Pediatric Dermatology MCQs

Which treatment choice would be contraindicated in a one-year old child who presents with monomorphous, nonpruritic flat-topped papules on the face, buttocks, extremities, palms and soles? 1

Advil

2

Acetaminophen

3

Hydration

4

Corticosteroids

5

Observation Q/Q(M)-476495 Report a Problem

Which treatment choice would be contraindicated in a one-year old child who presents with monomorphous, nonpruritic flat-topped papules on the face, buttocks, extremities, palms and soles? 4

Corticosteroids

Gianotti-Crosti or papular acrodermatitis of childhood is ass ociated with a variety of viral infections. infecti ons. Patients have a typical cutaneous manifestation, low-grade fever, mild lymphadenopathy and diarrhea. Corticosteroids should be avoided as they may have an adverse effect. Q/Q(M)-476495 Report a Problem Most common malignancy associated with multiple lesions similar t o the attached image is: 1

Acute myelogenous leukemia

2

Chronic myelogenous leukemia

3

Acute lymphocytic leukemia

4

Chronic lymphocytic leukemia

5

Melanoma Q/Q(M)-481905 Report a Problem

Most common malignancy associated with multiple lesions similar t o the attached image is: 2

Chronic myelogenous leukemia

The image shown is juvenile xanthogranuloma. The most commonly associated malignancy is chronic myelogenous leukemia. Other associations include Neurofibromatosis type 1 and aquagenic pruritus. Reference: Zvulunov A, Barak Y, Metzker A. Juvenile xanthogranuloma, neurofibromatosis, and juvenile chronic myelogenous leukemia. World statistical analysis. Arch Dermatol 1995;131:904-908. Q/Q(M)-481905 Report a Problem A newborn has a nodule over his lumbar spine. Skin biopsy reveals a lipoma. The next appropriate step is: 1

Observation 1

2

Excision of the lesion

3

Genetic testing

4

Imaging study

5

Malignancy work up Q/Q(M)-476957 Report a Problem

A newborn has a nodule over his lumbar spine. Skin biopsy reveals a lipoma. The next appropriate step is: 4

Imaging study

The skin can provide an important clue to the presence of an underlying neural tube defect, such as meningomyelocele and encephalocele. Cutaneous lesions along the midline of the spine should always prompt consideration of this possibility. Although, Although, midline neural tube defects are uncommon, early recognition and diagnosis of a spinal dysraphism can have important implications for earl y surgical correction and minimizing loss of neurologic function. Clues to the diagnosis include a midline dimple, tuft of hair, lipoma, or vascular lesion. In these instanc es, imaging studies (MRI, CT, ultrasound) should be promptly initiated. Q/Q(M)-476957 Report a Problem A patient presents with multiple juvenile xanthogranulomas, axillary freckli ng, multiple caf �-au-lait macules, three neurofibromas and a family history of NF-1. What other condition is this patient at increased risk for? 1

AML

2

CML

3

CLL

4

Medulloblastoma

5

Pancreatic carcinoma Q/Q(M)-480185 Report a Problem

A patient presents with multiple juvenile xanthogranulomas, axillary freckling, multiple caf �-au-lait macules, three neurofibromas and a family history of NF-1. What other condition is this patient at increased risk for? 2

CML

A patient with Neurofibromatosis type I and JXG �s present is at increased risk for CML. Medulloblastomas are increased in patients with Basal Cell Nevus syndrome. Pancreatic carcinoma occurs at an increased risk in patients with mutations in CDKN2A in the familial melanoma syndrome. Q/Q(M)-480185 Report a Problem Schimmelpenning-Feuerstein-Mims syndrome may be associated with which of the following: 1

Osteopokilosis

2

Polyostotic fibrous dysplasia

3

Osteopathia striata

4

Chondrodysplasia punctata

5

Hypophosphatemic rickets Q/Q(M)-476261 Report a Problem 2

Schimmelpenning-Feuerstein-Mims syndrome may be associated with which of the following: 5

Hypophosphatemic rickets

Epidermal nevus syndrome, also known as Schimmelpenning-Feuerstein-Mims syndrome, may be associated with hypophospatemic, vitamin D resistant rickets. Q/Q(M)-476261 Report a Problem The differential diagnosis of zinc deficiency is least likely to include: 1

Granuloma gluteale infantum

2

Biotin deficiency

3

Multiple carboxylase deficiency

4

Cystic fibrosis

5

Holocarboxylase synthetase deficiency Q/Q(M)-473684 Report a Problem

The differential diagnosis of zinc deficiency is least likely to include: 1


All of the options result in an eczematous acrodermatitis enteropathica-like eruption except granuloma gluteale infantum. As the name suggests, the lesions lesi ons of granuloma gluteale infantum are granulomatous. Q/Q(M)-473684 Report a Problem Multiple cylindromas are associated with: 1

Myotonic dystrophy

2

Cowden syndrome

3

Carney complex

4

Trichoepitheliomas

5

Pilomatrichomas Q/Q(M)-473703 Report a Problem

Multiple cylindromas are associated with: 4

Trichoepitheliomas

The Brooke-Spigler syndrome is defined by the presence of multiple trichoepitheliomas and c ylindromas. Q/Q(M)-473703 Report a Problem A full term neonate is noted to have small pustules with no underlying erythema present at delivery. The pustules are easily removed with clearing of the vernix and a collarette appears. A gram stain is done showing predominately neutrophils without bacteria. What is the most li kely diagnosis? 1

Miliaria

2

Erythema toxicum neonatorum

3

Transient neonatal pustular melanosis

4

Congenital candidiasis

5

Urticaria pigmentosa 3

Q/Q(M)-473678 Report a Problem A full term neonate is noted to have small pustules with no underlying erythema present at delivery. The pustules are easily removed with clearing of the vernix and a collarette appears. A gram stain is done showing predominately neutrophils without bacteria. What is the most likely diagnosis? 3


Transient neonatal pustular melanosis typically begins with sterile pustules that leave a characteristic collarette when ruptured. The lesions heal with hyperpigmented macules. Q/Q(M)-473678 Report a Problem Which of the following may be associated? 1

Paronychia

2

Cleft palate

3

AVM

4

Seizure disorder

5

Atrial septal defect Q/Q(M)-474924 Report a Problem

Which of the following may be associated? 4

Seizure disorder

Nevus sebaceus can very rarely be associated with multiple anomalies. Schimmelpenning syndrome syndrome can include seizure disorder, mental retardation, coloboma, as well as skeletal, cardiac and genitourinary abnormalities. Q/Q(M)-474924 Report a Problem What syndrome can accessory tragi be associated with? 1

Goldenhar syndrome

2

Turner syndrome

3

Neurofibromatosis

4

Ichthyosis

5

Birt Hogg Dube Q/Q(M)-482064 Report a Problem

4

What syndrome can accessory tragi be associated with? 1

Goldenhar syndrome

Accessory tragus is the most common congenital defect of the external ear and linked to maldevelopment of the first branchial arch. It can be associated with several s yndromes including Goldenhar syndrome, in which epibulbar dermoid and vertebral defects are also common. An access ory tragus usually it appears as a small skin-colored tag or nodule arising near the tragus; it is composed of normal epidermis with dermal adipose tissue, pilosebaceous units, eccrine glands, elastic fibers, and cartilage. Q/Q(M)-482064 Report a Problem Which of the following is a potential complication of subcutaneous fat necrosis of the newborn? 1

Cholestrol clefts in fat cells

2

Hypercalcemia

3

Acute renal failure

4

Hepatitis

5

Elevated uric acid levels Q/Q(M)-475869 Report a Problem

Which of the following is a potential complication of subcutaneous fat necrosis of the newborn? 2

Hypercalcemia

Subcutaneous fat necrosis of the newborn is a rare condition characterized by firm, reddish or purple nodules which appear on the arms, thighs, buttocks, back and cheeks. This condition is believed to result from cold injury. Crystallization occurs within the lipocytes, and this can be seen on histopathology with an associated granulomatous reaction. This is thought to occur due to the higher melting point of neonatal fat, which contains more saturated fatty acids. Onset of this condition occurs within the first two weeks of life and usually resolved over a period of weeks to months. Occasionally, lesions heal with atrophy. Hypercalcemia is an infrequent complication of subcutaneous fat necrosis of the newborn. Associated symptoms can include irritability, weight loss, vomiting and failure to thrive. Repeated serum calcium tests are advised until one month after all the cutaneous lesions have resolved. Q/Q(M)-475869 Report a Problem 5

What is the most likely diagnosis is this 16 year old patient who developed generalized eruption 2 weeks after onset of sore throat due to strep infection? 1

Psoriasis

2

Pityriasis rosea

3

Cutaneous T-cell lymphoma

4

Contact dermatitis

5

Lichen planus Q/Q(M)-476801 Report a Problem

What is the most likely diagnosis is this 16 year old patient who developed generalized eruption 2 weeks after onset of sore throat due to strep infection? 1

Psoriasis

Although the exact mechanism is not known, there is a well-known correlat ion between a history of upper respiratory infection secondary to group A beta-hemolytic streptococci and the s ubsequent development of guttate psoriasis. The eruption may resolve spontaneously or with treatment ( topical steroids or ultraviolet therapy). Some portion of these patients eventually develop chronic, plaque-type psoriasis. Q/Q(M)-476801 Report a Problem What systemic abnormalities are associated with neonatal lupus? 1

Thrombocytopenia, transaminitis

2

Thrombocythemia, transaminitis

3

Anemia, hypocomplementemia

4

Thrombocytopenia, hypocomplementemia

5

Transaminitis, hypocomplementemia Q/Q(M)-482854 Report a Problem

What systemic abnormalities are associated with neonatal lupus? 1

Thrombocytopenia, transaminitis

Thrombocytopenia and transaminitis are associated with neonatal lupus and may indicate a worse prognosis. 6

Q/Q(M)-482854 Report a Problem What is the best therapeutic option? 1

Oral cephalexin

2

Topical immune modulator

3

Oral acyclovir

4

Topical mupirocin

5

Oral prednisone Q/Q(M)-474923 Report a Problem

What is the best therapeutic option? 3

Oral acyclovir

The condition shown is eczema herpeticum. The most appropriate treatment is oral acyclovir. Q/Q(M)-474923 Report a Problem A full term newborn develops erythematous, indurated plaques on the upper back. Which of the following tests should be performed? 1

Calcium

2

Complete blood count

3

Blood culture

4

Thyroid function test

5

Alkaline phosphatase Q/Q(M)-473674 Report a Problem

A full term newborn develops erythematous, indurated plaques on the upper back. Which of the following tests should be performed? 1

Calcium

The most likely diagnosis is subcutaneous fat necrosis of the newborn. Hypercalcemia may result and serum calcium levels should be monitored up to 4 weeks after resolution of the skin. Q/Q(M)-473674 Report a Problem The causative agent of Roseola is: 1

A ssDNA virus 7

2

A dsDNA virus

3

A ssRNA virus

4

A dsRNA virus

5

Streptococcus Q/Q(M)-473695 Report a Problem

The causative agent of Roseola is: 2

A dsDNA virus

Roseola is caused by Human Herpesvirus 6, a double stranded DNA virus. Q/Q(M)-473695 Report a Problem GLUT-1 stains placental tissue. What other tissue t issue shows positive GLUT-1 staining? 1

capillary vascular malformation

2

salmon patch

3

glomus tumor

4

infantile hemangioma

5

angel's kiss Q/Q(M)-482561 Report a Problem

GLUT-1 stains placental tissue. What other tissue t issue shows positive GLUT-1 staining? 4

infantile hemangioma

The correct answer is infantile hemangioma, which reliably displays GLUT-1 positivity. GLUT-1 stains infantile hemangioma with high sensitivity and specificity. Glomus tumor, capillary vascular malformation (also called port-wine stain), salmon patch, and angel's kiss are other vascular lesions that do not stain with GLUT-1. Q/Q(M)-482561 Report a Problem Which of the following is not true about Gianotti-Crosti? 1

It is associated with viral infections like enterocirus, EBV, and CMV

2

It typically affects children between the age of 3 months and 15 years

3

It is characterized by monomorphic symmetric flat topped papules in acral areas

4

It may have associated fevers, lymphadenopathy, and diarrhea

5

Corticosteroids should be given to alleviate the pruritus of the lesions Q/Q(M)-482241 Report a Problem

Which of the following is not true about Gianotti-Crosti? 5

Corticosteroids should be given to alleviate the pruritus of the lesions

Gianotti-Crosti, or papular acrodermatitis of childhood, is typically nonpruritic and corticosteroids should be avoided as they may have adverse effect. All the other statements are true. The condition typically resolves after 2-3 weeks. Q/Q(M)-482241 Report a Problem

8

Which of the following clinical signs is most likely associated? 1

Clitoral hypertrophy

2

Neurofibromas

3

Axillary freckling

4

Hypertrichosis

5

Alopecia Q/Q(M)-476798 Report a Problem

Which of the following clinical signs is most likely associated? 1

Clitoral hypertrophy

The �coast of Maine� caf � au lait macule is associated with McCune-Albright syndrome. Endocrine abnormalities, including precocious puberty and polyostotic fibrous dysplasia are associated feat ures. The genetic mutation which results in this genodermatosis is due to a post-somatic mutaion in the alpha subunit of stimulatory G-protein. Q/Q(M)-476798 Report a Problem The most likely etiology of Jacquet �s diaper dermatitis is: 1

Candida

2

Trichophyton rubrum

3

Group A beta-hemolytic streptococcus

4

Herpes simplex virus, Type 2

5

Multifactorial Q/Q(M)-473682 Report a Problem

The most likely etiology of Jacquet �s diaper dermatitis is: 5

Multifactorial

Jacquet�s diaper dermatitis is a multifactorial process. Yeast, irritants and moisture all contribute to the occurrence of this eruption. Q/Q(M)-473682 Report a Problem A newborn presents with a well-defined, shiny patch with complete alopecia on the vertex of the scalp along the suture lines. Which of the following is the first step in diagnosis? 9

1

MRI

2

Calcium

3

Skin biopsy

4

Skull x-ray

5

Fungal culture

Q/Q(M)-473675 Report a Problem A newborn presents with a well-defined, shiny patch with complete alopecia on the vertex of the scalp along the suture lines. Which of the following is the first step in diagnosis? 4

Skull x-ray

The newborn most likely has aplasia cutis congenita. A skull x-ray would be the simplest, most cost effective means of identifying any underlying bony abnormality. Q/Q(M)-473675 Report a Problem A child presents with high fever, strawberry tongue, lymphadenopathy l ymphadenopathy,, and a polymorphous exanthem. Approximately how many of these patients will have coronary artery aneurysms if left untreated? 1

Two percent

2

Five percent

3

Ten percent

4

Twenty-five percent

5

Sixty percent Q/Q(M)-482264 Report a Problem

A child presents with high fever, strawberry tongue, lymphadenopathy l ymphadenopathy,, and a polymorphous exanthem. Approximately how many of these patients will have coronary artery aneurysms if left untreated? 4

Twenty-five percent

This child has Kawasaki's disease, or mucocutaneous lymph node syndrome. Other features include conjunctival injection and extremity erythema and edema. Twenty-five percent of untreated patients go on to develop coronary artery aneurysms. Treatment is with IVIG and aspirin. Q/Q(M)-482264 Report a Problem A 10 year old girl presents with desquamation of the fingertips. Which exam should be ordered? 1

Renal ultrasound

2

ASO titer

3

EEG

4

Eye exam

5

Chest x-ray Q/Q(M)-482471 Report a Problem

A 10 year old girl presents with desquamation of the fingertips. Which exam should be ordered? 10

2

ASO titer

Desquamation of the fingertips is commonly associated with infections with group A beta-hemolytic Strep and Staph aureus, including scarlet fever, perianal Strep, Staph scalded skin s yndrome, and toxic shock syndrome. Thus ASO titer would be indicated in this case. Fingertip desquamation is also a manifestation of Kawasaki disease. Q/Q(M)-482471 Report a Problem Which of the following is not a major criterion for Kawasaki �s disease: 1

Fever >5 days

2

Palmoplantar erythema > desquamation

3

Cardiac aneurysm

4

Strawberry tongue/ red lips

5

Cervical adenopathy Q/Q(M)-473697 Report a Problem

Which of the following is not a major criterion for Kawasaki �s disease: 3

Cardiac aneurysm

Cardiac aneurysm is a serious complication of Kawasaki �s disease. However, as the cardiovascular manifestations generally present 1 �5 months after presentation, they are not criteria for diagnosis. Q/Q(M)-473697 Report a Problem The most common age group for papular-purpuric gloves and socks syndrome is: 1

Newborns

2

Toddlers

3

6-10 year olds

4

Adolescents

5

Elderly Q/Q(M)-473696 Report a Problem

The most common age group for papular-purpuric gloves and socks syndrome is: 4

Adolescents

This unique presentation of parvovirus infection typically occurs in adolescents and young adults. Q/Q(M)-473696 Report a Problem What is the most common tumor associated with this condition? 1

Trichoblastoma

2

Syringocystadenoma papilliferum

3

Sebaceous carcinoma

4

Basal cell carcinoma

5

Trichoadenoma 11

Q/Q(M)-476259 Report a Problem

What is the most common tumor associated with this condition? 1

Trichoblastoma

Recent reviews have identified trichoblastoma as the most common tumor arising within nevus sebaceous. Q/Q(M)-476259 Report a Problem An infant presents with yellowish-brown, crusted papules with petechiae in a seborrheic distribution. Which of the following statements about this entity is likely to be true? 1

This presentation is consistent with Jacquet's dermatitis

2

CD1+, S100+ cells with comma-shaped nuclei should be seen on biopsy

3

This presentation occurs when the infant is weaned off of breast milk

4

An autosomal recessive defect in holocarboxylase synthetase is the cause

5

Maternal-fetal transmission most likely occured in the peripartum period Q/Q(M)-477791 Report a Problem

An infant presents with yellowish-brown, crusted papules with petechiae in a seborrheic distribution. Which of the following statements about this entity is likely to be true? 2

CD1+, S100+ cells with comma-shaped nuclei should be seen on biopsy

This infant has Langerhans Cell Histiocytosis (Letterer-Siwe disease). Multisystem involvement may be present. Jacquet's Erosive Dermatitis presents with severe erosive papules in the diaper region, and is multifactorial in etiology (yeast, irritant dermatitis, moisture). Acrodermatitis enteropathica (zinc deficiency) presents with brown, orange crusted plaques with vesicles and bullae, especially in perineal and perioral areas and distal extremities. The inherited form occurs when the infant is weaned off of breast milk. Biotin deficiency presents similarly; the neonatal form is attributed to an AR defect in holocarboxylase synthetase. Q/Q(M)-477791 Report a Problem An infant presents with the lesion depicted in the photo. What information do you provide the parents regarding this condition? 1

The lesion will go through rapid growth followed by stabilization and regression 12

2

The lesion will respond to laser therapy

3

The lesion will persist and may grow further

4

The lesion will resolve with antifungal therapy

5

The lesion is associated with a XO karyotype Q/Q(M)-474920 Report a Problem

An infant presents with the lesion depicted in the photo. What information do you provide the parents regarding this condition? 3

The lesion will persist and may grow further

The lesion represented in the photo is an arteriovenous vascular malformation. These lesions do not typically regress. Q/Q(M)-474920 Report a Problem Which finding is associated with Pachydermoperiostosis: 1

Cutis verticis gyrata

2

Palmoplantar hyperkeratosis

3

Mucosal keratoses

4

Osteopathia striata

5

Cutis marmorata Q/Q(M)-476266 Report a Problem

Which finding is associated with Pachydermoperiostosis: 1

Cutis verticis gyrata

The scalp change seen in pachydermoperiostosis is cutis verticis gyrata. Q/Q(M)-476266 Report a Problem Which presentation of psoriasis is more common in children: 1

Pustular psoriasis

2

Acrodermatitis continua of Hallopeau

3

Keratoderma blennorragica

4

Erythrodermic psoriasis

5

Guttate psoriasis Q/Q(M)-476267 Report a Problem 13

Which presentation of psoriasis is more common in children: 5

Guttate psoriasis

The majority of cases of guttate psoriasis occur in persons under the age of 30. Q/Q(M)-476267 Report a Problem What deficiency is responsible for this condition? 1

Thiamine

2

Niacin

3

Zinc

4

Vitamin C

5

Vitamin A Q/Q(M)-476670 Report a Problem

What deficiency is responsible for this condition? 3

Zinc

Acrodermatitis enteropathica is a rare inherited disorder transmitted in an autosomal recessive fashion. The disorder is caused by an inability to absorb zinc. the clinical syndrome is characterized by acral dermatitis, alopecia, and diarrhea. Zinc deficiency from other causes (including chronic wasting, poor oral intake, lack of supplementation in total parenteral nutrition) can cause similar clinical changes. Q/Q(M)-476670 Report a Problem All four subtypes of Phakomatosis Pigmentovascularis have which feature in common: 1

Nevus spilus

2

Epidermal nevi

3

Nevus flammeus

4

Nevus anemicus

5

Dermal Melanocytosis. Q/Q(M)-478707 Report a Problem

All four subtypes of Phakomatosis Pigmentovascularis have which feature in common: 3

Nevus flammeus

Phakomatosis Pigmentovascularis: Patients with a combination of vascular malformations and melanocytic or epidermal nevi are grouped into 4 subtypes of this disorder. All have nevus flammeus/capill ary malformation (CM). Type I: CM + epidermal nevus Type II: CM + der mal melanocytosis +/- nevus anemicus Type III: CM + nevus spilus +/- nevus anemicus Type IV: CM + dermal melanocytosis + nevus spilus +/- nevus anemicus. Q/Q(M)-478707 Report a Problem 14

What is the most likely diagnosis? 1

Psoriasis

2

Pityriasis rosea

3

Cutaneous T-cell lymphoma

4

Contact dermatitis

5



Contact dermatitis

This periumbilical eruption is classic for a contact nickel allergy. The metal snaps on pants are the cause in this case. Q/Q(M)-476441 Report a Problem Which of the following is the most common long term sequelae from congenital rubella syndrome? 1

Macrocephaly

2

Saber shins

3

Deafness

4

Nystagmus

5

Microcephaly Q/Q(M)-473687 Report a Problem

Which of the following is the most common long term sequelae from congenital rubella syndrome? 3

Deafness

Deafness may occur in up to 50% of infants with congenital rubella syndrome. Q/Q(M)-473687 Report a Problem Which of the following is a ssDNA virus: 1

Herpesvirus

2

Parvovirus

3

Picornovirus 15

4

Adenovirus

5

Parapox Q/Q(M)-473688 Report a Problem

Which of the following is a ssDNA virus: 2

Parvovirus

Parvovirus is the only ssDNA virus listed. Q/Q(M)-473688 Report a Problem A newborn infant presents with ring of long, dark, coarse hair surrounding a midline scalp patch of alopecia. What is associated with this finding? 1

Alopecia areata

2

Ectopic brain tissue

3

Thyroid disease

4

Nevus sebaceus of Jadassohn

5

Deafness Q/Q(M)-476777 Report a Problem

A newborn infant presents with ring of long, dark, coarse hair surrounding a midline scalp patch of alopecia. What is associated with this finding? 2

Ectopic brain tissue

The "hair collar sign" is associated associate d with ectopic brain tissue and is thought to arise from a congenital herniation through the skull. Caution must be used in evaluation as biopsy or needle aspiration may lead to retrograde infection. Q/Q(M)-476777 Report a Problem The association of Port-wine stains on a limb with soft tissue swelling with or without bony over growth is: 1

Bannayan-Riley-Ruvalcaba syndrome

2

Goldenhar's syndrome

3

Sturge-Weber syndrome

4

Klippel-Trenaunay syndrome

5

Proteous syndrome Q/Q(M)-478735 Report a Problem

The association of Port-wine stains on a limb with soft tissue swelling with or without bony over growth is: 4

Klippel-Trenaunay syndrome

The association of port-wine stain on a limb with soft tissue swelling with or without bony overgrowth is Klippel-Trenuanay syndrome. Klippel-Trenaunay syndrome is characterized by the Triad of port-wine malformations in association with deep venous system malformations, superficial varicosities, and bony and soft tissue hypertrophy. Sturge-Weber syndrome has 2 essential components: Facial port-wine stain and homolateral leptomeningeal angiiomattosis. The port wine stain most commonly involves the areas innervated by the ophthalmic(V1) and maxillary (V2) divisions of the trigeminal nerve. Complications of leptomeningeal angiomatosis are epilepsy, mental retardation, and occasionally, contralateral hemiplegia. Proteus Syndrome is characterized by vascular malformations including nevus flammeus, 16

hemihypertrophy, macrodactyly, verrucous epidermal nevus, soft-tissue subcutaneous masses, and cerebriform overgrowth of the plantar surface. Bannayan Riley Ruvalcaba syndrome may include mul tiple cutaneous and visceral venous, capillary, and lympathtic malformations, macroephaly, pseudopapilledema, systemic lipoangiomatosis, spotted pigmentation of the penis, hamartomatous intestinal polyps, and rarely trichilemmonmas. (multiple subcutaneous lipomas as well as acanthosis nigricans). Q/Q(M)-478735 Report a Problem What is the most common cause of neonatal purpura fulminans? 1

Strep

2

Staph

3

Varicella

4

Protein C deficiency

5

Factor V Leiden deficiency Q/Q(M)-482247 Report a Problem

What is the most common cause of neonatal purpura fulminans? 4

Protein C deficiency

In children, purpura fulminans may have several causes. It is a highly characteristic feature fe ature of meningococcal septicemia and may occur as a sequel se quel to a number of infections, including streptococcal infections, varicella, and measles. In the neonate, however, its occurrence is highly suggestive of homozygous protein C deficiency. Q/Q(M)-482247 Report a Problem Pachyonychia congenita type 2 is most commonly associated with which of the following: 1

Increased risk of malignancy

2

Poikiloderma

3

Natal teeth

4

Aplastic nails

5

Deafness Q/Q(M)-476264 Report a Problem

Pachyonychia congenita type 2 is most commonly associated with which of the following: 3

Natal teeth

Pachyonychia congenita type 2 may be associated with natal teeth and steatocystoma. Pachyonychia congenita type 1 is associated with benign leukoplakia. Q/Q(M)-476264 Report a Problem In a child with zinc deficiency, yet normal or near normal zinc levels, which test could be a valuable adjunctive test? 1

Magnesium

2

Niacin

3

Manganese

4

Alkaline phosphatase 17

5

Iron Q/Q(M)-477753 Report a Problem

In a child with zinc deficiency, yet normal or near normal zinc levels, which test tes t could be a valuable adjunctive test? 4

Alkaline phosphatase

The diagnosis of zinc deficiency should be consideredi in at-risk individuals with acral or periorificial dermatitis. Chronic diaper dermatitis in an infant s hould lead to the evaluation for zinc deficienc y. A low serum zinc level can usually confirm the diagnosis. If normal or near normal a low serum alkaline phosphatase, a zinc-dependent enzyme, may be a valuable valuable adjunctive test. Q/Q(M)-477753 Report a Problem Menkes kinky hair syndrome is associated most commonly with: 1

Trichorrhexsis invaginata

2

Trichostasis spinulosa

3

Pili multigemini

4

Pili torti

5

Plica neuropathica Q/Q(M)-473701 Report a Problem

Menkes kinky hair syndrome is associated most commonly with: 4

Pili torti

The X-linked recessive Menkes kinky hair syndrome is associated with multiple hair shaft abnormalities, most characteristically, pili torti. Q/Q(M)-473701 Report a Problem What is the most likely diagnosis: 1

Epidermolysis bullosa simplex

2

Epidermolysis bullosa dystrophica

3

Pemphigus vulgaris

4

Linear bullous IgA disease

5

Facticial dermatosis Q/Q(M)-474922 Report a Problem

18

What is the most likely diagnosis: 1


The bullous lesions represented are nonscarring and are associated with obvious nail dystrophy. Epidermolysis bullosa simplex, Dowling-Meara type, is the best option. Q/Q(M)-474922 Report a Problem 816 activating mutation in c-kit are found most often in which subset of patients with mastocytosis? 1

Adults with systemic disease refractory to imantinib

2

Adults with systemic disease associated with eosinophilia

3

Patients with familial history of mastocytosis

4

Adults with Telangiectasia Macularis Eruptiva Perstans

5

As a mosaic mutation in children with solitary cutaneous mastocytoma Q/Q(M)-482220 Report a Problem

816 activating mutation in c-kit are found most often in which subset of patients with mastocytosis? 1

Adults with systemic disease refractory to imantinib

Almost all sporadic adult onset mastocytosis patients demonstrate mutations in c-kit. Most of these are activating mutations linked to the 816 codon. Unfortunately, patients with this specific mutation tend to not respond to systemic therapy with oral imantinib, a tyrosine kinase inhibitor. Q/Q(M)-482220 Report a Problem Which of the following is most likely associated? 1

No additional abnormality

2

Deafness

3

Coarctation of the aorta

4

Bifid rib

5

Alopecia Q/Q(M)-476251 Report a Problem

19

Which of the following is most likely associated? 1

No additional abnormality

The majority of patients with accessory tragii tra gii do not have any associated abnormalities. Multiple anomalies, including deafness and midline defects, have been reported but are rare. Q/Q(M)-476251 Report a Problem What is the most likely diagnosis? 1

Incontinentia pigmenti

2

Bullous pemphigoid of infancy

3

Herpes zoster

4

Disseminated herpes simplex

5

Epidermolysis bullosa simplex Q/Q(M)-474927 Report a Problem



The vesicular lesions following the lines of Blaschko are typical for incontinentia pigmenti. Q/Q(M)-474927 Report a Problem Pastia�s lines are characteristic for which eruption: 1

Measles

2

Scarlet fever

3

Rubella

4

Kawasaki�s disease

5

Mumps Q/Q(M)-473689 Report a Problem

Pastia�s lines are characteristic for which eruption: 2

Scarlet fever 20

Pastia�s lines are defined as a linear accentuation of the erythematous sandpaper rash within the flexures. This is classically described with scarlet fever. Q/Q(M)-473689 Report a Problem What disorder is associated with a defect in LMX1B? 1

Nail patella syndrome

2

Steatocystoma

3

Monilithrix

4

Lhermitte-Duclos syndrome

5

Chediak-Higashi syndrome Q/Q(M)-473699 Report a Problem

What disorder is associated with a defect in LMX1B? 1

Nail patella syndrome

A LMX1B gene defect is associated with nail pate lla syndrome. Q/Q(M)-473699 Report a Problem An infant presents with the lesion depicted in the photo. Which of the following is least likely? 1

Sternal clefting

2

Supraumbilical raphe

3

Dandy-Walker malformation

4

Seizure disorder

5

Congenital cataracts Q/Q(M)-474926 Report a Problem

An infant presents with the lesion depicted in the photo. Which of the following is least likely? 4

Seizure disorder

Extensive facial hemangiomas are a component of the PHACES syndrome. Seizure disorder has not been described. Q/Q(M)-474926 Report a Problem The risk of fetal death with intrauterine parvovirus infection may occur with infection in which trimester: 1

First

2

Second 21

3

Third

4

First, Second and Third

5

None of these answers are correct

Q/Q(M)-473691 Report a Problem The risk of fetal death with intrauterine parvovirus infection may occur with infection in which trimester: 4

First, Second and Third

Fetal hydrops may occur with parvovirus infection during all three trimester s although the greatest risk is during the second trimester. Congenital anomalies are not a feature. Q/Q(M)-473691 Report a Problem A young girl presents with enlarged tongue, exomphalmos, and organomegaly and has history of Wilm's tumor. What cutaneous finding is most likely on physical exam? 1

Palmoplantar keratoderma

2

Lymphatic malformation

3

Acral edema

4

Midline capillary malformation

5

Angiokeratoma

Q/Q(M)-482385 Report a Problem A young girl presents with enlarged tongue, exomphalmos, and organomegaly and has history of Wilm's tumor. What cutaneous finding is most likely on physical exam? 4

Midline capillary malformation

This patient has Beckwith-Wiedemann syndrome, a sporadic condition that is a lso known as exomphalosmacroglossia-gigantism syndrome. It also features a midline capillary malformation, linear earlobe creases, intestinal malrotation, and other tumors such as rhabdomyosarcoma an hepatoblastoma. Q/Q(M)-482385 Report a Problem This patient had significantly elevated serum CPK. The likely diagnosis is: 1

Dermatomyositis

2

Lupus erythematosus

3

Psoriasis

4

Atopic dermatitis

5


This patient had significantly elevated serum CPK. The likely diagnosis is: 1

Dermatomyositis 22

The answer is dermatomyositis with the heliotrope color and distribution of er ythema or violaceous color. The skin over the metacarpal and proximal interphalangeal joints can become inflamed and erythematous forming Gottron�s papules. Q/Q(M)-474880 Report a Problem A neonate presents with a large segmental se gmental hemangioma of the V1 distribution. What are features may be associated with this finding? 1

Tram-track calcifications

2

Anterior fossa defect

3

Posterior fossa defect

4

M-paraproteinemia

5

Muscular dystrophy Q/Q(M)-476778 Report a Problem

A neonate presents with a large segmental se gmental hemangioma of the V1 distribution. What are features may be associated with this finding? 3

Posterior fossa defect

PHACES syndrome consists of posterior fossa defect, hemangiomas (often segmental and large), ar terial defects, cardiac defects, eye abnormaliteis, and sternal clefting. Posterior fossa defects include the DandyWalker malformtion. Q/Q(M)-476778 Report a Problem POEMS syndrome is associated with which of the following: 1

Premature aging

2

Odontogenic cysts

3

Eye abnormalities

4

M protein

5

Saddle nose deformity Q/Q(M)-476263 Report a Problem

POEMS syndrome is associated with which of the following: 4

M protein

The acronym POEMS stands for polyneuropathy, pol yneuropathy, organomegaly, organomegaly, endocrinopathy, M-protein, and skin changes. Q/Q(M)-476263 Report a Problem An infant with failure to thrive has multiple xanthomas x anthomas on skin exam and foamy histiocytes on bone marrow biopsy. Your diagnosis is: 1

Gaucher �s disease

2

Tay-Sach�s disease

3

Niemann-Pick disease

4

Fabry�s disease

5

Hunter �s syndrome 23

Q/Q(M)-477194 Report a Problem An infant with failure to thrive has multiple xanthomas x anthomas on skin exam and foamy histiocytes on bone marrow biopsy. Your diagnosis is: 3

Niemann-Pick disease

Niemann-Pick disease is an autosomal recessive disease caused by mutations in sphingomyelin sphingomyelin phosphodiesterase-1. Patients with Type A Niemann-Pick disease may have have xanthomas, progressive psychomotor deterioration, hepatosplenomegaly, hepatosplenomegaly, blindness, cherry red spots, and deafness. Q/Q(M)-477194 Report a Problem A patient presents with an epidermal nevus of the lower abdomen. You take a skin biopsy to confirm the diagnosis. The pathology report indicates that there were findings of epidermolytic hyperkeratosis. Which of the following defects is her offspring at risk ri sk for based on these findings? 1

Transglutaminase

2

Keratin 6a/16

3

Keratin 6b/17

4

Keratin 1/10

5

Keratin 2e

Q/Q(M)-473797 Report a Problem A patient presents with an epidermal nevus of the lower abdomen. You take a skin biopsy to confirm the diagnosis. The pathology report indicates that there were findings of epidermolytic hyperkeratosis. Which of the following defects is her offspring at risk ri sk for based on these findings? 4

Keratin 1/10

Women with epidermal nevi with epidermolytic hyperkeratosis histologically are at risk for offspring with the �full-blown� epidermolytic hyperkeratosis syndrome, which is due to Keratin 1/10 defects. Transglutaminase defects are seen in lamellar ichthyosis, Keratin 6a/16 and 6b/17 in Pachyonychia congenita types I and II and Keratin 2e in Ichth yosis bullosa of Siemens. Q/Q(M)-473797 Report a Problem An 8 year-old boy presents with pink, flushed cheeks and a low-grade fever. On week later, the following lacy eruption appeared. What is the most likely etiology? 1

Paramyxovirus

2

Togavirus

3

Group A streptococcus

4

HHV6

5

Parvovirus Q/Q(M)-474932 Report a Problem

An 8 year-old boy presents with pink, flushed cheeks and a low-grade fever. On week later, the following lacy eruption appeared. What is the most likely etiology? 5

Parvovirus

Fifth disease is caused by Parvovirus B19. Most cases start with prodrome of fever, malaise, headache and 24

rhinorrhea. Cutaneous reaction follows approximately 5-7 days later with erythema of the cheeks ("slapped cheeks") and reticulate rash of the trunk and extremities. Q/Q(M)-474932 Report a Problem The disorder caused by a defect in intestinal z inc-specific transporter SLC39A4 S LC39A4 is most likely to present: 1

At birth

2

Upon weaning from breast milk

3

Upon weaning from formula

4

In childhood

5

In adulthood Q/Q(M)-473680 Report a Problem

The disorder caused by a defect in intestinal z inc-specific transporter SLC39A4 S LC39A4 is most likely to present: 2

Upon weaning from breast milk

In acrodermatitis enteropathica, there is decreased absorption of zinc from the infant�s gastrointestinal tract. The zinc in breast milk has greater availability than nonmaternal sources thus protect ing the child from disease expression until weaning. Q/Q(M)-473680 Report a Problem 2 year old female with the skin eruption, also histor y of joint swelling, painful movement and mucosal lesions. What is the most likely diagnosis? 1

Pustular psoriasis

2

PAPA syndrome

3

Familial Mediterranean fever

4

DIRA

5

Subcorneal pustular dermatosis Q/Q(M)-482881 Report a Problem

2 year old female with the skin eruption, also histor y of joint swelling, painful movement and mucosal lesions. What is the most likely diagnosis? 4

DIRA

Deficiency of the IL-1 receptor antagonist(DIRA). These patients, early in their lives, present with severe pustulosis and ichthyosiform skin eruptions. Also they they can have joint pains and oral mucosal lesions. DIRA is a new addition to the spectrum of autoinflammatory disorders. It will not be encountered often, but its origin highlights the importance of IL-1? in skin function. Q/Q(M)-482881 Report a Problem Osteopathia striata is found in which disorder? 1

McCune-Albright syndrome

2

Neurofibromatosis I

3

Buschke-Ollendorff syndrome

4

Gorlin's syndrome 25

5

Focal dermal hypoplasia Q/Q(M)-477631 Report a Problem

Osteopathia striata is found in which disorder? 5

Focal dermal hypoplasia

Osteopathia striata (vertical striations in the metaphysis of long bones on x-ray) is seen is greater than 80% of cases of focal dermal hypoplasia h ypoplasia (or Goltz syndrome). Polyostotic fibrous dysplasia with recurrent fractures is seen in McCune-Albright syndrome. s yndrome. Sphenoid wing dysplasia dysplasia and thinning of long bone cortex is found in neurofibromatosis I. Osteopoikilosis is an asymptomatic x-ra y finding in patients with BuschkeOllendorf syndrome. Osteopoikilosis reflects ectopic calcification that does not increase risk of fracture. Bifid ribs, vertebral fusion/Sprengel deformity of the spine, and kyphoscoliosis can be seen i n basal cell nevus syndrome (Gorlin's syndrome). Q/Q(M)-477631 Report a Problem Which enanthem is most commonly seen in association with Exanthem subitum? 1

Koplik spots

2

Red strawberry tongue

3

Chapped lips; dry, red mucosa

4

Red macules and streaks on the soft palate

5

Palatal erosions Q/Q(M)-477795 Report a Problem

Which enanthem is most commonly seen in association with Exanthem subitum? 4

Red macules and streaks on the soft palate

Exanthem subitum (roseola or Sixth disease) is caused by HHV6, a dsDNA virus. It presents with high fever for several days followed by an exanthem of er ythematous macules and papules on the trunk that begins as the fever ends. An associated enanthem of red macules/streaks on the soft palate may be seen. Koplik spots are seen in measles; the red strawberry tongue (following the white strawberry tongue) is seen in Scarlet fever; chapped lips and dry, red mucosa ma y be seen in Kawasaki's disease; palatal erosions may be seen in Papular-purpuric gloves and socks socks syndrome. Q/Q(M)-477795 Report a Problem What is the most likely diagnosis? 1

Traction alopecia

2

Tinea capitis

3

Aplasia cutis congenita

4

Nevus sebaceous

5

Alopecia areata Q/Q(M)-476797 Report a Problem

26


Aplasia cutis congenita

Aplasia cutis congenita is characterized by the absence of a portion of skin, most commonly presenting as a solitary defect on the scalp, but sometimes it may occur as multiple lesions. The lesions les ions are noninflammatory and well demarcated appear as an atrophic, a trophic, membranous, ulcerated area with alopecia. The condition may be associated with other physical anomlies. Q/Q(M)-476797 Report a Problem This condition may be associated with which of the following: 1

Hypercalcemia

2

Hyperlipidemia

3

Vitiligo

4


5

Perinatal trauma Q/Q(M)-474919 Report a Problem

This condition may be associated with which of the following: 4


Aplasia cutis congenita may be associated with dystrophic epidermolysis bullosa (Bart syndrome.) Q/Q(M)-474919 Report a Problem A 6-year-old boy presents with osteoma cutis on his face. Which of the following genes is most likely defective? 1

GNAS

2

NSDHL

3

PTEN

4

SPINK5

5

SLURP1 Q/Q(M)-480188 Report a Problem 27

A 6-year-old boy presents with osteoma cutis on his face. Which of the following genes is most likely defective? 1

GNAS

Osteoma cutis in a child is associated with Albright �s hereditary osteodystrophy (pseudohypoparathyroidism), which is caused by a mutation i n GNAS1. Albright hereditary osteodystrophy is characterized by including short stature, obesity, round facies, subcutaneous ossifications, brachydactyly, and other skeletal anomalies . Some patients have mental retardation. AHO is often associated with pseudohypoparathyoidism, hypocalcemia, and elevated PTH levels (PHP Ia) (OMIM, 103580). NSDHL is defective in CHILD syndrome, s yndrome, PTEN in Cowden �s syndrome, SPINK5 in Netherton�s syndrome and SLURP1 in Mal de Maleda. Q/Q(M)-480188 Report a Problem A 2 day-old full term neonate develops blotchy erythematous macules with small central pustules over the upper trunk and extremities. A gram stain reveals predominantly eosinophils. What is the most likely diagnosis? 1


2


3

Urticaria pigmentosa

4


5

Miliaria Q/Q(M)-473677 Report a Problem

A 2 day-old full term neonate develops blotchy erythematous macules with small central pustules over the upper trunk and extremities. A gram stain reveals predominantly eosinophils. What is the most likely diagnosis? 1


Erythema toxicum neonatorum is a very common eruption in healthy newborns. A gram stain reveals sterile pustules containing eosinophils. Q/Q(M)-473677 Report a Problem A deficiency of the surface glycoprotein sialophorin is seen in which immunedeficient disease? 1

Wiskott-Aldrich syndrome

2

Chronic granulomatous disease

3

Job syndrome

4

Severe combined immunodeficiency syndrome

5

Leiner �s disease Q/Q(M)-477199 Report a Problem

A deficiency of the surface glycoprotein sialophorin is seen in which immunedeficient disease? 1


Wiskott-Aldrich syndrome is an X-linked recessive disorder caused by mutations in WAS gene. Patients with Wiskott-Aldrich may have unstable sialoglycoprotein CD 43 on the surface of lymphocytes. Patients with Wiskott-Aldrich have atopic dermatitis with increased risks for secondary infection, thrombocytopenia, and recurrent bacterial infections. They are also at an i ncreased risk for lymphoreticular malignancy (20%). 28

Q/Q(M)-477199 Report a Problem A healthy, full-term infant develops a pustular, erythematous eruption on her face and trunk on the third day of life. A smear taken from one of these pustules would show: 1

Gram-positive bacteria

2

Predominantly neutrophils

3

Multi-nucleated giant cells

4

Predominantly eosinophils

5

Hyphae Q/Q(M)-477632 Report a Problem

A healthy, full-term infant develops a pustular, erythematous eruption on her face and trunk on the third day of life. A smear taken from one of these pustules would show: 4

Predominantly eosinophils

This baby has developed erythema toxicum neonatorum, a benign, self-limited eruption that occurs in the majority of healthy, full-term infants. Erythema toxicum usually develops on the second or third day of life and resolves by day 10. A smear taken from one of the pustules will demonstrate eosinophils, which is sufficient to make the diagnosis. Viral infections of the skin may demonstrate multi-nucleated giant cells on Tzanck smear. Transient neonatal pustular melanosis is present at birth; neonates present with small, superficial pustules that rupture easil y. Some may have ruptured in utero, leaving pigmented macules. The pigmentation may persist for weeks to months in darkly-complexed darkly-complexed infants. A smear of the intracorneal/subcorneal pustule will show mostly neutrophils, but eosinophils may also be present. Q/Q(M)-477632 Report a Problem A 3 month-old girl with multiple hemangiomas along her right jaw is at increased risk for: 1

Bleeding complications

2

Underlying bone abnormalities

3

Subglottic hemangioma

4

Oral obstruction

5

Hearing defecits Q/Q(M)-477334 Report a Problem

A 3 month-old girl with multiple hemangiomas along her right jaw is at increased risk for: 3

Subglottic hemangioma

Hemangiomas are benign vascular tumors which have proliferating phase and then a spontaneous involution phase. Depending upon the location, some hemangiomas ma y have more long term sequelae s equelae such as scarring or structural malformation, and ra rely, consumptive coagulopathy. Infants with hemangiomas of the � beard� distribution should be evaluated for subglottic hemangiomas which may lead to airway obstruction. Q/Q(M)-477334 Report a Problem What the most likely diagnosis? 1

Atopic dermatitis 29

2

Lamellar ichthyosis

3

Rud syndrome

4


5

Keratosis pilaris Q/Q(M)-474935 Report a Problem

What the most likely diagnosis? 1

Atopic dermatitis

Ichythosis vulgaris and hyperlinear palms are both independent minor criteria for the diagnosis of atopic dermatitis in both children and infants. Q/Q(M)-474935 Report a Problem Rhinorrhea, condylomata lata, and mucous patches are all seen with which congenital disorder? 1

Rubella

2

Toxoplasmosis

3

Herpes simplex virus

4

Syphilis

5

Human papillomavirus infection Q/Q(M)-473686 Report a Problem

Rhinorrhea, condylomata lata, and mucous patches are all seen with which congenital disorder? 4

Syphilis

Signs of congenital syphilis include rhinorrhea, snuffles, rhagades, condylomata lata, and mucous patches. Condylomata lata or a generalized papulosquamous eruption of secondary syphilis may be present in diaper area. Q/Q(M)-473686 Report a Problem

An infant who presents with this abnormality will often have the following: 1

The lesion will be on the left-side in a segmental distribution

2

Airway restriction

3

Have multiple liver hemangiomas 30

4

Most likely be a male infant

5

An anterior fossa malformation Q/Q(M)-480558 Report a Problem

An infant who presents with this abnormality will often have the following: 1

The lesion will be on the left-side in a segmental distribution

PHACES syndrome represents posterior fossa malformations, hemangiomas, arterial abnormalities, cardiac abnormalities, eye abnormalities, and sternal defects. Facial hemangiomas are most often on the left-side of the face. The majority of reported cases are female. Q/Q(M)-480558 Report a Problem The genetic disorder depicted in this photo is caused by a mutation in: 1

MASH2

2

STK11

3

PTEN

4

Patched

5

TSC1 Q/Q(M)-474931 Report a Problem

The genetic disorder depicted in this photo is caused by a mutation in: 2

STK11

The multiple labial lentigo seen on this young boy are seen with Peutz-Jegher syndrome. A defect in the STK11 gene is responsible. Q/Q(M)-474931 Report a Problem A 4 month-old with diffuse blisters and erosions has a skin biopsy diagnostic of generalized mastocytosis. Which topical dressing should be avoided in this patient?

31

1

Mupirocin ointment

2

Polymyxin B ointment

3

Petrolatum

4

Neomycin ointment

5

Silver sulfadiazine Q/Q(M)-477328 Report a Problem

A 4 month-old with diffuse blisters and erosions has a skin biopsy diagnostic of generalized mastocytosis. Which topical dressing should be avoided in this patient? 2

Polymyxin B ointment

Mastocytosis comprises a group of diseases characterized chara cterized by increased number of mast cells cell s in the skin and other organs. Seventy-five percent of cases occur before the age of 2. Patients with mastocytosis should avoid potential mast degranulators including aspirin, codeine, opiates, procaine, spicy foods, cheese, alcohol, polymyxin B. Q/Q(M)-477328 Report a Problem Which of the following is a sign of EARLY postnatal congenital syphilis? 1

Clutton's joints

2

Higoumenaki's sign

3

Hutchinson's teeth

4

Saber shins

5

Wimberger's sign Q/Q(M)-482501 Report a Problem

Which of the following is a sign of EARLY postnatal congenital syphilis? 5

Wimberger's sign

Wimberger's sign is a sign of early earl y postnatal congenital syphilis (occurs < 2 years old). All other choices are signs of late postnatal congenital syphilis. Wimberger's sign is a radiographic sign showing a sawtooth appearance of the proximal tibia. Clutton's joints are nontender and represent synovitis with effusions of the knees and elbows. Higoumenaki's sign is a unilateral clavi cular enlargement secondary to periostitis. Hutchinson's teeth are centrally notched, wide spaced, peg shaped upper incisors. Saber shins ar e anterior bowing of the tibia. Q/Q(M)-482501 Report a Problem An eight year-old boy presents with pink, flushed cheeks and a low-grade fever. Eruptions then appeared. What is the most likely etiology? 1

Paramyxovirus

2

Togavirus

3

Group A streptococcus

4

HHV6

5

Parvovirus Q/Q(M)-476796 Report a Problem 32

An eight year-old boy presents with pink, flushed cheeks and a low-grade fever. Eruptions then appeared. What is the most likely etiology? 5

Parvovirus

Fifths Disease or erythema infectiosum, is caused by parvovirus B19, a single-stranded DNA virus. There are a few clinical presentations pres entations including, diffuse lacy rash on the trunk that spreads graduall y toward the distal extremities, papular-pruritic "gloves-and-socks" syndrome and aplastic crisis which does not have concomitant rash. Q/Q(M)-476796 Report a Problem The lesion depicted is most often associated with: 1


2

Chondrodysplasia punctata

3

Sphenoid wing hypoplasia

4

Cleft palate

5

Osteopoikilosis Q/Q(M)-474921 Report a Problem

The lesion depicted is most often associated with: 1


The �coast of Maine� caf � au lait macule is associated with McCune-Albright syndrome and polyostotic fibrous dysplasia. Q/Q(M)-474921 Report a Problem What syndrome is the disorder shown in the photo associated with? 1

Turner syndrome

2

Down syndrome

3

Noonan syndrome

4

Griscelli syndrome

5

Bloom syndrome Q/Q(M)-474928 Report a Problem

33

What syndrome is the disorder shown in the photo associated with? 3

Noonan syndrome

The photo shows keratosis pilaris atrophicans faceii and surgically corrected ptosis. Both of these findings are associated with Noonan syndrome. Q/Q(M)-474928 Report a Problem Psammomatous melanotic schwannomas are associated with: 1

Bloom syndrome

2

Carney complex

3

Neurofibromatosis Type 1

4


5

Tuberous sclerosis Q/Q(M)-473702 Report a Problem

Psammomatous melanotic schwannomas are associated with: 2

Carney complex

Psammomatous melanotic schwannomas have been described in the Carney complex, a defect in the tumor suppressor gene, PRKAR1A. Q/Q(M)-473702 Report a Problem What is the diagnosis? 1

Unilateral laterothoracic exanthem

2

Roseola

3

Rubella

4

Urticaria

5

Contact dermatitis Q/Q(M)-474929 Report a Problem

34

What is the diagnosis? 1

Unilateral laterothoracic exanthem

The child in the photo depicts the �Statue of Liberty� sign of unilateral laterothoracic exanthem. Q/Q(M)-474929 Report a Problem Which of followings syndromes characterized by Seborrheic-like or exfoliative dermati tis 1

Leiner's disease

2

Hyperimmunoglobulinemia Hyperimmunoglobulinemia E syndrome

3


4


5

X-linked agammaglobulinemia Q/Q(M)-482136 Report a Problem

Which of followings syndromes characterized by Seborrheic-like or exfoliative dermati tis 1

Leiner's disease

Immunodeficiency disorders may be associated with a variet y of cutaneous abnormalities, and recognition of these clinical features may allow an early diagnosis of primary immunodeficiency. Cutaneous abnormalities may include cutaneous infections, atopic- or seborrheic-like dermatitis, macular erythemas, alopecia, poor wound healing, purpura, petechiae, telangiectasias, pigmentary dilution, cutaneous granulomas, extensive warts, angioedema, and lupus-like changes. Leiner's disease or e rythroderma desquamativum is a complication of seborrheic dermatitis in infants (dermatitis seborrhoides infantum) There is usually a sudden confluence of lesions, leading to a universal scaling redness of the skin (erythroderma). The young patients are severely ill with anemia, diarrhea, and vomiting.The disease is both a familial and a nonfamilial form. The former is noted for having a functional deficiency of C5 complement, resulting in defective opsonization. These patients respond to antibiotics and infusions of fresh frozen plasma or whole blood. The other choices in the questions can all be associated with atopiclike dermatitis. Q/Q(M)-482136 Report a Problem Which of the following can present as collodion baby? 1

Ichthyosis vulgaris

2

X-linked ichthyosis

3

Lamellar ichthyosis 35

4

Bullous congenital ichthyosiform erythroderma

5

Sjogren-Larsson syndrome Q/Q(M)-477629 Report a Problem

Which of the following can present as collodion baby? 3

Lamellar ichthyosis

The most common presentation of collodion baby is lamellar ichthyosis, followed by congenital ichthyosiform erythroderma. Patients with ichthyosis vulgaris and x-linked ichthyosis are normal at birth. Bullous congenital ichthyosiform erythroderma or epidermolytic hyperkeratosis presents with widespread bullae, erthroderma, and denuded skin. Sjogren-Larsson presents with generalized ichthyosis and erythroderma in infancy. It is important to know the at-birth presentations of all the disorders of cornification. Q/Q(M)-477629 Report a Problem What is the function of the gene which is defective in ataxia-telangiectasia? 1

Gap junction protein

2

Cross-linking of structural proteins in the protein and lipid envelope of the upper epidermis

3

Pathway of cholesterol biosynthesis

4

DNA repair protein

5

Tumor supressor protein Q/Q(M)-477630 Report a Problem

What is the function of the gene which is defective in ataxia-telangiectasia? 4

DNA repair protein

The defective gene is ataxia-telangiectasia ataxia-t elangiectasia (Louis-Bar syndrome) is the ATM gene, which is res ponsible for DNA repair, especially after ionizing radiati on. Tumor suppressor genes mutations are responsible for basal cell nevus syndrome, xeroderma pigmentosum, Muir-Torre Muir-Torre syndrome, dyskeratosis congenital, Gardner syndrome, Peutz-Jeghers syndrome, Cowden syndrome, and MEN s yndromes. Connexins are gap junction proteins that are responsible for intercellular communication and signaling. Mutations in connexins are responsible for Vohwinkel syndrome and erythrokeratoderma variabilis. Mutations in the cholesterol biosynthesis pathways cause CHILD syndrome and Conradi-Hunermann syndrome. Tranglutaminase 1 (TGM 1) is involved in the normal cross-linking of structural proteins in the protein and lipid envelope of the upper epidermis. TGM 1 is mutated in lamellar ichthyosis and congenital ichthyosiform erythroderma. Q/Q(M)-477630 Report a Problem A 24 month-old infant presents with yellowish-brown, crusted papules with petechiae in a seborrheic distribution. A biopsy is done to confirm a diagnosis. Which histologic picture is most likel y? 1

CD1-, S100- cells with reniform nuclei

2

Foamy histiocytes with Touton giant cells

3

CD1+, S100+ cells with reniform nuclei

4

Mixed cellular infiltrate in a � ball and claw� pattern

5

Superficial perivascular infiltrate with mild spongiosis and neutrophil containing scale crust Q/Q(M)-473681 Report a Problem

36

A 24 month-old infant presents with yellowish-brown, crusted papules with petechiae in a seborrheic distribution. A biopsy is done to confirm a diagnosis. Which histologic picture is most likel y? 3

CD1+, S100+ cells with reniform nuclei

Langerhans cells are CD1 and S100 positive. The nuclei are described as kidney shaped, or reniform. Q/Q(M)-473681 Report a Problem Posterior auricular adenopathy is a common feature of which exanthem: 1

Measles

2

Mumps

3

Scarlet fever

4

Rubella

5

Erythema infectiosum Q/Q(M)-473690 Report a Problem

Posterior auricular adenopathy is a common feature of which exanthem: 4

Rubella

Lymphadenopathy with 1-7 days of malaise is commonly described with rubella infection. The rose-pink macular rash follows the prodrome. Q/Q(M)-473690 Report a Problem A 2 week-old infant is brought to the ER with a rash on her face. She is found to have a 3rd degree heart block. What is the risk that a second child born to this mother will have the same diagnosis? 1

5%

2

10%

3

25%

4

50%

5

100% Q/Q(M)-477634 Report a Problem

A 2 week-old infant is brought to the ER with a rash on her face. She is found to have a 3rd degree heart block. What is the risk that a second child born to this mother will have the same diagnosis? 3

25%

The diagnosis here is neonatal lupus erythematosus. Babies are normal at birth and develop skin lesions within the few months of life. About half of these babies will have an associated congenital heart block, usually 3rd degree, which is permanent. Most infants with NLE are girls a nd are born to mothers who are Ro/La positive. There is a 25% chance that a se cond child with have NLE. Q/Q(M)-477634 Report a Problem Subcutaneous fat necrosis of the newborn is usually treated with: 1

Aspirin

2

Topical calcipitriol

3

Excision

4

Observation 37

5

Retinoids Q/Q(M)-479620 Report a Problem

Subcutaneous fat necrosis of the newborn is usually treated with: 4

Observation

Subcutaneous fat necrosis of the newborn is rare and self-limited. It is thought to result from perinatal stress and resolves in 3-6 months. Treatment is usually not necessary unless hypercalcemia develops. Q/Q(M)-479620 Report a Problem A 10 year-old male presents with new pink-red papules distributed on his bilateral cheeks, elbows, knees, and buttocks. The lesions are not pruritic. His mother notes he has suffered from low grade fevers over the past few days, but is otherwise healthy. Which of the following is the most likely diagnosis? 1

rubella

2

rubeola

3

roseola

4

papular acrodermatitis of childhood

5

dermatitis herpetiformis Q/Q(M)-482829 Report a Problem

A 10 year-old male presents with new pink-red papules distributed on his bilateral cheeks, elbows, knees, and buttocks. The lesions are not pruritic. His mother notes he has suffered from low grade fevers over the past few days, but is otherwise healthy. Which of the following is the most likely diagnosis? 4

papular acrodermatitis of childhood

Papular acrodermatitis of childhood (Gianotti-Crosti syndrome) typically affects children 6 months to 10 years of age, and is characterized characteriz ed by symmetric papules (and sometimes papulovesicles) involving the face and buttocks ( �four cheeks�) and extensor surfaces of the upper and lower extr emities. Lesions tend to be asymptomatic. Multiple infectious etiologies have been implicated, including Epstein-Barr Epstein-Barr virus (most common in the U.S.) and hepatitis B (most common in Europe). However, the eruption is most often selflimited, resolving in 4 to 8 weeks, and thus further work-up (e.g. testing for hepatiti s) is indicated only if additional clinical symptoms are present. Q/Q(M)-482829 Report a Problem Hemangiomas of infancy are more likely to be: 1

GLUT-1 negative

2

Merosin positive but GLUT-1 negative

3

Lewis Y antigen positive, merosin negative

4

Glut-1 positive, merosin positive, Lewis Y antigen negative

5

Glut-1 positive, merosin positive, Lewis Y antigen positive Q/Q(M)-482865 Report a Problem

Hemangiomas of infancy are more likely to be: 5

Glut-1 positive, merosin positive, Lewis Y antigen positive

Hemangiomas show GLUT-1 positivity and stain positively with placental markers. 38

Q/Q(M)-482865 Report a Problem Late onset subungual keratotic tumors are associated with: 1


2


3

Carney complex

4

Cowden syndrome

5

Basal cell nevus syndrome Q/Q(M)-473704 Report a Problem

Late onset subungual keratotic tumors are associated with: 1


A NEMO gene defect can cause subungual keratotic growths. The typical age of presentat ion is early adulthood. Q/Q(M)-473704 Report a Problem

Variants of xeroderma pigmentosum are due to all of the following defects except: 1

Nucleotide excision repair

2

Helicase

3

Endonuclease

4

Thymidine kinase

5

Postrepliction repair Q/Q(M)-473698 Report a Problem

Variants of xeroderma pigmentosum are due to all of the following defects except: 4

Thymidine kinase

The photosensitivity of xeroderma pigmentosum is caused by defect in DNA repair mechanisms. Thymidine kinase is not affected. Q/Q(M)-473698 Report a Problem Rapp-Hodgkin is caused by a defect in the following gene: 1

Plakophilin

2

Desmoglein 1

3

Ectodysplasin A

4

P63

5

Connexin 30 Q/Q(M)-476265 Report a Problem

Rapp-Hodgkin is caused by a defect in the following gene: 39

4

P63

Rapp-Hodgkin, along with the other ectodermal dysplasia-clefting syndromes, is reportedly caused by a defect in the p63 gene. Q/Q(M)-476265 Report a Problem Individuals with which of the following syndromes characteristically present with photosensitivity, mental retardation, a "wizened" appearance, "bird-headed" facies, and "Mickey Mouse" ears? 1

Tay Syndrome

2

Rothmund-Thomson Syndrome

3

Hutchinson-Gilford Progeria Syndrome

4

Cockayne Syndrome

5

Werner Syndrome Q/Q(M)-478060 Report a Problem

Individuals with which of the following syndromes characteristically present with photosensitivity, mental retardation, a "wizened" appearance, "bird-headed" facies, and "Mickey Mouse" ears? 4

Cockayne Syndrome

Cockayne Syndrome is AR, caused by defective excision repair, cross-complementing group 8 gene (ERCC8). It presents with photosensitivity, mental retardation, and cachectic dwarfism. Patients have a characteristic "wizened" appearance, "bird-headed" facies, and "Mickey Mouse" ears. Cataracts, deafness, pigmentary retinopathy, dental caries, and skeletal, GU, and endocrine endocrine abnormalities may be seen. Q/Q(M)-478060 Report a Problem A 2 year-old has a high fever for three days, da ys, as the fever breaks, a generalized generaliz ed rash appears on the trunk. The most likely diagnosis is: 1

Measles

2

Mumps

3

Rubella

4

Erythema infectiosum

5

Roseola

Q/Q(M)-473694 Report a Problem A 2 year-old has a high fever for three days, da ys, as the fever breaks, a generalized generaliz ed rash appears on the trunk. The most likely diagnosis is: 5

Roseola

Roseola typically appears in toddlers. A high fever followed b y a generalized maculopapular rash is characteristic for this HHV6 infection. Q/Q(M)-473694 Report a Problem


1

Papular acrodermatitis of childhood

2

Mucocutaneous lymph node syndrome

3

German measles

4

Letterer-Siwe disease

5

Exanthem subitum Q/Q(M)-475959 Report a Problem


Papular acrodermatitis of childhood

Gianotti-Crosti syndrome is also known as papular acrodermatitis of childhood. The eruption is characterized by lichenoid papules in an acral distribution. Q/Q(M)-475959 Report a Problem Ankyloblepharon filiforme adnatum is seen with a defect in: 1

Plakophilin

2

Plakoglobin

3

Desmoglein

4

C-kit

5

p63

Q/Q(M)-473705 Report a Problem Ankyloblepharon filiforme adnatum is seen with a defect in: 5

p63

The ectodermal-clefting syndromes are caused by a defect in the p63 gene. Specifically, AEC or Hay-Wells syndrome is comprised of ankyloblepharon filiforme adnatum, ectodermal dysplasia and clefting. Q/Q(M)-473705 Report a Problem Which of the following is characteristic of Wooly Hair Nevus: 41

1

Typically occurs in the 5th decade

2

Ocular abnormalities may be associated

3

There are no nevi associated with this hair abnormality

4

Sponteneous improvement never occurs

5

It is hereditary

Q/Q(M)-478697 Report a Problem Which of the following is characteristic of Wooly Hair Nevus: 5

It is hereditary

Wooly Hair Nevus is the presence of Negroid hair on the scalp of persons of non-Negroid inheritance. The unruly hair presents at birth or in infancy, usually as a solitary problem inherited in an autosomal dominant fashion. Q/Q(M)-478697 Report a Problem Which of the following should be the next step in the management of this patient? 1

Barium swallow

2

spine x-ray

3

Cardiac evaluation

4

Administration of oral antibiotics

5

Pulmonary function studies Q/Q(M)-477846 Report a Problem

Which of the following should be the next step in the management of this patient? 3

Cardiac evaluation

This patient must be evaluated thoroughly for PHACES syndrome. PHACES is an acronym for Posterior fossa malformations (Dandy-Walker malformation is most common), Hemangiomas, Arterial anomalies, Coarctation of the aorta, Eye abnormalities, and Sternal cle ft defects. This patient should have a complete cardiac evaluation, neuroimaging, and ophthalmologic exam. If the facial hemangioma involves the beard area, this may indicate laryngeal lar yngeal involvement and appropriate imaging and evaluation is mandated. Systemic steroids at high doses (5 mg/kg) are usuall y administered. If caught early, the sequelae of PHACES syndrome can be minimized. Q/Q(M)-477846 Report a Problem 42

A boy is noted at birth to have coarse scales over his trunk and extremities. The face, palms, soles and flexures are spared. What is the least likely association? 1

Corneal opacities

2

Cryptorchidism

3

Ectropion

4

Prolonged maternal labor

5

Neurologic abnormality Q/Q(M)-474934 Report a Problem

A boy is noted at birth to have coarse scales over his trunk and extremities. The face, palms, soles and flexures are spared. What is the least likely association? 3

Ectropion

X-linked ichthyosis is characterized by small, dark, firml y adherent scales accentuated on the sides si des of the neck and trunk. The face, palms, soles, antecubital and popliteal fle xures are generally spared. Associated extracutaneous findings include corneal opacities (50%), undescended testes (20%), and prolonged maternal labor (usual). Neurological or mental retardation ret ardation are rare but documented associations as XLI can be associated with a contiguous gene syndrome with Kallman syndrome, mental retardation and X linked recessive chrondrodysplasia punctata. Q/Q(M)-474934 Report a Problem What is the best test to confirm a diagnosis? 1

DsDNA antibody

2

Anti Ro antibody

3

Biopsy

4

KOH

5

Gram stain Q/Q(M)-476258 Report a Problem

What is the best test to confirm a diagnosis? 43

4

KOH

The picture depicts tinea faceii. A KOH exam should be performed to look for hyphae. Q/Q(M)-476258 Report a Problem What is the best treatment option? 1

Oral cephalexin

2

Oral acyclovir

3

Observation

4

Topical tretinoin

5

Topical ketoconazole Q/Q(M)-476794 Report a Problem

What is the best treatment option? 4

Topical tretinoin

The lesions shown in the photo are comedones of infantile acne. Topical tretinoin would be the best treatment option. Q/Q(M)-476794 Report a Problem A young boy presents with a port-wine stain, a Mongolian spot, and a nevus spilus. Which phakomatosis pigmentovascularis does he have? 1

Type I

2

Type II

3

Type III

4

Type IV

5

Type V

Q/Q(M)-482384 Report a Problem A young boy presents with a port-wine stain, a Mongolian spot, and a nevus spilus. Which phakomatosis pigmentovascularis does he have? 4

Type IV

This patient has type IV phakomatosis pigmentovascularis characterized b y a port-wine stain, Mongolian spot, and nevus spilus. All types have a nevus flammeus. In addition, type II demonstrates a Mongolian spot (may be associated with granular cell tumor), type II a speckled nevus, and type V cutis marmorata telangiectasia. Type II-IV can also have ha ve a nevus amenicus. Phakomatosis pigmentovascularis is thought to be caused by the "twin spot" phenomenon. phenomenon. Q/Q(M)-482384 Report a Problem

44

Which of the following is the most common complication associated with cutis marmorata telangectatica congenita? 1

Seizure disorder

2

Atrial septal defect

3

Systemic lupus erythematosus

4

Hypercalcemia

5

Limb hypertrophy or atrophy

Q/Q(M)-473676 Report a Problem Which of the following is the most common complication associated with cutis marmorata telangectatica congenita? 5

Limb hypertrophy or atrophy

Hypertrophy or atrophy of the affected limb is the most li kely consequence of cutis marmorata telangectatica congenita. Orthopedic evaluation should be a part of the patient �s routine management. Q/Q(M)-473676 Report a Problem Juvenile self-healing papular mucinosis is associated with which of the findings? 1

Arthralgias

2

Cataracts

3

Type I diabetes

4

Calcinosis cutis

5

Xeropthalmia

Q/Q(M)-482478 Report a Problem Juvenile self-healing papular mucinosis is associated with which of the findings? 1

Arthralgias

Self-healing papular mucinosis is a rare disorder characterized by small, nontender, flesh-colored papules commonly seen on the head, neck, trunk, and periarticular areas of chil dren. Self-healing papular mucinosis is associated with systemic findings during acute s tages, including fever, arthralgias, weakness, and often carpal tunnel syndrome. A handful of cases have been reported in adults, a lthough systemic symptoms are not typically found in this population. Q/Q(M)-482478 Report a Problem Which of the following syndromes is associated with development of giant cell epulides? 1

Cherubism

2

Gardner syndrome

3

Cowden syndrome

4

Multiple hamartoma syndrome

5

CHILD syndrome

Q/Q(M)-482834 Report a Problem Which of the following syndromes is associated with development of giant cell epulides? 1

Cherubism 45

Cherubism is an autosomally-dominant inherited disorder characterized by asymptomatic fibro-oss eous hyperplasia and replacement of normal bone which commences in childhood. Typically the mandible is affected, leading to progressive enlargement and swollen �cherub-like� cheeks. Similar involvement of maxillary bones results in periorbital enlargement, resulting in the tendency of affected individuals to �look up towards the sky. � Frequently the disorder is self-limited and self-regressive. Mutations in SH3BP2 have been identified as a candidate gene defect. Affected i ndividuals may also present with single or multiple giant cell epulides (singular: epulis), which is a benign, bluish-red nodule arising on the gingiva, near deciduous incisors or bicuspids. It bears histologic similarit y to giant cell tumor of the tendon sheath. Q/Q(M)-482834 Report a Problem What is the most likely diagnosis? 1

Psoriasis

2

Langerhans cell histiocytosis

3

Seborrheic dermatitis

4

Contact dermatitis

5

Perianal streptococcal disease Q/Q(M)-476793 Report a Problem


Psoriasis

The well-defined erythematous plaque is an example of psorisis in the diaper area. The moist nature of the diaper environment results in a loss of the classic silvery scale. Q/Q(M)-476793 Report a Problem Neonatal acne is associated with species of which organism: 1

Staphylococcus

2

Propionibacterium

3

Candida

4

Malassezia

5

Streptococcus Q/Q(M)-475879 Report a Problem 46

Neonatal acne is associated with species of which organism: 4

Malassezia

Acne which develops within the first 30 days of life is termed neonatal acne. Neonatal acne has a predilection for the face, chest, back and groin appearing as small, discrete papules at 2 to 4 weeks weeks of age, and persisting for up to 8 months. As these lesions are self-resolving, no treatment is necessary, necessa ry, though 2.5% benzoyl peroxide may hasten resolution. Neonatal acne is quite common and is postulated to occur as a result of hyperplasia of premature sebaceous glands coupled with transient increases in circulating cir culating androgens. More recent data suggests that Malassezia species may be implicated at etiologic factors in neonatal acne. These organisms have been cultured from the skin of aff ected patients, though their exact role in unclear. Q/Q(M)-475879 Report a Problem Most common location of cutaneous lesions in neonatal lupus erythrematosus is 1

Cheeks

2

Nose

3

Scalp

4

Perioral

5

Periorbital

Q/Q(M)-482134 Report a Problem Most common location of cutaneous lesions in neonatal lupus erythrematosus is 5

Periorbital

neonatal lupus erythrematosus(NLE) is a form of subacute cutaneous lupus er ythrematosus(SCLE) that occur in infants whose mothers have anti-Ro autoantibodies. Unlike SCLE in adults, lesions of NLE have a predilection for the face especially periorbital region. Other sites include scalp, arms, legs, trunk and groin. Q/Q(M)-482134 Report a Problem A 3 month old baby girl is brought to your office for consultation. You note an infantile hemangioma on the tip of the nose. Imaging reveals no underlying structural anomalies in the cerebro-vasculature. You discuss starting propranolol. What side effect do you need to monitor for? 1

Hypertension

2

Hypercalcemia

3

Hypoglycemia

4

Tachycardia

5

Hypernatremia

Q/Q(M)-482515 Report a Problem A 3 month old baby girl is brought to your office for consultation. You note an infantile hemangioma on the tip of the nose. Imaging reveals no underlying structural anomalies in the cerebro-vasculature. You discuss starting propranolol. What side effect do you need to monitor for? 3

Hypoglycemia

In the treatment of infantile hemangiomas with propranolol, the most common serious adverse effects are bradycardia and hypotension. Other potential potential adverse effects include bronchospasm and hypoglycemia. Sustained hypoglycemia in infancy has been associated with longterm neurologic sequelae. Infants with very large hemangiomas or miliary hemangiomatosis are also at ris k for high-output cardiac compromise. Q/Q(M)-482515 Report a Problem 47

An infant presents with red-purple, granulomatous nodules occurring in the diaper area. The etiology is secondary to local irritation, maceration and Candida albicans. What is the most likely diagnosis? 1


2

Langerhans cell histiocytosisc.

3


4

Biotin deficiency

5

Psoriasis

Q/Q(M)-473683 Report a Problem An infant presents with red-purple, granulomatous nodules occurring in the diaper area. The etiology is secondary to local irritation, maceration and Candida albicans. What is the most likely diagnosis? 1


The etiology of granuloma gluteale infantum is multifactorial, resulting from the unique environment of the diaper area. Treatment consists of topical antifungal agents, barrier creams, and anti-inflammatory agents as needed. Q/Q(M)-473683 Report a Problem The Carney complex is associated with a defect i n: 1

PRKAR1A

2

LYST

3

PTEN

4

MASH2

5

MLH1

Q/Q(M)-473700 Report a Problem The Carney complex is associated with a defect i n: 1

PRKAR1A

A PRKAR1A gene defect is associated with the Carney complex. Q/Q(M)-473700 Report a Problem Koplik spots typically appear: 1

Before the exanthem

2

At the same time as the exanthem

3

1 week after the exanthem

4

6 weeks after the exanthem

5

8 weeks after the exanthem

Q/Q(M)-473693 Report a Problem oplik spots typically appear: 1

Before the exanthem

The enanthem of measles precedes the morbilliform eruption. Q/Q(M)-473693 Report a Problem

48



2

Acropustulosis of infancy

3

Neonatal cphalic pustulosis

4

Miliaria

5

Erythema toxicum neonatorum Q/Q(M)-476868 Report a Problem


Neonatal cphalic pustulosis

In neonatal cephalic pustulosis (neonatal acne), papules and pustules are present but comedones are absent. Spontaneous remission occurs. Q/Q(M)-476868 Report a Problem What is the most likely diagnosis? 1

Psoriasis

2


3


4

Contact dermatitis

5

Perianal streptococcal disease Q/Q(M)-476438 Report a Problem

49


Perianal streptococcal disease

The bright red erythema of perianal streptococcal dise ase can also involve the creases of the groin and axillae. Q/Q(M)-476438 Report a Problem Hemangiomas of infancy are more likely to occur: 1

a. In males

2

b. In Asians

3

c. In children who are large for gestational age

4

d. In multiple gestation

5

e. In post term infants

Q/Q(M)-482873 Report a Problem Hemangiomas of infancy are more likely to occur: 4

d. In multiple gestation

D. is Correct. Hemangiomas of infancy occur more commonly in girls, in Caucasians, in babies who are premature and low birth weight. They are more likely to occur in multiple gestation Q/Q(M)-482873 Report a Problem A 6 month-old presents with orange-brown crusted plaques around the mouth and groin. Several bullae are present on the fingers and toes. Which of the following following laboratory values is likely to be abnormal? 1

Hematocrit

2

Calcium

3

Platelet count

4

ALT

5


Q/Q(M)-473679 Report a Problem A 6 month-old presents with orange-brown crusted plaques around the mouth and groin. Several bullae are present on the fingers and toes. Which of the following following laboratory values is likely to be abnormal? 5


The most likely diagnosis is acrodermatitis enteropathica. Alkaline phosphatase is a zinc dependant enzyme that is decreased in response to low serum zinc levels. Q/Q(M)-473679 Report a Problem Trichothiodystrophy classically includes which of the following findings: 1

Trichostasis

2

Trichoschisis

3

Pili torti

4

Trichorrhexis invaginata

5

Trichorrhexis nodosa Q/Q(M)-476262 Report a Problem

Trichothiodystrophy classically includes which of the following findings:

50

2

Trichoschisis

Trichothiodystrophy or PIBIDS is associated with the �tiger-tail� abnormality of trichoschisis. Q/Q(M)-476262 Report a Problem

�Slapped cheeks � followed by a lacy eruption on extremities: 1

ssDNA virus

2

dsDNA virus

3

ssRNA virus

4

dsRNA virus

5

Streptococcus Q/Q(M)-473692 Report a Problem

�Slapped cheeks � followed by a lacy eruption on extremities: 1

ssDNA virus

The erythematous eruption on the cheeks precedes the generalized la cy rash of Fifth disease. The causative agent is parvovirus B19, a single stranded DNA virus. Q/Q(M)-473692 Report a Problem Which of the following bullous disorders is due to a target antigen that is a 97 kD protein which is a member of BPAG-2? 1

Bullous Dermolysis of the Newborn

2

Neonatal Pemphigus

3

Linear IgA Bullous Dermatosis

4

Junctional epidermolysis bullosa (Herlitz)

5


Q/Q(M)-477809 Report a Problem Which of the following bullous disorders is due to a target antigen that is a 97 kD protein which is a member of BPAG-2? 3

Linear IgA Bullous Dermatosis

Linear IgA Bullous Dermatosis is due to a target tar get antigen that is a 97 kD protein which is a member of BPAG-2. Transient bullous dermolysis of the newborn is due to a transient defect i n intracytoplasmic packaging or in the transport of type VII collagen collagen within basal keratinocytes. Neonatal pemphigus is due to maternal transfer of IgG antibodies reacting reacti ng to Desmoglein 3 (pemphigus vulgaris). Epidermolysis bullosa simplex is due to keratin 5/14 abnormalities and junctional EB (Herlitz) is due to defects in Laminin 5. Q/Q(M)-477809 Report a Problem A newborn presents with a pustular rash, joint swelling, oral mucosal lesions, and pain with movement. Over time, cutaneous pustulosis, ranging from discrete crops of pustules to generalized severe pustulosis and ichthyosiform lesions develop. In addition the newborn develops sterile multifocal osteomyelitis and periostitis. What is this condition? 1

Muckle-Wells syndrome

2

Deficiency of the interleukin-1-receptor antagonist (DIRA)

3

Familial Mediterranean fever

4

Hyper-IgD syndrome 51

5

PAPA syndrome

Q/Q(M)-482549 Report a Problem A newborn presents with a pustular rash, joint swelling, oral mucosal lesions, and pain with movement. Over time, cutaneous pustulosis, ranging from discrete crops of pustules to generalized severe pustulosis and ichthyosiform lesions develop. In addition the newborn develops sterile multifocal osteomyelitis and periostitis. What is this condition? 2

Deficiency of the interleukin-1-receptor antagonist (DIRA)

This condition is deficiency of the interleukin-1-receptor antagonist (DIRA) and due to a mutation in the IL1RN gene. Muckle-Wells syndrome is due to a mutation in the CIAS1 gene and presents with sensorineural deafness, recurrent hives, and amyloidosis. Familial Mediterranean fever is most often due to a mutation in the MEFV gene. Hyper-IgD syndrome is a rare periodic fever syndrome characterised by attacks of fever, arthralgia, skin lesions le sions including cyclical mouth ulcers, and diarrhea due to mutations in the mevalonate kinase gene. PAPA syndrome presents with pyogenic arthritis, pyoderma gangrenosum and acne and is associated with mutations in i n the CD2 binding protein 1 (CD2BP1). Q/Q(M)-482549 Report a Problem Which of the following disorders is more likely to occur in children with chronic fecal incontinence? 1


2

Perianal pseudoverrucous papules and nodules

3

Perianal streptococcal disease

4


5


Q/Q(M)-473685 Report a Problem Which of the following disorders is more likely to occur in children with chronic fecal incontinence? 2

Perianal pseudoverrucous papules and nodules

The warty papules of perianal pseudoverrucous papules and nodules are seen more commonly in the setting of chronic fecal incontinence. Q/Q(M)-473685 Report a Problem A 12 y/o female with autoimmune hepatitis on oral prednisone now with eruption on the face for 3 weeks. What is the most likely diagnosis? 1

Tinea facei

2

Acne vulgaris

3

Keratosis pilaris

4

Pityrosporum Folliculitis

5

Diagnosis not listed Q/Q(M)-482890 Report a Problem

A 12 y/o female with autoimmune hepatitis on oral prednisone now with eruption on the face for 3 weeks. What is the most likely diagnosis? 4

Pityrosporum Folliculitis

It presents as 1-2mm pruritic monomorphic papules and pustules on chest, back, upper arms, someti mes 52

face. The presence of pruritus may help to distinguish this e ntity from acne. It has been associated with wit h oral cortico-steroid use, diabetes and other immune suppressed states. Q/Q(M)-482890 Report a Problem Which of the following diseases with immunodeficiency has an increased r isk of lymphoreticular malignancy? 1


2


3

Job syndrome

4

Severe combined immunodeficiency syndrome

5

Leiner �s disease Q/Q(M)-477213 Report a Problem

Which of the following diseases with immunodeficiency has an increased r isk of lymphoreticular malignancy? 1


Wiskott-Aldrich syndrome is an X-linked recessive disorder caused by mutations in WAS gene. Patients with Wiskott-Aldrich have atopic dermatitis with increased risks for secondary infection, thrombocytopenia, and recurrent bacterial infections. They are also at an i ncreased risk for lymphoreticular malignancy (20%). Q/Q(M)-477213 Report a Problem The standard of care of patients with acute Kawasaki �s disease is: 1

Prednisone

2

Supportive care

3

Aspirin and IVIG

4

Penicillin

5

Acetaminophen and IVIG Q/Q(M)-477150 Report a Problem

The standard of care of patients with acute Kawasaki �s disease is: 3

Aspirin and IVIG

Kawasaki disease, also called mucocutaneous lymph node syndrome, is an acute febrile disorder based on the clinical criteria of changes in peripheral extremities, polymorphous exanthema, conjunctival injection without exudates, changes in the lips or oral cavit y, acute cervical lymphadenopathy. Fever must be present, lasting more than 5 days. Treatment is aimed to prevent prevent coronary aneurysms and myocardial infarction. Treatment for acute Kawasaki disease is intravenous immunoglobulin 2 g/kg over 10-12 hours and aspirin therapy. Q/Q(M)-477150 Report a Problem

53

Pediatric Dermatology MCQs

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