg per kg body weight or 250 mg twice a day), ketoconazole (200 mg daily), itraconazole (200 mg daily or one week pulse of 400 mg daily for 2 to 3 months), fluconazole (6 to 8 mg per kg body weight daily or 150 mg, 300 mg or 450 mg per week for 3 to 6 months) and terbinafine (250 mg daily or 250 mg twice a day) are the drugs used systemically. The skin and hair infection require shorter course of therapy (6-8 weeks therapy with griseofulvin) whereas nail infection require longer therapy (9-12 months with griseofulvin).

Fungal Infections

TINEA VERSICOLOR (PITYRIASIS VERSICOLOR, DERMATOMYCOSIS FURFURACEA, CHROMOPHYTOSIS) • It was thought that a single polymorphic yeast Pityrosporum ovale, or two species P. ovale and P. orbiculare, were the causative agents, but it is now recognized that this genus was invalid. There are at least 7 separate species of lipophilic yeast- Malassezia on the human skin: M-sympodialis (most commonly found on the normal skin), M.globosa (most frequently associated with tinea versicolor), M. restricta, M. dermatis, M.slooffiae, M.obtusa and M. furfur. • Predisposing factors- Warm and humid climate of tropics, pregnancy, serious underlying diseases, immunocompromised host, Cushing’s syndrome, malnutrition. • The term “versicolor” refers to the presence of both hyper-and hypopigmented lesions • A peculiar aspect of tinea versicolor is its propensity to present as either hypopigmented or hyperpigmented, finely scaling, round or perifollicular coalescing macular patches found primarily over the trunk (Figs 8.15 and 8.16). The hypopigmentation is explained on the basis of dicarboxylic acids produced by Malassezia species (e.g., azelaic acid) causing competitive inhibition of tyrosinase and perhaps a direct cytotoxic effect on

Fig. 8.15: Tinea versicolor–hypopigmented macules with fine powdery scales

Fig. 8.16: Tinea versicolor–hyperpigmented macules with fine scaling

hyperactive melanocytes. The hyperpigmentation may be due to abnormally large melanosomes and thicker keratin layer. To elicit fine branny powdery scales, candle grease sign or coup d’ongle sign is elicited. • The eruption is almost always asymptomatic and only of cosmetic significance • Other cutaneous disorders associated with Malassezia yeasts are seborrhoeic dermatitis, Malassezia folliculitis, Confluent and reticulate papillomatosis, and sebopsoriasis.

Diagnosis • KOH preparation-reveals numerous short, straight and angular hyphae and clusters of thick walled round and budding yeasts “Spaghetti and meat ball appearance” or “Banana and grapes appearance”(Fig. 8.17). • Cellophane tape examination and staining with periodic acid Schiff’s (PAS) reagent and India ink of the material taken from skin (Figs 8.18 and 8.19).

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Fig. 8.17: KOH mount–clusters of spores with short mycelial filaments. “Spaghetti and meat ball” appearance

Fig. 8.18: Periodic Acid Schiff’s (PAS) stained smear showing cluster of spores with short stumpy mycelial filaments- Malassezia

• Culture- Sabouraud’s dextrose agar overlaid with sterile olive oil or lanolin. Antibiotics such as penicillin, streptomycin and cycloheximide are incorporated to reduce growth of contaminants. • Wood’s light examination-golden to orange fluorescence.

Differential Diagnosis 1. Pityriasis alba– characterized by poorly marginated, hypopigmented, slightly scaly patches on the cheeks of young children.

Fig. 8.19: Parker ink staining of hyphae and spores of tinea versicolor

2. Indeterminate leprosy– usually seen in children with one or more macules, hypopigmented to faintly erythematous with an ill-defined to well defined edge. Lesions may be on the face or the limbs. Slight anaesthesia may be demonstrable. 3. Vitiligo– characterized by depigmented macules without any scaling 4. Erythrasma– Hyperpigmented tinea versicolor can be confused with erythrasma, but satellite lesions are less common in erythrasma and there is coral red fluorescence under Wood’s lamp. 5. Seborrhoeic dermatitis– May occur in areas of tinea versicolor distribution, but patches have an erythematous yellowish tint and scales are soft and greasy

Therapy – Topical– Selenium sulfide suspension (2.5%), zinc pyrithione, ketoconazole (2%) shampoo, sodium hyposulfite (25%), propylene glycol and imidazole derivatives are used topically. – Systemic– Ketoconazole (200 mg daily for 5 to 10 days), itraconazole (200 mg daily for 5 to 7 days) or fluconazole (single oral dose of 400 mg) are effective systemically.

Fungal Infections

CANDIDIASIS –

–

Causative agent –Candida albicans. This yeast like fungus is normally found on the mucous membranes, skin, in the gastrointestinal tract and in the vagina. Under certain circumstances, it changes from a commensal organism to a pathogen. Apart from Candida albicans, few other species of Candida like C. tropicalis, C. dubliniensis, C. parapsilosis, C. guilliermondii, C. krusei, C. glabrata may be involved Predisposing factors– Local factors are moisture, warmth, maceration, and/or occlusion and systemic factors are antibiotics therapy, corticosteroids, oral contraceptive pills, pregnancy, diabetes, immunocompromised state including HIV infection.

can be confused with coated tongue due to dehydration, mucositis due to chemotherapeutic drugs, aphthous ulcerations, herpetic infections, oral hairy leukoplakia, erythema multiforme, pemphigus, lichen planus, discoid lupus erythematosus, Pernicious anemia, histoplasmosis, leukoplakia, secondary syphilis. • Perleche–sore angles of mouth. • Intertriginous type– Well-defined peeling border around moist red macerated lesions and surrounded by satellite papules or pustules- occur in body folds such as groin, inframammary, axillary, perianal and interdigital areas (Fig. 8.22). Differential

Clinical Manifestations • Oral thrush– It presents as curdy, white, easily detachable deposits on tongue or oral mucosa with underlying bright red and moist surface (Figs 8.20 and 8.21). This form of candidiasis is also known as acute pseudomembranous candidiasis. It is the most common form of oral candidiasis. Other forms of oral candidias is include acute and chronic atrophic candidiasis, chronic hyperplastic candidiasis, and median rhomboid glossitis. Oral thrush

Fig. 8.21: Oral candidiasis with angular cheilitis in a HIV adult case

Fig. 8.20: Oral candidiasis–curdy white deposits on the hard palate in a baby

Fig. 8.22: Intertriginous candidiasis–toe cleft showing moist macerated lesion

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Essentials in Dermatology diagnosis includes tinea cruris, seborrhoeic dermatitis, flexural psoriasis and bacterial intertrigo. • Paronychia– inflammatory boggy swelling of posterior and lateral nail folds of digits of fingers (Fig. 8.23), commonly seen in house wives, maidservants, due to continuous wet job • Vulvovaginitis-frequently associated with itching and vaginal discharge. The vaginal mucosa shows erythema, edema and curdy white deposits (Fig. 8.24). Candidal balanoposthitis occurs as a counter part in males (Fig. 8.25).

Fig. 8.25: Candidal balanoposthitis–glazed erythema with pustulation over glans and prepuce

Diagnosis • KOH preparation-reveal budding yeasts with or without hyphae or pseudohyphae. • Gram’s stained smear-gram positive organisms longer than bacteria. • Confirmed by culture on Sabouraud’s media. Whitish mucoid colonies grow within 2 to 5 days.

Fig. 8.23: Chronic paronychia–swelling of proximal and lateral folds of many fingers with nail changes

Fig. 8.24: Candidal vulvovaginitis–vulval erythema with white deposits

Therapy • Evaluation and treatment of underlying medical conditions • Nystatin, imidazoles and broad-spectrum triazoles are the agents of greatest use in the treatment of candidal infection. Gentian violet and Castellani’s paint are older effective topical remedies. • Potassium permanganate soaks are more effective in moist Candidal intertrigo. • Recurrent or recalcitrant infections require oral medication with nystatin (to rid the gut and vagina of candida organisms), fluconazole (50 mg daily for 14 days), ketoconazole (200 mg daily) or itraconazole (200 mg daily). • For vaginal candidiasis, 500 mg clotrimazole vaginal tablet once or 200 mg miconazole tablet at bed time for 3 days or single oral dose

Fungal Infections of 150 mg of fluconazole or twice daily dose of itraconazole (200 mg twice a day).

CHRONIC MUCOCUTANEOUS CANDIDIASIS • It is a rare syndrome which is characterized by recurrent and persistent candidal infection of skin, nail and mucous membrane. It has autosomal dominant or recessive inheritance. Several endocrinopathies (e.g. hypothyroidism, hypoadrenatism, etc.) may be associated. • Treatment: Treatment of this condition depends critically on systemic antifungal chemotherapy. Attempts have been made to restore T cell function, by the use of transfer factor, or thymosin, or grafting compatible T lymphocytes and non specific measures like the restoration of normal iron stores. Prolonged and repeated use of systemic antifungals like fluconazole, itraconazole and ketoconazole may be necessary.

PIEDRA (PIEDRA = STONE) (TRICHOSPOROSIS) • Causative agent– White piedra-Trichosporon beigelii (Trichosporon cutaneum), a yeast seen in temperate region – Black piedra-Piedra hortae, a mould, occurs mostly in the tropics • Pinhead sized, hard nodes occurs on the hairs of the scalp, brows, lashes, or beard • KOH examination and culture clinch the diagnosis • Differential diagnosis– Nits, hair casts, developmental hair defects and trichomycosis axillaris may be differentiated by doing a microscopic examination. • Treatment is by cutting hair. Oral terbinafine 250 mg daily for 6 weeks has been shown to be effective against black piedra. For white piedra, imidazoles, selenium sulfide, zinc pyrithione, etc. are effective agents.

Deep fungal infections

TINEA NIGRA

MYCETOMA

• Causative agent- Phaeoannellomyces werneckii (syn. Exophiala werneckii), a mould which produces a melanin like pigment. • This characteristic disorder manifests as one or several brown or black spots that resemble silver nitrate or India ink stains. • Most frequently lesions occur on the palms but also on the soles. • The fungus can be easily demonstrated by KOH examination and culture. • Differential diagnosis are junctional naevi, melanoma, Addison’s disease, and chemical stains, however, tinea nigra can be easily differentiated by doing a KOH examination which shows brown coloured hyphae and spindle shaped yeast cells. • Topical imidazoles such as clotrimazole, miconazole, ketoconazole, etc. are effective.

• Mycetoma is a deep fungal infection, characterized by a clinical triad of swelling, discharging sinuses and discharge containing granules (Figs 8.26 and 8.27). It commonly occurs on the foot, hence also called as Madura foot. • Mycetoma-caused by species of fungi is known as eumycetoma, and that caused by aerobic actinomycetes or filamentous bacteria as actinomycetoma. Type of mycetoma Causative organisms Eumycetoma

Actinomycetoma

Madurella mycetomatis Exophila jeanselmei Pyrenochaeta romeroi Fusarium species Actinomadura madurae Nocardia brasilensis Actinomadura pelletieri

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Fig. 8.26: Mycetoma–a triad of features – swelling, discharging sinuses and discharge containing granules seen commonly over the foot

• Differential diagnosis 1. Chronic osteomyelitis of bacterial or tuberculosis origin (characteristic grains are not discharged) 2. Elephantiasis (no sinus tracts) 3. Primary cutaneous actinomycosis (develops on the exposed sites, very rare type of actinomycosis, caused by Actinomyces isralei, a normal inhabitant of human mouth, thus it is an endogenous infection). • Actinomycetomas generally respond to antibiotics such as a combination of dapsone with streptomycin or sulfamethoxazoletrimethoprim plus rifampin or streptomycin. Amikacin may also be used in recalcitrant Nocardia infections. • Of the fungal causes of mycetoma, M. mycetomatis may respond to ketoconazole (200 mg daily over several months). For the others, a trial of therapy with griseofulvin or itraconazole is worth attempting. Surgery, usually amputation, is the definitive procedure and may have to be used in advanced cases.

CHROMOMYCOSIS (CHROMOBLASTOMYCOSIS)

Fig. 8.27: Mycetoma over the upper back

• The organisms are usually soil or plant saprophytes that are only incidental human pathogens. • KOH examination, Gram’s staining, histopathology of lesion and culture helps in confirmation of the diagnosis.

• Chromoblastomycosis is primarily a disease of tropical or subtropical regions. • At least five distinct organisms are well known to cause chromoblastomycosis: Fonsecaea pedrosoi, Fonsecaea compactum, Cladosporium carrionii, Phialophora verrucosa, and Rhinocladelia (Acrotheca) aquaspersa. • The characteristic feature of a well-developed lesion is a pruritic warty plaque of the extremities in an agricultural worker, which drains purulent material (Fig. 8.28). • The pathology of chromoblastomycosis consists of striking epidermal thickening (pseudoepitheliomatous hyperplasia) over-

Fungal Infections

Fig. 8.28: Chromomycosis–verrucous itchy plaque lesion over the dorsa of the hand

Fig. 8.29: Rhinosporidiosis–pink friable polypoidal growth projecting from the nares

lying a suppurative granulomatous dermatitis. At least three appellations are frequently used to designate the tissue form of these fungi: Medlar bodies, sclerotic bodies, muriform bodies or the descriptive “copper pennies”. • Differential diagnosis: 1. Blastomycosis (presence of sharp border with minute abscesses and by the presence of pulmonary lesions) 2. Cutaneous tuberculosis and leishmaniasis (biopsy and culture will establish the diagnosis) 3. Elephantiasis verrucosa nostras (mossy foot). • The known treatments may be divided into three groups: surgery, physical modalities (heat, cryotherapy, electrosurgery, and radiation therapy), and systemic antifungal medications(amphotericin B, 5-fluorocytosine, triazole derivatives especially itraconazole, and thiabendazole).

• It is seen most often in southern India and Srilanka. • It is more common in adult males and is possibly transmitted to man by direct contact with spores through dust, infected clothing or fingers and swimming in stagnant waters. • Rhinosporidiosis frequently involves the nasopharynx (70%) presenting as a painless, friable, polypoidal growth, which may hang, anteriorly from the nares (Fig. 8.29) or posteriorly into the pharynx. The lesions are pink or purple red and studded with minute white spots which are the sporangia containing spores. Nasal obstruction and bleeding are the most common symptoms. The conjunctiva and lacrimal sac are involved in 15% of cases. • Occasionally, it affects the lips, palate, uvula, maxillary antrum, epiglottis, larynx, trachea, bronchus, ear, scalp, vulva, vagina, penis, rectum or the skin (Figs 8.30 and 8.31). • Since the causative agent cannot be cultured, the diagnosis can be confirmed by demonstrating typical sporangia and spores in histopathology and imprint smears. • Differential diagnosis is nasal polyps, warts, and condylomas.

RHINOSPORIDIOSIS • Rhinosporidiosis is a chronic granulomatous mycosis caused by Rhinosporidium seeberi.

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Fig. 8.30: Rhinosporidiosis–pink friable polypoidal growth over the root of the nose

Fig. 8.32: Subcutaneous phycomycosis–shiny disk like indurated lesion over the thigh, can be insinuated at the margin with fingers

Fig. 8.31: Rhinosporidiosis–noduloplaque lesions over the arms

• Surgical removal and electro desiccation are the treatments of choice.

SUBCUTANEOUS ZYGOMYCOSIS • Subcutaneous zygomycosis or subcutaneous phycomycosis (SP) has two clinically and mycologically distinctive entities termed as basidiobolomycosis (etiological agent: Basidiobolus ranarum) and conidiobolomycosis (etiological agent: Conidiobolus

coronatus). These organisms belonging to Entomophthorales cause granulomatous infection that usually affects healthy people. • SP due to Conidiobolus is uncommon. Clinically, the disease is characterized by nasal obstruction due to the inflammation of the submucosa of the nostril, usually in the vicinity of the inferior turbinate. • SP is also caused by Basidiobolus. The site of infection is usually confined to the limb girdles or proximal limbs. It occurs chiefly in children. Characteristically, it manifests as painless, well-circumscribed, firm to hard subcutaneous masses, which grow slowly at the periphery and may envelop parts of or a whole limb (Fig. 8.32). The border is smooth, rounded, clearly defined, and can be raised up by inserting fingers underneath it. This is thought to be an almost diagnostic clinical feature of the disease. There is no involvement of the regional lymph nodes.

Fungal Infections • Differential diagnosis: 1. Lymphatic edema (no distinctive edge) 2. Subcutaneous malignant lymphoma (grows more rapidly) 3. Subcutaneous morphea • A therapeutic trial with potassium iodide in a clinical setting may be considered as an important criterion for diagnosis where facilities to culture the organism do not exist.

SPOROTRICHOSIS • It is caused by Sporothrix schenckii and is characterized by nodulo-ulcerative and crusted lesions arranged in a linear fashion over the extremities with intervening lymphatics thickened like a cord. • The best sources of diagnostic material are smears, exudates, and biopsies (to look for “Asteroid bodies”). S. schenckii is very rarely seen in direct microscopic examination because yeasts are usually present only in small numbers; the organism can be readily isolated on Sabouraud’s agar. • Differential diagnosis: 1. Fish tank granuloma 2. Cutaneous leishmaniasis • Potassium iodide (saturated solution) is effective in the cutaneous types of sporotrichosis. Other deep fungal infections: Cryptococcosis and aspergillosis are ubiquitous throughout the world. In south east Asia, penicillinosis is common whereas coccidioidomycosis and histoplasmosis are restricted to certain geographic regions.

CRYPTOCOCCOSIS • Cryptococcosis is an opportunistic infection caused by the encapsulated yeast Cryptococcus neoformans.

• Virtually all infections involve the central nervous system, with meningitis the most frequent manifestation. • Cutaneous dissemination occurs in 10% to 20% of patients, has a variable presentation and may precede other signs of infection. • Initial signs of cryptococcosis include cellulitis, genital or oral ulcerations, or molluscum-, herpes simplex-, or Kaposi’s sarcoma- like lesions. • Diagnosis can be made by performing curettage on a lesion, by making a potassium hydroxide preparation, India ink preparation, isolation of fungus on culture or by a biopsy of lesion. Cryptococcal antigen is present in these patient sera and can be detected by latex particle agglutination. • Intravenous amphotericin B alone or with flucytosine and oral fluconazole is highly effective in the treatment of cryptococcus infection.

PENICILLIOSIS • Penicillium marneffei is the only penicillium species that is dimorphic and can cause systemic mycosis in human beings, particularly those who are immunocompromised. • Features of the infection frequently include fever, anemia, marked weight loss, cough and diarrhea, but skin eruptions occur in the majority. • Cutaneous manifestations usually consist of a generalised papular eruption, in which the papules may be umbilicated (due to central necrosis), although necrotic papules, nodules, folliculitis, macular rash and mouth ulcer have also been reported. • Diagnosis depends on isolation of the organism from blood or tissue. • Treatment includes systemic amphotericin B, itraconazole or fluconazole.

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Infestations

• Causative agent: Sarcoptes scabiei var. hominis (Fig. 9.1) • Morphology: The mite has an ovoid body, flattened dorsoventrally. The body is creamy white marked with transverse corrugations, and on its dorsal surface by bristles and spines (denticles). The mite has four pairs of short legs. The rear two pairs of legs of female mites end in long bristles called setae. Adult female mite measures about 0.4 × 0.3 mm whereas adult male about 0.2 × 0.15 mm. The mite prefers non-hairy skin and areas of low sebum production.

• Life cycle: Copulation occurs in burrows excavated by female mite in stratum corneum. After copulation, the pregnant female enlarges the burrows and begins egg laying. It travels 5 mm per day and lays 4050 eggs during its life span of 4-6 weeks (Figs 9.2 and 9.3). These eggs hatch in a week and reach maturity (eggs-larvae-nymph-adult) in about 3 weeks. • Most infected adults harbor 10 to 12 mites • Mode of spread-close personal contact, but may be transmitted through clothing, or towels. • Incubation period- when a human is infested for the first time, symptoms usually

Fig. 9.1: Sarcoptes scabiei mite (300-400 microns)

Fig. 9.2: Eggs and fecal pellets of sarcoptes

SCABIES (THE ITCH, SEVEN YEAR ITCH)

Infestations

Fig. 9.3: An egg of sarcoptes

Fig. 9.5: Scabies – periumbilical papular lesions

Fig. 9.4: Scabies – typical finger web spaces involvement with papular, vesicular and crusted lesions

Fig. 9.6: Scabies – genital and thigh area involved by papular and excoriated lesions

develop after 3-6 weeks while after reinfestation, they occur within 1 or 2 days. • The classic symptom is intense pruritus especially at night in bed • The sites of predilection are the interdigital spaces (Fig. 9.4), wrists, points of elbows, antecubital fossae, the anterior axillary folds, the umbilicus (Fig. 9.5), and the genitalia (Fig. 9.6) especially the gluteal cleft (Fig. 9.7) (“circle of Hebra”). • The most diagnostic or pathognomonic finding is an intact “S” shaped or linear burrow with a papule or vesicle at its end housing the mite (Fig. 9.8). Most common

Fig. 9.7: Scabies – papular lesions over the gluteal area

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Fig. 9.8: Scabies—typical burrows of scabies of the shaft of the penis

•

•

•

•

sites are webspaces of the hands, wrists, and lateral aspect of palms. Generalized urticarial papules, excoriations and eczematous changes are secondary lesions caused by sensitization to the mite. Tiny scaly papules on the nipple and male genitalia (glans, shaft and scrotum) are pathognomonic of scabies. Infants and small children often have vesicular lesions on the palms, soles, head and neck (Figs. 9.9 and 9.10). Scabies in babies is generally more extensive in distribution of burrows, vesicular or vesicopustular lesions on the hands and feet, extensive eczematization, multiple crusted nodules on the trunk and limbs. Nodular scabies- in some cases, itching nodules (5–20 mm in diameter, red, pink, tan or brown in color) persist for several months. They are found most commonly on the scrotum (Fig. 9.11). Burrow may be seen on the surface of early nodules.

Fig. 9.9: Scabies in an infant—papulovesicular lesions in finger web spaces

Fig. 9.10: Scabies in an infant – vesicular, pustular and crusted lesions on the ankle and feet

• Scabies incognito means modified clinical picture of classical scabies which mimic other dermatoses due to inappropriate use of topical steroids. • Complications of scabies: Secondary infection of skin lesions, eczematization, nephritogenic strains of streptococci may produce secondary sepsis, and glomerulonephritis particularly in tropics.

Infestations

•

Fig. 9.11: Nodular scabies—nodules seen over the scrotum

• Diagnosis: If a mite is demonstrated, one needs no diagnostic criteria. Typical lesions on the penis and nipple, the presence of burrows even without a mite and interdigital lesions are almost diagnostic. Severe pruritus, especially at night, of short duration or in family members is also very suggestive. • Burrow identification (Ink method): The suspected burrow is smeared with blue or black fountain pen ink and then wiped off with an alcohol swab after some time. The dye that enters the burrows is highlighted as a dark line. • Microscopic examination: The burrow is scraped with 15 no. blade and examine the material with 10% KOH or mineral oil under light micrscope. Presence of mite, egg or fecal concretions (scybala) confirms diagnosis of scabies. • Under dermoscope, mite in burrow resembles “jet with contrail”. • Differential diagnosis: A. For pruritic localized or generalized rash: In infants: Papular urticaria, infantile acropustulosis, In children: Papular urticaria, insect bite reactions,

• • • •

atopic dermatitis, animal scabies, In adults: acute generalized lichen planus, adverse drug reactions, contact dermatitis, pediculosis pubis, pediculosis corporis, different forms of prurigo, In elderly: Dermatitis herpetiformis, senile pruritis, delusional parasitosis. B. For pruritic nodules: Urticaria pigmentosa, papular urticaria (insect bite), and pseudolymphoma. Therapy: Permethrin (5%) cream is treatment of choice (single overnight application below neck all over the body with a second application after an interval of a week). It is the treatment of choice for infants (application includes head and neck also). Sulfur and crotamiton are safe in pregnancy. Other agents used are gamma benzene hexachloride lotion (1%), benzyl benzoate lotion (12.5% for infants and children, 25% for adults), esdepallethrine 0.63%, malathion 0.5% lotion, ivermectin 0.8% lotion, and monosulfiram (25%) diluted with two or three parts of water to form an emulsion. Ivermectin 200 microgram per kg body weight single oral dose is also effective in many cases of ordinary scabies, but presumably because of lack of ovicidal activity, higher cure rates are obtained with two doses separated by an interval of a week. It is a useful modality of treatment for institutional outbreaks of scabies as it is cheap, effective and easy to administer. Pruritus may persist for up to 1-2 weeks after the end of effective treatment. Treat infested individuals as well as close physical contacts simultaneously. Bedding and clothing should be washed in hot cycle of washing machine. Intralesional triamcinolone 5-10 mg / ml in each lesion is used for nodular scabies besides routine scabies treatment.

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CRUSTED SCABIES (NORWEGIAN SCABIES) • Very uncommon variant of scabies. • Though the first report of crusted scabies was in patients with leprosy, recent reports have described an increasing incidence of this form of scabies in patients with immunosuppression due to immunosuppressive agents, malignancy or acquired immunodeficiency syndrome (AIDS). • In this form of scabies, the host’s response to the mites is modified, allowing them to multiply. As a result of this, the mite population becomes enormous and may number millions. • The large adherent crusts are most often seen over the knees and elbows as well as the hands (Fig. 9.12) and feet. There may even be an erythroderma. • Frequently, it can be confused with psoriasis, chronic eczema (contact dermatitis), Langerhans cell histiocytosis, or neurodermatitis (Fig. 9.13). • Ivermectin seems to be ideal drug for this condition; otherwise prolonged therapy with topical scabicidal agents is required. Methotrexate is another option.

Fig. 9.12: Crusted scabies – hyperkeratotic scaly lesions over the palm and finger web space

Fig. 9.13: Crusted scabies—scaly cursted lesions over the side of the buttock

ANIMAL SCABIES • Contracted from pet animals-cats, dogs, or birds. • No burrows seen, only itching and papulovesicular lesions are seen over the site of contact-abdomen, thighs and arms. • Treatment of pet animal with scabicidal agent and antipruritic agents for controlling itching are required. • Human skin lesions are self limiting; resolve spontaneously if further contact with affected animal is stopped.

Fig. 9.14: Pediculus humanus capitis

Infestations

Fig. 9.15: Phthirus pubis

are oval, 1 mm long and firmly attached to the hair (Fig. 9.16) (or seems of clothing), hatch in about 7-9 days and become mature in another week. • Body louse is the vector for the following organisms: 1. Rickettsia prowazekii (Endemic typhus), 2. Bartonella quintana (Trench fever), 3. Borrelia recurrentis (Relapsing fever). • Extreme pruritus is the primary characteristic of pediculosis. • Incubation period—It takes 6 weeks (approximately 30 days) for the pruritus to develop in non-sensitized individual and only 24 to 48 hours with repeat exposures.

Pediculosis Capitis • Most common in children especially girls with long hairs. • Mode of spread- human to human contact or sharing combs, brushes and towels. • Scalp-favorite site is area behind the ear (Fig. 9.17) or occiput. • Nits may be found most easily on the hairs. Adult lice often impossible to find. • In patients with scalp pruritus, secondary pyoderma (with cervical lymphadenopathy) and dermatitis, one should always search the

Fig. 9.16: Nits attached to the hair

PEDICULOSIS (LICE INFESTATION) • Pediculus humanus capitis (2 to 4 mm long)head louse (Fig. 9.14). • Pediculus humanus corporis-body louse. • Phthirus pubis-pubic louse (Fig. 9.15). • Life cycle— these wingless six legged blood sucking insects are obligate parasites specific for humans, lays eggs. The ova (nits), which

Fig. 9.17: Pediculosis capitis – nits on the hair

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Fig. 9.18: Plica polonica—matting of the scalp hair

hairs behind the ears carefully for nits. The hairs may become matted from repeated scratching (plica polonica) (Fig. 9.18). • Differential diagnosis: Dandruff, here flakes come off easily from hair on manipulation. Hair casts look similar to nits but form an encompassing cylinder whereas nits are attached at an angle. Piedra is much less common and consists of clumps of fungi. • Permethrin 1% cream rinse (10 minute application) is the treatment of choice. Repeat application after one week is required to kill hatched out nits. Pyrethrins, lindane, malathion and benzyl benzoate are other agents, which can be used topically. Systemically, ivermectin, 200 µg/ kg body weight, two doses at 7 days interval may be used. Co-trimoxazole 1 tablet twice a day for 3 days, following that second course 7-10 day later. • For nit removal: After treatment, hairs are soaked with vinegar and water (50:50) and then combed with fine toothed nit comb.

• Itching is the main symptom , which is due to sensitivity to salivary antigens. • Patients harbouring body lice for long time develop hyperpigmentation of skin, develop numerous excoriations (frequently most noticeable on the side of trunk and back), secondary infection and even lymphadenopathy-vagabond’s skin, vagabond’s itch, or vagabond disease (morbus errorum). • For diagnosis: Closely examine the clothing for lice and nits along the seams. • Differential diagnosis: All pruritic dermatoses especially scabies, contact dermatitis, atopic dermatitis. • Therapy: Clothes must be disinfected. Topical antipruritics or corticosteroids are given to the patient. A course of antibiotic may be needed to take care of secondary infection.

Pediculosis Pubis • Transmission in adults occurs mostly during sexual intercourse. • The pubic or crab louse is smaller than the head or body louse, has resemblance to a crab (for prominent claws in second and third pairs of legs). • Pruritus or crawling sensation is modest in the pubic area.

Pediculosis Corporis • Infestation associated with poor personal hygiene, a disease of homeless. • The body louse feeds on body and lays its eggs on seems of clothing.

Fig. 9.19: Phthriasis pubis – adult louse clung to the hair

Infestations of insects such as mosquitoes, fleas, and bedbugs. • This condition is mainly seen in childhood. • Treatment involves use of insect repellants, topical antipruritic agents and antibacterial with or without steroids.

LARVA MIGRANS ERUPTION

Fig. 9.20: Phthriasis palpebrarum – nits attached to eyelashes

• Adult lice may be difficult to find and are usually located at the base of hairs resembling small freckles, scabs or moles (Fig. 9.19). • Other than pubic hair, body and axillary hair as well as eyelashes (phthiriasis palpebrarum) (Fig. 9.20) and beard should be examined for nits. • A classical clinical finding is the maculae cerulea—flat, indistinct, blue grey or slate colored macules in the infested area. • All the treatments suggested for pediculosis capitis are effective. Shaving the area is another option. Infestation of the eyelids is treated with twice daily applications of petrolatum for 7 to 10 days. Alternative treatments include anticholinesterase (physostigmine) eye ointments, yellow oxide of mercury, or fluorescein. The simplest technique for the treatment of eyelid lice is direct removal of the lice and nits with a fine forceps. Cryotherapy may provide a fast cure. Application of 1% gamma benzene hexachloride cream and pyrethrin ointment are other options.

• Larva migrans eruption is usually caused by filariform larvae of the dog and cat hookworms, mainly Ancylostoma braziliensis and rarely A. caninum, Uncinaria stenochepala, Bunostomum phlebotomun, or the human larvae of Necator americanus and Ancylostoma duodenale. • Adult hookworms live in the intestines of dogs and cats and their ova are deposited in the animals’ feces. Under favorable conditions of humidity and temperature, the ova hatch into infective larvae, which will penetrate human skin. Sandy, warm, moist,

PAPULAR URTICARIA • Papular urticaria occurs as episodic, symmetrically distributed, pruritic, urticarial papules that are caused by bites

Fig. 9.21: Larva migrans eruption – serpentine erythematous tract of larva migrans over the thigh of a child

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shaded areas are particularly favorable and humans acquire numerous infections. It is commonly known as creeping eruption for its distinctive feature that the lesion creep or migrate and is due to the presence of moving parasite in the skin. An irregularly linear, thin, raised burrow, 2-3 mm wide, manifests this exceedingly itchy eruption (Fig. 9.21). The larva moves a few mm to a few cm per day. The eruption is self-limited because humans are abnormal hosts. The feet and buttocks are the areas most commonly involved. Larva migrans can be accompanied by Loeffler’s eosinophilia, particularly in severe infestations. The classic clinical picture of wandering, serpentine and itchy lesion is easily recognized. Biopsy in larva migrans is of little value as the larvae have advanced beyond the clinical lesions. Differential diagnosis is granuloma annulare, migratory myiasis and larva currens. Larva currens caused by filariform larvae of Strongyloides stercoralis is characterized by wheal and flare response along the path of larvae, especially around anus and buttocks. The linear or serpiginous tracks of urticarial papules move at the rate of several cm/ hour. Demonstration of larvae of Strongyloides spp. in faeces confirms the diagnosis. The treatment of choice is the topical application of 10% thiabendazole (not available in India). Even the oral preparation of thiabendazole or two tablets of 500 mg thiabendazole crushed and formulated in 10 g petrolatum may be applied topically twice daily. Systemic use of thiabendazole, though effective, is contraindicated due to its possible poorly tolerated side effects. Ivermectin, in a single dose of 200 microgram

Fig. 9.22: Myiasis—maggots in chromomycosis lesion on the hand

per kg body weight seems best. Albendazole 400 mg daily by mouth for 3 days is safe and often effective. Flubendazole is currently at an experimental stage, appears to offer good prospects.

MYIASIS • Myiasis is the presence of fly larvae (maggots) in the tissue. • Usually they need an entry site, such as a chronic ulcer (trophic ulcer of leprosy) or wound. • Most maggots only eat necrotic debris, although some attack healthy tissue. • Clinically, myiasis is classified according to the part of the body affected-cutaneous myiasis (Fig. 9.22) (wound, furuncular), nasopharyngeal myiasis, ophthalmomyiasis, intestinal and urogenital myiasis. • Therapy-remove the maggots either after anesthetizing the area or suffocating them with turpentine oil or petrolatum.

LEISHMANIASIS Leishmaniasis is a parasitic infection caused by many species of the protozoa Leishmania, manifested clinically as four syndromes.

Infestations

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Fig. 9.23: Cutaneous leishmaniasis— noduloulcerative lesion over the forearm

• Old world type—Cutaneous leishmaniasis. • New world type—Mucocutaneous leishmaniasis. • Diffuse Cutaneous Leishmaniasis • Visceral leishmaniasis—Kala-azar. • Transmitted by infected female sand fly— Old world leishmaniasis by Phlebotomus spp. and New world leishmaniasis by Lutzomyia, Psychodopygus spp. • Life cycle: The parasite exists in two forms: a. Amastigote form in vertebrate host in cells of reticuloendothelial system or dermis. It has no flagellum. b. Promastigote form in gut of female sand fly and in culture media. It is motile with an anterior flagellum. • Oriental sore (Delhi boil, Baghdad boil) is an old world cutaneous leishmaniasis caused by Leishmania major, seen in Rajasthan and adjoining areas in India. • Unclothed parts of the body such as face, neck, and forearms are usually involved. It

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begins as papule that enlarges, ulcerates and becomes crusted nodule with surrounding induration (Fig. 9.23). Satellite lesions may occur around the main lesion. Most of the lesions heal by 6 months. Post kala-azar dermal leishmaniasis (PKDL) occurs in 20% of the patients treated for visceral leishmaniasis (Kala-azar) caused by Leishmania donovani. It is mainly confined to West Bengal, Orissa, Jharkhand and Bihar in India. The lesions appear after a year of therapy and manifest as hypopigmented macules, papules and nodules over the trunk and face. They closely resemble lepromatous leprosy, secondary syphilis and yaws. Espundia is a type of mucocutaneous leishmaniasis caused by Leishmania braziliensis. Chiclero ulcers are caused by Leishmania mexicana. Diagnosis is by demonstration of amastigotes inside macrophages from the lesion on a slit skin smear and culture in NNN media. Treatment of cutaneous leishmaniasis. A. Local therapy—Heating the sore (40-42°C), cryotherapy, B. Local infiltration of 1-2 ml of sodium stibogluconate or meglumine antimoniate, C. Systemic therapy: Antimonials—Sodium stibogluconate or meglumine antimoniate 20 mg/kg/day for 14-21 days. Pentamidine isethionate- 4 mg salt / kg – weekly doses may be used. D. Other drugs: Amphotericin B, ketoconazole, itraconazole, dapsone, rifampicin, etc.

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Papulosquamous Disorders

Papulosquamous disorders are characterized by raised, scaly papules and plaques, include psoriasis, lichen planus, pityriasis rosea, lichen nitidus, pityriasis rubra pilaris and other conditions. These are discussed below.

PSORIASIS • Psoriasis is a common chronic disfiguring, inflammatory and proliferative epidermal skin disorder mediated by T cells that affects approximately 1 to 3% of the world population. • Multifactorial inheritance most likely, a family history of psoriasis is found in 30% of patients. • The histocompatibility antigen HLA Cw6 is most strongly associated with psoriasis and the coexistence of HLA B17 or B27 portends more severe skin disease or associated psoriatic arthritis. • The pathogenesis of psoriasis remains unclear. The initial reaction is possibly an intrinsic defect of keratinocytes with increased cytokine production which leads to expansion of CD45RO + T cells with resultant production of type 1 cytokines. Subsequently, it results in epidermal proliferation, migration of neutrophils into

the epidermis, and proliferation of vessels in the papillary dermis. • The time necessary for psoriatic epidermal cell to travel from the basal cell layer to the surface and be cast off is 3 to 4 days, in marked contrast to the normal 26 to 28 days. • Precipitating factors— Stress and anxiety, alcohol and smoking, drugs (chloroquine, lithium, betablockers, ACE inhibitors, NSAID’s, etc), sudden withdrawal of systemic steroids, irritants, infections.

Classification of Psoriasis 1. Based on Morphological Types • Chronic stable plaque psoriasis (psoriasis vulgaris) (Figs 10.1 and 10.2) • Guttate psoriasis • Pustular psoriasis • Erythrodermic psoriasis • Rupioid, elephantine and ostraceous psoriasis. 2. • • • • • •

Based on Site of Involvement Scalp psoriasis Flexural (inverse) psoriasis (Fig. 10.3) Nail psoriasis Palmoplantar psoriasis Genital psoriasis Psoriatic arthritis.

Papulosquamous Disorders 3. • • • • • • • • • •

Atypical Forms of Psoriasis Linear and zonal lesions Follicular Photosensitive Seborrheic psoriasis Annular psoriasis Circinate psoriasis Nummular psoriasis Serpiginous psoriasis Geographic psoriasis Mucosal lesions— annular plaques, diffuse areas of erythema, geographic tongue; very rare • Ocular lesions— blepharitis, conjunctivitis, keratitis, xerosis, symblepharon, trichiasis, uveitis; extremely rare.

size, sharply circumscribed, dry, and usually covered with layers of silvery white, micaceous scales (Figs 10.1 and 10.2). • Grattage test involves scrapping a scaly lesion to look for type of scales. Silvery white

Clinical Features • Most lesions of psoriasis are asymptomatic. • Its typical age of onset is in the third decade, though it may develop at any time from birth onward. • Erythematous papules and plaques characterize it. The lesions are of variable

Fig. 10.2: Psoriasis vulgaris–plaque lesions over the trunk

Fig. 10.1: Psoriasis vulgaris–plaque lesions over the extensor surface of limbs and trunk

Fig. 10.3: Flexural psoriasis–psoriatic plaque involving axilla

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micaceous scales are found on doing grattage test in psoriasis. Auspitz’s sign is typical of psoriasis. It consists of three components: 1. Silvery white micaceous scales on scrapping (due to parakeratosis) 2. Shiny membrane called Bulkley’s membrane on continued scrapping (due to suprapapillary thinning of epidermis) 3. Bleeding points on removal of membrane (due to dilated capillaries in papillary dermis). Extensor surfaces of the extremities (Fig. 10.1), the scalp (Fig. 10.4) and lumbosacral region are commonly involved. Lesions of active psoriasis often appear in areas of epidermal injury-Koebner phenomenon. An acute variant, guttate or eruptive psoriasis is often seen in younger patients and is characterized by an abrupt eruption of small drop shaped lesions. It is associated with acute group A beta hemolytic streptococcal infection of pharynx in the preceding 7 to 10 days.

Fig. 10.4: Psoriasis vulgaris–lesions extending beyond hair line

• Nail involvement is common (50% of psoriasis patients). The most frequent alteration of nail plate surface is the presence of pits (Fig. 10.5). Nail matrix changes in psoriasis are pitting, longitudinal ridging, grooves, leukonychia, erythema of lunula (mottled erythema on lunula due to matrix inflammation), thickening of nails, crumbling of nail plate and trachynonychia. Nail bed changes include subungual hyperkeratosis, distal onycholysis, salmon (oily) patches, splinter hemorrhages (thin red or black longitudinal lines in distal portion of nail due to psoriatic involvement of nail bed capillaries, which are longitudinally oriented). Paronychia results from involvement of periungual skin of proximal nail fold with retention of scales between ventral nail fold and nail plate. • Psoriatic arthritis is of various types— Distal interphalangeal joint arthritis, asymmetrical oligoarthritis, polyarthritis (rheumatoid arthritis like), arthritis mutilans and predominantly axial arthritis (psoriatic spondylitis and/ or sacroiliitis). Psoriatic

Fig. 10.5: Psoriasis vulgaris–nail pitting

Papulosquamous Disorders arthritis characteristically involves the terminal interphalangeal joints (Fig. 10.6), but frequently the large joints are also affected, resembling rheumatoid arthritis. However, the rheumatoid factor is absent. • In severe cases, the disease may affect the entire skin and presents as psoriatic erythroderma. • Psoriasis rarely presents as generalized pustular psoriasis (von-Zumbusch typeacute exanthematic type (Fig. 10.7) or generalized pustular psoriasis of pregnancyImpetigo herpetiformis). Localized pustular psoriasis occurs as acrodermatitis continua of Hallopeau (localized to the distal portions of the hands and feet) (Fig. 10.8) or pustulosis palmaris et plantaris (chronic, relapsing disorder occurring on the palms, soles, or both).

Diagnosis • Psoriasis may be confused with seborrheic dermatitis, secondary syphilis, dermatophyte infections, cutaneous lupus erythematosus,

Fig. 10.6: Psoriatic arthritis—characteristically involving distal interphalangeal joint leading to swan neck deformity

eczema, lichen planus, pityriasis rosea, Bowen’s disease or lichen simplex chronicus. • Diagnosis is mostly clinical supplemented at times of doubt by histopathology of skin lesion.

Fig. 10.7: Generalized pustular psoriasisgeneralized pustular eruption in a child

Fig. 10.8: Acrodermatitis continua—nail dystrophy and finger tip involved by pustular psoriasis

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Essentials in Dermatology • Histopathology: The fully developed lesion of psoriasis shows: 1. Hyperkeratosis and parakeratosis. 2. Within parakeratotic areas of the horny layer, accumulations of neutrophils forms, which are called as Munro microabscesses. 3. Hypo or absent granular layer. 4. Regular acanthosis. 5. Spongiform pustule of Kogoj— neutrophils accumulation in Malpighian layer 6. There is regular elongation of the rete ridges with thickening in their lower portion, looking like elephant feet. 7. There is thinning of the supra papillary portion of the epidermis. 8. The dermal papillae contain enlarged and tortuous capillaries that are very close to the skin surface and impart a characteristic erythematous hue to the lesions. 9. Sparse lymphocytic infiltrate in the upper dermis.

Differential Diagnosis of Psoriasis • Seborrhoeic dermatitis— In the scalp, lesions are lighter in color, less well defined, covered with dull or branny or greasy scales, with absence of corona. • Pityriasis rosea— Papular or annular eruption usually confined to upper arms, trunk and thighs. Herald patch is followed by disseminated eruption, which has christmas tree pattern. Typically individual lesion shows collarette of scaling. Duration of disease is few weeks (6-8 weeks), and it has self limiting course. • Pityriasis rubra pilaris resembles closely the psoriasis especially in the erythrodermic phase. The follicular accentuation, focal areas of sparing (islands of normal skin), sometimes more salmon coloration of pityriasis rubra pilaris and the acquired

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palmoplantar keratoderma with yellow orange tinge help to differentiate the condition clinically; biopsy sections of pityriasis rubra pilaris may show follicular plugging, parakeratotic shoulder and perifollicular lymphohistiocytic infiltrate. Dermatophyte infection (tinea corporis)— Itchy annular erythematous plaque/s with peripheral scaling, KOH examination demonstrates dermatophytes. Discoid lupus erythematosus— Discrete erythmatous plaques on face and scalp (usually on sun-exposed areas), associated with atrophy, scaling and alopecia. Scales of discoid lesions are grayish and adherent. There is follicular plugging and carpet-tack sign. Subacute lupus erythematous— Presents with annular erythematous or psoriasiform lesions associated with systemic component, may have other components of systemic lupus erythematosus. Eczema at times develops a psoriasiform appearance especially on the legs. Hyperkeratotic eczema of palms is a common cause of misdiagnosis. Color, scratch evoked scaling and well defined margins are suggestive of psoriasis and nail changes may be diagnostic. Lichen planus— It involves flexor surface of forearm and wrists, and shins and ankles. Pruritic purple flat topped papules and plaques with scanty and tightly adherent scales. Scalp is much less frequently involved. Nails are longitudinally ridged, thickened or thinned out, with pterygium a characteristic finding. No nail pitting. Linear lichen planus may mimick linear psoriasis but the individual lesion characterstics helps in differentiating. Secondary syphilis— Asymptomatic eruption, brownish sparse scales, generalized lymphadenopathy, mucous patches, condyloma lata, positive serological test for syphilis.

Papulosquamous Disorders • Drug eruptions— Always take a drug history; many drug reactions are psoriasiform, and in some instances lead to true psoriasis in susceptible host. • Pityriasis lichenoides chronica— It can closely resemble guttate psoriasis but the lesions are less evenly scattered, have brownish red or orange brown color and are capped by an opaque soft mica like scale.

Treatment • It is important to emphasize that psoriasis is a treatable condition. The treatment pyramid for psoriasis is shown in Fig. 10.9. • Despite virtual explosion in therapeutic options, the management of psoriasis remains a challenge to clinicians.

Topical Therapies • Emollients— Such as coconut oil, vaseline, or liquid paraffin. • Coal tar (2, 5, 10% ointment)— It may be used effectively as monotherapy or in combination with other treatment modalities. Goeckerman therapy consists of black crude coal tar application all over the patient’s body. This tar is left on for 24 hours per day. UVB phototherapy is administered before the tar is applied or after it is washed off. The Goeckerman regimen remains an intensive daily therapy that is usually conducted for a period of several weeks. • Anthralin (0.1 to 10% cream or ointment)— It is moderately effective and quite safe in plaque psoriasis. The major side effect is staining of skin and clothing and occasional irritation. Ingram regimen consists of a combination of anthralin and UVB phototherapy. Short contact treatment of 30 minutes or less is likely to reduce irritation. • Topical steroids/ intralesional steroidsTopical corticosteroids are used for treatment over face and neck, flexures and genitalia, where tar or anthralin would cause irritation and cannot be used. Systemic steroids

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generally not recommended due to the wellknown risk of rebound and pustular conversion. Vitamin D analogues are both naturally occurring – calcitriol (1α, 25-dihydroxyvitamin D 3 ) and synthetic analoguescalcipotriol, tacalcitol, maxacalcitol. Vitamin D molecule is dispensed as ointment, cream or solution and has risk of producing hypercalcemia in less than 1% of cases. Tazarotene (0.1 to 0.05% gel)— It is a modified vitamin A molecule formulated as a topical agent. It is recommended for use in patients with total body surface involvement of 20% or less. Topical methotrexate gel-used for palmoplantar psoriasis with limited efficacy. Tacrolimus (0.03, 0.1%) effective for facial plaques and inverse psoriasis.

Medications Used in Phototherapy • Concurrent use of topical or oral medications, psoralen (5 methoxy psoralen, 8 methoxy psoralen, trimethyl psoralen) along with UVA or UVB phototherapy is done. • Psoralen along with UVA from sunlight (PUVASOL). • Narrow band UVB. • Methotrexate and PUVA combination (Me-PUVA). • Retinoids and PUVA combination (RePUVA). • Excimer LASER (308 nm). Systemic Medications for Psoriasis • Methotrexate (0.4 to 0.6 mg per kg body weight)— Used for severe cases of psoriasis, taken orally just once a week. It is an effective and well tolerated drug in appropriately selected patients who are receiving folate supplementation, and who are adequately monitored for potential toxicities. • Cyclosporine (3-5 mg per kg body weight). Cyclosporine has dose-related nephrotoxicity and hypertension that impede its use as a

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long-term agent for most patients. Nevertheless, cyclosporine is an effective drug for rapid clearing in most patients. Cyclosporine, when used in the appropriate patient at the appropriate dose (usually 2.5 to 5.5 mg/kg daily), is an excellent “bridging agent” that can be used safely for periods of 2–12 months. Etretinate and Acitretin (0.5-1 mg per kg body weight)— They are the treatment of choice for generalized pustular psoriasis. Acitretin is less effective when used as monotherapy for plaque psoriasis. Hydroxyurea (maximum up to 500 mg three times a day). Sulfasalazine (3-4 gm/day). Mycophenolate mofetil ( 1-2 gm/day, can increase maximum up to 4 gm/day). Oral tacrolimus ( 0.05- 0.15 mg/kg body weight per day). Fumaric acid esters 30 mg per day up to 240 mg three times a day. Many of these medications are used in combination and rotated periodically. HIV associated psoriasis may respond to zidovudine based HAART regimen.

Biologic Agents Biologics are agents that selectively block the immunologic steps in the pathogenesis of psoriasis. Alefacept, efalizumab, etanercept, and infliximab are currently approved for the treatment of adults with moderate to severe plaque psoriasis, and phase 3 trials for adalimumab are ongoing (Table 10.1). • All biologics approved to date for the treatment of psoriasis are recommended for patients who are candidates for systemic or photo-therapy. Table 10.1: Biologic agents and their mechanism of action

Strategy

Biologic agents

Reduction of the number of pathogenic T cells

Alefacept, denileukin diftitox

Inhibition of T-cell activation and migration

Efalizumab, daclizumab, siplizumab, CTLA4Ig, galiximab,

Modulation of the immune system

Ilodecakin, oprelvekin

Blockage of the activity of inflammatory cytokines

Etanercept, infliximab, adalimumab, ABX-IL8

Fig. 10.9: Treatment pyramid of psoriasis

Papulosquamous Disorders • Efalizumab is favorable in patients with high risk of latent tuberculosis or evidence of demyelinating disease. • Infliximab is advantageous where rapid disease control is required (e.g. unstable erythroderma). • Etanercept is the biologic of choice in stable psoriasis where a TNF-α-blocking strategy is appropriate. • Some biologics are also approved for treatment of psoriatic arthritis; thus, patients with psoriatic arthritis may benefit from a biologic for both the articular and dermatologic manifestations.

• Cutaneous lesions occur in about half of affected patients. The cutaneous eruption has limpet like scales (Figs 10.10 and 10.11). Lesions have a predilection for the glans penis (balanitis circinata) (Fig. 10.12), the palms and soles (keratoderma blennorrhagica) and the subungual areas. • HLA B 27 is present in about 80% of cases • Most cases of Reiter’s disease are probably caused by infection with micro-organisms that infect urogenital or gastrointestinal systems such as Chlamydia trachomatis,

Course and Prognosis • The course of psoriasis is prolonged but unpredictable. REITER’S DISEASE • Reiter’s disease predominantly affects young men and consists of the triad of urethritis, arthritis, and conjunctivitis. As few patients present with classic triad, the American College of Rheumatology recognizes criteria for limited manifestations of this syndrome including peripheral arthritis of more than one month duration in association with urethritis, cervicitis or bilateral conjunctivitis.

Fig. 10.10: Reiter’s disease–typical limpet like scales

Fig. 10.11: Reiter’s disease–typical lesions over legs

Fig. 10.12: Reiter’s disease-circinate balanitis

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Essentials in Dermatology Ureaplasma urealyticum, Shigella, Salmonella, etc. • Differential diagnosis includes rheumatoid arthritis, ankylosing spondylitis, gout, psoriatic arthritis, gonococcal arthritis, acute rheumatic fever, etc. • Mucocutaneous lesions are generally self limited and clear with topical steroids. Refractory lesions are managed like psoriasis. Joint disease may require NSAIDS, methotrexate or biologics (infliximab).

LICHEN PLANUS • Lichen planus (Greek leichen, “tree moss”; Latin planus, “flat”) is a subacute or a chronic dermatosis that may involve skin, mucous membranes, hair follicles and nails. • The cause of lichen planus is unknown, but several etiologies have been proposed. It is likely that both endogenous-genetic and exogenous-environmental components such as drugs or infection may interact to elicit the disease. • The prevalence of chronic liver diseases, including primary biliary cirrhosis, alcoholic cirrhosis, hepatitis B, and especially hepatitis C, is increased. • HLA-B8 is more common in patients with oral lichen planus as the sole manifestation,

Fig. 10.13: Lichen planus–violaceous, flat-topped papules

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and HLA-Bw 35 is more strongly associated with cutaneous lichen planus. At least two-thirds of cases occur between the ages of 30 and 60 years. Cutaneous eruption is characterized by small, flat topped, shiny, polygonal, violaceous papules that may coalesce into plaques (Fig. 10.13) The papules often show a network of white lines known as Wickham’s striae. Koebner phenomenon commonly seen (Fig. 10.14). Itching is usually pronounced. The four P’s – purple, polygonal, pruritic, papule- is the abbreviation used to recall the constellation of symptoms and skin findings that characterize lichen planus. The disease has a predilection for the flexor surfaces of the forearms (Fig. 10.15), legs (Fig. 10.16), trunk (Fig. 10.17), and the genitalia including the glans penis (Figs 10.18 to 10.20).

Fig. 10.14: Lichen planus–wrist involved, Koebner phenomenon also seen

Papulosquamous Disorders • The oral lesions of lichen planus are frequently found, either as sole manifestation of the disease or associated with cutaneous involvement. Most often consist of a lacy, reticular network of coalescent papules over the buccal (Fig. 10.21) or glossal mucosa. Besides this, it forms plaque like, atrophic, papular, erosive and bullous lesions. • The nails are involved in about 10% of cases and show roughening, longitudinal ridging, thinning and dystrophy. Pterygium

Fig. 10.17: Lichen planus–trunk involved by typical violaceous papular lesions

Fig. 10.15: Lichen planus–flexor aspect of arm involved

Fig. 10.18: Lichen planus–an annular lesion over the glans penis

Fig. 10.16: Lichen planus–hypertrophic violaceous papules and plaques over legs

Fig. 10.19: Lichen planus–male genitalia involved by lichen planus

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Fig. 10.20: Lichen planus–female genitalia involved by lichen planus

Fig. 10.21: Lichen planus–lacy white network over the buccal mucosa

Fig. 10.22: Lichen planus–pterygium unguium formation in some finger nails

Fig. 10.23: Lichen planus–transeversely aligned lichen planus along Blaschko’s lines

formation is a frequent finding (Fig. 10.22). • Common variants of lichen planus (LP) are classified on the basis of: A. Configuration (annular, linear, zosteriform/along Blaschko’s lines (Fig. 10.23), B. Morphology of lesions (hypertrophic, atrophic, vesicobullous, erosive, ulcerative, follicular [planopilaris], actinic (Fig. 10.24), LP pigmentosus, perforating, guttate), C. Site of involvement (palms and soles, mucous membranes, nails, scalp),

D. Special forms (drug induced, overlap syndrome of lichen planus/lupus erythematosus, LP pemphigoides).

Diagnosis • The appearance of the typical papule of lichen planus is usually sufficient to make the correct diagnosis. • Histopathology: Microscopic features, similar to the gross morphology, are diagnostic. The two major pathologic findings in lichen planus are basal epidermal keratinocyte damage and lichenoid-interface lympho-

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• • Fig. 10.24: Lichen planus actinicus–typical hypopigmented halo around pigmented violaceous lesions over the face

cytic reaction. The histologic features are summarized here: 1. Hyperkeratosis 2. Wedge shaped hypergranuloses which is responsible for the Wickham’s striae seen clinically 3. Irregular acanthosis 4. Damage to basal cell layer 5. The rete ridges are pointed at their lower end and the papillae between rete ridges are often dome shaped thus resembling the old styled bridges on rivers having tapering down pillars and domes in between. This pattern is also called as “saw-tooth” pattern. 6. A band-like dermal lymphocytic infiltrate closely hugs the epidermis. 7. Max-Joseph spaces are seen in some cases as a sub-epidermal clear zone. Melanin incontinence and vascular inflammatory reaction probably give purple colour to LP lesions.

Differential Diagnosis • Classic lesions: Lichenoid drug eruption (large scaly lesions in sun-exposed areas, devoid of Wickham striae, residual pigmentation common), lichen nitidus

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(asymptomatic pinhead sized shiny papular lesions, rarely involves mucous membranes), secondary syphilis, pityriasis lichenoides et varioliformis acuta, early pityriasis rubra pilaris. Hyperkeratotic lesions: Lichen simplex chronicus, prurigo nodularis, lichen amyloidosis, warts. Linear lesions: Lichen striatus, linear epidermal naevus, linear psoriasis. Annular lesions: Granuloma annulare, secondary syphilis, psoriasis Lichen planopilaris: For early lesionskeratosis pilaris, other follicular keratoses, Darier’s disease, early pityriasis rubra pilaris. For advanced lesions-discoid lupus erythematosus, and other forms of scarring alopecia. Wide-spread erosive oral lesions must be differentiated from those of candidiasis (curdy white deposits which on removal leaves an erythematous base. KOH scrapping shows budding yeasts, pseudohyphae), aphthous ulcers (well circumscribed shallow ulcers with a necrotic gray centre and an erythematous halo. It usually heals in around six weeks) pemphigus (ill defined irregular buccal or palatal non healing painful erosions. Other mucosal sites may also be involved. Nikolsky’s sign positive), cicatricial pemphigoid (vesicles, persistent extensive erosions, desquamative gingivitis with eroded bleeding gums, adhesions between buccal mucosa and alveolar process and around uvula and tonsillar fossae, involvement of other mucosal sites including conjunctiva, genital mucosa, larynx and esophagus, and decreased mouth opening due to fibrosis), carcinoma (usually malignant ulcers are painless unless secondarily infected. On palpation induration of base may be present), and erythema multiforme (usually involves lips,

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Essentials in Dermatology palate and gingival. On lips, target lesions may be identified. Vesicles, erosions and crusting are present over oral mucosa. Associated skin lesions- target lesions may be seen over acral extensor sun-exposed areas).

Treatment • Topical therapy: Topical glucocorticoids are typically used for mucosal and limited cutaneous disease. • Intralesional therapy: Intralesional triamcinolone acetonide (5 to 10 mg/mL) is effective in treating oral and cutaneous lichen planus. • Systemic therapy: 1. Systemic glucocorticoids are often useful and effective in doses greater than 20 mg/day (e.g. 30 to 60 mg prednisolone) for 4 to 6 weeks with subsequent taper over 4 to 6 weeks. 2. PUVA photochemotherapy is usually successful in generalized cutaneous lichen planus. 3. The systemic retinoids demonstrate antiinflammatory activity and have been used in the treatment of lichen planus. 4. Cyclosporine (500 mg) rinses in oral lichen and systemic cyclosporine in recalcitrant lichen planus has been used successfully. 5. Other agents used in lichen planus are dapsone, griseofulvin, cyclophosphamide, methotrexate, phenytoin and extracorporeal photochemotherapy. In mucosal lichen planus, topical corticosteroids, tetracycline, betamethasone mouthwashes 0.5 mg 3-4 times daily, topical tretinoin gel, cyclosporine mouth rinses, tacrolimus, and pimecrolimus have been used. Maintenance of good oral hygiene and replacement of amalgam or gold dental restorations with composite material is frequently of considerable benefit. Erosive oral lichen planus may respond to oral dapsone.

Course Lichen planus is a benign disease with spontaneous remissions and exacerbations. Apart from hypertrophic lichen planus, most of LP lesions tend to involute after several months to a year. LICHEN NITIDUS • Lichen nitidus is a chronic dermatitis usually asymptomatic which begins commonly in childhood or early adulthood • It is characterized by minute, round, flat or dome shaped, shiny, flesh colored papules 2 to 3 mm in diameter that may occur in groups • Predominantly, the arms, trunk (Fig. 10.25) or penis (Fig. 10.26)are involved • Koebner phenomenon may be observed (Fig. 10.27) • The histology of a typical papule is characteristic. A circumscribed epithelioid cell granuloma is situated immediately below the epidermis. The rete ridges at the margin of the infiltrate are elongated and tend to encircle it “claw clutching a ball appearance”. • Differential diagnosis: 1. Keratoses pilaris (horny follicular papules on extensor surfaces),

Fig. 10.25: Lichen nitidus–flat, shiny, flesh colored papules on the trunk

Papulosquamous Disorders 2. Lichen scrofulosorum (grouped follicular papules in small patches on trunk), 3. Lichen planus, 4. Sarcoidosis, 5. Disseminated granuloma annulare, 6. Eruptive xanthomas,

7. Plane warts (more variable in size, have verrucous surface and present with fewer lesions with few anatomic sites involved) • As the disease is often asymptomatic and eventually self-limiting, no treatment is required in most cases • Topical steroids may be recommended if treatment is demanded.

PITYRIASIS ROSEA

Fig. 10.26: Lichen nitidus–flat, shiny, flesh colored papules on the glans penis

Fig. 10.27: Lichen nitidus- over the arm showing Koebner phenomenon

• Pityriasis rosea is a self limited dermatitis lasting from 4 to 7 weeks. • The etiology is unknown, though a viral origin has been suggested. • Predominantly occurs in adolescents and young adults. • May be asymptomatic or pruritic (50% of cases). • It frequently starts with herald patch (mother patch, plaque primitive, primary medallion, primary plaque) (most commonly on the trunk) followed by a disseminated eruption (the trunk and proximal extremities) within several days to 2 weeks (Fig. 10.28). Sometimes, herald patch may be missing, or present as double or multiple lesions often close together.

Fig. 10.28 Pityriasis rosea–herald patch with a few daughter lesions showing “collarette of scaling”

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Essentials in Dermatology • Round to oval salmon colored patches following the lines of cleavage (Christmas tree like pattern) and showing peripheral attached thin cigarette paper like scales (“collarette of scaling”) (Fig. 10.28). When individual lesion is stretched along the long axis, the scales tend to fold across the lines of stretch, so called “hanging curtain sign”. • Variants of pityriasis rosea are vesicular, inverse (Fig. 10.29), cervicocephalic, pityriasis rosea gigantea (pityriasis circinata et marginata of Vidal), pityriasis rosea urticata, purpuric pityriasis rosea, pityriasis rosea perstans, pustular pityriasis rosea, and eczematous pityriasis rosea.

white micaceous scales), parapsoriasis, pityriasis lichenoides chronica, lichen planus, and, most importantly, secondary syphilis (maculopapular lesions, no herald patch, genital and oral mucosal involvement).

Treatment • Most patients require no treatment. • Topical corticosteroids or short course of oral corticosteroid may be required in severe cases. • Oral erythromycin may be helpful. • Itching alleviated with antipruritic lotions or antihistamines. PITYRIASIS RUBRA PILARIS (PRP)

Diagnosis • Usually easily diagnosed by its morphology and distribution. Differential Diagnosis • Pityriasis rosea must be differentiated from tinea corporis, tinea versicolor (well defined hypopigmented coalescing macules with branny scales), drug eruptions (acute onset without herald patch, pruritic protracted rash and a tendency for lesions to become lichenoid), psoriasis (papules and plaques with silvery

• Pityriasis rubra pilaris is an erythematous squamous disorder characterized by seborrheic dermatitis like rash which progresses cephalo-caudally, follicular plugging and perifollicular erythema that coalesces to form orange red scaly plaques (Fig. 10.30) that frequently contain islands of normal appearing skin (Fig. 10.31), proximal phalanges showing typical follicular plugging and palms and soles keratotic sandal (palmoplantar keratoderma) (Fig. 10.32).

Fig. 10.29: Inverse pityriasis rosea – lesions over the face

Fig. 10.30: Pityriasis rubra pilaris – follicular papular lesions coalescing to form plaques over the trunk

Papulosquamous Disorders • • • •

Fig. 10.31: Pityriasis rubra piliaris–islands of normal skin in a patient having erythroderma

Fig. 10.32: Pityriasis rubra pilaris–palmoplantar keratoderma (keratotic sandal)

• It may progresses to erythroderma. • Both familial and acquired forms of the disorder have been described.

Types of PRP • Type I—adult onset classical, most common type. • Type II—adult onset atypical. • Type III—juvenile onset, classical.

Type IV—juvenile onset, circumscribed. Type V— juvenile onset, atypical. Type VI—HIV associated. Histopathology reveals hyperkeratosis and follicular plugging with shoulder of parakeratosis. Parakeratosis may alternate both vertically and horizontally producing a checkerboard pattern. • Differential diagnosis: Closely resembles psoriasis but prominent facial involvement or follicular lesions speak against it. Early scalp involvement is often mistaken for atopic dermatitis or seborrhoeic dermatitis. Early diffuse lesions are confused with lichen planus and pityriasis lichenoides et varioliformis acuta. Circumscribed pityriasis rubra pilaris mimics phrynoderma or keratosis pilaris. • Treatment: Topical therapy with keratolytic agents, vitamin D analogues and systemic therapy with vitamin A, acitretin, isotretinoin (systemic retinoid), methotrexate and retinoid plus psoralen and ultraviolet A (RePUVA) has been tried with variable efficacy. Antiretroviral therapy is of value in pityriasis rubra pilaris like eruption of HIV infection. • The inherited form of the disorder is persistent throughout life while the acquired disease usually shows remissions and exacerbations.

ERYTHRODERMA (EXFOLIATIVE DERMATITIS) • Erythroderma is a condition which is characterized by erythema, infiltration and scaling involving more than 90% of the body surface area or near universal involvement of the body (Fig. 10.33). • Erythroderma can be caused by various dermatological disorders: eczema, psoriasis, drugs, lymphoma, leukaemia, pemphigus foliaceus, ichthyosiform erythroderma, pityriasis rubra pilaris, lichen planus,

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Essentials in Dermatology • In erythroderma where the etiology is unknown, called as idiopathic erythroderma (10% of cases). • Chronic erythroderma of unknown origin with prolonged course in the elderly is called as ‘red man syndrome’. • Sézary syndrome manifest with erythroderma, generalized lymphadenopathy and atypical cells in peripheral smear (more than 10%). • It can be a manifestation of internal malignancy.

Fig. 10.33: Erythroderma – near universal involvement by erythema, scaling and infiltration

dermatomyositis, dermatophytosis and crusted scabies. • The drugs causing erythroderma are phenytoin, carbamazepine, cimetidine, lithium, gold, chloroquine, isoniazid, mercury, thiazide, quinidine and pyrazolone derivatives.

Complications • Erythroderma leads to hemodynamic and metabolic disturbances such as high out put cardiac failure, hypothermia, dehydration and hypoalbuminemia. • Temperature regulation is affected and patient behaves like a poikilothermic animal. • Death may occur due to cardiac failure, pneumonia and septicemia. Management It includes maintenance of the homeostasis and treatment of the primary disease.

Eczema

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Eczema

The word eczema is derived from the Greek word “ekzein” meaning “to boil out” or “to effervesce”. Eczema is an inflammatory skin reaction characterized histologically by spongiosis with varying degrees of acanthosis, and a superficial perivascular lympho-histiocytic infiltrate. The clinical features of eczema may include itching, redness, scaling and clustered papulo-vesicles. The condition may be induced by a wide range of external and internal factors acting singly or in combination. The terms ‘dermatitis’ and ‘eczema’ are nowadays generally regarded as synonymous, although some authors still use the term ‘dermatitis’ to include all types of cutaneous inflammation, so that all eczema is dermatitis, but not all dermatitis is eczema.

CLASSIFICATION OF ECZEMA Exogenous Eczema • Irritant dermatitis • Allergic contact dermatitis • Photoallergic contact dermatitis • Eczematous polymorphic light eruption • Infective eczema • Dermatophytide • Post-traumatic eczema.

Endogenous Eczema • Atopic dermatitis • Seborrheic dermatitis • Asteatotic eczema • Discoid eczema • Pompholyx • Pityriasis alba • Stasis dermatitis • Hand eczema • Lichen simplex chronicus • Prurigo nodularis • Lichen striatus • Juvenile plantar dermatosis • Metabolic eczema or eczemas associated with systemic disease • Eczematous drug eruptions. It must be remembered that, endogenous and exogenous factors may co-exist. For example, hand eczema, which is an endogenous eczema, is often aggravated by exogenous factors. Some of the important endogenous (Atopic dermatitis, seborrheic dermatitis, nummular eczema, pompholyx, pityriasis alba, stasis dermatitis, asteatotic eczema, lichen simplex chronicus, prurigo nodularis, lichen striatus) and exogenous eczema (Contact dermatitis, infective eczema/ dermatitis, polymorphous light eruption) are discussed as follows.

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ATOPIC DERMATITIS • Atopic dermatitis (AD) or eczema is a common chronic or relapsing dermatitis characterized by severe pruritus, occurring primarily in infants and children. • The age of onset is between 2 and 6 months in the majority of cases, but it may start at any age, even before the age of 2 months in some cases. • The disease arises as a result of a complex interplay between various genetic, immunological and environmental factors. • The environmental factors include (a) physical factors like sweating, climate, warm surroundings, detergents and soap, synthetic or woollen fabrics, cigarette smoke, (b) psychological factors, (c) food items (including tomato, orange and citrus fruits juices, meat, fish) (d) allergens such as house dust mite, animal hair, pollen, plants and others such as Staphylococcus aureus and release of exotoxins (superantigens) and saliva in small children. • Majority of cases are associated with a sensitization to environmental allergens and increased serum IgE (extrinsic AD), but about 10-30% of all cases lack any link to the classical atopic diathesis and are labelled as intrinsic AD. • There is no laboratory gold standard for the diagnosis of AD. A detailed history and a characteristic clinical picture would establish the diagnosis. Hanifin and Rajka have laid down certain major and minor criteria for making a diagnosis of AD.

Must have Three or More Major Findings • Pruritus • Typical morphology and distribution (Flexural lichenification or linearity in adults; Facial and extensor involvement in infants and children).

• Chronic or chronically relapsing dermatitis • Personal or family history of atopic dermatitis (e.g. asthma, allergic rhinitis, atopic dermatitis).

Plus Three or More Minor Findings • Xerosis • Ichthyosis • Palmar hyperlinearity • Keratosis pilaris • Immediate (type 1) skin test reactivity • Elevated serum IgE level • Early age of onset • Tendency towards extraneous infections (especially with Staphylococcus aureus and herpes simplex) or impaired cell mediated immunity • Propensity towards nonspecific dermatitis of the hand or foot. • Nipple eczema • Cheilitis • Recurrent conjunctivitis • Dennie -Morgan infraorbital fold • Keratoconus • Anterior subcapsular cataracts • Orbital darkening • Pityriasis alba • Facial pallor or facial erythema • Anterior neck folds • Itch when sweating • Intolerance to wool and lipid solvents • Food intolerance • White dermographism or delayed blanch. Course of atopic dermatitis may be divided in three phases: 1. Infantile phase 2. Childhood phase 3. Adult phase The distribution of lesions varies with age: Face (Fig. 11.1)and scalp involvement is common in infants as well as the extensor surfaces of the extremities and the trunk. In the childhood phase of AD 18-24 months onwards, eczema is

Eczema

Fig. 11.1: Atopic dermatitis—face

Fig. 11.2

Fig. 11.3

Figs 11.2 and 11.3: Atopic dermatitis–discoid eczematous lesions also appearing on flexor aspect of limbs in a child

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Essentials in Dermatology observed on the flexural surfaces, including the neck, antecubital or popliteal fossae, wrists and ankles (Figs 11.2 and 11.3). There is an adult phase, which occurs in adolescents and adults, where lichenification of the flexures and the hands commonly occurs. Some important signs: Thinning of lateral eyebrows, Hertoghe’s sign is sometimes present. Hyperkeratosis and hyperpigmentation producing a “dirty neck” appearance is also frequent in atopic individuals. Atopic individuals often exhibit perioral, perinasal and periorbital pallor “headlight sign”. Differential diagnosis: The diagnosis of atopic dermatitis (AD) is rarely aided by investigations. In the individual patient, one must consider a number of other conditions. Scabies should always be excluded, and can cause confusion when superimposed on pre-existing AD. In the first few months of life, the differentiation of infantile seborrhoeic dermatitis from atopic dermatitis can be difficult, although with time the distinction becomes apparent. The most useful distinguishing feature between atopic dermatitis and seborrheic dermatitis is the increased number of lesions on the forearms and shins in the former and axillae in the latter. The development of the lesions solely in the diaper area favors a diagnosis of infantile seborrheic dermatitis. Absent to mild pruritus is considered a significant feature of infantile seborrheic dermatitis. Immunodeficiency states should also be considered in infants in whom the disease is unusually severe, when there are recurrent systemic or ear infections and if there is failure to thrive, malabsorption or petechiae. Then appropriate investigations should be performed, for example, immunoglobulin levels and subclasses, IgE levels, white cell count, platelets, complement levels, and function, and T, B, and phagocyte numbers and functions. If clinically appropriate, one may also consider testing for

HTLV-I and human immunodeficiency virus (HIV). Treatment: No disease is more complicated to treat than atopic dermatitis. It is absolutely essential to counsel and educate the patient and their parents. Avoidance of exacerbating factors like wool clothes, house dust mites, detergents, dietary or aero-allergens, stress, etc. is advised wherever implicated. The treatment is aimed at suppressing the symptoms and controlling or preventing complications. Besides emollients for routine skin care, topical corticosteroids (TC) are the mainstay of treatment for AD to control acute exacerbations and can be used safely if certain precautions are taken. Topical calcineurin inhibitors (TCIs: Tacrolimus 0.03% and 0.1% and Pimecrolimus 1%) are recommended in patients of AD who are unresponsive to or intolerant to other conventional therapies, should be applied over the affected areas twice daily. Tacrolimus 0.03% and 0.1% formulations are recommended for the use in adults, whereas only 0.03% formulation is recommended for use in children aged 2-15 years, often first line for face, intertriginous areas and genitalia, useful in early flare, continue treatment till disease clears (approximately 1 month), and is useful for maintenance therapy. Systemic corticosteroids have a limited role in tiding over occasional flares of severe AD. Sedative antihistamines such as hydroxyzine and promethazine are preferred to non-sedative ones to control pruritus. Avoidance of provoking factors is paramount to achieve control. • Other treatment modalities include phototherapy and photochemotherapy, cyclosporin, evening primrose oil, azathioprine, Chinese herbal medicines, interferons, thymopentin, human interferon gamma and plasmapheresis. • Algorithmic approach to management depending upon severity of AD.

Eczema

1.

Mild AD

Bland emollients and parents/patient counseling Mild to moderate TCs, followed by TCI or TCI as first line of therapy Sedative anti-histamines

2.

Moderate AD

Bland emollients and parents/patient counseling Initial control with TCs and at the earliest shift to TCI’s Anti-histamines Narrow band UVB Systemic corticosteroids for short period

3.

Severe AD

Bland emollients and parents/patient counseling Anti-histamines Systemic antibiotics Oral corticosteroids/cyclosporine/azathioprine

• Course and prognosis— Atopic dermatitis follows a highly variable course with exacerbations and remissions. About 95% of children with AD remit around puberty, but relapses m ay occur and the disease may persist well into adulthood.

SEBORRHEIC DERMATITIS (SD) • Seborrheic dermatitis is a common, endogenous eczema that is usually easily recognized. • It affects infants and adults and is often associated with increased sebum production (seborrhea) of the scalp and the sebaceous follicle–rich areas of the face and trunk. • Various genetic factors, hormonal factors and increased colonization of Malassezia may play a role in the causation and perpetuation. • Seborrheic dermatitis has two age peaks, one in infancy within the first 3 months of life and the second around the second to the third decade of life. Men are affected more often than women in all age groups.

Classification of Seborrhoeic Dermatitis Infantile • Scalp (Cradle cap) • Trunk (including flexures and napkin areas) • Leiner’s disease.

Adult • Scalp: — Dandruff — Inflammatory • Face (may include blepharitis and conjunctivitis) • Trunk: — Petaloid — Pityriasiform — Flexural — Eczematous plaques — Follicular. • Generalized (may be erythroderma) • In infants, the scalp (the frontal and parietal scalp regions are covered with an oilylooking, thick, often fissured crust -crusta lactea (milk crust, or cradle cap) (Fig. 11.4), face and diaper areas are often involved. Leiner’s disease is due to complement C5 deficiency, presents as erythroderma. • Clinically, adult patients develop erythema and greasy scale on the scalp (with often pruritus), paranasal areas, eyebrows, nasolabial folds, central chest and intertriginous folds (seborrheic areas of the body). Rarely generalized lesions may occur. Dandruff is usually the earliest manifestation of seborrheic dermatitis.

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Fig. 11.4: Seborrheic dermatitis-cradle cap in an infant

Fig. 11.5: Seborrheic dermatitis in an adult–greasy scaling with erythema involving eyebrows, nasolabial folds and beard area in a HIV case

• ‘Corona seborrheica’, when SD on the scalp extends beyond the frontal hairline onto the forehead. • Sebopsoriasis: Early stage of scalp psoriasis may closely resemble seborrheic dermatitis with similar clinical and histological features. Some believe that seborrheic dermatitis may be a precursor of scalp psoriasis. • SD occur more commonly in variety of medical disorders like parkinsonism, myocardial ischemia, malabsorption, epilepsy, obesity and alcoholic pancreatitis. • SD can be a cutaneous marker of early HIV infection (Fig. 11.5). • Patients with HIV infection often have severe recalcitrant disease. • The disease is usually protracted over weeks to months. The prognosis is good. • Differential diagnosis: 1. HIV must be ruled out as patients with HIV tend to have extensive/severe SD.

2. Scalp psoriasis: The lesions are well defined, palpably thickened, brighter pink in colour with silvery scales. 3. Infective dermatitis complicating pediculosis may mimic SD. 4. Truncal SD should be differentiated from pityriaisis rosea, tinea versicolor. 5. Flexural SD should be differentiated from tinea, candidiasis, erythrasma. 6. Follicular SD should be differentiated from Darier’s disease. 7. SD-like lesions can occur in acrodermatitis enteropathica. 8. Drugs producing SD-like rash: Methyl dopa, chlorpromazine, cimetidine. • Treatment: In general, therapy is directed towards loosening and removal of scales (daily shampoo containing selenium sulfide, zinc pyrithione, ketoconazole, cetavalon, salicylic acid, coal tar; topical corticosteroids; topical antifungals like imidazoles) and

Eczema crusts, inhibition of yeast colonization, control of secondary infection, and reduction of erythema and itching. For unresponsive cases, narrow band UVB, oral ketoconazole, itraconazole or terbinafine may be used. Other treatment modalities include topical metronidazole (1%) gel, topical lithium succinate, vitamin D 3 analogues, and oral isotretinoin. • Patients should be informed about the chronic nature of the disease and understand that therapy works by controlling the disease rather than by curing it.

NUMMULAR ECZEMA (NE) (DISCOID ECZEMA) • The word nummular means (Latin nummulus) –‘coin like’. • Etiology of this is unknown. • It mainly affects men aged 55-65 years. • The eruption is characterized by pruritic, coin shaped, eczematous lesions (Fig. 11.6) • The lesion tends to develop on the extensor surfaces of the extremities and trunk.

• Differential diagnosis: NE may simulate tinea corporis (where scaling of the edge is more conspicuous, scrapings shows presence of mycelia). Exogenous dermatitis and irritant dermatitis occasionally presents with discoid response. In psoriasis, the lesions are dry, the scaling is more prominent and the irritation is milder. • NE has a chronic course with frequent relapses. • Treatment: open wet compresses, topical steroids and antibiotics for secondary infection.

POMPHOLYX (DYSHIDROSIS) • Pompholyx is characterized by itchy deepseated vesiculation on the palms and sides of the fingers. Lesions appear ‘sago-like’. • It may involve the soles of the feet. • It occurs bilaterally symmetrically, has relapsing course. • Differential diagnosis: 1. Id eruptions: Id eruptions are pompholyxlike eruptions due to distant focus of infections (e.g. kerion or bullous tinea pedis) 2. Pustular psoriasis of palms and soles: Sterile pustules, absence of clear vesicles, and characteristic residual brown marks as the lesions subside 3. Pemphigoid lesions occurring on the palms 4. Bullous tinea pedis • Treatment: open wet compresses, topical steroids and antibiotics for secondary infection.

PITYRIASIS ALBA

Fig. 11.6: Nummular eczema—coin shaped eczematous lesions over the leg

• Pityriasis alba is a common disorder in children that is characterized by an asymptomatic, hypopigmented, slightly elevated, fine, scaling plaque with indistinct borders.

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Essentials in Dermatology • It usually disappears by adulthood. • The condition commonly affects the face. • Differential diagnosis: 1. Indeterminate leprosy: Lesion is usually solitary to a few, hypopigmented macule with ill-defined margins and equivocal sensory loss. 2. Tinea versicolor: Lesions are well-defined hypopigmented macules with branny scales. KOH examination shows ‘spaghetti and meatball’ appearance. 3. Vitiligo-early lesions may mimic but lack scaling. 4. Mycosis fungoides although relatively rare may present with lesions clinically resembling pityrasis alba. • Treatment: Emollients, mild topical steroid or topical pimecrolimus or tacrolimus application may suffice.

STASIS DERMATITIS • Stasis dermatitis occurs as a result of venous stasis on the lower portions of the legs. • The dermatitis may be acute, subacute or chronic. • It differs from other forms of dermatitis, firstly by showing brownish black pigmentation and secondly, by resulting in some instances in ulceration and atrophic scarring. • The surrounding skin may show changes due to venous stasis–hyperpigmentation, induration of skin and lipodermatosclerosis in late stage. • Differential diagnosis: 1. Allergic contact dermatitis usually caused by topical medications. Patch testing is helpful. 2. Infected ulcer with infective eczematoid dermatitis around the ulcer responds to appropriate antibiotic therapy. 3. Discoid eczema is common on lower legs usually on the anterior or anterolateral aspect.

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4. Asteatotic eczema commonly affects the legs of elderly. 5. Dermatophyte infection may present as diffuse erythema and scaling and can be difficult to recognize, particularly if it has been treated with topical steroids. 6. Psoriasis may present as a single irritable plaque on the leg but is usually more scaly and clearly marginated Treatment: The underlying venous hypertension should be controlled. Well fitted stockings can be helpful if worn regularly. The legs should be elevated when patient is recumbent. Topical steroids may be used to relieve irritation.

ASTEATOTIC ECZEMA (ECZEMA CRAQUELE, WINTER ECZEMA, DERMATITIS SICCA) • It is an eczema associated with a decrease in skin surface lipids. • The eczema occurs after excessive drying, especially during the winter months and among the elderly and those with atopic dermatitis or ichthyosis vulgaris. • The skin of the limbs and trunk is erythematous, dry and itchy and shows a fine crazy-paving pattern of fissuring. • Differential diagnosis: Atopic dermatitis, various forms of ichthyosis especially acquired ichthyosis. • Treatment: Central heating should be humidified where possible. Avoidance of frequent bath, use of synthetic detergent instead of soap, regular lubrication of the skin especially after bathing helps. Weak topical steroids are often prescribed.

LICHEN SIMPLEX CHRONICUS (NEURODERMATITIS) • Lichen simplex chronicus is characterized by lichenified plaque lesion or lesions due to

Eczema repeated rubbing or scratching as a habit or due to “stress”. • Lichenification of the skin follows chronic scratching and/or rubbing. This may occur without a predisposing dermatosis, or may follow any pruriginous dermatosis. The term lichen simplex is used when there is no predisposing dermatosis. • It is characterized histologically by acanthosis and hyperkeratosis and clinically by thickened appearance of the skin, with accentuation of skin markings so that the affected skin surface resembles tree bark.

Fig. 11.7: Lichen simplex chronicus—thickening, and pigmentation of ankle

Fig. 11.9: Lichen simplex chronicus-involving female external genitalia

• The areas most commonly affected are those that are conveniently reached. • These areas are ankles (Fig. 11.7) and wrists, nape of neck (Fig. 11.8), genitalia (scrotum or mons pubis) (Fig. 11.9), etc. • It is common in Indian subcontinent amongst adults (30-50 years). • Differential diagnosis: The morphological diagnosis of lichenification is not usually difficult-lichen planus, lichen amyloidosis, and psoriasis have to be excluded and typical lesions sought in other sites. • Treatment: Patient should be given some assistance in reducing their tension. Sedation is often needed and sedative antihistaminics may be helpful. In most cases, a steroid cream is the treatment of choice. To prevent the scratching and to improve the outcome of the treatment, steroid may be applied under polythene occlusion.

PRURIGO NODULARIS (PN, HYDE’S PRURIGO)

Fig. 11.8: Lichen simplex chronicus— involving nape of neck

• Prurigo is a Latin word for itching. • PN is an intensely pruritic disorder in which persistent rubbing and scratching in particular area leads to formation of distinctive hemispherical nodules with raised warty surface (Fig. 11.10).

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Fig. 11.11: Lichen striatus—linearly arranged flat topped papular lesions over the chest of a child

Fig. 11.10: Prurigo nodularis—raised itchy nodular lesions over the legs and dorsa of feet

• Disease occur in 20-60 years of age, both sexes are equally affected. • The cause is unknown. The initial trigger for itching in PN may include local trauma, insect bite reactions and atopic eczema, etc. • Differential diagnosis: 1. Hypertrophic lichen planus: Usually violaceous, may be associated with typical lichen planus lesions elsewhere. 2. Pemphigoid nodularis may present as nodular prurigo for sometime before typical urticated plaques and blisters supervene. 3. Allergic contact dermatitis may result in papulonodular eruptions. 4. Systemic causes of pruritus that can give rise to lesions resembling nodular prurigo include renal failure, liver disease, lymphoma, and HIV infection. • Treatment: Oral antihistamines, emollients, topical steroids with salicylic acid and

intralesional steroids, cryotherapy, and systemic agents such as thalidomide, cyclosporine and azathioprine.

LICHEN STRIATUS • It is an acquired self-limited inflammatory linear skin disorder • Exact etiology is unknown • Children aged 5 to 15 years are commonly affected • The most characteristic lesion is a linear band of hypopigmented lichenoid papules that follow Blaschko’s lines (Fig. 11.11) • The lesions commonly occur on the arm or leg • Course of the lesion may extend for 2 weeks to 4 months. • Differential diagnosis: Epidermal naevi (persist indefinitely), linear lichen planus or psoriasis (easily differentiated clinically, even in the absence of typical lesions in other sites which should always be sought for) • Treatment: Usually none is necessary, topical steroids, topical tacrolimus and intralesional steroids in persistent cases.

Eczema

HAND ECZEMA • Hand eczema is not a single diagnostic entity. It is rather a morphological presentation of various types of eczemas largely confined to the hands and can have a multitude of factors responsible for causing it, singly or in combination. Causes of hand eczema are broadly grouped into two groups as shown below:

Exogenous 1. Contact irritants: Chemicals (e.g. soaps, detergents, solvents) Physical (e.g. friction, minor trauma, cold dry air) 2. Contact allergens: Delayed hypersensitivity (e.g. chromium, rubber) Immediate hypersentivity (e.g. seafood) 3. Ingested allergens (e.g. drugs, nickel, chromium) 4. Infection (e.g. bacterial infections of hand wounds) 5. Secondary dissemination (e.g. dermatophytide reaction to tinea pedis). Endogenous 1. Idiopathic (e.g. Discoid, hyperkeratotic palmar eczema) 2. Immunological or metabolic defects (e.g. atopics) 3. Psychosomatic: Stress aggravates, but may not be causative 4. Dyshidrosis: Increased sweating aggravates, but may not be causative. Morphological types of Hand Eczema 1. Pompholyx 2. Recurrent focal palmar peeling 3. Hyperkeratotic palmar eczema 4. Ring eczema 5. ‘Wear and tear’ dermatitis

6. Fingertip eczema 7. Apron eczema 8. Discoid eczema 9. Chronic acral dermatitis 10. Gut eczema. • Differential diagnosis: The diagnosis of hand eczema is usually self evident but distinction from psoriasis is very difficult. In most cases of psoriasis on hands, however, the silvery nature of the scales, involvement of knuckles, sharply demarcated scalloped edges to the erythema along the borders of the hands and fingers, and the relative absence of pruritus are helpful pointers. Family history of psoriasis and the presence of nail pits in the absence of nail fold lesions are also suggestive. Tinea can also be missed especially when it is extensive and irritable or secondarily infected. Unilateral scaling of palm should always suggest tinea manuum. • Treatment of hand eczema: Avoidance of irritants, frequent application of emollients, and sparing use of topical steroids.

JUVENILE PLANTAR DERMATOSIS (FOREFOOT ECZEMA) • Occurs mainly in children aged 3-14 years of age. • The presenting features are redness and pain on the plantar surface of the forefoot, which assumes a glazed and cracked appearance. • The condition is most severe on the ball of the foot and toe pads, and tends to spare the non-weight-bearing instep. • The symmetry of the lesions is a striking feature. • Differential diagnosis: The toe clefts are normal, and this helps to distinguish the condition from tinea pedis. Patch testing to exclude foot wear allergy may be done. • Treatment: Most cases will clear spontaneously during childhood or

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Essentials in Dermatology adolescence. Patients are advised to use emollients, 100% cotton socks and leather shoes or sandals.

CONTACT DERMATITIS Contact dermatitis is an eczematous dermatitis caused by exposure to substances in the environment. Common types of contact dermatitis are discussed here.

Irritant Contact Dermatitis (ICD) • ICD is a non-immunological eczematous reaction which occurs without previous sensitization in most exposed individuals. • Most common type of contact dermatitis. • Common irritants include alkalis (soaps, detergents), dye and ammonia containing compounds. • The clinical features vary from mild xerosis to erythema/chapping or even severe ulceration depending on the concentration of the irritant, the skin area exposed, mode of contact and the substance itself. • The reaction remains localized and does not spread to become generalized and it does not cause systemic symptoms. • Acute irritant dermatitis is characterized by vesicles and bullae (e.g. cement dermatitis, savlon induced reaction). • Chronic irritant dermatitis manifests as hyperkeratosis, fissuring and scaling (e.g. house wife dermatitis). • Healing usually occurs within 2 weeks of removal of noxious stimulus. • In cases of chronic subcritical level of irritation, some develop tolerance or hardening. • Differential diagnosis: Same as for allergic contact dermatitis • Treatment: avoidance of irritants, use of bland barrier creams, protective gloves and mild topical steroids.

Allergic Contact Dermatitis (ACD) • ACD results when an allergen comes in contact with previously sensitized skin. • It is a delayed hypersensitivity reaction, consisting of sensitization phase and when rechallenged-elicitation phase. • Occurs in individual of all ages, especially elderly. • Sensitization remains lifelong. • The allergens are extremely varied and may be nonprotein in nature. • Common causes of ACD include plants, nickel (Fig. 11.12), chromate, para phenylene diamine (hair-dye) (Figs 11.13 and 11.14),

Fig. 11.12: Contact dermatitis—dermatitis over abdomen due to trouser metallic hook

Fig. 11.13: Contact dermatitis—erythema and edema due to acute dermatitis caused by hair dye

Eczema

Fig. 11.14: Contact dermatitis—lichenoid dermatitis over the forehead due to hair dye

Fig. 11.16: Common agents involved in contact dermatitis of the face

Fig. 11.15: Contact dermatitis—dermatitis due to rubber chappal

rubber compounds (Fig. 11.15), fragrances and preservatives in compounds. • The eczematous reactions develops at the sites of skin contact with the allergen (Fig. 11.16) but occasionally spreads outside these limits and cause systemic symptoms. • The patch test is diagnostic and helps to identify allergens involved. • Patch test consists of application of substances suspected to be the cause of the contact dermatitis to the intact uninflammed skin, in nonirritating concentration.

• Most common plant allergen in India is Parthenium hysterophorus. • Most common metal causing ACD is nickel • Differential diagnosis: Main differential diagnostic consideration is irritant contact dermatitis. Others are erysipelas (rapidly spreading, non-pruritic, well defined erythematous area, and patient is sick), atopic dermatitis, nummular eczema, tinea (KOH examination), seborrhoeic dermatitis, polymorphous light eruption, and angioedema • Treatment: The most important step is avoidance of allergens. Acute dermatitis of any sort is best treated with moist compresses and high potency corticosteroid creams. In severe cases, a short burst of systemic corticosteroids tapered over 7-10 days is needed. More chronic cases can be treated with lower potency corticosteroids in an ointment base. Oral cyclosporine is indicated for therapy resistant chronic disease.

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DIFFERENCES BETWEEN IRRITANT CONTACT AND ALLERGIC CONTACT DERMATITIS Irritant contact dermatitis

Allergic contact dermatitis

*People at risk *Mechanism of response *Prior exposure required *Nature of substance

Everyone Nonimmunologic No Organic solvents, soap

*Concentration of substance required *Symptoms *Lesions *Involvement of non-exposed sites *Type of inflammatory cells *Patch test

Usually high Severe, stinging or burning sensation Large tense bullae with necrosis No Neutrophils Not useful

Genetically predisposed Delayed hypersensitivity Yes Low molecular weight hapten (metal, formalin) May be low Pruritus Papules or papulovesicles Yes Eosinophils and lymphocytes Diagnostic

INFECTIVE DERMATITIS (INFECTIOUS ECZEMATOID DERMATITIS, MICROBIAL ECZEMA) • Infective eczema is caused by microorganisms or their products, clears when organisms are eradicated. • It is seen predominantly around discharging wounds or ulcers or moist skin lesions of other types. • It presents as an area of advancing erythema, sometimes with microvesicles (Fig. 11.17). • Treatment: Factors predisposing to infection should be sought and when possible eliminated. Wet compresses, topical antibiotic in combination with steroid or systemic antibiotic.

POLYMORPHIC LIGHT ERUPTION (PMLE) • PMLE is the most common photodermatoses. • The disease may begin at any age and the symptoms are severe in spring and summer season. • It is an idiopathic eruption caused by UV exposure; appears in hours to days with varied morphology (papular variant most

Fig. 11.17: Infective dermatitis—oozing dermatitic lesions behind ear and trunk

common, papulovesicular, eczematous, erythematous, plaque like). Clinically, it presents as non scarring, erythematous, itchy papules, plaques or vesicles on exposed skin that usually heal without scarring. • The amount of light exposure needed to elicit an eruption varies greatly from one patient to another.

Eczema • Light sensitivity decreases with repeated sun exposure, this phenomenon is referred to as hardening. • Differential diagnosis: Lupus erythematosus, photosensitive drug eruption, prurigo nodularis, and photoallergic contact dermatitis need to be differentiated.

• Treatment: Photoprotection by clothing and sunscreens, phototherapy, and topical steroids. In severe resistant cases, consider the use of azathioprine 50-100 mg daily for 3 months. Use antioxidants, one week before exposure may help in prevention.

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12

Vesiculobullous Disorders

Vesiculobullous disorders cause significant morbidity and mortality in dermatology. While a number of disorders of varied etiology can present with vesicles or bullae, two distinct groups of disorders are the genetic blistering diseases (epidermolysis bullosa) and the acquired autoimmune blistering disorders (pemphigus, bullous pemphigoid, dermatitis herpetiformis, chronic bullous dermatosis of childhood, epidermolysis bullosa acquisita). Vesiculobullous disorders are diseases where the primary lesion is a vesicle or bulla. It occurs due to pathology in the epidermis or dermoepidermal junction or dermis. In this chapter we are not dealing with secondary causes of vesiculation like to burns, suction blisters, ischemia leading to blisters (vasculitis, diabetic bulla), drug eruptions like bullous fixed drug eruption, insect bite reaction, infections (varicella, herpes simplex, herpes zoster), dermatitis, miliaria, etc. This chapter would discuss primary vesiculobullous disorders. In epidermal autoimmune vesiculobullous disoders, the mechanism behind blister formation is primarily acantholysis in most cases. Decreased stability of basement membrane is responsible for a variety of subepidermal vesiculobullous disoders which could be either inherited or of acquired autoimmune origin.

CLASSIFICATION OF VESICULOBULLOUS DISORDERS 1. Inherited/genetic blistering disorders: a. Epidermolysis bullosa simplex b. Junctional epidermolysis bullosa c. Dystrophic epidermolysis bullosa. 2. Acquired autoimmune blistering disorders: a. Intraepidermal immunobullous diseases 1. Pemphigus vulgaris and its variant pemphigus vegetans. 2. Pemphigus foliaceous and its variants i. Endemic pemphigus foliaceus (Fogo selvagem; wild fire). ii. Pemphigus erythematosus (SenearUsher syndrome) iii.Pemphigus herpetiformis. 3. Drug induced pemphigus. 4.. IgA pemphigus i. Intraepidermal neutrophilic type ii. Subcorneal pustular dermatosis type. 5. Paraneoplastic pemphigus. 6. Subcorneal pustular dermatosis (Sneddon –Wilkinson disease). 3. Subepidermal immunobullous diseases a. Bullous pemphigoid and its variants b. Mucous membrane pemphigoid (cicatricial pemphigoid)

Vesiculobullous Disorders c. Pemphigoid gestationis (Herpes gestationis) d. Linear IgA disease/Chronic bullous dermatosis of childhood e. Dermatitis herpetiformis f. Epidermolysis bullosa acquisita g. Bullous systemic lupus erythematosus. The following discussion deals with these two groups of disorders with special focus on the more common entities.

EPIDERMOLYSIS BULLOSA • Epidermolysis bullosa (EB) comprises a group of genetically determined skin disorders characterized by blistering of the skin (Fig. 12.1) and mucosae at birth or soon afterwards, following mild mechanical trauma (due to increased fragility of skin). Thus an alternative term for these disorders could be the ‘mechanobullous’ disorders. • There are three main types of EB: – EB simplex (intraepidermal split due to disruption of basal keratinocytes) – Junctional EB (split through the basement membrane zone) – Dystrophic EB (split in the subepidermal level).

Fig. 12.1: Epidermolysis bullosa—baby showing multiple erosions

• EB simplex is the commonest and mildest form of EB of autosomal dominant inheritance. It is characterized by onset of blistering over trauma prone sites at birth or infancy. Lesions heal without scarring. Mucosae, nails and hair are essentially uninvolved. • Junctional EB are autosomal recessive disorders and are broadly classified into two main types, the lethal and non-lethal forms. They present at birth or soon after with severe fragility of the skin leading to extensive blistering and denudation. Oropharyngeal mucosae may be severely involved. Teeth may be malformed and prematurely lost and nails may be shed. This is the most fatal type of EB. • Dystrophic EB is characterized by skin fragility, scarring with milia (Fig. 12.2), nail changes (Fig. 12.3) and have either autosomal recessive or dominant inheritance. The more severe autosomal recessive form is characterized by:

Fig. 12.2: Epidermolysis bullosa dystrophica–knee showing hemorrhagic bullae with scarring, milia and pigmentation

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Essentials in Dermatology • Gene therapy appears as a realistic goal in the future.

PEMPHIGUS

Fig. 12.3: Epidermoloysis bullosa dystrophica– albopapuloid lesions over the dorsa of feet and some of the toenails completely lost

– Onset at birth or early infancy – Blistering of skin mainly over trauma prone sites – Oral blisters and scarring leading to ankyloglossia and microstomia – Esophageal lesions causing painful dysphagia and later esophageal strictures. – Perianal blistering, erosions and scarring causing stenosis and fecal retention. – Ocular complications—symblepharon, corneal erosions and opacity – Repeated blistering and progressive scarring—contractures and deformities (e.g. ‘Mitten hands’). • Diagnostic techniques include skin biopsy, electron microscopy to ascertain the level of split and structures involved, antigen mapping and immunohistochemistry. No autoantibodies are demonstrated in the sera. Prenatal DNA testing can be advised to couples at risk of having affected children. • No specific treatment is available for EB and thus the mainstay of treatment is based on avoidance of provoking factors. Management of the neonate includes maintaining adequate nutrition and hydration and prevention of sepsis. Other aspects include care of the oral cavity, teeth, eyes and management of contractures and deformities.

• Pemphigus is derived from the Greek ‘pemphix’ meaning blister or bubble and is characterized by intraepidermal blistering at various levels in the epidermis. • The key pathogenic process in this group of disorders is disruption of the intercellular cementing substance due to an autoantibody attack on the cellular adhesion proteins (desmogleins) leading to acantholysis. • The pemphigus group of disorders includes two major types (and their variants) and several other minor types. The major types are: – Pemphigus vulgaris (variant – Pemphigus vegetans): level of split-suprabasal. – Pemphigus foliaceus (variant – Pemphigus erythematosus): level of split – subcorneal. • The other minor types of pemphigus include paraneoplastic pemphigus, drug induced pemphigus, IgA pemphigus and neonatal pemphigus.

Pemphigus Vulgaris • Most common form of pemphigus, accounting for up to 80% of pemphigus cases. Occurs at any age, most commonly between fourth- sixth decades. In India, it occurs at younger age. • It is due to IgG antibodies directed against epidermal cell adhesion molecules (desmoglein 3)- disruption of intercellular cementing substance—loss of adhesion between epidermal cells (acantholysis) intraepidermal blistering. Clinical Features • Almost all patients have mucosal lesions, 50–70% present with painful oral erosions. Lesions may be limited to oral cavity for months to one year (Fig. 12.4).

Vesiculobullous Disorders • Skin – flaccid bullae on normal or erythematous skin, with a predilection for scalp, face (Fig. 12.5), trunk (Figs 12.6 and 12.7), axillae and groins and pressure sites. Bullae rupture producing painful erosions that show no tendency to heal spontaneously. • Pruritus is absent or negligible. • Nikolsky’s sign and Bulla spread sign (Asboe-Hansen sign) are positive. (Other disorders with positive Nikolsky’s sign are

staphylococcal scalded skin syndrome, toxic epidermal necrolysis, etc.). • Other mucosae involved are conjunctiva, pharynx, larynx, oesophagus, urethra, vulva and cervix.

Fig. 12.4: Pemphigus vulgaris—painful erosions involving tongue and lips

Fig. 12.5: Pemphigus vulgaris—extending erosions without tendency to heal over the face

Fig. 12.6: Pemphigus vulgaris—involving chest

Fig. 12.7: Pemphigus vulgaris—involving back

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Essentials in Dermatology • Prognosis is poor without treatment but with systemic steroids mortality has been reduced to 5–15 %. • Pemphigus may be associated with other autoimmune diseases such as thymoma, myasthenia gravis and malignancies like lymphomas and bronchogenic carcinoma.

Diagnosis • Tzanck smear from the floor of the blister shows acantholytic cells. Acantholytic cell is a large, rounded epidermal cell with a large nucleus, perinuclear halo and peripheral condensation of cytoplasm (Fig. 12.8). • Histopathological examination of a blister shows a supra-basal cleft in the epidermis. The basal keratinocytes remain attached to the basement membrane but are separated from each other and stand like a ‘row of tombstones’. • Immunofluorescence studies are the gold standard in diagnosis of the autoimmune blistering disorders. In pemphigus vulgaris, direct immunofluorescence done on the lesional skin shows deposition of intercellular IgG throughout the epidermis in a ‘fish-net’ pattern (Fig. 12.9). Indirect immunofluorescence done to determine levels of pathogenic antibodies in the sera of the

Fig. 12.8: Tzanck smear showing large rounded epidermal cells with large nuclei, perinuclear halo and peripheral condensed cytoplasm (Acantholytic cell)

Fig. 12.9: Direct immunofluorescence showing fish net pattern

patients shows circulating intercellular IgG antibodies in 80–90% of the cases. Levels of these antibodies correlate with disease activity.

Pemphigus Vegetans It is a clinical variant of pemphigus vulgaris characterized by vegetating lesions primarily in the flexures (Figs 12.10 and 12.11). Initial lesions are bullae or pustules, which rupture and progress to form vegetating plaques. Pemphigus Foliaceous • This disorder, characterized by blistering at a higher level in the epidermis is less common than pemphigus vulgaris and accounts for only 15–20 % of pemphigus cases. • It is caused by IgG antibodies directed against intercellular adhesion molecules (desmoglein 1) found predominantly in the upper epidermis—disruption of intercellular cementing substance of upper epidermal cellssubcorneal blister formation. • Clinically, pemphigus foliaceous is less severe than pemphigus vulgaris and is characterized by crusted, moist, scaly lesions in a seborrheic distribution (Fig. 12.12) involving scalp, face, chest and upper back. Blistering may not be obvious due to the superficial level of the split (very transient nature of blisters).


Fig. 12.10: Pemphigus vegetans—vegetating moist lesions occurring in the axilla

Fig. 12.12: Pemphigus foliaceous—moist scaly lesions in seborrheic areas of the face

Fig. 12.11: Pemphigus vegetans—vegetating moist lesions in the retroauricular area

• Oral lesions are uncommon. • Nikolsky’s sign is invariably positive (Fig. 12.13).

Fig. 12.13: Nikolsky’s sign – tangential pressure with finger tip producing moist erosion due to peeling of skin

Diagnosis • Tzanck smear from fresh erosion shows acantholytic cells. • Histology shows a subcorneal cleft with acantholysis.

• Immunofluorescence findings are usually indistinguishable from pemphigus vulgaris. Prognosis of this disorder is better than pemphigus vulgaris. This benign disorder responds well to treatment.

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Pemphigus Erythematosus It is a variant of pemphigus foliaceous characterized by immunological features of both pemphigus and lupus erythematosus (LE), that is, intercellular IgG and C3 in the epidermis (as in pemphigus) and in the basement membrane zone (as in LE) and antinuclear antibodies (as in LE). Clinically, erythematous, scaly rash over the nose and cheeks simulate LE while lesions on the trunk are similar to those in pemphigus foliaceous. Other Variants of Pemphigus • Endemic pemphigus foliaceous (Fogo Selvagem) is a variant of pemphigus foliaceous, endemic to certain parts of South America and is postulated to be precipitated by bites of the black fly (Simuliidae). The burnt appearance and burning sensation gave the disease its name, fogo selvagem, meaning “wild fire”. • Drug induced pemphigus (penicillamine, captopril, pyritinol, penicillin, rifampicin)— clinically commonly present as pemphigus foliaceous. • Paraneoplastic pemphigus—a polymorphous blistering eruption with mucocutaneous ulcerations having an underlying neoplasm. • Pemphigus herpetiformis—superficial vesicles and inflammatory papules occur in herpetiform distribution. • IgA pemphigus—has bound and circulating IgA autoantibodies against intraepidermal cell surface antigens and clinically may resemble subcorneal pustular dermatosis. • Juvenile pemphigus— pemphigus occurring before 20 years of age • Neonatal pemphigus— due to transplacental transfer of maternal anti-intercellular cement substance antibodies to the fetus. Blisters resolve in 2 weeks.

Differential Diagnosis When skin is involved, other autoimmune vesiculobullous disorders need to be differentiated from pemphigus. On rare occasions, bullous impetigo, dyskeratotic acantholytic disorders (Darier’s disease, Hailey Hailey disease, Grover’s disease) can cause a problem. When only the oral mucosa is involved, the following should be considered-aphthous ulcerations, oral erosive lichen planus, herpetic gingivostomatitis, erosive candidiasis, and erythema multiforme. When both skin and oral mucosa are involved, it closely resembles erythema multiforme, Stevens Johnson syndrome, toxic epidermal necrolysis, bullous systemic lupus erythematosus, and generalized bullous fixed drug eruption. Treatment of Pemphigus • The mainstay of therapy in the pemphigus group of disorders is with systemic steroids, which can be given as conventional therapy (oral prednisolone in the dose of 1 mg per kg body weight) or as pulse therapy. An upcoming mode of therapy is the dexamethasone cyclophosphamide pulse regimen consisting of 100 mg of IV dexamethasone in 5% dextrose infusion on 3 consecutive days of each month combined with IV cyclophosphamide 500 mg bolus dose on day 1 of the pulse and oral cyclophosphamide 50 mg daily on other days. This therapy reduces the conventional side effects of steroids. • The other modalities of therapy include adjuvant therapy with dapsone, azathioprine, cyclosporine, methotrexate, gold salts, mycophenolate mofetil, intravenous immunoglobulin therapy and plasmapheresis and are essentially to reduce the side effects of steroids or to control the severe form of pemphigus.


BULLOUS PEMPHIGOID (BP) • Autoimmune subepidermal blistering disorder of the elderly, with onset usually after 60 years of age. • Basic pathogenic process is due to IgG antibodies against components of the basement membrane zone (BMZ – structure which binds epidermis to the underlying dermis and mesenchyme)— loss of structural integrity of the BMZ due to resultant inflammation separation of the intact epidermis from underlying dermissubepidermal cleft formation.

Clinical Features • Preceded by pruritus with or without urticarial wheals lasting usually for 1–3 weeks. • Tense bullae on normal or erythematous skin predominantly over flexural aspects of the limbs (Fig. 12.14), lower abdomen (Fig. 12.15), groins and axillae. Facial skin and scalp relatively spared. – Lesions rupture to leave erosions that heal spontaneously with postinflammatory hyperpigmentation. – Nikolsky’s sign usually negative; Bulla spread sign +/– Mucosal lesions rare and less severe than in pemphigus vulgaris and almost never the presenting feature. • Bullous pemphigoid may be associated with an underlying malignancy (gastric carcinoma commonly) and may be associated with diabetes mellitus, rheumatoid arthritis and psoriasis. Diagnosis • Tzanck smear will show numerous eosinophils with few neutrophils but no acantholytic cell.

Fig. 12.14: Bullous pemphigoid—itchy urticarial lesions over the thigh developing into tense bullae

Fig. 12.15: Bullous pemphigoid—involving lower back

• Histopathological examination reveals subepidermal cleft with intact epidermis forming the roof of the blister. • Direct immunofluorescence of perilesional skin shows linear IgG (45–90%) and C3 (80–100 %) deposition in the basement membrane zone. Indirect immunofluorescence studies done on the patient’s sera show circulating IgG autoantibodies in most cases but unlike in pemphigus, their titers do not correlate with disease activity.

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Differential Diagnosis Bullous pemphigoid can be easily differentiated from most other blistering disorders such as linear IgA disease, chronic bullous disease of childhood, dermatitis herpetiformis, erythema multiforme and pemphigus by histology and immunofluorescence. Most difficult diseases to differentiate from bullous pemphigoid are epidermolysis bullosa acquisita and cicatricial pemphigoid. • Prognosis of bullous pemphigoid is better than pemphigus and it runs a chronic selflimiting course. It may be fatal in the active stage in the elderly or debilitated patients.

Fig. 12.16: Cicatricial pemphigoid— tense bulla over the tongue

Treatment • The mainstay of treatment is a topical or systemic steroid depending on the severity of the disease. Pulse dexamethasone cyclophosphamide, methyl prednisolone or cyclophosphamide pulse may be used. Other modalities include cyclophosphamide, azathioprine, dapsone, tetracycline with nicotinamide, erythromycin, leflunomide, sulphapyridine, methotrexate, mycophenolate mofetil, intravenous immunoglobulin therapy and plasmapheresis.

Fig. 12.17: Cicatricial pemphigoid—face showing symblepharon

Clinical Variants of Bullous Pemphigoid Include • Cicatricial pemphigoid is a rare blistering disorder of the mucosa and skin that results in permanent scarring of the affected area. Mucosal lesions predominate and involve in decreasing order the oropharynx (Fig. 12.16), nasopharynx, conjunctiva, larynx, genitalia and esophagus. Sequelae include oropharyngeal adhesions, esophageal strictures, stridor, introital shrinkage, symblepharon (Fig. 12.17) and ‘statue eye’. Unlike bullous pemphigoid, this disorder is not self-limiting and has a chronic debilitating course.

• Herpes gestationis is a non-viral autoimmune blistering disease of young women that occurs in pregnancy (21–28 weeks of gestation) or within 1st week postnatally. Clinically, the disease starts as severe pruritus with urticarial wheals and plaques followed by blistering predominantly in the periumbilical area, lower abdomen and thighs. Mucosal involvement is rare and lesions improve postpartum. Recurrence may occur in subsequent pregnancies, premenstrually or with oral contraceptive pills (OCPs). Direct immunofluorescence shows linear C3 deposits at the BMZ with IgG in some cases.


Characteristic

Bullous pemphigoid

Pemphigus vulgaris

Age Pruritus Blister Sites Erosion Nikolsky’s sign Oral lesions

> 60 years ++ Tense Flexures, groins, axillae, abdomen Spontaneously heal Negative 40 % less severe, almost never a presenting feature Benign, self limiting

Middle age Not present Flaccid Face, scalp, trunk, pressure points Extend peripherally Positive 80–90 % severe commonly the presenting symptom Poor without treatment

Prognosis

DERMATITIS HERPETIFORMIS (DH) • DH is defined as an intensely pruritic, chronic, recurrent, papulovesicular disease with an underlying gluten-sensitive enteropathy which may be asymptomatic.

Clinical Features • Onset at any age, usually between 20–55 years of age. • Males outnumber females. • Pruritus is the first and predominant symptom followed by a symmetrical eruption of erythematous papules and papulovesicles, which are so rapidly excoriated that intact vesicles are difficult to demonstrate (Fig. 12.18). • Sites – extensor aspects of limbs (elbows and knees), buttocks, natal cleft, shoulders, upper back (Fig. 12.19), face and scalp. Grouping of lesions accounts for it being described as herpetiformis (not associated with herpes virus). • Oral lesions are common but asymptomatic. • Provocation of lesions occurs with iodides orally or in iodide patch testing. • Histological examination best done on lesions that have not blistered or ruptured and reveals neutrophilic microabscesses at the tips of dermal papillae.

Fig. 12.18: Dermatitis herpetiformis—extremely itchy tense grouped vesicular lesions over the shoulder area

Fig. 12.19: Dermatitis herpetiformis—bilateral symmetrical distribution of itchy eroded lesions over the extensor aspect of limb and trunk

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Essentials in Dermatology • Direct immunofluorescence is the most reliable diagnostic criterion and should be performed on clinically normal skin (preferably of the buttocks). It reveals granular IgA deposits in dermal papillae. • Indirect immunofluorescence is negative for anti-BMZ or dermal autoantibodies but antithyroid and antigliadin antibodies may be seen.

chronic bullous disease/dermatosis of childhood (CBDC), with onset in childhood and linear IgA disease which presents in adults. There is lot of overlap in the clinical presentation of the two entities but they differ in their age at presentation and few clinical signs. In linear IgA disease, periorificial and annular lesions are not as common as in CBDC. Hemorrhagic bullae may be present.

Differential Diagnosis Dermatitis herpetiformis can be confused with numerous conditions because of its pleomorphic manifestations and occasional lack of diagnostic lesions. Scabies, bullous pemphigoid before the development of blisters, and the prurigo group of disoders are the main issues. It also needs to be differentiated from chronic exudative eczema, papular urticaria, neurotic excoriations, and transient acantholytic dermatoses. A high index of suspicion is very helpful, even in the absence of primary lesions. Dermatitis herpetiformis can be diagnosed based on the typical in vivo bound granular IgA deposits in normal appearing skin.

Chronic Bullous Disease/dermatosis of Childhood (CBDC, Linear IgA disease of Childhood) • CBDC is defined as a chronic acquired autoimmune subepidermal blistering disease of children characterized by IgA BMZ antibodies. • Onset is usually at around 5 years of age (toddlers and preschool children). • Urticarial plaques with blistering at the edges – ‘string of pearls’ appearance and localization of lesions around orifices (perioral, perigenital) (Figs 12.20 and 12.21).

Treatment • Dapsone 100–200 mg/day (up to 400 mg /day) is the drug of choice. It is amazingly effective; hours to days after the first dose, the pruritus disappears and no new lesions erupt after 1-2 days of treatment. • Strict adherence to a gluten-free diet for prolonged periods (e.g. 6 to 12 months) may control the disease in some patients, obviating or reducing the requirement for drug therapy. LINEAR IgA DISEASE It is a chronic acquired subepidermal blistering disorder of children and adults, with skin and mucous membrane involvement and characterized by linear deposition of IgA at basement membrane zone. It consists of two main entities-

Fig. 12.20: Chronic bullous dermatosis of childhood— clustering of tense bullae around the mouth

Vesiculobullous Disorders combined with low dose steroids in refractory cases.

Fig. 12.21: Chronic bullous dermatosis of childhood– lower limbs showing “cluster of jewels” appearance at many sites

• Spontaneous remission usually occurs with age. • Direct immunofluorescence shows linear IgA at BMZ.

Differential Diagnosis In young infant, bullous impetigo may resemble the initial lesions, but its response to antibiotics differentiates it. Epidermolysis bullosa often present at birth and family history further differentiates it. Bullous papular urticaria rarely affects the face or genital region and it is usually of short duration. Childhood bullous pemphigoid may give us similar clinical picture but the deposition of IgG and C3 at the BMZ is diagnostic. Treatment is the same. Dermatitis herpetiformis only occurs in small children (usually occurs between 20 and 55 years of age) who are heterozygous for predisposing HLA genes. Treatment • Dapsone is usually effective (response usually occurs within 24-48 hours), and may be

Disorder

Site of split

Pathogenic antibody

P. vulgaris P. foliaceous/ erythematosus Paraneoplastic pemphigus BP / HG / CP EBA Dermatitis herpetiformis CBDC

Suprabasal Subcorneal

IgG IgG

Suprabasal

IgG

Subepidermal Subepidermal Subepidermal

IgG and C3 IgG IgA

Subepidermal

IgA

(Note: Epidermolysis bullosa are a group of genetic disorders with structural defects but NO autoantibodies.)

EPIDERMOLYSIS BULLOSA ACQUISITA (EBA) • It is defined as a chronic, acquired autoimmune blistering disorder characterized either by trauma-induced subepidermal blistering or with a clinical picture indistinguishable from bullous pemphigoid. • IgG class of autoantibodies directed against collagen VII causes it. Collagen VII is the major component of the anchoring fibrils found in the subepidermal zone; (differentiate from hereditary epidermolysis bullosa dystrophica in which there is a collagen VII structural defect leading to reduced or absent collagen VII in the anchoring fibrils). The five clinical presentations of EBA are: 1. Classical presentation– Noninflammatory tense bullae over trauma prone areas which heal with scarring and milia formation. Hemorrhagic bulla may be seen. It is a mechanobullous disease marked by skin fragility. 2. Bullous pemphigoid–Like presentation– widespread inflammatory vesiculobullous eruption over trunk, flexures and extremities.

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Essentials in Dermatology Tense bullae are present over inflamed or urticarial skin. Hemorrhagic bulla may be seen. Large areas of erythema and urticaria without blistering can be seen. Patient complains of pruritus and does not show prominent skin fragility, scarring or milia formation. The clinical picture is more like a bullous pemphigoid than a mechanobullous disorder. 3. Cicatricial pemphigoid-like presentation– is marked by widespread mucosal involvement. Clinical appearance is similar to cicatricial pemphigoid. Erosions and scars over mucosal surfaces of mouth, upper esophagus, conjunctiva, anus or vagina are present with or without similar lesions over glabrous skin. 4. Brunsting-Perry pemphigoid-like presentation– is a chronic recurrent vesiculobullous eruption localized to head and neck. It is characterized by bullae healing with scarring and has minimal to no mucosal involvement. 5. IgA bullous dermatosis-like eruption– is characterized by tense veskcles arranged in an annular pattern and mucous membrane involvement. It is also differentiated from other EBA types by its DIF findings of linear

•

•

•

•

deposition of IgA at basement membrane zone. Histology shows subepidermal cleft with neutrophilic infiltrate in the BP type while sparse inflammatory infiltrate in the mechanobullous type. Direct immunofluorescence (DIF) on perilesional skin shows thick polyclonal band of deposition of IgG and C3 and sometimes IgA and IgM in the BMZ. DIF on salt split skin shows deposits of IgG and C3 on dermal side whereas in bullous pemphigoid in 85% cases deposits are present on epidermal side and in 15% cases, it may be seen on both epidermal and dermal sides. EBA is a very difficult disease to treat. Steroids in combination with dapsone or adjuvant immunosuppressives (azathioprine, methotrexate, cyclophosphamide, cyclosporine, high doses of colchicine) are the usual line of therapy. Photopheresis and intravenous immunoglobulin have been used. The mechanobullous type is resistant to most modalities of treatment. Supportive therapy is warranted in all patients with EBA. This includes instructions in open wound care and strategies in avoiding trauma.

Cutaneous Tuberculosis and Atypical Mycobacterial Infections

13


Tuberculosis of the skin constitutes about 10% of all extra-pulmonary tuberculosis which in turn constitutes only a fraction of all cases of tuberculosis.

CLASSIFICATION OF CUTANEOUS TUBERCULOSIS (TB) Types

Mode of infection

Bacilli

Immunity

Tuberculin

Primary TB * TB chancre *Miliary TB

Inoculation Hematogenous

+++ ++

-

-

Secondary TB *Lupus vulgaris *TBVC *Scrofuloderma *TB gumma *Orificial TB

Inoculation Inoculation Contiguous Hematogenous Autoinoculation

+/+ ++ ++ ++

+++ +++ +++ + +

+++ +++ ++ + -

Tuberculids *Papulonecrotic *Lichen scrofulosorum *Erythema induratum

Hematogenous “ “

-

+++ +++ +++

+++ +++ +++

Cutaneous Tuberculosis can be Classified as Given Blow 1. Inoculation tuberculosis (exogenous source): a. Tuberculous chancre b. Tuberculosis verrucosa cutis c. Lupus vulgaris (some). 2. Secondary tuberculosis (endogenous source) a. Contiguous spread— scrofuloderma b. Autoinoculation— orificial tuberculosis.

3. Hematogenous tuberculosis— acute miliary tuberculosis, lupus vulgaris (some), tuberculous gumma. 4. Eruptive tuberculosis (tuberculids) a. Micropapular— lichen scrofulosorum b. Papular— papular or papulonecrotic tuberculids c. Nodular— erythema induratum of Bazin, nodular tuberculid.

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TUBERCULOUS CHANCRE

LUPUS VULGARIS

It occurs due to inoculation of M. tuberculosis into the skin of an individual without natural or acquired immunity to tubercle bacilli. • Usually seen in face or limbs of children • Presents as a brownish red papule or nodule that ulcerates to form a ragged ulcer with undermined edge • Associated with regional lymphadenopathy • If untreated, chancre heals slowly over months.

It is the most common type of progressive cutaneous tuberculosis occurring in people with moderate or high immunity. • Lupus vulgaris arises from normal skin but it can arise in a scar of scrofuloderma. • The initial lesion is a reddish-brown, soft, gelatinous plaque which increases in size and extends peripherally. • Peripheral extension occurs with resultant scarring (Figs 13.1 and 13.2). • Diascopy reveals ‘apple jelly’ nodules in the periphery. • The various morphological forms are plaque type, ulcerative and mutilating form, vegetative form and tumor like. • Nasal mucosa can be involved leading to resultant destruction of nasal septum. • Rarely squamous cell carcinoma can occur.

MILIARY TUBERCULOSIS Miliary TB occurs due to hematogenous dissemination of tuberculosis in infants and children or immunosuppressed • The skin lesions are varied— may manifest as crops of bluish papules, vesicles, pustules or hemorrhagic lesions • Diagnosis established by skin biopsy which shows acid fast bacilli • The primary source of TB should be identified and treated.

Fig. 13.1: Lupus vulgaris—plaque lesion with active infiltrated border with central scarring over the thigh

Differential Diagnosis • Discoid lupus erythematosus— Lesions begin as dull red macules or indurated plaques that develop an adherent scale (carpet tack sign), mainly over head and neck and evolve with atrophy, scarring and pigmentary changes.

Fig. 13.2: Lupus vulgaris—verrucous plaque with trailing scar and depigmentation in the groin

Cutaneous Tuberculosis and Atypical Mycobacterial Infections • Lupoid form of cutaneous leishmaniasis may be impossible to distinguish clinically. Histopathology distinguishes them. • Deep mycoses may resemble vegetating form of lupus vulgaris. It can be differentiated by histology and culture reports. • Leprosy— nodules of leprosy are firmer compared to those of lupus vulgaris. Other signs of leprosy would help in distinguishing it from lupus. • Sarcoidosis— nodules of sarcoidosis resemble grains of sand on palpation and the surface is shiny and waxy. • Psoriasis - usually multiple lesions over the extensor aspect of limbs and lumbosacral areas, but are less infiltrated compared to lupus vulgaris. They are associated with silvery white micaceous scales and there is no evidence of scarring. • Lichen simplex chronicus of perianal area may mimic lupus vulgaris. There will be no scarring and it will be itchy and hyperpigmented rather than reddish brown in lupus vulgaris.

TUBERCULOSIS VERRUCOSA CUTIS (WARTY TUBERCULOSIS, ANATOMIST’S WART) Tuberculosis verrucosa cutis (TBVC) manifests as a warty growth that occurs as a result of

Fig. 13.3: Tuberculosis verrucosa cutis—verrucous plaque with fissuring and foetid discharge over the sole

inoculation of mycobacteria into the skin of a previously infected individual with moderate to high immunity. • Lesions occur in exposed areas such as foot (Figs 13.3 and 13.4), hand, ankle and rarely buttock. • The lesions start as warty papules that enlarge to a verrucous plaque with serpiginous border.

Differential Diagnosis • TBVC should be differentiated from other warty lesions such as verruca vulgaris, lupus vulgaris (usually not hyperkeratotic, diascopy demonstrates apple jelly nodules), chromoblastomycosis, hypertrophic lichen planus (has multiple itchy lesions usually over lower legs with evidence of lichen planus elsewhere), leishmaniasis and tertiary syphilis. SCROFULODERMA Scrofuloderma results from breakdown and involvement of skin overlying a tuberculous focus such as lymph node, bone, joint, epididymis or lacrimal gland. • It manifests as bluish red nodules overlying lymph nodes or joints that break down to form undermined ulcers with a bluish edge (Figs 13.5 and 13.6).

Fig. 13.4: Tuberculosis verrucosa cutis—verrucous plaque with fissuring over the dorsum of great toe

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Fig. 13.5: Scrofuloderma—subcutaneous nodules and ulcerated lesions along with scarring in the neck

Fig. 13.7: Scrofuloderma—sinuses and fistulae healing with puckered scarring over the back

by multiple sinuses, puckered scarring and formation of new nodules that lead to an uneven indurated lumpy surface. Pus containing sulphur granules discharging from multiple sinuses is quite characterstic, distinguishing it from scrofuloderma.

TUBERCULOUS GUMMA (METASTATIC TUBERCULOUS ULCER)

Fig. 13.6: Scrofuloderma—ruptured bluish nodules with undermined edges in the axilla

• The resultant sinuses and fistulae heal with puckered scarring (Fig. 13.7).

Differential Diagnosis • Scrofuloderma should be differentiated from hidradenitis suppurativa, acne conglobata, syphilitic gumma and actinomycosis. Cervicofacial actinomycosis is characterized

It occurs due to hematogenous dissemination during periods of bacillemia and lowered resistance • Common in poorly nourished children • Presents as firm subcutaneous nodules that soften and ulcerate to form undermined ulcers.

ORIFICIAL TUBERCULOSIS Tuberculosis of mucosa or skin adjoining orifices in a patient with advanced internal tuberculosis is known as orificial TB (Fig. 13.8). • The affected patient is usually an adult of poor general health

Cutaneous Tuberculosis and Atypical Mycobacterial Infections • Lesions occur in the mouth, tongue, or genitalia as reddish nodules that break down to form painful shallow ulcers with bluish undermined edges.

TUBERCULIDS Tuberculids are hypersensitivity eruptions which arise in response to an internal focus of tuberculosis and clear with antituberculous therapy.

Papulonecrotic Tuberculid • It is an eruption of necrotizing papules, particularly affecting extremities and occurring in more or less symmetric crops. • The lesions are hard, dusky papules that crust or ulcerate to heal with atrophic scars (Fig. 13.9). • Papulopustular secondary syphilis, pityriasis lichenoides et varioliformis acuta, ChurgStrauss granuloma, lymphomatoid papulosis, perforating granuloma annulare, perforating collagenosis and necrotizing or septic vasculitis share clinical and histologic features with papulonecrotic tuberculid.

• The lesions are localized to legs (calf region) of middle aged women with erythrocyanotic circulation. • It must be distinguished from erythema nodosum (short duration, rapid development, affects chiefly anterior aspect of the leg, more painful tender nodules that do not ulcerate), nodular vasculitis, polyarteritis nodosa, tertiary syphilis (gumma is usually unilateral and single, serology and histology helps), and other infectious and inflammatory panniculitis.

Diagnosis Absolute Criteria • Positive culture for M. tuberculosis • Guinea pig inoculation • PCR for M. tuberculosis.

Others • • • • • •

Proven TB elsewhere in the body Presence of AFB in the lesion Histopathology Positive tuberculin test (Fig. 13.12) Clinical history and physical signs Response to therapy.

Lichen Scrofulosorum • It presents as grouped, closely set minute perifollicular papules over the trunk (Fig. 13.10) or extremities in children with tuberculous disease • Lichen nitidus, lichen planus, secondary syphilis and sarcoidosis should be considered in the differential diagnosis. Erythema induratum • Presents as persistent or recurrent erythematous tender nodular lesions (Fig, 13.11) (usually ulcerate in contrast to erythema nodosum) that occur secondary to a tuberculous focus elsewhere.

Fig. 13.8: Orificial tuberculosis—painful, shallow ulcers with undermined edges around the anus

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Fig. 13.9: Papulonecrotic tuberculid—atrophic scars over the thigh following papulonecrotic tuberculid

Fig. 13.10: Lichen scrofulosorum—micropapular eruption over the trunk

Fig. 13.11: Erythema induratum—erythematous indurated tender noduloplaque lesion over the leg

Fig. 13.12: Positive Mantoux reaction

Treatment Anti tubercular therapy with three or four drugs is given for a period of 6 to 9 months based on the type of tuberculosis. Most regimens contain isoniazid, rifampin, ethambutol and pyrazinamide.

ATYPICAL MYCOBACTERIAL INFECTIONS Atypical mycobacteria can present with varied cutaneous features in normal as well as immunosuppressed patients. The main features are summarized in the following table.


SUMMARY OF ATYPICAL MYCOBACTERIAL INFECTIONS Organism

Disease

Clinical Features

M.marinum

Swimming pool granuloma, fish tank granuloma

M.ulcerans

Buruli ulcer

Warty plaque or sporotrichoid lesions on knees, elbows and feet Subcutaneous nodules rupture to form shallow ulcers with necrotic fat in the floor Nodules, leg ulcers and papules Disseminated heterogenous infection in HIV patients Cellulitis and subcutaneous nodules Cutaneous abscesses, chronic ulcerative and nodular skin lesions, and cervical lymphadenitis

M.avium complex

M.chelonae Injection M.fortuitum abscess M. scrofulaceum Cutaneous abscesses

Treatment Rifampin and ethambutol or tetracycline Surgery is the treatment of choice followed by rifampin or cotrimoxazole Combined therapy of isoniazid, rifampin and streptomycin. Surgical debridement and amikacin or doxycycline or ciprofloxacin Treatment- surgical treatment of infected lymph node. Widespread disease- treatment same as in M avium complex

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Connective Tissue Disorders

The term “connective tissue disorders” (Collagen vascular disorders) encompasses a group of multi-system disorders which have certain features in common—the existence of autoimmunity in the form of antibody production or disordered cell mediated immunity, vascular abnormalities in the form of Raynaud’s phenomenon, occlusive vascular diseases or vasculitis, arthritis, arthralgia and skin disease. Involvement of skin is significantly predominant in this group of disorders. They are: systemic lupus erythematosus, systemic sclerosis, dermatomyositis, overlap-syndrome and mixed connective tissue disease.

LUPUS ERYTHEMATOSUS Lupus erythematosus (LE) is a multisystem disease of unknown origin characterized by the production of numerous diverse types of autoantibodies. Lupus erythematosus have wide spectrum of manifestations ranging from solitary chronic skin lesions in chronic discoid lupus erythematosus (DLE) to wide spread polymorphous lesions in subacute lupus erythematosus (SCLE) to multiple organ involvement in systemic lupus erythematosus (SLE).

DISCOID LUPUS ERYTHEMATOSUS (DLE) • Most common form of cutaneous lupus erythematosus. • Characterized by discrete, discoid, welldefined erythematous plaques covered with adherent scales (Figs 14.1 and 14.2). The lesions slowly expand with active inflammation at the periphery, leaving depressed scars, telangiectasias and permanent depigmentation (Fig. 14.3). • The central scarring with atrophy is very characteristic. • These lesions occur most often on the face, scalp, ears or neck. • Peeling the scale reveals an undersurface that looks like a carpet penetrated by several carpet tacks called “carpet tack sign”. • Scarring alopecia occurs in 60% of the cases (Fig. 14.4).

Types • Localized DLE: lesions occur only on the head or neck. • Generalized DLE: lesions are seen both above and below the neck (Figs 14.5 and 14.6).


Fig. 14.1: Discoid lupus erythematosus—well defined, discoid, erythematous plaques over the chest

Fig. 14.3: Discoid lupus erythematosus—discoid lesions evolving with central depigmentation and atrophy

Fig. 14.2: Discoid lupus erythematosus—discoid lesions involving the face

Fig. 14.4: Discoid lupus erythematosus—scarring alopecia

• Other types are hypertrophic, tumid, lupus erythematosus profundus, mucosal DLE, chilblain lupus, etc.

• The risk is higher in patients with disseminated DLE (22%).

Prognosis • DLE has a chronic progressive course. Patients with hematological and serological abnormalities have 6.5% risk of developing overt SLE.

Diagnosis • Based on clinical features and histopathology. Direct immunofluorescence (DIF)-deposits of IgG and C3 along the basement membrane in affected skin in up to 80%, but normal nonsun exposed skin always negative. Negative

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Essentials in Dermatology brown initially, later becoming red brown in color, have a characteristic translucence and woodgrains appearance, telangiectasia may be seen on the surface of the lesions, no prominent follicles), tinea faciei (KOH examination), granuloma faciale (brown color, no scarring), psoriasis (silvery scales), rosacea (pustules, ears spared) and Jessner’s lymphocytic infiltrate. In each case, histology is most helpful. The hypertrophic type of DLE may be confused with hypertrophic lichen planus, prurigo nodularis. LE profundus should be differentiated from other types of panniculitis. Mucosal DLE should be differentiated from mucosal lichen planus.

Fig. 14.5: Generalized discoid lupus erythematosus—discoid lesions involving the trunk

Fig. 14.6: Generalized discoid lupus erythematosus—discoid lesions involving the feet

or low titer ANA; sometimes higher titer in disseminated type. Exclude SLE.

Differential Diagnosis Lupus vulgaris (rarely symmetrical, has progression on one side and healing with scarring on the other side, apple-jelly nodules on diascopy), sarcoidosis (lesions are yellowish

Histopathology Characteristic histopathological changes are: • Hyperkeratosis with follicular plugging • Irregular atrophy of the stratum malphighi • Liquefactive degeneration of the basal cell layer and • Patchy perivascular and periappendageal lymphocytic infiltration. • Degenerative changes in the connective tissues—hyalinization, edema, fibrinoid change in the upper dermis. • Valuable clues are thickened periodic acidSchiff (PAS) positive basement membrane and deposition of mucin. Treatment • Photoprotection and topical sunscreens • Topical high potency or intralesional steroids • Topical calicineurin inhibitors (pimecrolimus, tacrolimus) • Systemic therapy for widespread recalcitrant disease are antimalarials (Hydroxychloroquine 200-400 mg daily or chloroquine 250 mg daily, monitoring by an ophthalmologist required every 6-12 months), dapsone (50-100 mg daily), thalidomide (50-200 mg daily), corticosteroids (prednisolone 40 mg

Connective Tissue Disorders daily short courses), oral auranofin, etretinate, isotretinoin, clofazimine, methotrexate, azathioprine, cyclophosphamide, etc.

SUBACUTE LUPUS ERYTHEMATOSUS (SCLE) • Characterized by scaly papules on the shoulders, extensor surfaces of the upper extremities, upper chest, upper back and neck in widespread and symmetric fashion. • Above lesions evolve into two morphological types either into papulosquamous or annular and polycyclic lesions. Rarely erythema multiforme-like with blisters (Rowell syndrome). • All patients with SCLE have mild systemic complaints and 50% of the patients fulfill the criteria for the diagnosis of SLE. • SCLE patients have antibodies to the cellular antigens Ro/SS-A and La/SS-B. • Children born to mother with SCLE may have congenital heart block (especially, those with anti Ro/SS-A antibody). • Differential diagnosis: To be differentiated from discoid lupus erythematosus, psoriasis, tinea corporis, annular erythemas, tinea versicolor and rarely erythema multiforme.

• It has been postulated that four or more genes are involved in predisposing an individual to SLE.

Pathogenesis LE is a multifactorial disease with genetic and immunopathologic abnormalities. The release of nuclear antigens because of enhanced apoptosis is a key factor. Important predisposing factors are genetic predisposition (HLA-B8, DR2, DR3, DQwl, DRB1), complement defects, exogenous factors (UV radiation, and medications), and individual factors (hormone status, altered immune status). The 1982 Revised Criteria for Diagnosis of SLE are: 1. Malar rash: Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds (Fig. 14.7). 2. Discoid rash: Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions.

Treatment Same as for discoid lupus erythematosus. SYSTEMIC LUPUS ERYTHEMATOSUS (SLE) SLE a multisystem disorder, primarily affects skin, joints and vascular system. The age of onset is usually between 16 and 42 years occurring more frequently in females (F: M:: 8:1).

Etiology • Exact cause is unknown but there is evidence to suggest the role of genetic, immune and various environmental factors.

Fig. 14.7: Systemic lupus erythematosus—malar and photosensitive rash over the face

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Essentials in Dermatology 3. Photosensitivity: Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation. 4. Oral ulcers: Oral or nasopharyngeal ulceration, usually painless, observed by a physician. 5. Arthritis: Nonerosive arthritis involving two or more peripheral joints, characterized by tenderness, swelling, or effusion. or 6. Serositis: a. Pleuritis—convincing history of pleuritic pain or rub heard by a physician or evidence of pleural effusion. b. Pericarditis—documented by ECG or rub or evidence of pericardial effusion. 7. Renal disorder: a. Persistent proteinuria > 0.5 g/day or greater than 3+ if quantitation not performed or b. Cellular casts—may be red cell, hemoglobin, granular, tubular, or mixed. 8. Neurologic disorder: a. Seizures—in the absence of offending drugs or known metabolic derangements, e.g. uremia, ketoacidosis, or electrolyte imbalance or b. Psychosis—in the absence of offending drugs or known metabolic derangements, e.g. uremia, ketoacidosis, or electrolyte imbalance. 9. Hematologic disorder: a. Hemolytic anemia—with reticulocytosis or b. Leukopenia < 4000/mL on two or more occasions or c. Lymphopenia < 1500/mL on two or more occasions or d. Thrombocytopenia—<100,000/mL in the absence of offending drugs. 10. Immunologic disorder: a. Anti-DNA—antibody to native DNA in abnormal titer or b. Anti-Sm—presence of antibody to Sm nuclear antigen or

c. Positive finding of antiphospholipid antibodies based on (1) an abnormal serum level of IgG or IgM anticardiolipin antibodies, (2) a positive test result for lupus anticoagulant using a standard method, or (3) a false-positive serologic test for syphilis known to be positive for at least 6 months and confirmed by Treponema pallidum immobilization or fluorescent treponemal antibody absorption test. 11. Antinuclear antibody (ANA): An abnormal titer of antinuclear antibody by immunofluorescence or an equivalent assay at any point in time and in the absence of drugs known to be associated with “drug-induced lupus” syndrome. Note: The proposed classification is based on 11 criteria. For the purpose of identifying patients in clinical studies, a person shall be said to have SLE if any 4 or more of the 11 criteria are present, serially or simultaneously, during any interval of observation.

Chief Cutaneous Features • Butterfly rash. • Photosensitivity. • Raynaud’s phenomenon. • Non-scarring alopecia-short hairs in the frontal region are referred to as lupus hairs. • Urticarial vasculitis. • Mouth ulceration. • Bullous lesions. • Chronic discoid LE lesion. • Cutaneous vasculitis. • Other features are vasculopathy, periungual telangiectasia, leg ulcers, erythema multiforme, thrombophlebitis, peripheral gangrene, etc. Investigations • Complete blood count, ESR. • Urine analysis for microscopic hematuria and proteinuria.

Connective Tissue Disorders • Skin biopsy- There is no single diagnostic pathological feature in the skin, but a combination of features aids diagnosis. Some changes are similar to DLE. The primary pathological lesions of SLE are fibrinoid necrosis, collagen sclerosis, necrosis and basophilic body formation and vascular endothelial thickening. • LE cell test to demonstrate LE cell (a neutrophil containing engulfed nuclear material) or rosette phenomenon (neutrophils surrounding nuclear debris, trying to engulf it). • Lupus band test- deposits of IgG and C3 along the basement membrane on normal, non-sun exposed skin suggest SLE but is no longer an accepted criterion. It positively correlates with the presence of anti-ds DNA antibodies and with risk for developing LE nephritis. • C3, C4, CH50 levels. • Serum globulins are frequently raised especially gamma globulin. • Rheumatoid factor occurs in approximate 40% cases. • ANA (Commonest pattern is homogeneous; peripheral pattern-predictor of renal involvement). • ndsDNA. • Antibodies-SSA (Ro), SSB (La), Sm, nRNP.

Differential Diagnosis SLE must be differentiated from dermatomyositis, erythema multiforme, polyarteritis nodosa, acute rheumatic fever, rheumatoid arthritis, pellagra, pemphigus erythematosus, drug eruption, hyperglobulinemic purpura, Sjogren’s syndrome, necrotizing angiitis and myasthenia gravis. In SLE, there may be fever, arthralgia, weakness, lassitude, diagnostic skin lesions, an increased ESR, cytopenias, proteinuria, immunoglobulin deposition at dermoepidermal junction and a positive ANA test. Biopsies of skin lesions and involved kidney may also be diagnostic.

Treatment SLE with only cutaneous lesions and arthritis: • Photoprotection. • NSAID’s, antimalarials, prednisolone ( 1-2 mg per kg body weight daily). • Dapsone most effective in urticarial vasculitis and bullous SLE. Severe Disease with End-organ Damage • Steroid pulse therapy. • Immunosuppressants such as azathioprine (100-150 mg daily), cyclophosphamide (50100 mg daily), methotrexate (7.5-20 mg weekly), chlorambucil, mycophenolate mofetil (2 gm daily) and cyclosporine (5 mg/kg body weight daily). Experimental Therapies • Intravenous immunoglobulins, plasmapheresis, anti CD20 (rituximab), -CD40 and – TNF alpha antibodies. Course and Prognosis The course of the SLE is very variable. Acute fulminating cases are much less common than subacute cases, which smoulder on for many years. SCLERODERMA Scleroderma (Gr. Skleros – hard, and derma – skin) is a connective tissue disorder characterized by generalized or localized sclerosis of the skin. The localized type is called morphea.

Classification Morphea – Localized (Circumscribed plaque, morphea profundus, bullous, linear, en coup de sabre) and generalized.

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Essentials in Dermatology Systemic sclerosis – LM (Limited cutaneous) SSc, DC (Diffuse cutaneous) SSc. Occupational scleroderma – Polyvinylchloride, perchlorethylene, trichloroethylene, organic solvents, malathion, DDT, epoxy resins, silicosis. Iatrogenic scleroderma – Bleomycin, carbidopa, pentazocine, cocaine, appetite suppressants, silicone or paraffin implants, GVHD. Pseudoscleroderma – Scleroedema of Buschke, Scleromyxoedema, prophyria cutanea tarda, phenylketonuria. Primary systemic amyloidosis, carcinoid syndrome, hypothyroidism. Miscellaneous – Toxic oil syndrome, eosinophilic fascitis.

MORPHEA Localized Morphea • Most common form of morphea. • Occurs most commonly in females than males and primarily in young adults. • Seen commonly on the trunk. • The lesion of morphea may begin as erythematous macule, evolve into ivory-

Fig. 14.8

colored center and violaceous bordered plaque. • Lesions slowly involute over 3 to 5 years period leaving permanent atrophic skin or normal appearing skin behind.

Generalized Morphea • Lesions are more numerous and larger • Often coalesce to involve extensive portions of the body. • Muscle atrophy may be associated. Pansclerotic Morphea (Morphea Profunda) • Sclerosis involves dermis, panniculus, fascia, muscle and bones. • There is disabling limitation of the joints. En Coup De Sabre • It is a variant of linear scleroderma involving scalp parasagitally on frontal scalp and forehead (Figs 14.8 and 14.9). • Often has the configuration of the stroke of a saber (en coup de sabre). Differential Diagnosis 1. Morphea-like lesions can occur in sarcoidosis and morpheic basal cell carcinoma.

Fig. 14.9

Figs 14.8 and 14.9: En coup de sabre—linear indurated depressed lesion in the midline over the forehead

Connective Tissue Disorders Histopathology is required to differentiate between them. 2. Lichen sclerosus atrophicus (ivory white plaques with follicular delling and atrophy) and subcutaneous zygomycosis may resemble morphea. Fingers can be insinuated below the plaques of subcutaneous zygomycosis. 3. Pseudoscleroderma especially porphyria cutanea tarda and graft versus host disease. 4. Drug reaction (bleomycin induced sclerosis, atrophic morphoeic plaques from intramuscular injection of vitamin K or subcutaneous corticosteroid injections).

Treatment • Natural history is towards spontaneous resolution. • Topical steroid, intralesional steroids and oral chloroquine. • Topical calcipotriol. • Bath PUVA or UVA1. • For widespread or rapidly advancing disease consider therapy usually reserved for systemic sclerosis (corticosteroids, d-penicillamine, cyclopsporine, low dose methotrexate, etretinate, phenytoin, plasmapheresis). • Physiotherapy may be helpful in preventing joint deformities.

• Endothelial cell injury. • Dysregulation of the immune system.

Classification • Diffuse disease: Characterized by extensive proximal and truncal skin induration. • Limited disease: Induration is confined to hands, forearm, face, and legs. Its variant is called CREST syndrome (ThibiergeWeissenbach syndrome) (Calcinosis, Raynaud’s phenomenon, Esophageal dysfunction, Sclerodactyly and Telangiectasia). American Rheumatism Association Criteria Major • Scleroderma proximal to the digits, affecting limbs, face, neck and trunk. Minor • Sclerodactyly (Fig. 14.10). • Digital pitted scarring (Fig. 14.11). • Bilateral basal pulmonary fibrosis. One major criterion or two or more minor criteria suggest the diagnosis of systemic sclerosis. Note: These criteria have 97% sensitivity and 98% specificity.

SYSTEMIC SCLEROSIS It is characterized by cutaneous and internal organ fibrosis. Raynaud’s phenomenon is the earliest feature and may precede the onset of disease by months or years. The heart, lungs, gastrointestinal, kidney and other organs may be involved.

Pathogenesis Exact cause of systemic sclerosis is unknown. Important steps in its pathogenesis include: • Excessive synthesis of collagen and matrix macromolecules.

Fig. 14.10: Progressive systemic sclerosis— sclerodactyly with calcification over the interphalangeal joints of fingers

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Cutaneous Manifestations Three phases of dermal involvement can be distinguished: 1. Edematous phase (stiff, puffy, fingers) 2. Indurative phase (hard, tight, hide bound) 3. Atrophic phase (softened skin, burnt out). Hands and Feet • Early: Raynaud’s phenomenon. • Swollen or tumid fingers and hands. • “Round finger pad sign”-fingers lose their normal peaked contour but rather appear as rounded hemisphere when viewed from the side. • Painful ulcerations at fingertips (Rat bite ulcer) with pitted scars. • Late: sclerodactyly (induration of skin over the fingers) with tapering of fingers (Figs 14.10 and 14.11). • Skin is tightly bound down. • Leathery crepitations over joints and flexion contractures. • “Heuck-Gottron sign”- loss of cuticle with telangiectases. • Bony resorption. • Atrophy of the pulp of the fingers. • Gangrene of the fingers. • Pigmentation. • Calcinosis.

Fig. 14.12: Progressive systemic sclerosis—mask like facies, thinning of lips and small sharp nose

Face • Periorbital edema is the early manifestation • Late manifestations include: Mask like facies (difficulty in eversion of lower eyelids), thinning of lips, microstomia, radial perioral furrowing, small sharp nose (Fig. 14.12), telangiectasia (mat-like) and diffuse hyperpigmentation. • Forehead is smooth and shiny, and skin is bound down and hard. • There is reduced wrinkling on the forehead and mandibular atrophy. Trunk

Fig. 14.11: Progressive systemic sclerosis—finger tip pits due to scarring

• Early: tense, stiff and waxy appearing skin that cannot be pinched and folded. • Late: impairment of respiratory movement of chest wall and of joint mobility. • “Neck sign”-ridging and tightening of the neck on extension due to sclerosis.


• •

• •

Fig. 14.13: Progressive systemic sclerosis—“salt and pepper pigmentation”—depigmentation with speckled hyperpigmented macules over the front of the chest

Other Changes “Salt and pepper” pigmentation (Fig. 14.13), gangrene of fingers, mat like telangiectasia, leg ulcers and livedo reticularis.

Organ Involvement in PSS • Esophageal fibrosis, pulmonary interstitial fibrosis, myocardial fibrosis, small intestinal fibrosis, large intestinal fibrosis, renal involvement, skeletal muscle atrophy and thyroid fibrosis. • Others are bone, eye, CNS, teeth, tendons. Investigations • Routine tests such as complete hemogram, liver function tests, sedimentation rate, C-reactive protein. • Skin biopsy- Dermal sclerosis typically results in rectangular punch biopsy specimen. As the dermis replaces the subcutaneous tissue, eccrine glands appear to be in the mid portion of the thickened dermis. The subcutaneous fat is quantitatively reduced and adventitial fat is lost. On DIF testing of skin the nucleolus may be stained in the keratinocytes if antinucleolar circulating antibodies are present and a “pepper dot” epidermal nuclear

pattern may be seen in CREST patients who have anticentromere antibodies in their serum. Nail fold capillary microscopy. ANA (+ve in more than 90%), Ab-SSA, SSB, Sm, nRNP, Scl-70 (specific for diffuse systemic sclerosis), anticentromere antibody (specific for CREST syndrome). Rheumatoid factor positive in 30% Organ workup: urine analysis, barium swallow, esophageal manometry, barium enema, chest x-ray and pulmonary function test.

Differential Diagnosis 1. Generalized morphea (Raynaud’s phenomenon is rare, systemic involvement is unusual, no atrophic stage, no facial telangiectasia or perioral furrowing, skin of trunk and limbs are equally involved) 2. Pseudosclerodermas (Specific features of each should be looked for) 3. Occupational and iatrogenic scleroderma (History of specific exposure should arouse the suspicion). 4. Other collagen vascular disorders- mixed collagen vascular disorders, overlap syndromes 5. Graft versus host disease Prognosis and Cause of Death • Course of the disease is variable. Death occurs from intercurrent infection, respiratory failure, cardiac failure, renal failure, sometimes, malignant hypertension and perforation of the gastrointestinal tract. Other Conditions Where Sclerodermoid Changes are Seen Phenylketonuria, progeria, Rothmund-Thomson syndrome, Werner’s syndrome, porphyria cutanea tarda, primary systemic amyloidosis, Hashimoto’s disease, carcinoid syndrome,

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Essentials in Dermatology childhood diabetes mellitus and drugs (bleomycin, pentazocine, carbidopa, and 5-hydroxytryptophan).

Treatment There is no specific treatment and no therapy is known to alter the course of a disease. Treatment is primarily directed towards complications. Pharmacological agents used for systemic sclerosis can be grouped into various categories. • Collagen modulators: D-penicillamine (125 mg alternate days, 750-1500 mg per day), relaxins, and interferons. • Vasoactive agents: Captopril, nifedipine and pentoxifylline. • Immunosuppressive agents: Systemic corticosteroids (prednisolone 0.5 mg mg per kg body weight daily), azathioprine (1-2 mg mg per kg body weight daily), cyclophosphamide (2 mg per kg body weight daily), cyclosporine (3-5 mg per kg body weight daily) and methotrexate (20-30 mg weekly). For internal organ involvement— Angiotensin converting enzyme inhibitors are treatment of choice for renal hypertension, proton pump inhibitors (omeprazole 20-40 mg daily) indicated for esophageal dysfunction and for pulmonary hypertension intravenous prostacyclin; interstitial lung disease may respond best to cyclophosphamide. Physical therapy can help avoid contractures and retain function.

Cutaneous Manifestations of Dermatomyositis Pathognomonic • Gottron’s papules: Violaceous, flat topped papules on interphalangeal joints and knuckles. Similar lesions may occur over other bony prominences such as knees, elbows and medial malleoli. • Gottron’s sign: Symmetric macular violaceous erythema with or without edema over the above mentioned sites.

Characteristic • Periorbital violaceous erythema with associated edema of eyelids and periorbital tissue (heliotrope rash) (Fig. 14.14). • Periungual telangiectasia with associated dystrophic cuticles. • Macular violaceous erythema overlying the dorsal hands, extensor forearms and arms, deltoids, posterior shoulders, nape of neck, V area of neck, upper chest and forehead. “Shawl sign”-erythema and scale (with or without poikiloderma) over the shoulder regions. • Mechanic’s hands: Bilaterally symmetrical confluent hyperkeratosis distributed along the

DERMATOMYOSITIS It is a systemic, inflammatory disease involving primarily skin and muscles. Symmetric, proximal muscle weakness occurs especially in the hips, thigh, and upper arm. Patients with only muscular symptoms and signs but no cutaneous findings are said to have polymyositis. These can be associated with malignancies especially in adulthood – carcinoma of lung, breast, female genital tract, stomach, kidney and testes.

Fig. 14.14: Dermatomyositis—“Heliotrope rash”— periorbital violaceous erythema with associated edema of eyelids

Connective Tissue Disorders ulnar aspects of the thumbs and radial aspects of the index and middle fingers with occasional extension to the palmar surface.

Compatible • Poikiloderma atrophicans vasculare • Calcinosis cutis. Systemic features include arthritis (25%), oral ulcers (20%), calcinosis (distinctive feature of juvenile dermatomyositis), pulmonary fibrosis (20%), gastrointestinal ulcerations and hemorrhages, occasionally myocarditis or myopathy.

Investigations • Skin biopsy, muscle biopsy, muscle enzymes, electromyography, ANA, Ab-SSA, SSB, Sm, nRNP, Jo-1and PM-1. Diagnostic Criteria 1. Progressive symmetric proximal muscle weakness. 2. Elevated muscle enzyme levels. 3. Abnormal electromyogram. 4. Abnormal muscle biopsy. 5. Characteristic cutaneous manifestations. Definite: 5 plus three other criteria. Probable: 5 plus two other criteria. Differential Diagnosis Includes other myopathies (inclusion body myositis, muscular dystrophy, neuromuscular atrophy, myasthenia gravis, thyrotoxic myopathy, Cushing’s disease, sarcoidosis, alcoholism), drugs (lipid lowering agents, hydroxyurea, NSAIDs), overlap syndromes, vasculitis, polymyalgia rheumatica and trichinosis. Treatment • Skin disease: Topical steroids, hydroxychloroquine. • Muscle disease: Systemic corticosteroids are first line therapy. Second line agents are

methotrexate (5-15 mg weekly) and azathioprine (1-3 mg per kg body weight daily). • Intravenous immunoglobulins are indicated for resistant cases, children with vasculitic component and those with steroid induced diabetes mellitus. • Bed rest during flares, physical therapy when stable. • Appropriate treatment of underlying malignancy where it is associated.

OVERLAP SYNDROME • The term overlap syndrome may be used when patients exhibit symptoms of more than one connective tissue disease. • Such patients may meet diagnostic criteria for one disease but also have atypical manifestations or findings suggestive of second diagnosis. • Systemic sclerosis combined with dermatomyositis is the most frequently seen overlap syndrome.

MIXED CONNECTIVE TISSUE DISEASE • This entity was first described by Sharp and colleagues in 1972. • These patients, predominantly female, show features of SLE, systemic sclerosis, dermatomyositis and polymyositis. • All patients have high titer of antibody to U1 RNP (ribonucleoprotein). • Prominent clinical features include myositis, pulmonary hypertension, Raynaud’s phenomenon, oesophageal hypomotility, swollen hands and sclerodactyly.

SJÖGREN’S SYNDROME (SS) • Sjögren in 1933 described a triad of keratoconjunctivitis sicca, xerostomia and rheumatoid arthritis.

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Essentials in Dermatology • Dry eyes and dry mouth occur in primary Sjögren’s syndrome or if associated with other connective tissue disease then referred to as secondary Sjögren’s syndrome. • Most patients are aged 50 years or older and are women. • Clinical features include xerostomia, rhinitis sicca, vaginal dryness and dry eyes (Fig. 14.15). • Skin manifestations of SS include vasculitis, xerosis and annular erythema. • Rheumatoid factor is usually positive. • 80% of patients have anti Ro/SSA antibodies. • Increased risk of developing lymphoreticular malignancies. • No specific treatments available, only symptomatic management.

eight” or “ hour glass” appearance (Fig. 14.16) • LSA of the penis in adults (Balanitis xerotica obliterans) presents with acquired phimosis or recurrent balanitis (Fig. 14.17). • LSA of vulva (Kraurosis vulvae) usually seen in postmenopausal women. • LSA of the genitalia have increased risk for squamous cell carcinoma (Fig. 14.18).

LICHEN SCLEROSUS ET ATROPHICUS (LSA) • It is a chronic disease of the skin and mucous membrane of unknown origin predominantly affecting the females. • Early lesions of LSA are ivory white, polygonal, flat-topped papules or plaques with follicular plugs (delling). The lesions may coalesce into large atrophic plaques. • In women, involvement of the vulvar and perianal areas leads to the typical “figure of

Fig. 14.15: Sjögren’s syndrome—face showing submandibular salivary gland enlargement

Fig. 14.16: Lichen sclerosus et atrophicus of the vulvatypical “figure of eight” or “hour glass” appearance

Fig. 14.17: Lichen sclerosus et atrophicus—ivory white indurated ring like lesion involvin preputial orifice


Fig. 14.18: Lichen sclerosus et atrophicus—male penis with squamous cell carcinoma

Differential Diagnosis On trunk- morphea, annular atrophic plaque type of DLE and vitiligo, female genitalia-vitiligo, chronic dermatitis, erosive lichen planus, autoimmune bullous diseases, male genitaliabalanitis in all its variants, idiopathic phimosis, erosive lichen planus.

Treatment Medical—Topical steroids or intralesional steroids, topical pimecrolimus or tacrolimus, topical testosterone, topical tretinoin, UVB and bath PUVA therapy. Surgical correction of adhesions may be required.

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Pigmentary Disorders

Human race has a wide variation in normal skin color (pale white, light brown, dark brown and black). Various skin chromophores like brown (melanin), red (oxyhemoglobin), blue (deoxyhemoglobin) and yellow-orange (carotene) are responsible for skin color. Of course, melanin is the major component of skin color. Skin color depends on the genetic make-up of an individual and environmental effects like ultraviolet radiation exposure. Melanocyte is the sole site of melanin synthesis. Pigmentary disturbances include decrease or absence or increase in pigmentation which could be either epidermal or dermal. These pigmentary changes are a result of changes in number of melanocytes or amount of melanin. Pigmentary disorders may be congenital or acquired, circumscribed or generalized, hypomelanotic (Vitiligo, albinism, piebaldism, Waardenburg’s syndrome) or hypermelanotic (Melasma, freckles, lentigo, Peutz Jeghers syndrome). In this chapter, only the common ones are discussed.

VITILIGO Vitiligo is defined as a common, dermatological disorder characterized by well-circumscribed, milky-white cutaneous macules devoid of

identifiable melanocytes. In India, the incidence of vitiligo is estimated to be between 3 and 4 %. The term vitiligo is probably derived from the Latin word “vitium”(“blemish”). Many ancient terms are applied to this condition— shwetakustha in the sacred Indian book, Atharva Veda (1400BC), and switra in Manusmriti (200 BC). In South India where the old Dravidian language of Tamil is spoken, the condition is known as ven kushtam—”white leprosy.”

Pathogenesis The white macules of vitiligo are the result of a loss of melanocytes. The mechanism(s) by which the melanocytes are lost may be multiple but have not been identified unequivocally. Seven hypotheses, not mutually exclusive, have been proposed to explain the causation of vitiligo: • Autoimmune: Strengthened by the demonstration of specific autoantibodies to melanocyte cell surface antigens and the association of vitiligo with a variety of autoimmune disorders. • Autocytotoxic: Also called the self-destruction theory, it proposes melanocyte destruction by intracellular retention of various precursors of melanin synthesis. • Neural: Especially proposed to explain the segmental type of vitiligo.

Pigmentary Disorders • Biochemical: Accumulation of pteridines (6biopterin and 7-biopterin) in the vitiliginous skin causes the depigmentation. • Antioxidant deficiency theory: There is an increased level of norepinephrine and catecholamine derivatives in vitiligo skin which leads to tissue ischemia and increased activity of monoamine oxidase enzyme, which in turn causes excess production of stress related hydrogen peroxide. Reduced catalase activity in vitiligo skin leads to impaired degradation of H2O2 and accumulation of superoxide radicals causing depigmentation. • Melanocyte growth factor reduction hypothesis: In vitiligo, there is reduced melanocyte growth factors derived from keratinocytes, fibroblasts and other tissues. • Intrinsic (genetic) theory: An underlying genetic/intrinsic factor predisposes some individuals to be more prone to develop vitiligo.

Clinical Features • The diagnostic lesion of vitiligo is the typical vitiligo macule, which is of variable size, round/oval in shape, has a milky white color and scalloped margins (Fig. 15.1). • May appear at any age, however, the peak age at onset has been reported to be five to thirty years. • Prevalence is the same in both sexes. • The natural course of the disease is of gradual progression, the lesions increasing both in number and size. In some cases there may be a rapid downhill course of vitiligo and this has been termed ‘galloping vitiligo’ or ‘vitiligo fulminans’. • Segmental vitiligo and vitiligo in children have a better prognosis. • Mucous membrane involvement is also noted in vitiligo and is commoner, or rather, easily detectable in dark-skinned races. • Leukotrichia refers to depigmentation of the hair and may occur in some patients.

Fig. 15.1: Zosteriform vitiligo—milky white macules in a zosteriform distribution over the back.

Classification of Vitiligo Localized • Focal vitiligo: This consists of one or more macules in one area but not clearly in a segmental or zosteriform distribution . • Segmental vitiligo: Number of macules involving a unilateral segment of the body. The lesions stop abruptly at the midline of the affected segment (Fig. 15.1). • Mucosal vitiligo: Vitiligo affecting mucous membranes of the lips, oral cavity or the genitalia (Fig. 15.2).

Fig. 15.2: Vulval vitiligo—female genitalia involved by milky white lesions

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Fig. 15.3: Acral vitiligo—vitiligo involving hands and feet

Fig. 15.5: Lip tip vitiligo-lesions affecting the tips of the digits and the lips

• Lip-tip vitiligo: Lesions affecting the tips of the digits and the lips (Fig. 15.5) • Mixed: Any combination- of vitiligo vulgaris and acrofacial vitiligo or of vitiligo vulgaris with segmental vitiligo. • Universal Vitiligo: This is the term used to describe complete or near complete depigmentation.

Fig. 15.4: Vitiligo vulgaris—multiple depigmented and hypopigmented macules over the chest and abdomen (Trichrome vitiligo)

Generalized • Acrofacial vitiligo: Lesions on the acral areas (hands and feet) (Fig. 15.3) and on the face, very often the perioral areas. • Vitiligo vulgaris: Multiple macules of variable sizes over widely scattered areas often tending to bilateral symmetry (Fig. 15.4).

Special Signs in Vitiligo • Trichrome vitiligo: The trichrome sign, also termed ‘vitiligo gradata’ describes a tan colored zone (intermediate colour) between the normal skin and the depigmented macules (Fig. 15.2). ‘Quadrichrome vitiligo’ implies the presence of a fourth color – dark brown – at the sites of perifollicular repigmentation. • Pentachrome vitiligo: Has five colors—white, tan, brown hyperpigmented, blue-grey hyperpigmented, and normal skin color. • Inflammatory vitiligo: Here skin lesions of vitiligo have erythematous raised margin. This should be differentiated from the erythema of vitiligo macule following exposure to sunlight. • Koebner’s sign: This phenomenon, a common feature of vitiligo, is defined as the development of lesions along the lines of specific trauma such as a cut, burn or abrasion. It may be a marker of disease activity.

Pigmentary Disorders

Association of Vitiligo with Other Diseases Vitiligo has been shown to be associated with autoimmune thyroid disease, Addison’s disease, pernicious anemia, diabetes mellitus and various dermatological disorders like alopecia areata, scleroderma, psoriasis and collagen vascular disorders. The Vogt Koyanagi Harada syndrome is an apparently rare, multisystem disease characterized by vitiligo, poliosis, uveitis, dysacousia, and alopecia.

Differential Diagnosis of Localized Vitiligo Includes Naevus depigmentosus (localized hypomelanosis present since birth), naevus anemicus (pale area due to vasoconsconstriction), leprosy (suspect in an endemic area, shows hypoanesthetic or anesthetic hypopigmented patch/ es), pityriasis alba (hypopigmented macular lesions have ill defined margins with fine scaling, self limiting course), postinflammatory hypomelanosis (such cases have preceding history of dermatoses, and the skin lesions are ill defined), tinea versicolor (typical localization to upper trunk, pigmented macular lesions have fine powdery scales, KOH +vity conclusive), ash-leaf macules and confetti depigmentation of tuberous sclerosis, and idiopathic guttate hypomelanosis (tiny porcelain white macules, 2-6 mm with distinct margins, localized to limbs in older individuals).

Differential Diagnosis of Generalized Vitiligo Includes Chemical leukoderma (there is history of exposure to phenolic germicides; confetti macules; localized to the site of contact), leprosy (suspect in an endemic area, has anesthetic hypopigmented patch/es), mycosis fungoides (unpatterned lesions, diagnosis confirmed by biopsy), postinflammatory hypomelanosis (hypopig-

mented macules with ill defined border, history of preceding dermatoses like psoriasis, eczema, pityriasis rosea, etc. in same areas with pattern mimicking it), tinea versicolor (has fine powdery scales over the lesions, in doubt do KOH examination of skin scrapings), Waardenburg’s syndrome, albinism and piebaldism (see next page for last three).

Treatment Treatment of vitiligo can be broadly divided into medical and surgical modalities. Medical management Topical therapy 1. Photoprotection: It prevents sunburn and Koebner phenomenon, prevents tanning of uninvolved skin and therefore lessens contrast between normal and depigmented skin. 2. Topical potent corticosteroids. 3. Intralesional corticosteroids: (Triamcinolone acetonide) especially for leukotrichia on scalp. 4. Human placental extract. 5. Topical immunomodulators: Such as tacrolimus (0.1%, 0.03%), pimecrolimus or tacrolimus (0.1%) combined with narrow band UVB three times a week. 6. Calcipotriol: Can be used as monotherapy or combined therapy (sunlight, PUVA, or narrow band UVB, clobetasol). 7. Topical pseudocatalase + calcium + UVB 8. Vitix®: Formulation containing superoxide dismutase and catalase. It removes hydrogen peroxide from skin, thereby helps in repigmentation. 9. Phenytoin local application: It inhibits release of norepinephrine and activity of monoamine oxidase, inhibits the production of superoxide anion and suppresses cytotoxic T- lymphocyte activity and induces type 2 like cytokine profile.

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Essentials in Dermatology 10. Dead sea climatotherapy in combination with pseudocatalase: Here patients take bath in dead sea for 15 minutes twice daily followed by a shower to wash off salt followed by application of pseudocatalase cream prior to sun exposure. 11. Topical prostaglandin analogues (PGE2). 12. Cosmetic camouflage where nothing works or when on treatment.

• Phenylalanine + UVA (PAUVA). Phenyalanine both topically and systemically can be used in combination with UVA. Phenyalanine can be used at a dose of 50-100 mg/ kg body weight 45 minutes prior to UVA exposure. • UVB radiation especially narrow band UVB • Targeted light therapy and Excimer laser (308 nm).

Systemic Therapy

Surgical Management Indications • Stable vitiligo (i.e. no new lesions for last two years) • Refractory to medical management.

1. Low dose oral corticosteroids-prednisolone 0.3 mg/kg body weight daily. 2. Oral dexamethasone or betamethasone pulse therapy- 0.5 mg/ every 5 kg body weight for two consecutive days in a week. 3. High dose methylprednisolone pulse therapy8 mg/kg/day IV over 30 minutes for three consecutive days every 4-8 weeks. 4. Multivitamin therapy (folic acid/vitamin B12/ vitamin C). 5. Antioxidants (β-carotene, α-tocopherol, methionine, ubiquinone, vitamin C). 6. Immunomodulators- Levamisole 150 mg on two consecutive days every week; cyclophosphamide 50 mg twice daily; cyclosporine 6 mg/kg/day; dapsone 100 mg/day; azathioprine. 7. Quinoline compounds- chloroquine 250 mg/ day and hydroxychloroquine 400 mg/day. They can be combined with psoralen therapy.

Phototherapy • Psoralen (stimulates melanogenesis in presence of ultra violet radiation)with ultraviolet A therapy (PUVA)- topical and systemic • Khellin + UVA (KUVA). Khellin is extracted from seeds of the plant Ammi visnaga. It can be given either topically or systemically. Khellin is given orally 50-100 mg/day, 2.5 hours prior to sun or UVA exposure up to 15 J/cm2. But due to systemic toxicity, topical preparation of khellin is recommended.

Modalities • • • • • • • •

Punch grafting Split skin thickness graft Blister grafting Melanocyte culture and transplantation Tattooing Therapeutic spot and regional dermabrasion Trypsinized autograft injection Topical 5- fluorouracil combined with epidermal abrasion.

For universal vitiligo options for depigmentation are: • Depigmentation with 20% monobenzyl ether of hydroquinone (for islands of residual repigmentation in extensive vitiligo). • Imatinib mesilate 400 mg/day for 15 days, then 300 mg once a day for 30 days has caused vitiligo-like depigmentation. It is a selective inhibitor of several tyrosine kinases.

ALBINISM • Autosomal recessive inherited disorder. • It is characterized by reduced melanin synthesis in the melanocytes of the skin, hair, and eyes, termed oculocutaneous albinism

Pigmentary Disorders (OCA), and hypopigmentation primarily involving the retinal pigment epithelium of the eyes termed ocular albinism (OA). • Due to genetic abnormalities of melanin synthesis associated with normal number and structure of melanocytes (differentiate it from vitiligo where melanocytes are reduced or absent). • Tyrosinase-related OCA, the most common type of albinism is produced by loss of function of the melanocytic enzyme tyrosinase resulting from mutations of the tyrosinase gene. • Affected individuals are born with white or blond hair and skin and blue eyes (Fig. 15.6).

PIEBALDISM • Piebaldism is an uncommon, autosomal dominant, congenital, stable leukoderma characterized by a white forelock and vitiligolike amelanotic macules, usually containing a few normally pigmented or hyperpigmented macules (Fig. 15.7).

Fig. 15.7: Piebaldism—typical white forelock

• Hyperpigmented macules within the amelanotic macules and on normally pigmented skin are characteristic of piebaldism.

WAARDENBURG’S SYNDROME WS (Waardenburg syndrome–Hirschsprung’s disease or Shab-Waardenburg syndrome) is a rare autosomal dominant disorder that is characterized by: • Lateral displacement of the inner canthi and of lacrimal puncta • Prominence of the nasal root and of the medial eyebrows • Congenital deafness • Heterochromic irides • White forelock • Hypomelanotic macules.

MELASMA (CHLOASMA)

Fig. 15.6: Albinism—Blond hair and white skin with actinic keratosis.

• Melasma is a common acquired hypermelanosis that occurs exclusively in sunexposed areas; it is exacerbated by sun exposure, pregnancy, oral contraceptives, and certain anti-epilepsy drugs. • Melasma presents in one of three usually symmetric facial patterns. The most common

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Fig. 15.8: Melasma—hyperpigmented light brown macules over the cheeks

is a centrofacial pattern involving the cheeks (Fig. 15.8), forehead, upper lip, nose, and chin. Less common are the malar pattern, involving the cheeks and nose, and the mandibular pattern. • Successful treatment of melasma involves the triad of sunblocks, bleach and time.

FRECKLE (EPHELIDES) It is an area of pale brown pigmentation usually less than 3 mm with poorly defined lateral margins • Freckle appears as result of functionally overactive melanocytes (though they are normal in number ) • They are seen only in fair skinned people • They are stimulated by ultra violet radiation and fade away during winter • Histology reveals excess of melanin pigment in the basal layer.

LENTIGO (PLURAL: LENTIGINES) It is a sharply demarcated brown pigmented macule usually circular or polycyclic in shape. • Lentigo appears as a result of increased number of melanocytes in the basal layer • They do not show seasonal colour variations ( not affected by ultra violet radiation). • Histology reveals linear increase of melanocytes in the basal layer

Syndromes Associated with Lentigines • LEOPARD syndrome: Lentigines (multiple), ECG abnormalities, Ocular abnormalities (hypertelorism), Pulmonary stenosis, Abnormalities of genitalia, Retardation of growth and Deafness (sensorineural). • NAME syndrome: Nevi, Atrial myxoma, Myxoid neurofibromas and Ephelides. • LAMB syndrome: Lentigines, Atrial myxoma, Mucocutaneous myxomas and Blue nevi. PEUTZ JEGHERS SYNDROME • Peutz-Jeghers syndrome is an autosomal dominant disorder characterized by pigmented macules (lentigines) of the buccal mucosa, lips, fingers, and toes and by gastrointestinal polyps. • It is caused by mutations in a novel serine threonine kinase, and its gene has recently been mapped to chromosome 19p. • The pigmented macules (dark brown or bluebrown) are most common on the buccal mucosa and lips, but also seen over palate and tongue. The macules on the skin are usually found on the face (around the mouth and eyes), dorsa of the hands and feet and periumbilically. • The diagnosis is particularly important because of the presence of gastrointestinal polyps, which are most frequent in the small bowel, particularly the jejunum, which may manifest with gastrointestinal bleeding. Malignant change may occur in these polyps.

Pigmentary Disorders • Any child with recurrent, unexplained abdominal pain should be examined for the typical mucosal, and periorificial pigmented lesions of Peutz-Jeghers syndrome.

DRUGS CAUSING HYPERPIGMENTATION • Generalized–Addisonian-like occurs with ACTH and generalized diffuse due to clofazimine, cyclophosphamide, minocycline, etc.

• Localized: – Melasma- like- estrogen, progesterone, phenytoin – Knuckle pigmentation- bleomycin – Palmoplantar- cyclophosphamide, doxorubicin – Linear – bleomycin (flagellate), zidovudine.

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Keratinization Disorders

ICHTHYOSIS Ichthyosis (from Greek ichthys “fish”) denotes a group of hereditary and acquired disorders of keratinization characterized by the development of dry rectangular scales.

Hereditary (Inherited) Ichthyoses Ichthyosis Vulgaris It is a common autosomal dominant disorder characterized by mild scaling on extensor aspect of the limbs (Fig. 16.1), more than trunk and spares the flexures. Increased palmar markings or frank palmoplantar keratoderma may be associated features. Onset is after 3 months of age. Scaling gets worse in winter.

side of the face and trunk are affected. This form of ichthyosis is associated with steroid sulphatase deficiency. Differential diagnosis: In contrast to ichthyosis vulgaris, no hyperlinear palms, no keratosis pilaris, flexural involvement and larger, darker scales.

Differential diagnosis: Atopic xerosis, eczema craquele, acquired ichthyosis, and Refsum’s disease.

X-linked Recessive Ichthyosis It appears in infancy and occurs in males. Females may be heterozygotes and female carriers are either totally spared or only mildly affected. Large dirty brown scales characterize it. Extensor and flexor aspects of the limbs are involved but spare rhomboidal spaces in body folds (Figs. 16.2 and 16.3). Palms and soles are spared but the neck,

Fig. 16.1: Ichthyosis vulgaris—scaling limited to extensor aspects of limbs (shin)


Fig. 16.2

Fig. 16.3

Figs 16.2 and 16.3: X-linked recessive ichthyosis—large dirty brown scaling involving flexor and extensor aspects of limbs and trunk but sparing rhomboidal spaces in body folds

Fig. 16.4: Lamellar ichthyosis—face involvement by erythema and scaling

Fig. 16.5: Lamellar ichthyosis—brownish plate like scales over the trunk

Lamellar Ichthyosis

the body including flexures. Facial involvement often results in ectropion and eclabium (Figs 16.4, to 16.6).

Lamellar ichthyosis is a rare form of ichthyosis, which presents at birth as “Collodion baby”; baby is encased in a taut inelastic membrane. In this autosomal recessive disorder, the baby later develops large, thick plate like scales all over

Differential diagnosis: Includes X-linked recessive ichthyosis and ichthyosiform eythrodermas with collodion presentation.

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Fig. 16.6: Lamellar ichthyosis—large plate like scales involving lower legs

Congenital Ichthyosiform Erythroderma It is of two types: 1. Bullous congenital ichthyosiform erythroderma: It is inherited as an autosomal dominant trait. At birth, the skin is erythematous, moist and tender. Blisters develop at or shortly after birth and later, thick dark gray brown scales form warty-ridged pattern (Figs 16.7 and 16.8). There is accentuation in the areas of flexures and on the palms and soles (Fig. 16.9). Normal appearing skin within a hyperkeratotic area is a valuable diagnostic sign. Skin colonization by Staphylococcus brevibacterium and possibly fungi produces a distinctive and embarrassing body odor. Ectropion and deformed ears are common. Differential diagnosis: At birth, may be confused with epidermolysis bullosa, staphylococcal scalded skin syndrome, herpetic infections and incontinentia pigmenti. In older patients, ichthyosis hystrix, ichthyosis bullosa of Siemens, and nonepidermolytic epidermal naevi may resemble bullous congenital ichthyosiform erythroderma. 2. Nonbullous congenital ichthyosiform erythroderma: It is an autosomal recessive disorder. Most infants with it are born as

Fig. 16.7

Fig. 16.8 Figs 16.7 and 16.8: Bullous congenital ichthyosiform erythroderma—no more blisters seen, body folds show thick dark gray brown scales forming warty, ridged pattern

Fig. 16.9: Bullous congenital ichthyosiform erythroderma—palms involved by keratoderma

Keratinization Disorders collodion baby. As the membrane is cast off in 10 to 14 days, generalized erythema and scaling are apparent. Scales may be large and plate like on the legs but are apt to be fine on the trunk, face and scalp. It has a tendency to improve at the time of puberty. Ectropion, deformities of the ears and sparsity of the scalp hair are common accompaniments. Differential diagnosis: Congenital infections such as candidiasis, congenital psoriasis, Netherton’s syndrome, immunodeficiency disorders, trichothiodystrophy and neutral lipid storage disease may mimic the cutaneous signs of non-bullous ichthyosiform erythroderma. • A collodion baby is the usual presentation of congenital recessive ichthyosis. Kollodes is the Greek word for glutinous or glue like. The child is born encased in a transparent, parchment-like membrane, which is taut and may impair respiration and sucking. Collodion presentation can develop into a wide spectrum of ichthyosis phenotypes as the child grows. Differential diagnosis: Harlequin fetus can be easily differentiated from collodion baby. Restrictive dermopathy or “Stiff baby syndrome” produces a generalized taut thick, tethered and unyielding skin at birth which does not desiccate in the neonatal period. Infective causes of desquamation such as staphylococcal scalded skin syndrome should be included in differential diagnosis. • Harlequin ichthyosis (fetus) is a dramatic, severe, and usually fatal presentation of ichthyosis. The child is often premature and born with massive, shiny plates of stratum corneum separated by deep, red fissures that tend to form geometric patterns. There are poorly developed or absent ears and marked ectropion and eclabium. These children are at great risk during the neonatal period and often die shortly after birth.

Differential diagnosis: As given for collodion baby. A variant of infantile systemic hyalinosis and the stiff skin syndrome (congenital fascial dystrophy) also present with tight skin.

Ichthyosiform Syndromes 1. Refsum’s syndrome (Heredopathia atactica polyneuritiformis) • Refsum’s syndrome is a rare autosomal recessive metabolic disorder in which there are characteristic neurological and cutaneous clinical features: • The underlying abnormality is deficiency of enzyme phytanic acid oxidase. • As a consequence of this deficiency, phytanic acid (found in green vegetables) accumulates and displaces some of the unsaturated fatty acids, such as linolenic acid, from the lipids through out the tissues. • It manifests usually in the second decade. • Skin is affected by an ichthyosis very similar to ichthyosis vulgaris. • Neurological changes include a cerebellar degenerative disorder (cerebellar ataxia), a progressive polyneuropathy, retinitis pigmentosa, and a sensory deafness. Rarely, cardiac abnormalities have been described. • Diagnosis—Histopathology of the skin shows some of the epidermal cells containing lipid vacuoles. No or very little phytanic acid is present in the blood. • Treatment by a phytanic acid free diet, in which green vegetables and dairy products are excluded, has been used. 2. Sjögren’s-Larsson syndrome: • This uncommon neuroectodermal genodermatosis appears to be inherited as an autosomal recessive disorder. • The underlying metabolic defect is deficiency of enzyme fatty alcohol: nicotinamide-adenine dinucleotide oxidoreductase.

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Essentials in Dermatology • The skin disorder becomes evident after the first few months of life with scaling over the body and hyperkeratosis of palms and soles. • The neurological component usually starts at 2-3 years of life and remains static after puberty, consists of a spastic diplegia (Fig. 16.10), or occasionally a tetraplegia with mental retardation. Other features are seizures and degeneration of retina. • Diagnosis—Histopathological changes are similar to lamellar ichthyosis. • Treatment—Diet lacking natural fat and containing medium chain triglycerides has been beneficial. 3. KID syndrome: • The acronym KID—Keratitis, Ichthyosis, and Deafness describes the salient clinical features of this syndrome. 4. Netherton’s syndrome: • It is a rare autosomal recessive disorder characterized by concurrence of ichthyosis linearis circumflexa, trichorrhexis invaginata (bamboo hair) and atopic dermatitis.

5. CHILD syndrome: • The acronym CHILD describes a very rare disorder comprising Congenital Hemidysplasia with Ichthyosiform erythroderma and unilateral Limb Defects mainly skeletal hypoplasia. 6. IBIDS syndrome (Tay’s syndrome): • The acronym IBIDS describes Ichthyosis, Brittle hairs, Impaired intelligence, Decreased fertility and short Stature.

Acquired Ichthyoses The distinction between dry skin (xerosis) from environmental causes and acquired ichthyoses is sometimes difficult. The sudden onset of generalized pronounced ichthyoses in an adult could be due to: 1. Lymphomas especially Hodgkin’s lymphoma 2. Internal malignancy 3. Malabsorption syndromes and malnutrition 4. Certain drugs like clofazimine 5. Hypothyroidism 6. Lepromatous leprosy 7. HIV disease. Treatment • Emollients for scaly skin especially after bath • Salicylic acid, urea, lactic acid in ointment form or propylene glycol-glycerine –lactic acid mixture for topical application • Oral retinoids – Etretinate or Acitretin. They are really useful in lamellar and congenital ichthyosiform erythrodermas.

DARIER’S DISEASE

Fig. 16.10: Sjögren's-Larsson syndrome—child having spastic diplegia with ichthyosis

• It is disorder of keratinization with an autosomal dominant mode of inheritance (chromosome 12). • Onset is during puberty when dirty, warty, greasy papules appear in the seborrheic distribution (Figs 16.11 to 16.13) • In due course of time, the lesions grow in size and form malodorous, papillomatous and vegetative growths


Fig. 16.11

Fig. 16.12

Figs 16.11 and 16.12: Darier's disease—dirty, warty greasy papules in seborrheic distribution over the trunk

Fig. 16.13: Darier's disease—dirty, warty greasy papules in seborrheic distribution over the scalp and face

• Other features include palmar pits (Fig. 16.14) and ‘V’ shaped nicking of nails. • Oral cavity shows cobble stone appearance of the mucosa (Fig. 16.15). • Acral areas may demonstrate dome shaped papular lesions known as acrokeratosis verruciformis of Hopf (Figs 16.16 and 16.17) • Exacerbation occurs following sun exposure • Histopathology shows dyskeratosis (corps ronds and grains) and acantholysis.

Differential diagnosis: Well developed cases distinctive; early lesions confused with other follicular keratoses. Same histologic picture can be seen in Grover’s disease as well as epidermal naevi and acquired acanthomas, so clinico pathologic correlation required. Treatment: Acitretin 25-50 mg daily is probably the best treatment; should be used until disease brought under control and then stopped if sideeffects develop. Topical retinoids and keratolytics

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Fig. 16.14: Darier's disease—palmar keratoses with pits

Fig. 16.15: Darier's disease—cobblestone appearance of hard palate mucosa

Fig. 16.16:

Fig. 16.17

Figs 16.16 and 16.17: Darier's disease—acrokeratosis verruciformis of Hopf lesions over the dorsa of hands

are disappointing at best. Some patients improve dramatically with antibiotics. Avoid sun exposure and use sun screens.

POROKERATOSIS • It is a chronic progressive disease which occurs due to abnormal proliferation of a clone of keratinocytes.

• Porokeratosis of Mibelli is the most common prototype of porokeratosis • The lesions are slightly atrophic plaques surrounded by elevated keratotic wall with a furrow (seen sometimes) (Figs 16.18 and 16.19) • Various types of porokeratosis are porokeratosis of Mibelli, disseminated superficial actinic porokeratosis, palmo-

Keratinization Disorders plantar porokeratosis, porokeratosis palmoplantaris et disseminata and linear porokeratosis (Fig. 16.20). • Histological hallmark is the column of parakeratosis known as ‘cornoid lamella’. Differential diagnosis: Multiple lesions can be mistaken for psoriasis, lupus erythematosus, pityriasis rubra pilaris or verrucous lichen planus. Solitary lesions often misinterpreted as tinea, warts, or actinic keratoses. Palmoplantar lesions are hard to diagnose clinically; one can consider

Fig. 16.18: Porokeratosis of Mibelli—an atrophic plaque surrounded by elevated keratotic wall with a furrow

Fig. 16.19: Porokeratosis of Mibelli—ridge made prominent by gentian violet paint

punctate palmoplantar keratoderma, arsenical keratoses and warts; only biopsy provides the answer. Treatment: Cryotherapy, CO2 and pulsed dye laser therapy or other destructive methods can be used for limited lesions. Porokeratosis is usually resistant to treatment but in some cases retinoids (acetretin) or PUVA have been used with success.

PALMOPLANTAR KERATODERMA (PPK) Palmoplantar keratoderma means thickening of palms and soles. They can inherited as well as acquired. Inherited PPK can be classified based on the presence or absence of transgradiens (spreading of keratoderma on to the extensor surface). • Transgradiens is seen in Mal de Meleda, Vohwinkel’s, Greither’s and Papillon-Lefevre syndrome. • Transgradiens is not seen in Thost-Unna (most common diffuse palmoplantar keratoderma), Vorner (diffuse PPK with epidermolytic hyperkeratosis) and focal types. • The important features of various inherited PPK are given in the following table.

Fig. 16.20: Linear porokeratosis— involving the arm

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Type of keratoderma

Important features

Thost –Unna (AD) Vorner type (AD)

Diffuse PPK with livid red border (Fig. 16.21) Marked hyperhidrosis Diffuse PPK similar to Thost –Unna type.Histology shows epidermolytic hyperkeratosis Honey combed palmoplantar thickening with constriction bands and mutilation, star fish shaped keratosis on dorsa of hands and fingers (Fig. 16.22) Palmoplantar keratoderma in a glove like distribution. Erythema is prominent with hyperhidrosis and malodor Diffuse PPK that is progressive. Extensor surfaces of hands, knees and elbows shows psoriasiform plaque (Fig. 16.23) Congenital PPK with perioral hyperkeratosis. Spontaneous amputation can occur PPK associated with periodontosis and increased frequency of pyogenic infection

Vohwinkel’s type (AD) Mal de Meleda (AR) Greither (AD) Olmsted syndrome (S) Papillon-Lefevre (AR)

Note: AD—Autosomal dominant, AR—Autosomal recessive, S—sporadic occurrence

Fig. 16.21: Palmoplantar keratoderma—diffuse keratoderma localized to palms

Fig. 16.23: Palmoplantar keratoderma—extending onto dorsa of hands and feet (transgradien palmoplantar keratoderma)

• Other types of palmoplantar keratodermas are focal kertodermas, striate keratodermas and puntate keratodermas (Fig. 16.24). • Various causes of acquired keratoderma are pityriasis rubra pilaris, malignancy (Tylosis), myxedema, Darier’s disease, keratoderma climactericum, psoriasis, lichen planus, and tinea pedis. Fig. 16.22: Palmoplantar keratoderma—leading on to mutilation

Treatment: Topical keratolytics, vitamin D3 analogues and mechanical debridement useful. Some cases respond to oral isotretinoin.


Fig. 16.24: Punctate keratoderma—punctuate keratotic lesions involving palmar creases

ACANTHOSIS NIGRICANS Acanthosis nigricans is a nonspecific reaction pattern involving major body folds and mucocutaneous regions characterized by hyperpigmented, velvety, soft, verrucous lesions in a symmetric fashion (Fig. 16.25). • It is broadly divided into benign and malignant form. • Benign acanthosis nigricans involves limited body areas and is less severe than malignant forms. • The various benign forms that are the most common include benign familial acanthosis nigricans, acanthosis nigricans associated with various syndromes, endocrine disease (especially insulin resistance diabetes mellitus, HAIR-AN syndrome [Hyper Androgenism, Insulin Resistance, and Acanthosis nigricans]), obesity (pseudoacanthosis Nigricans) and drugs (Nicotinic acidic, fusidic acid, stilbestrol, oral contraceptives, triazinate).

Fig. 16.25: Acanthosis nigricans—hyperpigmented, velvety, soft, verrucous involvement of neck

• Malignant acanthosis nigricans is associated with extensive, widespread lesions and mucosal involvement. They may precede, follow or occur simultaneously with onset of malignancy. Thickening of palms, especially fingertips produces accentuated dermatoglyphics with deep sulci called as Tripe palms. • Gastric carcinoma is the most commonly associated tumor; other sites include the bronchus, pancreas, ovary, bile duct, gallbladder, endometrium, breast and thyroid. Differential diagnosis: Confluent and reticulated papillomatosis, tinea versicolor, X-linked ichthyosis, retention hyperkeratosis, dirty neck of atopic dermatitis. Treatment: As it is a reaction pattern to some of the underlying conditions, treatment need to be directed at them. Topical retinoids may bring modest improvement.

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Urticaria, Angioedema and Pruritus

URTICARIA AND ANGIOEDEMA Urticaria Is a dermal vascular reaction of the skin characterized by the appearance of itchy wheals, which are elevated (edematous), pale or erythematous, transient and evanescent plaque lesions. These lesions usually do not last beyond 24 hours. They change in size and shape by

Fig. 17.1

peripheral extension or regression during few hours (Figs 17.1 and 17.2). Wheals itch. The intensity of itch varies.

Angioedema (Quincke’s Edema) Is also a vascular reaction, which involves subcutaneous or submucosal tissues (rather than dermis in urticaria) so skin overlying the swelling

Fig. 17.2

Figs 17.1 and 17.2: Urticaria—showing itchy erythematous wheals of various shapes and sizes


Fig. 17.3: Angioedema—swelling of chin and lip of sudden onset

is normal in color and margins of swelling are indistinct (diffuse swelling). It commonly results in asymptomatic swelling of the lips (Fig. 17.3), eyelids, etc. Due to laryngeal involvement, patient may develop respiratory distress. Gastrointestinal involvement may cause vomiting and abdominal pain. Angioedema may be classified into hereditary angioedema [Type I (85%)-C1 esterase inhibitor absent, Type II (15%)-C1 esterase inhibitor not functional] and acquired angioedema (Acute angioedema due to allergic IgE mediated, e.g. due to drugs, food, insects, contact dyes, serum sickness, cold urticaria or Chronic recurrent angioedema-(Idiopathic in most cases; rarely due to acquired C1 esterase inhibitor deficiency). Urticaria may be acute or chronic

Acute Urticaria Means urticaria of less than 6 to 8 weeks duration. Common causes are drugs especially penicillin, foods, especially shell fish, and rarely infections. In India, insect bites are another important cause. No apparent cause can be found in at least half of acute attacks. Even if a cause can be identified and withdrawn, it is often several days before the urticaria subsides.

Chronic Urticaria If urticaria lasts for more than 6 to 8 weeks duration, it is called chronic urticaria. Culprit in the great majority of cases remains obscure. Up to 55% of patients with chronic idiopathic urticaria possess functional IgG antibodies directed against the high affinity IgE receptors or less commonly against IgE itself. These autoantibodies release histamine from human skin mast cells and blood basophils and appear to be the cause of the disease (autoimmune urticaria). Immunoglobulin and complement components may be deposited perivascularly, and can be visualized by direct immunofluorescence examination. Basic pathogenic mechanisms of urticaria are immune and nonimmune mechanisms. Immune mechanism consists of type I hypersensitivity mechanism (IgE mediated), through complement activation, immune complex mechanism, or autoimmune process. Nonimmune mechanism works through direct release of histamine and various mediators from mast cells, vasoactive stimuli, aspirin, dietary pseudoallergens and due to angiotensin converting enzyme inhibitors. Etiological Factors(6 I’s + others ) 1. Ingestants a. Foods – cheese, eggs, nuts, fish, mushrooms, etc. b. Food additives – tartarzine dyes, etc. c. Food preservatives d. Drugs – penicillin, salicylates, sulphonamides, etc. 2. Injectants a. Insect bites b. Injection – drug, sera, blood, etc. 3. Inhalants • Pollens, animal dander, etc. 4. Infestation by parasites • Amoebiasis • Giardiasis

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Fig. 17.4: Dermographism–stroking the skin with blunt metallic instrument resulted in an exaggerated triple response forming wheals along them

5. 6. 7.

8.

• Hookworm • Round worm. Infection –focus in teeth, tonsils, sinuses or elsewhere. Implants—prosthetic and hormonal Physical causes/factors (Urticarial attacks are brief lasting for 30 to 60 minutes): a) Dermographism (write-on-skin) – stroking the skin with blunt metallic instrument results in an exaggerated triple response (Fig. 17.4). b) Pressure urticaria–pressure c) Cholinergic urticaria–increase in core body temperature (manifest as small intensely pruritic papules) d) Cold urticaria–cold air or water (Fig. 17.5) e) Heat urticaria–heated object f) Solar urticaria–sun exposure g) Exercise induced–exercise h) Aquagenic pruritus/urticaria–contact with water i) Vibratory urticaria–handling vibratory instruments. Contact urticaria – Contact urticaria refers to a wheal and flare reaction following external contact with a substance, e.g. on coming in

Fig. 17.5: Cold urticaria—ice cube test producing urticaria

contact with potato, onion, nitrogen mustard, etc. develops urticaria at site of contact. It usually appears within 30 minutes and clears completely within hours without residual signs of irritation. 9. Rule out underlying collagen vascular disorders, malignancy or any psychogenic cause. 10. Chronic idiopathic urticaria (CIU), defined as the occurrence of daily, or almost daily, wheals and itching for at least 6 weeks, with no obvious cause. Idiopathic – no cause found.

Fig. 17.6: Proceeding in a case of urticaria


Diagnosis How to proceed in a case of urticaria? 1. Urticaria due to physical causes or drugs excluded by history and examination (Fig. 17.6). 2. Complete food elimination followed by gradual introduction of one dietary element at a time helps in detection of food induced urticaria. 3. Mask use/nasal filter use/change of place may work for inhalants. 4. Stool examination by concentration method on 3 consecutive days – infestations are detected and treated accordingly. 5. Look for a focus of infection. If not possible to detect, give a course of antibiotics. Still no response, change the antibiotic. 6. The major advance in our understanding of chronic idiopathic urticaria (CIU) in recent years has been the discovery that in 30-50% of patients with so labeled chronic idiopathic urticaria, the disease is due to an autoimmune process, and therefore is not strictly ‘idiopathic’. 7. The autologous serum skin test is a useful screening test for autoimmune chronic urticaria (AICU). In this test, 0.05 ml of the patient’s serum, removed during a period of disease activity, is injected intradermally into the same patient’s uninvolved forearm skin, along with equal volumes of saline and histamine (10 µg/ml) at adjacent sites. The test is read 30 min later. A positive result is recorded if the diameter of the wheal at the serum-injected site is 1.5 mm greater than that of the bleb at the saline-injected site. The sensitivity and specificity of the test are 65-81% and 71-78%, respectively. Patients with AICU are more treatment-resistant, and their disease runs a more aggressive course, than those with non-autoimmune CIU. 8. If still getting urticaria, then look for other causes and treat symptomatically.

Differential Diagnosis of Urticaria Includes all dermatologic conditions with an urticarial component like cutaneous mastocytosis, urticarial vasculitis, insect bite reactions (papular urticaria), acute febrile neutrophilic dermatosis, pre bullous pemphigoid, acute facial contact dermatitis, erythema multiforme, collagen vascular disease, porphyria, pityriasis rosea, psoriasis and last but not least scabies. Differential Diagnosis of Angioedema Infections (cellulitis), trauma, superior vena cava syndrome, subcutaneous emphysema, Melkersson –Rosenthal syndrome. Treatment Acute urticaria • Minimize heat and stress, • Avoid alcohol, NSAIDs and opiates. • Soothing lotions such as calamine for topical application given during attack of urticaria. • Antihistaminics: H1 antagonists or H2 antagonists or Both together Old sedative antihistaminics are still better during acute episodes. • Corticosteroids may be required to tide over a crisis-tapering regime commencing with 30 mg prednisolone daily, with or without concurrent H1 antagonist administration. • Adrenaline used in anaphylaxis. • Other measures like intravenous fluids, oxygen use may be required.

Chronic Urticaria • Antihistamines still remain the mainstay of drug treatment. • Doxepin 25 to 50 mg at night time may be added. • Corticosteroids-occasionally as short tapering courses given in autoimmune chronic

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Essentials in Dermatology urticarias as they fail to respond to antihistamines. • Cyclosporine-2.5 to 5 mg /kg body weight/ day is another option. Control of urticaria usually occurs within 1 week of commencing treatment. • Intravenous immunoglobulin. • Other drug treatments are ketotifen (1-2 mg daily or at night), disodium cromoglycate, beta agonist (terbutaline 2.5 mg three times daily), calcium channel blockers (nifedipine), leukotriene antagonists (montelukast, zileuton), and anti IgE monoclonal antibody.

PRURITUS (ITCHING) Pruritus may be defined simply as that unpleasant sensation which produces the desire to scratch. It may be localized or generalized. If generalized, needs investigations for any systemic cause. Certain areas of the skin, particularly susceptible to pruritus are ear canals, perianal and genital areas, eyelids, etc. Localized pruritus may give clues to the etiology and is less likely to be associated with widespread or systemic disease. In the scalp, pruritus may be due to psoriasis or an ongoing folliculitis, in the inguinal or anal region due to pruritus ani, pruritus vulvae, pruritus scroti, venereal diseases, ectoparasites (scabies, pediculosis), in the hands and feet due to psoriasis, pompholyx, contact dermatitis, in the mid back due to notalgia paresthetica, macular amyloidosis, and in the lower limbs in older patients due to asteatosis. Causes of pruritus can be classified as follows: 1. Dermatological disorders 2. Systemic diseases 3. Psychogenic pruritus 4. Iatrogenic pruritus.

Dermatological Disorders Common dermatoses known to produce pruritus are scabies, pediculosis, insect bites, eczema,

urticaria, prickly heat, lichen planus, dermatitis herpetiformis, bullous pemphigoid, psoriasis, seborrhoeic dermatitis, dermatophytosis (tinea), urticaria pigmentosa, varicella and rubella, papular and pruritic eruption seen in AIDS patients, etc.

Systemic Disorders Three important systemic disorders most likely to play role in acquired acute pruritus are renal disease, hepatic disease and abnormalities of the hematopoietic system. • Renal – chronic renal insufficiency • Hepatic – intrahepatic and extrahepatic biliary obstruction (cholestasis). • Abnormalities of the hematopoietic system – iron deficiency anemia, polycythemia vera (is associated with bath pruritus), leukemia, Hodgkin’s lymphoma, mycosis fungoides, mast cell disorders. • Endocrine – diabetes mellitus, myxoedema, hyperthyroidism, hypoparathyroidism. • Intestinal parasites – infestation with hookworm, round worm, pin worm. • Collagen vascular disorders – SLE, sicca syndrome. • Neurological – multiple sclerosis, brain tumor. • Pregnancy.

Psychogenic Pruritus Psychosomatic disorders resulting in itch commonly occur in the middle aged or older individuals. Iatrogenic Pruritus Commonly induced by opium alkaloids, CNS stimulants, antidepressants, and belladona alkaloids.

Diagnosis History and clinical examination is of paramount importance. In addition, investigations outlined

Urticaria, Angioedema and Pruritus below may be undertaken to find the underlying cause. 1. Hemogram including ESR. 2. Liver function tests. 3. Kidney function tests. 4. Urine examination for albumin, sugar and cells. 5. Blood sugar. 6. Stool examination for occult blood, worms and ova. 7. Chest X-ray. 8. Thyroid function tests. 9. Hepatitis C screening. 10. HIV testing. 11. Uric acid estimation and also acid phosphatases. 12. Skin biopsy for mast cells and immunofluorescence. 13. Patch testing, photopatch testing, immunoglobulin levels. 14. Screening for underlying malignancy. 15. Serum protein electrophoresis.

Treatment 1. Eliminate causative disease if possible or treat it appropriately. 2. Avoid provocative influences: • Friction from rough clothing. • Overheating and vasodilatation, e.g. alcohol, hot drinks. • Keep nails short. • Soothing lotions, e.g. calamine in urticaria, moisturisers for dry skin. • Occlusive bandaging may be done to retain moisture in the skin.

• Emollients of bland nature, e.g. coconut oil, liquid paraffin. • 1% menthol in 90% ethanol is of significant value. • Steroid creams. • Topical antipruritic drugs, e.g. doxepin, crotamiton. • Avoidance of strong soaps in elderly individuals. 3. Systemic treatment: Antihistamines: H1 antagonists-especially sedative ones may be preferred Systemic steroids: Sometimes required to control urticaria Doxepin 4. Specific treatments: For uremic pruritus- Naltrexone (50 mg oral daily), activated charcoal, UVB phototherapy, ondansetron (4 mg twice daily), cholestyramine For cholestatic pruritus- cholestyramine, phenobarbitol, UVB phototherapy, rifampicin (300-450 mg daily), naltrexone (50 mg oral daily), and danazol. For hematologic disease-cimetidine, cyproheptadine, pizotifen (0.5 mg three times daily), danazol, interferon alpha 2b, UVB phototherapy, iron replacement therapy. Lidocaine and intralesional steroids (for localized pruritus and trigger points) may be tried as last resort.

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Drug Eruptions, Erythema Multiforme, Stevens-Johnson Syndrome, and Toxic Epidermal Necrolysis

DRUG ERUPTIONS • A drug may be defined as a chemical substance, or combination of substances, administered for the investigation, prevention or treatment of diseases or symptoms, real or imagined. • Drug eruptions are some of the most common skin disorders. • They are seen in 2 to 3% of hospitalized patients. • These eruptions may closely mimic other skin disorders.

Characteristics of Drug Eruptions 1. There is a history of drug intake preceding the eruption. The history of drug intake must include all systemic drugs, nonprescription drugs, home remedies and topical medications. A previous history of allergic reaction may increase the risk of development of an allergic reaction. 2. Drug eruption is sudden in onset. 3. Generalized eruption is often pruritic. 4. Eruption is bilateral and symmetrical, exception to this is fixed drug eruption. 5. Regression of eruption occurs on withdrawal of drug.

6. Similar type of rash recurs on re-exposure to the same or similar drug. Undesirable cutaneous or mucocutaneous reactions to systemically absorbed drugs occur through two mechanisms. 1. Immune mechanisms: All the four hypersensitivity mechanisms may be involved. Type I – IgE dependent reactions cause urticaria, pruritus, bronchospasm and laryngeal edema within minutes, hours or days. Type II – Cytotoxic reactions may cause thrombocytopenia. Type III – Immune complex dependent reactions result in serum sickness, urticarial or leukocytoclastic vasculitis within a week or so. Type IV – Cell mediated immune response may lead to eczematous and other types of eruptions in 3 to 4 weeks time. 2. Non-immune mechanisms: They include drug induced hemolysis (G6PD deficiency), mast cell degranulation (codeine, radiocontrast media), exacerbation of disease (psoriasis by lithium or beta blocker), drug deposition in skin, alopecia, etc.

Drug Eruptions, Erythema Multiforme, Stevens-Johnson Syndrome

General Rules • Drug allergy is most frequent in older individuals and may be related to development of immune response and increased exposure to drugs. • Topical application of drugs has the greatest propensity to induce allergy, followed by the intravenous route and the oral route. • The drugs most often responsible for the eruptions are antimicrobials and antipyretic/ anti-inflammatory analgesics. • The appearance of the eruption may provide some clues to its cause (e.g. fixed drug eruptions associated with sulphonamides). • Always keep in mind the fact that drug eruptions are great imitators of other skin diseases. • The most common morphologic patterns of drug eruptions are exanthematous (40%), along with urticaria and/or angioedema (37%), fixed drug eruption (6%), erythema multiforme and others.

4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19.

Acneiform eruptions (Fig. 18.1). Drug hypersensitivity syndrome. Lupus like eruptions. Lichenoid eruptions. Pityriasis rosea like eruptions. Phototoxic (Fig. 18.2)and photoallergic eruptions. Vesicobullous eruptions. Eczematous eruptions. Alopecia. Skin pigmentation. Vasculitic eruptions. Fixed drug eruptions. Erythroderma. Erythema multiforme. Stevens-Johnson syndrome. Toxic epidermal necrolysis.

Types of Drug Eruptions 1. Exanthematous eruptions (Maculopapular eruptions) (occur within 1 week). 2. Urticarial eruptions. 3. Serum sickness.

Exanthematous eruptions: These are the most frequent of all cutaneous reactions to drugs, and usually appear within 1 week of the causative drug being started. A rash may also start within 4-7 days of the offending drug being stopped. Lesions most often start first and clear first from head and upper extremities. They may be accompanied by fever, pruritus and eosinophilia. Antibiotics especially semisynthetic penicillins and sulfonamides are the most common causes of this reaction pattern.

Fig. 18.1: Acneiform drug eruption— monomorphous papular eruption over the back

Fig. 18.2: Phototoxic drug eruption—exaggerated sunburn reaction limited to exposed areas

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Essentials in Dermatology Urticarial eruptions: It is the second most common type of cutaneous drug eruption. Angiotension converting enzyme inhibitors are the frequent causes of angioedema often without urticaria. Serum sickness-like reaction: Serum sicknesslike eruption consists of fever, a rash with usually urticarial features and arthralgias occurring within 1 to 3 weeks of initiation of the drug. Lymphadenopathy and eosinophilia may also be found. Drug hypersensitivity syndrome (DHS): Also known as drug rash with eosinophilia and systemic symptoms (DRESS) syndrome or as drug induced delayed multiorgan hypersensitivity syndrome (DIDMOHS). It consists of an exanthema, hepatitis and fever. Eighty percent of the cases have an exanthem type eruption whereas others will develop more serious Stevens Johnson syndrome. DHS may be life threatening and requires prompt discontinuation of the drug and systemic corticosteroids.

with burning or stinging sensation (Fig. 18.3). These lesions sometime develop into bullous lesions (Fig. 18.4) and when they heal leave behind slate gray colored pigmentation (Fig. 18.5). Mucocutaneous junctions are commonly affected. Erythroderma: It is a generalised erythema, infiltration and scaling of the skin (involving more than 90% of the surface area of the body). It may be a manifestation of a drug eruption but usually occur due to dermatological disorders like psoriasis, contact dermatitis, pityriasis rubra pilaris, etc.

Fixed drug eruption: It differs from other eruptions in that it occurs and then recurs at fixed sites. Single or multiple circular or oval erythematous macule/s or plaque/s develop

Fig. 18.4: Fixed drug eruption–circular, slate gray colored pigmented macular lesion developing bullae over it

Fig. 18.3: Fixed drug eruption—typical slate grey oval macule with halo of erythema over the trunk

Fig. 18.5: Fixed drug eruption—multiple fixed drug eruptions over the chest

Drug Eruptions, Erythema Multiforme, Stevens-Johnson Syndrome Erythema multiforme: Erythema multiforme is characterized by many types of lesions including urticaria, target lesions (iris lesions or bull’s-eye lesions) (Figs 18.6 and 18.7) and vesicles and bullae, bilaterally symmetrically over acral areas of the body. They may also involve mucous membranes. Stevens-Johnson syndrome: It is a severe form of erythema multiforme associated with constitutional symptoms (fever) and visceral organ involvement, e.g. kidneys. The skin as well as mucous membranes are both involved (Figs 18.8 and 18.9).

Toxic epidermal necrolysis (TEN): The term toxic referred to a presumed toxin responsible for the prodrome and the eruption, epidermal to the presence of significant epidermal damage and necrolysis to the pathologic findings of necrosis and clinical findings of epidermolysis. It is a potentially life threatening, severe mucocutaneous reaction pattern characterized by fever, systemic toxicity, erythema and tenderness of skin followed by flaccid bullae formation resembling a burns case (Fig. 18.10). Nikolsky’s sign is positive. Mortality from TEN is 11 to 33%.

Fig. 18.6: Erythema multiforme—chest showing typical target lesions

Fig. 18.7: Erythema multiforme—target lesions showing three zones of color

Fig. 18.8: Stevens-Johnson syndrome—facial eruption with hemorrhagic crusting of lips

Fig. 18.9: Stevens-Johnson syndrome—atypical target lesions over the back

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Essentials in Dermatology serum creatinine, urinanalysis and TSH may be indicated in patients with suspected drug induced hypersensitivity syndrome. Third step is the invitro testing which include Radio Allergosorbent Assays (RAST) and Enzyme Linked Immunosorbent Assays (ELISA). Other tests include Lymphocyte Transformation Test (LTT), Macrophage migration Inhibition Factor (MIF), Lymphocyte Toxicity Assay (LTA), Basophil Degranulation Test and Histamine Release tests. Fig. 18.10: Toxic epidermal necrolysis—sheets of necrotic tender epidermis over the back

Diagnosis Diagnosis is basically based on suspicion and history of drug intake. A thorough and stepwise approach is essential to proper diagnosis of a drug- induced skin reaction. First step in patient evaluation should include (1) a comprehensive drug history, (2) awareness of various clinical manifestations of drug allergy and cutaneous reaction, (3) awareness of factors that favor development of allergic reactions to drugs, and (4) awareness of the immunologic and nonimmunologic mechanisms involved in cutaneous reaction to drugs. A general rule of thumb is that drugs started within one week of the onset of the eruption are the most likely suspects. Second step is skin biopsy and in vivo testing. This includes patch testing, scratch/ prick testing and dechallenge/ rechallenge tests. Dechallenge/ rechallenge continues to be regarded as the most definitive method for ascertaining drug-induced reactions. However, it is often not an option if the patient has experienced a life threatening condition or if suspected agent cannot be continued. Blood work-up may also aid in the clinical diagnosis. A CBC with differential count may show atypical lymphocytosis, leukopenia, leukocytosis, eosinophilia and so on. Liver function tests,

Differential Diagnosis Consider differential diagnoses such as infections, collagen vascular disease, primary skin conditions and neoplasia. Culture of skin, blood, tissue, erythrocyte sedimentation rate, ANA, and other tests may be ordered to help confirm or rule out other conditions. If palpable purpuric lesions are present, a complete physical and laboratory examination is required with an eye towards ruling out vasculitic involvement of other organ systems and other causes of vasculitis such as infections or collagen vascular disease. Treatment • Withdrawal of all drugs • Antihistamine (H1 antagonists) • Soothing lotion for topical application • Corticosteroids – topical steroids may provide some relief. Systemic corticosteroids are indicated if signs and symptoms are severe • Adrenaline in case of anaphylaxis • Other measures like fluid and electrolyte balance maintenance, wet compresses, etc. ERYTHEMA MULTIFORME It is a self limited, usually mild but relapsing exanthematic reaction of skin probably triggered by circulating immune complexes, most often related to recurrent herpes simplex infection,

Drug Eruptions, Erythema Multiforme, Stevens-Johnson Syndrome characterized by skin lesions with varied morphology (typically target lesions) in acral areas, frequently associated with mucous membrane lesions. • Herpes virus infection is the single most important cause of erythema multiforme (EMF). • Other important agents include Mycoplasma pneumoniae, drugs and malignancies. • EMF is common in young adults. • Prodromal symptoms are minimal to absent. • The lesions seen are urticarial papules, target lesions, vesicles and bullae. • The lesions occur in a symmetric fashion on the extensor aspects of limbs, palms and soles. • The target lesions /iris lesions have three zones of color: The central dark cyanotic area, pale edematous zone and the surrounding erythema (Figs 18.6 and 18.7). • The iris lesions result from centrifugal spread of a red maculopapule as the center becomes cyanotic, purpuric or vesicular.

Differential Diagnosis Urticaria, urticarial vasculitis, figurate erythemas (slow evolving lesions, usually asymptomatic, no mucosal involvement), acute febrile neutrophilic dermatosis, disseminated lesions of contact dermatitis, bullous pemphigoid, linear IgA dermatosis, herpes gestationis, lupus erythematosus, exanthematous or morbilliform drug eruption (accompanied by fever, pruritus, eosinophilia, involving trunk and extremities, sparing the face and pressure areas, fade with desquamation and postinflammatory hyperpigmentation), viral exanthem (start on face and involve the trunk, maculopapular/ urticarial/ vesicular/petechial lesions associated with fever, conjunctivitis, and lymphadenopathy, fades without pigmentation and scaling), fixed drug eruption (solitary first, later new lesions appear with repeated attacks, common on limbs, hands and feet, genitalia and perianal area, also

periorbital and perioral), Kawasaki’s disease (lips are red, dry with crust, transient red macules, conjunctivitis without exudates, strawberry tongue, lymphadenopathy), paraneoplastic pemphigus (severe necrosis of lips, eyes, oral mucosa, with polymorphous skin lesions, biopsy shows acantholysis and immunofluorescence confirms the diagnosis), Stevens Johnson syndrome (severe episodic acute mucocutaneous reaction, most often elicited by drugs and occasionally by infections, characterized by rapidly expanding irregular macules or atypical target lesions involving body surface area less than 10%, involvement of more than 2 mucosal sites), toxic epidermal necrolysis (mucosal erosions and epidermal detachment more than 30% of body surface area, skin tenderness, Nikolsky’s sign positive, histopathology shows full thickness epidermal necrosis with subepidermal cleft formation), acute graft versus host disease (also precipitating factor for Stevens Johnson syndrome, similar lesions, histopathology also can’t differentiate, lesions begin over the palms and soles first) and acute hemorrhagic edema of infancy (is a manifestation of leukocytoclastic vasculitis in infants and small children, it is characterized by symmetric erythematous and purpuric concentric rings in acral locations similar to target lesions that spontaneously resolve in about a week). • Treatment is symptomatic. If the herpetic lesions are clinically evident, acyclovir needs to be given.

STEVENS-JOHNSON SYNDROME— TOXIC EPIDERMAL NECROLYSIS SPECTRUM Stevens-Johnson syndrome (SJS) and Toxic epidermal necrolysis (TEN) are closely related severe acute mucocutaneous intolerance reactions most often elicited by drugs and less so by infections (Table 18.1).

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Essentials in Dermatology Table 18.1: Differentiating features between erythema multiforme and SJS-TEN

Features 1. 2. 3. 4.

Etiology Course Prodrome Eruption

5. Typical lesions 6. Mucosal involvement 7. Body surface area 8. Constitutional symptoms 9. Pathology

10. Internal organs 11. Duration 12. Complications

13. Mortality rate 14. Healing

Erythema multiforme

SJS-TEN

HSV (in majority) Acute, self limited, recurrent Absent to moderate Disseminated, symmetric, acral, face

Drugs (80-95%) Acute, self limited, episodic Intense, skin tenderness Disseminated, confluent, symmetric, on face, neck, trunk Fixed plaques, target lesions, blisters, Macules, flat atypical target, central Nikolsky’s sign –ve necrosis, Nikolsky’s sign +ve Frequent, mild oral Prominent, severe, 2-3 mucosal involvement <10% <10% to >30% Absent to moderate Prominent to severe Satellite cell necrosis, DEJ blister formation, Massive keratinocyte necrosis, prominent mononuclear cell infiltrate, papillary sloughing of epidermis, dermal dermis edematous infiltrate slight to absent Not involved Not infrequent 1-3 weeks > 2-6 weeks None Septicemia, pneumonia, gastrointestinal hemorrhage, renal and cardiac failure 0% 1-50% Without scarring Sequelae due to mucosal scars

They have been classified based on the area involved as: • SJS— mucosal erosions and epidermal detachment below 10%. • SJS/TEN overlap— mucosal erosions and epidermal detachment between 10-30%. • TEN— mucosal erosions and epidermal detachment more than 30% .

STEVENS-JOHNSON SYNDROME (SJS) • The important drugs causing SJS are phenytoin, phenobarbitone, sulfonamides, penicillins and NSAID’s. • It is common in children and young adults. • The disease is preceded by a nonspecific prodrome with fever, myalgia, rhinitis and cough. • Skin lesions occur abruptly and are purpuric macules, atypical target lesions and papules.

• Bullous lesions may occur in oral, genital and anal mucosa. • Ulcerative stomatitis with hemorrhagic crusting is the most characteristic feature (Fig. 18.8). • Corneal erosions may lead to symblepharon, synechiae and opacities. • Constitutional symptoms may be severe during active stages. • Differential diagnosis: As given for erythema multiforme. • The treatment includes immediate withdrawal of all potential causative agents, skin care, fluid and electrolyte balance, eye care, supportive care with antibiotics, diet and others. Patients have to be managed in an intensive care set up. Specific medications include steroids, intravenous immunoglobulins, plasmapheresis, hemodialysis, cyclophosphamide, and cyclosporine.

Drug Eruptions, Erythema Multiforme, Stevens-Johnson Syndrome

TOXIC EPIDERMAL NECROLYSIS (LYELL’S SYNDROME) TEN is a rare life threatening mucocutaneous reaction characterized by widespread sheets of erythema, necrosis and bullous detachment of epidermis. • Incidence: 1 to 3 cases per million. • Pathogenesis: In predisposed patients, the drug metabolites may bind to epidermis and trigger an immune response leading to immunoallergic cutaneous adverse reaction. • Drugs causing TEN are antiepileptics (phenytoin, phenobarbital, carbamazepine), sulfonamides, ampicillin, allopurinol, antituberculous drugs (thiacetazone, isoniazid) and NSAID’s.

Clinical Features • TEN begins with sheets of erythema covering wide areas. • In hours, the skin lesions become painful and extremely tender and small vesicles and bullae appear over the involved skin (Fig. 18.10). • The epidermis can be separated from dermis by slight tangential pressure (Nikolsky’s sign).

• Mucosal erosions and conjunctival erosions are a constant feature. • Septicemia and bronchopneumonia are the important causes of death.

Differential Diagnosis • As given for erythema multiforme. Staphylococcal scalded skin syndrome (SSSS) can be differentiated from TEN by occurrence in children, absence of mucosal lesions and absence of systemic features. Moreover in SSSS, the involved skin is dry and parchment- like while in TEN it is erythematous, purpuric and necrotic. Physical and chemical injury such as scalding and burns, solar erythema and chemical burns are other differential diagnosis, difficult in an unconscious patient. Treatment • Patients have to be managed in an intensive care set up with proper maintenance of fluid and electrolytes like Stevens Johnson syndrome. • Role of steroids is controversial. • Intravenous immunoglobulins have been found to be useful. • Plasmapheresis, hemodialysis, cyclophosphamide, cyclosporine, N-acetylcysteine, thalidomide, etc are other treatment options.

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Disorders of Sebaceous, Eccrine and Apocrine Glands

DISORDERS OF THE SEBACEOUS GLANDS Acne Vulgaris Acne vulgaris is a self-limited disease, seen frequently in adolescents, primarily involves the sebaceous follicles. Most cases of acne are pleomorphic, presenting with a variety of lesionscomedones, papules, pustules, nodules, and as sequelae to active lesions-pitted or hypertrophic scars. Pathogenesis Four major factors are involved in the pathogenesis: 1. Increased sebum production 2. Hypercornification of the pilosebaceous duct 3. Microbial flora 4. Inflammation.

Increased Sebum Production Acne patients, male and female, excrete on average more sebum than normal subjects and the level of secretion correlates with the acne severity.

Ductal Hypercornification Hypercornification of pilosebaceous ducts presents histologically as microcomedones and

clinically as blackheads and whiteheads. Thus comedones represent the retention of hyperproliferating ductal keratinocytes in the duct.

Bacteria Acne is not infectious. However, three major organisms isolated – Propionibacteria (P. acnes, P. granulosum, P. avidum), Staphylococcus epidermidis and Malassezia furfur. Environment of bacteria (i.e. low pH, reduced oxygen tension and bacterial lipases, proteases, etc.) more important than absolute numbers.

Inflammation The dermal inflammation is not caused by bacteria in the dermis but from inflammatory mediators that diffuse from the follicle where they are produced by P. acnes.

Natural History • Usually starts in adolescence and resolves by mid-twenties. • At least some degree of acne affects 95% and 83% of adolescent boys and girls. • Acne develops earlier in females than in males. • At the age of 40 years, acne may persist in 1% of males and 5% of females.


Clinical Features • Occurs predominantly on the face (99%), back (60%) and chest (15%). Infraorbital area spared even in severe acne. • Two types of lesions: i. Non-inflammatory (comedones) ii. Inflammatory • Comedones are the pathognomonic lesions of acne (Fig. 19.1). They are conical, raised lesions with a broad base and a plugged apex. Two types of comedones – blackheads/open comedones (black color due to oxidation of melanin) and whiteheads/closed comedones. • 25% of the whiteheads resolve within three days while another 75% develop into inflamed lesions. • Inflammatory lesions include papules, pustules and nodules or nodulocystic lesions (Figs 19.2 and 19.3). • Nodules occur more frequently in males and may be interconnected with sinuses. • In its most severe variant, acne can present with cysts and abscesses. • Some amount of scarring in 90%. These could be hypertrophic scars, keloids, atrophic scars or ice-pick scars. • Acne severity can be graded into mild (multiple open and closed comedones are present, with

Fig. 19.1: Acne vulgaris–face showing comedones, papules, and pustules

few inflammatory papules), moderate (with erythematous papules and pustules which are the predominant lesions, and disease is limited to the face), moderately severe (with erythematous papules, pustules, and nodules that are present on the face), and severe (with multiple painful nodules that are present on the back in spite of aggressive topical and oral interventions, respond only to isotretinoin).

Differential Diagnosis Acne is rarely misdiagnosed. The commonest mistaken diagnosis is rosacea (has facial flushing, typically pustules occurring over erythematous background, localization of lesions to mid-face,

Fig. 19.2: Acne vulgaris–face showing comedones, papules, pustules and nodules

Fig. 19.3: Acne vulgaris–nodulocystic lesions in addition to other acne lesions over the cheek

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Essentials in Dermatology absence of seborrhea, comedones, nodules, cyst or scarring). Perioral eczema/dermatitis can be confused with acne in females, but lesions itch, the skin is dry and there are no comedones. Whiteheads may be confused with milia (which are predominantly infraorbital in distribution and are whiter). Acneiform drug eruptions are sudden onset, follicular monomorphous eruptions characterized by papules and pustules resembling acne, induced by skin exposure to various industrial chemicals or medications. Folliculitis due to Gram-negative organisms can complicate acne therapy and rarely folliculitis due to Candida may also present as multiple pustular eruptions as may S. epidermidis folliculitis. Demodex folliculitis can present as nonresponsive acne, which best responds to metronidazole or topical permethrin. Rarely acne vulgaris need to be differentiated from Pityrosporum folliculitis, acne agminata, adenoma sebaceum lesions of tuberous sclerosis, pseudofolliculitis barbae, plane warts, acneiform eruption of Behcet’s disease.

Treatment Treatment involves counseling, acne assessment and appropriate and ethical prescribing based on the history, acne severity, lesion type and the psychological effects of the disease. The various modalities of treatment can be classified into topical and systemic therapies (Table 19.1).

Topical • Predominantly comedolytic: – Tretinoin (0.025%, 0.05%, 0.1% creams, gels) (reduce number of existing comedones and prevent formation of new comedones by loosening ductal keratinocytes) - The best treatment for comedones. Almost every patient benefits from topical retinoids. – Adapalene (0.1% cream, gel, solution). – Azelaic acid (20% cream).

• Predominantly antimicrobial: – Clindamycin (1% gel, solution). – Erythromycin (1.5-2% gel, solution, ointment). – Benzoyl peroxide (2.5-10% gel, lotion, cream). – Nadifloxacin. – Clarithromycin. – Tetracycline. – Sodium sulfacetamide- sulphur combination. • Predominantly anti-inflammatory – Adapalene. – Topical antibiotics. – Combination preparations. – Zinc and erythromycin. – Benzoyl peroxide and erythromycin.

Systemic • Oral antibiotics (oral tetracycline 250-500 mg 1-4 times a day, oral minocycline 50-100 mg daily, doxycycline 50-100 mg daily, erythromycin 250-500 mg 2-4 times a day, trimethoprim 300 mg twice a day, pulse dosing with azithromycin -250 mg daily for three days in a week for 4-6 months or roxithromycin). • Hormones (anti-androgens like 2 mg cyproterone acetate with 35 microgram ethinyl estradiol, flutamide-250 mg twice a day, oral contraceptive pills, spironolactone 100-200 mg daily, finasteride 2.5-5 mg daily): Indicated for women with acne located primarily on the lower face and neck, those with PCOS and late onset acne, late onset adrenal hyperplasia or other identifiable endocrinologic conditions. • Isotretinoin: It is the only anti-acne agent that affects all four of the known major etiologic mechanisms: sebum production, comedogenesis, Propionobacterium acnes (P. acnes) colonization of ductal and skin surface, and monocyte chemotaxis-induced inflammation. It is indicated for nodulocystic acne, acne

Disorders of Sebaceous, Eccrine and Apocrine Glands conglobata, acne fulminans, moderate acne relapse, significant psychosocial impairment, marked concomitant seborrhea, Gramnegative folliculitis, and scarring or persistent dyschromia. Acne therapy is usually initiated at a dose of 0.5 mg/kg daily for the first 2-4 weeks and then increased to 1.0 mg/kg/ day for the remainder of the 20 weeks course. The chance of a prolonged remission is greater when a total dose of 120 to 150 mg per kilogram of body weight is achieved. • Corticosteroids (co-prescribed with isotretinoin in severe acne variants).

Miscellaneous • • • • • • •

Oral zinc. Dapsone (nodulocystic acne). Clofazimine (acne fulminans). NSAIDs (to reduce inflammation). Chemical peels. Cryotherapy. Blue light therapy.

Surgical Procedures • • • •

Comedone extraction. Aspiration of cysts. Incision and drainage (large cysts). Intralesional steroid (0.1 ml triamcinolone injected into base of the cyst; reduces scarring).

Course and Prognosis The course and prognosis of acne is highly variable. All acne causes scars. ACNE VARIANTS WITH MARKED INFLAMMATION Acne Conglobata It is a rare type of acne, which is highly inflammatory and presents with comedones, nodules, abscesses and draining sinus tracts. Healing occurs with severe scarring. It usually starts in adult life. Oral isotretinoin is the best treatment for patients in whom antibiotics are unsuccessful or in patients with very severe deep acne like this one. Follicular Occlusion Tetrad (Acne tetrad) It is a combination of acne conglobata, acne inversa/hidradenitis suppurativa, dissecting cellulitis of scalp and pilonidal sinus. It is more common in men. SAPHO Syndrome It is characterized by Synovitis, Acne (conglobata, fulminans), Pustulosis (pustular psoriasis, palmoplantar pustulosis), Hyperostosis and Osteitis. Acne Fulminans It is characterized by the sudden appearance of severe acne with systemic signs and symptoms (fever, leukocytosis, osteomyelitis, polyarthralgia,

Acne Scar Surgery • Dermabrasion. • Laser abrasion. • Chemical peels.

Table 19.1: Treatment guidelines for acne vulgaris

Grade 1(Mild)

Grade II-III(Moderate)

Grade IV(Severe)

Maintenance therapy

Topical retinoids Benzoyl peroxide (BPO) or topical antibiotic (A/B)

Topical retinoids BPO or topical A/B

Isotretinoin or Topical retinoids, oral A/B, hormone therapy

Topical retinoids + BPO

+ Oral A/B Hormone Rx

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Essentials in Dermatology polymyalgia). The disease exclusively occurs in teenage boys. The face is often not involved. Treated initially with high dose corticosteroids to control inflammation and then retinoids

SPECIAL TYPES OF ACNE Acne from Drugs and Chemical Agents (Acneiform Eruptions) Due to topical or systemic glucocorticoids, anabolic steroids, INH, cyclosporine, iodides and bromides, lithium, etc. Oil Acne It is due to lubricating and cutting oil application and is seen mainly on front of thighs and forearms, predominantly as comedones. Chloracne or Industrial Acne It is due to topical or systemic exposure to toxic chlorinated hydrocarbons and causes severe comedonal acne. Excoriated Acne It is seen in young women. Small acne spots are picked, squeezed and altered by manual interference. Resulting papules are crusted and inflamed. ROSACEA It is a centrofacial disease, which is characterized by papules and papulopustules against a vivid erythematous background with telangiectases, preceded by episodes of flushing (Fig. 19.4). Later, there may be diffuse hyperplasia of connective tissue with enlarged sebaceous glands, particularly of the nose (rhinophyma). Women are more often affected than men in their third and fourth decade. Ophthalmological complications are common, occurring in over 50% of the patients with rosacea. These include sensation of grittiness

Fig. 19.4: Rosacea—face showing erythema topped by erythematous papules and pustules

or irritability of the eyes, often accompanied by visible reddening of the conjunctiva. Blepharitis, keratitis, episcleritis, chalazion, and hordeolum are also common.

Differential Diagnosis Important differential diagnoses include acne vulgaris (typical acne lacks the redness, telangiectasia, and flushing of rosacea), lupus erythematosus (DLE—scarring, scaling and follicular plugging are not features of rosacea, SLE-butterfly erythema is not pustular and is usually associated with systemic symptoms), perioral dermatitis (lesions itch, the skin is dry), seborrhoeic dermatitis (scaling in typical seborrhoeic areas) and demodex folliculitis (clearly mimics rosacea). Nasal sarcoidosis (lupus pernio) is the differential diagnosis for rhinophyma. Treatment Papulopustular rosacea responds well to treatment. Topical treatment with metronidazole (1% cream, 0.75% gel, cream, lotion) is effective. Additional topical therapies reported as effective include tetracycline, clindamycin, erythromycin, azelaic acid 20% cream and 15% gel, 0.025%

Disorders of Sebaceous, Eccrine and Apocrine Glands retinoic acid and 10% sulphur cream. Topical imidazoles are also gaining popularity in the treatment of rosacea. Effective oral treatment includes tetracycline or oxytetracycline 250 mg twice daily and erythromycin 250 mg twice daily. More recently developed tetracyclines such as minocycline, lymecycline and doxycycline are often used. Oral metronidazole (750-1500 mg in divided doses) is also effective. Oral isotretinoin (10-60 mg/day) is an alternative in resistant rosacea and can even improve rhinophyma. Flushing and burning are the most difficult features of rosacea to treat. Non-cardioselective beta-blockers such as propranolol 40 mg twice daily or nadalol 40 mg daily, clonidine 50 mcg twice daily, and rilmenidine 1 mg daily. Advent of vascular laser and intense pulsed light sources has provided a range of highly effective treatments for ablation of telangiectasia. Surgical treatment is a very successful treatment in rhinophyma. Excellent cosmetic results can be obtained by scalpel or razor remodeling, electrocoagulation, cryosurgery, excision and vaporization with argon, carbon dioxide or Nd: Yag lasers.

PERIORAL DERMATITIS It is facial dermatosis predominantly affecting females of childbearing years. The dermatosis is characterized by an erythematous, micropapular, fine scaling eruption classically affecting the nasolabial folds, chin, and upper lip, sparing a rim of skin at the vermilion border. In nearly every patient, there is striking dependency on topical steroids. A frequently heard story is that the patient presents with the earliest signs of rash and is given a potent topical steroid. The condition then shows improvement but any attempt to stop the treatment dramatically worsens the condition after a few days.

Differential Diagnosis The important differential diagnoses include rosacea (usually no telangiectasia or flushing in perioral dermatitis), lip licking cheilitis (seen predominantly in 7-15 years old age group, the rash is caused by repeated licking of skin around the mouth and is marked by a scaling, pink band around the mouth and involvement of lips), seborrhoeic dermatitis (not usually circumoral and the scalp, ears and eyebrows are commonly involved), contact allergic dermatitis (does not spare the immediate perioral area), late-onset acne vulgaris (evidence of comedones, large papules and cysts in wider distribution and responds more slowly to treatment), acne agminata (difficult to distinguish if confined to perioral area but can be differentiated histologically) and facial AfroCaribbean childhood eruption (FACE) (do not spare the perilabial skin, pustules do not occur, occur often in males). Treatment The most important measure is usually to discontinue the application of the topical corticosteroids. The patient should be warned that an initial flare may develop after the withdrawal of a topical corticosteroid. A four week course of oral tetracycline or its analogues such as minocycline, doxycycline or lymecycline is usually all that is required. Topical metronidzole, topical erythromycin, and topical tetracycline have also been used. DISORDERS OF ECCRINE SWEAT GLANDS Disorders of eccrine sweat glands may be broadly classified into four-chromhidrosis, hyperhidrosis, hypo- or anhidrosis and miliaria.

Chromhidrosis It means secretion of colored sweat. It is an exceedingly rare functional disorder of the

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Essentials in Dermatology apocrine sweat glands. The colored sweat may be yellow (most common), blue, green, or black. Colored sweat fluoresces and is caused by lipofuscin. Topical capsaicin satisfactorily reduces facial and nipple chromhidrosis.

Hyperhidrosis It means excessive sweating which could be localized or generalized. It may occur as consequence of a number of causes. Most commonly excessive sweating of the palms and soles occurs during mental stress, and may be associated with tachycardia and vasomotor instability.

Palmoplantar Hyperhidrosis (Emotional Hyperhidrosis) This type of hyperhidrosis is usually localized to the palms, soles or/and axillae. Frequently there is a family history and commonly begins in childhood or around puberty. There is a tendency of spontaneous improvement after the age of 25 years. Sweating may be intermittent (triggered by anxiety, stress, or fear) or may be constant. Apart from the embarrassing nature of the disorder, complications include pompholyx, contact dermatitis, and pitted keratolysis.

Gustatory Hyperhidrosis Sweating on the lips, forehead and nose after eating certain foods is known as gustatory sweating. Gustatory sweating may be idiopathic, post-peripheral nerve injury (parotid surgery, auriculotemporal nerve syndrome or von Frey syndrome, cervical sympathectomy), sensory neuropathy (diabetes mellitus, post herpes zoster).

Treatment Topical anticholinergics (0.5% glycopyrronium bromide cream), formalin 1% soaks, glutaraldehyde 10% in a buffered solution, 20% aluminium chloride have been used. Iontophoresis either using tap water or anticholinergic

drugs like glycopyrronium bromide is a satisfactory method. Direct current is usually used, with each palm or sole being treated for 30 min with 20 mA initially three times a week. Botulinum toxin injection into 4 cm2 areas on the palms, soles or axillae dramatically reduces sweating. The hypohidrosis continues for an average of 7 months. Axillary hyperhidrosis may be effectively controlled by excision of the most active sweating portion of the axillary skin, alternatively liposuction removal may be used.

Anhidrosis (Hypohidrosis) It can result from poral occlusion, congenital or acquired absence of sweat glands, damage to sweat gland function or dysfunction of sympathetic nerves in neuropathies. It may be localized or generalized. Miliaria It results from the obstruction of free flow of eccrine sweat to the skin surface and retention of the sweat within the skin. It manifests with a variety of signs and symptoms. Miliaria crystallina (Sudamina) consists of non-inflammatory, superficial subcorneal translucent vesicles that easily rupture when rubbed with a finger. Miliaria rubra (prickly heat) causes pruritic inflammatory papules around the sweat pores, follows repeated episodes of sweating in a hot humid environment. Some of the eruptions of miliaria rubra become pustular resulting in miliaria pustulosa. Miliaria profunda results when sweat leaks into the dermis and presents as multiple discrete, flesh colored papules. The most common complications of miliaria are secondary infection and disturbance of heat regulation. Periporitis staphylogenes is the name given to multiple staphylococcal abscesses superimposed on miliaria rubra in young infants (Fig. 19.5). Postmiliarial hypohidrosis invariably occurs following miliaria and sweating may be depressed to half the normal amount for as long


Fig. 19.5: Miliaria complicated by secondary infection of sweat glands—periporitis

as 3 weeks following miliaria. Affected persons may show decreasing efficiency, irritability, anorexia, drowsiness, vertigo and headache. Tropical anhidrotic asthenia is a rare form of miliaria with long lasting poral occlusion, which produces anhidrosis and heat retention.

Treatment The most effective treatment is to place the patient in a cool environment as this will avoid further sweating. Avoidance of excessive clothing, friction with clothing, excessive use of soap and contact of the skin with irritants will reduce the incidence. Calamine lotion is effective in the relief of discomfort. Oral ascorbic acid 500 mg twice daily has been found to diminish the severity of miliaria. DISORDERS OF APOCRINE GLAND The capacity to produce an unpleasant odor is best-known property of apocrine gland. Physicians in the past relied heavily on detecting special body odors in diagnosis of diseases, e.g. fruity odour in diabetic coma, butcher shop odor in yellow fever, freshly baked bread odor in typhoid fever, etc.

Bromidrosis (Bromhidrosis or Osmidrosis) Excessive odor arising from apocrine sweat, also known as fetid sweat or malodorous sweating. It is chiefly encountered in axillae. Often the patient complains of offensive axillary sweat but actually have no offensive odor. The complaint represents a phobia, delusion, paranoia or a lesion of the central nervous system. True bromidrosis is usually not recognized by the patient. Antibacterial soaps, deodoarants, frequent bathing, changing of underclothes, shaving of the axillae and application of aluminium chloride are all helpful measures. Fish Odor Syndrome (Trimethylaminuria) Affected individuals are unable to oxidize tertiary trimethylamine produced by oxidation of choline and carnitine in food. This results in formation of excessive amounts of offensively smelling (rotting fish) sweat. Defect in flavin containing monooxygenase 3 (FMO3) gene causes the disease. Diet low in carnitine and choline may help. Chromhidrosis Coloured apocrine sweat. Fox Fordyce Disease It is a disorder of the apocrine sweat gland comparable to prickly heat of the eccrine glands. It is a chronic itchy papular eruption of apocrine gland bearing areas-axillae (Fig. 19.6) and pubic area, classically seen in women between the ages of 15 and 35 years. Pruritus is often exacerbated by emotional or physical stimuli of the apocrine glands. Differential Diagnosis Includes lichen planus, lichen nitidus, infective folliculitis, chronic dermatitis, and syringoma.

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Fig. 19.6: Fox Fordyce disease–chronic itchy papular eruption in the axilla of a female

Fig. 19.7: Hidradenitis suppurativa—nodules, sinuses and puckered scarring in the axilla

Treatment Estrogen therapy in the form of oral contraceptive pills is universally effective. Topical tretinoin, clindamycin solution, UV phototherapy have been tried. Treatment response with topical steroids or intralesional steroids is not satisfactory.

epidermoid cyst. Other dermatoses that produce fistulas and sinuses should be excluded, such as scrofuloderma, actinomycosis, tularemia, and cat scratch disease. In the inguinal region, lymphogranuloma venerum, granuloma inguinale, Crohn’s disease, and ulcerative colitis should be excluded.

Hidradenitis Suppurativa (Apocrinitis, Hidradenitis Axillaris, Acne Inversa) It is a chronic and cicatricial disease of apocrine gland bearing skin areas-axillae, anogenital skin (Fig. 19.7). The disease occurs in both sexes, usually in the second or third decade. It is rare before puberty. Affected patients are often overweight. Differential Diagnosis A solitary abscess in the early stage resembles a carbuncle, lymphadenitis, or an infected

Treatment Earliest lesions often heal quickly with intralesional steroid therapy. Concomitant use of antibiotics (minocycline, cephalosporins, ciprofloxacin) is recommended. Isotretinoin (1 mg/kg/day) is effective in some patients. In severe disease, removal of the affected area is often required. Wide local excision and healing by second intention is considered the surgical treatment of choice.

Disorders of Hair and Nails

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DISORDERS OF HAIR Alopecia Alopecia is loss of hair. It may be diffuse or patchy, scarring or non-scarring. Diffuse Non-scarring Alopecia 1. Androgenetic alopecia: Terminal hair is converted into vellus hair. In men, recession of the frontal hairline near the temples and thinning over the vertex occurs (Fig. 20.1). In women, the scalp hair is generally diffuse but more so in the vertex area. Treatment includes in males- topical minoxidil (2 and 5% solution) or oral finasteride – competitive inhibitor of type II 5 α-reductase -1 mg/day and in females – topical minoxidil 2% and antiandrogens (spironolactone, cyproterone acetate, flutamide) and surgical treatment such as scalp reduction and hair transplantation.

induces a synchronous growth of hair follicles 6-10 weeks later such that more synchronous hair fall is seen. It commonly follows delivery of a baby, high fever, surgery or other stress. Drugs such as enalapril, beta blockers, retinoids, oral contraceptive pills, lithium, levodopa may be responsible. Treatment is reassurance, removal of stress or drug responsible. Hair density usually returns to normal in 6-12 months.

2. Endocrine and nutrition related: Endocrine abnormalities of the thyroid, pituitary or adrenals can cause diffuse alopecia. Malnutrition and zinc deficiency or iron deficiency may also cause diffuse alopecia. 3. Telogen effluvium: Certain events can induce hair follicles to enter the telogen phase. This then

Fig. 20.1: Androgenetic alopecia—scalp showing receding frontal hairline

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Fig. 20.2: Anagen effluvium – diffuse non-scarring alopecia following use of cytotoxic drugs

Fig. 20.3: Alopecia areata – rounded bald smooth patches over the scalp

4. Anagen effluvium: Abrupt cessation of hair growth follows use of cytotoxic drugs (cyclophosphamide) (Fig. 20.2), heparin, warfarin, colchicine, vitamin A, etc.

Patchy non-scarring alopecia Alopecia Areata • Alopecia areata is an unpredictable usually patchy, non scarring hair loss condition. The word alopecia is derived from the Greek, “Alopex”, meaning “Fox mange” (baldness). Any hair bearing surface may be affected. • It is a common condition, probably autoimmune in etiology. Associated autoimmune diseases are Hashimoto’s disease, vitiligo and myasthenia gravis. • It affects men and women equally. • It generally starts in the 2nd or 3rd decade. Patients are frequently quite young, sixty percent of them present before 20 years of age. • The characteristic initial lesion is commonly a round or oval, totally bald, smooth patch

Fig. 20.4: Alopecia areata – many bald patches coalescing over the scalp

involving the scalp (Figs 20.3 and 20.4) or any hair bearing area on the body. • A frequent feature in an alopecia areata patch is “Exclamation mark” hairs that are broken

Disorders of Hair and Nails short hairs tapering proximally. The pull test may be positive at the margins of the patch indicating very active disease. • Alopecia areata progresses as a wave of follicles, enter telogen prematurely. • The scalp is the most common site but any site can be involved. When the perimeter of the scalp is involved, it is called ophiasis (so called from its resemblance to a snake) (Fig. 20.5), which is associated with poor prognosis. • Alopecia areata can occur as diffuse loss of hair from the scalp but spares the grey hair (Fig. 20.6).

• If there are multiple patches on the scalp, they may become confluent to result in total loss of hair from the scalp, called as alopecia totalis (Fig. 20.7). Total loss of hairs from the whole body is called alopecia universalis. • Nail dystrophy is associated with alopecia areata in 10 to 66% of cases. Nail pitting “Hammered brass” (Fig. 20.8), trachyonychia (longitudinal striations), Beau’s line, onychorrhexis, thinning or thickening, onychomadesis, punctate or transverse

Fig. 20.7: Alopecia totalis

Fig. 20.5: Alopecia areata—ophiasis pattern

Fig. 20.6: Alopecia areata—diffuse hair loss sparing grey hair

Fig. 20.8: Alopecia areata – fine pitting of nail

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leukonychia, red spotted lunula and nail dystrophy may be seen in the nails.

Classification of Alopecia Areata According to Pattern 1. Patchy alopecia areata-round or oval patches of hair loss (most common) (Fig. 20.3). 2. Reticulated pattern of patchy hair loss (Fig. 20.4). 3. Ophiasis, a band-like alopecia areata, hair loss occurs in the parietal temporo-occipital scalp (Fig. 20.5). 4. Ophiasis inversus(sisaipho), a rare band like pattern of hair loss in the frontal parieto temporal scalp (the exact opposite of ophiasis) 5. Diffuse alopecia areata, a diffuse decrease in hair density over the entire scalp.

According to the Extent of Involvement 1. Alopecia areata, partial loss of scalp hair 2. Alopecia totalis, 100% loss of scalp hair (Fig. 20.7). 3. Alopecia universalis, 100% loss of hairs on the scalp and body.

Indicators of a Poor Prognosis 1. 2. 3. 4. 5. 6. 7.

Underlying atopy The presence of other autoimmune diseases Family history of alopecia areata Young age at onset Nail dystrophy Extensive hair loss Ophiasis.

Diagnosis The characteristic non scarring patchy loss of hairs, the presence of exclamation point hair on the periphery, a hair pull test indicating telogen hair excess and in atypical cases skin biopsy showing specific findings (a peribulbar and at the lower one third of the follicle, a lymphocytic infiltrate, “swarm of bees”, with no scarring) all points towards the diagnosis.

Differential Diagnosis Alopecia areata needs to be differentiated from tinea capitis, trichotillomania and secondary syphilis (moth-eaten appearance in beard or scalp). Treatment • Various modalities of treatment are available, the simplest of which are the use of counter irritants like phenol, benzoyl, tincture iodine, dithranol, etc. Physical modalities like UVB in erythema doses, grenz rays and thorium X-ray may be used with varied success. • Considering the autoimmune etiology of the disease various immunosuppressive drugs have been used. Among them, corticosteroids are important which can be used topically, intralesionally and systemically. • Photochemotherapy, contact immunotherapy (using dinitrochlorobenzene [DNCB], squaric acid dibutylester [SADBE], diphencyprone), topical minoxidil or topical cyclosporine may be used. Course and Prognosis Alopecia areata, however, a serious cosmetic problem, has unpredictable course. Tinea capitis: Discussed earlier under dermatophytosis. Trichotillomania • It is the term (Greek-“hair pulling madness”) first used by the French dermatologist Hallopeau to denote “a morbid impulse to pull one’s own hair”. • It manifests as one or a few small oval patches of hair loss which may occur in any hair area of the body in children or adults. • The patches are unusually irregular, asymmetric, and ill defined and have many broken hairs. • Common sites of involvement include scalp (Fig. 20.9), eyebrows, eyelashes, and pubic area.


Fig. 20.9: Trichotillomania – unusually irregular patchy loss of hair over the temple area of scalp due to pulling of hairs

Fig. 20.10: Perifolliculitis decalvans-scarring alopecia with folliculitis

• Trichophagy is rarely reported with trichotillomania, and when it occurs, trichobezoars (obstructive hair balls in the gastrointestinal tract) are the most severe complication. • Clinical differentiation from scalp alopecia areata is usually based upon the presence of short, broken hairs within the patch, and more irregular, often angular outlines of the area. Differentiation from tinea capitis may require Wood’s light examination, potassium hydroxide, or culture. The scalp in trichotillomania is usually healthy with broken hairs rather than scaly or erythematous in tinea capitis. • Any method of preventing grasping of hairs for a period of time (restraint, heavy gloves) will clarify the diagnosis and in one way serve as treatment, since hairs will grow normally in the area in the absence of trauma. One form of treatment consists of simply shaving the area. Psychological intervention and drugs such as clomipramine or fluoxetine may be given.

Secondary syphilis: Moth-eaten alopecia may be produced.

Traumatic alopecia: It is due to traction or pressure.

A. Ovarian (Polycystic ovarian disease, hyperthecosis),

Patchy/Diffuse Scarring Alopecia 1. Physical and chemical injury. 2. Infection – Kerion, favus, herpes zoster, folliculitis decalvans (Fig. 20.10). 3. Lichen planus and DLE. 4. Pseudopelade: Pseudopelade describes a scarring alopecia which represents the end stage of an idiopathic or unidentified destructive inflammatory process in the scalp. Characteristically, involves the vertex area of the scalp with crab like extensions (Fig. 20.11). 5. Malignancy – Basal cell carcinoma, squamous cell carcinoma. Excess hair Hirsutism: It refers to hair growth in a woman in areas of the body where hair growth is under androgen control and in which normally only post-pubescent males have terminal hair growth. These areas include the moustache, beard, chest and inner thigh. Causes are

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Fig. 20.11: Scarring alopecia of scalp-crab like extensions over the scalp-pseudopelade

Fig. 20.12: Clubbing of nails

B. Drugs (androgens, oral contraceptive pills, glucocorticoids, diazoxide, minoxidil, phenytoin).

A. Shape Abnormalities

C. Endocrine –Adrenal (Congenital adrenal hyperplasia, 21 and 11 hydroxylase deficiency, ACTH dependant Cushing’s syndrome).

Clubbing: It means increased transverse and longitudinal curvature of nails with hypertrophy of the soft tissue components of the digit pulp (Fig. 20.12). To confirm it, one can oppose dorsal aspects of two fingers from opposite hands, between the two opposed nails normally a window of light is seen which get obliterated in clubbing(window closes).

Others- hypothyroidism, acromegaly and D. Physiological – puberty, pregnancy. Treatment includes removal of precipitating cause/s, drug therapy with oral contraceptive pills, spironolactone, flutamide, finasteride, cyproterone acetate, and glucocorticoids, physical methods such as shaving, bleaching, waxing, laser, or electrolysis and topical therapy with eflornithine hydrochloride cream (slows hair growth by blocking ornithine decarboxylase).

Anonychia: It means absence of nails. Usually a developmental anomaly.

Clubbing Causes CLUBBING

DISORDERS OF NAILS

• Cyanotic Heart Disease • Lung disease (hypoxia, lung cancer, bronchiectasis, cystic fibrosis) • UC/Crohn’s disease • Biliary cirrhosis • Birth defect (harmless) • Infective endocarditis • Neoplasm (esp. Hodgkin’s) • GI malabsorption.

Nails may show their abnormalities—in shape, surface and color.

Koilonychia: Reverse curvature in the longitudinal and transverse axis of nail gives a

Hypertrichosis: It specifically refers to hair density or length beyond the accepted limits of normal for a particular age, race, or sex. The excess hair may be generalized or localized and may consist of lanugo, vellus, or terminal hair.


Fig. 20.13: Koilonychia—spoon like deformity of finger nails

Fig. 20.15: Onychogryphosis – thickened great toe nail growing upwards

Pterygium: When a central fibrotic tongue like band from proximal nail fold joins the nail bed dividing the nail proximally into two, it is known as pterygium. For example, it occurs in lichen planus. Subungual hyperkeratosis: It means excessive collection of squamous debris under the free edge of the nail. For example, it occurs in psoriasis, onychomycosis, etc. Onychogryphosis: Here nail gets thickened and grows upwards in a spiral manner attributed to chronic trauma (Fig. 20.15).

Fig. 20.14: Onycholysis with pitting of finger nails in psoriasis

Pachyonychia congenita: Here hypertrophy of nails is associated with nail bed and hyponychial hyperkeratosis (Figs 20.16 and 20.17).

B. Surface Abnormalities

concave dorsal aspect to the nail, such nail is thought to have koilonychia (Fig. 20.13). Commonly, it is due to iron deficiency anemia.

Beau’s lines: They are transverse grooves on nails which arise out of temporary interference with nail formation (Fig. 20.18).

Macronychia and micronychia: Too large or too small nail in comparison with other nails of digits.

Pitting: These are punctate erosions on the nail surface (Fig. 20.14). Commonly seen in psoriasis, and alopecia areata.

Onycholysis: Distal or lateral separation of the nail from the nail bed (Fig. 20.14). May be seen in psoriasis, onychomycosis, etc.

Median canaliform dystrophy: Nail shows midline split with fir tree like appearance of ridges

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Fig. 20.16

Fig. 20.17

Figs 20.16 and 20.17: Pachyonychia congenita—thickening of nails with subungual hyperkeratosis

Onychoclasis: Breaking of the nail. Onychomadesis: Separation of the proximal part of nail plate from the matrix and bed with subsequent shedding of nail. Onychomalacia: Softening of nail. Onychorrhexis: Longitudinal ridging and fissuring of nail plate with brittleness and breakage. Onychotillomania: Compulsive picking or tearing of nail. Fig. 20.18: Beau’s lines—transverse depressions equidistant from the nail folds

angled backwards. Commonly, it affects the thumb nail and involvement is symmetrical. Onychoschizia: It is characterized by transverse splitting of nail into layers at or near the free edge of the fingers and toes. Onychauxis: Nail plate appears to be thickened due to subungual hyperkeratosis of nail bed seen in psoriasis, eczema, distal subungual hyperkeratosis.

Trachyonychia: Nails are rough and often thinned (20 nail dystrophy or sand paper nail) seen in alopecia areata, lichen planus, psoriasis, etc.

C. Color Abnormalities Color changes in nail may be due to exogenous causes or endogenous causes. Leukonychia: This term is used for white discoloration of nails attributable to nail matrix dysfunction. Melanonychia: Means streaky hyperpigmentation of nails.


Fig. 20.19: Half and half nail – proximal half white and distal half brownish

Terry’s nail: Nail is white proximally but normal distally. It occurs in congestive cardiac failure, cirrhosis or adult onset diabetes mellitus. Red lunulae: Nail shows erythema of all or part of lunula. Mostly affects thumb nail. Dotted red lunula occurs in psoriasis. Other causes are congestive cardiac failure, cirrhosis of liver, systemic lupus erythematosus, etc. Splinter hemorrhages: They represent longitudinal hemorrhages in the nail bed, resembling the pattern of subungual vessels. They may occur in dermatoses like psoriasis, dermatitis, fungal infection or systemic diseases like endocarditis, systemic lupus erythematosus, mycosis fungoides, rheumatoid arthritis, etc. Half and half nails: It is onychopathological index of renal diseases and nail exhibits either normal or whitish proximal half and distinctly abnormal, brownish distal portion (Fig. 20.19).

Infections of Nails and Nail Folds Acute paronychia: It presents with painful swelling of the nail fold. Most commonly caused by staphylococci. Chronic paronychia: It presents with chronic swelling of nail folds of one or more fingers, often middle and index fingers, and is associated with candida and/or pseudomonas infection. Herpetic paronychia (Herpetic whitlow): It is due to primary inoculation of herpes simplex virus on to the finger and presents as single/grouped vesicles close to the nail. Tinea unguium: It is the fungal infection of nail complex by a dermatophyte and may be distal subungual infection, proximal subungual infection, or superficial white onychomycosis Onychomycosis: It includes tinea unguium and also infection by non-dermatophytic fungi including yeasts.

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Metabolic and Nutritional Disorders

PORPHYRIA • Porphyrias are hereditary disturbances in the synthesis of heme involving well-defined enzymatic defects. • The term “porphyria” is derived from the Greek word for “purple” and originally referred to the red to purple color of the urine of patients affected by acute intermittent porphyria. Other forms of porphyria produce urine that varies from pink to red to brown. Unlike findings with hematuria and pigmenturias (e.g., hemoglobinuria due to hemolysis, myoglobinuria caused by rhabdomyolysis), routine dipstick tests are negative for the presence of heme in porphyria patients. • Porphyrins also account for the fluorescence of urine viewed with a Wood’s lamp. • Almost all are inherited in an autosomal dominant pattern except congenital erythropoietic porphyria which is inherited in an autosomal recessive manner. • In addition, although the enzyme defects are genetic and permanent, the symptoms are often intermittent and do not appear until puberty except in congenital erythropoietic porphyria and hepatoerythropoietic porphyria (both manifest in infancy or

childhood, resemble clinically each other). This is true in porphyria cutanea tarda, acute intermittent porphyria, and variegate porphyria. • Photosensitivity in porphyria is due to absorption of UV radiation in soret band (400-410 nm). • Factors which precipitate are four m’s: medication (ethanol, oestrogens, iron, hexachlorobenzene), menses, malnutrition and medical illness.

Clinical Classification • These disorders can be classified into those without vesicobullous lesions and those associated with them. • The former includes delta aminolevulinic acid dehydratase deficiency porphyria and acute intermittent porphyria (have no cutaneous manifestations), and erythropoietic protoporphyria (can cause photosensitivity, but vesicobullous lesions are rare). • The latter includes congenital erythropoietic porphyria (Gunther’s disease), hepatoerythropoietic porphyria, hereditary coproporphyria, variegate porphyria (most prevalent in white population of South Africa) and porphyria cutanea tarda (PCT).


Congenital Erythropoietic Porphyria • Congenital erythropoietic porphyria manifests in infancy or early childhood. • It is the result of defective activity of the enzyme uroporphyrinogen cosynthase. • Clinical manifestations include severe bullous cutaneous photosensitivity (Fig. 21.1), hypertrichosis (Figs 21.2 and 21.3),

erythrodontia (fluorescent teeth), hemolytic anemia with splenomegaly, and bone abnormalities. However, the first clue to the diagnosis in infancy is generally not one of these striking cutaneous changes, but rather the pink or burgundy discoloration of urine (staining of diapers) due to massive porphyrinuria (Fig. 21.4). Diagnosis Markedly elevated levels of uroporphyrin I and coproporphyrin I in urine, stool and RBCs.

Fig. 21.1: Congenital erythropoietic porphyria– blistering over the fingers

Fig. 21.2

Differential Diagnosis Other childhood photosensitivity disorders • Treatment of this disorder is aimed at the cutaneous photosensitivity or at the anemia and its complications. Sun protection (clothing better than sunscreens) and ingestion of beta carotene may ameliorate some portion of the photodamage. Other modalities,

Fig. 21.3

Figs 21.2 and 21.3: Congenital erythropoietic porphyria—a child having photosensitivity and hypertrichosis of face and trunk

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Fig. 21.4: Congenital erythropoietic porphyria–pink to red discoloration of urine in the test tube

Fig. 21.5: Porphyria cutanea tarda– darkening of the urine

chronic transfusion regimens and splenectomy, the risks must be weighed against the severity of the disorder in each individual case. Bone marrow transplantation successful in some cases.

Porphyria Cutanea Tarda • PCT was first recognized by Waldenstrom in the 1930s, who identified a group of patients with excessive porphyrins in the urine, skin lesions in light exposed areas and a late (‘tarda’) onset in adulthood (in contrast with Gunther’s disease), so he called the disease ‘porphyria cutanea tarda’. • Most common type of porphyria due to deficiency of enzyme coproporphyrinogen decarboxylase. • Clinical signs include darkening of the urine (Fig. 21.5) and cutaneous photosensitivity manifested as fragility and bullae of sun exposed skin, scarring (Fig. 21.6), hypertrichosis and pigmentary and sclerodermoid changes. In severe cases, the clinical

Fig. 21.6: Porphyria cutanea tarda–scarring and pigmentary changes following photosensitivity

appearance may be similar to that of congenital erythropoietic porphyria. Diagnosis Wood’s lamp examination of urine shows orangered fluorescence. Total porphyrins and uroporphyrins are raised in urine and plasma; coproporphyrins raised in stool; porphobilinogen

Metabolic and Nutritional Disorders and delta aminolevulinic acid are normal in urine. Immunofluorescence of skin demonstrates IgG and other immunoglobulins at dermo-epidermal junction, and in and around blood vessels. Differential Diagnosis Some forms of epidermolysis bullosa acquisita look similar as does drug induced pseudoporphyria (usually furosemide) in renal dialysis patients • Avoid hepatotoxic agents. Unlike other porphyrias, it is usually not inherited and responds to different treatments (venesection and antimalarials). If hepatitis C is documented, treat it with interferon and ribaverin

•

•

•

•

PRIMARY CUTANEOUS AMYLOIDOSIS • Primary cutaneous amyloidosis is defined as cutaneous amyloidosis in the absence of other systemic or dermatological disease. • The various localized forms of primary cutaneous amyloidoses include the more

Fig. 21.7: Lichen amyloidosis–pruritic brownish lichenoid papules affecting the extensor aspect of lower leg

•

common papular (lichen amyloidosis) and macular types and the rare nodular or tumefactive form. Single or multiple nodules involving the trunk or limbs characterize the nodular form. It is due to localized plasma cell dyscrasia. Lichen amyloidosis is the most common form characterized by numerous pruritic, brownish lichenoid papules distributed over the extensor surface of legs (Fig. 21.7) and forearms and upper back. Macular amyloidosis typically manifests as brownish patches with a reticulate or rippled pattern, involving the upper back (Fig. 21.8), arms and lower extremities. In some patients features of both lichen and macular amyloidosis can coexist and the term ‘biphasic amyloidosis’ has been coined for these. Extensive variants of both macular and papular amyloidosis have also been described. On the other hand, amyloidosis cutis dyschromica (ACD) is a rare distinct type of primary cutaneous amyloidosis, which is characterized by reticulate hyperpigmentation with hypopigmented spots seen almost all over the body without any papulation.

Fig. 21.8: Macular amyloidosis—rippled pigmentation over the upper back

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Essentials in Dermatology Differential Diagnosis For macular amyloidosis is postinflammatory hyperpigmentation, atopic dermatitis, lichen simplex chronicus and fixed drug eruption. Lichen amyloidosis needs to be differentiated from lichen planus and lichen simplex chronicus. Nodular amyloidosis, clinically needs differentiation from naevus lipomatosus, lipoma, cysts, lymphoma and histologically from all forms of amorphous deposits, such as gout and nodular elastosis. • Treatment is symptomatic with topical corticosteroids (under occlusion), dimethyl sulfoxide (DMSO) and sometimes dermabrasion.

XANTHOMATOSES Xanthomas are composed of masses of lipid containing histiocytes forming papular, nodular, and plaque like lesions in the skin, tendons and sometime in the internal organs. Xanthomas are important clinical finding as they often evolve in the presence of elevated blood lipids and lipoproteins. Lipoproteins are macromolecular complexes that carry hydrophobic plasma lipids, particularly cholesterol and triglycerides, in the plasma. An elevation of serum lipid levels is called hyperlipidemia, or hyperlipoproteinemia while the term dyslipoproteinemia signifies

Fig. 21.9: Xanthelasma palpebrarum

abnormalities in serum lipoproteins, whether or not serum lipid levels are categorically elevated or not. Of clinical interest is the fact that different species of lipoproteins typically produce different types of xanthomas. Thus, the type of xanthoma observed in a particular patient provides important clues as to the type of hyperlipoproteinemia.

Xanthelasma Palpebrarum • Soft, velvety papules and plaques arranged around eyelids. • Common sites – upper eyelid, inner canthus (Figs 21.9 and 21.10) • Signify a systemic hyperlipidemia – usually LDL elevations • Usually occur in normolipidemic individuals but may be seen in familial hypercholesterolemia, dysbetalipoproteinemia, mixed hyperlipidemia. Secondary causes are obstructive liver disease, myxedema, and diabetes mellitus. • Treatment options includes surgical excision, electrofulguration, trichloroacetic acid chemical cautery , and CO2 laser.

Tuberous Xanthomas • Firm, yellow-orange, often with an erythematous halo, small papules (0.5 cm in

Fig. 21.10: Arcus juvenilis


Fig. 21.11: Tuberous xanthomas over the buttocks

Fig. 21.12: Tendinous xanthoma near the left 5th toe laterally

diameter) to lobulated tumors (2.5 cm or more) (Fig. 21.11). • Usually painless but may be tender on pressure. • Sites – knees, elbows, buttocks and pressure points, typically bilateral. • Seen with raised LDL levels – Familial hypercholesterolemia, dysbetalipoproteinemia and secondary hyperlipidemias (hypothyroidism, chronic biliary disease)

Tendinous Xanthomas • Slowly enlarging subcutaneous nodules attached to tendons, ligaments, fascia and periosteum (sub-periosteal) (Fig. 21.12). • Overlying skin appears normal. • Sites – extensor tendons of hands and feet, Achilles tendon, sub-periosteal bony prominences such as malleoli and elbows • Occur in severe hypercholesterolemia with raised LDL – Familial hypercholesterolemia, less frequently in secondary hypercholesterolemia (cholestasis).

Eruptive Exanthomas • Pinhead sized asymptomatic yellow papules with a reddish base, usually fleeting in nature and appear in crops.

Fig. 21.13: Eruptive papular and tuberous xanthomas over the elbows and knees

• Sites – buttocks, shoulders and extensor surfaces of extremities (Fig. 21.13). • Occasionally, these papules may coalesce and overlie a tuberous xanthoma – tuberoeruptive xanthomas. • Associated with pure or mixed hypertriglyceridemia and a high concentration of VLDL or chylomicrons.

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Essentials in Dermatology • May occur in secondary hyperlipidemia of diabetes mellitus.

Plane Xanthomas • Yellow-orange macules or slightly palpable plaques. • May occur at any site. • Plane xanthomas over palmar creases – ‘xanthoma palmaris et striata’ – pathognomonic of Type III dysbetalipoproteinemia. • Plane xanthomas may occur in secondary hyperlipidemias (biliary cirrhosis, cholestasis, gammopathy).

• May be associated with myeloma, macroglobulinemia or lymphoma; rarely with normal plasma lipids. • 50% of patients may have hypolipidemia with low LDL levels.

Intertriginous Xanthomas • They appear as flat to slightly raised yellow dermal plaques with corrugated surface within or adjacent to finger webs (Fig. 21.14), axillae, buttocks and antecubital and popliteal fossa. • They are pathognomonic of type II hypercholestrolemia.

Generalized Plane Xanthomas • These cover large areas of the face, neck and thorax and also may involve flexures and palms.

Fig. 21.14: Intertriginous xanthomas in the finger web spaces

Table 21.1: Abnormalities of lipoprotein metabolism with their cutaneous features

Lipid phenotype

Lipoprotein phenotype (Friedrickson)

Isolated Hypercholesterolemia Familial hypercholesterolemia

IIa

Familial defective apo B100 Polygenic hypercholesterolemia

IIa IIa

Isolated Hypertriglyceridemia Familial hypertriglyceridemia

I, V

Familial lipoprotein lipase deficiency Familial apo CII deficiency

I, V I, V

Hypertriglyceridemia and Hypercholesterolemia Combined hyperlipidemia Dysbetalipoproteinemia

II b III

Cutaneous features

Tuberous xanthomas, tendon xanthomas subperiosteal xanthomas Tendon xanthomas Usually none None, occasionally eruptive xanthomas Eruptive xanthomas, plaques Eruptive xanthomas, plaques Usually none; Tuberous xanthomas, tuberoeruptive xanthomas, xanthoma striata palmaris


Cerebrotendinous Xanthomas • Rare autosomal recessive disorder with xanthomas in the tendons and the brain. • It is due to mutation in the sterol 27-hydroxylase (CYP27A) gene leading to defective conversion of cholesterol to bile acids with accumulation of cholestanol. • Severe neurologic disease and tendency to coronary artery disease. • Tendon xanthomas. • Urinary gas chromatography is the specific test for this disease. • Treated with oral deoxycholic acid to replace the bile acid pool.

Fig. 21.15: Lipoid proteinosis – infiltrated lip and buccal mucosa

LIPOID PROTEINOSIS (URBACH – WIETHE DISEASE, HYALINOSIS CUTIS ET MUCOSAE) • Lipoid proteinosis is a rare disorder characterised by infiltration of hyaline material into the skin, oral cavity and internal organs. • It is inherited as a monogenetic autosomal recessive disorder of normal chromosomal pattern and is due to mutations in the extracellular matrix protein 1 gene. • The earliest manifestation is hoarseness that develops in infancy, which can progress to complete aphonia without breathing difficulty. Examination of larynx frequently reveals infiltration, thickening and nodularity of cords. The mucosa of lip, pharynx and tongue (Fig. 21.15) may also develop firm yellow nodules giving it a cobblestone appearance. The tongue is enlarged and firm on palpation with decreased range of movements. Ankyloglossia can occur due to involvement of the lingual frenulum. Recurrent parotitis can occur. • In early stages, the skin lesions are crops of bullae, pustule, that leave behind acne like scars. Later on, patients develop hyperkeratotic wart like or nodular skin lesions over

Fig. 21.16: Lipoid proteinosis–beaded eyelid margins

dorsal aspect of hands, finger, elbows and knees, and patchy alopecia. • Deposition of hyaline material along the eyelid margins results in typical beaded papules – ‘moniliform blepharosis’. This eyelid beading is the single most typical clinical feature of this disease (Fig. 21.16). In later stages, there may be total loss of eyelashes. Further infiltration of eyelids leads to its malfunction and can cause corneal ulcer. • Neurological abnormalities include calcification in the hippocampus and falx cerebri and temporal lobe epilepsy.

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Essentials in Dermatology • Drusen of Bruch’s membrane seen in fundi in half the patients. • Sickle-shaped calcifications dorsal and lateral to sella turcica in skull X-ray films are pathognomonic. • The nature of hyaline material and underlying metabolic defect is unknown. It may represent an underlying lysosomal storage disorder with single or multiple enzyme defects, others have postulated it to be a disorder of collagen metabolism or collagen synthesis. • Distinctive histologic features include extreme dilation of blood vessels and thickening of their walls, progressive hyalinization of sweat glands, and infiltration of the dermis and subcutaneous tissue with extracelluar hyaline deposits and also demonstrable in walls of vessels. • Differentiation from erythropoietic protoporphyria may be difficult histologically. Xanthomatoses and amyloidosis can be excluded histologically. In adults, differential diagnosis is from lichen myxoedematosus and myxedema with hoarseness have to be considered. • There is presently no effective therapy for lipoid proteinosis. Hoarseness may be relieved temporarily by surgical removal of vocal cord infiltrates. Facial lesions may be treated by dermabrasion, chemical skin peeling and blepharoplasty. Treatment by oral dimethyl sulphoxide has been claimed to be successful.

PHRYNODERMA • Phrynoderma is a type of follicular hyperkeratosis typically seen in vitamin A deficiency. • This eruption has also been associated with deficiencies of vitamin B complex, C, and E, calories, and essential fatty acids. • The morphology of these lesions is variable and may range from filiform papules to small

Fig. 21.17: Phrynoderma hyper keratotic follicular papules over the extensor aspect of the forearm

conical papules to large papules with large horny centers. They may be of the same color as the surrounding skin or may be slightly hyperpigmented. Elbows (Fig. 21.17), knees, anterolateral thighs, posterolateral superior forearms, extensor aspect of limbs, shoulders, abdomen, back and buttocks are the sites of involvement. • Differential diagnosis includes keratosis pilaris and Darier’s disease. • The treatment is oral vitamin A 100,000 IU/ day for 2-3 days followed by the recommended dietary requirements.

PELLAGRA • Pellagra is a nutritional disorder that occurs due to deficiency of niacin or tryptophan or both. Therapy with isoniazid, 5-fluorouracil, 6-mercaptopurine may provoke pellagra. Rare causes are carcinoid tumors, and Hartnup disease. • The term pellagra is derived from the Italian words ‘pelle agra’ meaning rough skin. • It is still endemic in areas of Africa and Asia due to poor nutrition and intake of certain cereals such as maize and jowar (Indian millet) as staple diet.

Metabolic and Nutritional Disorders • In the present day context, in western world, pellagra is confined to individuals who have improper food intake such as psychiatry patients, alcoholics and recluses. • Pellagra is a clinical syndrome characterised by (1) symmetrical photosensitive skin eruption (2) gastrointestinal manifestations (3) neurological and psychiatric disturbances. These well-known group of symptoms are traditionally remembered as pellagra’s four D’s -dermatitis - diarrhea - dementia and when untreated, death - is very seldom seen. But most of the manifestations are borderline and/ or less typical in nature. • The initial manifestation is an erythematous, photosensitive pruritic rash that occurs on the dorsa of hands. • The usual sites affected are the face, neck and dorsal surfaces of hands, arms and feet. • The dorsa of the hands are the most frequent site from where it may extend up to arm to

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form the ‘glove’ or ‘gauntlet’ of pellagra (Figs 21.18 and 21.19). The dermatosis is strikingly symmetrical and clearly demarcated from the normal skin. The feet is commonly involved and it may also affect the front and back of the leg to form a boot (Fig. 21.20). In the face, an erythematous rash extending from the nose to the cheeks, chins, lips, may resemble lupus erythematosus “Butterfly rash”. Rarely eyelids and ears may be affected. Facial rash usually occurs concurrently with lesions elsewhere. This eruption forms a broadband or collar around the neck, known as Casal’s ‘necklace’ (Fig. 21.19). In many instances, the necklace has an anterior continuation, also known as `cravat’. Differential diagnosis includes drug eruption, photodermatitis, lupus erythematosus, and actinic reticuloid. Classical pellagra responds dramatically to oral administration of nicotinamide or niacin (nicotinic acid) 100-300 mg/day in three divided doses. The mental changes disappear within 24-48 hours but skin lesions may take 3-4 weeks to disappear.

Fig. 21.19

Figs 21.18 and 21.19: Pellagra–chest and upper limb shows Casal’s necklace, and glove of hyperpigmented flaking dermatosis

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Fig. 21.21: Ariboflavinosis–erythema and scaling of nasolabial folds

Fig. 21.20: Pellagra—”boot” of pellagrus dermatosis affecting lower legs

ARIBOFLAVINOSIS (ORO-OCULO-GENITAL SYNDROME) • This syndrome occurs due to vitamin B2 (Riboflavin) deficiency and is characterized by mucocutaneous lesions of angular stomatitis, cheilosis, glossitis, seborrhoeic dermatitis (especially around the nose) (Fig. 21.21), scrotal (Fig. 21.22) and vulvar dermatitis, and increased corneal vascularity. • It usually responds promptly and dramatically to riboflavin supplementation with 5 to 15 mg of riboflavin daily for 2 weeks and correction of dietary errors.

ACRODERMATITIS ENTEROPATHICA • Acrodermatitis enteropathica, an autosomal recessive disorder which appears to be due to defective absorption of zinc from the gastrointestinal tract. • It may be a presenting sign in cystic fibrosis or AIDS.

Fig. 21.22: Ariboflavinosis–scrotal dermatitis

• It manifests insidiously between the ages of three weeks and 18 months (often when the baby is switched from breast milk to cow’s milk) with periorificial (mouth, nose, eyes, ears, and perineum) (Figs 21.23 to 21.25) and acral (extensor surfaces of the major joints, fingers, and toes) dermatitis, alopecia and diarrhea (Mnemonic DAD to remember its clinical features).


Fig. 21.23: Acrodermatitis enteropathica– perioral dermatitis

• The primary cutaneous eruption is vesicobullous, which is symmetrical and grouped. The lesions soon evolve into erosive vesicobullous eruption or psoriasiform patches. Secondary infection of skin lesions with Staphylococcus aureus is common and impairs wound healing. • At the same time or shortly afterward, diffuse loss of hair and gastrointestinal disturbances manifest chiefly by diarrhea, occur. • Mental depression, listlessness, loss of appetite, perleche, photophobia, and blepharitis may occur during exacerbations. • Differential diagnosis includes atopic dermatitis, seborrheic dermatitis and psoriasis. • Laboratory verification of deficient plasma or serum zinc levels may be undertaken where facilities exist, otherwise, all these cases respond to zinc sulphate / gluconate (1 to 2 mg/kg body weight/day) given once

Fig. 21.24

Fig. 21.25 Figs 21.24 and 21.25: Acrodermatitis enteropathica—perianal psoriasiform dermatosis

or twice daily. Skin lesions heal within one to two weeks. Diarrhea ceases and variability with depression of mood improves within 24 hours.

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Genetics and Genodermatoses

GENETICS IN DERMATOLOGY Human genetics is the study of the range of biological variations in human beings with each individual showing considerable variability in his/her expression of disease. Human genome, the genetic material is packaged into units called chromosomes (Chromo-colored, somes-bodies). Each somatic cell contains 46 including 2 sex chromosomes. The karyotype of an individual identifies the number and structure of chromosomes. This is usually derived from peripheral blood cells (T lymphocytes) that are stimulated to divide. They are classified by the position of the centromeres and the proportion of long and short arms. Phenotype is the bodily manifestation of genotype.

Congenital is one that is present at/before birth, not necessarily genetically determined.

Genetic Principles Gene is the sequence of bases on DNA that code for one polypeptide. Locus is the precise position of the gene on chromosome. Alleles are genes at a single locus, which may be heterozygous or homozygous. Dominant means full effect in heterozygous state. Expression means effects of a gene are variable. Penetrance is the frequency with which a gene produces an effect. If trait is non-penetrant, expressivity is zero.

Genodermatoses are genetically determined skin disorders, course of events altered little by environmental agents with single gene disorders.

Genetic heterogeneity means similar/identical phenotypic features associated with genes at different loci, e.g. albinism, ichthyosis, cutis laxa, etc.

Familial means a condition more common in relatives of an affected individual than in general population.

Phenocopy means environmental causes mimicking genetic diseases.

Inherited means that disorder is transmitted from one generation to next.

Pleiotropy means multiple phenotypic effects due to primary action of an abnormal genotype, e.g. pachyonychia congenita, neurofibromatosis.

Genetics and Genodermatoses Mutation occurs due to point substitution or deletion and may be somatic or gamete mutation. Mosaicism relates to an individual with 2 or more cell lines of different genotypes derived from a single zygote.

Autosomal Dominant Inheritance In this mode of inheritance, the gene locus is on an autosome and the trait is transmitted from generation to generation. • Both males and females are affected in equal proportions • Affected individuals are heterozygous • Every affected person will have affected parent • 50% of the children will be affected • Severity varies considerably in the family • Few show lack of penetrance. Examples: Ichthyosis, tuberous sclerosis, neurofibromatosis. Autosomal Recessive Inheritance In these pedigrees, the trait does not appear in successive generations. Parents appear entirely normal. • Both males and females are affected with equal frequency • Affected individuals are homozygous (means one copy received from each parent “double dose of the gene”) • One fourth or 25% of children are affected • No family history may be there • High frequency of consanguinity can be expected • Disorders are very severe. Examples: Xeroderma pigmentosum, phenylketonuria, etc. X-linked Recessive Inheritance In these conditions, the trait does not appear in successive generations. • Males are uniquely affected, whereas female siblings are carriers of the trait. Affected males are in different generations

• Females are healthy carriers, pass on disease to ½ of their sons and ½ of their daughters are carriers • All of the daughters of an affected male are obligate carriers of the trait • Mother’s brothers are affected • Family history always not positive. Examples: Fabry’s disease, Menkes-Kinky’s disease.

X-linked Dominant Inheritance Successive generations show the trait. • Females are predominantly affected • There is family history of recurrent losses of pregnancy early on. Although the losses may not be identified, it is often the case that the male fetus is preferentially aborted. • Hemizygous are males and heterozygous females. In most X-linked dominant traits the hemizygous male is more severely affected than the heterozygous affected female • Affected males transmit disorder to all his daughters, but none of his sons • Affected females transmit disease to ½ of their sons and ½ of their daughters. Example: Incontinentia pigmenti. Multifactorial Traits In single gene traits, transmission follows Mendel’s classical laws. In multifactorial inheritance, often referred to as polygenic inheritance, a definite familial tendency for development of the trait exists but the proportion of affected relatives is much less than expected for a single gene trait. Multiple genes modified by environmental factors influence the expression of the characteristic. Genetic Counseling Genetic counseling is the process undertaken to help patients to better understand the genetic basis of a medical problem and to plan for the future. By this definition, genetic counseling

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Essentials in Dermatology includes helping the patients to comprehend the medical facts, including the diagnosis, the probable course of the disorder and available management, the hereditary basis of the disorder, and the risk of recurrence in specific relatives. The counseling also includes a discussion of the course of action that seems appropriate in view of the risk and the family goals.

Symbols in Common use in Preparation of a Family Pedigree

PHAKOMATOSES (NEUROCUTANEOUS SYNDROMES) Phakomatoses or neurocutaneous syndromes are a rare group of disorders including neurofibromatosis, tuberous sclerosis, von HippelLindau syndrome, and Sturge-Weber syndrome.

Neurofibromatosis Type 1 (NF-1) • Neurofibromatosis type1 (NF-1), also known as von Recklinghausen’s disease, classic neurofibromatosis, or peripheral neurofibromatosis is the most common form of neurofibromatosis. • The NF1 gene responsible codes for “neurofibromin” on chromosome 17. • The clinical expression of NF-1 is highly variable. Roughly, half of NF-1 cases arise via spontaneous mutation and are not associated with any family history. • NF-1 is a multisystem disorder characterized by cafe-au-lait macules (CALMs), neurofibromas, Lisch nodules, optic gliomas, bony dysplasia, intertriginous freckling and autosomal dominant inheritance. • Neurofibromatosis is named after neurofibroma tumors. There are three types of neurofibromas: cutaneous, subcutaneous and plexiform. A single plexiform neurofibroma or two of any type are considered diagnostic of NF-1. Cutaneous neurofibromas (mollusca fibrosa) are soft lilac –pink tumors, sessile and dome shaped, sometimes pedunculated, most numerous on the trunk and limbs, hundreds may be present, ranging from a few millimeters to several centimeters in diameter. They can be easily pushed into underlying dermal defect with light digital pressure - “button holing”. When pressed, the soft tumors tend to invaginate through a small opening in the subcutaneous tissue, giving the feeling of a seedless raisin or a scrotum without a testicle (Fig. 22.1). The subcutaneous type may feel hard like a pencil eraser. They often cause localised pain or tenderness. On the other hand, plexiform neurofibromas are congenital and are pathognomonic for NF-1 (Figs 22.2 and 22.3). They feel like a “bag of worms” because of the many interdigitating

Genetics and Genodermatoses elements and can be thought of as combination of cutaneous and subcutaneous types. They present as a diffuse elongated fibroma along the course of the nerve, frequently involving the trigeminal or upper cervical nerves.

Fig. 22.3: Neurofibromatosis type 1—plexiform neurofibroma affecting foot

Fig. 22.1: Neurofibromatosis type 1 – multiple neurofibromas over the trunk

Fig. 22.2: Neurofibromatosis type 1 – plexiform neurofibromas affecting both lower limbs

Neurofibromas of the female areola and nipple are virtually pathognomonic for NF-1. • Cafe-au-lait macules (CALMs), another manifestation of NF-1, are discrete, well circumscribed, round or oval, uniformly pigmented patches (may be seen in normal population, segmental neurofibromatosis, familial CALMs, tuberous sclerosis, Mc Cune Albright syndrome, ataxia telangiectasia, Bloom’s syndrome, Watson’s syndrome, Rubinstein-Taybi syndrome) (Figs 22.4 and 22.5). Solitary CALMs are a common finding, affecting up to one third of normal children. Multiple CALMs are rare, particularly in the white population. A diagnosis of multiple organ disorder, the most common being NF-1, should be considered in this instance. The minimal number of 6 lesions of CALMs was established as a criterion for the diagnosis of NF-1 (more than 1.5 cm in diameter in adults). They are the first feature of the disease to appear in all children. • Freckling is a useful and often overlooked sign of NF-1. Freckling involving areas of hyperpigmentation up to 2 or 3 mm in diameter occurs frequently in the axilla as well as other intertriginous regions. Basically, there are two kinds of neurofibromatosis freckles—those that are basically very small CALMs ordinarily present at birth or in the

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Essentials in Dermatology • Systemic features include: 1. Skeletal manifestations include bony dysplasias especially tibia, pseudoarthrosis, cysts and scoliosis of thoracic region. 2. CNS manifestations—Approximately 50% of children with NF-1 exhibit a learning

Fig. 22.4: Neurofibromatosis type 1 – circumscribed pigmented patch of café-au-lait macule over the trunk

Fig. 22.5: Neurofibromatosis type 1 – back showing neurofibromas and café-au-lait macules

first year of life and distributed over the entire body and those that develop later in intertriginous regions (Crowe’s sign) (Fig. 22.6). Multiple melanotic macules of palms with varying size from 2 to 4 mm may be noted in 90% of Indian cases (Fig. 22.7). This sign has been named as ‘Patrick Yesudian sign’ by various medical schools, in south India. • Lisch nodules are pigmented iris hamartomas which appear as dome shaped lesions on the surface of iris. They are one of the most common manifestations of NF-1 and have significant diagnostic value.

Fig. 22.6: Neurofibromatosis type 1—axillary freckling

Fig. 22.7: Neurofibromatosis type 1—palmar freckling “Patrick Yesudian sign”

Genetics and Genodermatoses disability, as well as attention deficit disorder. More severe developmental delay, including mental retardation occurs in about 5%. Other features of NF-1 include macrocephaly, short stature, and hypertension (due to renal artery stenosis or rarely pheochromocytoma). 3. Endocrine disturbances of many types may be associated. • Diagnosis: The spectrum of clinical findings in NF-1 is broad, but only six features constitute the seven established diagnostic criteria. These criteria were described at a National Institutes of Health (NIH)—sponsored consensus conference in 1987. These were updated in 1990. In the absence of a single “test” that can confirm the diagnosis, physicians often rely on these criteria. Two criteria are required for a definitive diagnosis and one is required for a presumptive diagnosis. 1. Six or more cafe-au-lait macules (CALM’s) measuring 5 mm or more before puberty or 15 mm or more after puberty; 2. Axillary or inguinal freckling (Crowe’s sign), 3. Two or more neurofibromas or a plexiform neurofibroma; 4. Two or more Lisch nodules; 5. Optic nerve glioma; 6. Characteristic skeletal dysplasias (tibial or orbital dysplasia) and 7. Affected first degree relative. • Three of these criteria manifest themselves on to the skin (A single plexiform neurofibroma or two of any type, minimal number of 6 lesions of CALMs, and axillary frecklingCrowe’s sign). It is often the dermatologist who suggests or confirms the diagnosis based upon cutaneous manifestations. • Management consists of genetic counseling, anticipatory guidance, and surveillance for complications. Tests such as MRI or X-rays

should be done for clinical indications only. Children should be monitored for learning disabilities and provided cognitive and educational assessments as needed. Physical examinations should include measurement of height, weight, head circumference, and blood pressure. Ophthalmologic check up is a must in all cases. Surgery is indicated in only select cases. • Risk of malignancy: NF-1 is also associated with increased risk of malignancy (malignant peripheral nerve sheath tumors in second or third decades). • Prognosis: Variable, dependent upon severity of involvement and development of malignancy. Cosmetic disfigurement is progressive and worsens with time. Mild course during childhood is not a guarantee for mild disease in adulthood.

Tuberous Sclerosis (Bourneville’s Disease) • It is now frequently designated the tuberous sclerosis complex (TSC). It is an autosomal dominant human genetic disease characterized by widespread hamartomas, usually, occurring in the brain, eyes, skin, kidneys, liver, heart, and lungs. • This disorder derives its name from a description of its cerebral lesions by Bourneville in 1880. Its systemic nature was described by Vogt in the clinical triad (Epiloi-a) of epilepsy (seizures),low intelligence (mental retardation) and facial lesions termed adenoma sebaceum (a misnomer for angiofibromas). Recent studies have shown that full triad was evident in only about one third of patients. • Two third cases are sporadic and one third are autosomal dominant. • Two genetic loci have been identified TSC1 (Hamartin) and TSC2 (Tuberin) on chromosome 9 and 16 respectively. These are

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tumor suppressor genes, which when deficient result in Mtor disinhibition and abnormal proliferation of tissues resulting in hamartomas. Cutaneous features are the most frequent finding in TSC and if overlooked, will lead to a delay in diagnosis. Although, there is considerable variation in the age of expression of all the skin lesions, there is a trend towards the earlier expression of hypomelanotic macules and forehead plaques compared with facial angiofibromas and ungulal fibromas. Shagreen patches are usually present by puberty. The relatively vascular and fibrous components of adenoma sebaceum (angiofibromas) determines their clinical appearance (Fig. 22.8). They range from white or flesh colored to classical red pink papules, 1-10 mm in diameter, symmetrically distributed over the nasolabial folds, cheeks and chin, sparing the upper lips. They are regarded by many as a primary pathognomonic feature of TSC. Shagreen patches are not as diagnostically useful as facial angiofibromas. They most often appear as flat, slightly elevated areas of the skin, soft and skin colored plaques of variable size (1 to 10 cm) with a “pig skin”, “elephant skin” or “orange peel appearance”. Usually, these lesions are localized asymmetrically over the dorsal body surfaces, particularly over the lumbosacral area (Fig. 22.9). Periungual fibromas (Koenen’s tumors) common in adult patients with TSC, are much less frequent in children. They usually appear around puberty as smooth, firm, flesh colored excrescences and are usually 5-10 mm in length. They are located around or under the nail plate and arise from the bed under the nail plate or from skin of nail groove (Fig. 22.10). Regarded as angiofibromas, they are

classified as primary or pathognomonic feature of TSC. • Ash leaf macules/spots (hypomelanotic macules) are the most frequent lesions in TSC patients. Since, they resemble the leaf of European mountain ash tree, they are called as ash leaf spots. They are ovoid or leaf shaped white macules varying in size from 1-3 cm (Fig.

Fig. 22.8: Tuberous sclerosis – angiofibromas and forehead plaque seen over the face

Fig. 22.9: Tuberous sclerosis – “orange peel appearance” of a circumscribed area in the lumbosacral area – Shagreen patch


Fig. 22.10: Tuberous sclerosis – periungual fibromas, also known as Koenen’s tumors

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22.8) and may be the only skin lesions in infants and if associated with infantile spasms, strongly suggest the diagnosis of TSC. These ash leaf spots are predominantly distributed on the trunk and buttocks or limbs. Forehead fibrous plaque is classified histologically as an angiofibroma, although clinically it differs from the typical papulonodular angiofibroma because of fibromatous appearance (Fig. 22.5). It is one of the secondary features of tuberous sclerosis. Molluscum fibrosum pendulum (skin tags) are commonly seen in normal elderly people and are uncommon in adolescents and young adults, and should alert to the possibility of TSC. In individuals with TSC, molluscum fibrosum pendulum is commonly seen on the neck, groin, axillae and near flexures of limbs, especially in adults. Café-au-lait macules are not regarded as characteristic for TSC and are not included in the diagnostic criteria (Fig. 22.8). Systemic features include: 1. Neurological findings: Tuberosclerotic nodules of glial proliferation occur in cerebral cortex, basal ganglia, and ventricle wall (60-70%) and are causes of mental retardation. Once calcified, these lesions are visible on skull radiographs as

brain stones. Epilepsy is common, usually begins in infancy as infantile spasms or salam attacks. Autism, attention deficit disorder are other features. 2. Ocular: Retinal phakomas (lump of mulberry appearance) represent proliferation of astrocytes. Hypopigmented spots in iris may be seen. 3. Cardiac: Rhabdomyomas may be present at birth and regress spontaneously in first few years. 4. Renal: Polycystic kidney disease, isolated renal cyst, angiomyolipomas, renal cell carcinoma. 5. Pulmonary: Multifocal micronodular pneumocyte hyperplasia, pulmonary cyst, lymphangiomyomatosis. 6. Gastrointestinal: Hamartoma and polyposis of stomach, intestine and colon. • Investigations include: – X-ray (skull, hands and feet, lungs); – CT scan brain; – MRI (brain parenchymatous lesions); – EEG (seizures); – Renal ultrasonogram; – ECHO heart (rhabdomyomas); – Neurodevelopmental testing; – Opthalmological examination. Diagnositic Criteria (Tuberous Sclerosis Alliance, Consensus Conference 1998) Major features 1. Facial angiofibromas or forehead plaque 2. Non-traumatic ungual or periungual fibroma 3. Hypomelanotic macules (more than three) 4. Shagreen patch (connective tissue nevus) 5. Multiple retinal nodular hamartomas 6. Cortical tuber 7. Subependymal nodule 8. Subependymal giant cell astrocytoma, 9. Cardiac rhabdomyoma, single or multiple 10. Lymphangiomyomatosis 11. Renal angiomyolipoma.

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Essentials in Dermatology Minor features: 1. Multiple randomly distributed pits in dental enamel 2. Hamartomatous rectal polyps 3. Bone cysts 4. Cerebral white matter migration lines 5. Gingival fibromas 6. Non-renal hamartoma 7. Retinal achromic patch 8. “Confetti” skin lesions 9. Multiple renal cysts. Definite TSC: Either 2 major features, or 1 major feature with 2 minor features. Probable TSC: 1 major feature and 1 minor feature. Possible TSC: Either 1 major feature, or 2 or more minor features. • Treatment: 1. Facial angiofibromas: Dermabrasion, electrodessication, CO2, argon or pulsed dye laser 2. Seizures: For infantile spasms: ACTH/ steroids are useful. Seizures in children: vigabatrin is the drug of choice. • Prognosis is related to the extent of systemic involvement. Cardiovascular complications occur in 1st decade, brain tumor in 2nd decade, renal and pulmonary lymphangiomatosis in 4th decade are cause of morbidity and mortality.

Von Hippel-Lindau Disease (VHL) • VHL also referred to as CNS angiomatosis is inherited in an autosomal dominant fashion with incomplete penetrance with VHL gene located on chromosome 3p25. Both sexes are equally affected. Hippel first described retinal angiomatosis in 1904. Lindau subsequently recognized association with central nervous system tumors and hence the eponym von Hippel Lindau disease was coined.

• Cutaneous findings are portwine stains and café-au-lait macules. Dermal capillary malformation has predilection for the head and neck. Other manifestations are vascular malformations in the cerebellum and brain stem. Retina is also commonly affected. There may be cystic neoplasms or angiomatous lesions in the kidneys, liver and pancreas.

Sturge-Weber Syndrome (Encephalofacial angiomatosis) • Sturge -Weber syndrome (SWS) is defined as facial portwine stain in association with ipsilateral plial (i.e. leptomeningeal) vascular anomalies (with one or more symptoms; epilepsy early in life, hemiparesis or hemiplegia, gyriform intracranial calcifications and cerebral atrophy) and inconstant ipsilateral choroidal vascular lesions with glaucoma. • Portwine stain is usually unilateral, roughly involving the areas supplied by ophthalmic and maxillary divisions of trigeminal nerve, and bilateral in 50% of cases. • Portwine stains (naevus flammeus) are congenital vascular birthmarks that are present at birth and persist into adulthood. At birth, they are often pale pink macular lesions which with time, progress to become dark red to purple (Fig. 22.11) and even nodular. These changes occur as a result of progressive ectasia of cutaneous superficial vascular plexus. • Portwine stains can either occur as isolated cutaneous or be associated with structural abnormalities especially of those underlying the birth mark such as the choroidal vessels in the eye which produce glaucoma and leptomeningeal vessels in the brain which causes seizures, then is known as SWS.


Fig. 22.11: Sturge Weber syndrome—port wine nevus seen along ophthalmic and maxillary division of trigeminal nerve

• Freckles appear over face and hands, and later on neck, legs, lips and conjunctiva. Eventually freckles become permanent and progressively increase in number. • Continued sun exposure causes skin to become dry and parchment-like with pigmentation (hence the name xeroderma pigmentosum). • Next is the poikilodermatous stage characterized with atrophy and telangiectasia superadded to the existing freckles and hyperpigmentation (Fig. 22.12). • Superficial ulcers and atrophy may leave scars and contractures. • Ocular features include – photophobia with conjunctival injection, symblepharon, ectropion / entropion and loss of eye lashes due to atrophy of eyelid skin, keratitis leading to corneal opacity, pterygium and ocular neoplasms.

Xeroderma Pigmentosum (Pigmented Dry Skin) It is an autosomal recessive disorder characterized by photosensitivity, pigmentary changes, premature skin aging, neoplasia and abnormal DNA repair. • Eight different subtypes – complementation groups A to G and XP variants. • Main defect is in the DNA excision repair process (this is a process whereby damaged DNA is replaced with new DNA). • XP- variants have a normal nucleotide excision repair but the defect here is of a reduced molecular weight of newly synthesized DNA in UV radiated cells. Clinical Features • Skin normal at birth. • Earliest symptoms of dryness and freckling appear between six months and three years of age.

Fig. 22.12: Xeroderma pigmentosum—freckle like pigmentation with atrophy, telangiectasia and actinic keratoses

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Fig. 22.13: Xeroderma pigmentosum—freckle like pigmentation with fungating squamous cell tumor of the face and scalp

• Pre-malignant lesions like actinic keratosis and keratoacanthomas may occur in most cases. • Patients with XP under 20 years of age have a greater than 1000-fold increased risk of cutaneous basal cell or squamous cell carcinoma or melanoma (Fig. 22.13). The median age of onset of nonmelanoma skin cancer reported in patients with XP is 8 years. • Overall, there is a ten to twenty-fold increase in internal neoplasms (central nervous system, lung, gastric, breast, renal, etc.) in XP. • 20% have neurological complaints – mental retardation, areflexia, spasticity, ataxia, and sensorineural deafness. • Disease is often fatal under 10 years of age and two thirds of the cases die by 20 years of age. • De Sanctis –Cacchione syndrome consists of xeroderma pigmentosum, with microcephaly,

mental deficiency, dwarfism, hypogonadism, choreoathetosis, and ataxia. • Prenatal diagnosis by amniocentesis is possible. • Differential diagnosis includes ordinary freckling, other causes of photosensitivity and premature ageing syndromes such as Rothmund-Thomson syndrome, Bloom syndrome, Cockayne syndrome, progeria, and acrogeria. • Treatment: The mainstay of management is by ensuring maximum photoprotection (clothing, topical and systemic sunscreens, UV filter glasses). Ocular symptoms should be managed promptly to prevent complications. Early and adequate excision of all tumors is essential and topical 5-fluorouracil may be used for pre-malignant lesions. Oral retinoids have been found to decrease the occurrence of skin cancers in this condition.

Incontinentia Pigmenti (Bloch-Sulzberger Disease) • Incontinentia pigmenti is an uncommon genodermatosis of the developing neuroectoderm in which vesicular, verrucous and pigmented lesions are associated with developmental defects of eye, skeletal system and central nervous system. • Incontinentia pigmenti is a complex hereditary syndrome that principally affects female infants. It is inherited as an X-linked dominant disorder, caused by mutation of NEMO gene on chromosome Xq28. The gene is generally lethal in male fetuses. • This multisystem disorder has manifestations of dermatological, neurological, skeletal, ocular or dental origin. • It manifests at birth or during first weeks of life. • In the skin, the disorder characteristically progresses through four stages. The first stage


Fig. 22.14: Incontinentia pigmenti – linear whorled vesicular eruption along Blashko’s lines over the lower limbs

(vesicular phase) is characterized by linear whorled vesicular eruption along Blaschko’s lines (Fig. 22.14). The eruption typically favors acral locations. Peripheral leucocytosis and

eosinophilia may occur during this stage. This stage is followed 2 to 6 weeks later by verrucous or lichenoid lesions on the sites of the former vesicular eruption in 30% of patients. The third stage (pigmentary phase) starts between 12th and 20th week and is characterized by hyperpigmented lesions. A fourth stage, rarely seen in some adult females is characterized by faint hypochromic or atrophic linear macules over extremities. The hair is usually normal, but in 25% of cases, cicatricial alopecia may be seen. • Incontinentia pigmenti achromians (hypomelanosis of Ito) suggest the negative image of incontinentia pigmenti characterized by unilateral or bilateral hypopigmentation along the lines of Blaschko. • Usually, no treatment is necessary other than the control of secondary infection.

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Skin in Systemic Diseases

Hormones secreted by endocrine glands have physiological effects on pigmentation, hair growth, sebaceous glands and connective tissue. Endocrine disorders are associated with various cutaneous manifestations which offer clue to the diagnosis of the underlying endocrine disorder.

sarcoidosis, or iatrogenic) leading to multiple hormone deficiency. Cutaneous manifestations are non-specific and include xerotic skin, fine wrinkling around eyes and mouth, pallor, generalized hypopigmentation, increased sunburn tendency, thinning of scalp and body hair, reduced sweat and sebaceous gland activity and thin brittle nails.

SKIN IN PITUITARY DISORDERS

SKIN IN ADRENAL SYNDROMES

Acromegaly: It results from excessive secretion of growth hormone by pituitary adenomas (98% of cases) in adolescents or adults. Patients are tall and have prognathism, frontal bossing, elongated, blunt and thickened fingers. Dermatological features include thickening of skin, accentuation of facial, neck and scalp creases imparting corrugated appearance (cutis verticis gyrata), widened triangular shaped nose, large ears, large and protruding lower lip, edematous thick eyelids, macroglossia, numerous skin tags (fibroma molluscum), seborrhea, acne, hyperhidrosis, hyperpigmentation, acanthosis nigricans, coarse scalp and body hair, wide and thickened nails.

Cushing’s syndrome and Cushing’s disease: Cushing’s disease refers to hypercortisolism (glucocorticoid excess) from ACTH overproduction, most commonly by pituitary adenoma, whereas Cushing’s syndrome refers to hypercortisolism, resulting from any cause, such as pituitary ACTH overproduction, ectopic ACTH production, or from adrenal adenoma, carcinoma or hyperplasia, or from glucocorticoid therapy. Skin is atrophic, smooth and transparent (paper thin), and there is facial fullness and plethora (moon facies), buffalo hump (Fig. 23.1), fullness of supraclavicular fossa, truncal obesity and relative lack of fat in lower extremities “lemon on stick appearance”, poor wound healing, striae particularly over abdomen, arms, and thighs, purpura, petechiae, easy bruising and purplish mottling (cutis marmorata), acneiform eruptions, hirsutism,

SKIN IN ENDOCRINE DISORDERS

Panhypopituitarism: It results from destruction of pituitary gland from various causes (post partum hemorrhage “Sheehan’s syndrome”, adenoma, craniopharyngioma, tuberculosis,


Fig. 23.1: Cushing’s syndrome—buffalo hump

Fig. 23.2: Addison’s disease—hyperpigmentation of palmar creases

male pattern baldness in females, addisonian like pigmentation, and increased incidence of fungal infections.

Pheochromocytoma: It is a rare adrenal tumor (mostly benign). Main clinical feature is hypertension, but can lead to flushing episodes.

Addison’s disease (primary adrenal insufficiency): It refers to insufficient secretion of adrenocortical hormones mainly cortisol and mineralocorticoids due to destruction of adrenal glands. Various causes of adrenal gland damage are autoimmune disorders in 70% of cases, tuberculosis, fungal infections, sarcoidosis, metastasis, and hemorrhage. Secondary adrenal insufficiency results from hypothalamic or pituitary diseases leading to decreased ACTH secretion, or steroid withdrawal after long term use. General features of Addison’s disease are fatigue, dizziness, anorexia, nausea, vomiting, diarrhea, abdominal pain and hypotension. Cutaneous findings are hyperpigmentation of skin due to elevated ACTH and MSH, mainly over light exposed areas, elbow, knee, knuckles, axillae, areolae, umbilicus, genitalia, palmar creases (Fig. 23.2), tongue and mucous membranes, scars, and nails. Fibrosis and calcification of ear may occur. Hyperpigmentation is absent in secondary adrenal insufficiency (referred to as “white Addison’s disease).

SKIN IN THYROID DISEASES Hyperthyroidism It may be a manifestation of Grave’s disease (most common cause), toxic multinodular goiter, adenoma, Hashimoto’s thyroiditis (early stage) and iatrogenic. Grave’s disease: It is an autoimmune disease characterized by antithyroid antibodies such as long acting thyroid stimulator (LATS). Skin is warm, moist and smooth, and there is facial flushing, palmar erythema, generalized hyperhidrosis more so on palms and soles, pruritis, urticaria, addisonian hyperpigmentation, hyperpigmentation of eyelid (Jellinek’s sign), and thyroid swelling in the neck. Scalp hair become fine, soft and friable, telogen effluvium can occur. Nails grow rapidly, become thin and soft, develop distal onycholysis (Plummer’s nails). In addition to these features of hyperthyroidism, patients of Grave’s disease may have pretibial myxoedema, thyroid acropachy and exophthalmos (Diamond’s triad).

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Fig. 23.3: Pretibial myxoedema—bilateral firm hyperpigmented plaques with orange peel texture over shins

Pretibial myxoedema is characterized by bilateral asymmetric, firm, hyperpigmented, plaques or nodules, with orange peel texture (peau d’orange) mainly over shins and feet due to cutaneous accumulations of glycosaminoglycans (Fig. 23.3). Localized hypertrichosis and hyperhidrosis can occur over these lesions . Thyroid acropachy is characterized by digital clubbing, soft tissue swelling of hands and feet, and diaphyseal proliferation of periosteum in acral and distal long bones (tibia, fibula, ulna and radius). Exophthalmos is due to orbital deposits in orbital fossa, always bilateral. Autoimmune diseases such as vitiligo, alopecia areata can occur in association with Grave’s disease.

Hypothyroidism (Myxoedema) Deficiency of thyroid hormone may be caused by Hashimoto’s thyroiditis, iodine deficiency, iatrogenic following total thyroidectomy,

radioiodine therapy, drugs such as lithium, congenital hypothyroidism and secondary hypothyroidism due to reduced TSH from pituitary. The dermal manifestations are mainly due to accumulation of mucopolysaccharides (chondroitin sulphate and hyaluronic acid) in the skin. Skin appears rough, cool, dry, swollen, waxy and pale with increased skin creases, and there can be reduced sweating, asteatotic eczema, pruritis, and palmoplantar keratoderma (palms and soles, yellow-orange due to carotenemia). Patient may have characteristic expressionless facies with puffy and drooping eyelids, broad nose, swollen lips, and macroglossia. There is delayed wound healing, purpura, ecchymosis and xanthomatosis may occur. Nails grow slowly, become thick and brittle with longitudinal and transverse striations. Scalp and body hairs are sparse, coarse, dry and brittle, tend to fall out easily resulting in diffuse or partial alopecia. Loss of lateral third of eyebrows (madarosis) -Hertoghe’s sign In juvenile hypothyroids, waxy yellowish skin change is more prominent, but puffiness may be less apparent. In cretinism, infants will have periorbital puffiness, macroglossia, swollen hands and feet, cold and dry skin, cutis marmorata and umbilical hernia (pathognomonic). Unlike in adult hypothyroid, eyebrows tend to be confluent.

SKIN IN PARATHYROID DISEASES Hyperparathyroidism Primarily occurs due to adenoma, hyperplasia or carcinoma of parathyroid glands and secondary due to chronic renal failure. Subcutaneous calcifications can present as linearly arranged white papules or as infiltrating plaques usually over large joints. Subcutaneous calcification and calciphylaxis mainly occur in secondary hyperparathyroidism.


Hypoparathyroidism Congenital absence of glands (Di George’s syndrome), thyroid surgery, hemochromatosis, metastatic cancer, or idiopathic in origin may be its causes. Skin changes may be similar to that of hypothyroidism. The skin becomes dry, hyperkeratotic and puffy with sparse and coarse hair. Chloasma and pellagra like pigmentary changes can occur. Nails become opaque and brittle with transverse ridges. Impetigo herpetiformis, psoriatic flares and eczematous dermatitis have been associated with hypoparathyroidism. Normalization of serum calcium levels with calcium and vitamin D usually reverse skin abnormalities. SKIN IN DIABETES MELLITUS Diabetes mellitus (DM) is characterized by relative or absolute deficiency of insulin, leading to gross defects in glucose, fat and protein metabolism. Type I DM (Insulin dependant DM) is due to insulin insufficiency from antibody mediated destruction of beta cells of pancreas. Type II (Non-insulin dependant DM) results from hyperglycemia mainly due to peripheral insulin resistance. Nearly all patients with DM have some skin manifestations. For most manifestations the pathogenesis remains unknown, and others result from damage to vascular, neurologic or immune systems. Macro and microangiopathy contribute significantly to the cutaneous complications of DM.

Cutaneous Manifestations of DM 1. Skin Infections DM patients are at higher risk of contracting bacterial and fungal infections when compared to normal population. Staphylococcal and streptococcal pyodermas (folliculitis, furuncle,

carbuncle), malignant otitis externa (caused by Pseudomonas aeruginosa), necrotizing fasciitis, and clostridial gangrene occur more frequently in diabetics. Candida albicans is the most common pathogen, causing intertrigo, vulvovaginitis, balanitis, glossitis, angular cheilitis, paronychia and onychomycosis. Tinea pedis and onychomycosis of toe nails are common. Rhinocerebral mucormycosis, a fatal condition caused by mucor and rhizopus species occur more commonly in diabetics.

2. Markers of DM Acanthosis nigricans (AN) presents as brown to gray black papillomatous cutaneous thickening of the flexural areas including posterolateral neck (most common site), axillae, groin and abdominal folds. Affected area will have velvety appearance, and it may involve mucous membranes also. AN can also occur in association with obesity, internal malignancy and with drugs such as corticosteroids and estrogens.

Fig. 23.4: Diabetes mellitus—generalized granuloma annulare

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Essentials in Dermatology Generalized granuloma annulare presents with numerous small annular plaques formed by 1-2 mm flesh colored papules (Fig. 23.4). These lesions are symmetric and appear more commonly over the abdomen, chest, thighs and extensor of elbows, most frequently in older adults. Necrobiosis lipoidica (NL) is a cutaneous disorder, often but not always associated with diabetes mellitus. Sixty to seventy percent of patients have DM, so patients presenting with NL should be investigated for DM. More commonly affects middle aged women. The skin lesions start as skin colored or brownish red papule which evolve slowly into well demarcated waxy plaque of variable size. They have sharply defined, broad violet red or pink, elevated border and depressed yellow-orange centre with telangiectasias. Ulcerations commonly occur within the plaques and heal slowly resulting in depressed scars. Almost always affects shins, but can involve thighs, trunk, and face, scalp (scarring alopecia). Rarely disseminated NL can occur called as granulomatous disciformis chronica and progressiva. Bullosis diabeticorum is characterized by abrupt onset of bullae, mainly over the lower legs, feet, toes, and occasionally over dorsa of hands and fingers. Bullae appear over non inflamed skin, and measured from few millimeter to 3-5 cm in size, non-pruritic and painless. Healing occurs within 2 to 5 weeks and rarely leaves scar. Pathogenesis of bulla formation is unknown. Increased skin fragility due to glycosylation end products may be involved. Scleredema diabeticorum is characterized by insidious onset of painless, symmetric induration of skin and subcutaneous tissue mainly over upper back, neck, and rarely face, shoulder and anterior torso. Skin has non pitting, woody,

“peau d’ orange” quality. There is decreased sensation to pain and light touch over the affected area, and decreased range of movements in neck and upper extremities. Most patients have type II diabetes. It is due to thickening of collagen bundles and deposition of glycosaminoglycans (mainly hyaluronic acid).

3. Complications of Diabetes Macroangiopathy Atherosclerosis induced by DM can lead to ischemic gangrenous changes in legs and feet, hypothermia, dry skin and nail dystrophy.

Microangiopathy Microangiopathy causes many cutaneous manifestations, which include: 1. Wet gangrene of foot–occurs as a late complication. 2. Erysipelas like erythema–well demarcated, red areas occur on the legs or feet usually in elderly diabetics. 3. Diabetic rubeosis-peculiar rosy reddening of the face, rarely of hands and feet in long standing diabetics. 4. Diabetic dermopathy (diabetic shin spots or Binkley spots)— Most common dermatosis associated with diabetes. They start as crops of asymptomatic, oval, dull red papules 0.5-1cm in diameter, which gradually evolve into atrophic hyperpigmented lesions mainly over shins, also over forearms, thighs and bony prominences. If 4 or more are present, the specificity is high for microvascular disease in other tissues. An association seems to exist between dermopathy and other serious complications such as retinopathy, nephropathy and neuropathy.

Diabetic Neuropathy And Diabetic Foot Diabetic foot is mainly caused by peripheral neuropathy but micro and macroangiopathy and infections also contribute. Diabetic neuropathy

Skin in Systemic Diseases is usually distal, polyneuritic, sensory and motor neuropathy. Motor neuropathy causes weakness and wasting of muscles, thereby uneven pressure on feet while walking. Sensory neuropathy predisposes to trauma and autonomic involvement causes hypohidrosis. All these factors combine to produce neurotrophic ulcers mainly over bony prominences; most commonly over the ball of great toe. The ulcer is usually painless, circular and punched out surrounded by a ring of callus. Some patients present with burning sensation in the feet.

4. Skin Diseases Commonly Associated with Diabetes Eruptive xanthomas, skin tags, vitiligo, lichen planus, acquired perforating disorders such as Kyrle’s disease, reactive perforating collagenosis, uremic pruritis occur commonly in association with diabetics.

5. Complications of Diabetic Therapy Oral hypoglycemic agents may produce allergic reactions, and photosensivity. Insulin causes erythema, urticaria and lipodystrophy at injection site.

SKIN IN PRIMARY SYSTEMIC AMYLOIDOSIS Primary systemic amyloidosis involves mesenchymal tissue, the tongue, heart, gastrointestinal tract and skin. Cutaneous manifestations occur in approximately 40% of cases of primary systemic amyloidosis. The cutaneous eruptions usually begin as shiny, smooth, firm, flat topped or spherical papules of waxy color and have the appearance of transluscent vesicles because of their tenseness. These lesions coalesce to form nodules and plaques of various sizes. The regions about the eyes, nose, mouth and mucocutaneous junctions are commonly involved. Purpuric lesions and

Fig. 23.5: Systemic amyloidosis—macroglossia showing indentations of the tongue with gum hyperplasia

ecchymoses resultant of infiltration of blood vessels, chiefly affect the eyelids, limbs and oral cavity. Purpura typically occurs after trauma (pinch purpura). Purpuric lesions also classically appear after actions or procedures that result in increased pressure in the vessels of the face such as after proctoscopic examination. Glossitis with macroglossia may be an early symptom and can lead to dysphagia. The tongue becomes greatly enlarged and furrows develop. The lateral aspects show indentations from the teeth (Fig. 23.5). Diagnosis is confirmed by evaluation of patient’s serum and urine for immunoglobulin fragments and by demonstration of amyloid deposits in the skin.

SKIN IN VASCULITIS The term vasculitis refers to inflammation and necrosis of blood vessels. The vasculitides are best classified according to the size of the involved vessels into large, medium and small vessel vasculitis.

Classification of the Vasculitides A. Large Vessel Vasculitis 1. Giant cell arteritis 2. Takayasu arteritis

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B. Medium-sized Vessel Vasculitis 1. Kawasaki disease 2. Polyarterites nodosa (PAN) 3. Benign cutaneous PAN

C. 1. 2. 3. 4. 5.

Small Vessel Vasculitis Henoch-Schönlein purpura Cutaneous leukocytoclastic angiitis Microscopic polyangiitis Wegener’s granulomatosis Churg – Strauss syndrome Blood vessels have limited ways of responding to vessel wall injury. They may respond with increased permeability that leads to edema and purpura, attenuation of the vessel wall lead to aneurysm formation or hemorrhage, and intimal proliferation or thrombosis may cause stenosis or occlusion with tissue ischemia or infarction. The definitive diagnosis of a specific “vasculitis” remains dependent upon histopathologic confirmation of vasculitis in conjunction with the appropriate clinical picture and the exclusion of diseases that can cause secondary vascular inflammation such as infection (e.g., meningococcemia, gonococcemia, and endocarditis). The principal clinical clue to diagnosis of a specific vasculitic disorder is the pattern of organ involvement. Identification of qualitative pathologic changes in affected organs (pattern of necrosis, presence of granulomas, eosinophilic infiltration) further delineates the differential diagnosis. The identification of a vasculitic disorder may not always be easy because of the varied clinical presentations. More than one system is often involved and the manifestations can, therefore, be very heterogenous. However, there are some clinical pointers that may suggest the presence of a vasculitic disorder. These are1. Prolonged fever of unknown origin. 2. Suggestive skin lesions e.g. palpable purpura (Fig. 23.6), gangrene.

Fig. 23.6: Vasculitis—palpable purpuric lesions over the legs of hypersensitivity vasculitis

3. Unexplained peripheral neuropathy especially mononeuritis multiplex. 4. Arthralgia/arthritis, myositis, serositis. 5. Obscure pulmonary, cardiovascular or renal disease especially when there is multi-system involvement. 6. Laboratory parameters indicative of ongoing inflammation: e.g. leukocytosis, elevated ESR/CRP, eosinophilia, hypocomplementemia, cryoglobulinemia, circulating immune complexes. Kawasaki disease and Henöch-Schonlein purpura are the commonest vasculitides seen in children while giant cell arteritis, polyarteritis nodosa and Wegener’s granulomatosis are more common in the adults.

Giant Cell Arteritis (GCA) GCA generally affects individuals older than 50 years of age and is variably associated with fever, headache, masticatory muscle claudication, peripheral vascular disease, inflammatory aortic aneurysms, and retinal

Skin in Systemic Diseases ischemic syndromes. Cutaneous manifestations include rare cases of scalp or tongue ischemia and necrosis. The diagnosis is established by biopsy of the superficial temporal artery, which shows chronic mononuclear cell infiltrate, and giant cells.

Takayasu Arteritis (TA) TA is a chronic, idiopathic inflammatory disease primarily affecting the large vessels, such as the aorta and its branches. It is also known as ‘pulseless disease’ and ‘reverse coarctation’. TA is more commonly associated with aortic and aortic branch vessel stenoses and aneurysms than GCA. TA is the most common cause of giant cell arteritis in young patients. It mainly affects females in the age group of 10-30 years. The precise etiology of TA is still unknown. The most common complaints at the onset of the disease include headache, dyspnea, palpitations, arthralgia/ arthritis and myalgia. Constitutional features can be in the form of fever, night sweats, weight loss and anorexia which may be seen in >50% of patients. The onset of hypertension and/ or absence of upper limb pulses are often the complaints, which lead to a correct diagnosis. Polyarteritis Nodosa (PAN) This is rare in childhood, commonly occurs in adults. Clinical presentation of PAN is extremely variable. The initial symptoms can be rather vague and ill defined with fever, malaise and weight loss being prominent complaints. Hypertension is present in more than 80% of patients and the ESR is usually elevated. CNS involvement occurs in 50 to 70% of children and may first bring the patient to the attention of the physician. The other features that suggest the possibility of PAN are the presence of a typical livedo reticularis rash, abdominal pain, arthritis, myalgia and peripheral gangrene.

Kawasaki Disease (KD) KD is an acute systemic vasculitis of infancy and childhood. This clinical entity is also known as mucocutaneous lymph node syndrome. The clinical diagnosis of KD is usually quite straightforward, provided one keeps it in mind, whenever one encounters a young child with a febrile illness for which no other cause can be found. KD is a syndrome in which there is a constellation of clinical findings, which appear sequentially, with none of the features taken individually being of any significance. Diagnostic Criteria for Kawasaki Disease 1. Fever lasting for at least 5 days. 2. Presence of four of the following five conditions: i. Bilateral conjunctival injection ii. Changes of the mucosae of the oropharynx, including injected and/or fissured lips, strawberry tongue iii. Changes of the peripheral extremities such as edema and/or erythema of hands and/ or feet, desquamation usually beginning periungually iv. Rash, primarily truncal; polymorphous but non-vesicular v. Cervical lymphadenopathy 3. Illness not explained by other known disease process. Henoch-Schönlein Purpura (HSP) HSP is one of the most common vasculitides of childhood. HSP is a clinical diagnosis and is characterized by the presence of nonthrombocytopenic palpable purpura, arthralgia or arthritis, abdominal pain and gastrointestinal hemorrhage. These symptoms may occur over days to weeks. HSP is usually associated with vascular and renal deposition of IgA-containing immune complexes.

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Cutaneous Leukocytoclastic Vasculitis (Hypersensitivity Vasculitis, Small Vessel Vasculitis) This is the most common form of vasculitis that exclusively affects capillaries and venules, almost invariably involves the skin. It is frequently associated with immune complex deposition (with underlying infections, drugs, malignancy, collagen vascular disorders, etc). This form of vasculitis has also been termed as hypersensitivity vasculitis. This small vessel vasculitis may be limited to the skin or may be associated with visceral involvement including pulmonary hemorrhage, intestinal ischemia or hemorrhage, and glomerulonephritis. Common manifestations include purpura and/or urticaria, abdominal pain, gastrointestinal bleeding or intussusception, arthralgias, arthritis or periarthritis, and glomerulonephritis. Visceral symptoms may precede the skin lesions, leading to diagnostic confusion. Purpura tends to occur in crops of lesions of similar age. Palpable purpuric lesions are generally more pronounced in gravitydependent areas like legs (Fig. 23.6). Papules, urticaria/angioedema, pustules, vesicles, ulcers, necrosis, and livedo reticularis may be seen. Paraneoplastic vasculitis is a term used to describe cutaneous necrotizing vasculitis with associated malignant conditions, including Hodgkin’s disease, lymphosarcoma, adult T cell leukemia, mycosis fungoides, myelofibrosis, acute and chronic myelogenous forms of leukemia, IgA myeloma, diffuse large cell leukemia, hairy cell leukemia, squamous cell bronchogenic carcinoma, prostatic carcinoma, renal carcinoma, and colon carcinoma. Urticarial vasculitis represents a peculiar subset of small vessel vasculitis, characterized by typical wheals or serpentine papules formation, sometimes with angioedema. Individual lesions are slower to resolve than typical urticaria and

often last for several days. There is frequently a burning sensation or discomfort from the lesions. These lesions often heal with skin discoloration—hyperpigmentation or an ecchymotic area. Most cases of urticarial vasculitis are idiopathic; they may be associated with an underlying autoimmune disorder such as SLE or Sjögren’s syndrome, IgM paraproteinemia (Schnitzler’s syndrome), viral infections (hepatitis, acute Epstein-Barr), and HenochSchönellein Purpura.

Microscopic Polyangiitis Microscopic polyangiitis (MPA) involves vessels ranging in size from capillaries and venules to medium-sized arteries. Glomerulonephritis, especially rapidly progressive glomerulonephritis, and alveolar hemorrhage are particularly common in MPA and uncommon in PAN. Antibody to myeloperoxidase, a type of PANCA, is detected in sera from 60 percent of patients with MPA. Benign Cutaneous PAN (BC PAN) BC PAN is a rare vasculitic entity mostly seen in adults. The appearance of painful, violaceous, palpable nodules or ridges of variable size along the course of arterioles characterize this relatively benign condition. Mild constitutional symptoms and arthritis of the weight bearing joints may occur. The exact etiology of this condition is not known. Wegener’s Granulomatosis (WG) WG is a potentially lethal, necrotizing, granulomatous angiitis affecting small and medium sized vessels with a predilection for the sinuses, nasal passages, pharynx, lungs and kidneys. In some instances, the lesions may be widely scattered and may involve the skin, heart, CNS, GI tract and joints. Constitutional symptoms such as fever and weight loss are usually quite prominent.


Churg-Strauss Syndrome (CSS) (Allergic Angiitis and Granulomatosis) CSS, like WG, affects small- to medium-sized arteries and veins. Clinically, CSS and WG have similar patterns of organ involvement and pathology, especially in regard to upper and lower respiratory system disease and glomerulonephritis. CSS differs most strikingly from WG by its usual occurrence in patients with a history of atopy, asthma, or allergic rhinitis, which is often ongoing. In the prevasculitic atopy phase, as well as during the systemic phase of the illness, eosinophilia is characteristic and often of striking degree. Systemic features of CSS include some combination of pulmonary infiltrates, cardiomyopathy, coronary arteritis, pericarditis, polyneuropathy (symmetric or mononeuritis multiplex), ischemic bowel disease, eosinophilic gastroenteritis, ocular inflammation, nasal perforations, glomerulonephritis, and cutaneous nodules and/or purpura. Early diagnosis and prompt treatment (with corticosteroids or immunosuppressive drugs) can go a long way in decreasing the morbidity and mortality associated with these disorders. METASTATIC DEPOSITS OF MALIGNANCIES Five to ten percent of patients with cancer develop skin metastases. Usually metastases occur as numerous firm, hard or rubbery masses with predilection for the chest, abdomen, or scalp, in an adult over the age of 40 years who has had a previously diagnosed carcinoma. They are most commonly intradermal papules, nodules or tumors that are firm, skin colored to reddish, purplish, black or brown. Inflammatory carcinoma (carcinoma erysipeloides) is characterized by erythema, edema, warmth and a well defined leading edge similar to erysipelas

Fig. 23.7: Metastatic deposits of malignancies— Sister Mary Joseph nodules

in appearance. This is usually caused by breast carcinoma. The so-called Sister Mary Joseph nodule (Fig. 23.7) is formed by localization of metastatic tumors to the umbilicus. The most common primary sites are the stomach, large bowel, ovary and pancreas. Dissemination to the skin is often a late finding and metastases to other organs have usually occurred. A poor prognosis is thus the rule. Most cases of Hodgkin’s disease of the skin usually originate in the lymph nodes from which the extension to the skin is either retrograde through the lymphatics or by direct extension. Lesions present as papules or nodules with or without ulceration. Cutaneous B-cell lymphoma may present with solitary or multiple papules, plaques or nodules (Figs 23.8 and 23.9). The most common morphology of leukemic infiltration of the skin in all forms of leukemia is multiple papules or nodules or infiltrated plaques. They are rubbery on palpation and ulceration is uncommon. Extensive involvement of the face may lead to leonine facies.

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Fig. 23. 9: Metastatic deposits of malignancies— infiltrated nodules of lymphoma over the arm Fig. 23.8: Metastatic deposits of malignancies— infiltrated nodules and plaques of lymphoma over the abdomen

PARANEOPLASTIC DISORDERS Paraneoplastic disorders refer to cutaneous reaction patterns that have a statistical association with neoplasia of various internal organ systems. Schnyder criteria for paraneoplastic disorders: • No other explanation for skin findings. • Skin changes improve with cancer treatment and may reappear if cancer recurs. • Skin changes appear at about the same time as the systemic cancer: They may precede any clinical manifestation of the underlying malignancy. • Statistical connection is shown.

Paraneoplastic Disorders can be Categorized as 1. Disorders of Keratinization Acanthosis nigricans (AN) is characterized by velvety thickening and hyperpigmentation of the neck and body folds giving the skin a dirty appearance. It occurs in association with DM, obesity, internal malignancy and with certain

Fig. 23.10: Paraneoplastic disorders—rugose thickening of palms—tripe palms

drugs. Malignant acanthosis nigricans usually occurs in adults over 40 years of age. If differs from other forms of AN by its rapid onset and spread, and there is marked velvety hyperkeratosis and hyperpigmentation. There is more pronounced involvement of mucosa, perioral area, palms (tripe palms or acanthosis palmaris) and associated weight loss and wasting. Adenocarcinoma of stomach is most commonly associated with malignant AN.


Acquired ichthyosis: Sudden onset of ichthyosis in adults should arouse the suspicion of internal malignancy. Associated neoplasms are nonHodgkin’s lymphoma, mycosis fungoides, multiple myeloma, carcinoma of breast, lung, cervix and liver.

Necrolytic migratory erythema (Glucagonoma syndrome) is caused by excessive production of glucagon by alpha cell tumor of pancreas. It is more commonly seen in women of 45-65 years. The skin lesions start as circinate, annular or arcuate erythematous plaques that spread peripherally with central clearing. Borders show vesicles, bullae, crusting and scaling giving polycyclic or geographical appearance. These lesions mainly localize around periorificial, acral, and intertriginous areas, closely resembling acrodermatitis enteropathica. Other features are glossitis, angular cheilitis, blepharitis, wasting, anemia, glucose intolerance. Zinc replacement, orctreotide, surgical excision of tumor gives cure only in 30% of cases, because of persistent metastasis.

Acrokeratosis of Bazex: This condition is characterized by symmetric, erythematous psoriasiform eruption mainly involving hands, feet, ears and nose. Nails are involved early and severely. It is commonly associated with carcinoma of pharynx, esophagus, tongue and lung.

Erythema annulare centrifugum usually idiopathic rarely associated with malignancies particularly myeloproliferative disorders. It is characterized by slowly expanding annular erythematous plaques with central clearing and scaling at trailing edge, commonly involves buttocks, thighs (Fig. 23.11), and upper arms.

Erythroderma is commonly associated with leukemia, lymphoma especially cutaneous T cell lymphoma (CTCL), and carcinoma of lung, liver, stomach, colon, pancreas and prostate. Erythroderma secondary to solid tumors may resolve after resection of the tumor.

3. Bullous Eruptions

Associated features may be Leser – Trelat sign, florid cutaneous papillomatosis, and pachydermoperiostosis. Malignant AN typically improves with treatment of underlying malignancy. Tripe palms is characterized by rugose thickening of the palms and accentuation of dermatoglyphic ridges (Fig. 23.10). It may occur alone or along with acanthosis nigricans. It is commonly associated with carcinoma of stomach, and lung.

Paraneoplastic pemphigus (PN) is a bullous disease characterized by severe and resistant

2. Migratory Erythemas Erythema gyratum repens is characterized by mobile, concentric, often palpable, erythematous, wave-like bands (migrating at the rate up to 1 cm per day), giving “wood grain appearance” to skin. A peripheral scale or collarete may be present, and frequently involves the trunk. Tumors associated are lung cancers, myeloproliferative disorders and many other solid cancers. Resection of the tumor may result in resolution of lesions.

Fig. 23.11: Paraneoplastic disorder—erythema annulare centrifugum over the thigh

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Essentials in Dermatology polymorphous mucocutaneous lesions. Pemphigus like, pemphigoid like, erythema multiforme like, graft versus host disease like and lichen planus like lesions occur. Associated neoplasms are Hodgkin’s lymphoma, chronic lymphocytic leukemia, Castleman’s tumor, and thymoma. Pemphigus vulgaris has well defined association with thymoma. Hodgkin’s lymphoma and many solid tumors have also been associated. Bullous pemphigoid is a subepidermal autoimmune bullous disease, mainly affecting elderly individuals characterized by large tense blisters over normal or erythematous skin distributed mainly over central abdomen and flexural aspect of limbs. Significant association with gastric carcinoma has been found. Adult linear IgA disease is a subepidermal bullous disease characterized by vesicles, bullae, and urticarial plaques mainly over trunk and extremities. An increased association with lymphomas has been found. Epidermolysis bullosa acquisita is a subepidermal bullous disease characterized by bullae, and vesicles at trauma prone sites. Association with myeloma and leukemia has been found.

mainly on the anterior surface of legs. It is usually associated with streptococcal infections, and sarcoidosis. Associated neoplasms are Hodgkin’s and non-Hodgkin’s lymphoma, leukemia and renal cell carcinoma. Subcutaneous fat necrosis has been reported in association with pancreatic carcinoma (mainly acinar cell type).

5. Collagen Vascular Diseases Dermatomyositis is an autoimmune disease characterized by inflammation of skin and skeletal muscles. Associated neoplasms are carcinoma of lung, ovary, stomach, pancreas, colon and lymphoma. Adults rather than children with dermatomyositis should be thoroughly investigated for underlying malignancy. Systemic lupus erythematosus is rarely associated with lymphoma or thymoma. Scleroderma was found to be associated with carcinoma of lung, but this could be due to lung tumor developing secondary to chronic pulmonary fibrosis.

Dermatitis herpetiformis is occasionally associated with intestinal lymphoma. Herpes gestationis has been described in association with hydatiform mole and germ cell tumor. Erythema multiforme like lesions occur in association with carcinoma, lymphoma and leukemia.

4. Panniculitides Erythema nodosum is characterized by crops of painful, erythematous nodules and plaques

Fig. 23.12: Paraneoplastic disorder—pyoderma gangrenosum

Skin in Systemic Diseases cancer. Generalized telangiectasias can occur with malignant angioendotheliomatosis and carcinoid tumors. Purpura: Lymphoma, acute leukemia, multiple myeloma can cause purpura. Vasculitis: Leukocytoclastic vasculitis is rarely associated with squamous cell carcinoma of bronchus, renal carcinoma, leukemia and lymphoma.

Fig. 23.13: Paraneoplastic disorder—Sweet’s syndrome lesions over the upper limb

6. Neutrophilic Dermatoses Pyoderma gangrenosum (PG) particularly bullous PG is associated with myeloproliferative disorders. Solid tumors such as carcinoma of colon, prostate, bladder breast, bronchus and ovary have also been reported in association with PG (Fig. 23.12).

Digital ischemia: Persistent digital ischemia often progressing to gangrene has been associated with carcinoma of pancreas, stomach, small bowel, ovary and kidney as well as with lymphoma. Monder’s disease is thrombophlebitis of anterior chest wall, which may be associated with breast cancer.

Sweet’s syndrome is characterized by abrupt onset of painful, tender, erythematous to violaceous plaques and nodules (Fig. 23.13) mainly over trunk and proximal extremities. Associated malignancies are acute myeloid leukemia (most common), other leukemias, multiple myeloma, and lymphoma, less commonly with carcinoma of testis, ovary, stomach, breast, prostate and rectum.

7. Vascular and Blood Abnormalities Flushing: Marked flushing of central face and upper trunk can be a feature of carcinoid syndrome. Palmar erythema can occur in liver failure secondary to either primary or metastatic tumor of liver. Telangiectasias: Localized, grouped telangiectasia of anterior chest wall may indicate breast

Fig. 23.14: Paraneoplastic disorder—wide-spread lesion of seborrheic keratoses over the trunk “Sign of Leser Trelat”

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8. Hormone Related Disorders Cushing’s syndrome: Tumors of lung and pancreas producing excessive ACTH can lead to Cushing’s syndrome. Hirsutism: Androgen secreting ovarian tumors can produce hirsutism. Gynecomastia: Estrogen secreting tumors of testis, lung tumor can cause gynecomastia.

9. Others Xanthomas: Diffuse plane xanthoma can be caused by multipl e myeloma.

Hypertrichosis lanuginosa acquisita refers to acquired excessive growth of lunugo (vellus) hairs mainly over face and ears but eventually over all hair bearing areas. It is usually associated with painful glossitis. Associated tumors are tumors of colon, rectum, bladder, lung, pancreas, uterus, breast, and lymphoma. Multiple eruptive seborrheic keratosis “Sign of Leser – Trelat” refers to sudden development of numerous often pruritic seborrheic keratosis, in association with internal malignancy (Fig. 23.14). Associated neoplasms are adenocarcinoma of GIT, breast, tumors of reticuloendothelial system, and transitional cell carcinoma of bladder.

Skin Changes of Pregnancy and Old Age

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SKIN CHANGES OF PREGNANCY Skin changes occurring during pregnancy can be grouped into physiological variations, changes in pre-existing dermatoses, and pregnancy specific dermatoses.

Physiological Variations Physiological changes are most likely caused by hormonal changes. These include pigmentation of nipples, areolae (Fig. 24.1), and external genitalia. The linea alba becomes the linea nigra. Chloasma or melasma, a mask like hyper pigmentation of the face occurs in more than 50%

Fig. 24.1: Hyperpigmentation of nipple and areola with prominent Montgomery tubercles

of women. Pre-existing nevi or ephelides frequently darken during pregnancy. Mild to moderate hirsutism is frequently seen during pregnancy. After delivery, the resulting telogen effluvium may be severe, resulting in significant hair loss from 1 to 5 months postpartum. Striae distensae (Fig. 24.2) occurs in up to 90% of pregnant women. It is commonly seen over the abdomen, hips, buttocks, and sometimes breasts. Up to two-third of women develop palmar erythema and/or spider angiomas during pregnancy. Edema and varicosities commonly occur in hands and feet. Pregnancy epulis (granuloma gravidarum/pregnancy tumor of

Fig. 24.2: Striae distensae over the abdomen

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Essentials in Dermatology gingiva) is a pyogenic granuloma that may appear in the second or third trimester and resolves shortly after delivery.

Cholestyramine and ursodeoxycholic acid may be effective. Vitamin K to diminish the risk of postpartum hemorrhage can be given before delivery.

Changes in Pre-existing Dermatoses Acne, acne inverse, psoriasis and sarcoidosis tend to improve with pregnancy. Autoimmune collagen vascular disorders (lupus erythematosus, dermatomyositis, systemic sclerosis), autoimmune bullous diseases, invasive or metatastic melanoma, porphyria cutanea tarda, EhlersDanlos syndrome, pseudoxanthoma elasticum, viral and fungal infections tend to worsen. Atopic dermatitis and pustular psoriasis may improve or worsen.

Pemphigoid (Herpes) gestationis: It is the most specific dermatosis of pregnancy which is an autoimmune inflammatory bullous disease with onset during second trimester or during the postpartum period. Typically, pruritic urticarial papules and plaques develop around the umbilicus and extremities, which evolve into tense vesicles and bullae. Remit spontaneously usually within 3 months of delivery.

Pregnancy Specific Dermatoses Recurrent cholestasis of pregnancy (Prurigo gravidarum): It is a hepatic condition occurring late in pregnancy characterized by severe generalized pruritus that is often followed by the appearance of clinical jaundice. It has an estimated incidence of up to 2% of pregnancies. A slight increase in fetal mortality and prematurity has been reported. Postpartum hemorrhage is also more likely in these women. The condition remits within few days after delivery but tends to recur in subsequent pregnancies. Treatment includes bland emollients, topical antipruritic regimens, antihistamines, and UVB phototherapy.

Fig. 24.3

Pruritic urticarial papules and plaques of pregnancy (PUPPP): Also called as polymorphic eruption of pregnancy. It is a very common, intensely pruritic dermatosis that usually occurs late in the third trimester. It typically affects primigravidas. It is characterized by erythematous papules that begin in periumbilical striae distensae (Figs 24.3 and 24.4) and then spread to involve the buttocks and thighs yet spares the upper chest and face. Spontaneous resolution occurs most commonly few days after delivery.

Fig. 24.4

Figs 24.3 and 24.4: Pruritic urticarial papules and plaques of pregnancy involving striae and arms


Fig. 24.5: Senile comedones over the face

Fig. 24.6: Cherry angiomas over the back

Topical corticosteroids form the main stay of management.

alone; photoaging (extrinsic aging), the superimposition on intrinsic aging of changes attributable to chronic sun exposure, which are preventable. Intrinsic aging changes include epidermal atrophy, reduced wound repair, few Langerhans cells and generally impaired immune response, decreased sensation and fewer hairs, decreased sweating, drier skin (less sebum and epidermal lipids) and less vascular reactivity. Chronic exposure to the ultraviolet (UV) component of solar radiation leads to photoaging which is characterized clinically by fine and coarse wrinkles, dyspigmentation (freckling and lentigines), telangiectasia, laxity, roughness and a sallow appearance. Light exposed skin may develop periorbital comedones (Fig. 24.5) (maladie de Favre et Racouchot), purpura, and venous lakes. Over a period of time, these extrinsic aging changes develop into solar elastosis, accelerated cell death and vascular changes (Fig. 24.6), actinic keratoses, basal cell carcinoma, squamous cell carcinoma and malignant melanoma. Topical retinoids and hydroxyacids are the most effective treatment in preventing and partially reversing photodamage. A healthy balanced diet, avoidance of smoking, a good skin care regimen and regular exercise, coupled with sun protection in those with sensitive skin and outdoor lifestyles helps in preventing photoaging.

Prurigo gestationis (Besnier): It is a rare pruritic dermatosis that may occur any time during the fourth to ninth months of gestation. It is characterized by small papules that are excoriated on the proximal limbs and trunk. The eruptions tend to resolve quickly after delivery and it is uncommon to recur in subsequent pregnancies. Therapy with topical glucocorticoids is helpful. Impetigo herpetiformis: It is a form of pustular psoriasis that occurs during pregnancy and may be life-threatening. It tends to occur in third trimester of pregnancy. It is characterized by erythematous patches with peripheral pustules and central scaling favoring the abdomen and upper inner thighs. Systemic signs and symptoms include fever, chills, nausea and vomiting. The disease tends to remit promptly after delivery but may recur in subsequent pregnancies. Systemic glucocorticoids are the treatment of choice in impetigo herpetiformis.

SKIN CHANGES OF OLD AGE Cutaneous aging includes two distinct phenomena: Intrinsic aging, a universal, inevitable change attributable to passage of time

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Pediatric Dermatology

Some skin disorders are transient in newborn but make the parents nervous and anxious about their outcome, while other skin disorders develop later on during infancy or childhood. Some of these disorders are exclusive to children (Cradle cap, napkin dermatitis, acrodermatitis enteropathica, primary herpetic gingivostomatitis) while others are common in children (Pediculosis capitis, scabies, molluscum contagiosum, warts, chicken pox, tinea capitis, atopic dermatitis, oral thrush, Henoch-Schönlein purpura, acute hemorrhagic edema of infancy, and papular urticaria).

Transient Cutaneous Lesions in Newborn • Vernix caseosa—golden yellow staining of vernix caseosa occurs in hemolytic disease of the newborn and post-maturity. • Acrocyanosis—cyanosis seen in acral areas in a neonate • Erythema neonatorum—within a few hours of birth- erythema fades spontaneously within 24-48 hours. • Harlequin color change—seen when the infant is placed on one side-upper half of the body becoming pale and the lower half a deep red color, with a sharp midline demarcation between the two, staying for half a minute to 20 minutes, in a full term or preterm newborn seen during first week of life.

• Cutis marmorata—this marbling change due to reticulate blue vascular pattern seen in infancy, occurs on exposure to decreased temperature and disappears on rewarming. • Physiological scaling of the newborn—seen in up to 75% of the normal neonates. • Sebaceous gland hyperplasia characterized by multiple, uniform, pin point yellowish papules, most prominent on the nose, cheeks, upper lip and forehead, but may be visible on the upper trunk, especially the areolae, genitalia and the limbs. This phenomenon is associated with milia which represents minute follicular epidermal cysts. • Linea alba becomes pigmented in about 8% of babies. • Exaggerated pigmentation of the scrotum occurs in about 30% of oriental neonates, generally associated with Mongolian spots. • Epstein pearls—are one or more 1-2 mm yellowish white keratinous cysts along the alveolar ridges or in the midline at the junction of the hard and soft palate. • Milia represent miniature epidermal inclusion cysts that originate from sebaceous apparatus of vellus hair (Fig. 25.1). Epstein pearls are clinically and histologically the intra-oral counterpart of facial milia.


Fig. 25.1: Milia – tiny pearly white keratinous inclusion cysts over the face

• Succulent gums (Sucking pads)—a whitish hue to the oral mucosa. • Sucking blisters—blisters or erosions on the upper limb/upper lip, present at birth, due to intrauterine sucking. • Erythema toxicum neonatorum (Toxic erythema of the new born, erythema neonatorum)—presents as an asymptomatic macular erythema on the trunk within first 48 hours of birth. Subsequently, it may evolve into urticarial papules or pustules. Recovery occurs in 3 days. Smears of the pustule contents demonstrate inflammatory cells, more than 90% of which are eosinophils. There is an associated blood eosinophilia in 50% of cases. • Mongolian spots—are blue gray pigmentation areas present at birth on the sacral area of the normal infants in dark skinned races. Edwin Baez, thinking this as a characteristic of Mongolian race, named it as Mongolian spot. The Chinese people believed that the mark is the imprint of their “God of birth”. As the child is born, the God gives it a ‘spank’ or ‘kick’ to give it a start in life, whereas the

Japanese believed that the spot was the consequence of coitus performed during pregnancy. It usually disappears during the first decade and in a small percentage (3-4%) persist beyond this into adult life. • Acne neonatorum (Neonatal acne)— develops within the first 30 days of life. • Perianal dermatitis is an erythema centered on the anus, occasionally accompanied by erosion and bleeding at 4th to 7th day of life • Transient neonatal pustulosis (Transient neonatal pustular melanosis) invariably presents at birth. It is characterized by the presence of fragile superficial pustules mainly over the chin, neck, forehead, back, and buttock. Hyperpigmented macules develop subsequently. They may persist for 3 months and affected infants are otherwise normal. Pustules are formed due to subcorneal collection of neutrophils with a few eosinophils. Bacterial culture is negative. Pigmented macules demonstrate basal and supra-basal increase in pigmentation only, apparently without pigmentary incontinence.

Skin Disorders in Children (Pediatric Dermatoses) Skin disorders are extremely common in our population and approximately 6% of visits to all physicians entail a problem of the skin, hair or nails. However, dermatologists see only approximately 40% of these patients. Dermatological problems manifesting as primary and secondary cutaneous complaints, constitute at least 30% of all outpatient visits to a pediatrician and 30% of all visits to dermatologists involve patients of pediatric age group. The vast majority of complaints related to the skin in children can be easily recognized and treated by non-specialist. These common diseases have been classified in the Table 25.1.

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Essentials in Dermatology Table 25.1: Classification of common pediatric dermatoses 1. Infestations and infections (a) Parasitic infestations—Pediculosis capitis, scabies (b) Bacterial infections— Pyodermas (c) Viral infections—Molluscum contagiosum, warts, herpes simplex, chicken pox, herpes zoster (d) Fungal infections-Tinea capitis, tinea corporis, pityriasis versicolor, candidiasis 2. Dermatitis and eczema—Infantile seborrheic dermatitis, diaper or napkin dermatitis, atopic dermatitis, infective dermatitis 3. Urticaria 4. Exanthems—Viral exanthems (Measles, rubella, roseola infantum, erythema infectiosum) 5. Drug eruptions 6. Pigmentary disorders—Postinflammatory pigmentation, hypopigmentary disorders (Pityriasis alba, vitiligo, leprosy, nevus achromicus, ash leaf macule, albinism), hyperpigmentary disorders (Mongolian spots, café au lait macules) 7. Diseases of hair and nails—Tinea capitis, alopecia areata, diffuse alopecia, twenty nail dystrophy 8. Genetic diseases of the skin-Ichthyoses, acrodermatitis enteropathica 9. Collagen vascular diseases—Connective tissue diseases (Lupus erythematosus, scleroderma, and dermatomyositis) and vasculitic syndromes (Henoch-Schönlein purpura, acute hemorrhagic edema of infancy, and polyarteritis nodosa) 10. Miscellaneous conditions—Papular urticaria, miliaria rubra, miliaria crystallina, psoriasis, hemangiomas, chilblains

SOME IMPORTANT VIRAL EXANTHEMS IN CHILDREN Measles (Rubeola, Morbilli) • The term measles is thought to come from Latin “misellus” or “misella”, a diminutive of Latin “miser”, meaning miserable. • It is caused by measles virus (a paramyxovirus, RNA virus). • Incubation period is 10-11 days. • Measles, is a universal highly contagious disease of children. It has a characteristic prodrome of 3-4 days that consists of high fever, cough, coryza, a striking palpebral conjunctivitis with photophobia, and Koplik’s spots, which precede the appearance of florid generalized macular and papular rash. • The first lesion to appear on the soft palate is blotchy erythema, but the most pathognomonic lesion of the prodrome, if present are Koplik’s spots, which appear as tiny white lesions surrounded by an erythematous ring (grain of sands). • Koplik’s spots precede the onset of generalized rash by 1-2 days, remain for two to three days

•

• •

•

and are usually heavily clustered on the buccal mucosa opposite the second molar. The purplish red rash on the body appears first behind the ears and over the forehead, and then spreads slowly to involve the entire body by third day. The eruption extends downwards over the neck, shoulders and trunk and then distally over the upper and lower extremities. The rash fades with brown pigmentation followed by desquamation. Uncomplicated measles runs a self limited course lasting about 10 days. Complications of measles include encephalitis, thrombocytopenia, otitis media, pneumonia, exacerbation of tuberculosis and subacute sclerosing panencephalitis. There is no specific therapy for measles.

Rubella (German Measles) • Rubella virus is a togavirus, commonly recovered from pharynx. • Incubation period is 14-21 days. • Rubella is a common communicable infection of children and young adults characterized by a short prodromal period; enlargement of

Pediatric Dermatology cervical, suboccipital and postauricular glands and a rash of approximately 2 to 3 days duration. • An enanthem, Forschheimer’s sign, is present in up to 20% of patients during the prodromal period or on the first day of the rash. Dull-red macules or petechiae are confined to the soft palate. • The disease has rare sequelae apart from devastating effect on the fetus.

Exanthem Subitum (Sixth Disease, Roseola Infantum) • Caused by Human herpes virus type 6 (DNA virus). • Incubation period is 10-15 days. • Most common exanthem with fever in children under age group of 2 years. • Prodromal fever is usually high. Fever drops on fourth day. • Convulsions and lymphadenopathy may accompany it. • Clinically, a morbilliform erythema consisting of rose colored discrete macules appears on the neck, trunk, and buttocks. • Often there is a blanched halo around the lesions. • The lesions resolve in 1 to 2 days. • Other common associated findings include otitis media, diarrhea and meningoencephalitis. • In adults HHV-6 infection resembles acute infections mononucleosis. • Treatment – acyclovir, ganciclovir. Erythema Infectiosum (Fifth Disease) • Exanthematous disease occurring in patients with primary human parvo virus B19 infection (DNA virus). • Incubation period is 4-14 days. • More commonly seen in school children.

• Infection spreads by respiratory droplets during the prodrome. • Constitutional symptoms are absent or very minimal.

Three Stages of Rash are 1. “Slapped cheek” appearance (1 to 4 days). 2. Erythematous papular eruption over the upper and lower extremities spreading to trunk. Assumes a lace-like or reticulated appearance as it fades. 3. Recurrent evanescent stage (for weeks or months) is precipitated by a variety of skin irritants such as sunlight, hot showers. • Papular – purpuric ‘ gloves and socks’ syndrome seen in older children and adolescence. • Adults may present with atypical rash and arthritis. • Complication—Hydrops foetalis (maternal infection) and aplastic crisis. • Treatment—supportive.

Gianotti-Crosti Syndrome • Infantile papular acrodermatitis, or the Gianotti-Crosti syndrome, presents with symmetric erythematous lichenoid papules on the face, extremities, and buttocks, usually sparing the trunk. • The eruption is not pruritic and may be accompanied by splenomegaly, hepatitis, and lymphadenopathy. • The process often occurs in young children after an upper respiratory tract illness. • Pathologic specimens show a perivascular infiltration of lymphocytes and histiocytes in the upper portion of the dermis. • While the syndrome has been associated with hepatitis B and enterovirus infection, several cases have been associated with acute EBV infection.

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SOME OTHER IMPORTANT ENTITIES Henoch Schönlein Purpura • Henoch Schönlein purpura is an IgA mediated vasculitis syndrome affecting skin, joints, GIT and kidneys. • It usually occurs below the age of 20 years. • The eruption may begin as crops of palpable purpuric lesions or urticarial rash in the lower legs and buttocks. • Abdominal symptoms such as colic, vomiting and diarrhea are seen in two-third of the patients. • Polyarthralgia is seen in most patients, commonly affecting the knees and ankles. • Renal involvement is usually mild, consisting of proteinuria/hematuria, but can rarely lead to end stage renal disease. • Diagnosis: Histopathology shows leucocytoclastic vasculitis and immunoflorescence demonstrates IgA deposition around blood vessels. • Treatment: No specific treatment available. Antibiotics and corticosteroids can be given to alleviate symptoms.

Fig. 25.2

Urticaria Pigmentosa Urticaria pigmentosa is the most common type of cutaneous mastocytosis that occurs due to the accumulation of mast cells in the skin. • The skin lesions are itchy small, yellow tan to reddish brown maculopapules/plaques/ nodules scattered all over the body (Figs 25.2 and 25.3). • Mild trauma such as scratching or rubbing the lesions may cause urtication and erythema around macules and this is known as Darier’s sign. • Urticaria pigmentosa is associated with pruritus which may be exacerbated by temperature, friction, spicy foods, alcohol and drugs. • Diagnosis: Histopathology shows mast cell infiltration in the dermis • Treatment: Avoidance of precipitating factors and antihistaminies. Langerhans Cell Histiocytosis (LCH) Langerhans cell histiocytosis is a disease that results due to the proliferation of cells like Langerhans cells, called as LCH cell (share the

Fig. 25.3

Figs 25.2 and 25.3: Urticaria pigmentosa—multiple brownish pigmented infiltrated lesions over trunk and extremities


Fig. 25.4: Letterer-Siwe disease–seborrheic dermatitis like rash involving scalp extending on to forehead and ears

Fig. 25.5: Letterer-Siwe disease—hypopigmented macular as well as purpuric papular lesions seen over the abdomen

ultrastructural features with Langerhans cellscontaining Birbeck granules) in any organ. This “LCH cell” is about four to five times larger than small lymphocytes; has an irregular and vesiculated nucleus; is often reniform (kidney shaped); and has abundant, slightly eosinophilic cytoplasm.

Fig. 25.6: Letterer-Siwe disease—marked hepatosplenomegaly

• It is broadly classified into entities such as Letterer-Siwe disease, Hand-Schüller Christian disease and eosinophilic granuloma. • Eosinophilic granuloma occurs due to localized proliferation of “LCH cells” in the bones, skin, lymph nodes, lung, liver and spleen . • Hand-Schuller-Christian syndrome is a chronic multisystemic disease known by the triad of exophthalmos, multiple skull lesions and diabetes insipidus. • Letterer-Siwe disease is usually seen in children less than 1 year. It is characterized by seborrheic dermatitis like rash (Fig. 25.4) with hemorrhagic papular (Fig. 25.5), vesicular, pustular and ulcerated lesions in the intertriginous regions and trunk. • Letterer-Siwe disease is often associated with hepatosplenomegaly, dysfunction of liver, lungs and hematopoietic system. Lytic lesions may be seen in skull bones (Fig. 25.6). • Diagnosis: Histopathology shows LCH cell infiltration of the lesions. • Treatment: Localized forms can be treated with intralesional steroids or surgery. Multisystemic involvement needs chemotherapy.

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Benign, Pre-malignant and Malignant Tumors of the Skin

Skin tumors can be broadly classified into various types based on cell of origin. Broadly they can be classified as benign, premalignant and malignant tumors. Some of these common cutaneous tumors are discussed in this chapter.

BENIGN SKIN TUMORS Seborrhoeic Keratosis (Senile Wart, Senile Keratosis, Seborrheic Verruca, Basal Cell Papilloma) • A benign tumor, more common in the elderly people. • Seborrheic keratosis (SK) occur on any body site, most frequent on the face and the upper trunk. • Seborrheic keratoses typically begin as flat, sharply demarcated, brown macules. Follicular prominence is one of the hallmarks of seborrheic keratoses. Later on, typical asymptomatic, slowly increasing, verrucous plaque develops and have a “stuck-on” appearance. • Surface of SK has loosely adherent greasy keratinous scales on the surface. • Sometimes, sudden eruptive lesions appear which may be due to underlying malignancy. Then it is called ‘Leser-Trelat sign’. • Classical SK shows features of hyperkeratosis and numerous horn cysts in histology.

• Treatment – curettage, cryotherapy or electrodessication.

Naevi • The word naevus is derived from the Latin term meaning spot or blemish, originally used to describe the congenital lesion or birth mark (Mother’s mark). • In modern usage, it denotes a cutaneous hamartoma or benign proliferation of cells. • Nevi can be broadly classified into various types according to the predominant cell type. • The various types are keratinocyte nevi (Figs 26.1 and 26.2), follicular nevi, sebaceous nevi, apocrine nevi, eccrine nevi, connective tissue nevi, smooth muscle nevi, elastic nevi, fat nevi (Fig. 26.3), and vascular nevi (Figs 26.4 to 26.7).

Melanocytic Nevi • They are benign tumors derived from melanocytes. They are broadly classified into acquired or congenital. • Acquired melanocytic naevi are subdivided into junctional, compound (Figs 26.8 and 26.9) and dermal. • They begin as proliferative naevus cells along the dermal-epidermal junction (forming a junctional naevus)


Fig. 26.1

Fig. 26.2

Figs 26.1 and 26.2: Nevus—verrucous epidermal (keratinocyte) nevus occurring on one side of the body

Fig. 26.3: Nevus-nevus lipomatosus cutaneous superficialis presenting as lobulated mass over the thigh

• With continued proliferation, they extend from the dermal-epidermal junction into the dermis (forming a compound naevus). • The junctional component of the melanocytic naevus may resolve leaving only an intradermal component (intradermal naevus). • Acquired melanocytic naevi may resolve spontaneously.

Fig. 26.4: Nevus—Portwine stain over the face

• Congenital melanocytic nevi may be defined as melanocytic naevi present at birth (Figs 26.10 to 26.13). • Those measuring more than 20 cm in greatest diameter are referred to as Giant congenital melanocytic nevi or bathing trunk nevi. • Treatment: surgical excision.

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Fig. 26.5

Fig. 26.6

Figs 26.5 and 26.6: Nevus—Hemangioma of infancy over the nape of neck and face

Fig. 26.7: Angiokeratoma circumscriptum of the tongue

Fig. 26.8: Melanocytic nevus—compound nevus over the cheek

Skin Tags (soft warts; Acrochordons) • Common benign lesion, occurs on the neck, axilla and groin • The lesions are round, soft, pedunculated connected to the skin by a narrow pedicle.

They vary in size from 1 mm to 1 cm long (Fig. 26.14). • The skin tags are of three types which include–multiple small furrowed papules (12 mm long), single or multiple filiform


Fig. 26.9: Halo nevus over the cheek

Fig. 26.10: Congenital melanocytic nevus over the face of a child

smooth growths (2-5 mm) and solitary baglike–pedunculated growth (1 cm). • Histopathologically skin tags consist of loose fibrous tissue covered by folded skin. • They have been found in association with colonic polyps, diabetes and acromegaly. • Simple snipping, electrocautery and cryotherapy are effective.

Fig. 26. 11: Congenital melanocytic nevus with hairiness over the back

Pyogenic Granuloma (Granuloma pyogenicum) • They are smooth surfaced, bright red, friable, sessile or pedunculated lesions of exuberant granulation tissue with a pale epidermal collarette around the lesion (Fig. 26.15) • Occurs following a minor injury or infection of the skin • They most often affect children or young adults • The hands, fingers and face especially the lips and gums are the most common sites. • The lesion once developed may persist indefinitely unless destroyed. It may occur in pregnancy in gingiva–called as “Epulis Gravidarum” or “Pregnancy tumor”. • Differential diagnosis includes Kaposi’s sarcoma and bacillary angiomatosis • Treatment: excision and electrofulguration. Milia • They are tiny, white, globoid cysts that commonly occur on the face around eyes (Fig. 26.16). • Primary milia represent a keratinizing benign tumor. They arise spontaneously on the face in the predisposed individuals.

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Fig. 26.12

Fig. 26.13

Figs 26.12 and 26.13: Dermal melanocytosis—Nevus of Ota in the periorbital area with scleral pigmentation

Fig. 26.14: Skin tags—pedunculated soft lesions in the axilla

Fig. 26.15: Granuloma pyogenicum—pink colored nodule bleeds on manipulation

• Secondary milia are type of retention cyst that arises due to damage to the epithelium and occurs in diseases like bullous pemphigoid, porphyria cutanea tarda and others following trauma. • Treatment: Deroofing with hypodermic needle, trichloroacetic acid cautery or electrocautery.

Trichoepithelioma • Trichoepithelioma is benign appendageal tumor with follicular differentiation. • They appear at puberty as solitary or multiple, skin colored, translucent, rounded nodules on the face (Fig. 26.17). • The lesions are distributed predominantly in the nasolabial folds and eyelids.


Fig. 26.16: Milia—white keratinous cysts over the face

Fig. 26.18: Syringomas—multiple angulated papular lesions in the periorbital area

• Clinically lesions are multiple, skin colored, 1-3 mm sized, angular papules (due to, numerous small cystic ducts, as well as solid epithelial strands in the upper dermis and mid-dermis) distributed bilateral symmetrically, most commonly on the lower eyelids (Fig. 26.18), less commonly over the chest and neck. • Treatment with diathermy produces good results.

• A few telangiectatic vessels are often present on the surface of the large lesions, which resemble basal cell carcinoma. • Treatment with diathermy produces good results.

Hypertrophic Scar • Injury or surgery in a predisposed individual can result in an abnormally large scar called as hypertrophic scar • A hypertrophic scar represents excessive collagen deposition at the site of wound healing • Typically, hypertrophic scar starts as an asymptomatic, erythematous, smooth, firm scar seen at anatomic locations characterized by high tensions. With time, they flatten and become white in color. • Hypertrophic scars do not extend beyond the limits of the original trauma and heal by 6 months.

Syringoma • Benign tumors of eccrine differentiation • More common in females

Keloids • Keloids represent exaggerated collagen deposition at the site of wound healing.

Fig. 26.17: Trichoepithelioma—multiple skin colored translucent rounded nodules in the nasolabial folds

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Fig. 26.19: Keloid—indurated skin lesion showing peripheral extensions

Fig. 26.20: Dermatofibroma—single skin colored tender nodule over the shoulder

• They frequently proliferate beyond the wound margins onto the normal adjacent skin (Fig. 26.19). • A keloid appears as a firm, mildly tender, bosselated tumor present at a site of previous injury. They are commonly pruritic, erythematous, indurated lesions that show extensions like claws of a crab. They may even be the source of significant discomfort or pain. • They rarely undergo involution. • Treatment: Medical–topical steroids, intralesional steroids and silicone gel dressings. Surgical-surgical excision, cryosurgery and skin grafts.

Glomus Tumor • This arises within the glomus body, a neuromyoarterial receptor (the SucquetHoyer canal) that is sensitive to temperature variations and regulates arterial blood flow. • These tumors, more common on the fingers and toes and beneath the nail plate. • Usually seen in young adults between the third and fourth decade of life. • They can be solitary or multiple. • Classically presents as solitary, small, blue – red nodules on the hand (nail bed) that are characteristically associated with paroxysmal pain often elicited by changes in temperature. • Simple excision- treatment of choice.

Dermatofibroma • Most common benign dermal fibrous tumor. • Typical dermatofibromas appear as skin coloured to red-brown, firm, tender nodule(s), most commonly seen on the extremities (Fig. 26.20) • Lateral compression of the lesion with fingers results in the depression on the top–called ‘dimple sign’ • Histologically the fibroblasts and collagen bundles are arranged in a storiform or cartwheel pattern • Treatment of choice–simple excision.

PREMALIGNANT EPIDERMAL TUMOURS Actinic Keratosis (Senile or Solar Keratosis) • Most common premalignant skin tumor. • These lesions occur in sun-damaged skin of elderly people having light complexion • Clinically, these lesions are round to irregular keratotic papules surrounded by erythema.


Fig. 26.22: Erythroplasia of Queyrat—sharply defined brightly erythematous velvety plaque over the glans penis

Fig. 26.21: Actinic keratoses—Albino showing a number of actinic keratoses lesions along with two basal cell carcinoma lesions over the face

• The principal sites are the back of the hands, forearms and face (Fig. 26.21). • Squamous cell carcinoma can develop from actinic keratosis. • Histologically, there may be vacuolization of keratinocytes with numerous mitotic figures seen involving the lower layers of the epidermis (carcinoma in situ). • Treatment: Medical – topical tretinoin and topical 5-fluorouracil and surgical- curettage, electrodessication and cryotherapy.

Erythroplasia of Queyrat • This condition affects uncircumscised males. • Seen in the fifth and sixth decades of life. • The lesions present as sharply defined, brightly erythematous and velvety plaque with moist glistening or granular surface over the glans penis (Fig. 26.22). • Differentiated from benign inflammatory dermatoses such as psoriasis, lichen planus, Zoon’s plasma cell balanitis and fixed drug eruptions.

• Treatment with topical 5-fluorouracil produces satisfactory results.

Bowen’s Disease • Refers to cutaneous plaques of intraepidermal squamous cell carcinoma. • Chronic sunlight exposure, inorganic arsenicals are important etiologic factors. • Clinically lesions of Bowen’s disease appear as solitary, sharply defined, round or oval to irregular erythematous psoriasiform or eczematous plaque. • Ulceration is a sign of development of invasive carcinoma. • Histology shows proliferating atypical squamous cells through the full thickness of the epidermis (“wind- blown appearance”) • The most effective treatment for Bowen’s disease is surgical excision. Cutaneous Horn (cornu cutaneum) • Cutaneous horn (cornu cutaneum) is the term coined for horny skin excrescence, which in its form and consistency resembles an animal horn in miniature (Fig. 26.23) • The paramount consideration while making a clinical diagnosis is the height of the

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Fig. 26.23: Cutaneous horn—horny excrescence resembling an animal horn in miniature

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• • •

keratotic mass (at least one half of its largest diameter). It is a reaction pattern of exaggerated hyperkeratosis and is rarely, if ever seen nowadays. Its association with various dermatoses may be informative. The important issue is not the horn itself which is dead keratin, but rather the nature of underlying condition, which may be benign (seborrheic keratoses, viral warts, histiocytoma, inverted follicular keratosis, verrucous epidermal nevus, organoid nevus, ichthyosis hystrix, palmoplantar keratoderma, papilloma, keratoacanthoma, open epidermoid and trichilemmal cysts, lichen planus, corn and calluses, scar, molluscum contagiosum), premalignant (solar keratosis, arsenical keratoses, Bowen’s disease) or malignant (squamous cell carcinoma, rarely, basal cell carcinoma, metastatic renal carcinoma, granular cell tumor, sebaceous carcinoma or Kaposi’s sarcoma). In the majority of cases, these horns are benign. Most commonly, they are single and arise from a seborrheic keratosis lesion. They are encountered most frequently on the face and scalp, but may occur on the hands, penis and eyelids.

Basal Cell Carcinoma (BCC) (Rodent Ulcer) • Most common malignant skin tumor that rarely metastasizes. • It is a malignant tumor of the skin that is believed to arise from the hair follicle. • Its cells show a morphologic resemblance to the relatively undifferentiated cells of the basal layer of the epidermis. • BCC is more common in men. • Persons with fair complexion, having outdoor occupation and chronic sun exposure are at a higher risk to develop BCC. • The five clinical forms of BCC include–the noduloulcerative (most common), superficial type, pigmented type, sclerosing type and fibroepithelioma. • The most common clinical presentation of BCC is the noduloulcerative type, which appears as a dome-shaped papule with a telangiectatic surface and a “pearly” translucent border (Figs 26.24 and 26.25). It begins as small, shiny, waxy nodule increases in size, undergo central ulceration • It is locally invasive without any metastasis to regional lymph nodes • Treatment- surgical excision, electrodesiccation, irradiation. Kaposi’s Sarcoma • Kaposi’s sarcomas (KS) are multisystem vascular neoplasms, characterized by mucocutaneous violaceous lesions and edema as well as involvement of nearly any organ • Human herpes virus type 8 has been implicated in the pathogenesis • KS have been classified into five typesClassical type, African-endemic type, iatrogenic type, immunosuppression associated type and HIV associated type (Epidemic type)


Fig. 26.24

Fig. 26.25

Figs 26.24 and 26.25: Basal cell carcinoma—ulcerated nodule over the eyelid showing pearly translucent border with telangiectasia

• Presents as ecchymotic macules, which become purple and brownish with hemosiderotic halo. The lesion evolves into patches, plaques and nodules and are often arranged parallel to skin tension lines. • Classical KS lesion occurs on the leg at the mean age of 60 years. • Oral lesion can be the first manifestation of KS in the majority of cases, especially in those having underlying HIV infection. • Histologically, a characteristic sign of KS is the presence of solid cords and fascicles of oval or spindle shaped cells arranged between the jagged vascular channels. These structures dissect the collagen bundles of the entire dermis, leaving a spongy network of collagen. This biphasic tumor morphology, exhibiting both angiomatous and solid tumor patterns, changes to a clear-cut sarcomatous morphology. • Modalities of management include— radiation and chemotherapy.

Mycosis Fungoides Mycosis fungoides is a form of cutaneous T- Cell lymphoma (CTCL). • It is a chronic, slowly progressive disease that evolves from patches (patch stage) (Fig. 26.6)

to plaques (plaque stage) and subsequently nodules (tumor stage). • Pruritus is a prominent symptom. • The lesions coalesce and may ulcerate, leading to deep ulcers. • Prognosis depends upon the TNM staging. • Diagnosis: Clinically lesions can be confused with eczema and psoriasis. Histopathology reveals atypical lymphocytes infiltrating the epidermis without spongiosis (epidermotropism). The pathognomonic feature is presence of Pautrier microabscess (collection of atypical lymphocytes in the epidermis). The dermis has dense monomorphous lymphomononuclear infiltrate with grenz zone. • Treatment: It depends on the stage of the disease. Chemotherapy, retinoids, electron beam therapy, photochemotherapy, etc. have been used.

Sézary Syndrome Sézary syndrome is a rare special variant of cutaneous T-cell lymphoma characterized by a triad of features—erythroderma, peripheral lymphadenopathy and infiltrates of atypical cells in the skin and blood.

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Fig. 26.26: Mycosis fungoides—multiple hypopigmented patches over the chest and abdomen

Fig. 26.27: Sézary syndrome—face showing erythematous infiltrated plaques, in addition to diffuse erythema

• It occurs in people older than 60 years and more commonly in males. • There is generalized erythroderma (Fig. 26.27) with diffuse hyperkeratosis of palms and soles and diffuse hair loss.

• Peripheral smear shows more than 10% atypical lymphocytic cells in the blood. • Treatment is chemotherapy combined with electron beam therapy or photochemotherapy.

Topical Formulary and Key Systemic Drugs

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The use of topical remedies in the treatment of disease is as old as medicine itself. Because the skin is accessible and visible, it is uniquely amenable to external chemical and physical remedies.

Acute Exudative Dermatoses (Erythema, edema, vesiculation, oozing, crusting, infection, pruritus)

KEY POINTS IN TOPICAL THERAPY From treatment point of view, most of the common dermatoses fall into one of the two categories. 1. Dry dermatoses 2. Wet dermatoses The popular saying in treatment is “If the skin is dry, wet it, or if the skin is wet, dry it”. The most effective way to “dry” skin that is involved with acute exudative dermatoses is to apply cool, wet compresses with or without astringents, followed by open air or absorbent powders. Hence, for this use lotions (aqueous solutions) or gels are the vehicles of choice rather than ointments. Conversely, the effective way to “wet” the skin as in case of xerosis or chronic inflammatory and papulosquamous skin lesions is to apply greasy ointments, water in oil ointments or occlusive wrappings.

Chronic Inflammatory Dermatoses (Erythema, scaling, lichenification, dryness, pruritus) The term “vehicle” is used for those substances that bring specific drugs in contact with the skin. An ideal vehicle 1. Easy to apply and to remove. 2. Non-toxic. 3. Non-irritant. 4. Non-allergenic. 5. Chemically stable. 6. Homogeneous.

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Essentials in Dermatology 7. Bacteriostatic. 8. Pharmacologically inert. 9. Cosmetically acceptable. For a layman, it should not “Sting, stale/ stench and stain” For any drug to be effective topically, it must be formulated at the proper concentration and in the proper vehicle. In general, the most important vehicles for topical use may be divided into monophasic, biphasic, and triphasic forms. There are three basic constituents of a formulation, which are present singly or in combination to form a vehicle. They are: 1. Powders. 2. Greases. 3. Liquids. When only one of the basic constituent is present in a formulation vehicle, then it is called monophasic vehicle (Powders, liquids or greases/ oils). In case two of them are present, it is called biphasic vehicle(The combination of powder with water gives either a drying paste or a shake lotion. Grease or oil and powder will form either a liniment or a protective (fatty) paste. Water and grease, however, will mix to form emulsions in the presence of a surfactant or emulsifier. These then form oil in water (O/W) or water in oil (W/O) creams) and if all the three are present, it is called triphasic vehicle (Cooling pastes and cream pastes are triphasic vehicles that consist of oil-water-powder mixtures in varying proportion).

TERMINOLOGY IN TOPICAL FORMULATION Wet dressings are water or saline soaked cotton gauze or cloth compresses used in the treatment of acute inflammatory dermatoses with oozing, weeping and crusting, bullous diseases and ulcers.

Baths/soaks are a mode of drug delivery wherein a part (soak) or the whole body (bath) is immersed in medicated water. Lotions are liquid formulations where the active ingredient is present as powder suspended in aqueous solution by aid of a suspending agent known as shake lotion (need to be shaken before use) or active ingredients are dissolved to clarity called as clear solutions. Gels are soft semisolid preparations that are translucent to transparent in appearance. They liquefy on contact with the skin and dry to leave behind a thin greaseless film and are appropriate for use on hairy areas. Tinctures are alcoholic solutions with low concentration of active ingredients, e.g. tincture iodine. Paints are medicated solutions which leave a colored film on drying of the aqueous phase, i.e. they cause staining, e.g. gentian violet paint, Castellani’s paint. Ointments are semisolid soft to very firm greasy preparations and employ lipophilic bases. Petrolatum and white petrolatum are the most widely used ingredients in the ointments. Creams are semisolid soft to moderately firm washable emulsions and are generally of the oil in water type. Pastes are preparations of about 50% powdered ingredients in a greasy base such as petrolatum. Water based preparation of powder form drying pastes. One gram of cream covers an area approximately 10 by 10 cm. An ointment spreads up to 10 percent further. According to Arndt, the amount needed for the single application of a cream or ointment to the face or hands is 2 gm; to one arm or the anterior or posterior trunk, 3 gm; to one leg, 4 gm; and to the entire body, 30 gm.


KEY SYSTEMIC DRUGS—PENICILLINS Originally derived from Penicillium notatum are Beta-lactam antibiotic, inhibits transpeptidases by binding to penicillin binding proteins (PBPs) and thereby interferes with bacterial cell wall synthesis. Natural penicillins are benzyl penicillin (Penicillin G), sodium penicillin G (crystalline penicillin), and repository penicillin G (given deep IM) (such as procaine penicillin G, and benzathine penicillin G). Semisynthetic penicillins – 1. Acid resistant alternative to penicillin G – phenoxymethyl penicillin (Penicillin V). 2. Penicillinase resistant penicillins – methicillin, oxacillin, cloxacillin. 3. Extended spectrum penicillins. Aminopenicillins – ampicillin, amoxycillin Carboxypenicillins – carbenicillin, ticarcillin Ureidopenicillins – piperacillin, mezlocillin β -lactamase inhibitors – Clavulanic acid and sulbactam prevent inactivation by penicillinases produced by various organisms. Spectrum of activity is against gram positive cocci (non penicillinase-producing strains of Staph. aureus, Staph. epidermidis, Streptococci except group D or enterococci), gram negative cocci (Neisseria gonorrheae, Neisseria meningitides), gram positive bacilli (B. anthracis, C. diptheriae, Cl. tetani, Listeria), and spirochetes (Treponema pallidum). Majority of gram negative bacilli are totally insensitive to penicillin. Penicillin G is rapidly degraded by gastric acid (so preferred for IV administration). Penicillin V is preferred for oral administration. Benzathine and procaine penicillins are used for slow release (IM administration). Excretion mainly occurs via tubular secretion (dose adjustment in renal insufficiency).

Dosage • Sodium penicillin G (crystalline penicillin): 0.5-5 MU IM/IV 6-12 hourly. • Procaine penicillin G: 0.5-1 MU IM 12-24 hourly. • Benzathine penicillin G: 0.6-2.4 MU IM every 2-4 weeks. Indications • Syphilis. • Streptococcal infections. • Tetanus. • Gas gangrene. Adverse Effects/ Precautions • Hypersensitivity reactions ranging from mild rash/fever/eosinophilia to fatal anaphylaxis. • Jarisch-Herxheimer reaction – occurs within 2-12 hours of the first dose when using penicillin for syphilis; presents as headache, fever, chills, sweating, sore throat, myalgia/ arthralgia, tachycardia and increased blood pressure. The condition does not recur and does not need interruption of treatment. Aspirin and sedation offer symptomatic relief. • Pregnancy: category B. Drug Interactions • Probenecid – inteferes with tubular secretion of penicillin. AMPICILLIN Pharmacology • β-lactam antibiotic, aminopenicillin group. • Interferes with bacterial cell wall synthesis. • Greater activity than natural penicillins against enterococci and H.influenzae. • Maximal absorption when taken on empty stomach. • Primarily excreted in urine (dose adjustment in renal insufficiency).

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Dosage Oral • Adults and children >20 kg – 250-500 mg Q6h. • Children <20 kg – 50-100 mg/kg/day in divided doses Q6h.

Parenteral • Adults – 500-1500 mg IM/ 500-3000 mg IV Q4-6h (maximum 12 g/day). • Children – 100-400 mg/kg/day IM/IV in divided doses Q4-6h.

Indications • Skin and skin structure infections. Adverse Effects/Precautions • Hypersensitivity reactions (eosinophilia or rash). • Maculopapular rash (concomitant with viral infections, e.g. infectious mononucleosis). • GIT – nausea, vomiting, abdominal pain, diarrhea. • Black hairy tongue, glossitis, stomatitis, sore tongue. • Pregnancy: category B (FDA). Drug Interactions • Probenecid- interferes with excretion of ampicillin. • OCPs- Ampicillin may decrease the efficacy of OCPs. • Allopurinol-Concomitant allopurinol increases the risk of ampicillin rash. CEPHALEXIN Pharmacology • First generation cephalosporin; β-lactam antibiotic. • Same mechanism of action as penicillin but binds to different proteins. • Well absorbed orally. • Attains high concentration in bile. • Excreted unchanged in urine.

Dosage • Adults: 250-500 mg Q6h (maximum: 4 g/day). • Children: 25-50 mg/kg/day in divided doses Q6h. Indications • Skin and skin structure infections (staphylococcal/streptococcal). • Genitourinary tract infections (E. coli/P. mirabilis/K. pneumoniae). Adverse Effects/Precautions • Generally well tolerated. • GIT- nausea, vomiting, diarrhea. • Hypersensitivity reactions (cross allergenicity with penicillin). • Pregnancy: category B. ERYTHROMYCIN Pharmacology • Macrolide antibiotic; binds to the 50S ribosome subunit and interferes with protein synthesis. • Activity against mostly gram positive, and few gram negative organisms-Streptococcus pyogenes, Streptococcus pneumoniae, some Staph. aureus, N.gonorrheae, N.meningitides, B.pertussis, Treponema pallidum, Mycoplasma, Legionella, Chlamydia and Campylobacter. • Available as several different derivatives – estolate salt is the least susceptible to acid degradation and the best absorbed salt, compared to the base, stearate and ethylsuccinate formulations. • Maximum absorption when taken on empty stomach (except estolate – taken with food). • Extensively metabolized in the liver, and excreted in bile. • Penetration of CSF is negligible. Dosage All derivatives except ethylsuccinate: 250-500 mg every 6 hours (expressed in terms of base) – maximum 4g/day.

Topical Formulary and Key Systemic Drugs Ethylsuccinate: 400 mg is considered equivalent to 250 mg of base.

Indications As an alternative to penicillin for – Skin and skin structure infections of mild to moderate severity caused by Strept. pyogenes or Staph. aureus. – Erythrasma. – Streptococcal respiratory tract infections. – Diphtheria. – Tetanus. – Syphilis and gonorrhea. – Chlamydial infections. As a first choice drug for – Chancroid.

Adverse Effects/Precautions • GIT- nausea, vomiting, diarrhea. • Allergic reactions (more common with estolate). • Reversible cholestatic hepatitis (with estolate). • QT prolongation. • Estolate derivative contraindicated in hepatic dysfunction; all others used with caution. • Pregnancy: category B.

Drug Interactions • CYP3A4 inhibitor - Increased levels of theophylline, warfarin, phenytoin, carbamazepine, disopyramide and cyclosporine. • Increased risk of myopathy when used with HMG CoA reductase inhibitors (atorvastatin, simvastatin, lovastatin).

• Expanded spectrum of activity compared to erythromycin: retains similar gram positive cover, but has greater gram negative cover (N. gonorrheae, N. meningitidis, H. ducreyi, H. influenzae, M. catarrhalis, B. pertussis). • More acid stable than erythromycin. • Widely distributed; persists in pulmonary macrophages, polymorphonuclear leukocytes and genital/pelvic tissues for several days. • Negligible penetration of CSF. • Excreted primarily in feces.

Dosage • Adults: 500 mg on day 1, followed by 250 mg once a day for 5 days. • Chlamydia and chancroid: 1g single dose. Indications • Skin and skin structure infections (Staph. aureus, Strep. pyogenes, Strep. agalactiae). • Nongonococcal urethritis and cervicitis (Chlamydia). • Prevention of disseminated M. avium complex disease in HIV infected patients. • Chancroid. Adverse Effects/ Precautions • GIT- nausea, vomiting, diarrhea, abdominal pain. • Rare allergic reactions. • Reversible elevation of LFT. • Pregnancy: category B. Drug Interactions • Does not affect cytochrome P450 enzymes. • Warfarin – increased PT and INR can occur; close monitoring required.

AZITHROMYCIN Pharmacology • Macrolide antibiotic; inhibits bacterial protein synthesis by binding to the 50S ribosomal subunit.

TETRACYCLINE Pharmacology • Inhibits protein synthesis by binding to the 30S ribosomal subunit, thereby preventing

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Essentials in Dermatology attachment of the aminoacyl t-RNA to the A site of the mRNA-ribosome complex; primarily bacteriostatic. • In acne – inhibits growth of propionibacterium acnes and decreases free fatty acid concentration in sebum, resulting in decreased inflammation and microcomedo formation. • Maximal absorption on empty stomach (food decreases absorption by 50% or more). • Eliminated primarily unchanged in urine (needs dose adjustment in renal insufficiency).

Dosage • Acne vulgaris – 500-1000 mg/day in 4 divided doses for 1-2 weeks or till clinical improvement, followed by 125-500 mg/day. • Syphilis. – primary or secondary: 500 mg QID for 2 weeks – latent: 500 mg QID for 2 weeks (if duration of infection < 1 year) 500 mg QID for 4 weeks (duration of infection unknown or >1 year) Indications • Acne vulgaris. • Primary, secondary or latent syphilis in nonpregnant patients with documented penicillin allergy. Adverse Effects/ Precautions • GIT - nausea, vomiting, diarrhea, abdominal pain, epigastric burning, anorexia. • Maculopapular or erythematous rashes. • Hypersensitivity reactions. • Hemolytic anemia, thrombocytopenia, neutropenia and eosinophilia. • Photosensitivity. • Precipitation of renal failure in patients with underlying renal function. • Discoloration of teeth (in children < 8 years, or infants of pregnant mothers who ingested tetracycline).

• Superinfection with Candida (oral and anogenital). • Use of discarded or outdated tetracycline has resulted in an adverse event similar to Fanconi’s syndrome (patients should be instructed to discard any unused tetracycline after therapy). • Pregnancy: category D.

Drug Interactions • Absorption of tetracycline is impaired by dairy products, flouroquinolones, sucralfate, or any product containing iron, zinc, calcium, aluminium, magnesium or bismuth (separate administration by 4 hours). • OCPs – decreased efficacy of OCPs. • Warfarin – elevated PT. DOXYCYCLINE Pharmacology • Inhibits protein synthesis by binding to the 30S ribosomal subunit, thereby preventing attachment of the aminoacyl t-RNA to the A site of the mRNA-ribosome complex; primarily bacteriostatic. • In acne – inhibits growth of propionibacterium acnes and decreases free fatty acid concentration in sebum, resulting in decreased inflammation and microcomedo formation. • Spectrum of activity. – Cocci: all gram positive and gram negative cocci were originally sensitive but now many Strep. pyogenes, Strep. pneumoniae, Staph. aureus and enterococci have become resistant – Gram positive bacilli: Clostridia, Listeria, Corynebacteria, Propionibacterium acnes, Bacillus anthracis – Gram negative bacilli: H.ducreyi, Calymmatobacterium granulomatis, Vibrio cholerae, Yersinia pestis, Yersinia enterocolitica, Campylobacter, H. pylori, Brucella, Pasturella, Francisella

Topical Formulary and Key Systemic Drugs – Spirochetes: Treponema pallidum, Borrelia – Rickettsiae – Chlamydia – Mycoplasma, Ureaplasma • Maximum absorption on empty stomach (food may decrease absorption by up to 20%); should be taken with adequate fluids to reduce esophageal irritation/ulceration; can be given with food if gastric irritation occurs. • Equally excreted in feces and urine (unlike other tetracyclines); dose adjustment not needed in renal insufficiency.

Dosage • 100 mg twice a day. Indications Acne vulgaris 1st choice treatment in: – Lymphogranuloma venereum – Granuloma inguinale – Typhus, Rocky mountain spotted fever, Q fever (Rickettsiae) 2nd choice treatment in: – Tetanus, anthrax, actinomycosis, Listeria (to penicillin/ampicillin) – Gonorrhea (to ciprofloxacin/ceftriaxone, especially for penicillin-resistant, nonPPNG organisms and in penicillin allergic cases) – Chlamydial infections – non-gonococcal urethritis, endocervicitis, conjunctivitis, pneumonia (to azithromycin) – Chancroid (to cotrimoxazole)

Adverse Effects/Precautions • GIT- nausea, vomiting, diarrhea, abdominal pain, epigastric burning, anorexia. • Pseudotumor cerebri (increased risk when combined with isotretinoin).

• Photosensitivity. • Discoloration of teeth (in children < 8 years and infants of pregnant women taking the drug). • Superinfection with Candida (oral/ anogenital), pseudomembranous enterocolitis. • Pregnancy: category D.

Drug Interactions • Absorption of doxycycline is impaired by dairy products, flouroquinolones, sucralfate, or any product containing iron, zinc, calcium, aluminium, magnesium or bismuth (separate administration by 1-2 hours). • OCPs – decreased efficacy of OCPs. • Warfarin – elevated PT. MINOCYCLINE Pharmacology • Inhibits protein synthesis by binding to the 30S ribosomal subunit, thereby preventing attachment of the aminoacyl t-RNA to the A site of the mRNA-ribosome complex; primarily bacteriostatic. • In acne – inhibits growth of Propionibacterium acnes and decreases free fatty acid concentration in sebum, resulting in decreased inflammation and microcomedo formation. • Good activity against rickettsia, chlamydia, mycoplasma. • Also displays activity against Staph. aureus resistant to other tetracyclines. • Maximum absorption on empty stomach (food may decrease absorption by up to 20%); should be taken with adequate fluids to reduce esophageal irritation/ulceration; can be given with food if gastric irritation occurs. • Eliminated primarily via the hepatobiliary system.

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Dosage • Acne vulgaris: 50 mg OD – TID. • Infections due to susceptible organisms: 200 mg initial dose (orally or IV), followed by 100 mg Q12h. Indications • Moderate to severe inflammatory acne vulgaris. • Can be used to treat rickettsial, chlamydial, mycoplasmal infections (although doxycycline is the preferred drug). Adverse Effects/ Precautions • GIT – nausea, vomiting, diarrhea, abdominal pain, epigastric burning, anorexia. • Vestibular side effects – dizziness, ataxia, vertigo (dose-related, more common in women). • SLE – rare, reversible. • Pseudotumor cerebri (increased risk when combined with isotretinoin). • Reversible skin and mucous membrane pigmentation occurs with long-term use • Photosensitivity – may be less than other tetracyclines. • Discoloration of teeth (in children < 8 years and infants of pregnant women taking the drug). • Superinfection with Candida (oral and anogenital) less frequent than tetracycline and doxycycline. • Pregnancy: category D. Drug Interactions • Absorption of minocycline is impaired by sucralfate, or any product containing iron, zinc, calcium, aluminium, magnesium or bismuth (separate administration by 1-2 hours). • OCPs – decreased efficacy of OCPs. • Warfarin – elevated PT.

CIPROFLOXACIN Pharmacology • Fluoroquinolone antibiotic; inhibits bacterial DNA gyrase. • Most reliable for treatment of aerobic gram negative organisms. – Enterobacteriaceae – Neisseria gonorrheae, Neisseria meningitides – H. influenzae, H. ducreyi – Campylobacter jejuni – Yersinia enterocolitica • Moderately susceptible organisms include some streptococci, staphylococci, Pseudomonas aeruginosa, chlamydia, and mycobacteria. • Well absorbed after oral administration. • Widely distributed throughout the body; high tissue penetrability (except CSF). • Eliminated primarily via the kidney (dose adjustment needed in renal insufficiency).

Dosage 250-750 mg BD, depending on the indication.

Indications • • • •

Skin and skin structure infections. Gonorrhea. Bone and joint infections. Urinary tract infections (cystitis, pyelonephritis). • Intra-abdominal infections, diarrhea (in combination with metronidazole). • Typhoid. • Sinusitis, lower respiratory tract infections.

Adverse Effects/ Precautions • GIT – nausea, vomiting, bad taste, anorexia. • CNS – dizziness, headache, restlessness, anxiety, insomnia, impairment of concentration and dexterity (caution while driving), tremor, seizures.

Topical Formulary and Key Systemic Drugs • Hypersensitivity reactions – rash, pruritus, photosensitivity, urticaria, swelling of lips, fever, hepatic necrosis, fatal anaphylaxis. • Tendonitis and tendon rupture. • Cartilage damage in weight bearing joints (in animal studies) – not recommended for children. • Pregnancy: category C.

• Not effective against Pseudomonas, enterococci, and most anaerobes • Increased resistance being seen with H. influenzae, E. coli, staphylococci and streptococci • Well absorbed orally. • Excreted primarily in urine (dose adjustment needed in renal insufficiency).

Drug Interactions • Products containing multivalent cations such as iron, calcium, zinc, magnesium, aluminium (e.g. antacids, multivitamins, sucralfate) impair the absorption of ciprofloxacin – should be administered 6 hours before or 2 hours after ciprofloxacin. • Theophylline – ciprofloxacin can increase theophylline concentrations. • Warfarin – increased PT.

Dosage • All preparations contain TMP and SMX in the ratio of 1:5. • Oral – one double strength TMP/SMX (160/ 800 mg) tablet BD. • Intravenous – 8-20 mg/kg/day of TMP in 2-4 divided doses.

TRIMETHOPRIMSULPHAMETHOXAZOLE (SEPTRAN) Pharmacology • Trimethoprim is a diaminopyrimidine related to the antimalarial drug pyrimethamine, which selectively inhibits bacterial dihydrofolate reductase (DHFRase). • Sulphamethoxazole belongs to the class of sulfonamides, which are structural analogues of PABA, and thereby inhibit bacterial folate synthetase. • The combination (TMP-SMX) results in a synergistic bactericidal effect on gram positive and gram negative organisms – Strep. pneumoniae – Strep. pyogenes – Staph. aureus – H. influenzae – Moraxella catarrhalis – Nocardia species – Stenotrophomonas maltophila – Enterobacteriaceae (most) – Pneumocystis jiroveci

Indications • Chancroid (1st choice agent). • Granuloma inguinale (2nd choice agent). • Toxoplasmosis. • Nocardia infections. • Urinary tract infections – treatment and prophylaxis. • Respiratory tract infections (acute otitis media, acute exacerbations of chronic bronchitis, sinusitis). • Diarrhea and dysentery. • PCP pneumonia. Adverse Effects/ Precautions • GIT – nausea, vomiting, anorexia. • Hypersensitivity – rash, urticaria, StevensJohnson syndrome, TEN, erythema multiforme and exfoliative dermatitis. • Agranulocytosis, aplastic or hemolytic anemia (folate deficient patients at increased risk). • Fulminant hepatic necrosis. • HIV infected patients are at much greater risk of dermatologic and hematologic reactions, and desensitization may be required. • Crystalluria (advised to drink plenty of water). • Pregnancy: category C.

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Drug Interactions • Warfarin – antithrombotic effects can be potentiated by TMP/SMX. • Sulfonylureas – hypoglycemia as a result of displacement from protein binding sites. • Methotrexate – displacement from protein binding sites, and competition for renal tubular excretion, leading to bone marrow suppression. CORTICOSTEROIDS The corticosteroids bind to specific cytoplasmic receptors in the target cell and the steroid receptor complex then undergoes modification and is translocated to the nucleus where it causes formation of specific mRNA which then mediates the various biological effects. Their modes of actions are 1) Anti-inflammatory, 2) Antiallergic and 3) Immunosuppressive.

Dosage The intermediate acting steroid namely prednisolone is commonly used at a dose of 1 mg/kg body weight/day. Indications 1. Autoimmune bullous dermatoses (pemphigus, bullous pemphigoid, epidermolysis bullosa acquisita, etc.). 2. Drug eruptions (erythema multiforme, Stevens Johnson syndrome, TEN). 3. Connective tissue disorders (SLE, polymyositis, dermatomyositis, progressive systemic sclerosis). 4. Severe dermatitis (atopic dermatitis, contact dermatitis, photodermatoses). 5. Neutrophilic dermatoses (pyoderma gangrenosum, Sweet’s syndrome). 6. Sarcoidosis. 7. Leprosy in severe reaction. 8. Urticaria, angioedema and capillary hemangiomas.

Contraindications There are no absolute contraindications The relative contraindications are: Peptic ulcer disease, any focus of infection including tuberculosis, hypertension, glaucoma, cataract, hyperlipidemia, renal calculi, first trimester of pregnancy, and children. Side Effects 1. Metabolic disturbances: Diabetes, faciotruncal obesity, hyperlipidemia, protein hypercatabolism, etc. 2. Bone disturbances: Osteoporosis, avascular necrosis of the bone, growth retardation. 3. Muscular disorders: Steroid myopathy, amyotrophy, tendon rupture. 4. Cutaneous effects: Acneiform eruptions, atrophy, telangiectasia, easy bruising, alopecia/hypertrichosis, cutaneous infection, striae distense. 5. Electrolyte disturbances: Sodium retention and potassium depletion. 6. Endocrine changes: Inhibition of the hypothalamo-hypophyseal suprarenal axis. 7. Infections: Acute bacterial infection, tuberculosis, viral infection, parasitic disease. 8. Neuropsychiatric disorders: Neuropathy, psychosis, pseudotumor cerebri. 9. Ocular effects: Posterior subcapsular cataract, open angle glaucoma, aggravation of an existing infection. 10. Gastric disturbances: Peptic ulcer disease. 11. Hematologic alteration: Leucocytosis, eosinopenia and lymphopenia. GRISEOFULVIN Griseofulvin is the most frequently used systemic drug in the treatment of dermatophytosis. It is a fungistatic agent derived from Penicillium griseofulvum with selective activity against dermatophytes only. In addition to its antifungal

Topical Formulary and Key Systemic Drugs activity, it is also a weak vasodilator and inhibits leucocyte chemotaxis.

herpes zoster, the dose is 800 mg orally 5 times a day for 7 to 10 days.

Dosage The daily recommended dose is 10-20 mg/kg body weight/day to a maximum of 1 gm daily, given in a single dose or in two divided doses. For ringworm of the trunk, hand or foot, it is given for a period of 4 to 6 weeks. For scalp infection 8 to 12 weeks and in nail infection for 12 to 18 months.

Side Effects

Side Effects The most commonly reported adverse effects are headache and gastrointestinal side effects namely dyspepsia, nausea and diarrhoea. Other side effects are photosensitivity, urticaria, erythema multiforme, maculopapular skin rash, serum sickness, angioedema and vesicular eruption. The other uncommon but serious adverse effects include hepatic and renal insufficiency, severe leukopenia, peripheral neuropathy, mental confusion and blurred vision. It can also precipitate SLE. ACYCLOVIR Acyclovir is a synthetic guanosine analogue which is widely used for the treatment of herpes simplex virus and varicella zoster virus infections. Acyclovir, a prodrug, initially undergoes monophosphorylation by herpes virus encoded thymidine kinase and is activated to acyclovir triphosphate (by cellular kinase) which is the active antiviral moiety. Acyclovir is highly selective because only virus infected cells are able to phosphorylate acyclovir.

Dosage The recommended dose for the treatment of HSV infection is 200 mg 5 times a day or 400 mg three times a day for 7 to 10 days. For varicella and

They are renal crystalluria, interstitial nephritis, seizures, tremors, etc.

ANTIHISTAMINES Classification Class I: First generation H1 type antihistamines – These traditional antihistamines are competitive receptor blockers (reversible) and can be displaced by high levels of histamines and they dissociate from the receptors easily. They are divided into 6 groups. 1. Alkylamine – Pheniramine maleate (25-50 mg 2-3 times daily) – Chlorpheniramine maleate (4 mg thrice daily) 2. Ethanolamine – Clemastine fumarate (1 mg twice daily) – Embramine hydrochloride (25-50 mg/ day) – Bromodiphenyl hydramine hydrochloride – Diphenhydramine hydrochloride(25-50 mg thrice daily), citrate 3. Ethylenediamine – Mepyramine maleate – Pyrilamine maleate – Tripelamine citrate, HCl 4. Phenothiazine – Promethazine HCl (25 mg twice daily) – Methdilazine HCl 5. Piperidine – Cyproheptadine HCl (4 mg thrice daily) – Azatidine maleate 6. Piperazine – Hydroxyzine hydrochloride (10 mg or 25 mg thrice daily).

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Essentials in Dermatology Mechanisms of action: The main effects are as follows: 1. Anticholinergic activity 2. Anti-inflammatory effect 3. Antiallergic effect 4. Antimotion sickness effects 5. Antiemetic activity 6. Inhibit most responses of smooth muscle to histamine.

Indications

Side Effects 1. Neurological side effects – Sedation, dizziness and tinnitus, blurred vision and diplopia, insomnia, reduced or disturbed concentration, irritability and tremors. 2. Gastrointestinal side effects – Anorexia, nausea, vomiting, epigastric distress, constipation (can be minimized by giving them along with food). 3. Anticholinergic effects – dry mucous membranes, difficulty in micturition and urinary retention, frequency and dysuria, impotence. 4. Cutaneous side effects –eczematous dermatitis, fixed drug eruption, photosensitivity, urticaria, petechiae.

3. Mast cell disorders: Ketotifen, cimetidine, azelastine and chlorpheniramine are very effective.

Class II: Second generation H1 antihistamines. They are non-competitive blockers and dissociate slowly from the receptors and have a longer duration of action. They are devoid of sedative and anticholinergic effects. These drugs are: acrivastine, astemizole, azelastine, cetirizine, ebastine, fexofenadine, ketotifen, loratidine, mizolastine, terfenadine. The drugs commonly used are cetirizine (10 mg/day), loratidine (10 mg/day), terfenadine (60 mg BD) and astemizole (10 mg/day), with a loading dose of 10 mg tds x 3 days). Class III: H 2 antihistamines – Cimetidine, ranitidine, famotidine, nizatidine. Class IV: Other drugs with antihistamine activity – Tricyclic antidepressants (doxepin), ketotifen, oxafomide.

1. Urticaria and angioedema: Both traditional and second generation antihistamines are found to be useful. The combination of both H1 and H2 antihistamines are very effective in the treatment of acute or chronic idiopathic urticaria and physical urticarias. 2. Pruritus of various causes: viz. dermatitis, papulosquamous disorders, insect bites, etc.

4. Pruritus due to: Myelofibrosis, polycythemia vera and carcinoid flush. Cimetidine is also effective. 5. The immunomodulatory effects of H 2 antihistamines may offer great therapeutic potential. 6. Other uses include, their use in motion sickness, vertigo, preanesthetic medication, as sedative, hypnotic and anxiolytic.

DAPSONE Dapsone is a 4,4’-Diaminodiphenyl sulphone. It is weakly bactericidal against M.leprae. It acts by inhibiting folate metabolism in M.leprae. In addition to its antibacterial action, it also has antiinflammatory activity–inhibits lysosomal enzymes, interferes with myeloperoxidase system. During daily treatment with 100 mg dapsone, it inhibits the generation of 5-lipoxygenase product in neutrophils.

Dosage Dose is 6 to 10 mg/kg body weight/week. The average adult dosage is 100 mg/day. For children of 10 to 14 years, it is 50 mg/day and of 6 to 9 years it is 25 mg/day. Indications Other than its antileprosy action, it is used in dermatology in pemphigus, bullous pemphigoid,

Topical Formulary and Key Systemic Drugs dermatitis herpetiformis, subcorneal pustular dermatosis, acne conglobata, systemic lupus erythematosus, vasculitis, lichen planus etc.

Side Effects 1. Hemolytic anemia especially in G6PD deficient individuals. 2. Agranulocytosis. 3. Hepatitis. 4. Allergic rashes including FDE and exfoliative dermatitis. 5. Dapsone syndrome (fever, jaundice, lymphadenopathy, hepatomegaly and exfoliative dermatitis). 6. Psychosis. CLOFAZIMINE Clofazimine is a riminophenazine dye. Like dapsone, it is also weakly bactericidal against M.leprae and virtually nontoxic in the usual dosage used. Clofazimine has both antibacterial and anti-inflammatory activities.

Indications Apart from its use in multidrug therapy in leprosy, it is used in dermatology for multidrug resistant tuberculosis, atypical mycobacterial infection, neutrophilic dermatoses (acne, pustular psoriasis), rhinoscleroma, leishmaniasis, etc.

Dosage The recommended dose is 50 mg daily.

Side Effects 1. Reversible dose related reddish to brownish black discoloration of sweat, hair, sputum, urine and feces. 2. Ichthyosis. 3. Phototoxicity, nonspecific skin rashes and acneiform eruptions.

4. Eosinophilic enteropathy. 5. Conjunctival pigmentation.

METHOTREXATE Methotrexate (MTX) is a folic acid antagonist and is widely used for the treatment of severe psoriasis.

Dosage 1. Weekly single oral or parenteral dosage schedule. 2. Intermittent oral schedule of three divided doses 12th hourly over a period of 36 hours each week. 3. Oral dose is 0.4 to 0.6 mg/kg body weight/ week (maximum 30 mg). 4. Parenteral dose is 15-25 mg/week. Indications 1. Psoriasis – psoriatic erythroderma, psoriatic arthritis, pustular psoriasis, extensive psoriasis vulgaris. 2. Reiter’s syndrome and pityriasis rubra pilaris. 3. Sarcoidosis. 4. Polymyositis and systemic lupus erythematosus. 5. Pemphigus vulgaris and other bullous disorders. Side Effects 1. Gastrointestinal – nausea, vomiting, stomatitis, diarrhoea, ulceration, etc. 2. Hepatitic – abnormal liver enzymes, hepatic, fibrosis and cirrhosis. 3. Hematopoietic – anemia, thrombocytopenia, leucopenia, pancytopenia. 4. Pulmonary – acute hypersensitivity, fibrosis, pneumonia. Most common side effect is nausea and vomiting. Serious late side effect is cirrhosis of the liver.

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PSORALENS Of the many psoralens isolated and synthesized, three are in routine clinical use. These include 8 methoxypsoralen (8-MOP), 5 methoxypsoralen (5-MOP) and trimethyl psoralen (TMP). They are active only when combined with UVA irradiation. It interferes with DNA synthesis and blocks epidermal cell proliferation, has immunomodulatory effects and immunosuppressive effects along with induction of epidermal thickening and melanogenesis.

Dosage Oral 8-MOP is given in a dose of 0.6 to 0.8 mg/kg body weight/day. Two hours later, the patient is exposed to ultraviolet radiation A (UVA) at a dose of 1 joule/square cm to begin with, which is gradually increased to minimal erythema dose depending on the skin type. Therapy is usually given two to three times per week. The number of exposures required for achieving control are usually between 15 and 25. Maintenance therapy involves less frequent treatments often as little as once every two to four weeks, with eventual discontinuation of treatment. Indications 1. Psoriasis. 2. Vitiligo. 3. Photodermatoses. 4. Cutaneous T-cell lymphomas. Side Effects 1. Nausea, vomiting. 2. Cutaneous – Acute sunburn and erythema, irreversible hyperpigmentation of the skin, PUVA lentigines, actinic keratoses, premature ageing of the skin. 3. Ocular – cataract. It can be prevented by using sunglasses on the day of treatment. 4. Carcinogenesis.

Other than oral PUVA therapy, psoralen may be used for topical PUVA therapy and bath PUVA therapy.

RETINOIDS Retinoids is a generic term that includes both naturally occurring molecules and also synthetic compounds showing specific biological activities resembling those of vitamin A(retinol). Synthetic retinoids are produced by chemical modification of vitamin A. Three generations are known today(nonaromatic, monoaromatic, and polyaromatic). First generation(Nonaromatic) includes retinal and compounds that can be derived from it metabolically e.g. retinyl palmitate(topical), retinyl aldehyde(topical), tretinoin(all trans retinoic acid)(topical), 9-cis-retinoic acid, a-14hydroxyretroretinol, fenretinide(N-[4-hydroxyphenyl]-retinamide), E 5166(polyprenoic acid). Second generation(Monoaromatic) by addition of an aromatic ring e.g. etretinate, isotretinoin, acitretin, isoacitretin(13-cis-acitretin), motretinide. Third generation(Arotinoids) e.g. temarotene, arotenoid acid, arotenoid ethyl ester, arotenoid ethyl sulphone, arotenoid methyl sulphone, adapalene(topical antiacne agent), tazarotene (topical antipsoriatic agent). Intracellularly retinoids interact with cytosolic proteins and then enter the nucleus. By changing the expression of growth factors, oncogenes, keratin or transglutaminases, retinoids could exert wide spread changes in growth and differentiation.

Indications of Systemic Retinoids 1. Psoriasis and related disorders. 2. Other disorders of keratinization. 3. Seborrhoea, acne and acneiform dermatoses. 4. Rosacea and other acne related dermatoses.

Topical Formulary and Key Systemic Drugs 5. Cancers-basal cell carcinoma, actinic keratosis, cutaneous T-cell lymphomas, HIV related Kaposi’s sarcoma. 6. Other dermatoses such as lichen planus, lupus erythematosus, lichen sclerosus et atrophicus, prurigo nodularis, photoageing and ageing, pigmentary disorders, etc.

Side Effects of Systemic Retinoids The adverse effect profile closely resembles those of hypervitaminosis A. Acute toxicity is dose dependent and reversible. 1. Cheilitis appears 2-3 weeks after initiation of therapy in 100% of patients and is regarded as a marker of sufficient absorption. Other mucocutaneous adverse effects are skin and

mucosal dryness, skin fragility and/or stickiness, retinoid dermatitis, palmoplantar desquamation, pruritus, hair loss, paronychia, and photosensitivity. 2. Eye symptomatology and pseudotumor cerebri. 3. Serum lipids and liver function abnormalities. Chronic Toxicity 1. Bone changes include hyperostosis, periostosis, osteoporosis, thinning of the bones, premature closure of epiphyses, DISH(diffuse idiopathic skeletal hyperostosis), extraspinal calcification, bone pain and acute arthritis. 2. Arthralgia and myalgias 3. Teratogenicity involving central nervous system, skeletal system, cardiovascular system, and other organs.

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Dermatosurgical Procedures

SKIN BIOPSY Skin biopsy is the commonest investigation performed by any dermatologist. The diseased tissue obtained is subjected to microscopic and/ or other investigations.

Indications 1. Most frequently skin biopsy is taken to confirm a clinical diagnosis or to aid in the establishment of a diagnosis where clinical diagnosis is not apparent. 2. Sometimes, biopsy is excisional for the treatment of skin lesions particularly malignant neoplasms and other lesions for cosmetic reasons. 3. Skin biopsy may be used for a variety of investigative procedures such as ultrastructural examination, immunofluorescence studies, enzyme histochemistry and immunohistochemistry, microbiological studies, tissue culture, etc. Site of biopsy: Ideally, the lesion biopsied should be an early and untreated lesion and representative of the skin disorder as a whole. If lesions are present at all stages of evolution, it may be appropriate to biopsy more than one lesion. Normal skin should be included with a diagnostic biopsy wherever possible.

Written consent is normally obtained from the patient and local anesthesia with 1% or 2% lignocaine with or without adrenaline is injected around the biopsy site. Techniques of skin biopsy: Elliptical surgical biopsy, punch biopsy, curettage or shave biopsy may be undertaken. Elliptical surgical biopsy: A reasonable size for an elliptical biopsy is about 12 x 5 mm. It is undertaken with a scalpel and the wound is sutured. Punch biopsy: The biopsy punch of at least 5 mm is required to obtain satisfactory specimen. The punch is pushed into the skin with a downward twisting movement and then removed. The tissue specimen is lifted and cut. The wound may be left to heal without suturing. The biopsy specimen is put in 10% formalin solution for histopathologic studies. It is properly labeled and sent to pathology department with full clinical details of the case.

ELECTROSURGICAL PROCEDURES IN DERMATOLOGY Electrical current of sufficiently high frequency not to stimulate nerves or muscles can pass through tissue with little effect other than the

Dermatosurgical Procedures production of heat. Heat is produced from the electrical resistance of the tissue as current travels from one electrode to the other. When the electrodes are both large (as in medical diathermy), the heat is dispersed over a large area, and no injury ensues. However, if the emitting electrode is reduced to a small tip, the heat produced at the point of contact is sufficiently intense to cause localized tissue injury. As might be anticipated, variation in the voltage, amperage, frequency, and method of application gives each of the electrosurgical modality its unique qualities. Equally important, however, is the waveform of each current. In practice, a spark-gap apparatus produces damped waves, whereas a vacuum tube or transistorized unit creates continuous waves. Examples of the former include the Hyfrecator (Birtcher Corp., El Monte, CA) commonly employed in dermatology.

Electrodesiccation and Electrofulguration The difference between electrodesiccation and electrofulguration lies in the placement of the elec-trode tip. In electrodesiccation (Lat. Siccus ‘dry’), the tip contacts the skin and causes a radial spread of current . In electrofulguration [Lat. fulgur ‘lightning’] the tip remains above the skin and the charge leaps to the surface in a diffuse, or “defocused,” pattern , causing flatter and more superficial tissue destruction. The histologic outcome of both modalities is one of tissue desiccation. As a result of the low heat production, electrodesiccation and electrofulguration are best suited for superficial and comparatively avascular lesions such as verrucae and seborrheic keratoses. Electrocoagulation Electrocoagulation [Lat. Coagulum ‘clot’ or ‘curd’] is produced by a high-frequency

alternating current of high amperage (2,5004,000 mA) and low voltage (<200 volts). This current is generated by a spark-gap apparatus applied biterminally through one active and one dispersive electrode; the patient is an integral part of the circuit. The depth of penetration makes electrocoagu-lation useful in the removal of thick lesions. The greater production of heat and conduction of cur-rent along vessels make electrocoagulation supe-rior to electrodesiccation for hemostasis in a “wet” field and for destruction of highly vascular lesions (e.g. pyogenic granuloma).

Electrosection Electrosection [Lat. secare ‘to cut’] may be achieved by using either an undamped continuous current or a mildly damped pulsed current. The former is generated by a vacuum tube or solid-state unit, whereas the latter is produced by a spark-gap apparatus. Continuous waves cause intense but sharply limited heat production, resulting in localized tissue destruction with little effect on the immediately adjacent tissue. Microscopically, an incision approximately 0. 1 mm wide results from disintegration of cells along the cutting line. Electrolysis Electrolysis [Gr. Lysis ‘dissolution’] makes use of low-voltage, low-amperage direct (galvanic) current flowing unidirectionally between polarized electrodes. The resulting chemical ionization causes release of acids and metallic ions at the positive pole, with formation of hydroxides and attraction of metallic ions at the negative pole. The major electrosurgical application of electrolysis has been in epilation. Two disadvantages of electrolysis are that it is difficult to master and is time consuming. Although “electrolysis” is an inaccurate term for the high-frequency method of hair removal, it continues as the common expression solely by

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Essentials in Dermatology virtue of its frequent misusage. “Thermoepilation” [Gr. Therme ‘heat’ + e ‘out’ + pilus ‘hair’] is a more accurate and descriptive appellation.

Electrocautery Although not a true form of electrosurgery (no current actually enters the patient), electrocautery [Gr. Kauterion ‘branding iron’] is, by convention, usually included among the other modalities. Low-voltage, high-amperage, direct or alternating current is passed through a needle tip that is heated to red-hot temperatures by its resistance to the flow of current. Application of the heated needle tip to bleeding vessels causes coagulation. As with electrosection, too little heat causes coagulated tissue to adhere to the needle, whereas too much heat causes unnecessary destruction of tissue. Safety Considerations Potential interference of cardiac pacemakers from electrosurgical current has been known. Close attention to proper grounding is important to prevent unintentional shocks or burns. Particularly with the mono-terminal currents used for electrodesiccation and electrofulguration, care should be taken by the operator to maintain a firm, broad-based, skin-to-skin contact with the patient. Alcohol is commonly used to cleanse skin pre-operatively. If sufficient drying time is not allowed prior to electrosurgery, the alcohol can be ignited and the resulting flame spectacular.

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CRYOSURGERY IN DERMATOLOGY The term cryosurgery is derived from a Greek word Kryo meaning ice. This type of surgery is carried out by using freezing agents. The freezing agents are called as cryogens. • The anesthetic properties of hypothermia are known since Hippocratic times. Salt and ice mixtures were used in the 19th century to

•

relieve pain. Prolonged skin anaesthesia follows even light freezing, but the main purpose of cryotherapy is not to reduce pain but to destroy tissue. “Cold kills!” James Arndt of London pioneered refrigeration and he is often described as the “Father of modern cryosurgery”. Most practitioners now use liquid nitrogen as the source of coldness, the cryogen. The ability of cryosurgery to destroy both benign and malignant lesions is now well established. The spot freeze method, using either cotton buds or sprays are discussed below. Cotton bud method: This is used for benign lesions, in particular viral warts. A cotton bud, slightly smaller than the wart, is dipped into liquid nitrogen and applied firmly and vertically onto the wart (achieve a temperature of –20o C). A halo of 1 mm wide should form around the base of the wart, this may take up to 30 seconds for a large wart and treatment must then be stopped. Spray method: Here the delivery of nitrogen can be controlled accurately. The portable machine, which can be fitted with spray nozzles of different apertures, is used (achieve a temperature of –196 o C). To treat a basal cell carcinoma, with clear-cut margins, the nozzle is held 0.5-1 cm from the skin. Spraying then leads to the formation of a white ice field of slowly increasing diameter. The frozen area should include a 5 mm ring of normal tissue. The area is allowed to thaw slowly, to ensure maximum cell death. Two freeze-thaw cycles are better than one for the routine treatment of malignant tumors. To minimize inflammation, aspirin may be taken before treatment and then later on for 5 days or so. An anti-inflammatory dressing containing clobetasol propionate should also be applied daily for 3 days.

Dermatosurgical Procedures depends on the lasing medium, the molecules of which have been excited (e.g. CO2, ruby, argon, dyes, ND-YAG, etc.).

LASERS IN DERMATOLOGY LASER: Light Amplified by Stimulated Emission of Radiation. (first pioneered by Leon Goldman).

Principle Inside a laser, one photon stimulates the emission of another identical photon from molecules that are in an excited state. Two mirrors are placed on each end of the laser in exact parallel alignment so that the photons bounce back and forth through the excited medium and the light is amplified by stimulating more and more photons. The result is an impressively bright, monochromatic, highly collimated, coherent, and controllable beam of light. The wavelength of the light released

Laser Skin Interaction When the light released by the laser beam comes in contact with the skin, various molecules known as chromophores absorb the energy. These chromophores include various molecules like water, melanin, hemoglobin, nucleic acids, proteins and urocanic acid. Different chromophores have the ability to absorb lights of specific wavelengths and thus the type of laser used depends on the chromophore that needs to be targeted. For example, hemoglobin specifically absorbs blue, green and yellow light and thus lasers emitting light in these wavelengths (480 – 600 nm) are used for treatment of hemangiomas. After absorption of the light, the skin tissue is destroyed or remodeled by the various mechanisms of photothermal, photochemical and photomechanical effects.

Type of laser

Wavelength, nm

Typical uses

Alexandrite

755

Epidermal/dermal pigmentation, tattoos (black, blue, green),hair removal

Argon, argon dye

488–630

Vascular lesions (Port wine stain, hemangiomas), light source for photodynamic therapy

CO2

10,600

Vaporization/ablation of rhytides, verruca, seborrheic keratoses, actinic cheilitis,scars, photodamage

Erbium:YAG

2940

Rhytides, scars, photodamage

Krypton

520, 568

520-pigmented lesions, 568-vascular lesions

Nd:YAG

532, 1064

532-epidermal pigmentation,red tattoos; 1064-Q-switched-nonablative dermal remodeling, black tattoos, hair removal

Pulsed dye (yellow)

577–600

Vascular lesions (children and adults)

Pulsed dye (green)

510

Epidermal pigment, red tattoos

Ruby

694

Epidermal/dermal pigmentation, tattoos (black, blue, green), and hair removal

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DERMABRASION Dermabrasion means surgical abrasion in the planes of the epidermis and dermis. It can be achieved with the help of a manual metallic dermabrader or a rapidly rotating wire brush or diamond fraise.

How it works? Lesions and defects of the epidermis, papillary dermis, and upper reticular dermis can be partially or completely removed by surgically planing to the level of the reticular dermis. During the maturation phase of wound healing, fibroblasts replace and remodel collagen bundles in the papillary and upper reticular dermis. Indications Include: • Facial acne scars • Surgical and traumatic scars • Photoaging • Benign conditions—such as rhinophyma, adenoma sebaceum, epidermal nevi, syringomas, small cysts, milia, and seborrheic keratoses. • Superficial malignancies, including Bowen’s disease and superficial basal cell carcinomas. • Pigmentary disorders such as melasma, tattoos, and lentigines Postoperative complications include scarring, pigmentary changes, persistent erythema, and infection. The deeper the dermabrasion, the greater the risk of scarring.

CHEMICAL PEELS Chemical peeling also called chemical resurfacing or chemoexfoliation, involves the

application of one or more exfoliating agents to the skin, resulting in the destruction of portions of the epidermis and/or dermis with subsequent regeneration. Based on wound depth, peels can be classified as superficial (epidermal injury), medium depth (upper dermal injury) and deep peels (mid-dermal injury).

Indications Include • Actinic keratoses • Pigmentary lesions in the form of melasma or postinflammatory hyperpigmentation. • Acne vulgaris scars and other depressed scars. • Photoaging

Chemicals which are Used for Chemical Peeling Include • Trichloroacetic acid 10 to 35% • Resorcinol paste. • Jessner’s combination of resorcinol, salicylic acid, and lactic acid in ethanol. • Salicylic acid. • Alpha-hydroxy acids (AHAs) are naturally occurring carboxylic acids normally found in many foods. The AHAs include glycolic, lactic, malic, citric, and tartaric acids. Lactic acid and glycolic acid are used most commonly. • Topical tretinoin. • 55% phenolic Baker-Gordon formula penetrates into the deep dermis. Complications include pigmentary changes, scarring, infection (esp. herpes virus infection), and prolonged erythema.

Historical Milestones in Sexually Transmitted Diseases

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Historical Milestones in Sexually Transmitted Diseases

“Know syphilis in all its manifestations and relations, and all other things clinical will be added unto you”.

Alexandre Donne (in 1836) He identified a flagellate protozoon which was subsequently named Trichomonas vaginalis.

Sir William Osler, 1897 “Knowing AIDS” is to “Know medicine”.

Albert Neisser (1855-1916) Isolation of distinct causative organism for gonorrhoea in 1879.

Late Dr. Jonathan M. Mann (director, Global Programme for AIDS) John Astruc, the French physician (in 1736) He described genital herpes in 1736, and the first English translation appeared in his ‘Treatus of Venereal Disease’ in 1754. Bell (in 1793) He recognized genital warts as a distinct disease. Philippe Ricord (1799-1889) French Venereologist 1. Established the specificity of two infectionssyphilis and gonorrhoea. 2. Classified syphilis into primary, secondary and tertiary stages. Rudolf Virchow (1821-1902) Documented the range of systemic manifestations of syphilis and gonorrhea.

Fritz Schaudinn (1871-1906) Protozoologist and Erich Hoffman (1868-1959) syphilogist Provided final evidence for the cause of syphilis Paul Ehrlich (1854-1915) Nobel Laureate Immunologist Announced the discovery of arsenic compounds Salavarasan, a chemotherapeutic cure for syphilis, “Magic bullets”. Kenneth MacLeod (1844-1922AD) He while working in Indian medical service, in days of British Raj first described donovanosis in 1881. Caesar Boeck, Norwegian Syphilologist (1845-1930) In order to determine more accurately the spectrum of outcomes in syphilis infection, a prospective

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Essentials in Dermatology study was setup by Norwegian syphilologist, Caesar Boeck. He collected patients between 1891 and 1910 and his successor at the Oslo Dermatological Centre, Bruusgaard reassessed them in 1929. The data was further reanalyzed by Gjestland in 1955. This study is usually known as Oslo Study.

Brassereau (in 1852) Chancroid, one of the five classic venereal diseases, was first described in France. Augusto Ducreyi (1860-1940 AD) The causative organism of chancroid was identified while he was working in Naples in 1889. Diday and Doyon (in 1886) They published the monograph ‘Les Herpes Genitaux’ in which they observed that herpes often appeared after a venereal infection such as syphilis, chancroid, or gonorrhea. Caddy (in 1902) He was the first to record cases of lymphogranuloma venereum in India under the title of ‘Climatic bubo’. Colonel Charles Donovan (in 1905) He described the intracellular bodies “Donovan bodies”, which bear his name, and was the codiscoverer of etiological agent in Kala-azar. Halberstaedter and Prowazek (in 1907) Microbiological investigations of chlamydial infections began in 1907 when Halberstaedter and Prowazek detected intracytoplasmic chlamydial inclusions in the conjunctival scrapings of non-human primates. Lipshutz (in 1920s) He inoculated material from genital herpetic lesions into the skin of humans, eliciting clinical

infection within 48-72 hours in six persons and 24 days in one person.

HIV origin (1930) Sometime around 1930, a strain of SIV was transferred from chimpanzees to humans who hunted and butchered chimps for ‘bush meat’ somewhere in what is now the Republic of Cameroon, near the borders of the Gabonese Republic and the Republic of Congo. The virus is thought to have adapted to become HIV-1, the most widespread form of HIV found today. National STD Control Programme in India (1946) Started in 1946, continued to operate till 1991. Gardner and Dukes (in 1955) The syndrome of bacterial vaginosis was first described as “Haemophilus vaginalis vaginitis” in 1955 by Gardner and Dukes. They concluded that it was an STI as the isolated etiological agent, H. vaginalis (now renamed Gardnerella vaginalis), was found in the male contacts of the female cases. Genital Herpes as Venereal Disease (1966) Genital herpes was rarely considered in the differential diagnosis of genital ulcers prior to 1965. It was only in 1966 that herpes genitalis was recognized as a venereal disease. Dr Robert Gallo (in January 26, 1984) In the US National Cancer Institute (NCI), he isolated the virus that causes AIDS which he called as HTLV-III. Evolution of Antiretroviral Therapy Drugs and Regimens (1985 onwards) Zidovudine was first tested on humans in 1985 and introduced for treatment in March 1987. Between December 1995 and March 1996, three protease inhibitors (PIs)-indinavir (IDV),

Historical Milestones in Sexually Transmitted Diseases saquinavir (SQV), and ritonavir (RTV) got approval for their use.

retroviral drugs, replaced the expression “triple combination therapy”.

On June 21 1996, the US FDA approved the first non-nucleoside reverse transcriptase inhibitor (NNRTI) drug, nevirapine.

In November 1999, enfuvirtide, a drug from a new class of drugs called a fusion inhibitor, began clinical trials.

1996 onwards, combination therapy, highly active antiretroviral therapy (HAART) began to spread irreversibly.

National AIDS Control Programme (1992)

In 1995, David Ho came out with his slogan “hit hard and early”. In 1997, Highly Active Anti-Retroviral Therapy (HAART), consisting of three or more anti-

The arrival/spread of HIV infection and because of its strong relation with STD, the programme “National STD Control Programme in India” was brought under the purview of National AIDS Control Organization in the year 1992.

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History Taking and Examination in Sexually Transmitted Diseases (STDs)

History taking and examination of an STD patient is similar to that done in general medicine. The examination should be preceded by reassurance and evincing confidence in the patient. Most clinicians encounter no difficulty during routine patient interviews but are uneasy when faced with sexually related diseases. This is unfortunate as detection of STDs and other sexual problems is facilitated by a thorough sexual history. The brief explanation of the importance of thorough sexual history assures the patient that physician goal is to solve his or her problem. Identification of sexual contact is best deferred until a specific diagnosis is confirmed. Name: Calling the patient by name will not only help to make a doctor-patient relationship but also reduce patient apprehension and anxiety. Age: It is important to know the age of the patient, as STDs are more prevalent in reproductive age group. Sex: It determines variations in manifestations of STDs in males and females. Occupation: It may give a hint of certain occupations in which STDs are more frequent e.g., commercial sex workers, truck drivers etc, whether living at home or away from home.

Address: Address of the patient helps us in follow up and contact tracing. Marital status: Whether the person is staying single, unmarried or married, divorced, widow or widower, matters in sexual history. Privacy: Whenever possible only the practitioner and patient should be present during consultation. Ideally it should be conducted in a private room which is adequately sound proofed and doors kept closed.

History of Present Illness I. Chief complaints and associated symptomatology. Patients usually present with complaint of either discharge per urethra/vagina or genital sore. For the discharge per urethra/vagina, we should enquire about: 1. Amount of discharge- copious (gonorrhoea) or scanty (chlamydial infection, balanoposthitis). 2. Colour of discharge- milky white (bacterial vaginosis), yellowish (gonorrhoea, trichomonas infection), or blood stained (malignancy) 3. Consistency of discharge- thin watery or thick (gonorrhoea)

History Taking and Examination in Sexually Transmitted Diseases (STDs) 4. Whether had any relationship to micturition or not 5. Present all the time or increased during any particular time 6. Odour of discharge 7. In females-whether related to periods 8. Burning micturition 9. Pain during defecation, urethral discharge during defecation. For genital sore: 1. Number of lesions- single or multiple 2. Duration of sore(s) 3. Pain-painful (chancroid, herpes) or painless (primary chancre) 4. Discharge from the sore- serous discharge (primary chancre) 5. Associated swelling 6. Preceding erythema or burning sensation 7. History of recurrent vesicles 8. Any similar lesions before 9. Any local medication applied 10. History of phimosis after ulcer (chancroid). For genital growth: 1. Duration 2. Progression-slow or fast 3. Whether bleeds to touch 4. Associated features. For urinary symptoms: 1. Burning micturition- at onset of micturition (urethritis) or after coming in contact with vaginal walls (vaginitis, vulvitis). 2. Frequency of micturition- increased or decreased. 3. History of associated hematuria. 4. History of urgency, loin pain, fever with rigors to rule out pyelonephritis and urinary tract infection. 5. Strangury-gonorrhoea. Other symptoms like rash, pruritus, adenopathy, abdominal pain, dyspareunia, diarrhoea,

hematochezia, anogenital pain, pain during defaecation, pus stained stools (rectal gonorrhoea) etc. II. After asking for chief complaints, we should enquire about onset and duration of symptoms- whether started after sexual exposure, duration-whether of long duration or short duration III. Then, we should enquire about recent dates of sexual intercourse. It is important for knowing the incubation period like in gonorrhoea and chancroid-2-5 days, herpes-4-5 days, non specific urethritis-814 days, syphilis-9-90 days, lymphogranuloma venereum-3days-3 weeks, donovanosis-8-80 days, venereal warts-6-32 weeks, etc. IV. Sexual orientation of the patient should be found, whether heterosexual, homosexual, or bisexual. • Approximate number of recent sexual partners and known STDs if any among sexual contacts. • Use of any barrier method for prevention of STDs. • Previous STDs and therapy utilized. • Time of last urination because this might affect the presence of discharge.

Past History Past history of exposure if any, STD, medical or surgical illness, hospital admission, blood transfusion or medical illness complicating the disease. Treatment History Present and recent medications, especially antibiotics, topical and vaginal preparations. History of drug sensitivity or any genitourinary instrumentation e.g. cystoscopy, abortion, dilatation, curettage etc.

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Personal History Personal history regarding education, socioeconomic status, occupation, income, habits (alcohol use, drug abuse, smoking), staying alone or with family, relationship with spouse and frequency of sexual intercourse may be essential. Sexual History It should be taken in detail. Exact dates of sexual exposure, place of exposure, whether heterosexual, homosexual or bisexual, use of barrier contraceptives during intercourse may be enquired. Details of intercourse- genito-genital, genito-anal, genito-oral may have relationship to the diagnosis at hand. Further enquiry about the sexual partner whether he/she was having genital sore/discharge or any other related problem, whether the concerned person has single or multiple sexual partners need to be known, and the frequency of sexual intercourse. HIV Risk Assessment Activities known to be associated with increased risk of acquisition of HIV should be specifically enquired about. These include involvement in prostitution, male homosexuality or bisexuality, having injected drugs, sexual partners from areas of high endemicity, history of transfusions with blood or blood products, sexual contact with intravenous drug users, or (in the case of females) hemophiliacs, or bisexual male partners. Menstrual History History regarding last date of previous menstruation, whether menses are regular or irregular, flow heavy or scanty, contains fresh blood or clots, associated pain and history of post coital bleeding or intermenstrual bleeding may be helpful. Obstetric History Enquire about methods of contraception used if any (barrier, intrauterine device, oral pills

intake), has ever become pregnant, number of children if any and their health, death of children if any and cause of their death, history of miscarriage, abortion or stillbirth if any.

Family History Health status of siblings, any family history of similar problem or death if any and cause of death may be ascertained. This is relevant in cases of suspected congenital syphilis as it is more common if the mother did not receive adequate antenatal care. General Physical Examination Consideration should always be given to having another member of health care worker present when carrying out an intimate physical examination on the patient, who will reassure the patient, able to assist the practitioner in carrying out the examination and provides a witness as protection against allegation of indecency or misconduct. Systematic approach to physical examination is the best means of reassuring a patient. Since the manifestations of STDs are not limited to the anogenital region, a general physical examination of each patient is ideal. Relevant systemic examination may be undertaken after thorough genital examination. Genital Examination It should be done in privacy, in the presence of natural light if possible or good light source. Examination of the Male Patient • Patient should be fully exposed and undergarments inspected for staining with the discharge. • External genitalia and inguinal skin are examined visually and by palpation with gloved hands. • Care should be taken to inspect and palpate penis with attention to the urethral canal and scrotal contents for evidence of pain,


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inflammation, induration or edema, warts, or rashes. Hairy regions should be evaluated for the presence of lice, rust coloured excreta or nits attached to hair shafts. In an uncircumcised male, the foreskin should be retracted and concealed portion of foreskin and glans examined. Urethral meatus is then visualized for evidence of discharge or other abnormalities. With the penis held firmly at midshaft with one hand, urethral contents stripped anteriorly with free hand to elicit any discharge. Genital ulcers, vesicles, erosions should be carefully examined for their characteristics such as whether single or multiple, site of involvement, shape (circular or irregular), size, margin (clear cut, everted, undermined), depth (superficial or deep), floor (clean, covered with necrotic material, healthy granulation tissue), tenderness, induration, tendency to bleed on touch. The inguinal, external iliac, axillary and cervical group of lymph nodes are examined in detail (unilateral or bilateral, single or multiple, discrete or matted, consistency, tenderness, mobility, erythema of overlying skin, fixity to underlying tissue). The spermatic cord on both sides should be palpated between thumb and index finger for tenderness, thickening and asymmetry. Scrotum examined for erythema, asymmetry, and suppurative lesions. Scrotal contents palpated to know any abnormality. Digital rectal, anoscopic and sigmoidoscopic examination are recommended in homosexual males especially in those complaining of gastrointestinal and rectal symptomatology.

Examination of the Female Patient • After a brief explanation of the necessity for pelvic examination and steps involved, the

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patient should assume the lithotomy position on a table equipped with adjustable stirrups. The examiner, seated at the foot end of the table, should have both hands gloved and is encouraged to maintain eye contact with the patient. Patient may be draped to remove her apprehension. The external genitalia and pubic hair should be carefully inspected. Then stepwise examination of inguinal lymph nodes, vulvar skin, urethral meatus, introitus, Bartholin’s ducts and glands, and Skene’s ducts is completed. Evaluate for erythema, excoriation, inflammation, vaginal discharge, urethral discharge, induration, edema, ulceration, erosion, discoloration, and areas of abnormal appearing epithelium. For palpation and stripping of the urethra, a gloved index finger, lubricated with warm water, is inserted into the vagina and the urethra is then palpated through the anterior vaginal wall. Feel for edema, inflammation, induration or anomalies such as diverticuli. Compressing the urethra anteriorly against the pubic symphysis and milking it toward the meatus may express exudate from the urethra. Prior to speculum insertion, the same-gloved finger should be pressed gently but firmly against the posterior vaginal wall for several seconds. This allows for easy insertion of a speculum (due to perineal relaxation), lubricated with warm water. Proper angulation of speculum on insertion results in ready visualization of the cervix. Cervix should be examined for discharge, erosions, ectropion, ulceration and friability. To test friability of cervix, cotton tipped swab may be used. The blades of speculum can be used to gently compress the cervix in an attempt to exude endocervical discharge. After specimen collection is completed, the speculum is slowly removed and the entire area of the vaginal mucosa carefully examined.

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Essentials in Dermatology • The vagina should be examined for rugal folds, erythema, malodour, discharge, purulence, ulcerations and other abnormalities. • It is important to ascertain the character and source of discharge (physiological versus vaginal versus cervical). • Following speculum examination, bimanual examination including both abdominal pelvic and genitorectal assessment is important part of the pelvic examination. With one hand on the abdomen and the index and middle finger of the other hand inserted in the vaginal canal, stepwise palpation of the abdomen, vagina, cervix, uterus and adnexal structures is completed. Uterine size and position should be carefully evaluated and superior displacement of the cervix performed to check for the presence of tenderness. • Prior to genitorectal examination, gloves should be changed to avoid possibility of iatrogenic rectal inoculation of pathogens. Genitorectal examination is done for evaluation of the rectum and cul-de-sac. • Anoscopic and sigmoidoscopic examination is suggested for females with proctocolitic symptomatology and those who have engaged in rectal intercourse.

Oropharyngeal Examination Routine examination of oropharynx is essential and may give useful clues to diagnosis or helps in detection of ulcerations or pharyngitis. Bedside Procedures 1. Two or three glass testis done to differentiate anterior urethritis from posterior urethritis or infection of the bladder. In the two glass test, patient is asked to void the urine. If the first glass is hazy and second glass clear, suggests anterior urethritis. In case, haziness is seen in second glass only, it means there is

posterior urethritis. If both the glasses contain hazy urine, it means there is cystitis. In three glass test, anterior urethra is irrigated till washings are clear (1:8000 oxycyanide of mercury solution) in the first glass. If second sample of urine obtained contains pus, it suggests posterior urethritis and if third glass has pus, means cystitis. 2. Saline preparation for clue cells, lactobacilli, leukocytes, Trichomonas vaginalis or Candida albicans 3. Wet mount for Trichomonas vaginalis 4. KOH examination on vaginal discharge or preputial sac scrapings 5. Whiff or Sniff test on vaginal discharge 6. Gram’s stain of urethral, vaginal, cervical or other discharge 7. pH estimation of vaginal discharge 8. Dark ground illumination test- for Treponema pallidum, Trichomonas vaginalis 9. Tissue smear for Donovan bodies in the mononuclear cells seen in donovanosis. 10. Tzanck smear for giant cells in herpes genitalis infection 11. Acetic acid test in case of genital warts 12. Scraping for mite if scabies is suspected 13. Microscopic examination for lice or nits 14. Bright field microscopic examination of unstained specimens is useful for examining uncentrifuged and centrifuged urine for leukocytes and bacteria 15. Specimens obtained may be sent for culture examination 16. Skin biopsy from skin lesions, genital sores etc for histopathology Based on clinical history and examination, a provisional diagnosis is made. All patients with history of premarital or extramarital sexual contact, and those with STDs are screened for syphilis and HIV infection. On the basis of clinical diagnosis, treatment is instituted. A proper history and examination should be followed by counselling which should include imparting knowledge about safe sex and creating awareness about STDs.


Guidelines for Evaluating STD’s 1. The presence of one STD signals the possibility of a second pathogen (overt, asymptomatic or in incubation phase). 2. All sexual contacts of an STD patient should be evaluated and treated. 3. Complications of STDs may be averted by early diagnosis, proper treatment, appropriate follow up, and detection of the asymptomatic cases. 4. Site of infection should be specified in the diagnosis.

5. Evaluation for pregnancy by history, physical examination and laboratory tests is essential 6. STDs must be considered in all cases of child abuse. 7. Geographic location and patient population determine the prevalence of a specific disease and affect the predictive values of laboratory tests. 8. A single, non-reactive non-treponemal serological test for syphilis (VDRL) does not exclude syphilis. 9. Appropriate patient follow up and documentation of cure are critical.

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31

Sexually Transmitted Diseases

Sexually transmitted diseases (STDs) are a group of communicable diseases that are transmitted predominantly by sexual contact and caused by

a wide range of bacterial, viral, protozoal, fungal pathogens and ectoparasites. Over 20 pathogens have been found to be spread by sexual contact.

A classification of these agents and the diseases caused by them are Pathogen

Disease or syndrome

Neisseria gonorrhoeae

Gonorrhoea, urethritis, cervicitis, epididymitis, salpingitis, PID, neonatal conjunctivitis

Treponema pallidum

Syphilis

Haemophilus ducreyi

Chancroid

Chlamydia trachomatis

LGV, urethritis, proctitis, epididymitis, infantile pneumonia, Reiter’s syndrome, PID, neonatal conjunctivitis

Calymmatobacterium granulomatis

Donovanosis

Hepatitis B

Acute and chronic hepatitis

Human papilloma viruses

Genital and oral warts

Herpes simplex virus

Herpes genitalis

Human immunodeficiency virus

AIDS

Molluscum contagiosum

Genital molluscum contagiosum

Candida albicans

Vaginitis

Trichomonas vaginalis

Vaginitis


GENITAL ULCER DISEASES Syphilis • Causative organism- Treponema pallidum, a spirochete, a motile, corkscrew-shaped, gramnegative, prokaryotic bacterium with a flexible, helically coiled cell wall. It belongs to the order Spirochaetales (“coiled hair”). • The majority of syphilis infections are acquired through direct sexual contact with an infected person in the early stages of the disease (acquired syphilis). • The secondary stage of syphilis is most contagious because of the greater amount and variety of lesions present. Acquired Syphilis Primary Syphilis • After an incubation period of 9-90 days, infection with treponema pallidum results in a painless chancre (primary chancre, “Hunterian chancre”, Hard chancre) at the site of inoculation. Five percent of chancres have extra-genital location. • Typical chancre of primary syphilis is a round or slightly elongated ulcer, 1-2 cm across with an indurated margin. The ulcer has a clear base, without an exudate being present (Fig. 31.1)

Fig. 31.1: Primary chancre—painless indurated clean looking single ulcer over the glans penis

• Modest enlargement of inguinal lymph nodes (nontender, nonsuppurative, firm, shotty, “indolent bubo”), frequently bilaterally is observed in the majority of patients who have genital lesions. • Without treatment, the chancre heals with scarring in 3 to 6 weeks. • Relapses of primary syphilis, termed monorecidive syphilis or chancre redux are rare.

Secondary Syphilis • Lesions of secondary syphilis result from the hematogenous dissemination of treponemes during the evolution of the primary syphilitic chancre and lesions appear 3-12 weeks after the initial appearance of primary lesion. • Patients with secondary syphilis may be ill with flu-like symptoms that include malaise, appetite loss, fever, headache, stiff neck, lacrimation, myalgias, arthralgias, nasal discharge, and depression. • The skin manifestations of secondary syphilis called syphilids are asymptomatic, polymorphous, variegate, bilateral and symmetrical (Figs 31.2 to 31.6). The initial finding in secondary syphilis is an evanescent macular rash, a few days later symmetric papular eruption appear involving the entire trunk and the extremities, including palms and soles. Papular lesions demonstrate Ollendorf sign. Even nodules may appear. They may be pustular, but never vesicular. Because of the variety of clinical manifestations, syphilis has been called the “the great imitator”. • Lesions on mucous membranes appear as raised gray white “mucous patches”. • Condylomata lata, the moist, smooth surfaced warty intertriginous plaques are considered to be most infectious lesion of syphilis because of the eroded surface and large number of spirochetes (Figs 31.7 and 31.8)

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Fig. 31.2: Secondary syphilis—papular and nodular syphilid over the face

Fig. 31.4: Secondary syphilis—papular and nodular syphilitic rash over the back

Fig. 31.3: Secondary syphilis—split papules at the angles of mouth

Fig. 31.5: Secondary syphilis—single noduloplaque over the back

• Another hallmark of secondary syphilis is generalized lymphadenopathy, which frequently affect the inguinal, the posterior cervical and the epitrochlear lymph nodes. Their characteristics are similar to that seen in primary syphilis. • “Moth eaten” alopecia may occur over the scalp.

• Rarely, central nervous system, eyes and other visceral organs are affected. • Untreated secondary syphilis spontaneously resolves after a period of 3 to 12 weeks leaving the patient in asymptomatic latent phase. • Individuals may remain asymptomatic for life, even though the T. pallidum organism continues to multiply or enter late symptomatic syphilis.


Fig. 31.6: Secondary syphilis—annular syphilid

Fig. 31.8: Secondary syphilis—condylomata lata lesions in the perianal area

Fig. 31.7: Secondary syphilis—condylomata lata at the root of penis and healed scar of primary chancre seen over the glans penis

Fig. 31.9: Tertiary syphilis—perforation of the palate

Tertiary Syphilis

• The fetus is at greatest risk when maternal syphilis is of less than 2 years duration. The ability of the mother to infect the fetus diminishes but never disappears in late latent stages (“Kassowitz’s law”). • Prenatal infection may also result in miscarriage or stillbirths. • Congenital syphilis is divided into early (of less than 2 years duration) and late (more than 2 years duration) congenital syphilis.

• Tertiary syphilis may be gummatous (nontender pink to dusky red nodules or plaques that vary in size from millimeters to many centimeters in diameter) (Fig. 31.9), cardiovascular or neurosyphilis.

Congenital Syphilis • In congenital syphilis, the treponemes cross the placenta and infect the fetus.

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Essentials in Dermatology • The early clinical signs of congenital syphilis begin to appear in the third to eighth week of life and in all cases by 3rd month of life. • Early congenital syphilis is known for snuffles (a persistent nasal dischargesyphilitic rhinitis), myriad of cutaneous lesions like secondary syphilis (scaling papules, plaques, bullae, desquamation, condylomata lata, mucous patches) (Figs 31.10 to 31.12), furuncle of Barlow, hepatosplenomegaly (Fig. 31.13), generalized lymphadenopathy, pseudoparalysis of Parrot (pain from osteochondritis of the long bones,

or epiphysitis, is exacerbated by movement, so the child keeps the affected limb still), osteochondritis with the “sawtooth” metaphysis seen on radiographs, syphilitic chorioretinitis (“salt and pepper” fundus) and late congenital syphilis for “Hot cross bun” deformity of skull, a saddle nose deformity, notched molars (Moon’s molars or Mulberry molars) and central incisors (Hutchinson’s teeth), Hutchinson’s triad (Hutchinson’s teeth, interstitial keratitis, eighth nerve deafness), rhagades, Clutton’s joints (painless swelling of joints- most commonly both knees), sabre tibiae, palatal perforation, neurosyphilis (tabes dorsalis, general paresis and local gummata) and paroxysmal cold hemoglobinuria.

Fig. 31.10: Congenital syphilis—papular lesions of early syphilis over the knee

Fig. 31.12: Congenital syphilis—perianal condylomata lata lesions in early syphilis

Fig. 31.11: Congenital syphilis—erythema and scaling of palms in early syphilis

Fig. 31.13: Congenital syphilis— hepatosplenomegaly in early syphilis


Stages of Acquired Syphilitic Infection Primary syphilis

10-90 days (average 21 days)

Chancre single or multiple, on skin or mucous membrane, regional lymphadenopathy

Secondary syphilis

6 weeks to 6 months

Multiple secondary lesions (skin or mucous membrane), lymphadenopathy, fever, condylomata lata, alopecia, asymptomatic CNS involvement

Latent syphilis

<1 year (early); >1 year (late)

Asymptomatic

Late syphilis

Months to years

Gummatous syphilis (monocytic infiltration, tissue destruction of any organ); Cardiovascular syphilis (Aortic aneurysms); Neurosyphilis (paresis, tabes dorsalis, meningovascular syphilis)

Diagnosis Acquired Syphilis • The most specific and sensitive method for verifying the diagnosis of primary syphilis is the finding of treponemes with characteristic appearance by darkfield microscopic examinations of fluid obtained from the surface of the chancre. The dark field examination is actually the only test that specifically establishes the diagnosis of primary syphilis. • The non-specific treponemal tests the Venereal Disease Research Laboratory (VDRL) and the rapid plasma reagin (RPR), typically become reactive within 4 to 7 days of chancre development. A titre of 1:8 or more is said to be significant. These titre results correspond with disease activity and should reduce four fold within 6 to 12 months of treatment and become undetectable several years there after. False positive results may occur with numerous conditions, such as collagen vascular diseases, several chronic infections such as HIV or tuberculosis, advancing age, narcotic drug use, chronic liver disease and several active infections such as herpes. • Because of the decreased specificity of above tests, positive results should be confirmed with the more precise treponemal tests, Treponema pallidum heme agglutination assay

(TPHA), microhaemagglutination assay for T.pallidum (MHA-TP) and fluorescent treponemal antibody absorptions test (FTAABS). In secondary syphilis all serologies should be reactive, whereas in late syphilis, non-treponemal tests may be negative or only weakly positive whereas treponemal tests are usually reactive. • CSF examination and chest X-ray should be done in tertiary syphilis. Sometimes, skin biopsy may be required to show characteristic histopathological changes (Endarteritis obliterans and predominantly perivascular infiltrate of lymphoid cells and plasma cells are basic pathologic changes of syphilis. Late in the course of syphilis, granulomatous changes also occur) and organisms in tissue can be demonstrated by silver staining.

Congenital Syphilis • A diagnosis of congenital syphilis can be made with confidence if the mother has reactive non-treponemal and treponemal serologies and the infant manifests classic signs of disease. • Congenital syphilis is also highly likely when the infant’s non-treponemal antibody titre is four fold or greater than the mother’s serum, even in the absence of physical findings. • IgM FTA –ABS test is specific for congenital syphilis, particularly if the titre rises.

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Treatment Acquired Syphilis Stage

Treatment

Follow up

Primary, secondary, or early latent syphilis

2.4 million IU intramuscular benzathine penicillin G once

Clinical and serologic exams at 3, 6, 9, 12, and 24 months

Late latent syphilis or syphilis of unknown duration

2.4 million IU intramuscular benzathine penicillin G weekly for 3 weeks

Clinical and serologic exams at 6, 12, 18, and 24 months

Neurosyphilis or ocular syphilis

3-4 million IU of intravenous aqueous crystalline penicillin G every 4 hours for 10-14 days or intramuscular procaine penicillin 2.4 million IU daily and oral probenecid 500 mg 4 times per day for 10-14 days

If CSF pleocytosis was initially present, CSF exams every 6 months for up to 2 years or until this parameter returns to normal.

If patient is allergic to penicillin in early syphilis, he/she may be treated with Doxycycline 100 mg bd PO × 15 days or Tetracycline HCl 500 mg qid PO × 15 days Alternative regimen for penicillin-allergic pregnant patients in early syphilis is Erythromycin, 500 mg orally, 4 times daily for 14 days. Alternative regimen for penicillin-allergic nonpregnant patients in late syphilis. Doxycycline, 100 mg orally, twice daily for 30 days. Or Tetracycline, 500 mg orally, 4 times daily for 30 days. Alternative regimen for penicillin-allergic pregnant patients in late syphilis. Erythromycin, 500 mg orally, 4 times daily for 30 days. Patients of early syphilis and late syphilis should be followed up clinically and serologically at 3 monthly intervals.

Congenital Syphilis

Early Congenital Syphilis Aqueous benzylpenicillin 100, 000–150, 000 IU/ kg/day administered as 50, 000 IU/kg/dose IV every 12 hours, during the first 7 days of life and every 8 hours thereafter for a total of 10 days. Or Procaine benzylpenicillin, 50, 000 IU/kg by intramuscular injection, as a single daily dose for 10 days. Note Some experts treat all infants with congenital syphilis as if the CSF findings were abnormal. Antimicrobials other than penicillin (e.g. erythromycin) are not recommended for congenital syphilis except in cases of allergy to penicillin. Tetracyclines should not be used in young children.

Late Congenital Syphilis Aqueous benzylpenicillin, 200, 000–300, 000 IU/ kg/day by intravenous or intramuscular injection, administered as 50, 000 IU/kg/dose every 4–6 hours for 10–14 days.

Sexually Transmitted Diseases Alternative Regimen for Penicillin-allergic Patients, after the First Month of Life Erythromycin, 7.5–12.5 mg/kg orally, 4 times daily for 30 days.

Chancroid (Soft sore, soft chancre, ulcus molle) • Three days to 2 weeks (1-5 days usually) after exposure to Haemophilus ducreyi (gram negative bacillus), a small inflammatory papule or pustule arises at the site of inoculation. • Within days, the lesion erodes to form an extremely painful, deep ulceration. • The characteristic ulcer is soft, friable and non-indurated, with ragged undermined margins, a foul smelling, yellow-grey exudate covering and surrounding erythema. • Within 1 to 2 weeks, painful inguinal lymphadenitis (“inflammatory bubo”), most often unilateral, develops in 30 to 60% of patients (about half the cases). Twenty five percent of patients have progression of the lymphadenitis into a suppurative bubo (unilocular abscess), which may spontaneously rupture and develop ulceration.

Diagnosis • H.ducreyi may be identified in the form of chains (“school of fish”) of gram-negative cocobacilli in the smear from the ulcer, or preferably of pus from bubo. • Ideally it should be cultured on one of the modern selective media at 33°C in an atmosphere of high humidity. • Ito-Reenstierna test- intradermal test with a vaccine containing killed H.ducreyi in suspension, producing an inflammatory papule (0.5 to 1 cm in diameter) after 48 hours • Because of the current difficulty with diagnosis, the CDC recommends that a probable diagnosis can be determined by the

presence of the following: one or more painful genital ulcers, a clinical presentation and associated lymphadenopathy, a negative laboratory evaluation for T.pallidum (serologic testing or dark field examination) and a negative testing for herpes simplex virus (HSV).

Treatment WHO 2003 and Center for Disease Control and Prevention (CDC) in 2006 recommends Azithromycin 1 g orally in a single dose, or Ceftriaxone 250 mg intramuscularly (IM) in a single dose, or Ciprofloxacin 500 mg orally twice a day for 3 days (contraindicated for pregnant and lactating women), or Erythromycin base 500 mg orally three times (CDC) or four times (WHO) a day for 7 days. NACO recommends erythromycin stearate / erythromycin base, 500 mg orally 4 times a day for 7 days or erythromycin ethyl succinate, 800 mg orally 4 times a day for 7 days (In case of concomitant syphilis, treatment can be given for 15 days) or ciprofloxacin, 500 mg orally twice a day for 3-5 days or till the clearance of lesions or ceftriaxone, 250 mg IM as a single dose or azithromycin, 1 g orally as a single dose or doxycycline, 100 mg orally twice daily for 7 days or trimethoprim (80 mg) + sulphamethoxazole (400 mg), 2 tabs orally twice a day for 2 weeks. Treatment should be given for the period indicated, or until such time, the lesions heal. Fluctuant bubo should be aspirated through the surrounding healthy skin. Aspiration should not be done from the dependent side. Incision and drainage or excision of bubo delays healing and is contraindicated.

Lymphogranuloma Venereum (LGV) • Causative organism-Chlamydia trachomatis immunotypes L1, L2, and L3. • The course of disease in LGV consists of 3 separate stages.

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Essentials in Dermatology • After an incubation period of 3-30 days after inoculation, the first stage begins with a small painless papule or pustule that may erode to form an asymptomatic herpetiform ulcer. This typically heals without scarring in 1 week and often goes unnoticed. • The second stage begins within 2-6 weeks after the onset of primary lesion. It is referred to as inguinal stage and consists of painful inflammation and infection of inguinal and/ or femoral lymph nodes. Involvement is limited to one groin in about two third of the cases. Enlargement of tender lymph nodes above and below the inguinal ligament may give the bubo a grooved appearance the “sign of the groove” (Fig. 31.14). Suppuration occurs in them with the formation of multiple small abscesses. These abscesses open on the skin surface to form multiple sinuses, finally healing with puckered scars. Constitutional symptoms are very variable. • The third stage is called as genitorectal syndrome. The interval between the early stage and later manifestations may vary from a year or two to many years. Patients may develop genital elephantiasis (Esthiomene) or anorectal syndrome. It more often develops in women and homosexual men who engage in receptive anal intercourse and includes proctocolitis, peri-rectal abscess, fistula.

Diagnosis • A definitive diagnosis can be achieved with isolation of the organism on culture and cell typing of the isolates. • The Frei test-an intradermal test with 0.1ml of Lygranum antigen, read at 48 to 72 hours- a raised, red papule, at least 6 mm across. • With appropriate clinical presentation, a complement fixation antibody titre of greater than 1:64 is considered diagnostic of LGV.

Treatment Center for Disease Control and Prevention (CDC) in 2006 recommends. • Doxycycline 100 mg orally twice a day for 21 days • Alternative treatment is erythromycin base 500 mg orally four times a day for 21 days. • Some STD specialists believe that azithromycin 1.0 g orally once weekly for 3 weeks is probably effective, although clinical data are lacking. WHO (2003) Recommends Doxycycline, 100 mg orally, twice daily for 14 days. Or Erythromycin, 500 mg orally, 4 times daily for 14 days. Alternative regimen Tetracycline, 500 mg orally, 4 times daily for 14 days.

Fig. 31.14: Lymphogranuloma venereum—“sign of the groove” due to enlargement of lymph nodes above and below inguinal ligament

Note Tetracyclines are contraindicated in pregnancy. NACO recommendations are same as WHO except for an additional therapy- trimethoprim (80 mg) + sulphamethoxazole (400 mg) 2 tablets twice daily for 21 days. Fluctuant lymph nodes should be aspirated through healthy skin. Incision and drainage or excision of nodes may delay healing. Some patients with advanced disease may require

Sexually Transmitted Diseases treatment for longer than 14 days, and sequelae such as strictures and/or fistulae may require surgery.

superficially in the area of the inguinal nodes called pseudobuboes (not lymphadenitis).

Diagnosis

Granuloma inguinale (Donovanosis) • Causative organism- Klebsiella granulomatis (formerly known as Calymmatobacterium granulomatis), related to the Klebsiella species, gram negative, obligate intracellular bacillus. • After an incubation period of 8 days to 12 weeks (an average of 17 days), single or multiple subcutaneous nodules or papules develop at the site of inoculation (genitalia, thigh, groin, or in the perineum). These lesions enlarge and erode to form painless, soft, beefy red, exuberant ulcerations with clean friable bases and distinct raised rolled margins (Fig. 31.15) • The clinical presentation can also include hypertrophic, necrotic, or sclerotic variants. • Inguinal enlargement may occur because of subcutaneous granulomas that arise

• The most reliable method of diagnosis involves direct visualization of the bipolar staining intracytoplasmic inclusion bodies. These “safety pin-shaped bodies” also called as “Donovan bodies” (Fig. 31.16) can be seen within histiocytes (vacuolated monocytes) of granulation tissue smears or biopsy specimens. Wright’s or Giemsa stains are both satisfactorily used. • Biopsy and histopathological examination

Fig. 31.15: Donovanosis—painless, soft, beefy red, exuberant ulcerations with raised rolled margins over the groin (pseudobubo) and genitalia

Fig. 31.16: Donovanosis—large mononuclear cell demonstrating intracytoplasmic “Donovan bodies” in tissue smear

Treatment Center for Disease Control and Prevention (CDC) in 2006 recommends Doxycycline 100 mg orally twice a day for at least 3 weeks, Alternative regimens Azithromycin 1 g orally once per week for at least 3 weeks and until all lesions have completely healed Or Ciprofloxacin 750 mg orally twice a day for at least 3 weeks and until all lesions have completely healed Or

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Essentials in Dermatology Erythromycin base 500 mg orally four times a day for at least 3 weeks and until all lesions have completely healed Or Trimethoprim-sulfamethoxazole one doublestrength (800mg/160mg) tablet orally twice a day for at least 3 weeks and until all lesions have completely healed WHO (2003) Recommends Azithromycin, 1 g orally on first day, then 500 mg orally, once a day Or Doxycycline, 100 mg orally, twice daily Alternative regimen Erythromycin, 500 mg orally, 4 times daily Or Tetracycline, 500 mg orally, 4 times daily Or Trimethoprim 80 mg/sulfamethoxazole 400 mg, 2 tablets orally, twice daily for a minimum of 14 days Treatment should be continued until all lesions have completely epithelialized. Note: The addition of a parenteral aminoglycoside such as gentamicin should be carefully considered for treating HIV-infected patients. NACO recommendations are same as WHO one’s except azithromycin Other option used-Injection of streptomycin 1 gram given daily for 14 days.

Herpes Genitalis • Both HSV-1 and HSV-2 produce primary as well as recurrent genital infections. Isolation of HSV-2 can be successful in 80% of instances. • HSV is the most common cause of genital ulceration and accounts for 20% to 50% of ulcerative lesions in patients attending sexually transmitted disease clinics (STD clinics).

• The incubation period for both HSV-1 and HSV-2 is between 3 to 14 days. • The outbreak begins with small grouped vesicles, which break and progress to ulcerative lesions in 2 to 4 days. • The first episode of genital herpes (primary or non-primary) usually has multiple lesions, which present bilaterally and coalesce to involve a larger surface. Painful inguinal lymphadenopathy is common. The dominant local symptoms of primary genital herpes are pain, itching, dysuria, and vaginal and urethral discharge. The ulcers of herpes genitalis are superficial and tiny. The severity of these symptoms increases for the first 6-7 days of the illness and peaks during the first week of illness in about 75% of patients. The course of primary genital herpes may last for 18-21 days and the virus shedding is present for about 11 days following the primary infection. • About 50% of males will have a recurrence in 4 months but the severity of symptoms, and duration of viral shedding are much shorter in recurrent episodes than in primary disease (Figs 31.17 and 31.18).

Diagnosis • Definitive diagnosis of genital herpes is made by viral culture of the lesions, which can distinguish between HSV-1 and HSV-2. • Biopsy and cytologic studies may be useful in the diagnosis, but they are unable to differentiate between HSV-1, HSV-2 and varicella zoster virus. • The use of specific monoclonal antibodies directed against HSV-1 and HSV-2 proteins have proved to be sensitive and specific. • PCR can be used to detect HSV DNA. • And a valuable bedside clinical approach in making a rapid diagnosis of herpes infection relies on Tzanck preparation, which can be of great value in resource poor setting.


Fig. 31.17

Fig. 31.18

Figs 31.17 and 31.18: Herpes genitalis—grouped vesicles and multiple, superficial erosions involving genitalia

Treatment WHO (2003) and Center for Disease Control and Prevention (CDC) in 2006 recommends First clinical episode of genital herpes Acyclovir 400 mg orally three times a day for 7–10 days, or Acyclovir 200 mg orally five times a day for 7–10 days, or Famciclovir 250 mg orally three times a day for 7–10 days, or Valacyclovir 1 g orally twice a day for 7–10 days. Recurrent episodes of genital herpes Acyclovir 200 mg orally, 5 times daily for 5 days (WHO) or 400 mg orally three times a day for 5 days, or Acyclovir 800 mg orally three times a day for 2 days (CDC), or Acyclovir 800 mg orally twice a day for 5 days, or Famciclovir 125 mg orally twice a day for 5 days, or Valacyclovir 500 mg orally twice a day for 3–5 days, or Valacyclovir 1.0 g orally once a day for 5 days. Suppressive therapy for recurrent genital herpes Daily suppressive anti-viral therapy may be employed in patients with frequent recurrences of genital herpes (six or more recurrences per year). Since daily antiviral suppressive therapy reduces the recurrence rate of herpes genitalis by more than 75%, option for daily suppressive therapy may be discussed with all such patients suffering from recurrent herpes genitalis.

Acyclovir 400 mg orally twice a day, or Famciclovir 250 mg orally twice a day, or Valacyclovir 500 mg orally once a day, or Valacyclovir 1.0 gram orally once a day. Periodically, once a year discontinuation of suppressive therapy should be discussed. NACO recommends only the use of acyclovir as given above. Sexual contact should be avoided as long as there are active lesions. Cervical cytology should be routinely done in females with herpes genital infection. Counseling of infected persons and their sex partners is critical to the management of genital herpes.

Differential Diagnosis of Genital Ulcer Disease Genital ulcers are defined as breach in the continuity of genital mucosa and /or skin. Genital ulcer disease (GUD) may be due to sexually transmitted diseases (STD) like syphilis, herpes genitalis, chancroid, lymphogranuloma venereum, donovanosis or non-STD like traumatic ulcers, Behcet’s disease, lichen planus, erythema multiforme, lichen sclerosus et atrophicus, bullous diseases, Fournier’s gangrene, squamous cell carcinoma (Fig. 31.19), etc.

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Fig. 31.19: Squamous cell carcinoma presenting as ulcerative destructive growth over the tip of penis

Differentiating features of the common sexually transmitted diseases are given below Characteristics

Syphilis

Herpes genitalis

Chancroid

LGV

Donovanosis

1. Incubation period

9-90 days (2-4 weeks) mean 21 days

2-7 days

1-5 days

3 days to 6 weeks (avg. 7 days)

1 to 4 weeks (8 to 80 days)

2. Causative organism

Treponema pallidum

Herpes simplex virus (HSV 2 and 1)

Haemophilus ducreyi

Chlamydia trachomatis sero-type L1-L3.

Calymmatobacterium granulomatis

3. Primary lesion

Papule

Vesicle

Pustule

Papule, pustule or vesicle

Papule

4. Number of lesions

Usually one

Multiple, may coalesce

Usually multiple

Usually one

Variable

5. Pain

Painless

Painful

Usually very tender

Variable

Uncommon

6. Diameter

5 to 15 mm

1 to 2 mm

2 to 20 mm

2 to 10 mm

variable

7. Edges/ margin

Sharply demarcated, elevated, round/oval

Erythematous, sharp

Sharply circumscribed, ragged undermined edges

Elevated, round or oval

Elevated, irregular

8. Depth

Superficial/deep Superficial

Excavated and deep

Superficial

Elevated

9. Floor

Smooth, clean

Variable

Beefy red granulation tissue, bleeds readily

Erythematous, Yellow covered with necrotic purulent serous exudates exudates

Contd...

Sexually Transmitted Diseases Contd... Characteristics

Syphilis

Herpes genitalis

Chancroid

LGV

Donovanosis

10. Base

Indurated

None

Nonindurated, soft

Occasionally firm

Firm

11. Inguinal Bilateral, firm, Bilateral firm lymphadeno- non-tender, and tender pathy discrete, shotty

30 to 60%, unilateral, tender, matted together, suppuration with formation of a unilocular abscess (bubo), may rupture

66% usually None, unilateral, less pseudobubo painful, Groove sign may be seen. positive, multilocular abcesses, rupture to form multiple sinuses

12. Spontaneous 3 to 8 weeks, healing time never exceeds 3 months

14 to 21 days

Self limiting but may be persistent.

2 to 5 days (transient)

No tendency to heal

13. Recurrence

Yes, in 80%

No

No

No

No.

URETHRITIS, CERVICITIS AND/OR VAGINITIS Gonorrhea • The term, gonorrhea is derived from Greek “flow of seed”. • Causative organism- Neisseria gonorrhoeae. The responsible organism, Neisseria gonorrhoeae was demonstrated by Neisser in 1879 in stained smears of urethral, vaginal and conjunctival exudates. It is a gramnegative coccus that is found in pairs (diplococci) within polymorphonuclear leukocytes in purulent material. • It infects noncornified epithelium most often of the urogenital tract and secondarily of the rectum, oropharynx and conjunctivae. • The incubation period ranges from 1-14 days or even longer (on an average 2-5 days). • It is transmitted sexually or perinatally. • Acute anterior urethritis is the most common manifestation of gonococcal infection in men (Fig. 31.20) and as any asymptomatic or minimally symptomatic endocervical colonization in women. • Predominant symptoms are urethral discharge or dysuria. Discharge initially is scant and mucoid or mucopurulent. In most

males, the urethral exudate becomes frankly purulent and relatively profuse within 24 hours of onset. On examination, mucoid or mucopurulent discharge is seen from the urethral meatus and the meatus is erythematous and edematous. Epididymitis, seminal vesiculitis and prostatitis may occur but usually much later.

Fig. 31.20: Gonorrhea- mucopurulent discharge per urethra

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Essentials in Dermatology • In females, if manifest, it leads to increased vaginal discharge, dysuria, intermenstrual bleeding or menorrhagia. Examination reveals mucopurulent cervical discharge, the cervix has erythema and oedema, swabbing the endocervix easily induces mucosal bleeding. Salpingitis and Bartholin gland abscesses are possible local complications. Ascending infection and bacteremic dissemination (disseminated gonococcal infection) are responsible for most of the serious morbidity. • Rectal mucosa is a frequent site of infection in homosexual men with symptoms ranging from minimal anal pruritus, painless mucopurulent discharge or scant rectal bleeding to overt proctitis. • Pharyngeal infection occurs in 3-7 percent of heterosexual men, 10 to 20 percent of heterosexual women, and 10 to 25 percent of homosexually active men. • Ophthalmia neonatorum may occur due to perinatal transmission.

Diagnosis • Isolation of N.gonorrhoeae is the diagnostic standard for gonococcal infections. • Urethral smear is sufficiently sensitive and specific, that the culture may be considered optional for routine care. Gram’s stain has been the most extensively studied. A diagnosis of urethritis is made if there are more than 5 pus cells present per oil immersion field. In addition, these pus cells have intracellular gram-negative diplococci, confirming the diagnosis of gonorrhea (Fig. 31.21).

or Ciprofloxacin 500 mg orally in a single dose, or Ofloxacin 400 mg orally in a single dose, or Levofloxacin 250 mg orally in a single dose, plus IF CHLAMYDIAL INFECTION IS NOT RULED OUT Azithromycin 1 g orally in a single dose or Doxycycline 100 mg orally twice a day for 7 days.

Alternative Regimens Spectinomycin 2 g in a single IM dose or Singledose cephalosporin regimens- ceftizoxime (500 mg, administered IM), cefoxitin (2 g, administered IM with probenecid 1 g orally), and cefotaxime (500 mg, administered IM) or Single-dose quinolone regimens - gatifloxacin 400 mg orally, norfloxacin 800 mg orally, and lomefloxacin 400 mg orally.

Other Treatment Options Used in the Past 1. Aqueous procaine penicillin 4.8 million units IM single dose with 1 gm probenecid half an hour before the injection penicillin. 2. Spectinomycin 2 gm IM single dose. 3. Norfloxacin 800 mg single dose. 4. Ampicillin 3 gm or amoxicillin 3.5 gm with 1 gm probenecid.

Non-gonococcal Urethritis (NGU) • NGU is diagnosed if gram-negative intracellular organisms cannot be identified in the discharge on gram stain in a patient

Treatment For uncomplicated gonococcal infections of the cervix, urethra, and rectum, Center for Disease Control and Prevention (CDC) in 2006 recommends Ceftriaxone 125 mg IM (intramuscular) in a single dose, or Cefixime 400 mg orally in a single dose,

Fig. 31.21: Gram’s stained urethral discharge showing pus cell (polymorphonuclear cell) containing intracellular gram-negative diplococci

Sexually Transmitted Diseases with urethral discharge and burning micturition. • Chlamydia trachomatis is responsible for 30 to 50%, Ureaplasma urealyticum for 10 to 40% and the rest are due to Trichomonas vaginalis, yeasts, Herpes simplex virus, Adenovirus, Hemophilus spp., Bacteroides etc. • The incubation period is 1 to 5 weeks. • In males, urethritis begins with dysuria and mucoid urethral discharge. In contrast to gonococcal urethritis, symptoms are usually mild. Most cases in females are asymptomatic. Diagnosis 1. Gram’s stained urethral discharge shows more than 5 pus cells per oil immersion field but no gram-negative intracellular diplococci are seen. 2. Culture: Urethral swabs are sent for N.gonorrhoeae, mycoplasma, and anaerobes culture. Chlamydial antigen detection may be done on swab. Differential diagnosis: Clinical features of gonococcal and non-gonococcal urethritis are tabulated below Characteristics Gonorrhoea urethritis

Non-gonococcal

1. Incubation period

2-3 weeks

2-5 days

2. Constitutional Fever, malaise symptoms

Absent

3. Discharge

Profuse, purulent and yellowish

Scanty mucoid or mucopurulent

4. Dysuria

Intense burning sensation

Smarting feeling in urethra on passing urine

Treatment Center for Disease Control and Prevention (CDC) in 2006 recommends – Azithromycin 1 gm PO once or – Doxycycline 100 mg PO BD for 7 days

Alternative regimens: – Erythromycin base 500 mg PO qid for 7 days or – Ofloxacin 300mg PO BD for 7 days or Levofloxacin 500 mg orally for 7 days.

Pelvic Inflammatory Disease (PID) PID comprises of a spectrum of inflammatory disorders of the female upper genital tract, including any combination of endometritis, salpingitis, tubo-ovarian abscess and pelvic peritonitis. It may be of the chronic type (caused by M. tuberculosis) or acute PID (caused by sexual pathogens). This section will be dealing with acute PID. Etiology • Cervical pathogens: N.gonorrhoeae, Chlamydia trachomatis. • Vaginal microorganisms: Anaerobic bacteria (e.g. Prevotella, Peptostreptococcus), H. influenzae, Gardnerella vaginalis, Mycoplasma hominis, and Ureaplasma urealyticum. Symptoms and signs Wide variations in symptoms in women depending on the pathogen and the site of pathology. • Malodorous, yellowish vaginal discharge • Midline abdominal pain, abnormal vaginal bleeding (due to endometritis) • Dyspareunia • Lower abdominal and pelvic pain (salpingitis) • Fever, nausea vomiting, abdominal tenderness and rigidity (due to peritonitis). (Fitz-Hugh-Curtis syndrome refers to perihepatitis with acute PID caused by gonococcus or chlamydia. Patient presents with pleuritic upper abdominal pain and violin-string adhesions over the liver seen on laparoscopy.)

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Diagnostic Criteria

Minimum Criteria • Uterine/adnexal tenderness • Cervical motion tenderness Additional Criteria • • • • •

Oral temperature >101o F Abnormal cervical or vaginal discharge WBCs on saline mount of vaginal discharge Elevated ESR and C-reactive protein Laboratory documentation of cervical infection with N.gonorrheae or C. trachomatis.

Specific Criteria (required only in Certain Cases) • Endometrial biopsy showing endometritis • Transvaginal sonography or MRI scan showing tubal abnormalities or tubo-ovarian abscess. • Laparoscopic findings of PID.

Treatment • Treatment should be initiated with minimal criteria and needs to cover all the etiological pathogens. Early treatment prevents sequelae. • Parenteral therapy is given for the initial 24 – 48 hours and thereafter oral therapy is continued.

Bacterial vaginosis (Leukorrhea; Haemophilus vaginalis vaginitis; Gardnerella vaginalis vaginitis; Anaerobic vaginosis; Vaginal bacteriosis) • Bacterial vaginosis (BV) is a common cause of abnormal vaginal discharge in women of reproductive age. • It represents a complex change in vaginal flora characterized by a reduction in the prevalence and concentration of hydrogen peroxide producing lactobacilli and increase in the prevalence and concentration of Gardnerella vaginalis; Mobiluncus species; Mycoplasma hominis; anaerobic gram-negative rods belonging to the genera Prevotella, Porphyromonas, and Bacteroides; and Peptostreptococcus species. • Amsel et al has proposed a set of practical diagnostic criteria for the clinical diagnosis of BV that is now often accepted as the “gold standard.” Diagnosis requires three or more of the following clinical/diagnostic features: 1. The presence of (excessive) homogeneous vaginal discharge 2. Elevated vaginal pH >4.5 3. Positive amine test (Whiff test) 4. 20% “clue cells” (vaginal epithelial cells adhered to by infective microbes) and typical Gram stain appearance upon microscopy of vaginal secretions including absence of lactobacilli (Fig. 31.22).

Regimens include – IV Cefoxitin plus oral doxycycline (after 24 hours IV drugs stopped and oral doxycycline continued) or IV clindamycin and gentamicin (24 hours) followed by oral doxycycline or IV ofloxacin with metronidazole or IV ampicillin plus doxycycline • Male sexual contacts (in the preceding 60 days) should be treated.

Fig. 31.22: Gram’s stained vaginal discharge smear showing a normal vaginal epithelial cell and another coated with bacteria


Treatment • All women who have symptomatic BV disease require treatment. • Metronidazole 500 mg orally twice a day for 7 days, or Metronidazole gel 0.75%, one full applicator (5 g) intravaginally, once a day for 5 days, or Clindamycin cream 2%, one full applicator (5 g) intravaginally at bedtime for 7 days are recommended treatments. • Alternatively, patient may be treated with Metronidazole 2 g orally in a single dose, Clindamycin 300 mg orally twice a day for 7 days, or Clindamycin ovules 100 g intravaginally once at bedtime for 3 days. Differential diagnoses of vaginal discharge Profile

Normal vaginal discharge

Candidal vulvovaginitis

Trichomonal vaginitis

Bacterial vaginosis

Etiology

Lactobacillus predominant

Candida spp. and other yeasts

Trichomonas vaginalis

Associated with G.vaginalis; anaerobic bacteria, and Mycoplasma hominis

Symptoms

None

Vulvar pruritus and/ or irritation; external dysuria, increased vaginal discharge

Profuse discharge, often malodorous; external dysuria and genital irritation often present

Malodorous, increased discharge (commonly present at the introitus)

Amount

Variable; usually Scant to moderate scant Clear or white White

Profuse

Moderate to profuse

White, yellow or green

White or gray

Nonhomogenous, Clumped, “Cheesy” flocculant adherent exudative plaques

Homogeneous, watery, often frothy

Homogeneous, uniformly coating vaginal walls

Color Consistency

pH

Usually <4.5

<4.5

Usually >5.0

Usually >4.5

Amine odor with 10% KOH

None

None

Usually present

Present

Associated inflammatory signs

None

Erythema, edema and / or erosions of vagina or external genitalia; vulvar dermatitis common

Erythema of vaginal mucosa, introitus; occasional cervical petechiae; vulvar dermatitis

None

Microscopy of discharge

Normal epithelial cells; lactobacilli predominate

Leukocytes, epithelial cells; yeast, mycelia, pseudomycelia seen

Leukocytes; motile trichomonads seen

Clue cells; rare leukocytes; lactobacilli outnumbered by mixed flora

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MISCELLANEOUS STDs Anogenital Warts • The etiologic agent of anogenital warts or condyloma acuminata is the human papilloma virus (HPV) a non-enveloped, double standard DNA virus. • More than 80 different genotypes of HPV have been identified. Forty-five of these are known to affect the genital epithelium and are considered to be sexually transmitted infections. • It is roughly estimated that up to 30% to 50% of sexually active adults are infected with HPV. The highest rates of infection are found in women, 19-22 years of age and in men, 2226 years of age. • HPV 6 and 11 are most commonly associated with genital warts, but at least 20 different HPV genotypes have been associated with condyloma acuminata. • The infectivity of HPV between sexual partners is estimated to be 60%. • The most common locations of primary infection include the fourchette in women and

Fig. 31.23

•

•

•

•

•

the subpreputial region in men. Men have been found to be at increased risk if they fail to wear a condom and if they are smokers. Most genital warts occur on the penis, scrotum, urethral meatus, and perianal area in men. The clinical types of anogenital warts are Condyloma acuminata (acuminate warts) (Figs 31.23 and 31.24), papular warts, verruca vulgaris type or keratotic warts, sessile warts, flat warts, intraepithelial neoplasiaBowenoid papulosis and Bowen’s Disease, and “Giant condyloma” (Buschke – Lowenstein tumor) Subclinical HPV infection is one that is not clinically visible with the unaided eye, requiring colposcopy with or without acetowhitening or histopathology. Latent HPV infection means the presence of HPV can be confirmed only by the detection of the HPV DNA Atypical lesions occur in immunocompromized patients such as HIV infected individuals

Fig. 31.24

Figs 31.23 and 31.24: Condyloma acuminata—genital outgrowths

Sexually Transmitted Diseases Diagnosis • Diagnosis is typically made on clinical basis. • The most sensitive method for detection of HPV DNA is polymerase chain reaction. • Acetowhitening is a diagnostic tool. • But the most commonly utilized and widely available method is histopathologic examination of a tissue biopsy.

Differential Diagnosis Morphologically, various conditions, which need to be differentiated from verrucous lesions of genital warts, are condyloma lata of syphilis, nonvenereal treponematosis, hypertrophic verrucous type of granuloma inguinale, tuberculosis verrucosa cutis, skin tags, and malignancy. For small genital warts, various differential diagnoses need to be considered are pearly penile papules (Fig. 31.25), vestibular papillomatosis, molluscum contagiosum, seborrhoeic keratosis, Fordyce’s spots, urethral caruncle, lichen planus, and foreign body granuloma. For subclinical infection (seldom appears reddish), candidial infection of the vulva and repeated topical application of antifungals need to be ruled out. Other benign tumors like neurofibroma, lipoma, fibroepitheliomata, and normal physiological glands like Tyson’s glands also need to be considered before appropriate diagnosis is made. Around the anus, prolapse and sentinel piles, and anal tags

Fig. 31.25: Pearly penile papules often misdiagnosed as genital warts

may be confused with anogenital warts. In unhygienic persons, dry smegma may appear like warts and hence cleaning the sub-preputial region is emphasized before the examination of warts in male genitalia. For the pathological specimen of genital warts, various differential diagnoses considered are seborrheic keratosis, molluscum contagiosum, psoriasis, lichen planus, condylomata lata, Bowen’s disease and squamous cell carcinoma.

Treatment Treatment options may be categorized into cytodestructive methods (surgical excision, cryotherapy, laser therapy, bichloroacetic acid / trichloroacetic acid, podophyllin and podophyllotoxin), antimetabolic therapy (5-fluorouracil), antiviral therapy (cidofovir and interferons [IFNs]) and immunomodulation (imiquimod) 1. Podophyllin 10-25% (applied to the warts using cotton tipped swab once or twice a week for up to six weeks) or podophyllotoxin 0.5% (the solution or cream is applied with cotton swab or finger respectively, over the condylomas also on normal appearing skin between the lesions twice daily for three days followed by four days of no therapy) for local use is first line therapy. 2. Imiquimod cream, supplied in single use sachets, is applied to the warts with fingers three times per week (every other night) and the area washed with mild soap and water the next morning. Treatment continued until wart clearance, or for a maximum of 16 weeks 3. Cryotherapy – treatment of choice for condyloma acuminata in pregnancy. 4. CO2 laser 5. Surgery 6. Interferons (as adjuvant to surgery), 5-fluorouracil (urethral warts), cidofovir, or retinoids are other treatment options 7. Since treatment is often unsatisfactory and is not directed at elimination of the virus, some

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Essentials in Dermatology progress has been made in the development of the HPV vaccines 8. Patient counseling- Advise female patients about regular participation in cervical cytology screening programmes. Use barrier protection with new sexual contacts until successful treatment has been completed

Molluscum Contagiosum • Molluscum contagiosum is a benign papular condition of the skin, which is often sexually transmitted in adults. • Molluscum contagiosum virus is the final remaining member of poxviridae family that specifically infects humans. • The incidence of molluscum contagiosum in sexually active adults has been increasing significantly throughout the past few decades. • Mechanical trauma appears to facilitate the transmission of molluscum contagiosum virus. There is no information about the efficiency of sexual transmission of MCV. • Lesions begin as tiny papules, which grow over several weeks to a diameter of 3 to 5 mm. The flesh coloured papules are smooth, firm and dome shaped with a highly characteristic central umbilication. In adults lesions most commonly occur on the thighs, inguinal region, buttocks and lower abdominal wall (Fig. 31.26). Scabies • Scabies can be sexually transmitted. • When scabies spreads through sexual transmission, the characteristic lesions are more likely to occur on the genitalia, lower abdomen or thigh. • The penis and bra lines are other favourite locations for mite. Pediculosis Pubis • Pediculosis pubis is primarily a sexually transmitted infection occurring predo-

Fig. 31.26: Molluscum contagiosum with herpes genitalis

minantly in the young sexually active population. • Phthirus pubis is the causative organism. • Main symptom is itching which leads to scratching, erythema, irritation and inflammation.

Chronic Viral Hepatitis Sexual activity may profoundly influence the risk for acquisition of both hepatitis A virus (HAV) and hepatitis B virus (HBV) infection. To some extent sexual behavior may also determine the risk of infection with hepatitis C virus (HCV) and HDV. Prevention of STDs • The most reliable way to avoid transmission of STDs is to abstain from sexual intercourse (i.e., oral, vaginal, or anal sex) or to be in a long-term, mutually monogamous relationship with an uninfected partner. • Both partners should get tested for STDs, including HIV, before initiating sexual intercourse. • If a person chooses to have sexual intercourse with a partner whose infection status is unknown or who is infected with HIV or another STD, a new condom should be used for each act of insertive intercourse.

Human Immunodeficiency Virus Infection (HIV) and AIDS

32

Human Immunodeficiency Virus Infection (HIV) and Acquired Immunodeficiency Syndrome (AIDS)

HISTORICAL BACKGROUND • AIDS was first recognized and described as a clinical entity in mid 1981 when FriedmanKien and Gottlieb et al described groups of gay (homosexual) men in the USA with Kaposi’s sarcoma and pneumocystis carinii pneumonia (PCP), both previously very rare diseases and known to be linked to immunosuppression. • In June 1983, Barré Sinoussi and Montagnier of the Pasteur Institute in Paris reported the discovery of a new virus isolated from a lymph node of a gay (homosexual) man with persistent lymphadenopathy. They called this new virus lymphadenopathy associated virus (LAV) and postulated that this was the cause of AIDS. • In 1984, Gallo reported the isolation of a new retrovirus that he called human T-cell lymphotropic virus III (HTLV III). Serum antibody testing for this virus was found to be positive in almost 100% of AIDS patients. • In 1987, an international nomenclature committee decided that the new virus should be termed human immunodeficiency virus or HIV rather than HTLV III or LAV. • In 1987, Clavel et al isolated a second retrovirus from AIDS patients in West Africa,

which he termed it as HIV-2. HIV-2 causes AIDS in an identical way to HIV-1 although with a longer disease free period. • The first reported cases of HIV infections diagnosed in India were among Madras commercial sex workers (CSWs) in May 1986. • The epidemic of HIV in India is similar in many ways to the experience in sub-Saharan Africa and Thailand. • The estimated number of HIV infected adult cases (15-49 years) in the country as on 2004 was 5.134 million (based on HIV sentinel surveillance data of National AIDS Control Organization). Causative Organism • Human immunodeficiency virus (HIV) is an RNA retrovirus. It is called retrovirus as it utilizes a DNA polymerase enzyme reverse transcriptase to convert viral RNA to DNA and finally DNA is transcribed to RNA. • It mainly targets CD4+T lymphocytes and thus causes a profound defect in cell mediated immunity leading to opportunistic infections and neoplastic processes.

MODE OF TRANSMISSION • HIV infection is primarily transmitted through sexual route and less commonly through blood transfusion, maternofetal

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310

Essentials in Dermatology transmission, intravenous drug abuse, needle stick injuries, etc. • In other words, HIV is transmitted through semen and vaginal fluids, infected blood and blood products, infected mother to her babybefore birth, during birth or through breast milk.

SPECTRUM OF HIV INFECTION • Infection with HIV produces a spectrum of disease that progresses from a clinically latent or asymptomatic state to AIDS as a late

manifestation. The pace of disease progression varies. • In untreated patients, the time between infection with HIV and the development of AIDS ranges from a few months to as long as 17 years (median: 10 years). • Most adults and adolescents infected with HIV remain symptom-free for extended periods, but viral replication is active during all stages of infection, increasing substantially as the immune system deteriorates. • In the absence of treatment, AIDS eventually develops in almost all HIV-infected persons.

Clinical Stages of HIV Infection Stage of HIV infection

Clinical features

CD4 count (cells/uL)

Duration (days)

1. Primary HIV infection

Acute seroconversion illness: fever, pharyngitis, fatigue, rash, mucocutaneous ulcers, lymphadenopathy (occipital, axillary, cervical), headache, meningitis, encephalitis, neuropathies

150-800

3-14 days

2. Asymptomatic

Lymphadenopathy, headache

>300

2-10 years or more

3. Early symptomatic

Recurrent varicella zoster, oral candidiasis, oral hairy leukoplakia, seborrheic dermatitis, psoriasis, skin and nail infections (impetigo, folliculitis, fungal intertrigo, paronychia), bacterial infections (pneumonia, bronchitis, sinusitis), unexplained fatigue/fever /sweats/ weight loss, diarrhea, vaginal candidiasis, cervical dysplasia, cervical carcinoma in situ, recurrent pelvic inflammatory disease, tuberculosis

150-500

1-5 years

4. Late symptomatic and advanced

Kaposi’s sarcoma, lymphoma, PCP pneumonia,toxoplasmosis, esophageal candidiasis, cryptococcosis, cryptosporidiosis, recurrent HSV infection, HIV-associated dementia, progressive multifocal leukoencephalopathy, tuberculosis, CMV retinitis, primary CNS lymphoma, Mycobacterium avium intracellulare (MAC) infection

Late symptomatic: 50-200 Advanced: <50

Late symptomatic: 1-4 years Advanced: <2 years

Human Immunodeficiency Virus Infection (HIV) and AIDS Because of the long asymptomatic phase of most retroviral infections, a positive screening test is often the first indication of a possible infection. With the use of supplemented confirmatory tests, the laboratory diagnosis of HIV infection can be accurate, timely and sensitive. There are several testing methodologies now available for detection of retrovirus infection.

Revised CDC Classification for HIV Infection and Expanded Case Definition for AIDS in Adolescents and Adults CD4 cell count

A

B

C

>500/mL (>29%)

A1

B1

C1

200 to 499/mL (14% to 28%)

A2

B2

C2

<200/mL (<14%)

A3

B3

C3

Patients in categories A3, B3 and C1-C3 are considered to have AIDS

Category A • Acute retroviral syndrome • Asymptomatic HIV infection • Persistent generalized lymphadenopathy Category B Symptomatic conditions occurring in a HIVinfected adolescent or adult that meet at least one of the following criteria: 1. They are attributed to HIV infection or indicate a defect in cell-mediated immunity 2. They are considered to have a clinical course or management that is complicated by HIV infection Examples include, but are not limited to the following: – Constitutional symptoms (fever of 38.5°C [101.3°F], diarrhea >1 month) – Idiopathic thrombocytopenic purpura – Peripheral neuropathy Bartonella henselae, B. quintana: bacillary angiomatosis

– Pelvic inflammatory disease – Candidiasis: oropharyngeal or recurrent vulvovaginal – Varicella-zoster virus infection: herpes zoster (2 distinct episodes/ more than one dermatomes) – Epstein-Barr virus infection: oral hairy leukoplakia – Human papillomavirus infection: Cervical squamous intraepithelial lesion (dysplasia/carcinoma in situ)

Category C – HIV encephalopathy – Progressive multifocal leukoencephalopathy – Lymphoma: Burkitt’s, immunoblastic or primary CNS lymphoma – Mycobacterium avium-intracellulare complex or M. kansasii infection: disseminated/ extrapulmonary – M. tuberculosis: pulmonary/ extrapulmonary – Mycobacterium: other species/ unidentified species - disseminated/ extrapulmonary – Pneumonia (recurrent with >2 episodes in 12 months) – Salmonella septicemia, recurrent (nontyphoid) – Candidiasis: esophageal, pulmonary – Cryptococcosis: extrapulmonary – Coccidioidomycosis: disseminated/ extrapulmonary – Histoplasmosis: disseminated/ extrapulmonary – Pneumocystis jiroveci (formerly carinii) pneumonia – Herpes simplex virus infection: esophageal, pulmonary, mucocutaneous ulcers of >1 month duration – Cytomegalovirus: other than liver, spleen or nodes

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Essentials in Dermatology – – – – – – –

Human herpesvirus-8 infection: Kaposi’s sarcoma Human papillomavirus infection: invasive cervical cancer Cryptosporidiosis: chronic intestinal (>1 month duration) Isosporiasis: chronic intestinal (>1 month duration) Toxoplasmosis of brain Kaposi’s sarcoma Wasting syndrome due to HIV (involving weight loss >10% of baseline body weight), associated with either chronic diarrhea (> 2 loose stools per day for > 1 month) or chronic weakness and documented fever > 1 month

Note AIDS in HIV-infected adolescents and adults aged >13 years defined by CDC includes any one of the following criteria: (a) <200 CD4+ cells/ mL; (b) a CD4+ cell percentage of total lymphocytes of <14 percent; or (c) any of the following three clinical conditions: pulmonary tuberculosis, recurrent pneumonia, or invasive cervical cancer.

WHO Criteria Used for Diagnosing HIV Infection/AIDS The WHO has proposed an alternative definition for AIDS, in geographic areas where only clinical parameters can be used. Provisional World Health Organization (WHO) clinical case definition for AIDS where diagnostic resources are limited. WHO case definition for AIDS surveillance:

Adults AIDS in an adult is defined by the existence of at least two of the major signs associated with at least one minor sign in the absence of known

causes of immunosuppression such as cancer or severe malnutrition or other recognized causes. Major Signs 1. Weight loss greater than 10 percent of body weight. 2. Chronic diarrhea for more than 1 month. 3. Prolonged fever for more than 1 month (intermittent or consistent) Minor Signs 1. Persistent cough for more than 1 month. 2. Generalized pruritic dermatitis. 3. Recurrent herpes zoster infection. 4. Oropharyngeal candidiasis. 5. Chronic progressive and disseminated herpes simplex virus infections 6. Generalized lymphadenopathy The presence of generalized Kaposi’s sarcoma or cryptococcal meningitis itself is sufficient for the diagnosis.

Children Pediatric AIDS is suspected in an infant or child presenting with at least two major signs associated with at least two minor signs in the absence of other known causes of immunosuppression. Major Signs 1 Weight loss or abnormally slow growth 2. Chronic diarrhea for more than 1 month 3. Prolonged fever for more than 1 month Minor Signs 1. Generalized lymphadenopathy 2. Oropharyngeal candidiasis 3. Repeated common infections (otitis, pharyngitis etc.) 4. Persistent cough for more than 1 month 5. Generalized dermatitis. 6. Confirmed maternal HIV infection.


WHO Case Definition for HIV Infection Adults and Children 18 Months or Older Positive HIV antibody testing (rapid/ ELISA) confirmed by a second HIV antibody test (rapid / ELISA) relying on different antigens or a different operating technique AND/OR Positive virological test for HIV or its components (HIV RNA or HIV DNA or ultrasensitive HIV p24 antigen) confirmed by a second virological test obtained from a separate determination.

Children Younger than 18 Months A positive virological test for HIV or its components (HIV RNA or HIV DNA or ultrasensitive HIV p24 antigen) confirmed by a second virological test obtained from a separate determination, taken more than 4 weeks after birth. Positive HIV antibody testing is not recommended for definitive/ confirmatory diagnosis of HIV infection in children until 18 months of age. Case definition for AIDS in India: Case definition for AIDS in India was revised in October, 1999. The new case definition is as follows: I. Case Definition of AIDS in Children (up to 12 years of age) 1. The positive tests for HIV infection by ERS (ELISA/RAPID/SIMPLE) in children above 18 months or confirmed maternal HIV infection for children less than 18 months. AND 2. Presence of at least two major and two minor signs in the absence of known causes of immunosuppression.

Major Signs a. Loss of weight or failure to thrive which is not known to be due to medical causes other than HIV infection. b. Chronic diarrhea (intermittent or continuous) > 1 month duration. c. Prolonged fever (intermittent or continuous) > 1 month duration.

Minor Signs a. Repeat common infections (e.g. Pneumonitis, otitis, pharyngitis etc.) b. Generalized lymphadenopathy c. Oropharyngeal candidiasis d. Persistent cough for more than 1month e. Disseminated maculo-papular dermatosis II. Case Definition of AIDS in adults (for persons above 12 years of age) 1. Two positive tests for HIV infection by ERS test (ELISA/RAPID/SIMPLE) AND 2. Any one of the following criteria: a. Significant weight loss (> 10% of body weight) within last one month/Cachexia (not known to be due to a condition other than HIV infection). AND Chronic diarrhea (intermittent or continuous) > 1 month duration or prolonged fever (intermittent or continuous) > 1 month duration b. Tuberculosis: Extensive pulmonary, disseminated, miliary, extra-pulmonary tuberculosis. c. Neurological impairment preventing independent daily activities, not known to be due to the conditions unrelated to HIV infection (e.g. trauma) d. Candidiasis of the esophagus (diagnosable by oral candidiasis with odynophagia) e. Clinically diagnosed life-threatening or recurrent episodes of pneumonia, with or without etiological confirmation f. Kaposi’s Sarcoma g. Other conditions: - Cryptococcal meningitis - Neuro toxoplasmosis - CMV retinitis - Pencillium marneffei - Recurrent herpes zoster or multidermatomal herpes infection - Disseminated molluscum contagiosum

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Diagnosis of HIV infection • HIV infection is diagnosed on the basis of blood tests using three different ELISA/ Rapid/simple tests using different antigen preparation. • AIDS cases are diagnosed on the basis of two different ELISA/Rapid tests on different antigens and presence of AIDS related opportunistic infections. • Western Blot test is used for confirmation of diagnosis of indeterminate ELISA tests.

Mucocutaneous Manifestations of HIV Infection Mucocutaneous diseases are amongst the first recognized clinical manifestations of AIDS. Over the past decade, it has become increasingly clear that cutaneous disorders are not only associated with terminal immunodeficiency but occur throughout the course of HIV infection. This may be due to impairment of the skin immune system as evidenced by the reduced epidermal Langerhans cell population, which explains the development of opportunistic infections and neoplasms of the skin and mucous membranes in these patients. Mucocutaneous disorders remain one of the most important clinical markers of the stages of HIV infection from time of seroconversion. They range from urticarial viral exanthema to a myriad of cutaneous opportunistic infections or malignancies. They function as visual markers in assessing the progression of HIV disease. Cutaneous disorders occur more frequently as HIV infection advances and immune function deteriorates. They affect between 80% and 95% of HIV infected patients. Cutaneous disorders during HIV infection are numerous. Some have drawn attention because of their onset, defines some of the Center for Disease Control and Prevention (CDC), acquired immunodeficiency syndrome (AIDS) clinical categories, e.g. oral candidiasis, zoster, herpes simplex, oral hairy

leukoplakia and Kaposi’s sarcoma, but most have been documented solely in case reports. Mucocutaneous disorders are commonly the indication for initial HIV serotesting and they are listed in table. Mucocutaneous disorders as indicators for HIV serotesting A. Disorders strongly associated with HIV infection 1. Candidiasis-oropharyngeal (Figs. 32.1 and 32.2) or recurrent vulvovaginal 2. Herpes zoster-necrotic (Fig. 32.3), multidermatomal or disseminated 3. Chronic herpes simplex infection (Fig. 32.4) 4. Oral hairy leukoplakia (Fig. 32.5) 5. Papular eruption of HIV (Fig. 32.6) 6. Molluscum contagiosum-face involvement in an adult or giant molluscum contagiosum (Fig. 32.7) 7. Proximal white subungual onychomycosis 8. Kaposi’s sarcoma B. Disorders commonly associated with HIV infection 1. Any sexually transmitted disease-indicative of unsafe sexual behaviour 2. Signs of injected drug use C. 1. 2. 3.

Disorders associated with HIV infection Generalized lymphadenopathy Psoriasis vulgaris-explosive onset Seborrheic dermatitis-in an adult extensive and refractory to therapy 4. Aphthous ulcers-recurrent, refractory to therapy 5. Crusted scabies

Xerosis/acquired ichthyosis and giant molluscum contagiosum are characteristically seen in group IV of HIV disease whereas oral candidiasis, oral aphthae, papular dermatitis of HIV and psoriasis are early warning signs. Moreover, with progression of HIV disease, the number of dermatoses goes on increasing. Common dermatoses assume aggressive postures (Figs 32.8 to 32.13) and some rare dermatoses (Figs 32.14 and 32.15) also present.

Treatment • There is no cure for AIDS at this time. However, treatments are available that can


Fig. 32.1: Oral candidiasis—curdy white deposits over the tongue

Fig. 32.2: Hyperplastic candidiasis of the tongue

Fig. 32.3: Herpes zoster—necrotic lesions of herpes zoster along a thoracic dermatome

Fig. 32.4: Herpes genitalis—non-healing impetiginized ulcers over the glans penis and prepuce

Fig. 32.5: Oral hairy leukoplakia—un-removable white striations on the side and dorsa of tongue

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Fig. 32.6: Papular eruption of HIV- itchy papular eruption over the back

Fig. 32.7: Molluscum contagiosum—giant lesions over the face

Fig. 32.8: Genital warts—multiple verruca vulgaris like genital warts

Fig. 32.9: Tinea versicolor—extensive macular lesions over the neck with cervical lymphadenopathy

Fig. 32.10

Fig. 32.11

Figs 32.10 and 32.11: Extensive tinea corporis and cruris lesions in a HIV patient


Fig. 32.12: Secondary syphilis—pigmented lesions in the palms

Fig. 32.14: Cryptococcosis—hemorrhagic crusted papular lesions over the face

Fig. 32.13: Secondary syphilis—hyperkeratotic scaly plaques over the lower legs

Fig. 32.15: Cryptococcosis—molluscum contagiosum like lesions over the chest

improve the quality of life of those suffering the infection. • Use of condom during sexual acts should be encouraged. • Patients need counselling at the first visit apart from the general and specific treatments.

• Antiretroviral therapy is discussed in next chapter. • Opportunistic infections are treated with appropriate therapeutic agents. With Highly Active Retroviral Therapy (HAART), the prevalence of mucocutaneous disorders in HIV infected patients is coming down.

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33

Antiretroviral Therapy (ART)

Development of Antiretroviral Therapy (ART) • Zidovudine was first tested on humans in 1985 and introduced for treatment in March 1987. • Zalcitabine (ddC), didanosine (ddI), and stavudine (d4T) were introduced between 1991-1994. • Between December 1995 and March 1996, three protease inhibitors (PIs)-indinavir (IDV), saquinavir (SQV), and ritonavir (RTV) got approval. • 1996 onwards, combination therapy, highly active antiretroviral therapy (HAART) began to spread irreversibly. • In 1995, David HO came out with his slogan “hit hard and early”. • By June 1996, first NNRTI (nevirapine), a third drug class of antiretroviral drugs was licensed. Soon after another PI, nelfinavir, also arrived. • In 1996-97, some patients began to complain of fat stomach and buffalo hump with PIs but retained their thin facies and thin legs. • In 1998, a term lipodystrophy was used to describe the characterstic fat distribution that patients on HAART had developed. • In1999 “Mitochondrial toxicity” hypothesis emerged as the reason behind lipodystrophy.

• By year 2000, many strict recommendations got revised. “Hit HIV hard, but only when necessary” now heard more than” hit hard and early”. • Despite all skepticism, HAART can often achieve miracles e.g., cryptosporidiosis and Kaposi’s sarcoma simply disappear. Once daily regimens, even twice weekly regimens are coming. • New classes of drugs are appearing- co receptor antagonists, attachment-, integrase, and maturation inhibitors. • Since 1995, protease inhibitors (PIs) have revolutionized the treatment of HIV infection, but in recent years they have been criticized for their high pill burden and side effects. They remain an essential component of HAART especially for treatment experienced patients. With growing knowledge of mitochondrial toxicity of nucleoside analogs and the introduction of easy to take PIs, boosted PIs, this class of drugs is currently experiencing something of renaissance. Boosting with small doses of ritonavir is based on the fact that ritonavir a very potent inhibitor of the isoenzyme 3A4, a subunit of the cytochrome P450 hepatic enzyme system, allows boosting of the most important pharmacokinetic parameters of all

Antiretroviral Therapy (ART) PIs (except nelfinavir), maximum concentration, trough levels, and half life. Boosting is usually indicated by addition of “/r” after the drug name. • Reduction in the cost of ART from INR 35000/month (in year 2000) to approx. INR 1000/month made ART affordable. There is further chance of 20% price reduction.

Goals of Antiretroviral Therapy Eradication of HIV infection cannot be achieved with available antiretroviral regimens. Therefore, once the decision is made to initiate therapy, the primary goals of antiretroviral therapy are: 1. Reduction of HIV-related morbidity and mortality 2. Maximal and durable suppression of viral replication 3. Preservation and/or restoration of immunologic functions 4. Improvement of quality of life Currently available antiretroviral drugs have been classified in the Table 33.1. Antiretroviral Drugs 1. Nucleoside reverse transcriptase inhibitors (NRTIs): Their target is the HIV enzyme reverse transcriptase. 2. Non-nucleoside reverse transcriptase inhibitors (NNRTIs): The target enzyme is

reverse transcriptase. However, NNRTIs bind directly and noncompetitively to the enzyme at a position in close proximity to the substrate binding site for nucleosides. The resulting complex blocks the catalyst activated binding site of the reverse transcriptase. This, in turn, can bind fewer nucleosides, slowing polymerization down significantly. In contrast to NRTIs, NNRTIs do not require activation within the cell. The three available NNRTIs are nevirapine, delavirdine and efavirenz. 3. Protease inhibitors (PIs): The HIV protease cuts the viral gag-pol polyprotein into its functional subunits. If the protease is inhibited and proteolytic splicing prevented, non-infectious virus particles will result. 4. Entry inhibitors: There are three crucial steps for entry of HIV into the CD4+ T-cell. a. Binding of HIV to the CD4 receptor (target of attachment inhibitors), b. Binding to coreceptors (target of coreceptor antagonists), and finally c. Fusion of virus and cell (target of fusion inhibitors) 5. Integrase inhibitors: Integrase, along with reverse transcriptase and protease, is one of the three key enzymes in the HIV replication cycle. This enzyme is involved in the integration of viral DNA into the host genome, and is essential for the proliferation

Table 33.1: Currently available antiretroviral drugs

NRTI

NNRTI

PI

Azidothymidine(AZT)

Nevirapine (NVP)

Indinavir (IDV)

Stavudine (d4T)

Efavirenz (EFV)

Nelfinavir(NFV)

Didanosine (ddI)

Delaviridine(DLV)

Saquinavir(SQV)

Zalcitabine (ddC)

Ritonavir(RTV)

Abacavir (ABC)

Amprenavir(APV)

Tenofovir (TFV)

Lopinavir(LPV)

Emtricitabine(FTC)

Atazanavir(ATV)

Lamivudine (3TC)

Fosamprenavir(FPV)

* Fusion inhibitor: Enfuviritide

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Essentials in Dermatology of HIV. Integrase inhibitors are occasionally falsely classified as entry inhibitors. However, unlike the latter, they do not prevent entry of the virus into the cell. 6. Maturation inhibitors: The so-called maturation inhibitors inhibit HIV replication in a very late phase of the HIV reproduction cycle, i.e., by the budding or maturation of new virions. As is the case for integrase inhibitors, 2005 can be counted as the introductory year.

Highly Active Antiretroviral Therapy (HAART) It is a combination of different classes of antiretroviral in order: to achieve maximal (below level of detection) and most durable suppression of viral replication; prevent emergence of drug resistant mutants and to improve survival and quality of life. HAART has drawbacks of high cost, not being a cure, has short and long term side effects, drug interactions, need for stringent adherence, and risk of viral resistance. The characteristics of an ideal first line ART are that it should be affordable, effective and well tolerated, potent and robust, minimal drug inter-/contra-actions, appropriate for use in TB and pregnancy, and stable in tropical conditions Indications for Antiretroviral Therapy • Antiretroviral therapy is recommended for all patients with history of an AIDS-defining illness or severe symptoms of HIV infection regardless of CD4+ T cell count. (AI) (WHO clinical stage IV). • Antiretroviral therapy is also recommended for asymptomatic patients with <200 CD4+ T cells/mm3(AI) (WHO clinical stage I, II, III with CD4 cell counts <200/mm3). • Asymptomatic patients with CD4+ T cell counts of 201–350 cells/mm 3 should be offered treatment. (BII) (WHO clinical stage III).

• For asymptomatic patients with CD4+ T cell of >350 cells/mm3 and plasma HIV RNA >100,000 copies/mL most experienced clinicians defer therapy but some clinicians may consider initiating treatment. (CII). • Therapy should be deferred for patients with CD4+ T cell counts of >350 cells /mm3 and plasma HIV RNA <100,000 copies/mL. (DII).

Recommendations of HAART Regimens: What to start with? WHO recommended (2003) first and second line regimens are given in Table 33.2. DHHS panel recommended NNRTI based regimen is efavirenz +zidovudine or tenofovir + lamivudine or emtricitabine and PI based regimen is lopinavir/ritonavir+ zidovudine+ lamivudine or emtricitabine. Various possible combinations with NRTI drugs (nukes) as backbone are given in Table 33.3. First Line Combination in India Today’s best available choice is combination of stavudine/lamivudine/nevirapine. It has the Table 33.2: WHO recommended 2003 ART regimens

First line regimen

Second line regimen

Stavudine (D4T) or Azidothymidine (AZT) +

Tenofovir (TFV) or Abacavir (ABC) +

Lamivudine (3TC)

Didanosine (ddI)

+

+

Nevirapine (NVP) or Efavirenz (EFV)

Lopinavir(LPV)or Saquinavir(SQV)

Table 33.3: HAART- Various possible combinations with nukes as backbone Column A • AZT + 3TC • d4T + 3TC • AZT + ddI

Column B

with

• • • • • •

NVP EFV NFV RTV + LPV RTV + IDV RTV + SQV

Antiretroviral Therapy (ART) advantage of being well tolerated, affordable, and easy to take. But it is ineffective against HIV2, and has drug interactions with rifampicin.

Alternative First Line Combinations in India • Stavudine/lamivudine/efavirenz (can be used with rifampicin, teratogenic) • Zidovudine/lamivudine/nevirapine (can be used in stavudine induced neuropathy, but has risk of anemia, and thus requires Hb monitoring).

• 3-NRTI combinations – Abacavir + tenofovir + lamivudine – Didanosine + tenofovir + lamivudine – Zidovudine + lamivudine + abacavir

Govt. Will Provision For Antiretroviral (ARV) Drugs With Following Combination • Stavudine (30 or 40 mg) BID or Zidovudine (300 mg) BID + Lamivudine (150 mg) BID+ Nevirapine (200 mg) BID or Efavirenz (600 mg) OD.

Pretreatment Evaluation It is essential to confirm HIV results, complete history and physical examination, treat OI (TB, Toxoplasmosis, and cryptococcosis), complete blood counts (CBC), chemistry profile, VDRL, HBsAg. Assessment of “readiness” for treatment and adherence is a must. It is desirable to get CD4+ T-lymphocyte count and optional to get plasma HIV RNA measurement if affordable. For patients with pretreatment HIV RNA >1,000 copies/mL genotypic resistance testing prior to initiation of therapy (BIII) should be considered; if therapy is to be deferred, resistance testing may still be considered (CIII).

ART Regimens Not Recommended • Monotherapy should not be used as it is less potent and does not have sustained antiviral activity. • Dual NRTI therapy has not demonstrated potent and sustained antiviral activity as compared to three-drug combination regimens. • Didanosine + stavudine combination can result in a high incidence of toxicities, particularly peripheral neuropathy, pancreatitis, and lactic acidosis. • Stavudine + zidovudine combination should be avoided because of the demonstration of antagonism in vitro and in vivo. • Didanosine + tenofovir combination has high failure rate of therapy, higher toxicity (pancreatitis). • Zalcitabine containing regimens are less convenient (given three times daily) and more toxic.

Follow-up of Patients on HAART Two surrogate markers are routinely used to determine indications for treatment and to monitor the efficacy of therapy: CD4+ T cell count and plasma HIV RNA (or viral load). The CD4+ T cell count should be determined every three to six months to (1)to determine when to start ART; (2) to assess immunologic response to ART; and (3) to assess the need for initiating chemoprophylaxis for opportunistic infections. Plasma HIV RNA (viral load) should be monitored every 2-8 weeks at initiation or for change in therapy and to confirm potency of the new regimen. In patients on stable ART, viral load should be monitored every 3-4 month or if clinically indicated. Efficacy of HAART is determined by clinical evaluation (weight gain, resolution of OI), viral load reduction (undetectable levels after 24 weeks), and CD4 cell counts (increase by 100-150 cells/mm3/ year).

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Adverse Effects of Antiretroviral Drugs Gastrointestinal problems are the most common side effects of almost all antiretroviral drugs nucleoside analogs, NNRTIs and particularly protease inhibitors – and occur especially during the early stages of therapy. Typical symptoms include abdominal discomfort, loss of appetite, diarrhea, nausea and vomiting. Heartburn, abdominal pain, meteorism and constipation may also occur. Nausea is a common symptom with zidovudine-containing regimens; diarrhea occurs frequently with zidovudine, didanosine and all PIs, particularly with ritonavir and nelfinavir, as well as with saquinavir, lopinavir atazanavir and tipranavir. Treatment with zidovudine rarely leads to a severe form of gastritic pain, nausea and vomiting in the early phase of therapy, in which case it should be discontinued. Serious Adverse Effects of HAART NRTI 1. Lactic acidosis mostly related to stavudine and didanosine, less in patients on zidovudine, abacavir and lamivudine. Risk factors are obesity, female sex, pregnancy and therapy with ribavirin or hydroxyurea, a diminished creatinine clearance and a low CD4+ T-cell nadir. 2. Hepatic steatosis, is probably caused by mitochondrial toxicity and usually occurs after more than 6 months on treatment 3. Pancreatitis - Up to 7 % of patients treated with didanosine suffer from pancreatitis. Occasionally, stavudine, lamivudine and zalcitabine cause pancreatitis too. 4. Hypersensitivity reactions-especially in patients on abacavir. 5. Anemia especially in patients on treatment with zidovudine 6. Peripheral polyneuropathy mostly caused by the zalcitabine, didanosine and stavudine. It can present as paresthesia and pain in hands

and feet or distal symmetrical sensorimotor paralysis 7. Myopathy, 8. Lipoatrophy

NNRTI 1. Rash-Nevirapine and delavirdine may cause a slight rash in 15 to 20% of patients, 5 to 10% of which discontinue treatment. The rash is seen less frequently on efavirenz therapy, where only 2 % of the patients discontinue the drug. The NNRTI allergy is a reversible, systemic reaction and typically presents as an erythematous, maculopapular, pruritic and confluent rash, distributed mainly over the trunk and arms. Fever may precede the rash. Further symptoms include myalgia (sometimes severe), fatigue and mucosal ulceration. The allergy usually begins in the second or third week of treatment. Women are more often and more severely affected. If symptoms occur later than 8 weeks after initiation of therapy, other drugs should be suspected. 2. Stevens Johnsons Syndrome 3. TEN 4. Hepatitis-hypersensitive hepatitis often occurs within the first 12 weeks. Liver toxicity occurs more commonly in patients on nevirapine than on other antiretroviral drugs. 5. Neuro-psychiatric-common in patients on treatment with efavirenz. It includes dizziness, insomnia, nightmares, mood fluctuations, depression, depersonalization, paranoid delusions, confusion and suicidal ideation. These side effects are observed mainly during the first days and weeks of treatment. Discontinuation of therapy becomes necessary in only 3 % of patients.

PI 1. Renal stones- occur particularly on indinavir treatment,

Antiretroviral Therapy (ART) 2. Hepatitis, PIs can lead to hepatotoxicity at any stage during the course of treatment. Patients with chronic viral hepatitis are particularly at risk. One possible cause is an immune reconstitution syndrome on HAART, with increased cytolytic activity against the hepatitis viruses. Among the PIs, toxic hepatitis is seen most frequently in patients on boosted atazanavir, indinavir and tipranavir, a novel non-petidic protease inhibitor. 3. Rash 4. Dyslipidemia 5. Glucose intolerance 6. Lipodystrophy 7. Osteoporosis

Change of Therapy Treatment Regimen Failure Virologic failure can be defined as incomplete or lack of HIV RNA response to antiretroviral therapy: • Incomplete virologic response is defined as repeated HIV RNA >400 copies/mL after 24 weeks or >50 copies/mL by 48 weeks in a treatment-naïve patient initiating therapy. • Virologic rebound is repeated detection of HIV RNA after virologic suppression i.e. repeated HIV RNA level >400 copies/mL after prior suppression of viremia to <400 copies/mL. Immunologic failure can be defined as failure to increase the CD4 cell count by 25-50 cells/mm3 above the baseline count over the first year of therapy, or a decrease to below the baseline CD4 cell count on therapy. Mean increases in CD4 cell counts in treatment-naïve patients with initial antiretroviral regimens are approximately 150 cells/mm3 over the first year. Clinical progression can be defined as the occurrence or recurrence of HIV-related events

(after at least 3 months on an antiretroviral regimen), excluding immune reconstitution syndromes.

Treatment regimen failure: Assessment • Review antiretroviral history • Assess adherence, tolerability, drug-drug interactions, pharmacokinetic issues • Physical exam for signs of clinical progression • Resistance testing where feasible (while patient is on therapy or within 4 weeks after regimen discontinuation ) • Identify other treatment options If adherence is good, switch entire regimen.

Changing therapy: Toxicity Change the offending drug in case of • Anemia on AZT, switch to d4T • Peripheral neuropathy on ddI/d4T, switch to AZT/3TC • Nephrolithiasis on indinavir, switch to another PI • Hepatitis or severe rash on nevirapine, switch to efavirenz Thus change of regimen is required in cases of virologic failure, immunologic failure, clinical failure, drug toxicity and drug resistance Immune reconstitution inflammatory syndrome: The immune reconstitution inflammatory syndrome (IRIS) is characterized by worsening clinical, laboratory, or radiologic findings despite improvements in the HIV RNA level and CD4 count after the introduction of antiretroviral therapy and is due to the restoration of pathogen-specific immune responses. It is basically pathological inflammatory response, where paradoxical worsening occurs after HAART, in either previously treated infections or subclinical infections with recovery of immune responses. It has been described with mycobacteria, C. neoformans, herpes viruses, and Pneumocystis

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Essentials in Dermatology jerovecii pneumonia. IRIS may occur during or shortly after the treatment of an opportunistic infection or as a “new” clinical syndrome resulting from a previously unrecognized occult infection. Risk factors for IRIS include a low CD4 count (<50/ml), simultaneous (<30 days) initiation of OI treatment and HAART, the presence of latent infection(s), and a robust virologic and immunologic response to HAART. In addition to infectious pathogens, IRIS is

associated with autoimmune or malignancyrelated conditions. HAART should be continued. Specific antimicrobial therapy, nonsteroidal antiinflammatory drugs, and/or steroids for managing immune reconstitution syndrome should be considered. The prognosis for most IRIS cases is favorable because a robust inflammatory response may predict an excellent response to HAART in terms of immune reconstitution and, perhaps, improved survival.

Historical Milestones in Leprosy

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Historical Milestones in Leprosy

Leprosy has tormented humans throughout recorded history. The earliest possible account of a disease that many scholars believe is leprosy appears in an Egyptian Papyrus document written around 1550 BC. Around 600 BC, Indian writings described a disease that resembles leprosy. In Europe, leprosy first appeared in the records of ancient Greece after the army of Alexander the Great came back from India and then in Rome in 62 BC coinciding with the return of Pompeii’s troops from Asia Minor. Danielssen and Boeck (1849)

Established leprosy as a clinical entity

G Armauer Hansen on 8 February 1873

Not only was the bacillus, the first mycobacterium, discovered, it was the first etiologic agent of a chronic disease of humans

Rafael Lucio (1819-1896) of Mexico

Distinguished leprologist after whom a type of leprosy “Lucio leprosy” and the “Lucio phenomenon” are named

First International Leprosy Congress

Held in Berlin in 1897, contagious nature of the disease contributed to policy of segregation

Second International Leprosy Congress

Held in Bergen with Hansen as President, reaffirmed the policy of isolation and segregation

Hayashi and Mitsuda (1919)

Established the lepromin reaction

Leprosy in India (1929)

The Indian Leprosy Association began publication of its journal “Leprosy in India” (now entitled the Indian Journal of Leprosy)

International Leprosy Association (ILA)

Was founded in Manila in1936, under the auspices of the Leonard Wood Memorial and launch of International Journal of Leprosy

First ILA Congress

Was held in Havana in 1948, the term ‘leper’ in designation of the patient with leprosy be abandoned and the person suffering from the disease be designated as “leprosy patient”

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Essentials in Dermatology Oil of the chaulmoogra nut

Until the late 1940s, leprosy doctors all over the world treated patients by injecting them with oil from the chaulmoogra nut.

Guy Faget (1943)

Soluble sulphone, Promin success in leprosy established at Carville, Louisiana, USA

Robert Cochrane (1943)

Dapsone (DDS) was introduced for the treatment of leprosy patient in Chingelput, India

Lowe and Smith (1949)

First to use dapsone orally.

Khanolkar in India, 1950s and 1960s

Leprosy became recognized as primarily an affliction of peripheral nerves

National Leprosy Control Programme (NLCP) in 1954-55

Started in 1954-55, the mainstay of NLCP was the early detection and sustained, regular treatment of patients with dapsone monotherapy

Chapman Binford (1956)

On the basis of Virchow’s observations in 1863 and on his own clinical observations in Hawaii, proposed that M.leprae grew selectively in the cooler areas of the hosti.e., ears, skin, eyes, peripheral nerves and testes. This concept led Binford to infect hamsters successfully (1958)

Shepard (1960)

Developed the mouse footpad model

Browne (1962)

Showed clofazimine to be an effective treatment for leprosy

Ridley and Jopling (1962)

Codified a classification system based on clinical, bacteriologic, immunologic and histopathologic criteria

Opromolla (1963)

First to use rifamycin SV (a potent antituberculosis drug) for the treatment of leprosy

Pettit and Rees (1964)

Detection of sulphone-resistant strains

Sheskin (1965)

First to use thalidomide as a sedative for severe erythema nodosum leprosum(ENL)

Kirchheimer and Storrs (1969)

Nine banded armadillo used as an experimental animal, inoculated animals developed a massive, disseminated infection

International Federation of Antileprosy Associations (ILEP) (1975)

Formed from earlier existing Coordinating Committee of European Leprosy Agency (ELEP) founded in 1966

Working Group under the chairmanship of Dr MS Swaminathan in 1980

In 1980 the Government of India declared its resolve to “eradicate” leprosy by the year 2000 and the then Prime minister Mrs. Indira Gandhi directed the constitution of a working group

Historical Milestones in Leprosy WHO Study Group on Chemotherapy in 1981

Introduction of multidrug therapy (MDT) in leprosy

National Leprosy Eradication Programme (NLEP) in 1983

Was launched in 1983 with the introduction of multidrug therapy for leprosy following emergence of dapsone resistance

WHO Study Group on Chemotherapy in 1993

Made important recommendation on fixed duration MDT

Modified Leprosy Elimination Campaign (MLEC) in 1997

A meeting of States Health Secretaries and State Programme Officers was held from 31st July to 1st August, 1997 to discuss the findings and recommendations of mid term review and actions required for strengthening the programme. Based on its suggestions, Government of India decided to implement MLEC in all states/UTs

Leprosy declared eliminated as a public health problem in India

In 2006.

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History Taking and Examination in Leprosy

The aim of history taking is to study the patient as an individual, his environment and the reasons for his illness. The patient should be allowed to tell his/her history in his/her own words. It is only then the clinician should ask the patient to elaborate on particular aspects of the symptoms. History may be obtained from relatives, friends or onlookers in a depressed or confined patient. Registration: It is required for follow up reference of the patient. Name: This will not only help to elicit the history properly, but will also be of psychological benefit to the patient. Age: Leprosy is known to occur at all ages ranging from early infancy to very old age, but it shows a peak at ages of 10 to 14 years followed by a depression, which in turn is followed by a rise and plateau covering ages 30-60 years. Sex: Males are more often affected by leprosy than females. Native place: Since leprosy is endemic in certain areas of India, it is important to know the native place of a person, more so the place of residence in the past 10 years. Marital status, occupation, religion, literacy and income may be recorded.

PRESENTING COMPLAINTS History regarding skin patches, loss of sensation over the patches, loss of sweating, pain in nerves, loss of eyebrows/eyelashes, nasal stuffiness/ epistaxis, edema of legs, hoarseness of voice, tingling and numbness in limbs, weakness of hands and legs, eye pain/blurring of vision/ photophobia, joint pain/fever/malaise/flaring of patches may be recorded.

HISTORY OF PRESENT ILLNESS • Further details regarding present complaints are explored e.g., age of onset of disease, number of patches, site of onset of them, size, shape, color and surface of patches, evolution of them, any associated features. • In longstanding cases, enquiry may be made for hoarseness of voice, tingling and numbness in fingers and toes, blistering and deformities of hands and feet. Factors responsible for acute episodes in leprosy if any are found out.

PAST HISTORY Whether, he had similar complaints in the past, surgical correction of deformities, or lepra reaction (type 1 or type 2).

History Taking and Examination in Leprosy

TREATMENT HISTORY

CUTANEOUS EXAMINATION

Whether the patient has taken any treatment before and if so, the duration of treatment. If discontinued, any reason for that, any adverse effects noted

Following features are noted: Type of lesions-macule/papule/plaque/nodule – Number of lesions-1-3, 10, 20, numerous, uncountable – Colour- erythematous/hypopigmented/ hyperpigmented – Margins-welldefined/illdefined - regular/irregular – Surface- smooth/rough/shiny/dry - scaly/non-scaly - flat/raised – Induration-peripheral/central/whole lesion/none – Sweating-present/absent/impaired – Hairs over the lesion (s) present or absent, leprous alopecia • Distribution –symmetrical/asymmetrical/ localized/others • Sensory functions of the affected area- normal/ impaired/ absent with regard to touch, pain and temperature. • The most common sign of autonomic nerve damage is loss of sweating which is shown by dryness and may lead to fissuring. It may be tested by pilocarpine test.

FAMILY HISTORY If any one in the family is suffering from leprosy, needs to be enquired. Health status of family members should be ascertained.

Personal History It should focus on accommodation facility, number of rooms available and number of persons staying together. GENERAL PHYSICAL EXAMINATION • One may look for pallor in longstanding cases. • Hair loss in the form of ciliary madarosis, superciliary madarosis and alopecia of axillary and pubic hair may be noted in lepromatous leprosy cases. Besides this, such cases may show xanthelasma over the eyelids, collapse of bridge of the nose or perforation of the nasal septum. • Examination of mouth and pharynx may show papules on lips, nodules on the tongue/ palate or palatal perforation. • Nails may demonstrate longitudinal ridges and become dry, lustreless and shrunken. • Hands and feet may show disabilities and deformities like wrist drop, foot drop, claw hand/toes, ape thumb etc. Digits may have autoamputation. • Testes may get involved and finally may become atrophic. • Gynaecomastia and sparse beard may be an indication of testicular involvement in lepromatous leprosy cases.

EXAMINATION OF PERIPHERAL NERVES In leprosy cases, one may proceed with peripheral nerve examination starting with inspection (any visible deformity of limbs or face, wasting of the muscles, trophic skin changes, scar or wound), palpation (temperature of paralysed parts, consistency of muscles, hyperesthesia of skin, scar tenderness), assessing muscle power (gradation of muscle power from 0-5) and palpation of peripheral nerves for thickening and tenderness (main nerve trunks as well as cutaneous nerves especially those feeding the patch). After the physical examination is complete, make a slit skin smear for acid-fast bacilli. If the

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Essentials in Dermatology diagnosis is still in doubt take a biopsy from a skin lesion or from an enlarged nerve that carries only sensory fibers.

Decide 1. Does the patient have leprosy? 2. If so, where does his disease lie on the spectrum? 3. What is the stage of activity?

4. Is the patient having a lepra reaction? What is the immunologic response to M. leprae? 5. What do I need to teach or tell the patient? 6. Should I refer him for specialized care? 7. If not, what treatment should I give: specific antileprosy treatment, anti-inflammatory treatment, treatment for complications of leprosy, or treatment for other illness? 8. What are the social implications of these decisions?

Clinical Leprosy

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Clinical Leprosy

Leprosy (Hansen’s disease) is a chronic infectious disease caused by Mycobacterium leprae. It mainly affects the peripheral nerves. It also affects the skin, muscles, eyes, bones, testes and internal organs. There is no infectious disease in man in which the clinical picture is as varied as that of leprosy. Even then, leprosy can be easily diagnosed if we remember the three cardinal features of leprosy. 1. Hypopigmented or erythematous hypoanesthetic patch (es). 2. Thickening of peripheral nerves with sensory or motor deficit. 3. Demonstration of AFB from skin lesions. Any one of them if present in a given case, you can make a diagnosis of leprosy (provided he has yet not completed full course of treatment).

Definition of a Case of Leprosy The WHO in 1998 defined a case of leprosy as a person having one or more of the following features and who has yet to complete a full course of treatment: 1. Hypopigmented or reddish skin lesion(s) with definite loss of sensation. 2. Involvement of peripheral nerves as demonstrated by a definite thickening with loss of sensation.

3. Skin smear positive for acid fast bacilli (AFB) This definition also includes treatment defaulters and cases who relapse, but does not include cured persons or persons with late reactions or residual disabilities.

Agent • Mycobacterium leprae was discovered by Gerhard Henrik Armauer Hansen in 1873. • It is an acid-fast bacillus measuring 1 to 8 microns in length by 0.3 microns in diameter. • The capsule of lepra bacilli contains a phenolic glycolipid 1 (PGL1) that renders it chemically unique and antigenically specific. • It is an obligate intracellular parasite that divides every 12-13 days in histiocyte and schwann cells • The optimal temperature for growth for lepra bacilli is 30-33 centigrade.

Incubation Period • It can show considerable variation and in most instances between 2 and 5 years. Mode of Spread • The various modes of spread are by inhalation, direct contact, ingestion and insects.

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SPECTRUM OF LEPROSY Ridley and Jopling classified leprosy taking into account the immunological and histopathological basis in addition to the clinical and bacteriological findings. The classification divides leprosy into • Tuberculoid tuberculoid (TT) • Borderline tuberculoid (BT) • Borderline borderline /Mid borderline/ dimorphous leprosy (BB) • Borderline lepromatous (BL) • Lepromatous lepromatous (LL) The two other types of leprosy, which does not fall under Ridley and Jopling classification but are included in Indian Leprologists association classification are indeterminate and polyneuritic leprosy.

Some General Facts • Cell mediated immune response of the host determine the type of leprosy • TT and LL patients are stable • TT leprosy often self heals • LL leprosy remains heavily infected unless given appropriate chemotherapy • BB leprosy is the most unstable, with most patients down grading if not treated • Leprosy can affect all ages and both sexes • The limited growth of M. leprae in the mouse foot pad provided a way to screen for therapeutic agents and to identify drug resistance in leprosy • The recognition of leprosy in the nine banded armadillo provided a source of large quantities of highly purified M. leprae for biochemical and immunologic studies including preparation of vaccine for leprosy • Rifampicin was the first drug to be identified as bactericidal for M. leprae and is now the cornerstone of most therapeutic regimens Tuberculoid Leprosy • 1-3 hypopigmented well defined macules or plaques (Fig. 36.1).

Fig. 36.1: Tuberculoid leprosy- well defined oval erythematous hypoanesthetic plaque lesion over the trunk

• The lesions are dry, hypoanesthetic or anesthetic with loss of sweating and hair. • Occurs commonly in areas not covered by clothes. • Peripheral nerve trunks are not involved, but nerve feeding the patch may be thickened.

Borderline Tuberculoid Leprosy • 3-10 or more hypopigmented macules or plaques with irregular margins (Figs 36.2 and 36.3) • The lesions are large, hypoanesthetic with loss of appendages • Satellite lesions are seen around the large plaques. • Cutaneous nerves supplying the plaque may be thickened (feeder nerves) (Fig. 36.4) • Peripheral nerve trunks are irregularly enlarged and involved in an asymmetrical pattern. • Prone to develop type 1 lepra reaction.

Clinical Leprosy

Fig. 36.2: Borderline tuberculoid leprosy-hypopigmented hypoanesthetic macule with irregular well defined margin

Fig. 36.4: Borderline tuberculoid leprosy-hand showing a thickened cutaneous nerve feeding an erythematous hypoanesthetic plaque over the index finger

Fig. 36.3: Borderline tuberculoid leprosy- a hypoanesthetic ichthyotic plaque over the thigh with a few satellite lesions

Borderline Lepromatous • Numerous but still countable lesions are seen • The copper colored macules, plaques and nodules show a tendency towards symmetry (Figs 36.5 and 36.6). • The lesions are shiny with minimal sensory loss • Multiple peripheral nerves are thickened

Borderline Borderline Leprosy • Also known as dimorphous leprosy • More than 20 lesions are seen • The lesion are well to ill defined with irregular borders presenting with polymorphic or geographic appearance • Swiss cheese or punched out lesions are typical of BB Hansen • Peripheral nerves are thickened.

Lepromatous Leprosy • Initially has cutaneous and mucosal lesions • Edema of lower legs and epistaxis are considered early signs • The early skin lesions are macules which are uncountable (Fig. 36.7) • The skin lesions are small, coppery colored, numerous and symmetrically arranged • Over a period of time, the entire skin becomes shiny and infiltrated, most often on the face, especially forehead, ear lobes (Fig. 36.8),

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Fig. 36.5: Borderline lepromatous leprosy-multiple, countable, hypopigmented, slightly hypoanesthetic macular lesions over the trunk

Fig. 36.6: Borderline lepromatous leprosy-multiple, shiny, infiltrated plaques showing tendency to symmetrical distribution over the back

Fig. 36.7: Lepromatous leprosy-coppery colored shiny macules of uniformly small size over the upper back in early lepromatous leprosy

Fig. 36.8: Lepromatous leprosy- infiltrated pinna showing papules and nodules in a more advanced case

eyebrows, nose, and malar eminences, finally producing ‘leonine facies’(Fig. 36.9) • As the untreated lepromatous leprosy evolves, papules and nodules appear (Figs 36.10 and 36.11) • Nasal involvement occurs in the form of epistaxis, septal perforation and collapse of nasal bridge

• Neural changes occur later • Glove and stocking anesthesia, and thickening of peripheral nerve trunks (Fig. 36.12), bilaterally and symmetrically is a feature of advanced and progressive lepromatous leprosy • Gynecomastia (Fig. 36.13)and testicular atrophy (Fig. 36.14) occurs in late stages in males

Clinical Leprosy

Fig. 36.9: Lepromatous leprosy-Leonine facies

Fig. 36.10: Lepromatous leprosy- infiltrated shiny skin along with nodular gross lesions over the knee in an advanced lepromatous leprosy case

Fig. 36.11: Lepromatous leprosy-shiny diffuse infiltrated lesions on the back

Fig. 36.12: Lepromatous leprosy-thickening of greater auricular nerve

Fig. 36.13: Lepromatous leprosy-gynaecomastia

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Fig. 36.14: Lepromatous leprosy-sparse beard hairs due to testicular atrophy

• Multiple organs are involved due to bacillemia in the absence of cell mediated immune response against M leprae

Fig. 36.15: Histoid leprosy-waxy shiny nodules of histoid leprosy over apparently normal skin over the elbow and knee

Indeterminate Leprosy • Usually occurs as solitary, hypopigmented, vaguely defined macules • Common in children • Sensory loss is equivocal • Most lesions heal spontaneously • Mitsuda negative patients are more likely to develop lepromatous leprosy

• It is characterized by the occurrence of well demarcated cutaneous and subcutaneous nodules and plaques over apparently normal skin (Fig. 36.15) • Histoid leprosy lesions show the highest loads of bacilli (the bacteriological index is frequently 6+) and the majority are solid staining, arranged in clumps like sheaves of wheat • The histiocyte (macrophage) tissue reaction is unusual in that the cells frequently become spindle shaped and oriented in storiform pattern, similar to those of fibrohistiocytoma • The presence of these spindle shaped histiocytes in histopathological section has given this entity the name of ‘histoid leprosy’

Polyneuritic/Pure Neuritic Leprosy • Manifests only with neural signs without any clinical evidence of skin lesions • Peripheral nerves are thickened and associated with sensory loss and motor weakness • The deep reflexes are normal in contrast to other peripheral neuropathies Histoid Leprosy • A distinct, stable and unique variant of lepromatous leprosy • It frequently follows incomplete chemotherapy or acquired drug resistance

Lucio Leprosy • Occurs in Mexico ( in South America), also called as lepra bonita /beautiful leprosy • Diffuse infiltration occurs without distorting the body contour • May mimic myxedema

Clinical Leprosy

DIAGNOSIS Slit Skin Smear (SSS) • Consists of obtaining tissue smear from the skin and staining with modified Ziehl-Neelsen stain • Smear should be taken from six sites: both ear lobes and four representative active skin lesions • In case of single patch, smear should be taken from two sites diagonally opposite to each other • A SSS is positive if at least 104 bacilli are present per gram of tissue • Grading of SSS is based on Ridley’s logarithmic scale _ 1 to 10 bacilli in 100 fields -1 + _ 1 to 10 bacilli in 10 fields -2 + _ 1 to 10 bacilli in an average field -3 + _ 10 to 100 bacilli in an average field -4+ _ 100 to 1000 bacilli in an average field -5 + _ Clumps and globi in an average field -6+ (Fig. 36.16) • Bacteriological index gives a quantitative measure of M. leprae from the skin • BI falls by 1 unit every year after starting the patient on MDT • Morphological index is given as the percentage of regularly stained bacteria

Fig. 36.16: Slit skin smear showing red rods of acid fast bacilli of M leprae packed as globi inside macrophages (6+ on Ridley’s logarithmic scale)

• The MI falls to zero by 1 month after starting the patient on multibacillary treatment.

Lepromin Skin Test • Lepromin is prepared from M.leprae and used as skin test antigen • The two lepromin commonly used are Mitsuda lepromin (commonly armadillo derived) and Dharmendra lepromin (human tissue derived) • Intradermal injection of lepromin evolves into two types of responses, the early (Fernandez) and delayed (Mitsuda) reactions • Fernandez reaction (within 48 hours) is a delayed hypersensitivity response to bacillary antigen • Mitsuda reaction (after 4 weeks) is a reliable indicator of cell mediated immunity against lepra bacilli • Both lepromin produce early and late reaction but Fernandez reaction is prominent with Dharmendra lepromin and Mitsuda reaction is prominent with Mitsuda lepromin • Lepromin is strongly positive in TT Hansen and gradually decreases across the spectrum and becomes negative in LL Hansen. Histopathology Conclusive proof of leprosy is the demonstration of an intraneural granuloma or the presence of acid-fast bacilli in nerves. • TT Hansen- well defined epithelioid granulomas with Langhan giant cells eroding into the epidermis • BT Hansen- epithelioid granuloma is seen with giant cells in the upper dermis • BB Hansen- few epithelioid granulomas with absence of giant cells and scanty lymphocytes. AFB are demonstrable • BL Hansen- granuloma composed of histiocyte, lymphocytes and macrophages. Nerves show schwann cell proliferation (onion peel appearance)

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Essentials in Dermatology • LL Hansen- subepidermal zone is free (Grenz zone) with diffuse infiltration of foamy macrophages (Virchowcytes) in the dermis. Abundant AFB, even globi may be seen.

DIFFERENTIAL DIAGNOSIS OF LEPROSY The first step towards diagnosing leprosy is to think of the possibility of leprosy. The diagnosis can easily be made if the case is classical i.e., having characteristic skin changes (hypopigmented patch with loss of sensation) and nerve involvement, but this is not always the case. Leprosy produce wide variety of clinical situations depending upon the interaction between M. leprae and host’s immune system such as repeated epistaxes , paresthesias, mono or unilateral neuralgia, muscle weakness, paralysis, deformities, painless trauma or ulcer, chronic asymptomatic skin lesions, madarosis, upgrading and downgrading reactions, etc. Active leprosy gives rise to either mono or polymorphous clinical pictures where different skin lesions (macules, papules, plaques, nodules, diffuse infiltration) are variously assorted. Before diagnosing a case of leprosy one should be aware of following problems: 1. Bacteriological negative skin smear doesn’t rule out diagnosis. 2. AFB detection in nasal smear by itself is not sufficient for diagnosis. 3. Lepromin test does not have any diagnostic value. 4. Sometime, histopathological examination may not show any evidence from suspected leprosy lesions. 5. Leprosy presenting with only skin lesions or nerve lesions 6. Contact with leprosy case can not be regarded as diagnostic criteria. Therefore to formulate a diagnosis of leprosy, clinical, microbiological and histopathological findings must be taken into account. The typical

association of skin lesions and symptoms of nerve involvement is peculiar to leprosy even though not constantly occurring.

Differential Diagnosis of Cutaneous Manifestations Macules in leprosy are fixed, round or oval in shape, nonpruriginous, nonvesiculate, chronic in evolution and resistant to topical therapies. They may be erythematous, hypopigmented and rarely hyperpigmented. Differential diagnosis of erythematous macule (seen in all forms of leprosy) are early fixed drug eruption (initial lesion is an erythematous patch, have a burning sensation, commonly seen in perioral area, or genital areas, repeated episodes occur at the same site, preceding history of drug intake- sulfonamides, NSAIDs, tetracycline, metronidazole, phenobarbitone etc), morphea (one or few erythematous macules, gradually loose their color, become centrally white, and sclerotic), pityriasis rosea (self-limiting disorder, starts with a ‘herald patch’ 2-5 cm in diameter predominantly on the trunk or proximal extremities, daughter lesions follow Christmas tree pattern, individual lesion shows collarette scaling “Hanging curtain sign”), tinea corporis (itchy annular scaly erythematous patches with papulovesicular border, KOH examination demonstrates dermatophytes), syphilitic roseola (initially as erythematous macules called as ‘roseolar syphilide’, in a few days it evolve into papular form, nonscaly, non-itchy lesions follow lines of cleavage on back, history of sexual exposure, VDRL reactive), postkalazar dermal leishmaniasis “PKDL” (occurs after recovery from insufficiently treated visceral leishmaniasis, characterized by hypopigmented and erythematous macules, usually located on the perioral regions and cheeks in a butterfly distribution), seborrheic dermatitis (hypopigmented or erythematous papules and plaques with scaling, in adolescent or adult age group, with sites of

Clinical Leprosy predilection- scalp chest, back and face, presence of pruritus helps in differentiating it from leprosy, may have a history of cradle cap during infancy, treatment with topical steroids and oral antifungals like fluconazole helps in resolution), gyrate erythemas-erythema chronicum migrans (skin manifestation of Lyme’s disease, a spirochete (Borrelia burgdorferi) infection, caused by the bite of Ixodes ticks, starts as erythematous macule at bite site, extends peripherally to become annular lesion which may persist for months and associated with fever, headache, myalgia and arthralgia, titration of anti-Borrelia antibody helps in diagnosis), erythema annulare centrifugum (characterized by annular or polycyclic lesions with raised erythematous border, cause is unclear but in some cases hypersensitivity to tinea infection or association with malignancy is suspected, persists for weeks to months and tends to recur over the years), erythema gyratum repens (characterized by concentric raised erythematous bands moving in waves over the body surface in a “wood-grain” pattern at a speed of approximately 1 cm/day, always associated with internal malignancy), erythema marginatum (eruption has a serpiginous pink indurated border, blanches on pressure and changes its shape and is often hidden by the clothing, occurs in association with rheumatic fever), erythema multiforme (acute onset eruption in acral areas, typical lesion is target lesion, recurrent form is associated with herpes labialis) Differential diagnoses of hypochromic macule are nevus anemicus (a developmental anomaly, presents as a circular patch of pale skin of normal texture, does not flare on stroking, intradermal injection of histamine produces a flare whereas in leprosy the flare is delayed, feeble or entirely absent), nevus depigmentosus (achromicus) (birthmark, poorly demarcated hypopigmented patches -resemble splashed paint in a dermatomal pattern, diascopy does not show any change in color at the edge), vitiligo (presents with

depigmented macules but can present with hypo pigmented macule in previtiligo, also shows leucotrichia, peripheral and perifollicular hyperpigmentation, and normal histamine test, no loss of sensation), tinea versicolor (hypo pigmented usually on dark skin, well defined, multiple, asymptomatic or mildly itchy macules with fine branny scales, mainly on upper back, shoulder and chest, KOH examination shows banana and grape appearance, gold to orange fluorescence on Wood’s light examination), pityriasis alba (seen in children, on the face as hypopigmented macules with powdery scaling, has a seasonal variation and is more in summer seasons, a manifestation of atopic diathesis, if the lesion persists, it is often difficult to differentiate from indeterminate leprosy as leprosy patch on the face is not always anesthetic), postinflammatory lesions (occurs most commonly with pityriasis rosea, the lesions show a characteristic ‘Christmas tree’ pattern on the trunk), early PKDL ( as described earlier), yaws and pinta (in secondary stage of yaws and late stage of pinta, hypochromic macules may occur), onchocerciasis (confluent achromic macules occur on pretibial areas). Differential diagnosis of hyperchromic macule are residual lesions of fixed drug eruption, Kaposi’s sarcoma (hyperpigmented macules are seen in regressive stage), erythema dyschromicum perstans (eruption often begins with erythematous macules that rapidly evolves into blue-gray color with elevated edge, mainly seen on trunk, limbs and face), tinea nigra (a fungal infection produces brownish or black macules on palms and soles), and hyperchromic form of tinea versicolor. Differential diagnoses of papular lesions are sarcoidosis (a granulomatous multisystem disease presents with variable skin manifestations such as macule, papule, nodule and plaques, histopathologically shows characteristic naked epithelioid granulomas), granuloma

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Essentials in Dermatology annulare (presents typically with asymptomatic papules arranged in a ring like fashion over the hands and feet, disseminated lesions may develop later on, these lesions may disappear after a biopsy, the histology shows areas of necrosis with collagen degeneration), granuloma multiforme (can be confused with tuberculoid type of leprosy, the lesions are seen on the extremities-sun exposed areas as annular, with well marked papular edges, evolving simultaneously towards central healing, coalescing of annular lesions gives rise to circinate lesions, surroundings skin shows photo damaging changes, the lesions persists indefinitely or at least for several years), lichen planus (a papulosquamous disorder characterized by itchy, violaceous flat topped, polygonal papules especially on the flexor aspect of wrists, shins and trunk), late secondary syphilis (papules occur on the trunk and limbs in secondary syphilis, associated with mucosal erosions or ‘snail track’ ulcers, “Buschke Ollendroff” sign positive i.e. tenderness if lesion is pressed with a blunt object in the center, serological tests (VDRL) confirm the diagnosis, histopathological examination shows endarteritis with plasma cell infiltrate, lesions disappear in a few weeks if adequately treated with penicillin or resolve in two months and pass on to the early latent stage) and PKDL. Differential diagnoses of nodular lesions are Kaposi’s sarcoma (a vascular tumor characterized by the presence of bluish red or dark brown plaques or nodules, on the distal portion of lower extremities, may have lymph node involvement and visceral lesions in 10 percent of patients, the tumors may be associated with AIDS or human herpes virus 8 (HHV8), histopathology shows vascular formations with predominance of endothelial cells and spindle cell formations containing vascular slits), cutaneous leishmaniasis (single or multiple nodules, seen on the exposed parts i.e. at the site of insect bites.

Cutaneous leishmaniasis due to Leishmania ethiopica presents with lesions on the face with a few satellite papules, the lesion may ulcerate. In diffuse cutaneous leishmaniasis, multiple lesions are seen on the face and have to be differentiated from lepromatous leprosy. Leprosy has to be excluded by doing a slit skin smear or a biopsy. In leishmaniasis, the organisms are seen inside the macrophages and appear as a ‘dot and a dash’), mycetoma (a deep fungal infection, characterized by triad of nodules, discharging sinuses and discharge containing granules, commonly occurs on the foot), sarcoidosis (systemic granulomatous disease of unknown etiology, presents with various types of skin lesions including nodules,histology is characteristic, which show a naked epithelioid cell granuloma), Histoid leprosy (nodules of Histoid leprosy have to be differentiated from molluscum contagiosum, keloid and dermatofibroma and steatocystoma multiplex), neurofibromatosis (has to be differentiated from leprosy as it produces not only nodules but also thickened nerves like lepromatous leprosy, other signs seen in neurofibromatosis helps in easy differentiation such as Cafe-au-lait macules, axillary freckling “Crowe’s sign”, or palmar freckling “Patrick-Yesudian sign”, button-hole sign and Lisch nodules or iris hamartomas on slit lamp examination), dermatofibroma (a benign tumor with hard consistency, appears as small dome shaped nodule located on the extremity), molluscum contagiosum (pearly white or skin colored, asymptomatic nodules with central umblication seen commonly on the face and genitalia), cutaneous deposits in lymphoproliferative deposits, onchocerciasis, and PKDL. Differential diagnoses of plaque lesions are lupus vulgaris (a form of cutaneous tuberculosis, presents typically as a plaque, heals on one side and progresses on other side, shows apple jelly nodules on diascopy, histology shows tuberculoid

Clinical Leprosy granulomas with normal nerves), necrobiosis lipoidica (observed with diabetes mellitus, commonly over the legs and thighs as sharply marginated, firm, depressed waxy, yellow brown plaques with glistening surface and telangiectasia, heals with atrophy and scarring. Histopathology shows palisading granuloma, which is characteristic), late secondary syphilis, mycosis fungoides (Cutaneous T cell lymphoma (CTCL) affects the skin primarily, but ultimately, lymph nodes and visceral organs are involved. Skin lesions vary from erythematous patches to plaques, tumors and erythroderma. Plaque stages or tumor stages resemble lepromatous leprosy. Skin biopsy shows lymphocytic infiltrate with epidermotrophism. In the plaque stage, mycosis cells may be seen along with a band like dermal infiltrate), and Mycobacteria marinum infection (Solitary erythematous nodule or plaque, sometimes ulcerate and appear crusted, a skin smear from the lesion may contain AFB similar in appearance to M. leprae, but the organism can be cultured on suitable media, a skin test using PPD from M. marinum is positive). Other differential diagnoses include parapsoriasis, sarcoidosis, cutaneous leishmaniasis, erythema multiforme. Differential diagnoses of diffuse infiltration of the skin are lymphoma, actinic reticuloid (a severe form of persistent photosensitivity producing erythema, edema and thickness of the skin on the face, neck and hands), systemic sclerosis (patient develops a taut and thickened skin which slowly becomes bound to subcutaneous tissues, recurrent ulcerations develop at the ends of fingers and terminal phalanges become absorbed, polyarthritis of small joints is common and finger contractures may develop), myxedema (has many similarities to lepromatous leprosy, thickening of the skin, thinning of eyebrows, a hoarse voice, edematous leg, normocytic normochromic anemia and carpal tunnel syndrome as a complication), and pachydermoperiostosis (a familial condition

predominantly affecting the males in which the facial appearance with deepening of the lines of face and forehead closely resembles lepromatous leprosy, bone changes take the form of proliferative periostitis, fingers become thickened and there is clubbing of fingers and toes).

Differential Diagnosis of Regional Manifestations Differential diagnosis of madarosis in lepromatous leprosy is lymphoma, follicular mucinosis, hypothyroidism, secondary syphilis and alopecia areata. Differential diagnoses of earlobe infiltration/ nodules in lepromatous leprosy is lupus vulgaris, coccidioidomycosis, chondrodermatitis nodularis helices, lupus erythematosus, sarcoidosis. Differential diagnoses of deformity of nose (cartilage destruction) in lepromatous leprosy, leishmaniasis (cartilage destruction), tertiary yaws, relapsing polychondritis and congenital syphilis (bone destruction).

Differential Diagnosis of Peripheral Nerve Involvement Differential diagnoses of only palpable nerve thickening are excessive muscular development (Localized - great auricular nerve thickening in people carrying heavy weights on the head or Generalized – in professional wrestlers), and pachydermoperiostosis (Generalized thickening of skin, periosteum and bone, there is clubbing and acromegalic features, furrowing due to the thickened skin of forehead, can easily be mistaken for the leonine facies of lepromatous leprosy). Differential diagnoses of palpable nerve thickening with regional anesthesia, with or without muscle wasting are primary amyloidosis of peripheral nerves (Inherited disorder, onset in second or third decades, usually affects lower limbs, with impaired sensations, muscle wasting

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Essentials in Dermatology and dropped foot. Late effects are loss of tendon reflexes and trophic ulceration of feet, associated with autonomic neuropathy leading to orthostatic hypotension, diarrhea, loss of bladder control or impotence. Histological examination discloses amyloid infiltrating the affected nerves), familial hypertrophic interstitial neuritis (Djerine— Sottas neuropathy) (Hereditary motor and sensory neuropathy type 3, autosomal recessive, has onset in childhood or adolescence, characterized by slowly progressive muscular atrophy of limbs, commencing distally with claw hands, dropped foot, anesthesia and loss of tendon reflexes). Differential diagnosis of regional anaesthesia with or without muscle wasting but with palpable nerve thickening in some cases are recurrent or chronic progressive (endotoxic) polyneuritis (an acquired disorder of nerves, cause unknown, symptoms first appear in adult life and tendon reflexes are diminished or absent), and peroneal muscular atrophy (Charcot— Marie—Tooth type) (an inherited disorder, onset in childhood, with muscle weakness in lower limbs, pes cavus, hammers toes and callosities of feet, tendon reflexes of lower limbs are diminished or absent). Differential diagnosis of regional anaesthesia with or without muscle wasting but without palpable nerve thickening are syringomyelia (anesthesia and muscle wasting develop in upper or lower limbs depending on the localization of the cord lesion, there is dissociated anesthesia loss of pain and temperature sensation with preservation of touch, tendon reflexes are diminished or lost, and the histamine test is positive), tabes dorsalis (characterized by dysfunction of posterior nerve roots has triad of symptoms-lightning pain, dysuria and ataxia and triad of signs-Argyll—Robertson pupils, areflexia and loss of proprioception), peripheral neuropathy (a mononeuropathy can result from

compression of nerve or nerve plexus and may simulate pure neural leprosy, e.g., cubital tunnel compression syndrome, carpal tunnel syndrome, cervical rib and meralgia paraesthetica causing sensory changes in one or both thighs or multiple neuropathy which has a large number of causes, some of which like diabetes result in plantar ulceration, depression of histamine flare in anesthetic skin is similar to that in leprosy, but there are no thickened nerves and tendon reflexes are likely to be lost), hereditary sensory radicular neuropathy (Loss of sensation and sweating is most severe in lower limbs, but muscular coordination is normal compared to tabes, chronic painless plantar ulceration is classical, together with high-tone deafness. Loss of ankle jerk is usual and X- ray of feet reveals bone changes similar to those in leprosy) and hysteria.

Differential Diagnosis of Trophic Ulcers Many conditions, which produce sensory motor neuropathy, may also produce trophic ulcers. The following conditions are to be considered like diabetes mellitus, syringomyelia, Moorvan’s syndrome, tabes dorsalis, diastometamyelia, Thevanard syndrome and congenital sensory neuropathy. Differential Diagnosis of Thickening of Peripheral Nerves The following disorders are associated with thickening of peripheral nerves-Déjérine-Sotta’s disease, neurofibromatosis, schwannoma, lipofibromata, Tooth Charcot Hoffmann’s syndrome, Thevanard’s syndrome, syringomyelia, amyloidosis, hereditary motor and sensory neuropathy type 3 and Guillain-Barre’s syndrome. TREATMENT The most important development in the leprosy control in the last millennium has been the

Clinical Leprosy introduction of multidrug therapy (MDT) instead of dapsone monotherapy in the year 1982, following the recommendations of the WHO study group. There has been a dramatic downward revision of estimated number of leprosy patients in the world from 10 to 12 million in the mid 1980s to 0.62 million (point prevalence) in 2001. Although these figures are highly encouraging, the number of new cases detected annually has remained quite stable during the last 15 years. India harbours 65% of the world population of leprosy patients. The total number of registered cases in India has shown a steep decline over the years, from 3.4 million cases in 1986 to 0.266 million cases in 2004. The standard schedule of MDT for leprosy has not undergone any change after its introduction in 1982, except for the temporary introduction of ROM therapy for single skin lesionpaucibacillary (PB) leprosy. However there have been significant changes in the duration of therapy and in the criteria for allocating patients into PB and multibacillary (MB) groups for therapy. At the time of introduction of MDT, in 1982, this allocation was based on the grading of smear positivity, with patients with a bacteriological index (BI) < 2 classified as PB leprosy. In 1988 the WHO expert committee on leprosy recommended that only initially smearnegative patients should be classified as PB. With time, a division based on skin smear examination was not considered suitable in field conditions and a division based on the number of skin lesions gained favour. In the last 22 years, an increasing number of patients who were previously classified as PB for treatment purposes, were allocated to the MB group for treatment purposes, with skin smear examination no longer considered necessary for gouping. Patients with more than 5 skin lesions are now being classified as MB patients, and wherever facilities are available for a slit skin smear examination, all

smear positive patients are included in the MB group. The original recommendation of treating patients till smear negativity meant that the duration of treatment was for more than 2 years for MB patients. This was reduced to a fixed duration of 24 months in 1992, and further shortened to 12 months in 1998. WHO in 2003 has omitted any separate mention of the single lesion PB group or ROM therapy. At present, 12 months fixed duration therapy for MB patients is being followed all over India. From July 1997, the vertical NLEP programme is being integrated into general health services. WHO (2003) presently classifies leprosy into PB leprosy (patients with 1-5 skin lesions, slit skin smear negative) and MB leprosy (patients with more than 5 skin lesions, slit skin smear positive). The Directorate General of Leprosy, Government of India, had accepted the ROM (single dose of ROM-Rifampin 600mg, Ofloxacin 400mg and Minocycline 100 mg) regimen for single lesion PB leprosy and had incorporated it in the National Leprosy Eradication Programme (NLEP) in November 1997. However, they have recommended that patients on ROM should be under surveillance for 2 years after administration of the therapy but this mode of therapy has come under lot of criticism.

WHO/National Leprosy Eradication Programme (NLEP) Recommendations are: • Paucibacillary leprosy-6 months therapy Monthly supervised rifampin (600 mg) and dapsone (100mg) daily • Multibacillary leprosy-12 months therapy Monthly-supervised rifampin (600 mg) and clofazimine (300 mg), dapsone (100 mg) daily and clofazimine (50 mg) daily

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Essentials in Dermatology Laboratory monitoring for drugs used to treat leprosy

Drug

Laboratory studies

Initial studies for all drugs Dapsone Rifampicin Clofazimine

CBC, platelets, urine analysis, blood biochemistry Baseline CBC, reticulocyte count, G6PD only if hemolysis recorded every 6 months CBC, platelets, blood biochemistry every 3 months No recommended laboratory studies

Accompanied MDT (A-MDT) is designed to address a frequent problem in field programs. Patients often have to interrupt their treatment because of a shortage of drugs at the health centre, poor access to the health services or simply because no one is at the health center when they come to collect their treatment. A-MDT means providing patient with full course of treatment on their first visit to the leprosy clinic after diagnosis. On this occasion, the patient and accompanying person also receive information about leprosy in the form of printed material about the disease, its treatment and when and where to come for follow-up or in the event of complications. The term accompanied is adopted because someone close to or important to the patient assumes responsibility for helping the patient to complete the treatment.

Alternative MDT Regime Rifampicin resistant cases or those patients, who developed toxicity to rifampicin- Stop rifampicin, continue two other drugs in MB MDT. In addition, add ofloxacin 400 mg + minocycline 100 mg (or clarithromycin 500 mg) for 6 months, followed by ofloxacin 400 mg or minocycline 100 mg for a period of another 18 months Dapsone resistant cases -Dapsone to be continued with other drugs + add clofazimine in PB cases. Dapsone toxicity cases- In PB cases – stop dapsone and add clofazimine and in MB cases – stop dapsone and continue other two drugs.

Frequency

Clofazamine unacceptable cases(due to pigmentation)- Omit clofazimine from regimen, replace it with ofloxacin 400 mg or minocycline 100 mg can be used as substitute MB patients who were on dapsone monotherapy previously and negative smear-Give an additional course of MB MDT. Leprosy with concomitant HIV infectionRifampicin is contraindicated with protease inhibitors. Dose of rifabutine needs to be reduced to half if indinavir or nelfinavir is given. Leprosy and tuberculosis- Continue standard antituberculous treatment. Rifampicin is given in doses as for tuberculosis and other two antileprosy drugs are to be continued as per regime. Relapse- Treat with minimum two new antileprosy drugs in addition to rifampicin.

New Regimes of Short Duration (Under Trials) 1. MOR: minocycline(daily) + ofloxacin (daily) + rifampicin (monthly) 2. RMM: Rifapentine(monthly) + minocycline (daily) + moxifloxacin(daily) 3. U-MDT: Regimens for paucibacillary (PB) and multibacillary (MB) leprosy patients differ in their duration and components. So Uniform Multi Drug Therapy (U-MDT) with three drug combination of MDT drugs as given for MB cases has been recommended to overcome difficulties in classification which usually come in field conditions. Uniform triple drug

Clinical Leprosy therapy is given with fixed duration of 6 months for PB and 12 months for MB cases. 4. U-MDT with fixed duration therapy of 6 months. 5. Monthly administered ROM for MB and PB leprosy: Once a month ROM for 12 months in MB and 6 months in PB leprosy cases. 6. Fully supervised regimes such as RMM monthly regimes: Rifapentine + minocycline + moxifloxacin (monthly), RCM regimes: Rifampicin + clarithromycin ( 1000mg) + minocycline (200mg) monthly fully supervised dose, Ampicilin/ sulbactum, or Quadruple regime: Rifampicin (600mg) + ofloxacin (400mg) + clofazamine (100mg) + minocyclines (100mg) once weekly for 6 weeks. This regime is gaining importance.

Fig. 36.17: Type 1 lepra reaction- the previously existing skin lesion over the face had become erythematous and edematous

REACTIONS IN LEPROSY Reactions are acute episodes of hypersensitivity reactions due to fluctuations in the immune status of a leprosy patient. There are three types of reactions; type 1 reaction, type 2 reaction and Lucio phenomenon.

Type 1 Lepra Reaction (Reversal Reaction) • It is a type IV hypersensitivity reaction (Coombs and Gell type) • Seen in borderline spectrum (BT, BB, BL) • May manifest in the skin or nerve lesions of leprosy • Erythema, edema and hyperalgesia of existing skin lesions occurs (Fig. 36.17) • Occurrence of new lesions • Rarely ulcerations of skin lesions • Excruciatingly tender and thickened nerves, with sensory or motor deficit • Patient can develop nerve palsies and in severe reactions oedema of the hands and feet (Fig. 36.18)

Fig. 36.18: Type 1 lepra reaction- swelling of hands and arm in severe type 1 lepra reaction

• No constitutional symptoms usually seen • The principles of management of reversal reaction are the following: 1. Continued maintenance of ALT. 2. Prolonged anti-inflammatory therapy. 3. Adequate analgesia. 4. Physical support.

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Essentials in Dermatology • Most important aspect of type 1 lepra reaction is not the skin lesions but the condition of peripheral nerves • Mild reaction can be managed with analgesics • Severe reaction and neuritis needs oral steroids, commonly prednisolone is used in the dose of 30 to 40 mg per day. • Other drugs used are cyclosporin, azathioprine, clofazamine, intraneural injections of lignocaine • Management of nerve abscess by aspiration or neurolysis.

Type 2 Lepra Reaction • It is a type III or immune complex mediated lepra reaction • Occurs in lepromatous spectrum (most commonly in LL, occasionally in BL) • Triggered by infection, vaccination, drugs and stress • Characterized by tender, erythematous, evanescent nodules called as erythema nodosum leprosum (ENL) (Figs 36.19 and 36.20) • ENL occurs over the face and extensors • ENL does not occur in legs in contrast to erythema nodosum which occurs in legs • Constitutional symptoms such as fever, arthralgia, anorexia and myalgia occur • May have systemic involvement in the form of conjunctivitis, keratitis, iritis, iridocyclitis, orchitis, hepatomegaly and lymphadenopathy • The principles of treatment include. – Identifying and treating precipitating factors. – Continuation or start of ALT. – Treatment of reaction. – Identification and management of complications. • Primary drug therapy is with analgesics and corticosteroids. Thalidomide is very effective for both neuritis and pain in type 2 lepra reaction and works faster than corticosteroids; however it should be given under supervision.

Fig. 36.19: Type 2 lepra reaction- back showing erythematous papular and nodular, tender lesions in a case of lepromatous leprosy

Fig. 36.20: Pustular erythema nodosum leprosum

Other drugs used are clofazimine (up to 300 mg per day), pentoxifylline, levamisole, cyclosporin, azathioprine, cyclophosphamide and zinc.

Lucio Phenomenon • Occurs in Lucio leprosy due to bacterial endotoxins, precipitated by stress and strain • Characterized by painful, large, bizarre ulceration of skin and is not accompanied by systemic features (Fig. 36.21).

Clinical Leprosy • The lesions occur over legs, thighs and buttock • Starting of antileprosy treatment • Steroids and anti-inflammatory agents are main stay of therapy.

COMPLICATIONS Leprosy has been regarded as a dreaded and stigmatizing disease, which can lead to gross disabilities, deformities and mutilations.

• Nerve palsies: due to silent neuritis or reactions-ulnar claw hand (Fig. 36.22), median nerve palsy, facial palsy (Fig. 36.23), foot drop and claw toes. • Anesthetic complications: trophic ulcer (Fig. 36.24) leads to osteomyelitis, distortion of toes and shortening of hands and feet (Figs 36.25 and 36.26), tarsal disintegration and malignancy.

Fig. 36.21: Lucio phenomenon- angular hemorrhagic infarcts over the lower limb

Fig. 36.23: Complications- left-sided facial nerve palsy resulting in inability to close the eye

Fig. 36.22: Complications- ulnar claw hand due to ulnar nerve damage in borderline tuberculoid leprosy

Fig. 36.24: Complications- trophic ulcer on the sole

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Fig. 36.25

Fig. 36.26

Figs. 36.25 and 36.26: Complications- shortening of digits of hands and feet in lepromatous leprosy

OCULAR LEPROSY It is an important cause of preventable blindness • Due to direct leprous involvement: madarosis, conjunctivitis, superficial punctate keratitis,

pannus, corneal ulcer, iris pearls, chronic plastic iridocyclitis • Due to type 2 lepra reaction: iritis, iridocyclitis, episcleritis • Trigeminal nerve and facial nerve involvement also can cause ocular complications.

Multiple Choice Questions

Multiple Choice Questions Introduction with Tips for MCQs Entrance Examinations for Post- graduation courses in Medical Schools in India All India Entrance Examinations for admissions to Post-graduate Medical Studies (MS/MS/ Diploma) is conducted by All India Institute of Medical Sciences (AIIMS), New Delhi • As per Supreme Court Directives, All India Entrance Examinations for admissions to Post Graduate Medical Studies (MS/MS/Diploma) is conducted by All India Institute of Medical Sciences (AIIMS) New Delhi for 25% seats of all Government and Municipal Medical Colleges all over India and recognized by Medical Council of India. (MCI). • However, the examination does not cover the states of Andhra Pradesh and Jammu and Kashmir. The number of seats finalized through the entrance is approximately 1,800. Examination is held every year on first Sunday of January. • There will be one paper of 3 and 1/2 hours duration containing 300 Multiple Choice Questions (MCQs) from pre-clinical, paraclinical and clinical subjects. • All questions will be of objective type. Each answer with correct response shall be awarded

four marks. Negative marking will be adopted for incorrect responses. One mark will be deducted for each negative response. Zero mark will be given for the question not answered. More than one answer indicated against a question will be deemed as incorrect response and will be negatively marked. • The competitive entrance examination shall be of the standard of MBBS examination and shall cover all the subjects of the M.B.B.S course.

At PGIMER Entrance Examination 250 MCQ’s with five alternatives each and single/ multiple correct response are to be solved in 3 hrs. Negative marks will be given for incorrect responses marked correct.

At JIPMER Pre PG Entrance Examination • There will be one paper of 3 hours duration containing 250 Multiple Choice Questions (MCQs). 100 from pre/para clinical and 150 from clinical subjects. Exam usually occurs on 3rd Sunday of March every year.

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Essentials in Dermatology • All questions will be of objective type. Each answer with correct response shall be awarded one mark. There is negative marking. More than one answer indicated against a question will be deemed as incorrect response

MAHE Pre PG Entrance Examination • There will be one paper of 3 hours duration containing 200 Multiple Choice Questions (MCQs) of pre-clinical/para-clinical/clinical subjects and is online examination. • All questions will be of objective type. Each answer with correct response shall be awarded three marks. Negative marking will be adopted for incorrect responses. One mark will be deducted for each negative response. Zero mark will be given for the question not answered. More than one answer indicated against a question will be deemed as incorrect response and will be negatively marked. THE MULTIPLE-CHOICE QUESTIONNAIRE (MCQ) Why MCQs? The multiple-choice questionnaire (MCQ) has become one of the standard assessment methods in most medical schools over the past 30 years. MCQs were developed to address problems associated with traditional essay style testing. They are seen as a more objective test of students’ knowledge than essays. MCQs have clear right and wrong answers and so are free of any interpretation bias. They are generally regarded as being reliable and thus consistent and valid tests of students’ knowledge and information recall. Advantage of MCQs MCQs have many advantages for teachers. Once designed they are easy to administer and mark. Most MCQs are answered on a card, which can be read by a computer coupled with an optical card reader. So the whole process can be

automated leaving teachers free to do other things. The scoring is done by the computer, which also reports on the reliability and discriminatory power of the MCQ questions. So it seems that the MCQ is an assessment design that you are likely to encounter many times during your undergraduate and postgraduate career.

Disadvantage of MCQs It is important to note, however, what MCQs cannot do. They cannot measure clinical performance or competence. They can occasionally be used to test components of diagnostic reasoning and problem solving. MCQs have also been criticized for providing candidates with visual “clues” that suggest a correct answer, one that the candidate might not otherwise have recalled. For some courses in JIPMER, Pondicherry, they are combined with other test formats that test different aspects of candidates’ performance - for example, objective structured clinical examinations, short answer questions, etc. Marking Schemes for MCQs There are two types of marking commonly encountered in MCQs. These are “negative marking” where marks are deducted for incorrect answers and “neutral marking” where all questions should be attempted - marks are given for correct answers only and guessing is encouraged. Negative marking was introduced to reduce the amount of guessing by candidates by “punishing” incorrect responses with a negative mark. STRATEGIES IN ATTEMPTING MULTIPLE-CHOICE QUESTIONS Strategy before the test 1. Try to gather as many examples as you can of old papers and previous examples of MCQs used by the department or school in question in the past.

Multiple Choice Questions 2. Solve last year papers as 30-40% questions are repeated. At the time of final revision by solving mock test (like solve and send series) helps a lot. 3. Do not, however, try to memorize hundreds of responses to questions. The factual knowledge you will gain will be superficial and dissociated. It is better to look for the topic areas that recur frequently and ensure that you have a deeper knowledge of these topics. 4. Revise with friends and colleagues. You can share knowledge and techniques. 5. “Practice makes a man perfect”, is true for any competitive exam. Learning by heart helps very little, but if it is done with understanding and clarity in concepts followed by writing it out helps much better. It prepares you to perform in exams confidently and intelligently. 6. Practice is the key to success and attempting sufficient number of mock tests before the actual exams will help you improve your presentation and scoring skills. 7. It helps you to improve your speed; thereby you can complete your papers on time. 8. You never get a second chance to improve yourself or rectify your mistakes. Once done is final, something on which your whole future is dependent. Mock tests will help you realize your silly mistakes and thereby eliminate them before the actual exams. 9. You will get thoroughly conversant with the style and standard of entrance exam questions papers and assessment of answer papers. 10. Writing a number of mock tests shall relieve you of examination fear and develop tremendous confidence to score higher ranks. 11. You should know what type of MCQ is being set for you. Will there be negative marking? How much time will you have and how many questions will there be?

Strategies During the Test 1. Check that your understanding of the MCQ format is correct. “It is negative marking, there are 300 questions, and I have two hours to complete this.” Always read the stem for each question carefully. Have you understood the question? Are there any ambiguities? If so ask an invigilator who will alert an examiner. There are usually one or two in the room. Allocate three quarters of the time to answering the questions and a period at the end to checking answers and accuracy. 2. Preview the test: Read through the paper briefly to acquaint yourself with the task ahead. Make sure your copy has no missing or duplicate pages. Read the directions carefully. 3. Start with questions that you can readily answer: To build your confidence and to save time for the harder ones. When you identify a correct response carefully mark this on the question paper. If you are unable to make a choice and need to spend more time with the question, or you answered the question but are not at all sure that you made the correct choice, put a big question mark beside that question, and move on to the next. Avoid getting bogged down on one question. It is much better to move on and finish all those questions that you can answer and then come back later to process the problematic questions. Sometimes the answer will occur to you simply because you are more relaxed after having answered other questions. 4. Read each question carefully: Multiplechoice tests also examine your ability to read carefully and thoughtfully, as much as they test your ability to recall and reason. 5. Identify key words: Circle or underline key words, such as “except”, “true”, “all,” “always,” “never,” “none,” “not,” “few,” “many,” some,” and “sometimes.”

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Essentials in Dermatology 6. Identify subject area: Identifying what lecture, reading, or laboratory exercise the question is from which might help you narrow the choice of possible responses. 7. Identify what is being asked: Answer each question as teacher intended, that is, within the context of the course material that was taught. 8. The “cover up” strategy: Some students find it helpful to read the question and try to recall the answer from memory before looking at each of the four responses. 9. The “true/false” strategy: Identify if the question is looking for a true or false statement. Then label each of the four responses as “true” or “false” and eliminate those that do not correctly complete the question. 10. Read each of the four responses: And do not just stop when you come upon the one that seems likely. 11. Do not select a response just because you remember learning the information in the course; it may be a “true” statement in its own right, but not the best answer to the question. 12. Do not dismiss a response because it seems too obvious and simple an answer; if you are well prepared for the test, some of the questions may appear very straightforward. 13. Do not be persuaded by fancy terms in the question; do not say to you, “That sounds impressive, so it must be the right answer.” As you read through the possible responses, mark off the ones you know are wrong. This will save time if you have to come back to the question later.

14. Should I change an answer? : Change answers only if you have a good reason for doing so. (Changing your answer from response “b” because you selected “b” to the previous two questions is not a good reason.). The origin of the myth that students most often change correct answers to wrong answers is probably that it is the wrong answers that students remember most when reviewing the test (for you are less likely to remember the answers you changed from “wrong” to “right”). 15. If two responses appear to be equally correct: Eliminate the response that appears to be least related to the question being asked. Remember, you are looking for the best answer, not only a correct one. Some responses may be correct but are not directly related to the question. 16. If you are not certain of an answer, do not guess wildly: There is a stiff penalty for wrong answers (negative marking). Eliminate the responses you know are incorrect. Narrow down your selection to two responses and then compare them and identify how they differ. Finally, make an informed, intelligent guess. Do not make any wild guess, as they will do you more harm than good. 17. If the MCQ has a “neutral” marking design you should answer all questions. You will not be penalized for guessing, so have a go. You cannot score if you leave answers blank. 18. It is generally advised that you do not guess in situations where you “have not a clue “ and there is negative marking. The half and half rule applies both ways. You may improve your position; you could also worsen it considerably.


MCQs in Dermatology, Sexually Transmitted Diseases and Leprosy for MBBS Students for PG Entrance Examination

Set I (1-400) 1. A patient is said to have AIDS when the CD-4 count is A. 200 to 499 B. <200 C. <100 D. <50 2. About autosomal dominant inheritance all are true except A. It is expressed in homozygotes B. Always one parent is affected C. 50% of children affected D. Offspring of a non-diseased child of a diseased parent will not have the mutant gene. 3. Harlequin skin changes are associated with A. It is a normal phenomenon B. Seen in ichthyosis C. Septicemia D. Atopic dermatitis

B. Systemic griseofulvin C. Griseofulvin topically D. Amphotericin 7. Grayish white vaginal discharge with formation of bubbles is a feature of A. Trichomoniasis B. Bacterial vaginosis C. Candida albicans D. Gonorrhea 8. The most effective means of transfer of HIV is A. Sexual route B. Perinatal route C. Blood transfusion D. Needle prick 9. Myiasis means A. Infestation with Maggots B. Infestation with fungi C. Infestation with Bacillus anthracis D. Infection with Bartonella hensalae

4. Norwegian scabies usually affects A. Elderly B. Children C. Immunocompromised D. Infants

10. Chancroid is caused by A. Treponema pallidum B. Hemophilus ducreyi C. Calymmatobacterium granulomatis D. Chlamydia trachomatis

5. A 67-year-old patient presents with complaints of a pruritic red lesion on his forearm, which is having silvery white scales on a red base. The probable diagnosis is A. Psoriasis B. Leprosy C. Lichen planus D. Pityriasis alba

11. Which of the following fungi is most difficult to be cultured in vitro? A. Microsporum canis B. Rhinosporidium seeberi C. Sporothrix schenkii D. Coccidioides immitis

6. Which of the following is the treatment of choice for tinea unguium? A. Miconazole

12. Which of the following is NOT seen in acute intermittent porphyria? A. Abdominal pain B. Neuropsychiatric manifestations C. Dermatological manifestations D. Passing dark coloured urine

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Essentials in Dermatology 13. All of the following are used in urticaria except A. Antihistamines B. Topical glucocorticoids C. Cyproheptadine D. Hydroxyzine 14. Epstein Barr virus causes all of the following diseases except A. Burkitt’s lymphoma B. Nasopharyngeal carcinoma C. Hodgkin’s lymphoma D. Kaposi’s sarcoma

C. Pigment deposition occurs D. Blanches on pressure 20. Erysipelas is caused by A. Streptococci B. Staphylococcus aureus C. Hemophilus influenzae D. Bacillus anthracis 21. Tzanck test is done in infection with A. Herpes simplex B. Cytomegalovirus C. Epstein Barr virus D. Human papillomavirus

15. Oral candidiasis is seen in all of the following diseases except A. Pregnancy B. Diabetes mellitus C. Hypertension D. Immunodeficiency

22. Which of the following is true about rhinophyma A. Seen in tuberculosis B. Caused by K. scleromatis C. Seen in Rhinosporidiosis D. Seen in Rosacea

16. Topical preparation of Vitamin D (Calcipotriol) is used in which of the following skin conditions? A. Psoriasis B. Pityriasis rosea C. Lichen planus D. Lichen nitidus

23. Differentiation between multibacillary and paucibacillary leprosy is based on A. Morphological index B. Bacteriological index and skin lesions C. Lepromin test D. Skin lesions and lepromin

17. Incubation period for measles is: A. 2 – 7 days B. 10 – 14 days C. 14 – 28 days D. 3 – 4 weeks 18. According to CDC/ WHO criteria for AIDS, which of the following cannot be considered as a criterion? A. Cryptococcosis B. Oral candidiasis C. Kaposi’s sarcoma D. Disseminated Mycobacterium avium complex infection 19. Which is NOT true about purpura? A. Size more than 3 mm B. Bleeding into the dermis

24. Most common cause of pelvic inflammatory disease is A. Chlamydia trachomatis B. Tuberculosis C. Gonococci D. Group B streptococci 25. Golden yellow crust is seen in A. Impetigo B. Ecthyma C. Malignant pustule D. Pseudomonas infection 26. A neonate suspected to have congenital syphilis, diagnostic test to be done is A. FTA-ABS IgM B. FTA-ABS IgG C. TPI D. TPHA

Multiple Choice Questions 27. A child presents with seborrheic dermatitis, lytic skull lesions, ear discharge, hepatosplenomegaly; likely diagnosis A. Leukemia B. Lymphoma C. Histiocytosis X (Langerhans cell histiocytosis) D. Leiner’s disease 28. If urine sample darkens on standing, most likely condition is A. Phenylketonuria B. Alkaptonuria C. Mapple syrup urine disease D. Homocystinuria

33. A girl aged 19 with arthritis, alopecia, photosensitive rash on the cheek; diagnosis A. SLE B. DLE C. Dermatomyositis D. Systemic sclerosis 34. A child with itchy lesions of scabies over the groin and the genitalia. What will not to be advised? A. Bathe and apply scabicidal B. Treat family C. Burn clothes D. Antibiotics

29. All of the following are true regarding Ureaplasma urealyticum except A. Causes non gonococcal urethritis B. Associated with infertility C. Bacterial vaginosis D. Epididymitis

35. A man aged 19 years gets painless penile ulcer 10 days after sexual intercourse with a professional sex worker. Diagnosis is A. Chancroid B. Herpes genitalis C. Primary chancre D. Traumatic ulcer

30. A male patient, 20 years old, from Jaipur, has erythematous lesion with central crusting, on cheek; diagnosis A. SLE B. Lupus vulgaris C. Chilblains D. Cutaneous leishmaniasis

36. A boy aged 8 years from TN has a white non anesthetic non-scaly hypopigmented macule on his face A. Pityriasis alba B. Pityriasis versicolor C. Indeterminate leprosy D. Polymorphous light eruption

31. A child aged 3 years, has alopecia, boggy scalp swelling; hair easily pulled out; diagnostic test A. KOH examination B. Culture sensitivity C. Biopsy D. Gram’s staining

37. The most common organism causing Tinea capitis A. Trichophyton violaceum B. Microsporum canis C. Epidermophyton floccosum D. Candida albicans

32. A girl aged 19 years has light brown pigmentation over the malar eminences; diagnosis A. Chloasma B. SLE C. Photodermatitis D. Tinea versicolor

38. Which of the following is not effective in Tinea versicolor? A. Clotrimazole B. Ketoconazole C. Griseofulvin D. Selenium sulphide 39. The duration of therapy with Griseofulvin for tinea unguium is A. 6-9 months B. 4 months C. 2 months D. 1 month

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Essentials in Dermatology 40. Genital elephantiasis is seen in A. Donovanosis B. Lymphogranuloma venereum C. Herpes simplex D. Syphilis 41. A patient, Gopi, had lepromatous leprosy. Which of the following is true regarding globi in leprosy? A. Consists of lipid laden macrophages B. Consists of macrophages filled with bacteria C. Consists of neutrophils filled with bacteria D. Degenerated neural tissue 42. All the following are sexually transmitted infections except A. Candida B. Group B streptococcus C. Hepatitis B D. Echinococcus 43. Exfoliative dermatitis is seen in all the following except A. Pityriasis rosea B. Pityriasis rubra pilaris C. Psoriasis D. Drug reaction 44. In a patient, annular erythematous lesions on the trunk were seen circumscribed by collarettes scales. The diagnosis is most likely to be A. Pityriasis versicolor B. Pityriasis rosea C. Pityriasis rubra pilaris D. Lichen planus 45. Wickham’s striae are seen in A. Lichen sclerosis et atrophicus B. Lichen planus C. Lichen aureus D. Lichen nitidus 46. A patient with psoriasis was started on systemic steroids. After stopping treatment,

the patient developed generalized pustules all over his body. The cause is most likely to be A. Drug induced reaction B. Pustular psoriasis C. Bacterial infections D. Septicemia 47. A 6-month-old infant develops high-grade fever, which subsided after 3 days following which there was appearance of a generalized rash, which too subsided after 48 hours without any residual pigmentation. Which of the following is the most likely diagnosis? A. Fifth disease B. Roseola infantum C. Measles D. Rubella 48. A patient, Bindu, presents with vaginal discharge. Examination of the discharge reveals the presence of Chlamydial infection. The treatment of choice is A. Azithromycin + contact tracing B. Doxycycline + Metronidazole C. Fluconazole + Doxycycline D. Metronidazole 49. The most bactericidal drug in leprosy is A. Rifampicin B. Dapsone C. Clofazimine D. Ofloxacin 50. Steroids cause all the following except A. Hypertrophy of muscle B. Hypokalemia C. Neuropsychiatric symptoms D. Hyperglycemia 51. The treatment of pustular psoriasis is A. Retinoids B. Thalidomide C. Steroids D. Hydroxyurea 52. Pruritic ring-like lesion beneath the breast is likely to be A. Pityriasis rubra pilaris B. Trichophytosis C. Lichen planus D. Bacterial infections

Multiple Choice Questions 53. Wood’s lamp examination is helpful in the diagnosis of A. Tinea versicolor B. Psoriasis C. Lichen planus D. Varicella 54. A patient has scaly lesions on the elbow and knee joint. Which of the following is a bedside test for provisional diagnosis of the illness A. Auspitz’s sign B. KOH preparation C. Tzanck smear D. Slit skin smear 55. A male patient presents with multiple nodulocystic lesions on the face with multiple sinuses. The treatment of choice is A. Antibiotics B. Retinoids C. Antituberculous drugs D. Steroids 56. Granules coming out of the multiple discharging sinuses in mycetoma foot contains A. Fungal colonies B. Pus cells C. Inflammatory cells D. Granuloma 57. Babloo, a 4-year-old boy, presents with history of seizures. On examination there is hypopigmented patches on his face. There is mental retardation also. What is the most likely diagnosis? A. Neurofibromatosis B. Tuberous sclerosis C. Sturge Weber syndrome D. Incontinentia pigmenti 58. ANCA is not associated with which of the following diseases A. Wegener’s granulomatosis B. Henoch-Schönlein purpura C. Microscopic PAN D. Churg Strauss syndrome

59. A child presents with a solitary white welldefined patch of his right thigh. What is the diagnosis A. Piebaldism B. Nevus achromicus C. Acral vitiligo D. Albinism 60. A patient gets recurrent urticaria while doing exercising and on exposure to sunlight. Which of the following is the most likely cause A. Chronic idiopathic urticaria B. Universal dermographism C. Cholinergic urticaria D. Solar urticaria 61. Vasanti, a 28-year-old female, presents with complaints of tightness of fingers. There is also history of dysphagia. Which of the following is the probable diagnosis? A. Dermatomyositis B. Scleroderma C. Rheumatoid arthritis D. Systemic lupus erythematosus 62. A lady approaches a physician for contraceptive advice. On examination there were two symmetrical painless ulcers on vulva, which were well defined with a firm base. Which of the following is the most likely cause A. Primary syphilis B. Chancroid C. Herpes genitalis D. Malignancy 63. A girl presents with complaints of melena. On examination there are pigmented lesions involving her mouth and lips. Two of her sisters also had similar complaints. Which of the following is the most probable diagnosis? A. Cronkhite-Canada syndrome B. Peutz Jegher’s syndrome C. Gardner’s syndrome D. Behçet’s syndrome

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Essentials in Dermatology 64. A 30-year-old Basanti presents with light brown lesions involving both her cheeks. The lesions had never been erythematous. Which of the following is the most probable diagnosis A. SLE B. Chloasma C. Air borne Contact Dermatitis D. Photodermatitis 65. Babloo, 5 years old male, presents with small hypopigmented scaly macules on his cheek. Some of his classmates also have similar lesions. Which of the following is the most probable diagnosis A. Pityriasis alba B. Pityriasis rosea C. Pityriasis versicolor D. Indeterminate leprosy 66. Kallu, a 30-year-old man, presented with subcutaneous itchy nodules over the left iliac crest. On examination, they are firm, non-tender and mobile. Skin snips contain microfilaria and adult worms of A. Loa Loa B. Onchocerca volvulus C. Brugia malayi D. Mansonella perstans 67. All are true about Neisseria gonorrhea except A. Gram positive cocci B. Causes stricture urethra C. Involves seminal vesicles and spreads to epididymis D. Drug of choice is Ceftriaxone 68. A 30-year-old male, Kallu, with a history of sexual exposure comes with a painless granulomatous ulcer over the penis with everted margins. The diagnosis is A. Primary chancre B. Chancroid C. Lymphogranuloma venereum D. Donovanosis

69. All are features of peripheral neuritis in a patient with Hansen’s disease except A. Predominant sensory involvement B. Decreased tendon reflexes C. Mutilations D. Muscle paralysis 70. Vitamin D is synthesized in A. Stratum basale B. Stratum spinosum C. Stratum granulosum D. Stratum corneum 71. Tram track appearance on CT scan of the head is seen in A. Sturge Weber syndrome B. von Hippel Lindau disease C. Tuberous sclerosis D. Neurofibromatosis 72. A 40-year-old farmer with a history of recurrent attacks of pain abdomen, complains of itching when exposed to the sun and maculopapular rash on sunexposed areas. His symptoms are exaggerated in the summer. The diagnosis is A. Congenital erythropoietic porphyria B. Acute intermittent porphyria C. Variegate porphyria D. Porphyria cutanea tarda 73. An 8-year-old boy presents with a welldefined annular lesion over the buttock with central scarring that is gradually progressing over the last 8 months. The diagnosis is A. Annular psoriasis B. Lupus vulgaris C. Tinea corporis D. Annular lichen planus 74. An adult presents with oval scaly hypopigmented macules over the chest and the back. The diagnosis is A. Leprosy B. Lupus vulgaris C. Pityriasis versicolor D. Lichen planus

Multiple Choice Questions 75. The characteristic nail finding in lichen planus is A. Pitting B. Pterygium C. Beau’s lines D. Hyperpigmentation of the nails 76. In an 8-day-old child with no history of consanguinity in the parents, the mother reports blisters and peeling off of the skin at the site of handling and pressure. There was a similar history in the previous child, which proved to be fatal. The diagnosis is A. Bullous pemphigoid B. Congenital syphilis C. Epidermolysis bullosa D. Letterer-Siwe disease 77. An 8-year-old female child following URTI developed maculopapular rash on the face spreading onto the trunk which cleared on the 3rd day without desquamation and tender post auricular and suboccipital lymphadenopathy. The diagnosis is A. Measles B. Rubella C. Erythema infectiosum D. Kawasaki disease (Mucocutaneous LN Syndrome) 78. A patient presented with scarring alopecia, thinned nails, hyperpigmented macular lesions over the trunk and oral mucosal lesions. The diagnosis is most likely to be A. Lichen planus B. Psoriasis C. Secondary syphilis D. Scleroderma 79. A young boy presented with a lesion over his right buttock, which had peripheral scaling and central clearing with scarring. The investigation of choice would be A. Tzanck smear B. KOH preparation C. Biopsy D. Culture on Sabouraud’s agar

80. Acne vulgaris is due to involvement of A. Sebaceous glands B. Eccrine glands C. Hair follicles D. Apocrine glands 81. The term tuberculids includes all except A. Lichen scrofulosorum B. Lichen nitidus C. Erythema nodosum D. Lupus miliaris 82. According to the WHO revised criteria, all are true regarding the treatment of leprosy except A. All multibacillary cases should be treated for 12 months with multi drug therapy B. Treatment of paucibacillary leprosy involves Rifampicin 600 mg once a month and Dapsone 100 mg daily for 6 months C. Treatment of multibacillary leprosy involves Rifampicin 600 mg once a month, clofazimine 300 mg once a month and 50 mg OD and Dapsone 100mg OD for 6 months D. Treatment of multibacillary leprosy involves Rifampicin 600 mg once a month, clofazimine 300mg once a month and 50 mg OD and Dapsone 100mg OD for 12 months 83. Regarding Neurofibromatosis Type I, following are true except A. Lisch nodules are seen B. Multiple cafe-au-lait spots are seen C. Plexiform neurofibroma is associated D. Posterior capsular lenticular opacity is seen 84. Eczema herpeticum is associated with infection due to A. Herpes simplex B. Varicella C. HHV 8 D. Parvo virus

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Essentials in Dermatology 85. Tinea capitis (Endothrix) is most commonly caused by A. T.mentagrophytes B. E.floccosum C. T.tonsurans D. T.violaceum 86. Viable but not cultivable state is seen in which of the following organisms A. M leprae B. M. tuberculosis C. V. cholera D. Staph. aureus 87. Regarding Henoch Schonlein purpura, all are true except A. Associated with glomerulonephritis B. Palpable purpura C. Decreased complement D. Thrombocytopenia 88. Dyskeratosis is a feature of A. Darier’s disease B. Pemphigus vulgaris C. Psoriasis D. Lichen planus 89. Regarding AIDS all are true except A. Male to female transmission is more common than vice versa B. Condom provides 100% protection C. Kissing is considered to be safe D. Risk of transmission is more in the presence of associated STDs 90. Staphylococcus aureus classically causes A. Impetigo contagiosa B. Furuncle C. Ecthyma D. Blistering dactylitis 91. Which of the following is/are associated with Swimming pool granuloma? A. M. marinum B. M. avium complex C. M. ulcerans D. M. Chelonae

92. All are AFB stain positive except A. M. leprae B. M. tuberculosis C. Nocardia D. M. mycetomatis 93. Lyme’s disease is caused by A. Borrelia recurrentis B. Fusobacteria C. Spirochete D. Borrelia burgdorferi 94. All are true about rhinophyma except A. Fungal infection B. Acne rosacea C. Dermabrasion D. Electrosurgery 95. A child presents with slapped cheek appearance of face, diagnosis is A. Pityriasis alba B. Tinea versicolor C. Pityriasis rosacea D. Erythema infectiosum 96. Which of the following is not a feature of rhinosporidiosis? A. Bleeding polyp B. Russell bodies are seen C. Oral dapsone is useful in treatment D. Chiefly involves eyes 97. Which of the following is the commonest cause of urethritis with leukorrhea? A. Chlamydia trachomatis B. Gardnerella vaginalis C. Trichomonas vaginalis D. Candida albicans 98. Munro’s micro abscesses is seen in A. Stratum corneum B. Stratum basale C. Dermis D. Stratum spinosum 99. The drug of choice if available, for type 2 lepra reaction is A. Clofazimine B. Chloroquine C. Thalidomide D. Steroids

Multiple Choice Questions 100. A 6 months child, has developed irritability, photophobia, diarrhoea and hair loss of 2 months duration, following weaning from breast milk. The child has an eczematous rash around the perioral and perianal area. His ALP is 500 IU/L, Hb - 8.0 gm%, SGOT- 15 IU/L and SGPT- 20 IU/L. What is your diagnosis? A. Acrodermatitis enteropathica B. Selenium deficiency C. Protein energy malnutrition D. Biotin deficiency 101. Which of the following is not a pyoderma? A. Furuncle B. Ecthyma C. Pyoderma gangrenosum D. Carbuncle 102. Treatment of choice for disseminated gonococcal infection is A. Penicillin B. Ceftriaxone C. Doxycycline D. Erythromycin 103. Scabies in children differs from that in adults in that it affects A. Finger web spaces B. Face C. Genitalia D. Axilla 104. Swollen cherry red lip with strawberry tongue is a feature of A. Kawasaki disease B. Scarlet fever C. Toxic shock syndrome D. Staphylococcal scalded skin syndrome

C. Trichomonas vaginalis D. Leishmania brasiliensis 107. A smear from a genital lesion shows Donovan bodies. The diagnosis is A. Lymphogranuloma venereum B. Chancroid C. Granuloma inguinale D. Primary chancre 108. The minimum period of treatment for multi-bacillary leprosy is A. 1 year B. 2 years C. 6 months D. 9 months 109. Refsum’s disease is due to defect in enzyme A. Phytanic acid oxidase B. Nicotinamide-adenine dinucleotide oxidoreductase. C. Homogentisic acid oxidase D. Tyrosinase 110. Vesicular lesion is seen in A. Primary syphilis B. Secondary syphilis C. Tertiary syphilis D. Congenital syphilis 111. A patient comes with recurrent abdominal pain, and urinary examination reveals Ehrlich aldehyde test (+) for urobilinogen. The diagnosis is A. Porphyria B. Renal calculi C. Cholelithiasis D. Angina pectoris

105. Maculae cerulea is seen in A. Pediculosis corporis B. Pediculosis capitis C. Phthirus pubis D. None of the above

112. In urticaria pigmentosa, the cell most commonly seen in skin on microscopy is A. Mast cell B. Langerhans cell C. Langhan’s giant cell D. Fibroblasts

106. Which of the following is not sexually transmitted? A. Entamoeba histolytica B. Giardia lamblia

113. An elderly male with dry skin and fissuring scaly skin is likely to have A. Nummular eczema B. Venous eczema

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Essentials in Dermatology C. Asteatotic eczema D. Seborrheic dermatitis

C. Can cause arthritis D. It commonly occurs in flexural areas

114. Thimble pitting in nails is seen in A. Psoriasis B. Tinea versicolor C. Lichen planus D. Tuberculosis

121. Koebner phenomenon is seen in A. Erythema marginatum B. Pemphigus C. Lichen planus D. Pityriasis Rosea

115. Ridley-Jopling classification includes all except A. Lepromatous leprosy B. Borderline borderline leprosy C. Tuberculoid leprosy D. Neuritic leprosy

122. The following statements are true about gonococci except A. It causes vulvovaginitis in young girls B. Causes interstitial pneumonia in new born C. Causes non specific urethritis D. Causes endocervicitis

116. Which one of the following is due to type IV hypersensitivity A. Irritant dermatitis B. House wife dermatitis C. Air borne contact dermatitis D. Neurodermatitis

123. In a patient 28 years old presenting with infertility and recurrent history of PID with lower pelvic pain must be investigated for which of the following infections? A. Gonococci B. HIV C. Tuberculosis D. Chlamydia

117. Hidradenitis suppurativa is due to infection of A. Apocrine sweat glands of axilla B. Subcutaneous tissues C. Sebaceous glands D. Eccrine sweat glands

124. Tzanck test is used in the diagnosis of A. Epidermolysis bullosa B. Bullous impetigo C. Pemphigus vulgaris D. Tinea corporis

118. Podophyllin is used in the treatment of A. Plantar wart B. Plane wart C. Condyloma lata D. Condyloma acuminata

125. Pseudobubo is seen in A. Granuloma inguinale B. Lymphogranuloma venereum C. Chancroid D. Syphilis

119. Type 1 lepra reaction is seen in all except A. Tuberculoid leprosy B. Borderline tuberculoid leprosy C. Borderline borderline leprosy D. Borderline lepromatous leprosy

126. Apple-jelly nodules are seen A. Tuberculosis verrucosa cutis B. Scrofuloderma C. Tuberculous gumma D. Lupus vulgaris

120. All the following are true about psoriasis except A. Scalp is commonly involved B. Nails are involved

127. A 3-year-old child was brought by his mother with complaints of a rash on the scalp, which was not responding to therapy. Cutaneous examination showed

Multiple Choice Questions few scaly purpuric papules over trunk and greasy scaling over scalp. The child also had loosening of teeth. Biopsy of the papule showed large cells with reniform nuclei. Skiagram of skull showed a few lytic lesions. What is your diagnosis? A. Histiocytosis B. Neuroblastoma C. Seborrheic dermatitis D. Gaucher’s disease 128. Frei skin test is used in the diagnosis of A. Lymphogranuloma venereum B. Sarcoidosis C. Leishmaniasis D. Granuloma venereum 129. Strawberry vaginitis is seen in infection with A. Gonococcus B. Trichomonas vaginalis C. Hemophilus vaginalis D. Candida albicans 130. Hemophilus ducreyi is the causative agent of A. Hard chancre B. Urethritis C. Soft sore D. Granuloma inguinale 131. Commonest type of basal cell carcinoma is A. Noduloulcerative type B. Superficial spreading type C. Morphoeic type D. Pigmented type

C. Staphylococci D. Pneumococci 134. Endotoxins are implicated in all except A. Psoriasis B. Kawasaki disease C. Atopic dermatitis D. Seborrheic dermatitis 135. Skin lesions caused by Staphylococcus aureus include all except: A. Bullous impetigo B. Staphylococcal scalded skin syndrome C. Scarlet fever D. Furuncle 136. Ecthyma gangrenosum is caused by A. Staphylococcus aureus B. Pseudomonas aeruginosa C. Streptococcal infection D. All of the above 137. The most common site of carbuncle A. Nape of neck B. Thigh C. Face D. Legs 138. Botryomycosis is due to A. Bacteria B. Fungi C. Protozoa D. Algae 139. Toxic shock syndrome; all are true except A. Exotoxins – producing strain of S.aureus B. Seen in menstruating women C. Fever, hypotension and rash are seen D. Pastias lines are present

132. Chronic paronychia is caused by A. Mycobacterium tuberculosis B. Improper treatment of acute paronychia C. Fungal infection D. Repeated trauma

140. Blistering distal dactylitis is mainly caused by A. Group A streptococci B. Group B streptococci C. Staphylococci D. E. coli

133. Schwartzmann phenomenon is seen in A. Meningococcemia B. Syphilis

141. Purpura fulminans A. Follows group A streptococcal infection B. Seen with deficiency of protein C

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Essentials in Dermatology C. DIC occurs in this condition D. All are true 142. Pitted keratolysis is caused by A. Micrococcus sedentarius B. Dermatophilus congolensis C. Corynebacterium D. All the above 143. Erythrasma, all are true except A. Caused by corynebacterium minutissimum B. It is a gram negative rod C. Coral–red fluorescence is seen with wood’s lamp D. It is treated using clotrimazole cream 144. All are true of trichomycosis axillaris except A. It is caused by fungus B. Nodular thickening of hair shaft present C. It involves axilla commonly D. Treatment is shaving 145. Russell bodies are seen in A. Rhinoscleroma B. Leishmania C. Syphilis D. Leprosy 146. All are true about bacillary angiomatosis except A. Caused by B. henselae and B. quintana B. Seen in AIDS patients C. Diagnosed by Warthin – Starry stain D. Treatment is by ciprofloxacin 147. All are seen in anthrax except A. Malignant pustule is seen B. It is usually painful C. Parenteral crystalline penicillin is the drug of choice D. Incision and debridement should be avoided 148. Apple – jelly nodules are seen in A. Sarcoidosis B. Lupus vulgaris

C. Leprosy D. Cat-scratch disease 149. Scrofuloderma involves all except A. Lymph nodes B. Bone and joints C. Epididymis D. Penis 150. Buruli ulcer is caused by A. M. ulcerans B. M. marinum C. M. bovis D. M. leprae 151. About staphylococcal scalded skin syndrome all are true except A. It is caused by phage II S. aureus B. It is a exotoxin mediated condition C. Can be seen in infants and adults D. Diagnosis is by demonstration of staphylococcus by gram stain 152. All are true about Orf except A. Ecthyma contagiosum is a synonym B. Caused by parapox virus C. Treatment is acyclovir D. Disease widespread in sheep and goats 153. All are true of molluscum contagiosum except A. May be seen in HIV infection B. Umbilicated vesicles are characteristic C. Caused by molluscipox virus D. Treatment is by curettage 154. Papular purpuric gloves and sock syndrome is caused by A. EBV B. Parvovirus – B19 C. HSV D. HPV 155. Gianotti Crosti syndrome is due to A. Hepatitis B B. Coxsackie virus C. EBV D. All of the above 156. Forscheimer’s sign is seen in A. Polio B. Rubella C. Toxoplasma D. Roseola infantum

Multiple Choice Questions 157. All are due to HPV infection except A. Epidermodysplasia verruciformis B. Buschke – Lowenstein tumour C. Epidermoid plantar cysts D. Condylomata lata 158. EBV causes A. Oral hairy leukoplakia B. Gianotti Crosti syndrome C. Lipshutz ulcer D. All of the above 159. Roseola infantum is caused by A. HHV-4 B. HHV-5 C. HHV-6 D. HHV-7

C. Pemphigoid D. PLE 164. Drug induced pemphigus is seen in all except A. Penicillamine B. Rifampicin C. Chloroquine D. Captopril 165. Acantholysis is seen in A. Epidermis B. Dermis C. Dermal-epidermal junction D. Subcutaneous tissue 166. Subepidermal bulla is seen in A. Pemphigus vulgaris B. Pemphigoid C. Hailey Hailey disease D. Transient acantholytic dermatoses

160. All are true about herpes zoster except A. Occasionally pain is not followed by eruption B. Multidermatomal zoster is a clinical marker for AIDS C. Postherpetic neuralgia occurs 2 weeks after eruption D. Eruption can be bilateral

167. Drug of choice in dermatitis herpetiformis A. Dapsone B. Tetracycline C. Psoralen D. Steroids

161. Kaposi’s varicelliform eruption is caused by A. Herpes simplex virus B. Coxsackie virus C. Vaccinia D. All of the above

168. Bullous disorder associated with gluten sensitive enteropathy A. Pemphigus B. Bullous pemphigoid C. Both A and B D. None of the above

162. About herpes simplex virus infection all are true except A. It is a common cause of erythema multiforme B. Both HSV-1 and HSV-2 cause herpes genitalis C. Mucocutaneous herpes simplex infection of 3 weeks is a CDC criteria for AIDS D. It does not cause herpes gestations

169. Row of tomb stone appearance seen in A. Pemphigus B. Pemphigoid C. Epidermolysis bullosa D. None of the above

163. Intraepithelial bulla is a feature of A. Pemphigus B. Dermatitis herpetiformis

171. Asboe-Hansen sign (Bulla spread sign) elicited in A. Bullous pemphigoid

170. Nikolsky’s sign is positive in A. Pemphigus vulgaris B. Bullous pemphigoid C. Pemphigus follaceous D. Both A and C

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Essentials in Dermatology B. Dermatitis herpetiformis C. Pemphigus vulgaris D. Friction blister 172. Acantholysis is due to dissolution of intercellular cement substance in the A. Epidermis B. Sub-epidermis C. Dermis D. Basement membrane 173. A 60 years old man presented with itchy tense hemorrhagic blisters over thigh and lower abdomen without any oral lesions diagnosis A. Bullous pemphigoid B. Pemphigus C. Both A and B D. Dermatitis herpetiformis 174. Koebner phenomenon seen in A. Lupus erythematosus B. Syphilis C. Lupus vulgaris D. Psoriasis 175. Not transmitted sexually A. Syphilis B. Yaws C. Gonorrhea D. Chancroid 176. Diagnostic method of choice in contact dermatitis A. Tzanck test B. Intradermal test C. Skin biopsy D. Patch test 177. Patch test read after A. 12 hours B. 24 hours C. 48 hours D. 72 hours 178. Tzanck cell is a A. Keratinocyte C. Neurtrophil

B. Lymphocyte D. All of the above

179. Drug of choice for T.unguium A. Amophotericin-B B. Miconoazole C. Griseofulvin D. Nystatin

180. Test not used for diagnosis of syphilis A. VDRL B. Frei’s test C. Reagin test D. TPI 181. Most common precipitant of contact dermatitis A. Gold B. Nickel C. Copper D. Silver 182. Mode of inheritance of ichthyosis vulgaris A. Autosomal recessive B. Autosomal dominant C. X-linked dominant D. X-linked recessive 183. Hyperpigmentation is not seen in A. Addison’s disease B. Cushing’s disease C. Hypothyroidism D. Graves disease 184. Treatment of choice for nodulocystic acne A. Systemic steroids B. Benzyl peroxide C. Retinoids D. Estrogens 185. Fordyce spots are A. Ectopic sebaceous glands B. Ectopic eccrine glands C. Ectopic apocrine glands D. Ectopic mucosal glands 186. Target lesions seen in A. Urticaria B. Lichen planus C. Scabies D. Erythema multiforme 187. Site not involved in psoriasis A. Skin B. CNS C. Nail D. Joints 188. Gonococcal infection does not affect A. Testes B. Ovary C. Cervix D. Fallopian tube

Multiple Choice Questions 189. Cicatrical alopecia seen in A. Tinea capitis B. Psoriasis C. DLE D. Alopecia areata 190. Erythema nodosum is seen in all except A. Tuberculosis B. Sulphonamide treatment C. Sarcoidosis D. Giant cell arteritis 191. Skin doubling time A. 2 weeks B. 4 weeks C. 8 weeks D. 12 weeks 192. Alopecia areata is due to A. Androgenic stimulation B. Estrogenic stimulation C. Autoimmune D. All of the above 193. Griseofulvin is used in all except A. T. versicolor B. T. Capitis C. T. corporis D. T. unguium 194. Palpable purpura is seen in all except A. HS purpura B. Vasculitis C. ITP D. Amyloidosis 195. Treatment of choice for exfoliative dermatitis due to gold is A. Steroids B. Cephalosporins C. Methotrexate D. Hemodialysis 196. Characteristic nail change in LP is A. Pitting B. Onycholysis C. Pterygium D. Subungual hyperkeratosis 197. Myrmecia warts are A. Plantar warts B. Plane warts C. Venereal warts D. Filiform warts 198. Fungi with no sexual stage A. Basidiomyecetes B. Phycomycetes C. Ascomycetes D. Fungi imperfecti

199. Rain drop pigmentation over the trunk is seen in A. Chronic lead poisoning B. Chronic arsenic poisoning C. Mercury poisoning D. All of the above 200. Oil drop sign seen in A. B. C. D.

Psoriasis of nails Lichen planus nails Clubbing Dermatophyte infection of nails

201. Dennie Morgan fold is seen in A. Mastocytosis B. Sarcoidosis C. Atopic dermatitis D. Seborrheic dermatitis 202. Black hairy tongue is due to intake of A. Psoralen B. Retinoids C. Broad spectrum antibiotics D. Steroid 203. All are seen in dermatomyositis except A. Heliotrope rash B. Gottron’s sign C. Gottron’s papule D. Sclerodactyly 204. Periungual telangiectasia seen in A. Dermatomyositis B. Scleroderma C. SLE D. All of the above 205. Drug induced lupus is seen with——— antibody A. Anti ds DNA B. Antihistone C. Anti Sm D. Anti ss DNA 206. Drug induced lupus occurs with all except A. INH B. Procainamide C. Hydralazine D. Aspirin

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Essentials in Dermatology 207. CREST syndrome contains all except A. Calcinosis B. Raynaud’s phenomenon C. ECG changes D. Sclerodactyly 208. Scl-70 is characteristic of A. CREST syndrome B. Diffuse systemic sclerosis C. Diabetes mellitus D. SLE 209. All are seen in mixed connective tissue disease except A. Sausage digits B. Anti U1-RNP positive C. Pulmonary involvement in common D. Usually of infective origin 210. Bywaters lesion, a nail fold infarct, is seen in A. Rheumatoid arthritis B. Psoriatic arthritis C. Gonococcal arthritis D. Osteoarthritis 211. Components of antiphospholipid syndrome except A. Recurrent abortions B. Arterial and venous thrombosis C. High VDRL D. A-partial prothrombin time decrease 212. What is vagabond’s disease A. Pediculosis corporis B. Pediculosis pubis C. Scabies D. Tinea cruris 213. Treatment of pediculosis includes A. Permethrin B. Cotrimoxazole C. Physostigmine ointment D. All of the above 214. Treatment of scabies includes all except A. 5% Permethrin B. 1% Lindane C. 0.5% Malathion D. 10% Sulphur

215. Nodular scabies is seen over A. Palms B. Scrotum C. Trunk D. Face 216. A 9-month infant presents to you with complaints of itchy papules over the face, and papulo-vesicles over palms and soles. What is your diagnosis A. Scabies B. Pediculosis C. Candidiasis D. Acropustulosis 217. All are true about Norwegian scabies except A. Seen in Down’s syndrome B. Methotrexate is used as treatment for this condition C. The condition is often extremely pruritic D. Patient should be isolated till cured 218. True about scabies incognito A. It is a type of animal scabies B. Classical burrows are seen in this condition C. Due to inappropriate use of topical steroids D. Does not need treatment 219. True about scabies A. It is caused by the male sarcoptes mite B. It is not an STD C. Lindane is used in the treatment of scabies in infants D. Burrow is diagnostic of this condition 220. What is true about treatment of scabies A. Ivermectin is used in outbreaks B. Sulphur is contraindicated in pregnancy C. Itching subsides immediately after therapy D. Lindane should be applied after hot bath 221. Wickham’s striae represent a network of fine white lines or puncta, are seen in many papules of A. Psoriasis B. Pityriasis rosea

Multiple Choice Questions C. Lichen planus D. Pityriasis rubra pilaris 222. The herald patch is seen in 50 to 90 percent of cases of A. Pityriasis rosea B. Pityriasis alba C. Pityriasis rubra pilaris D. Pityriasis lichenoides 223. A macule is a circumscribed, flat lesion that differs from surrounding skin because of its A. Colour B. Size C. Shape D. Texture 224. The Koebner reaction occurs in traumatized normal skin in A. Vitiligo B. Herpes zoster C. Sporotrichosis D. Linear scleroderma 225. Nikolsky’s sign refers to the sheet like removal of epidermis by gentle traction that can be observed in A. Bullous pemphigoid B. Dermatitis herpetiformis C. Epidermolysis bullous acquisition D. Pemphigus vulgaris 226. A rounded or flat-topped papule or plaque that is characteristically evanescent, disappearing within hours is known as A. Nodule B. Bulla C. Cyst D. Wheal 227. The appearance of pin point dots of blood at the top of ruptured capillaries when scale is forcibly removed from psoriatic plaques refers to A. Tzanck test B. Bioscopy C. Auspitz’sign D. Darier’s sign

228. The classical triad of dermatitis, diarrhea and dementia is seen in A. Phrynoderma B. Pellagra C. Beri Beri D. Scurvy 229. Probably the most frequent and the best known nail deformity in psoriasis is A. Thickening of nail B. Pitting C. Nail discoloration D. Onycholysis 230. Exclamation – mark hairs may be present at the margin of smooth alopecia patch in A. Tinea capitis B. Androgenetic alopecia C. Trichotillomania D. Alopecia areata 231. Oral hairy leukoplakia is a lesion specific to A. Human papilloma virus infection B. HIV disease C. Varicella zoster virus infection D. Cytomegalovirus infection 232. Most common form of oral candidiasis is A. Chronic hyperplastic B. Chronic atrophic C. Acute atrophic D. Acute pseudomembranous 233. Tinea capitis requires a systemic antifungal to penetrate the hair follicle such as A. Potassium iodide B. Griseofulvin C. Amphotericin D. Nystatin 234. The most common dermatophyte producing favus is A. T.verrucosum B. T. violaceum

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Essentials in Dermatology C. T. tonsurans D. T. schoenleinii 235. Bullous impetigo is caused by A. Staphylococcus aureus B. Streptococcus pyogenes C. Streptococcus epidermidis D. Streptococcus haemolyticus 236. The mean incubation period of varicella is A. 7 days B. 14 days C. 21 days D. 28 days 237. A subcutaneous tuberculosis leading to cold abscess formation and secondary break down of the overlying skin is referred to as A. Lupus vulgaris B. Scrofuloderma C. Tuberculosis verrucosa cutis D. Orificial tuberculosis

C. Seborrhoeic keratosis D. Bowenoid keratosis 242. Vitiligo involving distal digits and periorificial areas is named as A. Vitiligo vulgaris B. Acrofacial vitiligo C. Focal vitiligo D. Universal vitiligo 243. Acute vesicular eczema of hands and feet is known as A. House wife dermatitis B. Nummular eczema C. Atopic eczema D. Pompholyx 244. Most common photodermatosis is A. Polymorphic light eruption B. Actinic prurigo C. Hydroa vacciniformis D. Chronic actinic dermatitis

238. Leprosy is a chronic granulomatous infection primarily of A. Skeletal muscles B. Spinal cord C. Skin and nerves D. Central nervous system

245. “Clue cells” on wet mount are seen in vaginal discharge of A. Candidal vaginitis B. Bacterial vaginosis C. Trichomoniasis D. Genital mycoplasma infection

239. Erythema Nodosum Leprosum occurs most often in A. Tuberculoid leprosy B. Lepromatous leprosy C. Borderline borderline leprosy D. Borderline tuberculoid leprosy

246. Commonest form of cutaneous tuberculosis is A. Lupus vulgaris B. Scrofuloderma C. Tuberculosis verrucosa cutis D. Orificial tuberculosis

240. Syphilis is an infectious disease caused by Treponema pallidum that is pathogenic to A. Humans only B. Humans and birds C. Humans and amphibians D. Terrestrial animals 241. Leser-Trelat sign basically consists of an association of multiple internal malignancies with multiple eruptive A. Actinic keratosis B. Arsenical keratosis

247. Other than leprosy, dapsone is the drug of choice in A. Pemphigus vulgaris B. Dermatitis herpetiformis C. Bullous pemphigoid D. Epidermolysis bullosa 248. Griseofulvin is the most frequently systemically used drug in A. Tinea versicolor B. Dermatophytosis C. Candidiasis D. Tinea nigra

Multiple Choice Questions 249. Tinea versicolor lesions, under Wood’s light demonstrate A. Coral red fluorescence B. Bright red fluorescence C. Yellow fluorescence D. Bluish green fluorescence 250. An antihelminthic agent given systemically found to be effective in scabies is A. Mebendazole B. Thiabendazole C. Albendazole D. Ivermectin 251. KOH mount of skin scraping demonstrates “Spaghetti and meat balls” in A. Tinea versicolor B. Tinea corporis C. Tinea nigra D. Tinea capitis 252. Tinea imbricata is caused by A. Trichophyton rubrum B. Trichophyton mentagrophytes C. Trichophyton concentricum D. Trichophyton verrucosum 253. Auspitz’s sign occurs only in A. Pemphigus B. Psoriasis C. Lichen planus D. Lichen nitidus 254. Mucous patch is the best known and most characteristic lesion of A. Primary syphilis B. Secondary syphilis C. Latent syphilis D. Tertiary syphilis 255. Herpes zoster is almost invariably unilateral and most frequently affects A. Cervical dermatome B. Thoracic dermatome C. Lumbar dermatome D. Sacral dermatome 256. Oral hairy leukoplakia, a distinct corrugated white plaque on one or both sides of the tongue is an early clue to the diagnosis of A. HIV infection B. Human papillomavirus infection

C. Herpes simplex infection D. HTLV-I virus infection 257. Pellagra is due to deficiency of A. Thiamine B. Pyridoxine C. Riboflavin D. Niacin 258. Growth of the terminal hair in a male pattern in a female is called as A. Anagen effluvium B. Telogen effluvium C. Hypertrichosis D. Hirsutism 259. On average, number of mites present at the itching stage of scabies in an adult are A. 6 B. 12 C. 24 D. 36 260. After first being infested, how many weeks it takes for the hypersensitivity reaction to scabies mite to occur and express it as intense itching A. 0-4 weeks B. 4-6 weeks C. 6-8 weeks D. 8-12 weeks 261. Langerhans cells serves a primary role in immune reactions of A. Type I hypersensitivity B. Type II hypersensitivity C. Type III hypersensitivity D. Type IV hypersensitivity 262. Heliotrope rash is characteristic of A. Systemic lupus erythematosus B. Scleroderma C. Polymyositis D. Dermatomyositis 263. Scrofuloderma is a form of A. Syphilis B. Sarcoidosis C. Skin tuberculosis D. Sporotrichosis 264. Finger nail growth is A. 0.1 mm/day B. 0.2 mm/day C. 0.3 mm/day D. 0.4 mm/day

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Essentials in Dermatology 265. The commonest type of basal cell carcinoma is A. Ulcerative B. Pigmented C. Morphoeic D. Nodulo cystic/ Nodulo-ulcerative 266. Urticaria pigmentosa is characterized by excessive accumulation in many tissues A. Macrophages B. Fibroblasts C. Mast cells D. Langerhans cells 267. Increased sebum secretion and formation of horny plugs in follicular lumens are the earliest abnormalities in A. Seborrheic dermatitis B. Sebopsoriasis C. Acne rosacea D. Acne vulgaris 268. Wickham’s striae on the flat topped polygonal purple colored papule are typical of A. Lichen planus B. Psoriasis C. Pityriasis rubra pilaris D. Pityriasis rosea 269. The nails are often affected and may show the so called thimble pitting in A. Lichen planus B. Psoriasis C. Pityriasis rubra pilaris D. Pityriasis rosea 270. The definitive technique for diagnosing allergic contact hypersensitivity is A. Intradermal test B. Patch test C. “RAST” test D. Lymphocyte transformation test 271. Raised level of serum IgE and its relationship with severity of eczema is a feature of A. Nummular eczema B. Asteatotic eczema

C. Atopic dermatitis D. Seborrheic dermatitis 272. White dermographism is typically seen in A. Angioedema B. Urticaria C. Contact dermatitis D. Atopic dermatitis 273. Gluten enteropathy is a feature of the following blistering disorder A. Bullous pemphigoid B. Epidermolysis bullosa C. Pemphigus vulgaris D. Dermatitis herpetiformis 274. Target like lesions are commonly encountered in A. Erythema annulare centrifugum B. Erythema marginatum C. Erythema nodosum D. Erythema multiforme 275. Pediculosis capitis is recognized by louse eggs stuck to hair called as A. Hair casts B. Hair beading C. Piedra D. Nits 276. The best sites on which to find scabies burrows are A. Interdigital areas of the fingers B. Soles C. Abdomen D. Back 277. Herpes zoster caused by varicella zoster virus is primarily due to A. Primary infection of virus B. Autoinoculation of virus C. Reactivation of virus D. Re-infection of virus 278. Common warts of hands and fingers are also known as A. Myrmecia warts B. Plane warts C. Mosaic warts D. Verruca vulgaris

Multiple Choice Questions 279. Ritter’s disease (SSSS) is caused by A. Pseudomonas aeruginosa B. Bacillus anthracis C. Streptococcus pyogenes D. Staphylococcus aureus 280. Major skin disorder seen in AIDS patients is A. Dermatophytosis B. Seborrheic dermatitis C. Herpes simplex infection D. Candidiasis 281. Hutchinson’s triad consists of Hutchinson’s teeth, interstitial keratitis and A. Saddle nose B. Clutton’s joints C. Sabre tibiae D. VIII nerve deafness 282. Hardened deposits that result from drying up of serum, blood or purulent exudate on the skin surface is called A. Scale B. Crust C. Calcinosis D. Ossification 283. A group of autoimmune blistering diseases of skin and mucous membrane from intraepidermal blisters due to acantholysis is A. Pemphigus B. Pemphigoid C. Dermatitis herpetiformis D. Epidermolysis bullosa

C. Miliaria pustulosa D. Miliaria profunda 286. Tinea capitis caused by Microsporum species, under Wood’s lamp demonstrates A. Bluish green fluorescence B. Yellow fluorescence C. Coral red fluorescence D. Pink fluorescence 287. Dendritic cells among keratinocytes of the stratum spinosum of the epidermis, having distinct folded nucleus and Birbeck granules are A. Langerhans cells B. Melanocytes C. Merkel cells D. Fibroblasts 288. Deep seated vesicles resembling tapioca on the sides of the fingers, palms and soles characterize A. Pompholyx B. Nummular eczema C. Atopic dermatitis D. Xerotic eczema 289. Erythema nodosum leprosum occurs in half of patients with A. Indeterminate leprosy B. Tuberculoid leprosy C. Borderline tuberculoid leprosy D. Borderline lepromatous or lepromatous leprosy

284. Koenen’s periungual fibromas develop in 50% of cases of A. Ataxia telangiectasia B. Sturge Weber syndrome C. Neurofibromatosis D. Tuberous sclerosis

290. Safest as well as the most effective medication for scabies is A. Permethrin 5% B. Precipitated sulphur 6-10% C. Crotamiton 10% D. Benzoyl benzoate 25%

285. A common condition resulting from obstructed sweat, causing pruritic inflammatory papules around the sweat pores is known as A. Miliaria crystalline B. Miliaria rubra

291. Most effective sulfone in dermatitis herpetiformis is A. Dapsone B. Sulfapyridine C. Sulfasalazine D. Sulphamethoxazole

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Essentials in Dermatology 292. Slit skin smear examination is used to demonstrate organisms from the skin in A. Pyoderma B. Leishmaniasis C. Donovanosis D. Leprosy 293. A drug eruption which recurs at the same site/s and heals with slate gray pigmentation is A. Erythema multiforme B. Stevens Johnson syndrome C. Toxic epidermal necrolysis D. Fixed drug eruption 294. A small solid elevated lesion smaller than 0.5 cm in diameter is called as A. Macule B. Papule C. Vesicle D. Nodule 295. If touching of the papule of secondary syphilis with the head of a pin is exquisitely tender, then sign said to be positive is A. Ollendorf’s sign B. Auspitz’s sign C. Nikolsky’s sign D. Button holing sign 296. Sharply localized oedema or wheal with a surrounding erythematous flare occurring within seconds to minutes after the skin has been stroked, characterize A. Dermatographism B. Cholinergic urticaria C. Pressure urticaria D. Adrenergic urticaria 297. A collarettes of dermatitis in the ‘V’ area of neck called Casal’s necklace is seen in A. Vitiligo B. Ichthyosis C. Contact dermatitis D. Pellagra

298. Henderson-Paterson bodies are intracytoplasmic, ovoid inclusion bodies which are demonstrated in lesions of A. Herpes simplex B. Herpes zoster C. Molluscum contagiosum D. Warts 299. Thalidomide is the drug of choice in the treatment of A. Type 1 upgrading lepra reaction B. Type 1 downgrading lepra reaction C. Type 2 lepra reaction D. Lucio phenomenon 300. Drug of choice for erythrasma is A. Dapsone B. Griseofulvin C. Ketoconazole D. Erythromycin 301. In secondary syphilis, chancre is still present and active in A. 3% cases B. 33% cases C. 63% cases D. 93% cases 302. This type of eruption is not a feature of secondary syphilis A. Macules B. Papules C. Vesicles D. Pustules 303. The Wood’s light examination of erythrasma shows fluorescence which is A. Coral red B. Yellow C. Blue D. Green 304. A new effective topical agent available for the treatment of scabies is A. Calcipotriol B. Mupirocin C. Permethrin D. Azelaic acid 305. Circle of Hebra is related to A. Impetigo B. Syphilis C. Scabies D. Leprosy

Multiple Choice Questions 306. The human herpes virus associated with roseola infantum is A. Human herpes virus 6 B. Human herpes virus 1 C. Human herpes virus 2 D. Varicella Zoster virus

313. Gottron’s papules and Gottron’s sign are pathognomonic of A. Dermatomyositis B. Systemic lupus erythematosus C. Progressive systemic sclerosis D. Crohn’s disease

307. In gonorrhea, the risk of infection from a single exposure to an infected male, for females is A. 50-90% B. 10-50% C. 5-10% D. 1-5%

314. Acanthosis palmaris, a marker of internal malignancy occurs most commonly with A. Bronchial carcinoma B. Gastric carcinoma C. Colonic carcinoma D. Prostatic carcinoma

308. Myrmecia warts are A. Condylomata acuminata B. Common warts C. Verruca plana D. Plantar warts

315. Ollendorf’s sign is present in A. Pityriasis rosea B. Secondary syphilis C. Guttate psoriasis D. Lichen planus

309. Dimple sign is elicited in A. Neurofibroma B. Neurilemmoma C. Dermatofibroma D. Malignant melanoma

316. Lucio phenomenon is a type of lepra reaction confined to A. Tuberculoid leprosy B. Histoid leprosy C. Borderline leprosy D. Lucio leprosy

310. Epiloia is a triad of epilepsy, low intelligence and A. Acanthosis nigricans B. Acanthoma C. Astrocytoma D. Adenoma sebaceum 311. Sudden eruption of seborrheic keratosis lesions in association with internal malignancy is called as A. Leser Trelat sign B. Asboe Hansen’s sign C. Darier’s sign D. Tin tack sign 312. Depigmentation around melanocytic naevus is known as A. Meyerson naevus B. Sutton naevus C. Naevus of Ito D. Naevus of Ota

317. Pitted keratolysis is caused by A. Corynebacterium species B. Pseudomonas aeruginosa C. Hemophilus ducreyi D. Streptococcus pyogenes 318. Mucous patch is the best known and most characteristic lesion of A. Primary syphilis B. Secondary syphilis C. Latent syphilis D. Tertiary syphilis 319. The skin is one of the largest organs in the body making up about A. 16% of the body weight B. 26% of the body weight C. 36% of the body weight D. 46% of the body weight

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Essentials in Dermatology 320. The manifestations of leprosy depends on the degree of A. Type I hypersensitivity B. Type II hypersensitivity C. Type III hypersensitivity D. Type IV hypersensitivity 321. The main cell of the epidermis is the A. Keratinocyte B. Dendrocyte C. Melanocyte D. Langerhans cell 322. Collagen is the major structural protein of the dermis forming A. 30-40% of its dry weight B. 40-50% of its dry weight C. 60-70% of its dry weight D. 70-80% of its dry weight 323. A localized area of colour change in the skin without elevation or infiltration is called as A. Papule B. Nodule C. Macule D. Plaque 324. KOH mount of skin scraping demonstrates “spaghetti and meat balls” in A. Tinea versicolor B. Tinea corporis C. Tinea nigra D. Tinea capitis 325. The generalized eruption is preceded in most patients by the appearance of a herald patch in A. Psoriasis vulgaris B. Lichen planus C. Pityriasis rosea D. Secondary syphilis 326. The commonest Mycobacterium tuberculosis skin infection is A. Lupus vulgaris B. Scrofuloderma

C. Warty tuberculosis D. Tuberculosis cutis orificialis 327. Human papilloma virus subtypes 6, 11, 16, and 18 are associated with A. Plantar warts B. Plane warts C. Common warts D. Genital warts 328. The drug that given systemically is not effective in the treatment of tinea versicolor is A. Griseofulvin B. Ketoconazole C. Fluconazole D. Itraconazole 329. The most common opportunistic fungal infection in all stages of HIV disease is A. Candidiasis B. Cryptococcosis C. Histoplasmosis D. Coccidioidomycosis 330. Malar rash is a characteristic feature of A. Dermatomyositis B. Systemic lupus erythematosus C. Systemic sclerosis D. Rheumatoid arthritis 331. Incubation period of gonococcal urethritis is usually A. 2-5 days B. 5-15 days C. 15-20 days D. 20-25 days 332. Commonest pathogen involved in nongonococcal urethritis in males is A. Chlamydia trachomatis B. Ureaplasma urealyticum C. Trichomonas vaginalis D. Herpes simplex virus 333. Postherpetic neuralgia is a complication of A. Herpes simplex virus infection B. Varicella C. Herpes zoster D. Cytomegalovirus infection

Multiple Choice Questions 334. Allergic contact dermatitis is mediated by A. Type I hypersensitivity B. Type II hypersensitivity C. Type III hypersensitivity D. Type IV hypersensitivity 335. Tissue smear in donovanosis shows Donovan bodies inside vacuolated cytoplasm of large A. Neutrophils B. Basophils C. Eosinophils D. Mononuclear cells 336. The skin immune system comprises of epidermal keratinocytes, epidermotropic T cells and A. Langerhans cells B. Langhan giant cells C. Epithelioid cells D. Merkel cells 337. Heliotrope, a periorbital violaceous erythema with associated edema of the eyelids and periorbital tissue frequently occurs in A. Systemic lupus erythematosus B. Drug rash C. Discoid lupus erythematosus D. Dermatomyositis 338. Stratum lucidum, a layer in the epidermis of the skin is seen in A. Eyelids B. Scalp C. Palms and soles D. Nails 339. Intraepidermal blister (supra basal cleft) due to acantholysis is observed in A. Bullous pemphigoid B. Dermatitis herpetiformis C. Pemphigus vulgaris D. Cicatricial pemphigoid 340. Herald patch is frequently an initial feature of A. Psoriasis B. Lichen planus

C. Pityriasis rosea D. Eczema 341. Clue cells in vaginal discharge is the diagnostic feature of A. Bacterial vaginosis B. Vaginal trichomoniasis C. Candida vulvovaginitis D. Gonococcal cervicitis 342. The causative agent of donovanosis is A. Leishmania donovania B. Leishmania ethiopica C. Calymmatobacterium granulomatis D. Borrelia burgdorferi 343. Actinomycosis is caused by A. Actinomyces israelii B. Madurella grisea C. Madurella mycetomatis D. Histoplasma capsulatum 344. The commonly employed test in the diagnosis and monitoring of disease activity of syphilis is A. VDRL B. TPHA C. FTA-ABS test D. ELISA test 345. Which one of the following is not true in lepromatous leprosy A. Lepromatous leprosy initially has cutaneous and mucosal lesions B. Neural changes in lepromatous leprosy occur later C. The skin lesions are numerous and symmetrically arranged D. Lepromin test is positive 346. Conclusive proof of leprosy is the demonstration of a granuloma or the presence of acid fast bacilli in A. Nerves B. Arrector pilorum muscle C. Sweat glands D. Sebaceous glands

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Essentials in Dermatology 347. Erythema nodosum leprosum occurs most commonly in lepromatous leprosy and less frequently in A. Borderline lepromatous leprosy B. Borderline tuberculoid leprosy C. Tuberculoid leprosy D. Indeterminate leprosy 348. Which one of the following is not true in histoid leprosy A. Histoid leprosy is a distinctive variant of borderline lepromatous leprosy B. It frequently follows incomplete chemotherapy or acquired drug resistance C. It is characterized by the occurrence of well demarcated cutaneous and subcutaneous nodules D. Histoid leprosy lesions show the highest loads of bacilli and the majority are solid staining 349. Impetigo contagiosa is primarily an endemic disease of A. Adults B. Adolescents C. School children D. Preschool children 350. A way to screen for therapeutic agents and to identify drug resistance in leprosy was provided by the limited growth of Mycobacterium leprae in the A. Mouse foot pad B. Armadillo C. Monkey D. Nude mice 351. The classical staining method for demonstrating leprosy bacilli in lesions is A. Giemsa Staining B. Leishman staining C. Modified Ziehl Neelsen staining D. Gram’s staining

352. Most probable mode of transmission of leprosy other than direct skin to skin contact is A. Inhalation B. Ingestion C. Insect bite D. Inoculation 353. Mitsuda negative patients with indeterminate leprosy are more likely to develop A. Tuberculoid leprosy B. Borderline tuberculoid leprosy C. Borderline lepromatous leprosy D. Lepromatous leprosy 354. Which one of the following is not true in leprosy A. Tuberculoid and lepromatous leprosy patients are stable B. Tuberculoid leprosy often self heals C. Lepromatous leprosy remains heavily infected unless given appropriate chemotherapy D. Indeterminate leprosy is the most unstable with most patients down grading if not treated 355. Ecthyma is an ulcerative staphylococcal or streptococcal pyoderma, nearly always of the A. Shins or dorsal feet B. Trunk C. Thighs D. Face or upper limbs 356. Though bullous impetigo may occur at any age, it characteristically occurs in A. Newborn infants B. Children C. Adolescents D. Adults 357. Acute glomerulonephritis sometimes follows skin infections due to A. Group A beta hemolytic streptococci B. Staphylococcus aureus C. Staphylococcus epidermidis D. Pseudomonas aeruginosa

Multiple Choice Questions 358. Perifollicular chronic pustular staphylococcal infection of the bearded region is known as A. Pseudofolliculitis barbae B. Herpetic sycosis C. Sycosis barbae D. Acne vulgaris 359. Pterygium formation, a characteristic nail change in which the proximal nail fold fuses with the proximal portion of the nail bed occurs in A. Psoriasis B. Lichen planus C. Lichen nitidus D. Reiter’s syndrome 360. Pruritic small violaceous flat topped polygonal papules are almost pathognomonic and characteristic of A. Secondary syphilis B. Pityriasis rosea C. Lichen planus D. Psoriasis vulgaris 361. The skin disease most frequently beginning with a single herald or mother patch, usually larger than succeeding lesions is A. Guttate psoriasis B. Secondary syphilis C. Lichen planus D. Pityriasis rosea 362. The individual oval patches often desquamates leaving a “collarettes of scaling” in A. Pityriasis rosea B. Pityriasis rubra pilaris C. Pityriasis versicolor D. Pityriasis alba 363. The skin eruption, keratoderma blennorrhagica occurs in A. Secondary syphilis B. Pityriasis rosea C. Reiter’s syndrome D. Pityriasis rubra pilaris

364. Reiter’s syndrome is a characteristic triad consisting of urethritis, conjunctivitis and A. Arthritis B. Dermatitis C. Glomerulonephritis D. Stomatitis 365. The Goeckerman technique (a treatment with tar preparation) is an effective and frequently gratifying method of treatment in A. Pityriasis rubra pilaris B. Pityriasis rosea C. Psoriasis D. Pityriasis capitis 366. On removal of the scales from patches of psoriasis, bleeding points appear. This sign is named as A. Ollendorf’s sign B. Auspitz’s sign C. Nikolsky’s sign D. Button holing sign 367. Cradle cap consisting of yellow or brown scaling lesions on the scalp with accumulated adherent debris occurring in infants is a manifestation of A. Psoriasis vulgaris B. Seborrheic dermatitis C. Pityriasis rosea D. Pityriasis rubra pilaris 368. Pityrosporum ovale, the lipophilic yeast has role in A. Seborrheic dermatitis B. Pityriasis rosea C. Pityriasis rubra pilaris D. Erythroderma 369. Koebner phenomenon is observed in all except A. Psoriasis B. Lichen planus C. Vitiligo D. Pemphigus vulgaris

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Essentials in Dermatology 370. Diascopy is most useful in detection of nonblanchable raised purpura, the clinical hallmark of A. Cutaneous vasculitis B. Hallo nevus C. Nevus achromicus D. Nevus flammeus 371. An infant was brought by his mother with complaints of asymptomatic recurrent blistering over occiput, buttocks, legs and back of 2 months duration. Some of the blisters spontaneously healed on their own. Mucosa, nails and hair were normal. Your diagnosis. A. Pemphigus vulgaris B. Epidermolysis bullosa C. Miria D. Neonatal lupus erythematosus 372. Papillary tip microabscesses are seen in A. Pemphigus vulgaris B. Bullous pemphigoid C. Dermatitis herpetiformis D. Dermatitis repens 373. A 60-year-old male was admitted to the dialysis unit for chronic renal failure. Few days later he developed blisters over the dorsa of hands. On examination his blood sugar was 342 mg%, serum creatinine 5 mg%, serum potassium 5mg/dL, Hb: 6.0 mg%, and urine porphyrin level was normal. Your diagnosis. A. Diabetic bullae B. Pseudoporphyria C. Pemphigus vulgaris D. Bullous SLE 374. All are techniques in the investigation of blistering disorder A. Immunofluorescence B. Immunoblotting C. Electron microscopy D. All of the above

375. All are true about neonatal LE except A. Discoid lesions B. Positive anti Ro – antibodies C. All patients clear by 6 months D. Treatment is usually required 376. Erythema gyratum repens is associated usually with A. Lung carcinoma B. Breast carcinoma C. Renal cell carcinoma D. CTCL 377. All are components of glucagonoma except A. Neurolytic migratory erythema B. High serum levels of amino acids C. Resection of tumor is treatment D. Alpha cell tumor of pancreas 378. Relapsing febrile nodular panniculitis is called A. Erythema nodosum B. Weber – Christian syndrome C. Enzymic panniculitis D. Nodular panniculitis 379. Darier’s sign is classically seen in A. Mastocytosis B. Histiocytoma C. Non-Hodgkin’s lymphoma D. Mycosis fungoides 380. Pautrier’s abscesses are seen in A. Mycosis fungoides B. Pautrier’s neuroma C. Psoriasis D. Impetigo herpetiformis 381. Black piedra is caused by A. Piedraia hortae B. T. beigelii C. Exophila D. Fusarium 382. What is desert rheumatism A. Cryptococcosis B. Coccidioidomycosis

Multiple Choice Questions C. Blastomycosis D. Paracoccidioidomycosis 383. Ramu, a 52-year-old male came to the hospital with complaints of low-grade fever, chest pain, cough and hemoptysis of 2 months duration. Few nodules with ulceration were seen on the trunk. Histology of these nodules showed refractile spherical yeasts with broad based buds. What is your diagnosis? A. Blastomycosis B. Candidiasis C. Coccidioidomycosis D. Cryptococcosis 384. What is Darling’s disease A. Histoplasmosis B. Blastomycosis C. Cryptococcosis D. Infectious mononucleosis 385. Copper penny bodies are seen in A. Chromomycosis B. Sporotrichosis C. Mycetoma D. Cryptococcosis 386. Asteroid bodies are seen in A. Sporotrichosis B. Chromomycosis C. Tinea D. Paracoccidioidomycosis 387. Endothrix dermatophyte infection in India is due to A. T. tonsurans B. T. violaceum C. M. canis D. T.yaoundei 388. A six years old male child has well defined patch of alopecia over the vertex with no scaling. Few exclamation mark hairs are seen at the periphery. There is no history of conflict with the mother. What is your diagnosis? A. Alopecia areata B. Tinea capitis

C. Trichotillomania D. Traction alopecia 389. An eight-year-old child has multiple yellow crusts over the scalp of 2 months duration. KOH examination of the crusts yielded multiple air spaces with hyphae. Woods lamp shows grayish fluorescence. What is your diagnosis? A. Favus B. Kerion C. Black dot tinea capitis D. Gray patch tinea capitis 390. An ideal treatment of kerion to avoid scarring is A. Griseofulvin B. Oral steroids C. Shaving D. A and B 391. What is calabar swelling? A. Loiasis B. Onchocerciasis C. Trypanosomiasis D. Insect bite 392. A 25-year-old HIV seropositive male presented with fever, abdominal distension, cough, dyspnea and mental changes. On examination a petechial rash around the umbilicus is seen. Few linear tracks are seen around the trunk. How do you establish your diagnosis? A. Stool examination B. Chest X-ray C. Urine examination D. Ultrasound abdomen 393. Larva currens is due to A. Strongyloidiasis B. Ancylostomiasis C. Uncinaria D. Bubostomum 394. Leopard skin is seen in A. Onchocerciasis B. Loiasis C. Mycosis fungoides D. Psoriasis

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Essentials in Dermatology 395. All are true of Refsum’s disease except A. Autosomal dominant B. Phytanic acid oxidase deficiency C. Sensorineural deafness is present D. Chlorophyll should be avoided in diet 396. About acanthosis nigricans all are true except A. Seen in obese individuals B. Individual diabetes is insulin sensitive C. Seen in with use of nicotinic acid D. Associated with adenocarcinoma 397. Opera glass deformity is seen in A. Rheumatoid arthritis B. Osteoarthritis

C. Psoriatic arthritis D. Ankylosing spondylitis 398. Bowen’s disease is associated with ——— poisoning A. Mercury B. Selenium C. Arsenic D. Chromium 399. Koebner phenomenon is seen in all except A. Psoriasis B. Xanthoma C. Lichen planus D. Pityriasis rosea 400. In Munro’s microabscess, the inflammatory cell involved is A. Neutrophil B. Lymphocyte C. Monocyte D. Eosinophil


Set II (1-245) 1. Nodules are morphologically similar to papules, but they are more than____in diameter A. 0.5 cm B. 1 cm C. 1.5 cm D. 2 cm 2. Distinct intracytoplasmic organelles called Birbeck granules characterize A. Keratinocytes B. Langerhans cells C. Melanocytes D. Mast cells 3. Functionally, Langerhans cells are of the monocyte macrophage lineage and originate in A. Thymus B. Neuroectoderm C. Bone marrow D. Lymph nodes 4. The sebaceous glands in the buccal mucosa and vermilion border of the lip are termed as A. Meibomian glands B. Montgomery’s tubercles C. Tyson’s glands D. Fordyce spots 5. Finger nails grow on an average of 0.1 mm per day, require about_____ to replace a complete nail plate A. 1-2 months B. 2-3 months C. 3-4 months D. 4-5 months 6. By far, the most common cutaneous symptom of skin disorders is A. Pruritus B. Pain C. Burning sensation D. Allodynia 7. Pruritus induced by temperature changes in polycythemia vera is immediately relieved by A. H1 antagonists B. H2 antagonists C. Aspirin D. Ketotifen

8. An abnormal urge to pull out the hair results in irregular areas of scalp hair loss in A. Alopecia areata B. Androgenetic alopecia C. Traction alopecia D. Trichotillomania 9. Necrolytic migratory erythema is usually associated with A. Bronchogenic carcinoma B. Sarcoidosis C. Subacute cutaneous lupus erythematosus D. APUD cell tumour of the pancreas 10. Removal of the scale shows its undersurface to be covered with the horny plugs that filled the follicles, resembling carpet tacks, are observed in A. Pityriasis rosea B. Lichen planus C. Discoid lupus erythematosus D. Psoriasis vulgaris 11. Systemic lupus erythematosus specific, antinuclear antibody pattern (anti DNA) is A. Peripheral B. Homogeneous C. Particulate D. Nucleolar 12. Antinuclear antibody (ANA) test is positive in about a third of all connective tissue disorders, but in systemic lupus erythematosus in A. 5% of cases B. 25% of cases C. 55% of cases D. 95% of cases 13. A reddish purple scaling eruption over the knuckles and over the knees and elbows, referred to as Gottron’s sign, occurs in A. CREST syndrome B. Dermatomyositis C. Systemic lupus erythematosus D. Sjogren’s syndrome

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Essentials in Dermatology 14. Reiter’s syndrome is a characteristic clinical triad consisting of urethritis, conjunctivitis and _____ A. Arthritis B. Carditis C. Neuritis D. Uveitis 15. Impetigo of the scalp is a frequent complication of A. Pityriasis capitis B. Scalp psoriasis C. Pediculosis capitis D. Seborrheic dermatitis 16. Treatment of choice for erythrasma is A. Systemic erythromycin B. Topical erythromycin C. Topical clindamycin D. Topical tolnaftate solution 17. Ecthyma gangrenosum which occurs in debilitated persons, usually occurs as a manifestation of sepsis by A. Escherichia coli B. Streptococcus faecalis C. Clostridium septicum D. Pseudomonas aeruginosa 18. Granuloma inguinale is caused by the gram negative bacterium A. Calymmatobacterium granulomatis B. Haemophilus ducreyi C. Bartonella henselae D. Yersinia pestis 19. A rare form of secondary syphilis presenting with severe ulcerations, pustular or rupioid lesions accompanied by severe constitutional symptoms is known as A. Condylomata lata B. Pustular syphilid C. Lues maligna D. Rupial syphilid

20. The drug of choice for treatment of all stages of syphilis remains A. Penicillin B. Erythromycin C. Tetracycline D. Cephalosporin 21. The antinucleolar pattern of antinuclear antibody (ANA) is considered most specific for A. Systemic lupus erythematosus B. Scleroderma C. Sjogren’s syndrome D. Dermatomyositis 22. In infants, yellow or brown scaling lesions on the scalp known as cradle cap are manifestation of A. Atopic dermatitis B. Seborrheic dermatitis C. Psoriasis D. Reiter’s syndrome 23. As a rule, pityriasis alba is asymptomatic and chiefly occurs in A. Infancy B. Children and teenagers C. Adults D. Old age 24. The primary lesions of ____________ are characteristic, almost pathognomonic, small, violaceous, flat topped polygonal papules A. Lichen planus B. Psoriasis C. Pityriasis rosea D. Pityriasis rubra pilaris 25. Much evidence suggests that liberation of free fatty acids by the metabolic activity of _______________ is a major factor in the genesis of acne vulgaris A. Pityrosporum ovale B. Proteus C. Propionibacterium acnes D. Pseudomonas aeruginosa

Multiple Choice Questions 26. Cellulitis is a suppurative inflammation involving particularly the subcutaneous tissue caused most frequently by A. Escherichia coli B. Proteus C. Pseudomonas aeruginosa D. Streptococcus pyogenes or Staphylococcus aureus 27. Chancroid is an infectious, contagious, ulcerative, sexually transmitted disease caused by A. Treponema pallidum B. Hemophilus ducreyi C. Calymmatobacterium granulomatis D. Chlamydia trachomatis 28. Adenopathy above and below Poupart’s ligament produces the characteristic but not diagnostic, groove sign in A. Chancroid B. Primary chancre C. Donovanosis D. Lymphogranuloma venereum 29. A diagnosis of _________ must be considered in any patient with neurologic and cutaneous lesions A. Pityriasis alba B. Pityriasis versicolor C. Vitiligo D. Leprosy 30. The most effective drug for the treatment of dermatitis herpetiformis is A. Dapsone B. Sulfasalazine C. Colchicine D. Tetracycline 31. Button hole sign is seen in _________ A. Dermatofibroma B. Neurofibroma C. Lipoma D. Trichilemmal cyst 32. X-linked ichthyosis is associated with deficiency of A. Steroid sulphatase B. Aromatase

C. Phosphorylase D. Tyrosinase 33. Satellite macules are characteristic of_________ A. Tuberculoid leprosy B. Borderline tuberculoid leprosy C. Borderline borderline leprosy D. Borderline lepromatous leprosy 34. Larva currens is caused by__________ A. Ascariasis B. Toxocara C. Strongyloides D. Ancylostoma 35. Circle of Hebra is associated with A. Scabies B. Pediculosis C. Myiasis D. Psoriasis 36. The intraepidermal split in pemphigus vulgaris due to acantholysis is ______ A. Subcorneal B. Stratum spinosum C. Suprabasal D. Subepidermal 37. Munro microabscess, a histopathological feature of psoriasis is seen in A. Stratum corneum B. Stratum spinosum C. Stratum granulosum D. Stratum basale 38. Dennie Morgan fold is a clinical feature of_________ A. Seborrheic dermatitis B. Prurigo simplex C. Atopic dermatitis D. Allergic contact dermatitis 39. Heliotrope rash is a characteristic periorbital rash of ____________ A. Dermatomyositis B. Scleroderma C. Sjogren’s syndrome D. Systemic lupus erythematosus

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Essentials in Dermatology 40. Red grains of mycetoma are produced by_____________ A. Actinomadura madurai B. Actinomadura pelletieri C. Nocardia D. Streptomyces somaliensis 41. Ultra structurally, Langerhans’ cells are characterized by a folded nucleus a n d distinct intracytoplasmic organelles called A. Keratohyaline granules B. Birbeck granules C. Melanosomes D. Odland bodies 42. Mast cell granules stain metachromatically because of their high content of A. Heparin B. Histamine C. Tryptase D. Prostaglandin D2 43. Variously sized, circumscribed changes in skin colour without elevation or depression are named as A. Nodules B. Papules C. Wheals D. Macules 44. Chancroid is an infectious contagious, ulcerative sexually transmitted disease caused by A. Treponema pallidum B. Chlamydia trachomatis C. Herpes simplex virus D. Haemophilus ducreyi 45. A neurosis characterized by an abnormal urge to pull out the hair is known as A. Dermatothlasia B. Dysmorphophobia C. Trichotillomania D. Neurotic excoriation 46. Sixty percent of cases of atopic dermatitis present in the A. First year of life

B. Second year of life C. Third year of life D. Fourth year of life 47. Urticaria elicited by water or sea water at any temperature is called as A. Adrenergic urticaria B. Cold urticaria C. Solar urticaria D. Aquagenic urticaria 48. Antinuclear antibody (ANA) test is positive in about a third of all connective tissue disorders but in 95% of cases of A. Systemic sclerosis B. SLE C. Dermatomyositis D. Sjogren’s syndrome 49. Bullous impetigo is caused by A. Staphylococcus aureus B. Streptococcus pyogenes C. Pseudomonas aeruginosa D. Proteus 50. The presence of Donovan bodies in the indolent serpiginous ulcerations of the groins, pubes, genitalia and anus permit the diagnosis of A. Lymphogranuloma venereum B. Chancroid C. Granuloma inguinale D. Primary syphilitic chancre 51. The presence of either generalized Kaposi’s sarcoma or ——— is sufficient for the diagnosis of AIDS for surveillance purposes A. Oropharyngeal candidiasis B. Cryptococcal meningitis C. Extrapulmonary tuberculosis D. Invasive cervical cancer 52. ——— almost specific for clinical presentation of HIV infection A. Norwegian scabies B. Seborrheic dermatitis

Multiple Choice Questions C. Reiter’s syndrome D. Oral hairy leukoplakia 53. Zidovudine, an antiretroviral agent is A. Nucleoside reverse transcriptase inhibitor B. Non nucleoside reverse transcriptase inhibitor C. Nucleotide analogue D. Protease inhibitor 54. Indinavir, an antiretroviral agent is A. Nucleoside reverse transcriptase inhibitor B. Non nucleoside reverse transcriptase inhibitor C. Nucleotide inhibitor D. Protease inhibitor 55. The most important adverse effect of zidovudine is A. Vomiting B. Headache C. Bone marrow toxicity D. Myopathy 56. Most commonly used non-nucleoside reverse transcriptase inhibitor in India is A. Delaverdine B. Nevirapine C. Efavirenz D. Tenofovir 57. Which antiretroviral drugs are associated with body fat redistribution? A. Nucleoside reverse transcriptase inhibitor B. Non nucleoside reverse transcriptase inhibitor C. Nucleotide inhibitor D. Protease inhibitor 58. Dark field microscopy is used to identify— ——— in serous fluid from lesions of primary and secondary syphilis A. Treponema pallidum

B. Neisseria gonorrheae C. Trichomonas vaginalis D. Hemophilus ducreyi 59. Presence of “clue cells” in vaginal discharge Gram stained smear is highly diagnostic of A. Candidiasis B. Bacterial vaginosis C. Trichomoniasis D. Donovanosis 60. Wet mount is mainly used for the diagnosis of A. Bacterial vaginosis B. Candidiasis C. Trichomoniasis D. Donovanosis 61. Which test is indicated for the smear diagnosis of genital herpes A. Gram stained smear B. Wet mount C. Tzanck smear D. KOH wet mount 62. Whiff test or Sniff test is diagnostic of A. Candidiasis B. Bacterial vaginosis C. Trichomoniasis D. Gonorrhea 63. Acetic acid test is used to detect the subclinical genital ————— infection A. Human papilloma virus infection B. Herpes simplex virus infection C. Donovanosis D. Molluscum contagiosum 64. Dory flap sign due to hard chancre on the inner surface of prepuce is a feature of A. Donovanosis B. Chancroid C. Herpes genitalis D. Primary chancre 65. Wide spread eruptions of secondary syphilis are characteristically A. Non vesicular and non pruritic

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Essentials in Dermatology B. Vesicular C. Pruritic D. Vesicular and pruritic 66. The commonest sites of condyloma lata are perianal, vulva and A. Perineal region B. Angles of mouth C. Axillae D. Between toes 67. Lues maligna, an explosive form of syphilis is also known as A. Pustular syphilide B. Malignant syphilis C. Papular syphilide D. Macular syphilide 68. The fetus may be born apparently normal, but develops signs of early congenital syphilis during the first few weeks or months, called as A. Diday’s law B. Kassowitz law C. Colle’s law D. Profeta’s law 69. Syphilitic infant may not infect its own mother but is capable of infecting others. This observation is known as A. Colle’s law B. Profeta’s law C. Diday’s law D. Kassowitz law 70. Hutchinson’s triad of late congenital syphilis consists of interstitial keratitis, Hutchinson’s teeth and———— A. Clutton’s joints B. Wimberger’s sign C. Eighth nerve deafness D. Saber tibia 71. Parenteral———— is the treatment of choice for the treatment of all stages of syphilis A. Sulphonamide B. Penicillin C. Cephalosporins D. Macrolides

72. Jarisch- Herxheimer reaction occurs most frequently with greater severity in early syphilis more so in A. Primary syphilis B. Secondary syphilis C. Neurosyphilis D. Tertiary syphilis 73. Chancroid, a genital ulcer disease is caused by A. Treponema pallidum B. Haemophilus ducreyi C. Chlamydia trachomatis D. Calymmatobacterium granulomatis 74. The following is not true of chancroid A. The bubo is a multilocular abscess B. The overlying skin is erythematous and shiny C. If untreated, the bubo ruptures to form a single sinus D. The opening of the sinus may enlarge to form a giant ulcer 75. The following is true of donovanosis except A. The ulcer is usually painless, beefy red in color and bleeds easily on touch B. Genitalia most common site involved C. Lymph nodes are usually involved D. Phimosis or lymphedema of distal tissues is common in active phase of the disease 76. The identification of intracellular ——— inside large mononuclear cells is the gold standard for the diagnosis of granuloma inguinale A. Molluscum bodies B. Kamino bodies C. Donovan bodies D. Henderson Paterson bodies 77. —————— is the most common manifestation of gonococcal infection in males A. Anterior urethritis

Multiple Choice Questions B. Posterior urethritis C. Prostatitis D. Epididymorchitis 78. Lymphogranuloma venerum is a sexually transmitted infection caused by A. Chlamydia psittacii B. Chlamydia pneumoniae C. Chlamydia pecorum D. Chlamydia trachomatis 79. Groove sign of Greenblat due to femoral and inguinal lymph node enlargement is a feature of ………… A. Chancroid B. Primary chancre C. Donovanosis D. Lymhogranuloma venereum 80. Esthiomene due to chronic lymphatic edema of the vulva is a late complication of A. Donovanosis B. Lymhogranuloma inguinale C. Chancroid D. Primary chancre 81. The following is true of lymhogranuloma venereum bubo except A. Genital ulcer not present B. Bilateral in 1/3 cases C. Unilocular suppurative swelling D. Rupture to form multiple sinuses 82. The commonest cause of vaginal discharge in women in India is A. Bacterial vaginosis B. Trichomoniasis C. Candidiasis D. Gonorrhoea 83. Clue cells are———— covered with G. vaginalis A. Polymorphonuclear cells B. Vaginal epithelial cells

C. Macrophages D. Monocytes 84. Tzanck smear from the base of a vesicle or erosion of herpes genitalis may show A. Foreign body giant cells B. Langhan’s giant cells C. Touton giant cells D. Multinucleate giant cells 85. The definitive means for diagnosis of herpes genitalis is A. Tzanck smear B. Histopathology C. Serological study D. Viral culture 86. Anogenital warts are frequently caused by human papilloma virus types A. 6, 11 B. 16, 18 C. 72, 73 D. 33, 35 87. Trichomonas vaginalis in females, commonly infects A. Urethra B. Vaginal epithelium C. Bartholin’s glands D. Skene’s glands 88. ————— is the gold standard for the diagnosis of trichomoniasis A. Wet mount B. Culture C. PCR Test D. Urine sedimentation exam 89. The incubation period of gonrrhea ranges from A. 1 to 14 days B. 14 to 28 days C. 28 to 42 days D. 42 to 56 days

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Essentials in Dermatology 90. The incubation period of donovanosis ranges from A. 3 days to 3 months B. 3 months to 6 months C. 6 months to 9 months D. 9 months to 1 year 91. Donovanosis, a chronic destructive and slowly progressive disease is caused by A. Haemophilus ducreyi B. Gardnerella vaginalis C. Calymmatobacterium granulomatis D. Chlamydia trachomatis 92. Locomotor ataxia, a late manifestation of syphilis due to parenchymatous involvement of the spinal cord is called A. General paralysis of insane B. Tabes dorsalis C. Meningovascular syphilis D. Syphilitic amyotrophy 93. The incubation period of syphilis is A. 1-9 days B. 9-90 days C. 90-100 days D. > 100 days 94. Calymmatobacterium granulomatis is a — —— intracellular bacterium A. Gram negative B. Gram positive C. Gram variable D. Acid fast 95. The primary site of infection of gonorrhea in females is A. Urethra B. Vagina C. Vulva D. Endocervical canal

98. A linear loss of epidermis and dermis with sharply defined nearly vertical wall is known as A. Erosion B. Ulcer C. Excoriation D. Fissure 99. A small superficial keratin cyst with no visible opening is called as A. Comedone B. Milia C. Cyst D. Nodule 100. Eczema that appears as one or several coin shaped plaques is called as A. Nummular eczema B. Stasis eczema C. Asteatotic eczema D. Hyperkeratotic eczema 101. A distinctive reaction pattern of eczema presenting as symmetric vesicular hand and foot dermatitis is A. Finger tip eczema B. Hyperkeratotic eczema C. Pompholyx D. Housewife eczema 102. ———— occurs after excess drying, especially during winter months and among the elderly A. Stasis eczema B. Asteatotic eczema C. Nummular eczema D. Hyperkeratotic eczema

96. A circumscribed, flat discoloration that may be brown, blue, red or hypopigmentated is called as A. Macule B. Papule C. Plaque D. Nodule

103. Sensitization to ————is the leading cause of allergic contact dermatitis worldwide A. Mercury B. Chromium C. Nickel D. Gold

97. A circumscribed collection of free fluid up to 0.5 cm in diameter is known as A. Bulla B. Pustule C. Abscess D. Vesicle

104. The most common cosmetic allergen is……. A. Fragrance B. Preservative C. Emulsifier D. Hair dye

Multiple Choice Questions 105. —————— is a chronic, pruritic eczematous disease that nearly always begins in childhood and follows a remitting flaring course A. Seborrhoeic dermatitis B. Allergic contact dermatitis C. Irritant contact dermatitis D. Atopic dermatitis 106. —————is increased in the serum of many patients with atopic dermatitis A. IgA B. IgG C. IgM D. IgE 107. Infants typically develop the first sign of inflammation in atopic eczema during the—————————of life A. First month B. Second month C. Third month D. Fourth month 108. The most common and characteristic appearance of atopic dermatitis in childhood phase is inflammation in——— —— A. Extensor areas B. Flexural areas C. Facial areas D. Palms and soles 109. ——— is an important associated feature of the atopic state A. Dry skin B. Ichthyosis vulgaris C. Keratosis pilaris D. Pityriasis alba 110. ——— is the most common physical urticaria A. Solar urticaria B. Pressure urticaria C. Heat urticaria D. Dermographism 111. Ultra structurally, ——— are characterized by a folded nucleus and distinct intracytoplasmic organelles, Birbeck granules A. Keratinocytes B. Langerhans’ cells C. Fibroblasts D. Melanocytes

112. ——— in the epidermis function primarily in the afferent limb of immune response by providing for the recognition, uptake, processing and presenting of antigens to sensitized T lymphocytes A. Keratinocytes B. Melanocytes C. Langerhans’ cells D. Fibroblasts 113. Specialized aggregates of smooth muscle cells found between arterioles and venules are called as ——— A. Arrectores pilorum B. Tunica dartos C. Glomus bodies D. Vater-Pacini corpuscles 114. ——— are evanescent, edematous, plateau like elevations of various sizes A. Wheals B. Plaques C. Patches D. Macules 115. Erythema, edema and a purple (heliotrope) discoloration of the eyelids are indicative of ——— A. Systemic sclerosis B. Polymyositis C. Dermatomyositis D. SLE 116. ——— presents with intense pruritic vesicular eruption associated with a usually asymptomatic gluten sensitive enteropathy A. Bullous pemphigoid B. Dermatitis herpetiformis C. Linear IgA dermatosis D. Pemphigus herpetiformis 117. ——— is the most common form of photosensitivity A. Actinic prurigo B. Solar urticaria C. Polymorphous light eruption D. Hydroa vacciniforme

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Essentials in Dermatology 118. ——— are so named because they recur at the same site with each exposure to the medication A. Erythema multiforme B. Fixed drug reactions C. Toxic epidermal necrolysis D. Lichenoid reactions 119. Typical target lesions of erythema multiforme are best observed on the —— A. Palms and soles B. Face C. Trunk D. Extremities 120. Target lesions typically appear symmetrically and acrally, initially most frequently on the dorsal hands in ——— A. Fixed drug reactions B. Erythema multiforme C. Toxic epidermal necrolysis D. Lichenoid reactions 121. Typical erythema multiforme minor is usually associated with——— A. Varicella zoster virus infection B. Mycoplasma pneumoniae C. Radiation therapy D. Herpes simplex infection

125. ——— are associated with a syndrome that includes venous thrombosis, arterial thrombosis, spontaneous abortions, and thrombocytopenia in SLE A. Anti-La antibodies B. Anti-Ro antibodies C. Anti Sm antibodies D. Antiphospholipid antibodies 126. ——— is a generalized disorder of connective tissue in which there is fibrous thickening of the skin combined with fibrosis and vascular abnormalities in certain internal organs A. SLE B. Progressive systemic sclerosis C. Dermatomyositis D. Generalized morphea 127. ——— involvement (chiefly atony) is seen in more than 90 % of patients of progressive systemic sclerosis A. Esophageal B. Musculoskeletal C. Pulmonary D. Cardiovascular

122. ———are probably most frequent causes of urticaria and angioedema A. Drugs B. Food C. Infections D. Neoplasms

128. ANA testing is positive in more than 90% of patients with ——— A. Systemic scleroderma B. Morphea C. Discoid lupus erythematosus D. Eosinophilic fascitis

123. The safest and most beneficial systemic therapy in discoid lupus erythematosus is……. A. Dapsone B. Antimalarials C. Corticosteroids D. Azathioprine

129. On removal of the micaceous scales from scaly plaques, bleeding points appear (Auspitz’s sign) in ………. A. Parapsoriasis B. Pityriasis rosea C. Psoriasis D. Pityriasis capitis

124. The characteristic butterfly malar erythema is seen in patients with ——— A. Sjogren’s syndrome B. Scleroderma C. Dermatomyositis D. SLE

130. The characteristic nail changes of psoriasis are numerous ——— A. Pits B. Oil spots C. Onycholysis D. Subungual debris

Multiple Choice Questions 131. Goeckerman technique is an effective and frequently gratifying method of treatment in ——— A. Lichen planus B. Psoriasis C. Pityriasis rosea D. Lichen nitidis 132. ——— is a characteristic clinical triad consisting of urethritis, conjunctivitis, and arthritis A. Behcet’s syndrome B. Hutchinson’s triad C. Epiloia D. Reiter’s syndrome 133. ——— is a chronic skin disease characterized by small follicular papules, disseminated yellowish pink scaling patches and often, solid confluent palmoplantar keratoderma A. Pityriasis rosea B. Pityriasis rubra pilaris C. Pityriasis capitis D. Pityriasis alba 134. The primary lesions of ——— are characteristic, almost pathognomonic, small, violaceous, flat topped, polygonal papules A. Lichen planus B. Psoriasis C. Pityriasis rosea D. Pityriasis rubra pilaris 135. Pterygium formation is characteristic of — —— of the nails A. Psoriasis B. Lichen planus C. Lichen nitidis D. Lichen striatus 136. ——— is a chronic inflammatory disease of the pilosebaceous follicles, characterized by comedones, papules, pustules, cysts, nodules, and often scars A. Seborrhoeic dermatitis

B. Pityriasis capitis C. Seborrhea D. Acne vulgaris 137. ——— is a perifollicular, chronic, pustular staphylococcal infection of the bearded region A. Sycosis barbae B. Folliculitis C. Furunculosis D. Carbuncle 138. ——— is an ulcerative staphylococcal or streptococcal pyoderma, nearly always of the shins or dorsal feet A. Cellulitis B. Impetigo bullosa C. Impetigo contagiosa D. Ecthyma 139. Sharply delineated, dry, brown, slightly scaling patches in the intertriginous areas of erythrasma are caused by——— A. Corynebacterium diphtheriae B. Micrococcus sedantarius C. Clostridium perfringens D. Corynebacterium minutissimum 140. ——— test give quantitative as well as qualitative results and can be used to monitor response to therapy of syphilis A. VDRL B. FTA-ABS C. TPHA D. TPI 141. Which of the vascular anomaly disappears by first birthday A. Salmon patch B. Portwine stain C. Capillary haemangioma D. All of the above 142. Casal’s collar, a hyperpigmented dermatosis in the “V” area of neck is seen in A. Ariboflavinosis B. Pellagra C. Scurvy D. Rickets’ disease

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Essentials in Dermatology 143. Darier’s sign is demonstrated in the skin lesions of A. Urticaria pigmentosa B. Lichen planus C. Psoriasis vulgaris D. All of the above

150. Cutaneous signs of myxedema includes all except A. Madarosis B. Dry skin C. Macroglossia D. Increased sweating

144. Hutchinson’s triad of congenital syphilis consists of all except A. Interstitial keratitis B. Deafness C. Saddle nose deformity D. Notched Peg shaped incisor teeth

151. Café-au-lait macules are seen in A. Neurofibromatosis B. McCune Albright syndrome C. Small percentage of normal population D. All of the above

145. Malignant melanoma transformation is considered in a melanocytic naevus when there is A. Change in colour B. Change in shape C. Change in size D. All of the above 146. Pterygium unguis is a feature of A. Psoriasis vulgaris B. Lichen planus C. Alopecia areata D. All of the above 147. Trichrome sign is observed in A. Vitiligo B. Naevus achromicus C. Piebaldism D. All of the above 148. Drugs exacerbating psoriasis includes A. Lithium B. Beta blockers C. Gold D. All of the above 149. Munro’s microabscess, a collection of neutrophils, a pathognomonic pathological change is demonstrated in A. Stratum corneum B. Stratum basale C. Stratum germinativum D. Stratum spinosum

152. Pitting of nails is observed in A. Psoriasis B. Alopecia areata C. Both A and B D. None 153. Maculae cerulae is classically seen with A. Pediculosis pubis B. Pediculosis corporis C. Pediculosis capitis D. None of the above 154. ——— is due to subepidermal blistering A. Pemphigus vulgaris B. Bullous impetigo C. Bullous pemphigoid D. All of the above 155. Nikolsky’s sign is classically positive in A. Pemphigus foliaceous B. Bullous pemphigoid C. Dermatitis herpetiformis D. Linear IgA dermatosis 156. For lupus vulgaris, a form of cutaneous tuberculosis, true is A. Presence of apple jelly nodules on diascopy B. Advancing active edge with trailing scar C. Strongly positive Mantoux test D. All of the above

Multiple Choice Questions 157.In secondary syphilis, all are seen except A. Maculopapular rash B. Low VDRL titre C. Condyloma lata D. Moth eaten alopecia 158. Following is true for herpes zoster except A. Occurs as a result of reactivation of varicella zoster virus B. Grouped vesicles develop along a dermatome C. This reactivated infection is noninfectious D. Dose of acyclovir used is 800 mg 5 times a day for 7-10 days 159. True about pityriasis rosea is A. The skin lesions classically spares the trunk B. Long axis of the oval lesions is perpendicular to ribs C. It is self limiting disease D. Fine powdery scales on the surface of skin lesions are characteristic 160. The following is true about erythrasma except A. Caused by Corynebacterium minutissimum B. Usually symptomatic C. Coral red fluorescence on Wood’s lamp examination D. Can be treated with topical antifungals like azoles 161. Chancroid, an infectious, contagious, ulcerative sexually transmitted disease is caused by A. Chlamydia trachomatis B. Calymmatobacterium granulomatis C. Treponema pallidum D. Haemophilus ducreyi 162. Tinea nigra is caused by A. Trichosporon beigelii B. Piedrai hortai

C. Exophiala phaeoanellomyces D. Exophiala jaenselmi 163. Wavelength of Wood’s light is A. 365 nm B. 368 nm C. 370 nm D. 380 nm 164. Wickham’s striae is seen in A. Psoriasis B. Lichen simplex chronicus C. Lichen scrofulosorum D. Lichen planus 165. Mother patch is the initial lesion of A. Pityriasis rosea B. Pityriasis lichenoides chronica C. Pityriasis alba D. Psoriasis 166. Deep seated vesicles resembling tapioca over the palms and soles characterize A. Bullous pemphigoid B. Acropustulosis C. Pompholyx D. Keratolysis exfoliativa 167. Keratoderma blennorrhagicum is a feature of A. Ritter’s disease B. Psoriasis C. Reiter’s disease D. Pityriasis rubra pilaris 168. Goeckerman’s technique consisting of topical coal tar use and UV radiation is used for the management of A. Pemphigus B. Psoriasis C. Lymphoma D. Parapsoriasis 169. Auspitz’s sign is pathognomonic of A. Lichen planus B. Reiter’s disease C. Psoriasis D. Secondary syphilis

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Essentials in Dermatology 170. Munro micro abscesses in psoriasis are seen in A. Stratum corneum B. Stratum basale C. Stratum spinosum D. Stratum granulosum 171. Koebner’s response is seen in all except A. Psoriasis B. Lichen planus C. Lichen nitidus D. Lichen striatus 172. Neck sign is typically observed in A. Scleroderma B. Eosinophilic fascitis C. Porphyria cutanea tarda D. Graft versus host disease 173. Which antibody is significantly associated with neonatal lupus erythematosus A. Anti Ro B. Anti Sm C. Anti ds DNA D. Anti U1 RNP 174. Carpet tack sign is characteristic of A. Discoid lupus erythematosus B. Systemic lupus erythematosus C. Dermatomyositis D. Psoriasis 175. Gottron’s sign is pathognomonic of A. Polymyositis B. Dermatomyositis C. Sjogren’s syndrome D. Systemic lupus erythematosus 176. The “target” or “iris” lesion with three zones is classic of A. Erythema palmare B. Erythema marginatum C. Erythema multiforme D. Erythema repens 177. All are components of lichenification except A. Thickening B. Pigmentation C. Excoriation D. Increased skin markings

178. Buruli ulcer is caused by A. M ulcerans B. M kansasii C. M avium D. M intracellulare 179. Satellite lesions around the lesion are characteristic of A. Tuberculoid tuberculoid leprosy B. Borderline tuberculoid leprosy C. Boderline lepromatous leprosy D. Lepromatous leprosy 180. Treatment of choice for type 2 lepra reaction is A. Steroids B. Clofazimine C. Pentoxyphylline D. Thalidomide 181. ____________ produces more cases of allergic contact dermatitis than all other metals combined A. Cobalt B. Copper C. Gold D. Nickel 182. The leading cause of allergic contact dermatitis associated with cosmetics is from A. Fragrances B. Preservatives C. Hair dyes D. Hair shampoos 183. Erythema nodosum is a reaction process commonly associated with A. Tuberculosis B. Streptococcal infection C. Yersinia D. Salmonella 184. Mycobacterium leprae is most effectively cultivated in A. Mouse foot pad B. Armadillos C. Primates D. Slander lorris 185. In a leprosy patient, if organisms are found on skin smears, the patient is said to have A. Multibacillary leprosy B. Paucibacillary leprosy C. Determinate leprosy D. Indeterminate leprosy

Multiple Choice Questions 186. A 5-year old boy presents with multiple bizarre patches of hair loss (hairs broken at different lengths, no scarring) over vertex and parietal region of the scalp. What is your diagnosis? A. Trichotillomania B. Alopecia areata C. Tinea capitis D. Congenital syphilis 187. Dennie Morgan fold is seen in A. Atopic dermatitis B. Seborrheic dermatitis C. Infected eczematoid dermatitis D. Diaper dermatitis 188. A 30-year old female had erythematous plaques on the scalp with adherent scales extending into the dilated follicles to give positive Carpet-tack sign. Diagnosis is A. Seborrheic dermatitis B. Psoriasis C. Discoid lupus erythematosus D. Erythroderma 189. A 40-year old man complains of recurrent episodes of painful erosions over the glans penis healing with slate grey pigmentation. He suffers from migraine and often takes various painkillers. Diagnosis is A. Erythema multiforme B. Fixed drug eruption C. Herpes genitalis D. Candidiasis 190. Koebner phenomenon is seen inA. Psoriasis B. Lichen planus C. Vitiligo D. All of the above 191. Sign of Lesar Trelat refers to sudden eruptive appearance of following lesions associated with an underlying malignancy A. Ulcerations B. Seborrheic keratoses C. Urticarial vasculitis D. Erythema multiforme

192. Trichrome sign is seen inA. Psoriasis B. Lichen planus C. Vitiligo D. Ichthyosis vulgaris 193. A 40-year-old man presents unilaterally distributed painful vesicles on chest in a linear fashion of herpes zoster. Tzanck smear characteristically shows A. Neutrophils B. Eosinophils C. Acantholytic cells D. Multinucleated giant cells 194. Incubation period of herpes genitalis is around A. 2-7 days B. 7-14 days C. 14 days -6 weeks D. More than 6 weeks 195. A 25-year old male patient presents with a painful necrotic ulcers with non-indurated base and unilateral painful suppurative lymph node enlargement. The likely causative organism is A. Haemophilus ducreyi B. Calymmatobacterium granulomatis C. Herpes simplex virus D. Chlamydia trachomatis 196. Erythema nodosum leprosum occurs due to which type of hypersensitivity reactionA. Type I B. Type II C. Type III D. Type IV 197. WHO treatment of multibacillary leprosy with three drugs is A. 12 months of antileprosy treatment in 18 months B. 12 months of antileprosy treatment in 15 months C. 9 months of antileprosy treatment in 12 months D. 6 months of antileprosy treatment in 9 months

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Essentials in Dermatology 198. A 60-year old man presents with itchy tense blisters mainly on abdomen and upper limbs, the Tzanck smear shows a predominance of eosinophils and Nikolsky’s sign is negative, so what is your diagnosis A. Bullous pemphigoid B. Dermatitis herpetiformis C. Pemphigus foliaceus D. Pemphigus vulgaris 199. A 20-year-old male presents with well defined hypopigmented, slightly itchy macules on the trunk, with very fine branny scales of tinea versicolor. Wood’s lamp examination fluorescence will be A. Yellowish brown B. Brilliant green C. Pale green D. Blue 200. On diascopy, apple jelly nodules are seen in A. Lichen planus B. Lupus vulgaris C. Neurofibromatosis D. Sarcoidosis 201. Moth-eaten alopecia is irregularly distributed alopecia of the scalp, seen in 5% of patients with A. Tinea capitis B. Secondary syphilis C. Alopecia areata D. Androgenetic alopecia 202. Gottron’s papules (flat topped violaceous papules over the knuckles) are pathognomonic of A. Dermatomyositis B. Scleroderma C. SLE D. Sjogren’s syndrome

203. Most characteristic nail finding of psoriasis is A. Pitting B. Subungular hyperkeratosis C. Salmon patch D. Ridging 204. Treatment of acne vulgaris includes all except A. Isotretinoin B. Benzoyl peroxide C. Adapalene D. Anthralin 205. A 6-month-old baby presented with periorificial erosions, periungual erythema, scaling and pustules of 1 week duration. Child also has diarrhea and generalized alopecia. The most probable cause is deficiency of – A. Niacin B. Zinc C. Biotin D. Selenium 206. Berloque (Perfume) dermatitis is seen most frequently on the sides of the A. Neck B. Shoulders C. Breasts D. Face 207. Relatively immune to dermatophyte infection in adults is A. Trunk B. Face C. Hands D. Scrotum 208. The leading cause of allergic contact dermatitis associated with cosmetics is from A. Preservatives B. Fragrances C. Paraphenylene diamine D. Hair bleaches

Multiple Choice Questions 209. Round, circumscribed, erythematous, dry scaling plaques of various sizes covered by silvery white, imbricated and lamellar scales characterize A. Lichen planus B. Psoriasis C. Pityriasis rosea D. Seborrheic dermatitis 210. Small, violaceous, flat topped, polygonal papules are characteristic, almost pathognomonic of A. Psoriasis B. Pityriasis rosea C. Lichen planus D. Seborrheic dermatitis 211. Perifollicular, chronic, pustular staphylococcal infection of the bearded region is known as A. Sycosis vulgaris B. Psoriasis vulgaris C. Lupus vulgaris D. Lupus erythematosus 212. An ulcerative staphylococcal or streptococcal pyoderma, nearly always affecting the shins or dorsal feet is A. Necrotizing fascitis B. Tropical ulcer C. Desert sore D. Ecthyma 213. Treatment of choice in erythrasma is A. Tolnaftate solution B. Oral erythromycin C. Topical clindamycin D. Oral tetracycline 214. Ecthyma gangrenosum is usually a manifestation of sepsis due to A. Streptococcus faecalis B. Proteus C. Pseudomonas aeruginosa D. Bacteroides

215. Bacillary angiomatosis occurs primarily in the settings of immunosuppression, especially A. AIDS B. Lymphoma C. Leukaemia D. Immunosuppressive therapy 216. A sexually transmitted disease that is characterized by suppurative inguinal adenitis with matted lymph nodes, inguinal bubo with secondary ulceration, and constitutional symptoms is A. Granuloma inguinale B. Chancroid C. Primary syphilis D. Lymphogranuloma venereum 217. The causative fungus for tinea imbricata is A. T rubrum B. T mentagrophytes C. T concentricum D. T tonsurans 218. Mycobacterium ulcerans is the cause of A. Swimming pool granuloma B. Buruli ulcer C. Lupus vulgaris D. Tuberculous chancre 219. A dory flop phenomenon seen in a sore in the prepuce is typical of A. Chancroid B. Granuloma inguinale C. Primary syphilitic chancre D. Lymphogranuloma venereum 220. The most common procedure used in the office for diagnosis of herpes simplex is A. Tzanck smear B. Slit skin smear C. Tissue smear D. Gram’s stained smear 221. The dermatomes most frequently affected by herpes zoster are A. Cervical B. Thoracic C. Lumbar D. Sacral

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Essentials in Dermatology 222. False about acne A. Disease of adolescents B. Comedones are the early lesions in acne C. Fatty food increases the risk of acne D. Propionibacterium acne plays role 223. Which of the following drug is not used in the treatment of single lesion paucibacillary leprosy? A. Rifampicin B. Ofloxacin C. Metronidazole D. Minocycline 224. Which of the following is not true of Norwegian scabies? A. Seen in immunocompromised person B. Itching is severe C. Patient will harbour million of mites D. Methotrexate is one of the treatment 225. Which of the following is highly specific for systemic lupus erythematosus A. LE cell phenomenon B. Anti ds antibody C. Anti Ro antibody D. Anti sm antibody 226. Following is not true about larva migrans A. Larval migration begins 4 days after inoculation B. Topical ivermectin is preferred over systemic therapy C. Majority of cases are caused by Ancylostoma braziliense D. Occurs in people who go barefoot on the beach 227. All of the following drugs are used in the treatment of type 2 lepra reaction except A. Clofazimine B. Corticosteroids C. Rifampicin D. Thalidomide 228. Which of the following is not a topical antifungal agent? A. Terbinafine B. Tioconazole C. Tolnaftate D. Tazarotene

229. Regarding scabies, which of the following statement is false A. Positive diagnosis is made only by the demonstration of the mite under microscope B. Scabies is characterized by pruritic papular lesions, excoriations, and burrows C. Sarcoptes scabiei is the causative agent D. Oral ivermectin therapy is more effective than topical therapy 230. Regarding pediculosis capitis, following statement is false A. Shaving the head may not cure head lice B. Pediculosis capitis is more common in children C. Presents with intense pruritus of the scalp D. Permethrin is the most widely used pediculocide 231. Partial or complete absence of pigment in the skin, hair and eyes or the eyes alone occurs in A. Postinflammatory leukoderma B. Vitiligo C. Albinism D. Piebaldism 232. Preferred form of phototherapy to treat vitiligo is A. Broad band UVB B. Narrow band UVB C. PUVA D. Balaneotherapy 233. C-ANCA is positive in 75% to 80% of patients of A. Churg-Strauss syndrome B. Microscopic polyangitis C. Polyarteritis nodosa D. Wegener granulomatosis

Multiple Choice Questions 234. Untreated Wegener granulomatosis has mean survival time of 5 months and a 90% mortality over A. 1 year B. 2 years C. 3 years D. 4 years 235. Henoch-Schonlein purpura is characterized by purpura, arthralgias, abdominal pain and A. CNS disease B. Pancreatic disease C. Renal disease D. Cardiac disease 236. Fordyce spots are ectopically located A. Apocrine glands B. Eccrine glands C. Sebaceous glands D. Apo-eccrine glands 237. In which alopecia, the anterior hairline recedes on each side, in the “professor angles” so that the forehead becomes high A. Alopecia areata B. Androgenetic alopecia C. Telogen effluvium D. Anagen effluvium 238. The phrase “red as rose in summer and indigo blue in winter” has been ascribed to Osler in describing A. Polycythemia vera B. Cutaneous myelofibrosis C. Hypereosinophilic syndrome D. Leukemia cutis 239. The leukemic phase of mycosis fungoides, a cutaneous T-cell lymphoma is A. Leukaemia cutis B. Lymphomatoid papulosis C. Pagetoid reticulosis D. Sezary syndrome 240. A gyrate serpiginous erythema with characteristic wood grain pattern scales

nearly always associated with an underlying malignancy is A. Erythema elevatum diutinum B. Erythema annulare centrigugum C. Erythema marginatum D. Erythema gyratum repens 241. Trousseau’s sign (migratory thrombophlebitis) is usually associated with A. Gastric carcinoma B. Pancreatic carcinoma C. Colon carcinoma D. Lung carcinoma 242. When cutaneous lesions of mastocytosis are firmly stroked or vigorously rubbed, urticaria with surrounding erythematous flare develops denoting A. Auspitz sign B. Nikolsky’s sign C. Button hole sign D. Darier’s sign 243. The mnemonic of 3 D’s: diarrhea, dementia and dermatitis is used for A. Niacin deficiency B. Biotin deficiency C. Zinc deficiency D. Pyridoxine deficiency 244. Dapsone and gluten free diet are recommended as therapy for A. Bullous pemphigoid B. Dermatitis herpetiformis C. Epidermolysis bullosa acquisita D. Pemphigus vulgaris 245. Direct immunofluorescence of non involved perilesional skin reveals deposits of IgA alone or together with C3 arranged in a granular pattern at dermoepidermal junction in A. Linear IgA bullous dermatosis B. Dermatitis herpetiformis C. Epidermolysis bullosa acquisita D. Pemphigus vulgaris

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MCQs in Dermatology, Sexually Transmitted Diseases and Leprosy for Post-graduate Students

MCQs in Dermatology- Basic sciences 1. All of the following is true about kinogen except A. Seen in bald individuals B. A stage between anagen and catagen C. Follicle remains empty D. Constitute part hair cycle

8. Keratohyline granules are composed of all of the following except A. Profilagrin B. KIF C. Loricrin D. Desmoplakin 9. Which pairs of keratin are expressed in basal layer? A. k1, k10 B. k5, k14 C. k8, k18 D. k4, k14

2. Trigger for initiation of hair cycle lies in A. Hair matrix B. Dermal papilla C. Bulge area just below opening of sebaceous duct D. Hair cortex

10. ‘Spines’ in spinous layer are made up of A. Hemidesmosomes B. Desmosomes C. Desmogleins D. Periplakin

3. Hair cast represents A. Dandruff B. Remnant of inner root sheath C. Remnant of outer root sheath D. Nits

11. One epidermal melanocyte unit is composed of A. 25 keratinocytes B. 31 keratinocytes C. 42 keratinocytes D. 36 keratinocytes

4. Adamson’s fringe represents A. Beginning of keratinization B. Beginning of hair root sheath C. Beginning of infundibulum D. Beginning of bulb 5. Nail thinning is usually a sign of involvement of A. Nail matrix B. Nail bed C. Nail folds D. Hyponychium 6. Nail pitting is because of involvement of A. Proximal matrix B. Distal matrix C. Nail bed D. Nail folds 7. Which of the following is true regarding telogen hair? A. Sticky B. Bears outer sheath C. Club shaped D. Pigmented

12. Earliest development of hairs rudiments occur at the following sites except A. Eyebrows B. Scalp C. Chin D. Upper lip 13. Nail first begins to develop intrauterine at A. 3 months B. 4 months C. 5 months D. 6 months 14. In salt split, skin cleavage is seen at A. Lamina densa B. Lamina lucida C. Lamina fibrosa D. Supra basal

Multiple Choice Questions 15. ‘Odland bodies” are most abundant at which layer of skin A. Stratum corneum B. Stratum granulosum C. Stratum spinosum D. Stratum basale 16. Which layer is absent in nail bed and matrix? A. Stratum basale B. Stratum germinatum C. Stratum granulosum D. Stratum spinosum 17. Grenz zone is seen in all except A. Lepromatous leprosy B. Granuloma faciale C. Dermatofibroma D. Granuloma annulare 18. Following are various types of epidermal degenerations except the following A. Ballooning degeneration B. Hydropic degeneration C. Reticular degeneration D. Hyaline degeneration 19. “Dilapidated brick walls’’ are seen in A. Pemphigus vulgaris B. Pemphigus foliaceous C. Hailey-Hailey’s disease D. Subcorneal pustular dermatosis 20. Dyskeratosis is seen in A. Bowen’s disease B. Solar keratoses C. Basal cell carcinoma D. Squamous cell carcinoma 21. Abrupt keratinisation is seen in A. Pilomatricoma B. Eccrine spiradenoma C. Mucous cyst D. Dermatofibroma 22. “Tadpole like” appearance is seen in histopathology of A. Nevus sebaceous

B. ILVEN C. Syringomas D. Papilloma 23. ‘Miescher’s granuloma’ is seen in histopathology of A. Erythema nodosum B. Erythema induratum C. Nodular vasculitis D. Erythema marginatum 24. Naked granulomas are seen in A. Sarcoidosis B. Tuberculosis C. Histoplasmosis D. Cat-scratch disease 25. All are type- I hypersensitivity reactions except A. Anaphylaxis B. Urticaria C. Contact hypersensitivity D. Allergic rhinoconjunctivitis 26. All are type-II immunological reactions except A. Serum sickness B. SLE C. Complement deficiency D. Eczema 27. Following about skin prick test is true except A. Result available in 15 minutes B. Can be done in patient receiving antihistaminics C. Cheap D. Cannot be done in patient with extensive eczema 28. Type-1 and type-2 cryoglobulins seen in all except A. Myeloma B. Waldenstrom’s macroglobulinaemia C. Lymphoma D. Chronic infection

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Essentials in Dermatology 29. Chronic mucocutaneous candidiasis has all except A. Endocrinopathy B. Norwegian scabies C. Candidiasis of nail D. Malignancy

36. All are neutrophilic defects except A. Chronic granulomatous disease B. Chediak-Higashi syndrome C. Conradi-Smith syndrome D. Cyclic neutropenia

30. Ataxia telangiectasia constitutes all except A. Cutaneous telangiectasia B. Viral and bacterial infection C. T-cell mediated D. B-cell mediated

37. Hyper IgE syndrome has all except A. Eczema B. Staphylococcal infection of skin and lung C. Osteopenia D. Malignancy

31. Chediak–Higashi syndrome consists of all except A. Silver streaks in hair B. Oculocutaneous albinism C. NK cell D. Cutaneous pigmentation

38. Homogenous nuclear staining seen in all except A. SLE B. Drug induced LE C. Dermatomyositis D. Systemic sclerosis

32. Dyskeratosis congenita has all except? A. Cutaneous pigmentosis B. Nail dystrophy C. Oral leukoplakia D. Hyperhidrosis

39. In scleroderma, all are seen all except A. Speckled nuclear staining B. Nucleolar staining C. Centromere staining D. Ribosomal staining

33. Epidermolytic hyperkeratosis has all except A. Increase in thickness of granular layer B. Keratinocytes contain C. Perinuclear vacuolation D. Distinct cell body

40. Satellite cell necrosis is seen in all except A. Herpes zoster infection B. Erythema multiforme C. Steven Johnson D. TEN

34. Toll like receptor antagonist is useful in the treatment of all except? A. Acne B. Acute inflammation C. Chronic inflammation D. Viral warts 35. All are B-cell defects EXCEPT A. X-linked agammaglobulinaemia B. M- chain deficiency C. Hyper IgE syndrome D. X-linked hyper-IgM

41. Neutrophils granules consists of all of the following except A. BPI B. Azurocidin C. Myeloperoxidase D. Histamine 42. ‘Cycloxygenase pathway’ is responsible for formation of all of the following except A. PGD2 B. LTB4 C. PGE2 D. TXA2

Multiple Choice Questions 43. Primary acantholysis is seen in all except A. Pemphigus B. Darier’s disease C. Solar keratoses D. Transient acantholytic dermatosis

49. Apocrine duct open at A. Just above opening of sebaceous duct B. Just below opening of sebaceous duct C. Just above opening of arrector pilorosum D. Just below opening of arrector pilorosum

44. Hypergranulosis is seen all except A. Lichen planus B. Lichen simplex chronicus C. Psoriasis D. Warts

50. Eccrine and apocrine gland are supplied separately by A. Cholinergic and adrenergic fibres B. Adrenergic and cholinergic fibres C. Both D. None of these

45. Necrobiosis is seen in all except A. Granuloma annulare B. Necrobiosis lipoidica C. Rheumatoid nodules D. Sarcoidosis 46. Pautrier microabcess is composed of A. Neutrophils B. Lymphocytes C. Monocytes D. Eosinophils 47. Pacinian corpuscles are located at A. Epidermis B. Dermo-epidermal junction C. Deep dermis and subcutaneous tissue D. Subcutaneous tissue 48. Dermo-epidermal junction is composed of all of the following except A. Desmosomes B. Hemidesmosomes C. Laminin 5 D. Anchoring fibrils

51. Special stain for calcium salt is A. Von kossa B. Von Gieson C. Gomori D. Masson Fontana 52. Masson’s trichome stains the collagen A. Green B. Red C. Yellow D. Purple 53. Which of following is fungal stain? A. Methanamine silver B. Ziehl- Nelson C. Grocott’s D. Gomori’s stain 54. PAS stain which tissue component A. Glycogen B. Elastic fibers C. Collagen D. Reticulin fibers 55. Melanocytic markers are all of the following except A. S-100 B. HMB 45 C. HMB 35 D. Tyrosinase

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MCQs in Clinical Dermatology-Part I 1. “Truck-Driver’s disease” is the other name for A. Hidradenitis suppurativa of perianal region in male B. Tinea cruris C. HIV/AIDS infection D. Chronic folliculitis of the leg 2. True about tropical ulcer is all except A. Results from synergistic bacterial infection. B. Most lesions develop on exposed parts such as legs. C. Tender, enlarged regional lymphnodes is commonly seen. D. There is clustering of cases in the localities.

B. Pheochromocytoma C. Vitamin B12 deficiency D. Still’s disease 7. All are features of FranceschettiJadassohn syndrome except A. Yellowing of teeth B. Hypohidrosis C. Hyperkeratosis of palms and soles. D. Begins by 2 years of age. 8. All are true about tropical acne except A. Occurs in persons working in hot humid climates B. Comedones are the common lesions C. Face is not much involved D. Isotretinoin may be useful

3. True about erythromelalgia all except A. More common in middle-aged and elderly women B. Occurs most commonly in the feet C. Can occur in myeloproliferative disorders D. Individual attack lasts for hours

9. Kligman’s formula includes. A. 5% hydroquinone, 0.1% dexamethasone and 0.1% tretinoin B. 5% hydroquinone, 0.1% dexamethasone and 15% azelaic acid C. 0.1% dexamethasone, 0.1% tretinoin, and 15% azelaic acid D. 15% azelaic acid, 5% hydroquinone, and 0.1% tretinoin.

4. Which of the following is not a feature of acne fulminans? A. Commonly affects boys B. Marked leukocytosis with negative blood culture C. Squamous cell carcinoma is reported in long standing cases D. Osteolytic bone lesions occur

10. Trichotemnomania refers to A. A hair eating disorder B. A psychological disorder where the patient pulls out his or her hair C. A psychological disorder, where the patient cuts his/her hair with scissors D. A psychological disorder, where the patient washes his/her hair repeatedly

5. Suspected causes in the etiopathogenesis of perioral dermatitis are all except A. Use of fluorinated topical steroids. B. Candida C. Staphylococcus aureus D. Demodex mite

11. ‘V’- shaped lunulae are seen in A. Dolichonychia B. Pachyonychia congenita C. Nail patella syndrome D. Yellow nail syndrome

6. Diseases associated with addisonian like hyper pigmentation are all except A. Hepatolenticular degeneration

12. All is true above naevus comedonicus, except A. Hamartoma of the pilosebaceous follicles

Multiple Choice Questions B. Common over the face, neck, upper arm and chest C. Often has a linear configuration D. Spontaneous resolution reported 13. All are sports related dermatological problems except A. Lime burns B. Pyogenic infection. C. Prurigo nodularis D. Friction blister. 14. All are true about dermographism except A. It associated with atopy B. Most common form of physical urticaria C. Usually fades within 30 minutes D. Peak prevalence occurs in second and third decades 15. Valacyclovir is contraindicated in A. Immunocompromised patient B. Urethritis C. Pneumonia D. Thrombocytopenia 16. Erythrasma responds best to A. Oral erythromycin B. Clotrimazole cream C. Keratolytic compounds D. Whitfield ointment

19. Ecthyma contagiosum is a zoonotic infection from which animal? A. Dogs B. Horses C. Sheep D. Rat 20. Literal meaning of “pompholyx” means A. Vesicle B. Sago-grain C. Bubble D. Blister 21. Which type of lesion is not found in dermatitis herpetiformis. A. Vesicle B. Papule C. Bulla D. Nodule 22. Comby’s sign is seen in A. Erythema infectiosum B. Measles C. Rubella D. Scarlet fever 23. Hertoghe’s sign is seen in A. Atopic dermatitis B. Seborrheic dermatitis C. Darier’s disease D. Dermatomyositis 24. Which one of the following is not a cause of trachyonychia A. Psoriasis B. Lichen planus C. Sarcoidosis D. Alopecia areata

17. Drug of first choice for pustular psoriasis with HIV is A. Oral retinoids B. Zidovudine C. Steroids D. Puva

25. Follicular occlusion tetrad does not include A. Pilonidal Sinus B. Dissecting Cellulites of scalp C. Acne inversa D. Acne venenata

18. Which one of the following is not a bacterial infection? A. Ecthyma gangrenosum B. Veldt sore C. Ecthyma contagiosum D. Ecthyma pyogenicum

26. A traveller develops a linear arrangement of urticarial papules on the trunk after spending a night in hotel. What is the most likely cause for this eruption? A. Reduvid bug B. Demodex

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Essentials in Dermatology C. Bed bug D. Pediculosis humanus 27. Muehrcke’s nail (narrow white bands occurring in pairs) seen in A. Chronic hypoalbuminemiae B. Cirrhosis C. Congestive heart failure D. Diabetes mellitus 28. Term “atopy” was coined by A. Russel B. Milan C. Coca D. Fieldman 29. Crowe’s sign is seen in A. Neurofibromatosis B. Ataxia telangiectasia C. Sturge-Weber syndrome D. Tuberous sclerosis. 30. Other name for diabetic dermopathy is A. Rose spots B. Binkley’s spots C. Liver spots D. Forschcimer’s spots 31. Most commonly used (commercial) serotype of botulinum toxin in dermatology is A. A B. B C. C1 D. E 32. Organism implicated in blastomycosis like pyoderma all except A. E.coli B. Proteus C. Pseudomonas D. Corynebacterium 33. Good candidate for hormone therapy for acne vulgaris are all except A. Acne primarily located in lower face or neck B. Acne not responding to other oral/ topical therapies C. Women with polycystic ovarian disease D. Women with early onset acne 34. Cork screw hairs are seen in A. Vitamin B12 deficiency B. Trichotillomania

C. Scurvy D. Woolly hair syndrome 35. Selenium deficiency manifests as A. Woody edema B. Perifollicular petechiae C. Pseudoalbinism D. Seborrheic dermatitis like eruption. 36. Button hole sign is seen inA. Anetoderma B. Cutis laxa C. Linear focal elastosis D. Striae distensae 37. Louis Bar syndrome is the other name of A. Proteus syndrome B. Ataxia-telangiectasia C. von-Hippel-Lindau syndrome D. Pachyonychia congenita 38. Hutchinson’s sign is seen in A. Melanoma in nail matrix B. Blue nevus C. Zosteriform herpes simplex D. Herpetic whitlow 39. Most common color in chromhidrosis is A. Yellow B. Green C. Blue D. Black 40. Pseudo-Hutchinson’s sign is seen in A. Nicotine stain of nail plate B. Bowen’s disease C. Blue nevus D. Melanoma 41. All are true about ‘epidermodysplesia verriciformis’ except A. Inherited susceptibility to HPV B. Malignant changes do not occur C. Lesions resembling pityriasis versicolor may occur. D. Onset may occur at any age 42. ‘Black piedra’ also known as: A. Trichosporiasis nodosa B. Trichomycosis nodularis

Multiple Choice Questions C. Trichomycosis axillaries D. Tinea nigra 43. ALL of the following true about-‘bacillary angiomatosis’ except A. Found commonly in severe immunosuppression like in AIDS patient B. Two bortenella species- B.henselae and B. quintana have been associated C. Lesions closely resemble with pyogenic granulomas D. Drug of choice is ciprofloxacin. 44. Pattern of tinea unguium common with AIDS patients A. Distal and lateral subungal onychomycosis B. Endonyx onychomycosis C. Proximal subungal onychomycosis D. Superficial white onychomycosis 45. Species most frequently associated with pityriasis versicolor A. Malassezia globosa B. M sympodialis C. M restricta D. M obtusa 46. Gilchrist’s disease is another name of A. North American blastomycosis. B. San Joaquin valley fever. C. South American blastomycosis. D. Penicilliosis. 47. Loiasis is also known as A. Hookworm disease B. Larva currens C. African eye worm disease D. Seatworm disease 48. Chrysalis babies is name given toA. Neutral lipid storage disease. B. Congenital psoriasis. C. Babies with intermediate features between harlequin fetus and colloidion baby. D. Self healing colloidion baby.

49. Lotus leaves on water appearance seen in A. Lichen spinulosus. B. Keratosis follicularis squamosa. C. Keratosis follicularis spinulosa decalvans D. Phrynoderma. 50. Mechanism of action of fumaric acid esters in psoriasis appears to be A. Ability to promote secretion of Th2 cytokines( IL-10) B. Inhibition of retinoic acid 4-hydroxylase C. Inhibition of inosine-5’ monophosphatase enzyme D. Blockage of T-and B-cell proliferation 51. Jigsaw-puzzle appearance is seen in the histopathology of A. Syringoma B. Eccrine poroma C. Cylindroma D. Apocrine hydrocystoma 52. All are true about “Meyerson’s naevus” except A. It may be pruritic. B. Also known as Sutton’s naevus C. Assosiated with spongiotic dermatitis in histopathology. D. It is a melanocytic naevus surrounded by inflammatory reactions. 53. Which one of the following not seen in incontinentia pigmenti? A. Chinese figure distribution B. Peg shaped teeth C. Pancytopenia D. Mental retardation 54. All of the following is true regarding Naxos disease except A. Cardiomyopathy B. Epidermolytic palmoplantar keratoderma

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Essentials in Dermatology C. Non epidermolytic palmoplantar keratoderma D. Woolly hair 55. Fish odour syndrome is because of A. Triethylamine B. Trimethylamine C. Carnitine D. Choline 56. Necrosis of sweat glands seen in histopathology of A. Eccrine poroma B. Eccrine nevus C. Neutrophilic eccrine hidradenitis D. Melanocytic nevus 57. Reversal of perception of hot and cold is characteristic of A. Ciguatera fish poisoning B. Ray fish poisoning C. Frost bite D. Snake bite 58. Pigmented hairy epidermal nevus consists of all except A. Becker’s nevus B. Ipsilateral hypoplasia of breast C. Scoliosis D. Cataract 59. Urbach-Wiethe disease is also known as A. Gaucher’s disease B. Lipoid proteinosis C. Fabry’s disease D. Fucosidosis 60. Knuckle pigmentation is seen in deficiency of A. Vitamin B1 B. Vitamin B12 C. Vitamin B3 D. Vitamin B6 61. Mauserung phenomenon is seen in A. Icthyosis bullosa of Siemens B. BCIE C. Icthyosis hysterix D. Refsums syndrome

62. Congenital insensitivity to pain with anhidrosis is A. HMSN I B. HMSN II C. HMSN III D. HMSN IV 63. Burt Pumphery syndrome composed of all except A. Knuckle pads B. Leukonychia C. Sensorineural hearing loss D. Nail dystrophy 64. Swiss cheese effect is histopathological feature of which of the following disease A. Sclerema neonatorum B. Pancreatic panniculitis C. Sclerosing panniculitis D. Foreign body panniculitis 65. Vasculits is not a predominant feature of A. Erythema nodosum leprosum B. Erythema induratum C. Erythema nodosum D. Nodular vasculitis 66. Hertoghe’s sign (loss of lateral half of eyebrow) is seen in all of the following except A. Atopic dermatitis B. Hypothyroidism C. Systemic sclerosis D. SLE 67. Beighton’s sign means A. Passive apposition of the thumb to flexor aspect of forearm B. Passive dorsiflexion of the 5th finger beyond 90 degrees C. Passive apposition of the thumb to extensor aspect of forearm D. Passive dorsiflexion of the 5th finger beyond 180 degrees

Multiple Choice Questions 68. Russell’s sign is seen in A. Bullemia nervosa B. Anorexia nervosa C. Dermatitis artifacta D. Russel viper bite 69. Premalatha sign is seen in A. Pemphigus vulgaris B. Pemphigus vegetans C. Pemphigus foliaceous D. Fogo selvagam 70. All of the following diseases have a sign related to Hutchinson except A. Herpes zoster B. Syphilis C. Melanoma D. Basal cell carcinoma 71. Hair collar sign (circle of hair surrounding the lesion) is seen in all except A. Encephalocele B. Myelocele C. Hemangioma D. Aplasia cutis

75. All of the following are examples of predominantly lobular panniculitis except A. Cutaneous polyarteritis nodosa B. Lupus panniculits C. Morphea profundus D. Erythema nodosum 76. Which of the following vector is responsible for old world leishmaniasis? A. Lutzomyia B. Psychodopygus C. Reduvid bug D. Phlebotomus 77. Non destructive mucocutanous leishmaniasis is caused by A. L braziliensis B. L donovani C. L mexicana amazonensis D. L aethiopica 78. Pentamidine is drug of choice for which type of leishmaniasis A. L donovani B. L aethiopica C. L braziliensis D. L amazonensis

72. Deck chair sign is seen in A. Papuloerythroderma of Ofuji B. Malignant melanoma C. Hodgkin’s lymphoma D. Pseudolymphoma

79. All of the following are histopathological variant of basal cell carcinoma except A. Solid basal cell carcinoma B. Spindle cell basal cell carcinoma C. Adenoid nodular cell carcinoma D. Keratotic basal cell carcinoma

73. Causative agent for white piedra is A. Trichosporon beigelli B. Trichosporon ovoides C. Piedraia hortae D. Hortae werneckii

80. Bazex syndrome is associated with which of the following malignancy A. Squamous cell carcinoma B. Basal cell carcinoma C. Carcinoma of upper aerodigestive tract D. Colon carcinoma

74. Risk factors for development of SLE in SCLE patients are all except A. High titre of ANA B. Leucopenia C. Raynaud’s phenomenon D. Papulosquamous type of SCLE

81. All of the following is true about eccrine hidrocystoma except A. Multilocular B. Unilocular C. Absent papillary projection D. Lined with cuboid cell

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Essentials in Dermatology 82. Spongiosis is a histopathological feature of A. Lichen planus B. Lichen striatus C. Psoriasis D. Keratosis lichenoides chronicus. 83. Follicular hyperkeratosis is seen in deficiency of all except A. Essential fatty acid B. Vitamin K C. Vitamin A D. Vitamin E

89. Which of the following is not true for systemic sclerosis? A. Matlike telangiectasia characteristic B. Nail fold telangiectasia common C. Dysphasia due to pharyngeal dysmotility D. Gangrene of fingers and toes are frequent 90. Pharyngeal dysmotility is feature of A. Polymyositis B. SLE C. Localized scleroderma D. Systemic sclerosis

84. Hair color change is seen in deficiency of all except A. Selenium B. Biotin C. Vitamin B12 D. Manganese

91. Bullous eruptions in infant are commonly associated with A. Congenital syphilis B. Epidermolysis bullosa C. Bullous impetigo D. Urticaria pigmentosa

85. Mouse ear is an X-ray feature of A. Ochronotic arthropathy B. Psoriatic arthritis C. Rheumatoid arthritis D. HIV associated arthropathy

92. Type of malignant melanoma seen in Indians is A. Lentigo melanoma B. Acral lentiginous melanoma C. Superficial spreading melanoma D. Nodular melanoma

86. Which of the following disease is associated with atopy? A. Ichthyosis vulgaris B. X-linked icthyosis C. Lamellar icthyosis D. Epidermolytic hyperkeratosis 87. Area least likely to be affected in atopic dermatitis A. Lips B. Mid chest C. Dorsa of feet D. Eyelids 88. Which of the following not useful for diagnosis of dermatomyositis? A. Clinical examination B. EMG C. Muscle biopsy D. Raised SGOT levels

93. Malignant melanoma spreads by all of these modes except A. Continuity B. Contiguity C. Lymphatic D. Blood 94. All are the following features of seborrheic keratosis except A. Surface has follicular plugs B. Pigmented keratotic papule C. Sun exposure is contributory factors D. Mostly pruritic 95. Which is not predisposing factor for squamous cell carcinoma of the skin A. Radiotherapy B. Arsenic ingestion

Multiple Choice Questions C. Submucous fibrosis D. HIV infection 96. Likelihood of developing psoriatic arthritis in psoriasis is A. 0.1% B. 0.5% C. 1.0% D. 5.0% 97. Prognosis of localized scleroderma is A. Persists unchanged B. Gradually increase in number and size C. Increases to involve whole body D. Heals with atrophy and then persists 98. Photosensitivity is common in A. SLE B. DM C. Scleroderma D. Rheumatoid arthritis

103. Which of the following pigmented lesion has increased risk for developing malignant melanoma? A. Melasma B. Congenital melanocytic nevi C. Solar lentigo D. Café au lait macules 104. Which is not a sign of fungal infection of nail? A. Onycholysis B. Pits C. Thickening of nail plate D. Subungual hyperkeratosis 105. After treatment of pityriasis versicolor, skin colour returns to normal in A. One week B. Two weeks C. Two months D. Six months

99. Which of the following is not a specific sign of lupus erythematosus A. Discoid lesions B. Butterfly erythema C. Nail fold telangiectasia D. None of the above

106. Bullae are notable feature of which type of scabies A. Scabies incognito B. Scabies in infant C. Crusted scabies D. Scabies in bedridden

100. Preferred mode of biopsy in suspected malignant melanoma is A. Shave biopsy B. Punch biopsy C. Incision biopsy D. Excision biopsy

107. All of the following are site involved in nodular scabies except A. Penis B. Trunk C. Axilla D. Elbow

101. Commonest benign epithelial neoplasm of skin is A. Squamous cell carcinoma B. Actinic keratosis C. Radiation keratosis D. Seborrheic keratosis 102. Standard management of café au lait macules is A. Excision and grafting B. Cryotherapy C. Topical hydroquinone D. Reassurance

108. Pyoderma includes all of the following except A. Erythrasma B. Impetigo C. Carbuncle D. Ecthyma 109. Maximum number of hairs lost per day in normal person is A. 5-10 B. 20-25 C. 50-100 D. 100-200 110. One of the following is an ophthalmic association of alopecia areata A. Iridocyclitis B. Corneal opacities C. Cataract D. Retinal pigmentation

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MCQs in Clinical Dermatology-Part II 1. Recent infection with rubella can be diagnosed using A. Neutralising antibody B. Haemagglutination inhibiting antibody C. Complement fixing antibody D. Cold agglutinating antibody

C. Candida species D. Rhizopus species 8. Primary manifestation of Parvo virus B19 in adults is A. Acute arthropathy B. Rashes with slapped cheek appearance C. Anaemia D. Flu like syndrome

2. Blue berry muffin is seen in all of the following conditions except A. Congenital rubella B. Congenital leukemia C. Congenital syphilis D. Congenital CMV infection

9. Bacillary angiomatosis causing organism is best visualized using A. Warthin-starry stain B. Modified Warthin-starry stain C. Leishman stain D. Gram stain

3. Most common cause of acute paronychia is A. Staphylococcus aureus B. Pseudomonas aeruginosa C. Candida albicans D. Streptococcus pyogenes

10. Haverhill fever/Erythema arthriticum epidemicum is caused by A. Streptobacillus imiae B. Streptobacillus moniliformis C. Fransciella tularensis D. Leptospira

4. Hermann spots which are bluish grey areas on tonsils are seen in A. Measles B. Mumps C. Rubella D. Chicken pox

11. Jungle sore is A. Cutaneous diphtheria B. Cutaneous leishmaniasis C. Cutaneous tuberculosis D. Syphilis

5. True about Hermann spots A. Pathognomonic of measles B. Pin point elevations on soft palate C. Bright red spots on buccal mucosa opposite second molars D. Bluish grey areas on tonsils 6. Pitted keratolysis is caused by all except A. Micrococcus sedantarius B. Corynebacterium species C. Streptococci species D. Trichophyton species 7. Green nail syndrome with fruity odour is caused by A. Pseudomonas aeruginosa B. Aspergillus species

12. EBV infection is not associated with A. Cold induced acrocyanosis B. Oral hairy leukoplakia C. Gianotti crosti syndrome D. Langerhan cell histiocytosis 13. Woody fibrosis is seen in A. Actinomycosis B. Nocardiosis C. Botyromycosis D. Blastomycosis 14. Berliner’s sign “Heavy eyelids” is seen in A. Exanthem subitum B. Erythema infectiosum C. Infectious mononucleosis D. Erythema multiforme

Multiple Choice Questions 15. Hanging curtain sign is seen in A. Pityriasis rubra pilaris B. Pityriasis rosea C. Psoriasis D. Lichen planus 16. Tenting sign / Pup tent sign which is elevation and longitudinal splitting of nail plate due to nail bed involvement is seen in A. Psoriasis B. Lichen planus C. Alopecia areata D. Pityriasis rubra pilaris 17. False about proximal subungual onychomycosis is A. Caused by Trichophyton rubrum B. Infection of undersurface of proximal nail plate C. Indication for HIV testing D. Most common type of onychomycosis 18. Most common type of oral lichen planus is A. Ulcerative type B. Reticulate type C. Erythematous type D. Plaque type

22. Checkerboard pattern of parakeratosis is typically seen in A. Pityriasis rubra pilaris B. Pityriasis rosea C. Lichen planus D. Psoriasis 23. De Sanctis-Cacchione syndrome is associated with A. Xeroderma pigmentosum B. Porphyria cutanea tarda C. Fanconi anaemia D. Dyskeratosis congenital 24. Mickey Mouse ears are seen in A. Cockayne syndrome B. Bloom’s syndrome C. Xeroderma pigmentosum D. Trichothiodystrophy 25. Which of the following diseases with cellular hypersensitivity is not associated with cancer? A. Bloom syndrome B. Fanconi anaemia C. Dyskeratosis congenita D. Trichothiodystrophy

19. Rumple Leede test is done in A. Rocky mountain spotted fever B. Endemic typhus C. Epidemic typhus D. Ehlrichiosis

26. Woringer Kolopp disease is A. Localised type of pagetoid reticulosis B. Disseminated type of pagetoid reticulosis C. Mycosis fungoides associated follicular mucinosis D. Alopecia mucinosa

20. Jet with contrail appearance in dermoscopy is seen in A. Scabies B. Pediculosis C. Lupus vulgaris D. None of the above

27. Herpes virus type 8 is associated with all of the following except A. Castleman’s disease in the setting of HIV B. Kaposi sarcoma C. Primary effusion lymphoma D. Kimura’s disease

21. Doughnut sign is seen in A. Generalised lichen myxedematous B. Localised lichen myxedematous C. Dermatomyositis D. Scleredema

28. Diabetes mellitus is associated with A. Necrobiosis lipoidica B. Necrobiotica Xanthogranuloma C. Necrolytic migratory erythema D. Xanthoma disseminatum

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Essentials in Dermatology 29. Which of the following is false about mastocytosis syndrome? A. Respiratory tract and endocrine systems are rarely affected B. Patients suffer from recurrent bacterial/ viral/fungal infection C. Urticaria pigmentosa is the most common skin manifestation D. Darier’s sign is positive 30. Reynold’s syndrome is A. CREST syndrome + Primary biliary cirrhosis B. CREST syndrome + Lichen planus C. CREST syndrome + Sjogren’s syndrome D. CREST syndrome + Ulcerative colitis 31. Azure lunulae is seen in all except A. Wilson’s disease B. Argyria C. Following treatment with busulfan D. Lichen planus 32. Lupus pernio is a type of A. Sarcoidosis B. Tuberculosis C. Syphilis D. Amyloidosis 33. Necrolytic migratory erythema is seen in A. Glucagonoma syndrome B. Diabetes mellitus C. Internal malignancy D. Lyme’s disease 34. Which of the following is false about Erythema toxicum neonatorum A. Idiopathic condition B. Smear of vesicle shows eosinophils C. Lesions spare the palms and soles D. Lesions heal with residual pigmentation 35. Which of the following is false about Transient neonatal pustular melanosis? A. Idiopathic pustular eruption of newborn B. Smear of vesicle shows neutrophils C. Palms and soles are involved D. Lesions heal without residual pigmentation

36. Trichorrhexis nodosa is seen in A. Argino succinic aciduria B. Alkaptonuria C. Phenyl ketonuria D. Tyrosinemia 37. Pinch purpura due to vessel wall fragility is a feature of A. Amyloidosis B. Sarcoidosis C. Porphyria D. Mucinosis 38. Skin reactions on sun exposed areas are absent in which porphyria A. Variegate porphyria B. ALA dehydratase deficiency porphyria C. Congenital erythropoietic porphyria D. Porphyria cutanea tarda 39. All of the following are neurocutaneous disorders except A. Woolf’s syndrome B. Waardenburg’s syndrome C. Tietz’s syndrome D. Hermansky-Pudlak syndrome 40. Erythema migrans is cutaneous hallmark of A. Tuberculosis B. Glucagonoma C. Lyme borreliosis D. Diabetes mellitus 41. Pseudoscleroderma is seen in A. Acrodermatitis chronica atrophicans B. Borrelial lymphocytoma C. Actinomycosis D. Cutaneous tuberculosis 42. Russel’s sign which is abrasions and scars on knuckle pads is seen in A. Eating disorders B. Kwashiorkar C. Marasmus D. Hypervitaminosis A

Multiple Choice Questions 43. Classic carcinoid flush is seen in A. Bronchial carcinoid B. Gastric carcinoid C. Midgut carcinoid D. Pancreatic carcinoid

50. Which type of rash does not occur in Kawasaki disease? A. Morbilliform B. Annular C. Vesicular D. Urticarial

44. Erythema induratum is A. Nodular vasculitis B. Urticarial vasculitis C. Livedoid vasculitis D. None of the above

51. Plucked chicken appearance of skin is seen in A. Cutis laxa B. Pseudoxanthoma elasticum C. Xeroderma pigmentosa D. Dyskeratosis congenital

45. Livedo reticularis and Livedo vasculitis associated with ischaemic cerebrovascular lesions,hypotension and extracerebral arterial and venons thrombosis is A. Sneddon’s syndrome B. Schnitzler’s syndrome C. Parry Romberg syndrome D. None of the above

52. Which of the following porphyria follows autosomal dominant pattern of inheritance? A. Erythropoietic protoporphyria B. Congenital erythropoietic porphyria C. Hepato erythropoietic porphyria D. Plumboporphyria/ALA dehydratase deficiency porphyria

46. Which of the following agent is thought to play a role in morphea? A. Borrelia burgdorferi B. Rickettsia C. Burkholderia cepaciae D. None of the above

53. Which of the following is facultative tuberculid? A. Lichen scrofulosum B. Lichenoid tuberculid C. Erythema induratum D. Lupus miliaris disseminatus faciei

47. Neuroectodermal melanolysosomal disease is A. Elejalde syndrome B. Steijlen syndrome C. Bjornstad syndrome D. Cross syndrome

54. White superficial onychomycosis not caused by A. Trichophyton mentagrophytes B. Trichophyton rubrum C. Aspergillus D. Fusarium

48. Subcutaneous fat necrosis is a cutaneous marker of A. CA lung B. CA prostrate C. CA stomach D. CA pancreas

55. Perforating organs and eroding fronds appearance of histopathological examination is seen in A. White superficial onychomycosis B. Proximal subungual onychomycosis C. Distal subungual onychomycosis D. Candidal onychomycosis

49. Erythema gyratum repens is associated with A. Internal malignancy B. Diabetes C. Glucagonoma D. Tuberculosis

56. Valley fever/Desert Rheumatism is A. Coccidioidomycosis B. Paracoccidioidomycosis C. Blastomycosis D. Torulosis

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Essentials in Dermatology 57. Which type of Ehlers-Danlos syndrome has autosomal recessive inheritance A. Hypermobility type B. Kyphoscoliosis type C. Vascular type D. Arthrochalasia type 58. Prayer sign which is inability to approximate palmar surfaces of PIP and DIP with palms pressed together is seen in A. Cheiroarthropathy/Diabetic limited joint mobility B. Scleredema diabeticorum C. Psoriatic arthritis D. None of the above 59. Cigarette paper scars are characteristic of A. Ehlers Danlos syndrome B. Marfan’s syndrome C. Pseudoxanthoma elasticum D. Cutis laxa 60. Which of the following is pathognomonic of dermatomyositis? A. Gottron’s papules B. Heliotrope erythema C. Shawl sign D. Mechanic’s hand lesion 61. Hot tub folliculitis is caused by A. Pseudomonas aeruginosa B. Staphyloccocus aureus C. Clostridium perfringens D. Streptococcus pyogenes

64. Granulomatosis infantiseptica syndrome is due to A. Listeriosis B. Diphtheria C. Melioidosis D. Leptospieosis 65. Which among the following is not active against Mycobacterium avium intracellulare complex A. Clarithromycin B. Isoniazid C. Ethambutol D. Rifampin 66. Botryomycosis is caused by all except A. Pseudomonas aeruginosa B. Actinobacillus lignieressi C. Escherichia species D. Aeromonas hydrophila 67. Granulomatous slack skin is type of A. Primary cutaneous T cell lymphoma B. Primary cutaneous B cell lymphoma C. Cytotoxic lymphoma of skin(NK/T cell lymphom D. Plasmacytoma) 68. Congenital self healing reticulohistiocytosis is A. Hashimoto Pritzker disease B. Eosniophilic granuloma C. Letterer siwe disease D. Hand Schuller christian disease

62. Slavic leprosy is another name for A. Rhinoscleroma B. Leishmaniasis C. Variant of leprosy in Africa D. None of the above

69. Glove and stocking hyperpigmentation with a lemon yellow pallor of skin elsewhere is due to A. Vitamin C deficiency B. Vitamin B3 deficiency C. Vitamin B12 deficiency D. Vitamin K deficiency

63. Pretibial fever is a form of A. Leptospirosis B. Melioidosis C. Plague D. Tularemia

70. Hepatitis B is associated with all except A. Cryoglobulinemia B. Papular acrodermatitis of childhood C. Sweet’s syndrome D. Acquired angioedema

Multiple Choice Questions 71. Which of the following is associated with lentigenes? A. Danoff syndrome B. CHILD syndrome C. MIDAS syndrome D. None of the above 72. Lupoid sycosis is A. Chronic scarring form of sycosis barbae B. Chronic scarring form of tinea barbae C. Chronic scarring form of lupus vulgaris D. All of the above 73. LE profundus refers to those patients with A. LE panniculitis B. LE panniculitis + DLE C. TEN like ACLE D. None of the above 74. Congenital lipodystrophy is A. Lawrence Seip syndrome B. Rabson Mendenhall syndrome C. Leprechaunism D. Type A syndrome 75. Gilchrist disease is A. North American Blastomycosis B. African Histoplasmosis C. South American Blastomycosis D. Coccidioidomycosis 76. Plummer’s Nail is seen in A. Hyperthyroidism B. Hypothyroidism C. Cushing’s syndrome D. Addison’s disease 77. Bloch-Sulzberger disease is another name for A. Incontinenta pigmenti B. Congenital dyskeratosis C. Anhidrotic ectodermal dysplasia D. Angiokeratoma corporis diffusum 78. In Neonatal Lupus erythematosus the predominant auto antibodies are A. Anti-R0/SSA

B. Anti-U1 RNP C. Anti-La/SSB D. Anti-ds DNA 79. Drug induced SLE is associated with auto antibody to A. ds DNA B. Sm Ag C. Histones D. UI RNP 80. Most frequent follicular tumour arising in Nevus sebaceous is A. Malignant melanoma B. Trichoblastoma C. Basal cell carcinoma D. Squamous cell carcinoma 81. Hutchinson’s nail sign is seen in A. Subungual melanoma B. Pyogenic granuloma C. Myxoid cyst D. Keratoacanthoma 82. Stevens-Johnson syndrome is associated with autoantibody against A. Desmogleins B. Desmoplakins C. Desmocollins D. None of the above 83. Epidermolysis bullosa acquisita is characterised by autoantibody against A. Collagen type VII B. Desmoglein C. Basement membrane zone D. None of the above 84. Which of the following does not belong to central centrifugal scarring alopecia? A. Follicular degeneration syndrome B. Folliculitis decalvans C. Lichen planopilaris D. Pseudopelade 85. ‘Sweat that stains’ is seen in A. Phenylketonuria B. Alkaptonuria C. Arginosuccinic aciduria D. Tyrosinemia

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Essentials in Dermatology 86. Comma shaped bodies in histiocytes are seen in electron microscopy in all except A. Juvenile xanthogranuloma B. Papular xanthoma C. Benign cephalic histiocytosis D. Sinus histiocytosis 87. Pseudo-cushing syndrome is seen in A. Chronic alcoholism B. Hypothyroidism C. Diabetes D. Porphyria 88. Gold standard in the diagnosis of Sjögren syndrome is A. Lip biopsy B. Sialometry C. Sialography D. Buccal mucosal biopsy 89. False about Paraneoplastic acrokeratosis of Bazex is all except A. Nail changes occur late in the course of the disease B. More common in females C. Responds to etretinate even if primary is not treated D. Usually associated with neoplasia of genito urinary tract 90. Angel’s kiss is A. Nevus flammeus B. Port-wine stain C. Hemangioma D. Mongolian spots 91. Steinberg’s sign (protrusion of thumb on the ulnar side of the hand after making a fist enclosing the thumb is seen in A. Ehler Danlos syndrome B. Marfan’s syndrome C. Cutis laxa D. Pseudoxanthoma elasticum 92. Nasal pruritus indicates an underlying A. Brain tumour B. CA nasopharynx

C. Hodgkin’s lymphoma D. Polycythemia vera 93. Bath itch is characteristic of A. Polycythemia rubra vera B. Iron deficiency C. Senile pruritus D. Hodgkin lymphoma 94. “Addicted scrotal syndrome” refers to A. Use of high potency steroid to scrotum B. Use of antifungal creams to scrotum C. Use of anesthetic cream to scrotum D. Use of antibacterial cream to scrotum 95. In nostalgia parasthetica, itch is localized to A. Interscapular region B. Medial aspect of thigh C. Lateral aspect of thigh D. Inner aspect of arm 96. In brachioradial pruritus, itch is localized to A. Interscapular region B. Medial ascpect of thigh C. Lateral aspect of thigh D. Inner aspect of arm 97. In cholestatic pruritus, pruritogenic substance is A. Bile salt B. Bile acid C. Bile salt intermediates D. Bilirubin 98. Aspirin is effective in pruritus due to A. Hodgkin lymphoma B. Polycythemia rubra vera C. Mastocytosis D. Internal malignancy 99. Mediator of pruritus in Hodgkin’s lymphoma A. Histamine B. Opioid peptides C. Seratonin D. Leukopeptidase


MCQs in Sexually Transmitted Diseases 1. The incubation period of the following genital ulcer diseases are correct except A. Syphilis – 9-90 days B. Genital herpes – 14-21 days C. Chancroid – 1-7 days D. LGV – 3-30 days 2. The test of choice for the laboratory diagnosis of genital Chlamydia trachomatis infection is A. Enzyme immunoassay B. PCR C. Complement fixation test D. Micro-immunofluorescence 3. All the following are features of genital herpes in an immunocompromised host except A. Hyperkeratotic verrucous or warty lesions B. Vegetating plaques C. Chronic persistent ulceration D. Painless vesicles 4. The recommended drug of choice for treatment of neurosyphilis is A. Benzathine penicillin G B. Aqueous crystalline penicillin G C. Procaine penicillin D. Benzyl penicillin 5. The etiological agent of gonorrhoea was identified in 1879 by A. John Hunter B. Benjamin Bell C. Albert Ludwig Neisser D. Phillipe Ricord 6. The following media are used for culture of gonococci except A. Modified Thayer Martin medium B. Chacko Nair medium C. Chocolate agar D. Mueller Hinton agar

7. The following statement regarding HSV-1 vs HSV-2 infection is correct A. Newly acquired HSV-2 infections are more commonly symptomatic than HSV1 B. A HSV-1 seropositive person is more likely to develop classical signs and symptoms on infection with HSV-2 C. Recurrences are more common with HSV-1 D. HSV-2 is more commonly isolated than HSV-1 in aseptic meningitis 8. Cowper’s glands are A. Bulbourethral glands B. Mucous glands C. Sebaceous glands D. Sweat glands 9. Bartholin’s glands in the female are homologous to the following structure in males A. Cowper’s glands B. Littre’s glands C. Tyson’s glands D. Skene’s glands 10. The glans penis and clitoris drain into A. Deep inguinal lymph nodes B. Superficial inguinal lymph nodes C. Femoral lymph nodes D. External iliac lymph nodes 11. Gonococci are A. Extracellular gram positive diplococci B. Intracellular gram positive diplococci C. Extracellular gram negative diplococci D. Intracellular gram negative diplococci 12. Neisseria gonorrhoeae can be isolated from the following sites in disseminated infection except A. Blood B. Skin lesions C. Synovial fluid D. Oropharynx

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Essentials in Dermatology 13. Henderson Paterson bodies are pathognomonic of A. Molluscum contagiosum B. Measles C. Chlamydial infections D. Mycoplasma 14. The following are true about chancre redux or monorecidive chancre except A. Recurrence of a primary sore at the site of the original lesion B. A form of relapse C. Infectious lesion D. Treponemes cannot be obtained 15. “School of fish” or “railroad track” appearance is seen in A. Yersinia pestis B. Hemophilus ducreyi C. Neisseria gonorrhoeae D. Calymmatobacterium granulomatis 16. True about Donovan bodies are all except A. Gold standard for the diagnosis of donovanosis B. Coccobacilli C. Located within polymorphonuclear leukocytes D. Resemble safety pins 17. False about Treponema pallidum A. 6-20 microns in length B. regular tight spirals C. capsulated D. flagellated 18. All are true about primary chancre except A. Seen in more than 90% cases of syphilis B. Painless, indurated, less than 2 cm C. Usually single D. Highly infectious 19. Commonly involved tissues in secondary syphilis are all except A. Skin B. Bone

C. Mucous membranes D. Lymph nodes 20. Eruptions of acquired secondary syphilis can be all except A. Papulosquamous B. Macular C. Follicular D. Bullous 21. Monorecidive or chancre redux occurs due to A. Re-infection with Treponema pallidum B. Relapsing lesion at the site of primary chancre C. Lesion of secondary syphilis D. Gumma of tertiary syphilis 22. Highly infectious lesions of syphilis are all except A. Primary chancre B. Gumma C. Condylomata lata D. Mucous patches 23. Gummatous osteoperiosteitis of skull bones in syphilis is called A. Worm-eaten skull B. Moth-eaten skull C. Hole in bone appearance D. Pepper-pot skull 24. Syphilitic gumma commonly involves A. Left ventricle and septum B. Right ventricle C. Left atrium and septum D. Right and left ventricles 25. Bruit de Tabourka (loud and tambour-like second sound) is heard on auscultation in A. Hypertension B. Syphilitic aortitis C. Dissecting aortic aneurysm D. Atherosclerosis

Multiple Choice Questions 26. In congenital syphilis the fetus may be born apparently normal but develop signs of early congenital syphilis during the first few weeks or months. This is A. Kassowitz law B. Profeta’s law C. Colle’s law D. Diday’s law 27. The following are AIDS defining illnesses except A. Oral hairy leukoplakia B. Persistent generalized lymphadenopathy C. Oropharyngeal candidiasis D. Invasive cervical carcinoma 28. The incubation period for primary HIV infection or seroconversion illness is A. 2-6 weeks B. 1-7 days C. 3-6 months D. 2-10 years 29. Primary neutralizing determinant or V3 loop is located on A. Envelope surface protein (gp120) B. Transmembrane protein (gp41) C. Reverse transcriptase protein (p66) D. Core protein (p24) 30. Beta lactamase producing Neisseria gonorrhoeae – all are true except A. Also known as PPNG (penicillinase producing N. gonorrhoeae) B. Originated in Asia and Africa at about the same time C. Depends on chromosome resistance D. Plasmid mediated 31. All the following are characteristic of Chlamydia trachomatis except A. Have cell wall B. Contains DNA C. Obligatory intracellular parasite D. Cell wall is devoid of muramic acid 32. Chlamydia trachomatis infection in children causes all except A. Conjunctivitis

B. Pneumonia C. Serous otitis media D. Meningitis 33. Bacterial vaginosis can be diagnosed clinically by the presence of ALL EXCEPT A. Vaginal pH > 4.5 B. Thin homogeneous milky discharge C. Scanty or absent lactobacilli D. Clue cells at least 30% of total vaginal epithelial cells 34. The following condition is associated with acute biological false positive VDRL A. Lepromatous leprosy B. Old age C. SLE D. Pregnancy 35. An infant should be evaluated for possible congenital syphilis in all the following conditions except A. The mother is inadequately treated for syphilis during pregnancy B. If mother has received treatment more than 4 weeks before delivery C. Treatment of syphilis during pregnancy with non-penicillin regimens D. Treatment of syphilis which has not been documented 36. Painless genital ulcer is caused by all except A. Amoebiasis B. LGV C. Histoplasmosis D. Ulcerative penile carcinoma 37. Human papilloma virus is responsible for all except A. White patches on the cervix B. Koilocytosis on cervical smear C. Condyloma acuminata D. Polyps on the cervix

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Essentials in Dermatology 38. In patients with perianal warts, all are true except A. Have higher incidence of other STDs than general population B. Inevitably have had receptive anal intercourse C. May present with pruritus ani D. Should have proctoscopic examination to exclude anal canal warts 39. True about gonococcal proctitis in homosexual men A. Is acquired by orogenital intercourse B. Usually causes malaise C. Is easily diagnosed on gram stain of rectal exudates D. Should be treated with ceftriaxone 250 mg in a single dose plus doxycycline 100 mg twice daily for 7 days 40. The following is/are appropriate methods for diagnosis of chlamydial infection in patients with cervicitis A. High vaginal swab B. Wet film C. Direct immunofluorescence with Chlamydia trachomatis specific monoclonal antibody D. Gram stain 41. All are true about clue cells except A. Show slight enlargement of nuclei with dense cytoplasm covered by bacterial vaginosis organisms B. Cell border is obscured and has serrated appearance C. Has clear cytoplasm covered by bacterial vaginosis organisms D. First described by Gardner and Duke 42. Diagnosis of atypical syphilis in advanced HIV positive patients should be confirmed by A. VDRL B. TPHA

C. FTA-Abs D. Biopsy with special stains for T. pallidum 43. Widespread vaginal warts can be safely and effectively treated with all except A. TCA cautery B. Laser therapy C. Topical podophyllin 5% D. Cryosurgery 44. The following statements are true in the etiology of cervical cancer except A. Over 90% of invasive cervical cancer contain HPV 16 and 18 B. Cigarette smoking is probably important in development of cervical cancer C. All CIN III lesions will progress to cervical cancer if untreated D. Immunosuppression promotes progression of CIN III to cancer 45. Plasma cell balanitis – all are true except A. It is also called Zoon’s balanitis B. Results in painful penile ulcers C. Appears as red patches on the glans/ inner prepuce D. May be treated with topical steroids 46. CSF examination is indicated in all except A. Serological relapse in syphilis patients B. Syphilis with HIV infection C. Positive syphilis serology with neurological signs D. Latent syphilis in pregnant women 47. The recommended treatment of neurosyphilitic patients who have penicillin allergy is A. Tetracycline B. Erythromycin C. Ceftriaxone D. Penicillin test, desensitization, penicillin treatment

Multiple Choice Questions 48. The most specific test for neurosyphilis is A. CSF cell count B. CSF protein C. CSF immunoglobulin D. CSF VDRL 49. Which of the following HPV types are not associated with malignancy A. Type 6 B. Type 16 C. Type 18 D. Type 31 50. The following statement about chancroid is true A. If left untreated, bubo forms in 80-85% patients B. Bubo is more common in females C. It is self-limited and confers immunity D. Prolonged treatment with erythromycin is recommended for HIV patients who develop chancroid lesions 51. Trichomonas vaginalis does not invade A. Prostate B. Preputial sac C. Urethra D. Vagina 52. The following statements regarding trichomonas vaginalis are true except A. Vaginal discharge is seen up to 70% of women B. Vulvitis is seen in often C. Colposcopic appearance of cervix described as strawberry cervix D. Cervical ulceration is not uncommon 53. Antibiotic that is used in single dose in treatment of chlamydial urethritis is A. Tetracycline B. Erythromycin C. Azithromycin D. Clarithromycin 54. The risk of neonatal herpes following primary maternal herpes infection is A. 30-50% B. 10-30% C. 5-10% D. <5%

55. Blisters in the genital region could be a feature of all except A. Pemphigoid B. StevenJohnsons syndrome C. Herpes zoster D. Syphilis 56. Advantages of syndromic management include all except A. Allow for diagnosis and treatment at first visit B. No special tests are necessary to make the diagnosis C. Treatment aimed at a single infection D. Simple, inexpensive and reliable 57. Colpitis macularis-strawberry appearance of cervix is seen in A. Trichomoniasis B. Candidiasis C. Bacterial vaginosis D. Gonorrhea 58. Which part of HIV virus is used to attach to T helper lymphocyte A. p 24 B. gp41 C. gp120 D. Fusion or CXCR4 59. The following are HIV virion structural genes except A. Gag B. Pol C. Env D. Tat 60. The following is a product of HIV gag gene A. Gp120 B. P24 C. P66 D. P51 61. The reverse transcriptase protein is designated by 2 subunits A. Gp41 and gp120 B. Gag and pol C. P66 and p51 D. P6 and p9

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Essentials in Dermatology 62. Most HIV-1 isolytes belong to the following group-which is the main cause of AIDS pandemic A. groupM B. group O C. group N D. group A 63. The main receptor for HIV-1 on T cells is A. CD4 B. Fusin CXCR-4 C. CCR-5 D. CCR-2 64. The most important co-receptor for CD4 mediated entry of HIV-1 into T-cells is A. CCR-2 B. CCR-4 C. CCR-5 D. Fusin CXCR-4 65. There are _______ identified subtypes of HIV-1 under Group M in Los Alamos HIV sequence database A. 10 B. 11 C. 12 D. 13 66. The gene in HIV-1 that is replaced by VPX gene in HIV-2 is A. vpu B. vpr C. vif D. env 67. HIV-1 and HIV-2 viruses belong to which subfamily of retroviruses A. Oncovirinae B. Lentivirine C. Spumavirinae D. Retrovirinae

70. The duration from entry of HIV into one cell to the production of new progeny which infects new cells is A. 2.6 hours B. 2.6 days C. 2.6 weeks D. 2.6 months 71. Memory CD4 cells are designated A. CD 45 RO B. CD 45 RA C. CD 45 RB D. CD 45 RC 72. Mechanisms of CD4 T cell destruction by HIV are all except A. Direct or single cell killing B. Syncytia formation C. Apoptosis D. Phagocytosis 73. All the following are part of the spectrum of clinical autoimmunity associated with HIV except A. Sjogren syndrome B. Reiter’s syndrome C. SLE D. Polymyositis 74. The following associations in HIV patients are correct except A. JC virus – Creutzfeldt Jakob disease B. CMV – esophageal ulcer C. Toxoplasma – encephalitis D. Isospora – diarrhea

68. HIV-1 is most closely related to the simian immunodeficiency virus (SIV) isolated from A. Chimpanzees B. Sooty mangabeys C. Baboons D. Slow lorris

75. Gastrointestinal infection in HIV is seen with all the following organisms except A. Salmonella B. Cryptococcus C. CMV D. Candida

69. Risk of HIV infection from a single percutaneous exposure to needles or other sharp instruments contaminated with HIV infected blood is A. 3% B. 0.3% C. 0.03% D. 0.003%

76. In Asia, in HIV infected patients, a common opportunistic infection is A. Penicillium marneffei B. Rhodococcus equi C. Mycobacterium leprae D. HHV-8

Multiple Choice Questions 77. Giant molluscum-like lesions in HIV patients is seen in infection with A. Penicillosis B. Bacillary angiomatosis C. Histoplasmosis D. Cryptococcosis

84. Rash is commonly seen with all except A. Abacavir B. Tenofovir C. Efavirenz D. Nevirapine

78. Primary prophylactic antimicrobial therapy in HIV positive patients is given for all except A. Pneumocystis carinii pneumonia B. Mycobacterium avium complex C. Toxoplasmosis D. Cryptococcal meningitis

86. Pancreatitis is a side effect of A. Zidovudine B. Didanosine C. Lamivudine D. Abacavir

79. Drugs that can be used for prophylaxis against Pnemocystis carinii pneumonia are all except A. Dapsone B. Aerosolised pentamidine C. Cotrimoxazole D. Amoxicillin clavulanate 80. The following drugs can be given in pregnant HIV patients EXCEPT A. Zidovudine B. Lamivudine C. Nevirapine D. Efavirenz 81. The best indicator to predict progression of HIV is A. CD4 count B. Weight loss C. Viral load D. New opportunistic infection 82. In patients with AIDS, Kaposi’s sarcoma A. More commonly affects homosexuals and bisexuals B. Usually presents at a single site initially C. Commonly metastasizes to brain D. Should be treated early and aggressively 83. Peripheral neuropathy is a side effect of all except A. Didanosine B. Stavudine C. Zalcitabine D. Abacavir

85. Nephrotoxicity is seen with A. Lamivudine B. Nevirapine C. Indinavir D. Ritonavir

87. Lipodystrophy is seen with A. Nucleotide reverse transcriptase inhibitors B. Non nucleotide reverse transcriptase inhibitors C. Protease inhibitors D. Fusion inhibitors 88. The antitubercular agent to be avoided in patients receiving protease inhibitors (particularly ritonavir) is A. Isoniazid B. Rifampicin C. Ethambutol D. Pyrazinamide 89. Serious mitochondrial toxicity can occur with the following class of antiretroviral agents A. Nucleoside reverse transcriptase inhibitors B. Nucleotide reverse transcriptase inhibitors C. Non-nucleoside reverse transcriptase inhibitors D. Protease inhibitors 90. Complications of gonorrhea include all except A. Prostatitis B. Littritis C. Bartholinitis D. Tysonitis 91. Water-can perineum is seen in A. Gonorrhea B. Lymphogranuloma venereum C. Granuloma inguinale D. Chancroid

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Essentials in Dermatology 92. Acute gonococcal infection in females usually presents as A. Urethritis B. Vaginitis C. Endocervicitis D. Pelvic inflammatory disease 93. In the three glass test haze in the second glass indicates A. Anterior urethritis B. Posterior urethritis C. Cystitis D. Either posterior urethritis or cystitis 94. True about gonorrhea are all except A. The average incubation period is 2-5 days B. Usually presents as posterior urethritis in men C. Usually presents as endocervicitis in women D. Rectal and pharyngeal infection is common in homosexuals 95. True statements about disseminated gonococcal infection are all except A. The primary mucosal infection is usually asymptomatic B. Characteristic clinical findings include arthritis and skin lesions C. The organism is detected in blood culture in 90% cases D. Endocarditis is a fatal complication 96. Specimens in suspected gonococcal infection are preferably collected with A. Metallic swab sticks B. Wooden swab sticks C. Polyethylene terephthalate swabs D. Calcium alginate swabs 97. The drug of first choice for the treatment of uncomplicated lower genital tract infection due to Chlamydia trachomatis is A. Azithromycin B. Doxycycline C. Erythromycin D. Tetracycline

98. The first choice agent (according to CDC guidelines) for the treatment of Chlamydia trachomatis genital tract infection in pregnant women is A. Erythromycin B. Azithromycin C. Amoxycillin D. Doxycycline 99. Durand-Nicolas-Favre disease is A. Chancroid B. LGV C. Granuloma inguinale D. Gonorrhea 100. The new name proposed for the causative organism of granuloma inguinale is A. Calymmatobacterium granulomatis B. Chlamydia trachomatis C. Klebsiella granulomatis D. Klebsiella rhinoscleromatis 101. True about LGV – all except A. The causative organism is lymphotropic B. Primary lesion is seen in only 30% cases C. Primary involvement of deep iliac or perirectal lymph nodes is more common in women D. Frei test is the current diagnostic test of choice 102. The recommended drug and duration of treatment for LGV according to CDC guidelines is A. Doxycycline 100 mg BD x 21 days B. Azithromycin 1 g single dose C. Rifampicin 450 mg OD x 10 days D. Ciprofloxacin 500 mg BD x 1 week 103. All are true about chancroid except A. Painful non-indurated ulcer B. Ragged undermined edges with necrotic base C. Bleeds easily on touch D. Usually single

Multiple Choice Questions 104. True about chancroidal ulcer are all except A. Caused by organisms other than Hemophilus ducreyi B. Tender non-indurated ulcer C. Conspicuous lymphadenopathy present D. Usually single large ulcer

109. True about donovanosis are all except A. Variants of primary lesion are hypertrophic, phagedenic and sclerotic B. Pseudoepitheliomatous hyperplasia seen on HPE C. Lesions tend to rapidly enlarge and proliferate during pregnancy D. Untreated lesions heal within two weeks

105. True about bubo in chancroid A. Always unilateral B. Develops in all cases of chancroid C. Appears 3-4 weeks after the genital ulcer D. Unilocular

110. True about treatment of donovanosis – all except A. Trimethoprim-sulfamethoxazole is one of the recommended first line agent B. Duration of treatment is 1 week C. Erythromycin and azithromycin are alternative agents D. Gentamicin can be added if there is no improvement within the first few days of therapy

106. True about diagnosis of chancroid – all except A. Gram stained smears show bacilli with “school of fish” or “railroad track” appearance B. Organisms are frequently demonstrated in pus aspirated from bubo C. Culture is the gold standard for diagnosis D. PCR is available for simultaneous diagnosis of H.ducreyi, T. pallidum and HSV 1 and 2 (multiplex PCR) 107. First choice agents for the treatment of chancroid according to CDC guidelines include all except A. Azithromycin 1 g single dose B. Ceftrioxone 250 mg IM single dose C. Ciprofloxacin 500mg BD-3 days D. Doxycycline 100 mg BD-7 days 108. True about donovanosis are all except A. Initial lesion is fleshy beefy red granulation tissue B. Non indurated,non tender,bleeds on touch C. Lymphnode involvement results in bubo formation D. Extensive mutilation and destruction of genitalia can occur

111. The earliest feature of congenital syphilis is A. Corymbiform cutaneous lesions B. Nasal discharge C. Osteochondritis D. Anemia 112. Cutaneous lesions of early congenital syphilis include all except A. Corymbiform lesions B. Pemphigus syphiliticus C. Syphilitic furuncle D. Gumma 113. Involvement of which of the following lymph nodes characteristically occurs in congenital syphilis A. Cervical B. Axillary C. Epitrochlear D. Paraaortic 114. Pseudoparalysis of Parrot in congenital syphilis is due to A. Osteochondritis B. Myositis C. Neuropathy D. Dactylitis

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Essentials in Dermatology 115. Parrot’s nodes in congenital syphilis refer to A. Frontoparietal swellings secondary to osteochondritis B. Epitrochlear lymphadenopathy C. Generalized lymphadenopathy D. Syphilitic furuncle 116. Focal defects with cortical destruction seen on the medial aspect of proximal tibial metaphysis is known as A. Wimberger’s sign B. Celery stick appearance C. Bucket handle sign D. Sarcophagus sign 117. Longitudinal lines of rarefaction extending into the diaphysis is known as A. Cat bite sign B. Higoumenakis’ sign C. Celery stick appearance D. Bucket handle sign 118. Fractures through the degenerating metaphysis, followed by exuberant callus formation resulting in a cap over the metaphysis is called A. Wimberger’s sign B. Sarcophagus sign C. Parrot’s node D. Bucket handle sign 119. Periosteal new bone formation resulting in layers of marrow trapped between layers of subperiosteal bone is known as A. Celery stick appearance B. Sarcophagus sign C. Bucket handle sign D. Wimberger sign 120. Haematological manifestations of early congenital syphilis include all the following except A. Autoimmne hemolytic anaemia B. Leukemoid reaction

C. Basophilia D. Thrombocytopenia 121. Neurological manifestations of early congenital syphilis include all except A. Acute leptomeingitis B. Cerebrovascular accidents C. Tabes dorsalis D. Hydrocephalus 122. Late congenital syphilis in the child/ adolescent corresponds to ________ in the adult A. Secondary syphilis B. Early latent syphilis C. Late latent syphilis D. Tertiary syphilis 123. Syphilitic pneumonitis- all are true except A. Occurs due to gumma formation in the lung B. Intense obliterative fibrosis occurs in the interalveolar space C. Can be confused with ARDS D. Also called pneumonia alba 124. The fundamental histological lesion of congenital syphilis is A. Interstitial fibrosis B. Obliterative endarteritis C. Microscopic gumma D. Necrotizing funisitis 125. Chancre galeuse is A. Syphilitic chancre occurring in a cutaneous lesion of scabies B. Oral chancre of syphilis C. Monorecidive chancre of syphilis D. Condylomata lata 126. Supraorbital thickening in syphilis is called A. Olympian brow B. Hertoghe’s sign C. Hennebert’s sign D. Higoumenakis’ sign

Multiple Choice Questions 127. Thickening of the medial third of the clavicle in congenital syphilis is called A. Parrot’s node B. Higoumenakis’ sign C. Hennebert’s sign D. Wimberger’s sign 128. The Oslo study, a prospective study of the natural history of syphilis was initiated by A. Caesar Boeck B. Phillippe Ricord C. Eric Hoffmann D. Kenneth MacLeod

129. The organism causing chancroid was first identified by A. Von Wassermann B. Aldo Castellani C. Augusto Ducreyi D. Max Bockhart 130. Donovanosis was first described by A. Augusto Ducreyi B. Kenneth MacLeod C. Robert Gallo D. Charles Donovan

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MCQs in Leprosy 1. The minimum inhibitory concentration for dapsone is _____ µg/ml A. 0.0003 B. 0.003 C. 0.03 D. 0.03 2. All are true regarding dapsone syndrome, except A. Hepatitis B. Usually develops within 6 weeks of therapy C. Exfoliative dermatitis D. Arthritis 3. Monospot test is used for A. Monitoring compliance of dapsone B. Detecting compliance of clofazimine C. Detecting dapsone resistance D. Testing integrity of dermal nerve.

9. Rifampicin acts by inhibiting ____ of M .leprae A. DNA synthesis B. RNA synthesis C. Protein synthesis D. Cell wall synthesis 10. All are used in type 2 lepra reaction EXCEPT A. Clofazimine B. Ethionamide C. Chloroquine D. Cyclosporin 11. Drug used in recurrent ENL with steroid dependence A. Clofazimine B. Thalidomide C. Methotrexate D. Chloroquine 12. ROM therapy is not recommended for children below A. 3 years B. 5 years C. 8 years D. 10 years

4. “Wooly headedness” is the adverse effect of A. Dapsone B. Clofazimine C. Rifampicin D. Ethionamide

13. Therapy recommended for single lesion paucibacillary leprosy A. ROM B. RMM C. RCM D. Quadruple regime

5. The haematological adverse effects of dapsone include all except A. Agranulocytosis B. Methemoglobinemia C. Hemolytic anemia D. Thrombocytosis

14. All are used for immunotherapy in leprosy, except A. Levamisole B. Gamma IFN C. Cyclosporins D. IL-2

6. The minimum inhibitory concentration of rifampicin is ______ µg/ml A. 0.3 B. 0.03 C. 0.003 D. 0.0003 7. The half-life of clofazimine ranges about A. 50 days B. 60 days C. 70 days D. 80 days 8. The following is true regarding clofazimine A. Only bacteriostatic B. Only bactericidal C. Bacteriostatic, weekly bactericidal D. Bactericidal, weekly bacteriostatic

15. 12 pulses of MB-MDT to be completed in _____ months A. 12 B. 15 C. 18 D. 24 16. All are indication for systemic steroids in leprosy except A. Neuritis B. Severe ENL C. Recent muscle paralysis D. BT patch over hand 17. Splint used for ulnar nerve palsy is A. Knuckle–bender B. Cock-up C. Volkman’s D. Bohler-brawn

Multiple Choice Questions 18. “Stiles –Bunnel” procedure is done for A. Claw toe B. Foot drop C. Claw hand D. Lagophthalmos 19. Surgery is indicated for foot drop of more than ____ duration A. 3 months B. 6 months C. 1 year D. 2 years 20. Following surgeries are done in claw finger deformity EXCEPT A. Brand’s procedure B. Antia’s procedure C. Fowler’s procedure D. Gille’s procedure 21. Claw toe deformity is due to paralysis of A. Common peroneal nerve B. Posterior tibial nerve C. Deep peroneal nerve D. Superficial peroneal nerve 22. Most common paralytic deformity in leprosy is A. Ulnar claw hand B. Median claw hand C. Total claw hand D. Claw toe 23. ‘Intrinstic plus’ or “swan neck deformity” of fingers is due to necrosis and fibrosis of A. Lumbricals B. Interossei C. Hypothenar muscles D. Forearm flexor muscles 24. Most common site of trophic ulcer is A. Heel B. Ball of great toe C. Tip of fingers D. Lateral border of foot 25. All are true regarding ‘Hot foot syndrome’ in leprosy, except A. Foot is warm and swollen B. Results from neurological deficit C. Results from underlying osteomyelitis D. Early surgical intervention is necessary

26. The colour of blister pack used in MBMDT regime for adult is A. Green B. Red C. Orange D. Yellow 27. In patients on regular dapsone, monsopot test will show A. Orange color in center, yellow color in periphery B. Yellow color in center, orange color in periphery C. Orange color in center, green color in periphery D. Green color in center, orange color in periphery 28. Most common teeth to be affected in leprosy is A. Molar B. Premolars C. Upper incisors D. Lower incisors 29. Nasal deformity in lepromatous leprosy is due to A. Destruction of nerves B. Atrophy of mucosal glands C. Vascular pathology D. Unknown 30. Laryngeal involvement in lepromatous leprosy includes all of the following except A. Chronic laryngeal obstruction B. Acute laryngeal obstruction C. Involvement above the vocal cords D. Involvement below the vocal cords 31. True statement among the following is A. Lymph nodes in leprosy are matted and rubbery B. Mesenteric lymph nodes are never affected C. Bronchial lymph nodes are affected during reaction D. Histopathology shows focal collection of macrophages in cortex only

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Essentials in Dermatology 32. Renal lesions in leprosy includes all except A. Membranous glomerulonephritis B. Acute pyelonephritis C. Acute tubular necrosis D. Amyloidosis

B. Assess response to treatment C. Resistance to chemotherapy D. Number of solid staining bacilli calculated after examining 200 red staining elements lying singly.

33. AFBs are commonly found in all these site’s except A. Cutaneous nerve bundles B. Subepidermal zone C. Arrector pili muscle D. Eccrine glands

40. M. leprae was discovered by Hansen in the year A. 1911 B. 1899 C. 1873 D. 1940

34. Predominate cell type in indeterminate leprosy A. Lymphocytes B. Epithelioid cells C. Neutrophils D. Macrophages 35. The type of leprosy where many macrophages and numerous lymphocytes are found is A. BL B. LLp C. LLs D. BB 36. Upgrading reaction is characterized by all except A. Increase in neutrophils B. Edema C. Disorganisation of granuloma D. Increase in lymphocytes 37. Not true about Lucio leprosy is A. Common in Brazil B. Marked vasculitis and thrombosis of vessels C. Unusual variant of lepromatous leprosy D. Endothelial cells have abundant AFB 38. In Ridley’s logarithmic scale 4+ denotes A. 10 – 100 bacilli in an average field B. 1 – 10 bacilli in an average field C. 1 – 10 bacilli in 100 field D. 100 – 1000 bacilli in average field. 39. True about morphological index are all except A. Indicates the activity of the disease

41. First to succeed in growing M leprae in a laboratory animal A. Hansen B. Rees C. Shepard D. Rodriguez 42. Foreign body giant cell are commonly found in A. BT B. BL C. LL D. TT 43. The characteristic of leprous facial palsy A. Upper eyelid is involved preferentially B. Both lids are involved C. Lower eyelid is involved preferentially D. Ophthalmic branch is affected first. 44. The autoimmune skin disorder which is commonly associated with LL A. Vitiligo B. Pemphigus vulgaris C. Bullous pempigoid D. Hashimoto’s thyroiditis 45. Hoarseness of voice is the common symptom in laryngeal involvement of leprosy A. Fibrotic form B. Ulcerative form C. Reticulate form D. Both A and B 46. Satellite lesions are a feature of A. BT B. BL C. LL D. TT

Multiple Choice Questions 47. Geographic appearance and inverted saucer appearance are characteristic of A. BB B. BT C. BL D. LL 48. Tenosynovitis in leprosy in characterized by all except A. Occurs in type 1 lepra reaction B. Most common in BT C. Can be unilateral or bilateral D. Does not occur in feet 49. Lazarine leprosy occurs in A. BT and Lucio leprosy B. BL and Lucio leprosy C. BT and BL D. BB and BL 50. Lucio leprosy is characterized by all except A. Diffuse infiltration of face and trunk B. Diffuse alopecia C. Lucio phenomenon D. Good response to thalidomide 51. Following are true about pure neuritic leprosy except A. 60% develops skin lesion subsequently B. Male preponderance C. Children > adults D. Majority are mononeuritic 52. Components of “facies leprosa” includes all except A. Destruction of bony nasal spine B. Destruction of alveolar process of maxilla C. Erosion of zygomatic process D. Leonine facies 53. Manila’s classification divided leprosy into A. Cutaneous, neural, and mixed B. Nodular and anaesthetic C. Lepra tuberosa, lepra cutanae, and lepra nervorum D. Lepromatous, and neural

54. All are true about madarosis in leprosy except A. Reversible with treatment B. Unilateral in tuberculoid C. Bilateral in LL D. Hair transplantation is the treatment 55. The following are true about armadillo except A. Has long life span B. Has cooler core body temperature C. Nerves are deep seated D. Disease does not manifest after inoculation 56. The usual incubation period of leprosy is A. 3–5 years B. 1–10 years C. 10–15 years D. 15–60 years 57. The possible route of transmission of leprosy includes all except A. Skin B. GIT C. Respiratory tract D. Blood transfusion 58. The acid-alcohol mixture used in ZiehlNeelsen method of staining in leprosy A. 20% H2SO4 in 10% alcohol B. 1% HCl in 70% alcohol C. 5% HCl in 70% alcohol D. 10% H2SO4 in 70% alcohol 59. Bacterial Index at the end of 12 months of treatment in lepromatous patients will be A. Reduced by 50% B. Reduced by 25% C. Will be 0 D. No fall 60. The doubling time of M.leprae is A. 11 – 13 days B. 14 – 20 days C. 20 – 25 days D. 5 days

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Essentials in Dermatology 61. The M. leprae looses its acid fastness if stained with A. Povidone B. Pyridine C. Carbol fuschin D. H2SO4 62. In which year NLEP was formed? A. 1982 B. 1898 C. 1900 D. 1850 63. In which year Indian leper’s act came into existence? A. 1982 B. 1898 C. 1900 D. 1850 64. True about glycolipid in M.leprae are all except A. Forms foaming material inside the macrophages B. Species specific C. Antiglycolipid antibodies are used in ELISA D. Not species specific 65. True about lepromin reaction A. Early reaction is due to delayed type hypersensitivity B. Mitsuda reaction occurs at 48 hrs C. Mitsuda is strongly positive in LL D. Fernandez reaction is read after 48 hours.

68. Not true about histoid leprosy A. Presence of globi B. Pseudocapsule C. Spindle shaped histocytes with bacilli D. Normal skin BI- 0 69. Not true about ENL is A. A type IV hypersensitivity reaction B. Immune complex formation occur C. Increase in neutrophilic infiltrate D. Fibrinoid deposits around the vascular walls 70. The presence of ceroid in histological section of treated leprosy is due to A. Dead bacilli B. Clofazimine C. Destruction of granuloma D. Destruction of cell wall of bacilli 71. Average period of survival of M. leprae outside the body is A. 84 hrs B. 24 hrs C. 36 hrs D. 30 days 72. In which classification indeterminate leprosy was introduced? A. Manila B. Cairo C. Havana D. WHO

66. True about PGL based ELISA is A. Anti PGL – 1 Abs are demonstrated in all the spectrum of disease B. Antigenemia disappears after chemotherapy C. May be present in treated cases D. None of the above

73. The revised IAL classification, include A. Lepromatous, tuberculoid, maculoanesthetic, borderline, polyneuritic, and indeterminate leprosy B. Tuberculoid, lepromatous, borderline, indeterminate, and pure neuritic C. LL, TT, BT, BL, I, and pure neuritic D. Lepromatous, tuberculoid, and borderline leprosy

67. The substance which gives acid fastness in Fite-Faracco’s stain is A. Pyridine B. Carbol fuschin C. Arachis oil D. Methylene blue

74. The characterstic of BT leprosy are all except A. 10 – 20 lesions B. Well and ill defined margin C. Satellite lesions D. Nerves symptomatically involved.

Multiple Choice Questions 75. The types of histoid leprosy include all except A. Subcutaneous nodule B. Papular type C. Deep cutaneous nodule D. Plaques 76. The muscles supplied by ulnar nerve includes all except A. Adductor pollicis B. Palmaris brevis C. Opponens policis D. 3rd and 4th lumbricals 77. Sensation for the sole of foot is carried by A. Common peroneal nerve B. Anterior tibial nerve C. Posterior tibial nerve D. Deep peronial nerve 78. Dapsone induced peripheral neuropathy is characterized by A. Tender nerves and normal reflexes B. Non tender nerves and normal reflexes C. Non tender nerves and reduced reflexes D. Tender nerves and reduced reflexes 79. Not true about clofazimine is A. Reversible skin pigmentation B. Irreversible skin pigmentation C. Anticholinergic action D. Phototoxicity 80. In dapsone sensitive PB-patients, dapsone can be replaced with A. Ofloxacin B. Minocycline C. Clofazimine D. Clarithromycin 81. U-MDT means, A. MB- MDT for 18 months B. MB-MDT for 12 months C. MB – MDT for 6 months D. MB – MDT for life long 82. High – endemic area in leprosy means A. Prevalence rate > 3/1000 population B. Prevalence rate > 5/1000 population

C. Prevalence rate > 1/1000 population D. Prevalence rate >10/1999 population 83. Which of these combination is acceptable in HIV – leprosy patient? A. Rifampicin – Zidovudine B. Rifampicin – Saquinavir C. Rifampicin – Nelfinavir D. Rifampicin – Indinavir 84. “Small bowel syndrome” occurs with A. Clofazimine B. Ethionamide C. Pefloxacin D. Rifapentine 85. The zoological name of nine banded armadillo A. Dasypus novemcinctus B. Erinaceus europaeus C. Citellus tridecemlineatus D. Tamias sibiricus 86. Most common procedure done for lagophthalmos A. Medial tarsorrhaphy B. Lateral tarsorrhaphy C. Temporalis muscle facial transfer D. Frontalis muscle facial transfer 87. Chaulmoogra oil as a therapy for leprosy was first introduced formally by A. Hansen B. Mouat C. Danielssen D. Sheskin 88. Ridley and Jopling recommended five systems of classification of leprosy in the year A. 1987 B. 1962 C. 1943 D. 1981 89. The first use of dapsone for leprosy was by A. Cochrane B. Faget C. Johansen D. Peterson

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Essentials in Dermatology 90. Thalidomide was found to be used in the treatment of erythema nodosum leprosum by A. Mouat B. Danielssen C. Peterson D. Sheskin 91. WHO recommended the use of multi drug therapy for leprosy in the year A. 1981 B. 1976 C. 1957 D. 1990 92. The first person to use dapsone orally were A. Faget and Muir B. Lowe and Smith C. Wade and Rodriguez D. Motta and Zuniga.

93. Gerhard Henrik Armauer Hansen was a A. Mexican B. American C. Norwegian D. Canadian. 94. Evidence of leprosy in India dates back to A. 2000 BC B. 50 AD C. 1000 AD D. 600 BC 95. Prior to 1943, the treatment of leprosy continued to be A. Chaulmoogra oil B. Intravenous Promin C. Diasone D. Rifampicin

Some Useful Medical Mnemonics

Some Useful Medical Mnemonics

A mnemonic is a device or code that helps one to memorize something. It is derived from the Greek term mnemon meaning “mindful”. In other words, a mnemonic helps one to keep something in mind. Mnemonics have been established as a useful tool in teaching a subject. Although mnemonics have limitations and should not replace traditional methods of teaching and learning, they are a useful education tool. Good mnemonics can increase the speed and amount of retained factual information, especially during stressful situations, such as examinations. A mnemonic is of great help in recalling the features of certain conditions, for example in systemic lupus erythematosus, the mnemonic ANTINUCLEAR A for antinuclear antibody, N for neurologic disorders such as seizures or psychosis, T for thrombocytopenia or lymphopenia or leucopenia or hemolytic anemia, I for immunologic features like positive LE cell preparation or anti ds DNA or anti sm antibodies or a positive test for syphilis, N for nasopharyngeal or oral ulcers, U for urinary abnormalities like proteinuria or casts, C for cutaneous discoid rash,

L for light sensitivity, E for effusions – pleural or pericardial, A for arthritis of two or more joints and R for a rash in the malar area This mnemonic enables students to recall the modified criteria confidently and quickly when examining a patient. In the mnemonic BIT CAFÉ for café au lait spots, the commonest condition seen by clinicians is placed last B for blooms, I for idiopathic, T for tuberous sclerosis, C for congenita (dyskeratosis), A for ataxia telangiectasia, F for Fanconi’s and E for elephant man (neurofibromatosis) The possible causes of erythema nodosm are recalled by the mnemonic NODOSUM NO cause in 60%, D for drugs, O for oral contraceptives, S for sarcoidosis, U for ulcerative colitis and M for microbiology: bacterial, viral, yersinia, tuberculosis, leprosy, deep fungal infections Seven P’s in telogen effluvium pregnancy,

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Essentials in Dermatology protein depletion, pills, propranolol, pyrexia, parturition and psychic stress Seven P’s in lichen planus purple, polygonal, planar or flat, papules, pruritic, persistent and penile Cockayne’s syndrome lies in COCKAYNE’S C for CNS defects, O for lipOdystrophy, C for color which is hyperpigmentation, A for auditory symptom of deafness, Y for microencephalY, N for normal pressure hydrocephalus, E for eye lesion of salt and pepper retina and S for short Describing skin lesions LES. T CABS Location and distribution (e.g. bilateral/symmetrical) Erythema (e.g. yes or no) Surface features (e.g. scale, verrucous) Type (e.g. papule, nodule, vesicle) Color (e.g. violaceous, blue) Arrangement (e.g. grouped) Border/shape (e.g. linear, serpiginous) Special sites/systemic (e.g. oral, nails, scalp) Generalized skin hyperpigmentation “With generalized, none of the skin is SPARED” Sunlight Pregnancy Addison’s disease Renal failure Excess iron (hemochromatosis) Drugs (e.g. amiodarone, minocycline)

Painful cutaneous nodules BENGAL CO Blue rubber bleb nevus Eccrine spiradenoma Neurilemmoma/neuroma Glomus tumor Angiolipoma/angioleiomyoma/angiosarcoma Leiomyoma Cutaneous endometriosis/calcinosis cutis Osteoma cutis White patch of skin “Vitiligo PATCH” Vitiligo Pityriasis alba/post-inflammatory hypopigmentation Age related hypopigmentation (e.g. idiopathic guttate hypomelanosis) Tinea versicolor, tuberous sclerosis (ash-leaf macules) Congenital birthmark (e.g. hypomelanosis of Ito) Hansen’s disease (leprosy) Common causes of leukocytoclastic vasculitis “VASCULITIS” Viral (e.g. hepatitis B and C) Autoimmune (systemic lupus erythematosus, Sjögren’s syndrome, rheumatoid arthritis) Streptococci, staphylococci, Henoch-Schönlein purpura Cryoglobulins, cryofibrinogens, Churg Strauss/ Wegener’s granulomatosis Ulcerative colitis, urticarial vasculitis Lymphoproliferative disease (hairy cell leukemia) Infectious (endocarditis, meningococcemia, gonococcemia, rocky mountain spotted fever) Thiazides, phenothiazines, and other drugs Immune complex reactions, iodides, idiopathic Sulfa drugs (septran), penicillin, and other antibiotics Non-scarring alopecia TOP HAT Telogen effluvium, Tinea capitis Out of iron, zinc Physical-trichotillomania, traction alopecia Hormonal-hypothyroidism, androgenic Autoimmune-alopecia areata, anagen effluvium Toxins-heavy metals, chemotherapy

Some Useful Medical Mnemonics Behcet’s syndrome: Diagnostic criteria PROSE* Pathergy test Recurrent genital ulceration Oral ulceration (recurrent) Skin lesions (e.g. erythema nodosum, subcutaneous thrombophlebitis, cutaneous hypersensitivity) Eye lesions (e.g. iridocyclitis, chorioretinitis) * Oral ulceration is central criterion, plus any 2 others Focal dermal hypoplasia syndrome/Goltz syndrome “FOCAL” Female sex (85–90%) Osteopathia striata Coloboma Absent ecto-, meso-, and neuro-dermis elements Lobster claw deformity SLE (Systemic Lupus Erythematosus) diagnosis SOAP BRAIN MD* Serositis Oral/nasal ulcers Arthritis Photosensitivity Blood (cytopenia) Renal involvement ANA Immune (typical antibodies e.g. dsDNA, anti-Sm) Neurologic (e.g. Seizures, stroke) Malar rash Discoid rash * 4 out of 11 criteria needed for diagnosis Henoch-Schonlein purpura: signs and symptoms NAPA Nephritis Arthritis, arthralgias Purpura, palpable (especially on lower extremities) Abdominal pain (intussusception to be ruled out) Classification of hypersensitivity reactions “ACID”

Type I Anaphylaxis Type II Cytotoxic - mediated Type III Immune - complex Type IV Delayed hypersensitivity Multiple Endocrine Neoplasia: Each of the MENs is a disease of three or two letters plus a feature. MEN I is a disease of 3 P’s (pituitary, parathyroid, and pancreas) plus adrenal cortex MEN II is a disease of 2 C’s (carcinoma of thyroid and catacholamines [pheochromocytoma]) plus parathyroid for MEN IIa or mucocutaneous neuromas for MEN IIB (aka MEN III) WBC Count: “Never Let Mom Eat Beans” and “60, 30, 6, 3, 1” Neutrophils 60% Lymphocytes 30% Monocytes 6% Eosinophils 3% Basophils 1% LR6 SO4 3. Which cranial nerve controls which eye muscle? Lateral rectus sixth nerve, superior oblique fourth, rest third. Bones, stones, groans and psychic moanssymptoms of an elevated serum calcium level. C 3, 4 and 5, keeps your diaphragm alive. S 2, 3 and 4, makes a mess on the floor. Psoriasis: Pathophysiology PSORIASIS : Pink Papules/ Plaques/ Pinpoint bleeding (Auspitz sign)/ Physical injury (Koebner phenomenon)/ Pain Silver Scale/ Sharp margins Onycholysis/Oil spots Rete Ridges with Regular elongation Itching Arthritis/ Abscess (Munro) Stratum corneum with nuclei, neutrophils Immunologic Stratum granulosum absent/ Stratum Spinosum thickening

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Essentials in Dermatology Delay in wound healing DID NOT HEAL: Drugs Infection/ Icterus/ Ischemia Diabetes Nutrition Oxygen (hypoxia) Toxins Hypothermia/Hyperthermia Edema Acidosis Local anesthetics Cutaneous inflammation patterns “Pus of Pig Valve”· Remove the vowels: PSFPGVLV: Psoriasiform Spongiotic (eczematous) Folliculitis Panniculitis Granulomatous Vasculopathic Lichenoid Vesiculobullous Clubbing causes CLUBBING: Cyanotic heart disease Lung disease (hypoxia, lung cancer, bronchiectasis, cystic fibrosis) UC/Crohn’s disease Biliary cirrhosis Birth defect (harmless) Infective endocarditis Neoplasm (esp. Hodgkins) GI malabsorption Albinisim type I vs. type II “One has None. Two Accumulates”: Type I: have no pigment. Type II: No pigment at birth but accumulates as person ages. Malignant melanoma: 3 sites with poor prognosis BANS:

Back of Arm Neck Scalp Symptoms of hyperthyroidism Remember the following mnemonic when evaluating patients for hyperthyroidism: S: Sweating T: Tremor or Tachycardia I: Intolerance to heat, Irregular menstruation, and Irritability N: Nervousness G: Goiter and Gastrointestinal (loose stools/ diarrhea). 5 T’s of early cyanosis: Tetralogy, Transposition, Truncus, Total anomalous, Tricuspid atresia Causes of hematuria Use the mnemonic SITTT as an aid in evaluating the cause of hematuria: S: Stone I: Infection T: Trauma T: Tumor T: Tuberculosis Causes of postoperative fever Remember the following mnemonic when determining the possible cause (s) of fever in a patient who has recently undergone a surgical procedure: the 5 W’s (or 6 W’s) Wind: The pulmonary system is the primary source of fever in the first 48 hours. Wound: There might be an infection at the surgical site. Water: Check intravenous access site for signs of phlebitis. Walk: Deep venous thrombosis can develop due to pelvic pooling or restricted mobility related to pain and fatigue. Whiz: A urinary tract infection is possible if urinary catheterization was required. Also Wonder drugs - drug fevers.

Some Useful Medical Mnemonics Proven MI (Myocardial infarction), should be met by M.O.N.A. M = morphine O = oxygen N = nitrates A = aspirin Caveat: If right ventricular MI suspected, hold the Nitrates. Aortic Branches: ABC’S Aortic arch gives off the Brachiocephalic trunk, the left Common Carotid, and the left Subclavian artery Argyll-Robertson Pupil—syphilitic pupil: Accommodation reflex present, Pupillary reflex absent due to damage at pretectal area. Also called the “prostitute’s pupil” (accommodates but does not react). Arterial occlusion: pain pallor pulselessness paresthesias Causes of Pancreatitis BAD SHIT Black scorpion bite Alcohol Drugs (tetracycline, azathioprine, sulfa) Stones (gallstones) Hyperlipidemia Idiopathic Trauma Microcytic Anemia: “TICS”Thalassemia Iron deficiency Chronic disease Sideroblastic anemia Kawasaki’s disease: ”SCREAM FEVER” S - sausage fingers

C - conjunctival redness R - rash E - extremity involvement A - adenopathy M - mucosal erythema FEVER – fever Eosinophilia: “NAACP” N - Neoplasm A - Allergy A - Addison’s C - Cirrhosis, CVD P - Parasite (visceral larva migrans), Periarteritis nodosa Endocarditis: “FAME” F - FEVER A - ANEMIA M - MURMUR E - ENDOCARDITIS Tumors that go to bone: “Kinds of tumors leaping primarily to bone” K - Kidney O - Ovarian T - Testicular L - Lung P - Prostate T - Thyroid B – Breast Hypercalcemia: “SHAMPOO DIRT” S - Sarcoidosis H - Hyperparathyroidism, hyperthyroidism A - Alkali-milk syndrome M - Metastases, myeloma P - Paget disease O - Osteogenesis imperfecta O - Osteoporosis D - Vitamin D intoxication I - Immobility

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Essentials in Dermatology R - RTA T – Thiazides Henoch-Schonlein Purpura: “JARS” J - Joints A - Abdominal pain R - Renal S – Skin

Acute Rheumatic Fever: There are several for the major criteria, I use “JONES” J - Joints O - Obvious (cardiac) N - Nodules (subcutaneous nodules) E - Erythema marginatum S - Sydenham’s chorea

Terminology in Dermatology, Sexually Transmitted Diseases and Leprosy

Terminology in Dermatology, Sexually Transmitted Diseases and Leprosy

Abscess: A localized collection of pus in a deep-seated cavity. Acantholysis: Separation of epidermal keratinocytes by loss of their intercellular connections causing the cells to become rounded. Acanthosis: Increase in thickness of prickle-cell layer of skin. Acne: Term used for a chronic inflammatory disease of the pilosebaceous follicles showing a keratinous plug, commonly referred to as acne vulgaris. Acrochordon: Skin tag. Actinic: Pertaining to rays or beams of light. Actinomycosis: A chronic suppurative infection of man and animals due to a species of Actinomyces. Ray-fungus disease. Acuminate: Sharp, pointed e.g., condyloma acuminata. Ainhum: Spontaneous separation of digit by constricting fibrous band. Albinism: Congenital hypopigmentation of skin, hair and eyes. Alkaptonuria: An inborn error of tyrosine metabolism leading to deposition of oxidized homogentisic acid in fibrous and cartilaginous tissue and characterized by dark urine. Allergy: Originally ‘altered immunological reactivity to antigens’ Alopecia: Loss of hair from normal hairy regions of the body. Amiantacea (pityriasis): Name given to a condition in which soft, asbestos-like scales adhere to scalp hairs. Anaphylaxis: Allergic reaction in which antigen releases mediators (cytokines) from antibodysensitized mast cells or basophils. Anetoderma: Macular atrophy of the skin. Angiokeratoma: A vascular lesion combining ectasia of dermal blood vessels with hyperkeratosis. Anhidrosis: Lack of sweating.

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Essentials in Dermatology Anthrax: Acute infective zoonosis of herbivorous animals, sometimes transmitted to man, caused by Bacillus anthracis and produces “Malignant pustule” in the skin (Greek anthrax charcoal). Anthropophilic: Preferring humans as host. Aphthae: Painful ulcers of the mucosae (usually oral cavity), often multiple and recurrent. Aplasia: Failure or lack of growth or development. Apocrine sweat glands: These tubular glands consists of two main parts – the coiled secretary gland and the straight excretory duct which opens into follicular canal just above the openings of sebaceous glands. They are distributed along the mammary line, i.e. axillae, areolae, periumbilical area, mons pubis, genital and perianal areas. Appendages: The epidermis gives rise to derivative structures (pilosebaceous unit, nails and sweat glands) called as appendages. Aquagenic: Caused by contact with water. Arachnodactyly: Increased length of fingers and toes, from resemblance to spider’s legs. Argyria: Ashen-grey discoloration of the skin and conjunctivae, resulting from chronic exposure to silver or its salts. Arthus phenomenon: Immunological reaction at site of injection of a substance (antigen) to which the recipient has previously formed precipitating-type antibodies, resulting in formation of antigen— antibody complexes causing tissue damage, especially of a vasculitic nature. Atopic disorders: The anaphylactic-type of disorder that results from IgE mediated reactions. Atopy: State of excessive formation of lgE antibody following exposure to common environmental allergens. Atrophy: A wasting, shrinkage, diminution or lack of tissue. Balanitis: Inflammation of glans penis (or glans clitoris). Balanoposthitis: Inflammation of glans penis and prepuce. Ballooning degeneration of epidermis: A type of degeneration of epidermal cells causing marked swelling of the cells with loss of intercellular bridges, occurs in viral vesicles. Basement membrane zone: Located beneath the basal layer, consists of the basement membrane and of the lamina lucida, anchoring fibrils, and reticulum fibres. Basidiobolomycosis: Disease caused by a genus of fungus causing subcutaneous phycomycosis. Bazin’s disease (tuberculous): Erythema induratum affecting legs of young women. Beau’s lines: Transverse depressions on the nails following acute illnesses or debilitating medical events. Behçet’s syndrome: Triad of orogenital ulceration and iritis. Pyoderma may occur and central nervous system and other organs may be involved. Bence-Jones protein: A low-molecular-weight protein found in urine of patients with myeloma. Berloque dermatitis: A streaky form of pigmented contact photodermatitis, usually seen in the neck but occasionally elsewhere, due to psoralen, as in bergamot oil in perfume. Besnier’s prurigo: Flexural form of atopic dermatitis.

Terminology in Dermatology, Sexually Transmitted Diseases and Leprosy Blaschko’s lines: A system of lines in the human skin along which many linear naevi and dermatoses arrange themselves. Boil: A term for furuncle Bourneville—Pringle syndrome: Tuberous sclerosis; epiloia. Bowen’s disease: Precancerous dermatosis; a form of carcinoma in situ. Bromhidrosis: Foul-smelling sweat, usually of axillary apocrine origin. Bubo: A swollen inflamed lymphatic gland, especially in neck, axilla or groin. Buerger’s disease: Thromboangiitis obliterans. Bulla: Bleb, blister. An elevated lesion of the skin containing clear fluid and over 0.5 cm diameter. Burrow: A track, passage or tunnel in the skin that houses a parasite, particularly the mite of scabies Buruli ulcer: Mycobacterium ulcerans infection, named after an area of the Nile in Uganda. Buschke—Loewenstein tumor: Giant condyloma acuminata. Callosity and corn: A callosity is a superficial circumscribed area of hyperkeratosis at a site of repeated friction, usually over a bony prominence on the hands or feet whereas a corn is a painful conical hyperkeratosis that occurs principally over toe joints (hard corn) and between toes (soft corn). Canities: Graying or whitening of hair. Carbuncle: A necrotizing pyogenic infection of the skin and subcutaneous tissue composed of a group of furuncles (boils). Casal’s necklace: Collar area of erythema and pigmentation around neck seen in pellagra. Castellani’s paint: Consists of basic fuchsin, carbolic and boric acids, acetone and resorcinol. Once widely used for epidermophytosis, now less so, or in modified form. Chancre: Usually, syphilitic ulcer developing at site of infection with T. pallidum; also, rarely, for lesion at inoculation site of sporotrichosis, tularemia or tuberculosis. Cheilitis: Inflammation of the lips. Cheiropompholyx: Blistering eruption of the hands. Cheloid: A more correctly derived spelling for Alibert’s KELOID. Alibert noted resemblance to feet of crayfish or crabs and hence the name. Chilblain: Lesion chiefly affecting fingers, toes and ears caused by a vascular response to low temperature (cold) and humidity. Chlamydia: Genus of tiny intracellular parasites classified as bacteria; the cause of psittacosis — lymphogranuloma venereum — trachoma group of infection. Chloasma (melasma): Patchy brownish pigmentation affecting particularly malar area of face. Chrysiasis: Deposition of gold within tissue. Clutton’s joints: Painless joint effusion (commonly of knees) in late congenital syphilis. Coccus, cocci: Spherical bacillus grouped in pairs, chains or clusters. Collagen: Collagen is the most abundant protein in the body and constitutes about 70% of the dry weight of the dermis. It serves as its major structural component and has remarkable tensile strength.

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Essentials in Dermatology Colloid bodies: Another name for Civatte bodies, are round or oval, 10 micrometer eosinophilic homogeneous bodies formed due to degeneration of epidermal cells and are extruded into the dermis. Comedo: A greasy plug in a sebaceous follicle. Condyloma: A wart-like growth due to hypertrophy of prickle-cell layer, commonly used for genital and perianal lesions of viral (acuminata) or syphilitic (lata) origin. CREST (CRST) syndrome: Calcinosis, Raynaud’s phenomenon, (Esophageal involvement), Sclerodactyly, Telangiectasia. Cryo: Meaning cold. CTCL: Cutaneous T-Cell Lymphoma. Cullen’s sign: Bluish staining around umbilicus usually denoting acute pancreatitis or ruptured ectopic pregnancy. Cushing’s syndrome: Due to adrenocortical hypertrophy. The term ‘Cushingoid’ is applied to the appearance caused by high-dose corticosteroid therapy. Cutis laxa: Laxity or fold-like formation of skin. Cyst: Any closed cavity or sac (normal or abnormal) with an epithelial, endothelial or membranous lining and containing fluid or semisolid material. Dactylitis: Inflammation or infection of a finger. Dandruff: Popular term for pityriasis capitis, a mild form of seborrheic dermatitis. Darier’s disease: Keratosis follicularis. Decalvans: Causing loss of hair, e.g. folliculitis decalvans. Decubitus ulcer: Bedsore; pressure sore due to prolonged recumbency without movement de Morgan’s spots (Campbell de Morgan’s spots): Cherry angiomas. Dermabrasion: Surgical technique of removal of superficial lesions of the skin by abrasive methods such as with high-speed rotary drills, or hand used dermabrader. Dermal-epidermal junction: The dermal-epidermal junction (DEJ) is a basement membrane zone (BMZ) that welds the epidermis to underlying dermis. It is undulated, forming dermal papillae (upward projections of the dermis into the epidermis) and rete ridges (downward projections of epidermis into the dermis). This DEJ is weakest at lamina lucida. Dermatoglyphics: It is the study of the highly characteristic epidermal ridge patterns of the volar skin of palms, soles, fingers and toes. Dermis: The dermis is formed by connective tissue having fibres (collagen, elastic and reticulin) and ground substance. It varies in thickness from about 1 mm on the face to 4 mm on the back and thigh. Dhobi (dhobie, dhoby): Indian washerman, ‘dhobie itch’ a colloquial term used by servicemen for tinea cruris. Diascopy: Examination of skin with exclusion of blood by firm pressure of glass, etc.; vitropression. DIC: Disseminated Intravascular Coagulation Ducrey’s Bacillus: Haemophilus ducreyi, the cause of chancroid. Duhring-Brocq disease: Dermatitis herpetiformis.

Terminology in Dermatology, Sexually Transmitted Diseases and Leprosy Dyskeratosis: Means faulty and premature keratinization of individual keratinocytes occurs in certain acantholytic diseases and epidermal neoplasias. Dysidrosis (better, dyshidrosis or pompholyx) literally, difficult sweating. Name given for an acute vesicular eruption of palms and/or fingers resembling ‘small boiled sago grains’ and previously thought to be caused by poral occlusion and sweat retention, hence the above name. Ecchymosis: Synonymous bruise. A macular red or purple or colored hemorrhage in skin or mucous membrane more than 2 mm in diameter. Eccrine sweat glands: They are tubular structures, which open on to the skin directly. The three parts of them are coiled glandular portion, the straight dermal ductal portion and spiral intraepidermal ductal portion. Sweat glands are most abundant on the palms, soles, forehead and axillae. These glands are highly developed and responsive part of the thermoregulatory apparatus. Ecthyma: A deep crusted pyogenic infection, usually on legs. Eczema: An inflammatory skin reaction characterized histologically by spongiosis and clinically by a variety of features, notably vesiculation (Greek ek out, zeein to boil). Ehlers-Danlos syndrome: A group of heritable disorders of connective tissue, characterized by hyperextensibility, fragility, easy bruising and poor skin healing, to varying degrees in different types. Elastic fibers: Elastic fibres constitutes about 3% of the dry weight of the dermis. The characteristic property of elastic fibres is that they can be stretched by 100% or more and still return to their original dimensions. ELISA: Enzyme-Linked Immunosorbent Assay. EMLA: Eutectic Mixture of Local Anesthetics. ENL: Erythema Nodosum Leprosum. Ephelis: Commonly, a freckle. Epidermis: The epidermis is continually renewing, stratified, squamous epithelium that keratinizes and gives rise to derivative structures (pilosebaceous unit, nails and sweat glands) called appendages. It is approximately 0.4 mm to 1.6 mm in thickness. The majority of the cells in the epidermis are the keratinocytes. Epidermotropism: Means presence of mononuclear cells in the epidermis with little or no spongiosis occurring in mycosis fungoides. Epiloia: A term coined to denote a triad of Epilepsy, Low Intelligence and Adenoma sebaceum. Epithelium: The cellular covering of the skin and mucous membranes. Epstein—Barr virus: A herpesvirus causing infectious mononucleosis, oral hairy leukoplakia. Erysipelas: An acute infection of the skin by the hemolytic streptococcus, St Anthony’s fire Erythema: Redness of the skin produced by vascular congestion or perfusion. Esthiomene: A destructive lesion of vulva or clitoris occurring in lymphogranuloma venereum associated with lymphedema. Exocytosis: Means presence of mononuclear cells in the epidermis in acute and subacute dermatitis.

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Essentials in Dermatology Favus: A fungal infection of the scalp, usually caused by Trichophyton schoenleinii. It forms a characteristic cup-shaped crust (scutula), which, on removal, exposes an oozing red surface. Fernandez reaction: A delayed-type of hypersensitivity reaction to Dharmendra antigen in leprosy. Fibroblasts: Fibroblasts are the most numerous of the cells found in loose connective tissue of dermis. They are the source of the ground substance, collagen and elastic fibers. Follicular plug: Hyperkeratosis distending a hair follicle opening. Fordyce angiomata: Scrotal angiomata. Fordyce spots: Ectopic sebaceous glands of inner surface and vermilion of lip and oral mucosa, appearing as creamy-coloured symptomless lesions. Fournier’s gangrene: Fulminating gangrene of the external genitalia. Fournier’s sign: Bowing of the tibia seen in osteitis deformans, syphilis and yaws. Fox-Fordyce disease: (Synonyms lichen axillaris, apocrine miliaria) intensely pruritic papular eruption involving apocrine ducts in axillae, breasts or genital area. Freckle: (Synonym ephelis) light-brown macules occurring in sun-exposed skin, especially in fairskinned persons. Frei’s disease: Lymphogranuloma venereum. Frei’s test: A diagnostic intradermal test of limited value for lymphogranuloma venereum. Functions of the skin: Main functions of the skin are protection, thermoregulation, sensory, storage organ, vitamin-D formation, absorption, excretion, immune surveillance, mechanical and cosmetic function. Furuncle: A localized painful nodular pyogenic infection originating around a hair follicle. Gangrene: Commonly used to describe necrotizing and sloughing lesions but more properly referring to death of tissue resulting from ischaemia. Ghon’s focus: Primary tuberculous complex of the lung. Giant cell: A cell with more than one nucleus. Langhans giant cell has peripheral ring of nuclei whereas foreign body giant cell has haphazard arrangement of nuclei. Gibert’s disease: Pityriasis rosea. Glabrous: Smooth; commonly used to designate smooth, hairless skin; properly skin without any hair follicles. Gottron’s sign: Erythematous papules over metacarpal and proximal interphalangeal joints in dermatomyositis. Granuloma: A chronic inflammatory lesion showing accumulations of macrophages which have undergone epithelioid transformation, with or without lymphocytes and multi-nuclear giant cells; more loosely, a nodular chronic inflammatory lesion arising in response to a variety of stimuli. Graves’ disease: Hyperthyroidism due to diffuse toxic goitre. Grenz zone: A zone of normal dermis overlying deeper pathological changes. Ground substance: Ground substance is an amorphous material that consists mainly of water, electrolytes, plasma proteins and mucopolysaccharides. A mucopolysaccharide consists of a longchain aminated polysaccharide (glycosaminoglycan) linked covalently to a polypeptide.

Terminology in Dermatology, Sexually Transmitted Diseases and Leprosy Gumma: A characteristic though inconstant lesion of tertiary syphilis, of a rubbery consistency and with a fibrous capsule, it consists of necrotic tissue and epithelioid, multinucleated and plasma cells. Also (rarely) used for tuberculous. Gynaecomastia: Enlargement of breasts. Hair: Hair structurally consists of a cuticle, cortex and medulla. These keratinous fibers are of two types in adults. Terminal hairs are thick, pigmented and long and are seen on the scalp, eyebrows, axillae and genital areas, etc. Vellus hairs are small in diameter, short and nonpigmented and therefore difficult to discern. Hair cycle: Hair grows at the rate of 0.3 mm per day and they undergo growth cycle. These recurring cycles consist of anagen (active growth phase), catagen (static growth phase) and telogen (shedding phase) phases. On the scalp, 80 to 85% of hair is in anagen phase and 15 to 20% in catagen and telogen phase. The anagen phase lasts for 2 to 5 years, a short catagen of few days and a telogen phase of 3 months. Hailey—Hailey disease: A term for benign familial pemphigus. Hamartoma: A benign tumor or tumor-like lesion composed of tissue normal for the body but inappropriate for the site; or abnormally mixed or overgrown. Broadly, naevoid anomaly. Hansen’s disease: A term for leprosy. After Hansen, who discovered the Mycobacterium leprae bacillus in 1873. Hartnup disease: A rare metabolic disorder comprising pellagra, cerebellar ataxia and indicanuria. Heberden’s nodes: Bony swellings of the distal interphalangeal joints of the hands, indicative of osteoarthritis. Henoch - Schonlein purpura: Anaphylactoid purpura. “JARS” J – Joints, A-Abdominal pain, R – Renal, S – Skin. Herpes: Originally used to designate a spreading or ‘creeping’ eruption. Now applied to herpes simplex, herpes zoster and herpes gestationis — all conditions in which a vesicular eruption appears to extend or ‘creep’ in clusters or in a linear fashion. Herpetiform-herpes-like. Grouped vesicles resembling herpes. Hess (capillary) test: A tourniquet test used to determine capillary fragility. Hiemalis: Pertaining to winter, e.g., dermatitis hiemalis. Hippocrates: Greek physician (460 BC- 377 BC), ‘The Father of Medicine’. Hippocratic oath, facies; aphorisms of Hippocrates; etc. Hippocratic nails: Broad, convex, clubbed nails; acropachy. Hirsutism: The growth of hair in women in the male sexual pattern but more commonly any abnormal degree of coarse hairiness. HIV: Human Immunodeficiency Virus. Hives: Term for urticaria; ‘nettle-rash’. Holocrine: Shedding completely, as applied to sebaceous glands, the secretion of which is formed by the complete disintegration of the glandular cells. Horner’s syndrome: Miosis, enophthalmos, ptosis and absence of sweating due to lesion of ipsilateral cervical sympathetic chain or ganglia.

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Essentials in Dermatology Hutchinson J’ Sir: British surgeon (1828—1913 AD). Outstanding figure in late 19th-century British medicine. Possessed an extraordinary visual memory, he made wide-ranging contributions to many aspects of medicine and surgery. His output was prodigious. ‘A most careful observer and fascinating teacher.’ Hutchinson’s teeth: Barrel-shaped incisors with a deep concentric notch seen in congenital syphilis. ‘Pegged’, ‘screwdriver’ teeth. Hutchinson’s triad: Interstitial keratitis, nerve deafness and tooth deformities in congenital syphilis. Hydropic degeneration of basal cells: A type of degeneration causing vacuolization of the basal cells, also called as liquefaction degeneration. It occurs in lupus erythematosus, dermatomyositis, lichen sclerosus et atrophicus, lichen planus, etc. Hypergranulosis: Increase in thickness of the granular layer of the epidermis. Hyperkeratosis: Increase in the thickness of stratum corneum of the epidermis. Ichthyol: A natural tar-like substance derived from bituminous deposits rich in fossilized fish. Ichthyosis: A group of disorders of keratinization characterized by fine scaling and a feeling of dryness of the skin (Greek ichthya rough fish skin from ichthys fish, osis). -id, -ide: In dermatology the term ‘ide’, ‘id’, denotes a reaction occurring in a part remote from the primary lesion, usually but not invariably due to an immunological reaction to the agent concerned or to its component parts e.g. tuberculide, syphilide, trichophytide. Idiopathic: Having no known cause. Impetigo: A contagious eruption of the skin caused by the Streptococcus/Staphylococcus. Incontinence of pigment: Loss of melanin from the cells of basal layer due to damage to these cells. It is taken up by macrophages in the dermis. Intertrigo: Inflammation of apposed skin surfaces such as groins, axillae and inframammary areas. Ischaemia: State of lack of blood supply to a part or organ; arterial insufficiency. Jarisch- Herxheimer reaction: A focal exacerbation of lesions when a disease of infective origin is treated with potent antimicrobial agents. Seen in treatment of early syphilis with penicillin. Kala-azar: Visceral form of leishmaniasis (Hindi kala black, Persian azar disease, poison). Also known as Dum-Dum fever after a town near Calcutta, India. Kaposi’s sarcoma: Multiple idiopathic haemorrhagic sarcoma. Kaposi’s varicelliform eruption: Disseminated primary infection with viruses of herpes simplex, vaccinia and perhaps Coxsackie A16, seen especially in atopic subjects. Now termed ‘eczema vaccinatum’ and ‘eczema herpeticum’. Kawasaki’s disease: Mucocutaneous lymph node syndrome. “SCREAM FEVER” S - sausage fingers, C - conjunctival redness, R-rash, E - extremity involvement, A-adenopathy, M - mucosal erythema, FEVER-fever Keloid: Alternative term for CHELOID and preferred by some in common usage. Kerion: An inflammatory granuloma of hair-bearing areas due to superficial fungal infection, particularly of zoophilic species.

Terminology in Dermatology, Sexually Transmitted Diseases and Leprosy Koebner’s phenomenon, reaction: The induction of a lesion of certain diseases such as psoriasis and lichen planus following physical or non-specific trauma to the unaffected skin. Isomorphic effect. Koch’s postulates, law: The law of specificity of bacteria. Four conditions must be satisfied to establish the causative organism of a specific disease: the organism must be present in every case; it must be isolated and grown in pure culture; when this is inoculated into susceptible animals it must reproduce the disease; and the organism must be grown again in pure culture. Koenen’s tumors: Periungual fibromata occurring in tuberous sclerosis. Kogoj’s spongiform pustule: Epidermal pustule containing polymorphonuclear leucocytes seen in active or pustular phases of psoriasis. Koilonychias: Spoon-shaped nails Koplik spots: Irregular red spots with grayish centres seen in oral mucosa during the prodromal period of measles. Kveim (Kveim-Siltzbach) test: A diagnostic test for sarcoidosis using intradermal injection of sarcoid tissue. Kwashiorkor: A disease of malnutrition in which the (African) hair becomes a lighter, reddish color (Ghanaian term for ‘rejected one’). Lamellar: A layer, leaf or plate; used to describe scaling. Langer’s lines: Cleavage lines in skin determined by the disposition of connective tissue in reticular layer. Langerhans cells: Important specialized dopa-negative dendritic mesodermal cells found in keratinized epithelia. They are morphologically distinctive type of macrophages, which are characterized by a folded nucleus, and distinct intracytoplasmic organelles called Birbeck granules. Langerhans cells perform immunological function in the skin. Langerhans layer: Another name for granular cell layer of epidermis. Langhans cells: Multinucleated giant cells, as found typically in tuberculous granulomas. Laser- Light Amplification by Stimulated Emission of Radiation. Leiner’s disease (syndrome): A desquamative erythroderma of due to C-5 complement deficiency affecting young infants and accompanied by diarrhoea, weight loss and increased susceptibility to infections. Lentigo: A pigmented macule having an increased number of melanocytes at the dermo-epidermal junction. Leprosy (synonym Hansen’s disease): A chronic mycobacterial disease affecting the skin and peripheral nervous system Leser-Trelat sign: Rapid development of pruritic seborrheic keratoses associated with internal malignancy. Letterer- Siwe disease: An acute form of generalized histiocytosis of infancy, with seborrhoeic eczemalike eruption and purpura. Leucoderma: Lack of normal pigmentation of the skin.

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Essentials in Dermatology Leucoplakia: Persistent white patches on mucous membranes not attributable to a known disease process. Libman-Sacks endocarditis: A verrucous endocarditis that may be associated with systemic lupus erythematosus. Lichen: An old term now restricted to a relatively small number of papular eruptions, of which lichen planus is the most com-mon (Greek leichen a tree-moss, lichen or liverwort). Lisch nodules: Pigmented iris hamartomas occurring in neurofibromatosis. Livedo: A cyanotic discolouration of the skin of reticulate, patchy or mottled pattern due to venous congestion or arterial disease. Louse: An ectoparasitic insect belonging to the order Anoplura or Mallophaga; dermatologically, head or body louse (Pediculus) or pubic louse (Phthirus). Lucio’s phenomenon: A distinctive reactional state in the course of diffuse lepromatous leprosy “Lucio leprosy”, “Lepra bonita”, a beautiful leprosy. Lues: A disguised designation for syphilis but originally a term for plague. Lupus: A term applied to lesions having an eroded or gnawed quality. Now confined to form of cutaneous tuberculosis, especially lupus vulgaris, and lupus erythematosus where it is better fitted to describe the discoid form. Lyell’s syndrome: Another term for toxic epidermal necrolysis. Originally also included staphylococcal scalded skin syndrome. -lysis meaning loosening, separation or rupture. Macrocheilia: Abnormal enlargement of lips. Madura foot: Name for mycetoma, a tumid granulomatous fungal infection of the foot. Named after Madura now Madurai, a town in South India. Majocchi’s granuloma: A trichophytic granuloma of the dermis. Malassezia: Name previously given to the dimorphic form of the lipophilic yeast Pityrosporum orbiculare. Malpighian layer: The basal and prickle-cell layers of the epidermis, also called as stratum malpighi. Mantoux test: Intracutaneous tuberculin test for tuberculosis. Marfan’s syndrome: A heritable disorder of connective tissue characterized by abnormally long extremities, arachnodactyly and ocular and cardiovascular anomalies Marjolin’s ulcer : Squamous cell epithelioma arising in an irritated scar tissue. Martorell’s ulcer : Hypertensive ulcer of the leg. Mast cell: Mastocyte widely dispersed mesenchymal cells found in the bone marrow, dermis and other tissues, the cytoplasm of which contains numerous metachromatically staining granules. Mast cells liberate histamines and various mediators during IgE mediated hypersensitivity responses. Mees’ lines: Horizontal whitish striations observed on nails in acute arsenical poisoning but also seen with other systemic disease.

Terminology in Dermatology, Sexually Transmitted Diseases and Leprosy Melanocytes: Melanocyte is a dendritic cell, which structurally resembles the octopus. Each melanocyte is in contact with 36 keratinocyte, together they form the so-called epidermal melanin unit. The main function of melanocyte is to synthesize melanin (Eumelanin or Pheomelanin). Melasma: Hypermelanosis of the face, seen chiefly in women; chloasma. Melkersson- Rosenthal syndrome: Recurrent facial paralysis, facial oedema, granulomatous cheilitis and other associated features. Micro: Pref. meaning small. Microabscesses: Small accumulations of cells in the epidermis or the subepidermal papillae. Three types of microabscesses are (i) Munro microabscess, composed of disintegrated neutrophils in the parakeratotic layer in psoriasis, (ii) Pautrier microabscess, composed of mononuclear cells and mycosis cells in the stratum malpighi in mycosis fungoides, and (iii) Papillary tip microabscess, composed predominantly of neutrophils in dermatitis herpetiformis. Miliary: Similar in size to a millet seed. Milium: A tiny white cyst containing lamellated keratin. Mite: Any small insect, e.g., scabies mite. Mitsuda’s antigen: A suspension of heat-killed leprosy bacilli used in an intradermal test for leprosy (Mitsuda’s reaction). Mole: Strictly, a circumscribed pigmented lesion of the skin but often applied to any raised accumulation of melanocytic nevus cells irrespective of pigmentation. Molluscum: A soft cutaneous nodule. Montgomery’s tubercles: Apocrine glands in the areola of the breast enlarged during pregnancy. Morgan’s folds (synonym Dennie-Morgan folds): Single or double infraorbital folds in children thought to be indicative of the atopic state. Morphoea: A circumscribed form of scleroderma. Mosaic: Patterned arrangement of small pieces, closely set appearance resembling a mosaic. Mycetoma: A term given to tumid and inflammatory fungal infections of deep tissues usually of the foot, due to various species of fungus. Mycosis fungoides: A CTCL lymphoma that may present as fungating masses. Myiasis: Disease caused by dipterous (fly) larvae. Nevus: A term for a circumscribed developmental lesion of skin or mucous membrane involving excess or deficiency of any one of the normal structures; cutaneous hamartoma. Nail unit: It is yet another epidermal appendage. It consists of nail matrix just underneath the proximal nail fold which gives rise to nail plate – a keratinized structure. The rectangular nail plate rests on a nail bed and is bounded on two sides by lateral nail folds. The cuticle seals the space between nail folds and nail plate. Necrobiosis: Physiological or normal cell death in the midst of living tissue. Neoplasm: ‘New growth’, tumor. A benign, premalignant, or malignant lesion consisting of proliferating cells.

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Essentials in Dermatology Nigricans: Blackish; properly, becoming dark. Nikolsky’s sign: Intra-epidermal cell separation or blister formation evoked by firm sliding pressure on unaffected skin of patients with pemphigus (especially pemphigus foliaceus) and, sometimes, epidermal necrolysis. Nit: The egg of the louse; or the encased embryo. Norwegian scabies: A form of extensive crusted scabies described by Boeck and seen in elderly, immunocompromised or mentally defective patients. Nummular: Assuming shape of coin, discoid. Obliterans: Destroying, obliterating. Ochre: A pigment of clay and iron oxide, of great antiquity; hence, yellow-red in color. Ochronosis: Grayish or bluish discolouration of connective tissue and cartilage due to deposition of a pigment derived from oxidized homogentisic acid, occurs in ALKAPTONURIA and in chronic exposure to phenol and phenolic compounds. Ointment: Semi-solid preparation in suitable base for application to the skin. Commonly used term for greasy preparations as opposed to creams. Onychia: Inflammation of nail matrix. Onychogryphosis: Thickening and over curvature of the nail resembling a ram’s horn. Onychoschizia splitting of nail plate into layers Onychotillomania: Habit of fiddling with or pulling pieces off the nail, an obsessional or psychopathic trait. Ophiasis: Snake-like, curling; applied to a form of alopecia areata. Orf: Ecthyma infectiosum. Highly contagious disease of sheep and goats caused by a parapoxvirus. May infect man by contact. Osler’s nodes: Tender erythematous lesions occurring on pads of fingers and toes, or palms and soles of patients with subacute bacterial endocarditis. Oxyuriasis: Infestation by worm of genus enterobius; enterobiasis. Pachydermoperiostosis: Hypertrophic osteoarthropathy with thickened skin. Pachyonychia: Abnormally thick nails. Paget’s disease, extramammary: A malignant plaque occurring in anogenital area or axillae and containing Paget cells. Paget’s disease of the nipple; mammary Paget’s disease: A marginated scaly or crusted lesion of the nipple containing intra-epidermal paget cells and associated with intraductal carcinoma of the breast. Panniculus: A layer of fat, covering sheet or garment. Papilloma: A nipple-like mass projecting from the surface of the skin. Papillomatosis: Upward proliferation of subepidermal papillae. Parakeratosis: Means retention of nuclei in the horny layer associated with a marked underdevelopment or absence of the granular layer, seen in psoriasis and other disorders

Terminology in Dermatology, Sexually Transmitted Diseases and Leprosy Patch: A large macule, more than 2 cm in diameter; a term obsolete now. Pautrier’s abscess (microabscess): Focal collections of lymphocytes in the epidermis of patients with mycosis fungoides. Peau d’orange: A dimpling and induration of the skin, such as may overlie a mammary carcinoma (French, orange-skin). Pediculosis: A genus of parasitic lice infecting man and other primates, family Pediculidae Pelade: French term for alopecia areata. Pellagra: Sisease due to dietary deficiency of niacin and tryptophan (from Italian “pelle agra” rough skin). Pemphigus: A group of blistering diseases showing intra-epidermal bullae and acantholysis. Pemphigoid (‘bullous pemphigoid’): A blistering disease of the elderly with subepidermal bullae. Perleche: Angular cheilitis Petechia: A punctate haemorrhagic spot 1-2 mm in diameter. Peutz-Jeghers syndrome: Orificial and periorificial lentigines with hamartomatous intestinal polyps. Peyronie’s disease: Penile fibromatosis. Phagedaena: Rarely used term for a rapidly spreading or deepening ulcer. Phrynoderma: Dry skin with horny follicular papules, especially as seen in severe vitamin A or essential fatty acid deficiency (Greek phrynos toad, derma skin, from resemblance to toad skin). Phthiriasis: Infestation with any type of louse but usually confined to infestation with pubic lice. Phthirus (pubis): A genus of louse primarily affecting the pubic hair and occasionally other hairy areas. Piedra: A fungal infection of the hair forming superficial nodules on the shaft (Spanish piedra stone). Piezogenic: Name given to pedal papules formed of herniated fat, the result of weight bearing around the ankle. Pilosebaceous unit: It consists of a hair follicle containing hair and sebaceous glands opening into follicular canal of hair follicle. Plaque: An elevated area of skin 2 cm or more in diameter or lesion formed by coalescence of papules. Plummer-Vinson syndrome (synonym Patterson -Brown Kelly syndrome)- dysphagia, post-cricoid oesophageal webs, spoon-shaped fingernails and atrophic glossitis. Poikiloderma: Dappled or variegated pattern of pigmentation. Usually applied to condition combining this with telangiectasia and atrophy. Pompholyx: A general term for cheiro- (and podo-) pompholyx, used for an acute, often recurrent deeply seated vesicular or bullous eruption of palms and fingers of unknown cause (‘dysidrosis’). Porphyria: Name given to a group of inborn errors of metabolism (porphyrin synthesis). Prickle cell: An epidermal cell in the stratum spinosum, so named because of its spiny intercellular bridges. Prurigo: A term denoting a number of conditions characterized by intensely irritable papules with no obvious local cause.

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Essentials in Dermatology Pruritus: A sensation of itching. Pseudopelade: A morphological term for a pattern of follicular response to a variety of insults, often unknown, resulting in patches of complete loss of scalp hair. Psoriasis: A chronic, inflammatory skin disease consisting essentially of well-demarcated dull-red plaques, though with many variants. Mnemonic to remember features of PSORIASIS: Pink Papules/ Plaques/ Pinpoint bleeding (Auspitz’s sign)/ Physical injury (Koebner phenomenon)/ Pain Silver Scale/ Sharp margins Onycholysis/ Oil spots Rete Ridges with Regular elongation Itching Arthritis/ Abscess (Munro) Stratum corneum with nuclei, neutrophils Immunologic Stratum granulosum absent/ Stratum Spinosum thickening. Pterygium: Vascular lesions encroaching on the cornea; fusion of eponychium with proximal portion of nail (pterygium unguis); web of skin extending from mastoid to achromial process as in Turner’s syndrome (pterygium colli). Purpura: Focal haemorrhage into the skin. Pustule: A vesicle or bulla containing numerous neutrophils. Pyoderma: Any purulent skin condition. Queyrat, erythroplasia: An intra-epidermal carcinoma of male genital mucosa (glans and prepuce). Quinke’s oedema: Means angioedema. Rash: Term for any inflammatory skin eruption Raynaud’s disease: Primary idiopathic form of Raynaud’s phenomenon. Raynaud’s phenomenon: Paroxysmal pallor, numbness and coldness of the extremities, often followed by cyanosis, due to digital vasospasm. It occurs in a number of diseases, notably the collagen vascular diseases. Reiter’s syndrome: Non-specific urethritis, arthritis and iridocyclitis (uveitis). Patients may develop a rupioid psoriasiform skin lesions in soles (keratoderma blennorrhagica). Reticulate: Means lacy, webbed, net-like. Rhagades: Cracks; fissures, a term used in congenital syphilis sequelae. Rhinosporidiosis: A granulomatous nasal mycosis common in Sri Lanka and South India. Ringwom: Very old term for any ring-shaped eruption; more recently used for superficial fungal infections, dermatophytosis or tinea. Ritter’s disease: Another name for staphylococcal scalded skin syndrome. Rosacea: A condition characterized by erythema, telangiectasia and acneiform pustules commonly affecting the central part of the face, occasionally scalp or elsewhere. Also termed acne rosacea. Rowell’s syndrome: Lupus erythematosus with erythema multiforme-like lesions. Sabouraud’s medium (agar): A culture medium for fungi, containing peptone and dextrose. Samman’s syndrome (synonym yellow nail syndrome): Thick, curved, slow growing yellowish nails associated with lymphoedema.

Terminology in Dermatology, Sexually Transmitted Diseases and Leprosy Sarcoidosis: A multisystem disease of unknown cause, characterized by non-caseating granulomas. Sarcoptes: A genus of mites, the Sarcoptidac, ectoparasites of warm-blooded animals. Scabiesinfestation with Sarcoptes scabeii. Also called itch, mange, ‘seven-year itch’. Scale- a flat plate or flake of stratum corneum. Scar: Fibrous tissue replacing normal tissues destroyed by injury or disease. Scleroderma: A local or generalized hardening of the skin, usually referring to systemic sclerosis. Sclerosis: A localized or diffuse induration. Scratch: A linear incised superficial wound of the skin, as with the fingernails, thorn, etc. Scrofula: Primary tuberculosis of cervical glands, with or without ulceration of overlying skin. Scutularis: A saucer-shaped depressed lesion, especially as seen in adherent follicular lesions of favus. Sebaceous glands: Sebaceous glands are lipid secreting holocrine glands. Their maximum density is in seborrhoeic areas of the body, i.e. scalp, face, upper chest, etc. They get activated at puberty under the influence of androgen hormone. Seborrhoea: Abnormally copious secretion of sebum. Senear-Usher syndrome: Another name for pemphigus erythematosus. Sezary’s syndrome: A pruritic CTCL erythroderma associated with lymphadenopathy and the presence in the peripheral blood of more than 10% of atypical mononuclear cells (Sézary cells). Shagreen: A type of rough granular leather made from horses or asses skin. Shagreen patch seen over the back in tuberous sclerosis. Shingles: A term for herpes zoster Sister Joseph’s nodule: Umbilical metastasis from intra-abdominal neoplasm. Sjogren’s syndrome: A term for keratoconjunctivitis sicca and stoma often associated with rheumatoid arthritis, systemic lupus erythematosus or systemic sclerosis. When occurring without these associated diseases, known as SICCA SYNDROME. Spirochaete: Any member of the family Spirochaetaceae, which includes the pathogens Treponema, Borrelia and Leptospira. Spongiosis: A process in which intercellular oedema fluid accumulates between the squamous cells of the epidermis. It occurs classically in acute and subacute dermatitis. Sporotrichosis: A fungal infection of man and animals, caused by Sporothrix schenckii. SSSS- Staphylococcal Scalded Skin Syndrome. Stevens-Johnson syndrome: A severe form of erythema multiforme with mucosal involvement. Strophulus: An outmoded term previously used either for papular urticaria. Sturge-Weber syndrome: Unilateral facial port-wine naevus with ipsilateral encephalomeningeal angiomatosis. Subcutaneous fat (Hypodermis): The subcutaneous fat layer is constituted by adipocytes. Adipocytes synthesize so much fat in their cytoplasm that lipid displaces the nucleus to the periphery of the cell.

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Essentials in Dermatology Subcutaneous fat act as shock absorber, facilitate mobility of skin over structures that underlie and acts as an insulator for heat. Syndrome: A set of symptoms occurring together or the sum of signs in a morbid process. Syphilis: Infection with Treponema pallidum. Tabes (dorsalis): A form of neurosyphilis; locomotor ataxia. TEN- Toxic Epidermal Necrolysis. Thrush: Term for infection of the oral, pharyngeal and vaginal mucosae with Candida albicans. Tinea: A fungal infection of skin, hair or nails caused by one of the dermatophytes Microsporum, Trichophyton or Epidermophyton, except for its use in terms tinea amiantacea and tinea versicolor (Latin tinea gnawing worm, moth). Trachoma: Infection of conjunctiva and cornea by Chlamydia trachomatis. Trichotillomania- a compulsion to pluck or twist out hair (Greek thrix, trichos hair, tillein to pluck, mania compulsion). Ulcer: A well-defined area of loss of skin and subcutaneous tissue involving the whole thickness of the skin and the underlying tissues, caused by infection, trauma or necrosis. Urticaria: An eruption of itching wheals, of physical, systemic or (less commonly) contact origin; nettle-rash, hives. Vagabond’s disease: A term for infestation with pediculosis corporis (French vagabond, from Latin vagabundus one who strolls around, from vagare to wander). Varicella: Disease caused by varicella-zoster virus; chicken-pox. Vegetans: Exuberant; vigorous skin lesions. Vellus: The short downy hair that replaces lanugo before or soon after birth on all hair-bearing areas except the scalp (Latin vellus wool, down, fleece). Venereal: Relating to or associated with sexual intercourse (Latin venereus from Venus, Veneris goddess of love). Verruca: Wart, excrescence; now used to denote viral wart, particularly of foot. Vesicle: A small blister less than 5 mm in diameter. Vitiligo: A primary loss of pigmentation from skin and hair, of unknown cause (Latin vitiligo a skin disease possibly from vitium flaw, blemish). Vulgaris: Common, ordinary. Wart (synonym verruca): This term used for any horny excrescence on the skin but properly so for lesions induced by the papovavirus. Weal (wheal): A transient circumscribed oedematous swelling as seen in urticaria or dermographism. Whitfield’s ointment: 6% benzoic acid compounded with 3% salicylic acid as ointment. Whitlow: Acute paronychia or purulent infection (abscess) of distal finger pulp; felon. Also, specifically, herpetic or melanotic whitlow. Wickham’s striae: A characteristic pattern of white streaking seen on papules of lichen planus.

Terminology in Dermatology, Sexually Transmitted Diseases and Leprosy Wood’s light: A source of ultraviolet radiation from which most visible rays have been excluded by the use of a filter. This photoemission causes fluorescence in certain skin conditions, especially in some fungal infections (Tinea capitis, tinea versicolor) and porphyrins. Xanthelasma: Symmetrical yellow plaques of eyelids due to lipid deposition. Yeast: Term used generically for any of a group of saprophytic unicellular fungi e.g.,C. albicans. Zoster: A shortened term for herpes zoster.

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Answers

Answers

Set I (1-400) 1.B 11.B 21.A 31.A 41.B 51.A 61.B 71.A 81.B 91.A 101.C 111.A 121.C 131.A 141.D 151.D 161.D 171.C 181.B 191.B 201.C 211.D 221.C 231.B 241.C 251.A 261.D 271.C 281.D 291.A 301.B

2.A 12.C 22.D 32.A 42.D 52.B 62.A 72.C 82.C 92.D 102.A 112.A 122.C 132.C 142.D 152.C 162.C 172.A 182.B 192.C 202.C 212.A 222.A 232.D 242.B 252.C 262.D 272.D 282.B 292.D 302.C

3.A 13.B 23.B 33.A 43.A 53.A 63.B 73.B 83.D 93.D 103.B 113.C 123.D 133.A 143.B 153.B 163.A 173.A 183.C 193.A 203.D 213.D 223.A 233.B 243.D 253.B 263.C 273.D 283.A 293.D 303.A

4.C 14.D 24.A 34.C 44.B 54.A 64.B 74.C 84.A 94.A 104.A 114.A 124.C 134.D 144.A 154.B 164.C 174.D 184.C 194.C 204.D 214.C 224.A 234.D 244.A 254.B 264.A 274.D 284.D 294.B 304.C

5.A 15.C 25.A 35.C 45.B 55.B 65.A 75.B 85.D 95.D 105.C 115.D 125.A 135.C 145.A 155.D 165.A 175.B 185.A 195.A 205.B 215.B 225.D 235.A 245.B 255.B 265.D 275.D 285.B 295.A 305.C

6.B 16.A 26.A 36.C 46.B 56.A 66.B 76.C 86.A 96.D 106.D 116.C 126.D 136.B 146.D 156.B 166.B 176.D 186.D 196.C 206.D 216.A 226.D 236.B 246.A 256.A 266.C 276.A 286.A 296.A 306.A

7.A 17.B 27.C 37.A 47.B 57.B 67.A 77.B 87.D 97.C 107.C 117.A 127.A 137.A 147.B 157.D 167.A 177.C 187.B 197.A 207.C 217.C 227.C 237.B 247.B 257.D 267.D 277.C 287.A 297.D 307.A

8.C 18.D 28.B 38.C 48.A 58.B 68.D 78.A 88.A 98.A 108.A 118.D 128.A 138.A 148.B 158.D 168.D 178.A 188.A 198.D 208.B 218.C 228.B 238.C 248.B 258.D 268.A 278.C 288.A 298.C 308.D

9.A 19.D 29.C 39.A 49.A 59.B 69.B 79.C 89.B 99.C 109.A 119.A 129.B 139.D 149.D 159.C 169.A 179.C 189.C 199.B 209.D 219.D 229.B 239.B 249.C 259.B 269.B 279.D 289.D 299.C 309.C

10.B 20.A 30.D 40.B 50.A 60.C 70.A 80.A 90.B 100.A 110.D 120.D 130.C 140.A 150.A 160.C 170.D 180.B 190.D 200.A 210.A 220.A 230.D 240.A 250.D 260.B 270.B 280.C 290.A 300.D 310.D Contd...

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Essentials in Dermatology Contd... 311.A 321.A 331.A 341.A 351.C 361.D 371.B 381.A 391.A

312.B 322.D 332.A 342.C 352.A 362.A 372.C 382.B 392.A

313.A 323.C 333.C 343.A 353.D 363.C 373.B 383.A 393.A

314.A 324.A 334.D 344.A 354.D 364.A 374.D 384.A 394.A

315.B 325.C 335.D 345.D 355.A 365.C 375.D 385.A 395.A

316.D 326.A 336.A 346.A 356.A 366.B 376.A 386.A 396.B

317.A 327.D 337.D 347.A 357.A 367.B 377.B 387.B 397.C

318.B 328.A 338.C 348.A 358.C 368.A 378.B 388.A 398.C

319.A 329.A 339.C 349.D 359.B 369.D 379.A 389.A 399.D

320.D 330.B 340.C 350.A 360.C 370.A 380.A 390.D 400.A

7. C 15. C 23. B 31. B 39. A 47. D 55. C 63. A 71. B 79. D 87. B 95. D 103. C 111. B 119. A 127. A 135. B 143. A 151. D 159. C 167. C 175. B 183. B 191. B 199. A 207. D 215. A 223. C 231. C 239. D

8. D 16. A 24. A 32. A 40. B 48. B 56. B 64. D 72. B 80. B 88. B 96. A 104. A 112. C 120. B 128. A 136. D 144. C 152. C 160. B 168. B 176. C 184. B 192. C 200. B 208. B 216. D 224. B 232. B 240. D

Key to Set II (1-245) 1. B 9. D 17. D 25. C 33. B 41. B 49. A 57. D 65. A 73. B 81. C 89. A 97. D 105. D 113. C 121. D 129. C 137. A 145. D 153. A 161. D 169. C 177. C 185. A 193. D 201. B 209. B 217. C 225. D 233. D 241. B

2. B 10. C 18. A 26. D 34. C 42. A 50.C 58. A 66. A 74. A 82. A 90. A 98. D 106. D 114. A 122. A 130. A 138. D 146. B 154. C 162. C 170. A 178. A 186. A 194. A 202. A 210. C 218. B 226. B 234. B 242. D

3. C 11. A 19. C 27. B 35. A 43. D 51. B 59. B 67. B 75. C 83. B 91. C 99. B 107. C 115. C 123. B 131. B 139. D 147. A 155. A 163. A 171. D 179. B 187. A 195. A 203. A 211. A 219. C 227. C 235. C 243. A

4. D 12. D 20. A 28. D 36. C 44. D 52. D 60. C 68. D 76. C 84. D 92. B 100. A 108. B 116. B 124. D 132. D 140. A 148. D 156. D 164. D 172. A 180. D 188. C 196. C 204. D 212. D 220. A 228. D 236. C 244. B

5. D 13. B 21. B 29. D 37. A 45. C 53. A 61. C 69. A 77. A 85. D 93. B 101. C 109. A 117. C 125. D 133. B 141. A 149. A 157. B 165. A 173. A 181. D 189. B 197. A 205. B 213. B 221. B 229. D 237. B 245. B

6. A 14. A 22. B 30. A 38. C 46. A 54. D 62. B 70. C 78. D 86. A 94. A 102. B 110. D 118. B 126. B 134. A 142. B 150. D 158. C 166. C 174. A 182. A 190. D 198. A 206. A 214. C 222. C 230. A 238. A

Answers Key to MCQs in Dermatology- Basic Sciences (1-55) 1.B 5.A 9.B 13.A 17.D 21.A 25.C 29.D 33.D 37.C 41.D 45.D 49.A 53.C

2.C 6.A 10.B 14.B 18.D 22.C 26.D 30.D 34.D 38.D 42.B 46.B 50.A 54.A

3.B 7.C 11.D 15.B 19.C 23.A 27B 31.D 35.D 39.D 43.C 47.C 51.A 55.C

4.A 8.D 12.B 16.C 20.C 24.A 28.D 32.D 36.D 40.A 44.C 48.A 52.A

Key to MCQs in Clinical Dermatology-Part 1 (1-110) 1. A 9. A 17. A 25. D 33. D 41. B 49. B 57. A 65. C 73. B 81. A 89. C 97. D 105. C

2. C 10. C 18. C 26. C 34. C 42. B 50. A 58. D 66. D 74. C 82. B 90. A 98. A 106. B

3. A 11. C 19. C 27. A 35. C 43. D 51. C 59. B 67. A 75. D 83. B 91. C 99. C 107. B

4. C 12. D 20. C 28. C 36. A 44. C 52. B 60. B 68. A 76. D 84. B 92. B 100. D 108. A

5. C 13. C 21. D 29. A 37. B 45. A 53. C 61. A 69. B 77. D 85. B 93. B 101. D 109. C

6. C 14. A 22. B 30. B 38. A 46. A 54. B 62. D 70. D 78. B 86. A 94. D 102. D 110. C

7. B 15. A 23. A 31. A 39. A 47. C 55. B 63. D 71. C 79. B 87. B 95. C 103. B

8. B 16. A 24. C 32. D 40. B 48. C 56. C 64. D 72. A 80. B 88. D 96. D 104. B

7. A 15. B 23. A 31. D 39. D 47. A 55. A 63. A 71. A 79. C 87. A 95. A

8. A 16. B 24. A 32. A 40. C 48. D 56. A 64. A 72. A 80. B 88. A 96. C

Key to MCQs in Clinical Dermatology- Part II (1-99) 1. B 9. B 17. D 25. D 33. A 41. A 49. A 57. B 65. B 73. B 81. A 89. C 97. C

2. C 10. B 18. B 26. A 34. D 42. A 50. C 58. A 66. D 74. A 82. B 90. A 98. B

3. A 11. A 19. A 27. D 35. D 43. C 51. B 59. A 67. A 75. A 83. A 91. B 99. D

4. A 12. D 20. A 28. A 36. A 44. A 52. A 60. A 68. A 76. A 84. C 92. A

5. D 13. A 21. A 29. B 37. A 45. A 53. C 61. A 69. C 77. A 85. B 93. A

6. D 14. A 22. A 30. A 38. B 46. A 54. B 62. A 70. D 78. A 86. B 94. A

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Essentials in Dermatology Key to MCQs in Sexually Transmitted Diseases (1-130) 1. B 9. A 17. C 25. B 33. D 41. C 49. A 57. A 65. B 73. C 81. C 89. A 97. A 105. D 113. C 121. C 129. C

2. D 10. A 18. A 26. B 34. D 42. D 50. D 58. C 66. A 74. A 82. A 90. C 98. A 106. B 114. A 122. D 130. D

3. D 11. D 19. B 27. C 35. B 43. C 51. A 59. D 67. B 75. B 83. D 91. A 99. B 107. D 115. A 123. A

4. D 12. B 20. D 28. A 36. A 44. C 52. D 60. B 68. A 76. A 84. B 92. C 100. C 108. C 116. A 124. B

5. C 13. A 21. B 29. A 37. D 45. B 53. C 61. C 69. B 77. D 85. C 93. B 101. D 109. D 117. C 125. A

6. D 14. D 22. B 30. C 38. B 46. D 54. A 62. A 70. B 78. D 86. D 94. B 102. A 110. B 118. D 126. A

7. D 15. B 23. A 31. B 39. D 47. D 55. D 63. A 71. A 79. D 87. C 95. C 103. D 111. B 119. B 127. B

8. C 16. C 24. C 32. D 40. C 48. D 56. C 64. D 72. D 80. D 88. B 96. C 104. C 112. D 120. C 128. A

6. A 14. C 22. A 30. D 38. A 46. A 54. A 62. A 70. B 78. C 86. B 94. D

7. D 15. C 23. B 31. B 39. D 47. A 55. D 63. B 71. C 79. B 87. B 95. A

8. C 16. D 24. B 32. B 40. C 48. D 56. A 64. D 72. C 80. C 88. B

Key to MCQs in Leprosy (1-95) 1. B 9. A 17. A 25. C 33. D 41. C 49. A 57. D 65. D 73. B 81. C 89. A

2. D 10. B 18. C 26. B 34. A 42. A 50. D 58. B 66. B 74. A 82. B 90. D

3. A 11. B 19. C 27. A 35. A 43. C 51. C 59. D 67. C 75. B 83. A 91. A

4. A 12. B 20. D 28. C 36. D 44. A 52. D 60. A 68. A 76. C 84. A 92. A

5. D 13. A 21. B 29. D 37. A 45. D 53. A 61. B 69. A 77. C 85. A 93. C

DM Thappa - Essentials in Dermatology, 2nd Edition

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