pediatric dermatology mcqs
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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? 1Advil
2Acetaminophen
3Hydration
4Corticosteroids
5Observation
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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? 4Corticosteroids
Gianotti-Crosti or papular acrodermatitis of childhood is associated with a variety of viral infections. Patients have a typical cutaneous manifestation, low-grade fever, mild lymphadenopathy and diarrhea. Corticosteroids should be avoided as they may have an adverse effect.
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Most common malignancy associated with multiple lesions similar to the attached image is: 1Acute myelogenous leukemia
2Chronic myelogenous leukemia
3Acute lymphocytic leukemia
4Chronic lymphocytic leukemia
5Melanoma
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Most common malignancy associated with multiple lesions similar to the attached image is: 2Chronic 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.
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A newborn has a nodule over his lumbar spine. Skin biopsy reveals a lipoma. The next appropriate step is: 1Observation
2Excision of the lesion
3Genetic testing
4Imaging study
5Malignancy work up
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A newborn has a nodule over his lumbar spine. Skin biopsy reveals a lipoma. The next appropriate step is: 4Imaging 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, midline neural tube defects are uncommon, early recognition and diagnosis of a spinal dysraphism can have important implications for early 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 instances, imaging studies (MRI, CT, ultrasound) should be promptly initiated.
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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? 1AML
2CML
3CLL
4Medulloblastoma
5Pancreatic carcinoma
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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? 2CML
A patient with Neurofibromatosis type I and JXGs 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.
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Schimmelpenning-Feuerstein-Mims syndrome may be associated with which of the following: 1Osteopokilosis
2Polyostotic fibrous dysplasia
3Osteopathia striata
4Chondrodysplasia punctata
5Hypophosphatemic rickets
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Schimmelpenning-Feuerstein-Mims syndrome may be associated with which of the following: 5Hypophosphatemic rickets
Epidermal nevus syndrome, also known as Schimmelpenning-Feuerstein-Mims syndrome, may be associated with hypophospatemic, vitamin D resistant rickets.
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The differential diagnosis of zinc deficiency is least likely to include: 1Granuloma gluteale infantum
2Biotin deficiency
3Multiple carboxylase deficiency
4Cystic fibrosis
5Holocarboxylase synthetase deficiency
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The differential diagnosis of zinc deficiency is least likely to include: 1Granuloma gluteale infantum
All of the options result in an eczematous acrodermatitis enteropathica-like eruption except granuloma gluteale infantum. As the name suggests, the lesions of granuloma gluteale infantum are granulomatous.
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Multiple cylindromas are associated with: 1Myotonic dystrophy
2Cowden syndrome
3Carney complex
4Trichoepitheliomas
5Pilomatrichomas
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Multiple cylindromas are associated with: 4Trichoepitheliomas
The Brooke-Spigler syndrome is defined by the presence of multiple trichoepitheliomas and cylindromas.
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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? 1Miliaria
2Erythema toxicum neonatorum
3Transient neonatal pustular melanosis
4Congenital candidiasis
5Urticaria pigmentosa
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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? 3Transient neonatal pustular melanosis
Transient neonatal pustular melanosis typically begins with sterile pustules that leave a characteristic collarette when ruptured. The lesions heal with hyperpigmented macules.
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Which of the following may be associated? 1Paronychia
2Cleft palate
3AVM
4Seizure disorder
5Atrial septal defect
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Which of the following may be associated? 4Seizure disorder
Nevus sebaceus can very rarely be associated with multiple anomalies. Schimmelpenning syndrome can include seizure disorder, mental retardation, coloboma, as well as skeletal, cardiac and genitourinary abnormalities.
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What syndrome can accessory tragi be associated with? 1Goldenhar syndrome
2Turner syndrome
3Neurofibromatosis
4Ichthyosis
5Birt Hogg Dube
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What syndrome can accessory tragi be associated with? 1Goldenhar 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 syndromes including Goldenhar syndrome, in which epibulbar dermoid and vertebral defects are also common. An accessory 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.
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Which of the following is a potential complication of subcutaneous fat necrosis of the newborn? 1Cholestrol clefts in fat cells
2Hypercalcemia
3Acute renal failure
4Hepatitis
5Elevated uric acid levels
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Which of the following is a potential complication of subcutaneous fat necrosis of the newborn? 2Hypercalcemia
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.
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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? 1Psoriasis
2Pityriasis rosea
3Cutaneous T-cell lymphoma
4Contact dermatitis
5Lichen planus
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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? 1Psoriasis
Although the exact mechanism is not known, there is a well-known correlation between a history of upper respiratory infection secondary to group A beta-hemolytic streptococci and the subsequent 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.
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What systemic abnormalities are associated with neonatal lupus? 1Thrombocytopenia, transaminitis
2Thrombocythemia, transaminitis
3Anemia, hypocomplementemia
4Thrombocytopenia, hypocomplementemia
5Transaminitis, hypocomplementemia
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What systemic abnormalities are associated with neonatal lupus? 1Thrombocytopenia, transaminitis
Thrombocytopenia and transaminitis are associated with neonatal lupus and may indicate a worse prognosis.
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What is the best therapeutic option? 1Oral cephalexin
2Topical immune modulator
3Oral acyclovir
4Topical mupirocin
5Oral prednisone
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What is the best therapeutic option? 3Oral acyclovir
The condition shown is eczema herpeticum. The most appropriate treatment is oral acyclovir.
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A full term newborn develops erythematous, indurated plaques on the upper back. Which of the following tests should be performed? 1Calcium
2Complete blood count
3Blood culture
4Thyroid function test
5Alkaline phosphatase
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A full term newborn develops erythematous, indurated plaques on the upper back. Which of the following tests should be performed? 1Calcium
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.
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The causative agent of Roseola is: 1A ssDNA virus
2A dsDNA virus
3A ssRNA virus
4A dsRNA virus
5Streptococcus
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The causative agent of Roseola is: 2A dsDNA virus
Roseola is caused by Human Herpesvirus 6, a double stranded DNA virus.
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GLUT-1 stains placental tissue. What other tissue shows positive GLUT-1 staining? 1capillary vascular malformation
2salmon patch
3glomus tumor
4infantile hemangioma
5angel's kiss
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GLUT-1 stains placental tissue. What other tissue shows positive GLUT-1 staining? 4infantile 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.
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Which of the following is not true about Gianotti-Crosti? 1It is associated with viral infections like enterocirus, EBV, and CMV
2It typically affects children between the age of 3 months and 15 years
3It is characterized by monomorphic symmetric flat topped papules in acral areas
4It may have associated fevers, lymphadenopathy, and diarrhea
5Corticosteroids should be given to alleviate the pruritus of the lesions
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Which of the following is not true about Gianotti-Crosti? 5Corticosteroids 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.
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Which of the following clinical signs is most likely associated? 1Clitoral hypertrophy
2Neurofibromas
3Axillary freckling
4Hypertrichosis
5Alopecia
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Which of the following clinical signs is most likely associated? 1Clitoral 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 features. The genetic mutation which results in this genodermatosis is due to a post-somatic mutaion in the alpha sub-unit of stimulatory G-protein.
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The most likely etiology of Jacquets diaper dermatitis is: 1Candida
2Trichophyton rubrum
3Group A beta-hemolytic streptococcus
4Herpes simplex virus, Type 2
5Multifactorial
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The most likely etiology of Jacquets diaper dermatitis is: 5Multifactorial
Jacquets diaper dermatitis is a multifactorial process. Yeast, irritants and moisture all contribute to the occurrence of this eruption.
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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? 1MRI
2Calcium
3Skin biopsy
4Skull x-ray
5Fungal culture
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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? 4Skull 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.
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A child presents with high fever, strawberry tongue, lymphadenopathy, and a polymorphous exanthem. Approximately how many of these patients will have coronary artery aneurysms if left untreated? 1Two percent
2Five percent
3Ten percent
4Twenty-five percent
5Sixty percent
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A child presents with high fever, strawberry tongue, lymphadenopathy, and a polymorphous exanthem. Approximately how many of these patients will have coronary artery aneurysms if left untreated? 4Twenty-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.
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A 10 year old girl presents with desquamation of the fingertips. Which exam should be ordered? 1Renal ultrasound
2ASO titer
3EEG
4Eye exam
5Chest x-ray
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A 10 year old girl presents with desquamation of the fingertips. Which exam should be ordered? 2ASO 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 syndrome, and toxic shock syndrome. Thus ASO titer would be indicated in this case. Fingertip desquamation is also a manifestation of Kawasaki disease.
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Which of the following is not a major criterion for Kawasakis disease: 1Fever >5 days
2Palmoplantar erythema > desquamation
3Cardiac aneurysm
4Strawberry tongue/ red lips
5Cervical adenopathy
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Which of the following is not a major criterion for Kawasakis disease: 3Cardiac aneurysm
Cardiac aneurysm is a serious complication of Kawasakis disease. However, as the cardiovascular manifestations generally present 1 5 months after presentation, they are not criteria for diagnosis.
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The most common age group for papular-purpuric gloves and socks syndrome is: 1Newborns
2Toddlers
36-10 year olds
4Adolescents
5Elderly
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The most common age group for papular-purpuric gloves and socks syndrome is: 4Adolescents
This unique presentation of parvovirus infection typically occurs in adolescents and young adults.
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What is the most common tumor associated with this condition? 1Trichoblastoma
2Syringocystadenoma papilliferum
3Sebaceous carcinoma
4Basal cell carcinoma
5Trichoadenoma
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What is the most common tumor associated with this condition? 1Trichoblastoma
Recent reviews have identified trichoblastoma as the most common tumor arising within nevus sebaceous.
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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? 1This presentation is consistent with Jacquet's dermatitis
2CD1+, S100+ cells with comma-shaped nuclei should be seen on biopsy
3This presentation occurs when the infant is weaned off of breast milk
4An autosomal recessive defect in holocarboxylase synthetase is the cause
5Maternal-fetal transmission most likely occured in the peripartum period
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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? 2CD1+, 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.
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An infant presents with the lesion depicted in the photo. What information do you provide the parents regarding this condition? 1The lesion will go through rapid growth followed by stabilization and regression
2The lesion will respond to laser therapy
3The lesion will persist and may grow further
4The lesion will resolve with antifungal therapy
5The lesion is associated with a XO karyotype
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An infant presents with the lesion depicted in the photo. What information do you provide the parents regarding this condition? 3The lesion will persist and may grow further
The lesion represented in the photo is an arteriovenous vascular malformation. These lesions do not typically regress.
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Which finding is associated with Pachydermoperiostosis: 1Cutis verticis gyrata
2Palmoplantar hyperkeratosis
3Mucosal keratoses
4Osteopathia striata
5Cutis marmorata
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Which finding is associated with Pachydermoperiostosis: 1Cutis verticis gyrata
The scalp change seen in pachydermoperiostosis is cutis verticis gyrata.
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Which presentation of psoriasis is more common in children: 1Pustular psoriasis
2Acrodermatitis continua of Hallopeau
3Keratoderma blennorragica
4Erythrodermic psoriasis
5Guttate psoriasis
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Which presentation of psoriasis is more common in children: 5Guttate psoriasis
The majority of cases of guttate psoriasis occur in persons under the age of 30.
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What deficiency is responsible for this condition? 1Thiamine
2Niacin
3Zinc
4Vitamin C
5Vitamin A
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What deficiency is responsible for this condition? 3Zinc
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.
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All four subtypes of Phakomatosis Pigmentovascularis have which feature in common: 1Nevus spilus
2Epidermal nevi
3Nevus flammeus
4Nevus anemicus
5Dermal Melanocytosis.
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All four subtypes of Phakomatosis Pigmentovascularis have which feature in common: 3Nevus 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/capillary malformation (CM). Type I: CM + epidermal nevus Type II: CM + dermal melanocytosis +/- nevus anemicus Type III: CM + nevus spilus +/- nevus anemicus Type IV: CM + dermal melanocytosis + nevus spilus +/- nevus anemicus.
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What is the most likely diagnosis? 1Psoriasis
2Pityriasis rosea
3Cutaneous T-cell lymphoma
4Contact dermatitis
5Lichen planus
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What is the most likely diagnosis? 4Contact dermatitis
This periumbilical eruption is classic for a contact nickel allergy. The metal snaps on pants are the cause in this case.
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Which of the following is the most common long term sequelae from congenital rubella syndrome? 1Macrocephaly
2Saber shins
3Deafness
4Nystagmus
5Microcephaly
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Which of the following is the most common long term sequelae from congenital rubella syndrome? 3Deafness
Deafness may occur in up to 50% of infants with congenital rubella syndrome.
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Which of the following is a ssDNA virus: 1Herpesvirus
2Parvovirus
3Picornovirus
4Adenovirus
5Parapox
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Which of the following is a ssDNA virus: 2Parvovirus
Parvovirus is the only ssDNA virus listed.
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A newborn infant presents with ring of long, dark, coarse hair surrounding a midline scalp patch of alopecia. What is associated with this finding? 1Alopecia areata
2Ectopic brain tissue
3Thyroid disease
4Nevus sebaceus of Jadassohn
5Deafness
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A newborn infant presents with ring of long, dark, coarse hair surrounding a midline scalp patch of alopecia. What is associated with this finding? 2Ectopic brain tissue
The "hair collar sign" is associated 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.
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The association of Port-wine stains on a limb with soft tissue swelling with or without bony over growth is: 1Bannayan-Riley-Ruvalcaba syndrome
2Goldenhar's syndrome
3Sturge-Weber syndrome
4Klippel-Trenaunay syndrome
5Proteous syndrome
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The association of Port-wine stains on a limb with soft tissue swelling with or without bony over growth is: 4Klippel-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, hemihypertrophy, macrodactyly, verrucous epidermal nevus, soft-tissue subcutaneous masses, and cerebriform overgrowth of the plantar surface. Bannayan Riley Ruvalcaba syndrome may include multiple 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).
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What is the most common cause of neonatal purpura fulminans? 1Strep
2Staph
3Varicella
4Protein C deficiency
5Factor V Leiden deficiency
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What is the most common cause of neonatal purpura fulminans? 4Protein C deficiency
In children, purpura fulminans may have several causes. It is a highly characteristic feature of meningococcal septicemia and may occur as a sequel 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.
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Pachyonychia congenita type 2 is most commonly associated with which of the following: 1Increased risk of malignancy
2Poikiloderma
3Natal teeth
4Aplastic nails
5Deafness
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Pachyonychia congenita type 2 is most commonly associated with which of the following: 3Natal teeth
Pachyonychia congenita type 2 may be associated with natal teeth and steatocystoma. Pachyonychia congenita type 1 is associated with benign leukoplakia.
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In a child with zinc deficiency, yet normal or near normal zinc levels, which test could be a valuable adjunctive test? 1Magnesium
2Niacin
3Manganese
4Alkaline phosphatase
5Iron
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In a child with zinc deficiency, yet normal or near normal zinc levels, which test could be a valuable adjunctive test? 4Alkaline phosphatase
The diagnosis of zinc deficiency should be consideredi in at-risk individuals with acral or periorificial dermatitis. Chronic diaper dermatitis in an infant should lead to the evaluation for zinc deficiency. 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 adjunctive test.
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Menkes kinky hair syndrome is associated most commonly with: 1Trichorrhexsis invaginata
2Trichostasis spinulosa
3Pili multigemini
4Pili torti
5Plica neuropathica
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Menkes kinky hair syndrome is associated most commonly with: 4Pili torti
The X-linked recessive Menkes kinky hair syndrome is associated with multiple hair shaft abnormalities, most characteristically, pili torti.
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What is the most likely diagnosis: 1Epidermolysis bullosa simplex
2Epidermolysis bullosa dystrophica
3Pemphigus vulgaris
4Linear bullous IgA disease
5Facticial dermatosis
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What is the most likely diagnosis: 1Epidermolysis bullosa simplex
The bullous lesions represented are nonscarring and are associated with obvious nail dystrophy. Epidermolysis bullosa simplex, Dowling-Meara type, is the best option.
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816 activating mutation in c-kit are found most often in which subset of patients with mastocytosis? 1Adults with systemic disease refractory to imantinib
2Adults with systemic disease associated with eosinophilia
3Patients with familial history of mastocytosis
4Adults with Telangiectasia Macularis Eruptiva Perstans
5As a mosaic mutation in children with solitary cutaneous mastocytoma
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816 activating mutation in c-kit are found most often in which subset of patients with mastocytosis? 1Adults 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.
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Which of the following is most likely associated? 1No additional abnormality
2Deafness
3Coarctation of the aorta
4Bifid rib
5Alopecia
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Which of the following is most likely associated? 1No additional abnormality
The majority of patients with accessory tragii do not have any associated abnormalities. Multiple anomalies, including deafness and midline defects, have been reported but are rare.
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What is the most likely diagnosis? 1Incontinentia pigmenti
2Bullous pemphigoid of infancy
3Herpes zoster
4Disseminated herpes simplex
5Epidermolysis bullosa simplex
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What is the most likely diagnosis? 1Incontinentia pigmenti
The vesicular lesions following the lines of Blaschko are typical for incontinentia pigmenti.
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Pastias lines are characteristic for which eruption: 1Measles
2Scarlet fever
3Rubella
4Kawasakis disease
5Mumps
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Pastias lines are characteristic for which eruption: 2Scarlet fever
Pastias lines are defined as a linear accentuation of the erythematous sandpaper rash within the flexures. This is classically described with scarlet fever.
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What disorder is associated with a defect in LMX1B? 1Nail patella syndrome
2Steatocystoma
3Monilithrix
4Lhermitte-Duclos syndrome
5Chediak-Higashi syndrome
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What disorder is associated with a defect in LMX1B? 1Nail patella syndrome
A LMX1B gene defect is associated with nail patella syndrome.
Q/Q(M)-473699Report a Problem
An infant presents with the lesion depicted in the photo. Which of the following is least likely? 1Sternal clefting
2Supraumbilical raphe
3Dandy-Walker malformation
4Seizure disorder
5Congenital cataracts
Q/Q(M)-474926Report a Problem
An infant presents with the lesion depicted in the photo. Which of the following is least likely? 4Seizure disorder
Extensive facial hemangiomas are a component of the PHACES syndrome. Seizure disorder has not been described.
Q/Q(M)-474926Report a Problem
The risk of fetal death with intrauterine parvovirus infection may occur with infection in which trimester: 1First
2Second
3Third
4First, Second and Third
5None of these answers are correct
Q/Q(M)-473691Report a Problem
The risk of fetal death with intrauterine parvovirus infection may occur with infection in which trimester: 4First, Second and Third
Fetal hydrops may occur with parvovirus infection during all three trimesters although the greatest risk is during the second trimester. Congenital anomalies are not a feature.
Q/Q(M)-473691Report 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? 1Palmoplantar keratoderma
2Lymphatic malformation
3Acral edema
4Midline capillary malformation
5Angiokeratoma
Q/Q(M)-482385Report 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? 4Midline capillary malformation
This patient has Beckwith-Wiedemann syndrome, a sporadic condition that is also known as exomphalos-macroglossia-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)-482385Report a Problem
This patient had significantly elevated serum CPK. The likely diagnosis is: 1Dermatomyositis
2Lupus erythematosus
3Psoriasis
4Atopic dermatitis
5Lichen planus
Q/Q(M)-474880Report a Problem
This patient had significantly elevated serum CPK. The likely diagnosis is: 1Dermatomyositis
The answer is dermatomyositis with the heliotrope color and distribution of erythema or violaceous color. The skin over the metacarpal and proximal interphalangeal joints can become inflamed and erythematous forming Gottrons papules.
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A neonate presents with a large segmental hemangioma of the V1 distribution. What are features may be associated with this finding? 1Tram-track calcifications
2Anterior fossa defect
3Posterior fossa defect
4M-paraproteinemia
5Muscular dystrophy
Q/Q(M)-476778Report a Problem
A neonate presents with a large segmental hemangioma of the V1 distribution. What are features may be associated with this finding? 3Posterior fossa defect
PHACES syndrome consists of posterior fossa defect, hemangiomas (often segmental and large), arterial defects, cardiac defects, eye abnormaliteis, and sternal clefting. Posterior fossa defects include the Dandy-Walker malformtion.
Q/Q(M)-476778Report a Problem
POEMS syndrome is associated with which of the following: 1Premature aging
2Odontogenic cysts
3Eye abnormalities
4M protein
5Saddle nose deformity
Q/Q(M)-476263Report a Problem
POEMS syndrome is associated with which of the following: 4M protein
The acronym POEMS stands for polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes.
Q/Q(M)-476263Report a Problem
An infant with failure to thrive has multiple xanthomas on skin exam and foamy histiocytes on bone marrow biopsy. Your diagnosis is: 1Gauchers disease
2Tay-Sachs disease
3Niemann-Pick disease
4Fabrys disease
5Hunters syndrome
Q/Q(M)-477194Report a Problem
An infant with failure to thrive has multiple xanthomas on skin exam and foamy histiocytes on bone marrow biopsy. Your diagnosis is: 3Niemann-Pick disease
Niemann-Pick disease is an autosomal recessive disease caused by mutations in sphingomyelin phosphodiesterase-1. Patients with Type A Niemann-Pick disease may have xanthomas, progressive psychomotor deterioration, hepatosplenomegaly, blindness, cherry red spots, and deafness.
Q/Q(M)-477194Report 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 for based on these findings? 1Transglutaminase
2Keratin 6a/16
3Keratin 6b/17
4Keratin 1/10
5Keratin 2e
Q/Q(M)-473797Report 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 for based on these findings? 4Keratin 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 Ichthyosis bullosa of Siemens.
Q/Q(M)-473797Report 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? 1Paramyxovirus
2Togavirus
3Group A streptococcus
4HHV6
5Parvovirus
Q/Q(M)-474932Report 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? 5Parvovirus
Fifth disease is caused by Parvovirus B19. Most cases start with prodrome of fever, malaise, headache and 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)-474932Report a Problem
The disorder caused by a defect in intestinal zinc-specific transporter SLC39A4 is most likely to present: 1At birth
2Upon weaning from breast milk
3Upon weaning from formula
4In childhood
5In adulthood
Q/Q(M)-473680Report a Problem
The disorder caused by a defect in intestinal zinc-specific transporter SLC39A4 is most likely to present: 2Upon weaning from breast milk
In acrodermatitis enteropathica, there is decreased absorption of zinc from the infants gastrointestinal tract. The zinc in breast milk has greater availability than nonmaternal sources thus protecting the child from disease expression until weaning.
Q/Q(M)-473680Report a Problem
2 year old female with the skin eruption, also history of joint swelling, painful movement and mucosal lesions. What is the most likely diagnosis? 1Pustular psoriasis
2PAPA syndrome
3Familial Mediterranean fever
4DIRA
5Subcorneal pustular dermatosis
Q/Q(M)-482881Report a Problem
2 year old female with the skin eruption, also history of joint swelling, painful movement and mucosal lesions. What is the most likely diagnosis? 4DIRA
Deficiency of the IL-1 receptor antagonist(DIRA). These patients, early in their lives, present with severe pustulosis and ichthyosiform skin eruptions. Also 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)-482881Report a Problem
Osteopathia striata is found in which disorder? 1McCune-Albright syndrome
2Neurofibromatosis I
3Buschke-Ollendorff syndrome
4Gorlin's syndrome
5Focal dermal hypoplasia
Q/Q(M)-477631Report a Problem
Osteopathia striata is found in which disorder? 5Focal 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 (or Goltz syndrome). Polyostotic fibrous dysplasia with recurrent fractures is seen in McCune-Albright syndrome. Sphenoid wing dysplasia and thinning of long bone cortex is found in neurofibromatosis I. Osteopoikilosis is an asymptomatic x-ray finding in patients with Buschke-Ollendorf 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 in basal cell nevus syndrome (Gorlin's syndrome).
Q/Q(M)-477631Report a Problem
Which enanthem is most commonly seen in association with Exanthem subitum? 1Koplik spots
2Red strawberry tongue
3Chapped lips; dry, red mucosa
4Red macules and streaks on the soft palate
5Palatal erosions
Q/Q(M)-477795Report a Problem
Which enanthem is most commonly seen in association with Exanthem subitum? 4Red 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 erythematous 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 may be seen in Kawasaki's disease; palatal erosions may be seen in Papular-purpuric gloves and socks syndrome.
Q/Q(M)-477795Report a Problem
What is the most likely diagnosis? 1Traction alopecia
2Tinea capitis
3Aplasia cutis congenita
4Nevus sebaceous
5Alopecia areata
Q/Q(M)-476797Report a Problem
What is the most likely diagnosis? 3Aplasia 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 are non-inflammatory and well demarcated appear as an atrophic, membranous, ulcerated area with alopecia. The condition may be associated with other physical anomlies.
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This condition may be associated with which of the following: 1Hypercalcemia
2Hyperlipidemia
3Vitiligo
4Epidermolysis bullosa dystrophica
5Perinatal trauma
Q/Q(M)-474919Report a Problem
This condition may be associated with which of the following: 4Epidermolysis bullosa dystrophica
Aplasia cutis congenita may be associated with dystrophic epidermolysis bullosa (Bart syndrome.)
Q/Q(M)-474919Report a Problem
A 6-year-old boy presents with osteoma cutis on his face. Which of the following genes is most likely defective? 1GNAS
2NSDHL
3PTEN
4SPINK5
5SLURP1
Q/Q(M)-480188Report a Problem
A 6-year-old boy presents with osteoma cutis on his face. Which of the following genes is most likely defective? 1GNAS
Osteoma cutis in a child is associated with Albrights hereditary osteodystrophy (pseudohypoparathyroidism), which is caused by a mutation in 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, PTEN in Cowdens syndrome, SPINK5 in Nethertons syndrome and SLURP1 in Mal de Maleda.
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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? 1Erythema toxicum neonatorum
2Incontinentia pigmenti
3Urticaria pigmentosa
4Transient neonatal pustular melanosis
5Miliaria
Q/Q(M)-473677Report 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? 1Erythema toxicum neonatorum
Erythema toxicum neonatorum is a very common eruption in healthy newborns. A gram stain reveals sterile pustules containing eosinophils.
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A deficiency of the surface glycoprotein sialophorin is seen in which immunedeficient disease? 1Wiskott-Aldrich syndrome
2Chronic granulomatous disease
3Job syndrome
4Severe combined immunodeficiency syndrome
5Leiners disease
Q/Q(M)-477199Report a Problem
A deficiency of the surface glycoprotein sialophorin is seen in which immunedeficient disease? 1Wiskott-Aldrich syndrome
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 increased risk for lymphoreticular malignancy (20%).
Q/Q(M)-477199Report 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: 1Gram-positive bacteria
2Predominantly neutrophils
3Multi-nucleated giant cells
4Predominantly eosinophils
5Hyphae
Q/Q(M)-477632Report 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: 4Predominantly 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 easily. Some may have ruptured in utero, leaving pigmented macules. The pigmentation may persist for weeks to months in darkly-complexed infants. A smear of the intracorneal/subcorneal pustule will show mostly neutrophils, but eosinophils may also be present.
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A 3 month-old girl with multiple hemangiomas along her right jaw is at increased risk for: 1Bleeding complications
2Underlying bone abnormalities
3Subglottic hemangioma
4Oral obstruction
5Hearing defecits
Q/Q(M)-477334Report a Problem
A 3 month-old girl with multiple hemangiomas along her right jaw is at increased risk for: 3Subglottic hemangioma
Hemangiomas are benign vascular tumors which have proliferating phase and then a spontaneous involution phase. Depending upon the location, some hemangiomas may have more long term sequelae such as scarring or structural malformation, and rarely, consumptive coagulopathy. Infants with hemangiomas of the beard distribution should be evaluated for subglottic hemangiomas which may lead to airway obstruction.
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What the most likely diagnosis? 1Atopic dermatitis
2Lamellar ichthyosis
3Rud syndrome
4Wiskott-Aldrich syndrome
5Keratosis pilaris
Q/Q(M)-474935Report a Problem
What the most likely diagnosis? 1Atopic dermatitis
Ichythosis vulgaris and hyperlinear palms are both independent minor criteria for the diagnosis of atopic dermatitis in both children and infants.
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Rhinorrhea, condylomata lata, and mucous patches are all seen with which congenital disorder? 1Rubella
2Toxoplasmosis
3Herpes simplex virus
4Syphilis
5Human papillomavirus infection
Q/Q(M)-473686Report a Problem
Rhinorrhea, condylomata lata, and mucous patches are all seen with which congenital disorder? 4Syphilis
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)-473686Report a Problem
An infant who presents with this abnormality will often have the following: 1The lesion will be on the left-side in a segmental distribution
2Airway restriction
3Have multiple liver hemangiomas
4Most likely be a male infant
5An anterior fossa malformation
Q/Q(M)-480558Report a Problem
An infant who presents with this abnormality will often have the following: 1The 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.
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The genetic disorder depicted in this photo is caused by a mutation in: 1MASH2
2STK11
3PTEN
4Patched
5TSC1
Q/Q(M)-474931Report a Problem
The genetic disorder depicted in this photo is caused by a mutation in: 2STK11
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)-474931Report 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? 1Mupirocin ointment
2Polymyxin B ointment
3Petrolatum
4Neomycin ointment
5Silver sulfadiazine
Q/Q(M)-477328Report 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? 2Polymyxin B ointment
Mastocytosis comprises a group of diseases characterized by increased number of mast cells 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)-477328Report a Problem
Which of the following is a sign of EARLY postnatal congenital syphilis? 1Clutton's joints
2Higoumenaki's sign
3Hutchinson's teeth
4Saber shins
5Wimberger's sign
Q/Q(M)-482501Report a Problem
Which of the following is a sign of EARLY postnatal congenital syphilis? 5Wimberger's sign
Wimberger's sign is a sign of early 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 clavicular enlargement secondary to periostitis. Hutchinson's teeth are centrally notched, wide spaced, peg shaped upper incisors. Saber shins are anterior bowing of the tibia.
Q/Q(M)-482501Report 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? 1Paramyxovirus
2Togavirus
3Group A streptococcus
4HHV6
5Parvovirus
Q/Q(M)-476796Report 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? 5Parvovirus
Fifths Disease or erythema infectiosum, is caused by parvovirus B19, a single-stranded DNA virus. There are a few clinical presentations including, diffuse lacy rash on the trunk that spreads gradually toward the distal extremities, papular-pruritic "gloves-and-socks" syndrome and aplastic crisis which does not have concomitant rash.
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The lesion depicted is most often associated with: 1Polyostotic fibrous dysplasia
2Chondrodysplasia punctata
3Sphenoid wing hypoplasia
4Cleft palate
5Osteopoikilosis
Q/Q(M)-474921Report a Problem
The lesion depicted is most often associated with: 1Polyostotic fibrous dysplasia
The coast of Maine caf au lait macule is associated with McCune-Albright syndrome and polyostotic fibrous dysplasia.
Q/Q(M)-474921Report a Problem
What syndrome is the disorder shown in the photo associated with? 1Turner syndrome
2Down syndrome
3Noonan syndrome
4Griscelli syndrome
5Bloom syndrome
Q/Q(M)-474928Report a Problem
What syndrome is the disorder shown in the photo associated with? 3Noonan syndrome
The photo shows keratosis pilaris atrophicans faceii and surgically corrected ptosis. Both of these findings are associated with Noonan syndrome.
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Psammomatous melanotic schwannomas are associated with: 1Bloom syndrome
2Carney complex
3Neurofibromatosis Type 1
4Neurofibromatosis Type 2
5Tuberous sclerosis
Q/Q(M)-473702Report a Problem
Psammomatous melanotic schwannomas are associated with: 2Carney complex
Psammomatous melanotic schwannomas have been described in the Carney complex, a defect in the tumor suppressor gene, PRKAR1A.
Q/Q(M)-473702Report a Problem
What is the diagnosis? 1Unilateral laterothoracic exanthem
2Roseola
3Rubella
4Urticaria
5Contact dermatitis
Q/Q(M)-474929Report a Problem
What is the diagnosis? 1Unilateral laterothoracic exanthem
The child in the photo depicts the Statue of Liberty sign of unilateral laterothoracic exanthem.
Q/Q(M)-474929Report a Problem
Which of followings syndromes characterized by Seborrheic-like or exfoliative dermatitis 1Leiner's disease
2Hyperimmunoglobulinemia E syndrome
3Wiskott-Aldrich syndrome
4Chronic granulomatous disease
5X-linked agammaglobulinemia
Q/Q(M)-482136Report a Problem
Which of followings syndromes characterized by Seborrheic-like or exfoliative dermatitis 1Leiner's disease
Immunodeficiency disorders may be associated with a variety 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 erythroderma 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 atopic-like dermatitis.
Q/Q(M)-482136Report a Problem
Which of the following can present as collodion baby? 1Ichthyosis vulgaris
2X-linked ichthyosis
3Lamellar ichthyosis
4Bullous congenital ichthyosiform erythroderma
5Sjogren-Larsson syndrome
Q/Q(M)-477629Report a Problem
Which of the following can present as collodion baby? 3Lamellar 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.
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What is the function of the gene which is defective in ataxia-telangiectasia? 1Gap junction protein
2Cross-linking of structural proteins in the protein and lipid envelope of the upper epidermis
3Pathway of cholesterol biosynthesis
4DNA repair protein
5Tumor supressor protein
Q/Q(M)-477630Report a Problem
What is the function of the gene which is defective in ataxia-telangiectasia? 4DNA repair protein
The defective gene is ataxia-telangiectasia (Louis-Bar syndrome) is the ATM gene, which is responsible for DNA repair, especially after ionizing radiation. Tumor suppressor genes mutations are responsible for basal cell nevus syndrome, xeroderma pigmentosum, Muir-Torre syndrome, dyskeratosis congenital, Gardner syndrome, Peutz-Jeghers syndrome, Cowden syndrome, and MEN syndromes. 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)-477630Report 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 likely? 1CD1-, S100- cells with reniform nuclei
2Foamy histiocytes with Touton giant cells
3CD1+, S100+ cells with reniform nuclei
4Mixed cellular infiltrate in a ball and claw pattern
5Superficial perivascular infiltrate with mild spongiosis and neutrophil containing scale crust
Q/Q(M)-473681Report 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 likely? 3CD1+, S100+ cells with reniform nuclei
Langerhans cells are CD1 and S100 positive. The nuclei are described as kidney shaped, or reniform.
Q/Q(M)-473681Report a Problem
Posterior auricular adenopathy is a common feature of which exanthem: 1Measles
2Mumps
3Scarlet fever
4Rubella
5Erythema infectiosum
Q/Q(M)-473690Report a Problem
Posterior auricular adenopathy is a common feature of which exanthem: 4Rubella
Lymphadenopathy with 1-7 days of malaise is commonly described with rubella infection. The rose-pink macular rash follows the prodrome.
Q/Q(M)-473690Report 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? 15%
210%
325%
450%
5100%
Q/Q(M)-477634Report 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? 325%
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 and are born to mothers who are Ro/La positive. There is a 25% chance that a second child with have NLE.
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Subcutaneous fat necrosis of the newborn is usually treated with: 1Aspirin
2Topical calcipitriol
3Excision
4Observation
5Retinoids
Q/Q(M)-479620Report a Problem
Subcutaneous fat necrosis of the newborn is usually treated with: 4Observation
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)-479620Report 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? 1rubella
2rubeola
3roseola
4papular acrodermatitis of childhood
5dermatitis herpetiformis
Q/Q(M)-482829Report 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? 4papular acrodermatitis of childhood
Papular acrodermatitis of childhood (Gianotti-Crosti syndrome) typically affects children 6 months to 10 years of age, and is characterized by symmetric papules (and sometimes papulovesicles) involving the face and buttocks (four cheeks) and extensor surfaces of the upper and lower extremities. Lesions tend to be asymptomatic. Multiple infectious etiologies have been implicated, including Epstein-Barr virus (most common in the U.S.) and hepatitis B (most common in Europe). However, the eruption is most often self-limited, resolving in 4 to 8 weeks, and thus further work-up (e.g. testing for hepatitis) is indicated only if additional clinical symptoms are present.
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Hemangiomas of infancy are more likely to be: 1GLUT-1 negative
2Merosin positive but GLUT-1 negative
3Lewis Y antigen positive, merosin negative
4Glut-1 positive, merosin positive, Lewis Y antigen negative
5Glut-1 positive, merosin positive, Lewis Y antigen positive
Q/Q(M)-482865Report a Problem
Hemangiomas of infancy are more likely to be: 5Glut-1 positive, merosin positive, Lewis Y antigen positive
Hemangiomas show GLUT-1 positivity and stain positively with placental markers.
Q/Q(M)-482865Report a Problem
Late onset subungual keratotic tumors are associated with: 1Incontinentia pigmenti
2Neurofibromatosis Type 1
3Carney complex
4Cowden syndrome
5Basal cell nevus syndrome
Q/Q(M)-473704Report a Problem
Late onset subungual keratotic tumors are associated with: 1Incontinentia pigmenti
A NEMO gene defect can cause subungual keratotic growths. The typical age of presentation is early adulthood.
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Variants of xeroderma pigmentosum are due to all of the following defects except: 1Nucleotide excision repair
2Helicase
3Endonuclease
4Thymidine kinase
5Postrepliction repair
Q/Q(M)-473698Report a Problem
Variants of xeroderma pigmentosum are due to all of the following defects except: 4Thymidine kinase
The photosensitivity of xeroderma pigmentosum is caused by defect in DNA repair mechanisms. Thymidine kinase is not affected.
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Rapp-Hodgkin is caused by a defect in the following gene: 1Plakophilin
2Desmoglein 1
3Ectodysplasin A
4P63
5Connexin 30
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Rapp-Hodgkin is caused by a defect in the following gene: 4P63
Rapp-Hodgkin, along with the other ectodermal dysplasia-clefting syndromes, is reportedly caused by a defect in the p63 gene.
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Individuals with which of the following syndromes characteristically present with photosensitivity, mental retardation, a "wizened" appearance, "bird-headed" facies, and "Mickey Mouse" ears? 1Tay Syndrome
2Rothmund-Thomson Syndrome
3Hutchinson-Gilford Progeria Syndrome
4Cockayne Syndrome
5Werner Syndrome
Q/Q(M)-478060Report 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? 4Cockayne 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 abnormalities may be seen.
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A 2 year-old has a high fever for three days, as the fever breaks, a generalized rash appears on the trunk. The most likely diagnosis is: 1Measles
2Mumps
3Rubella
4Erythema infectiosum
5Roseola
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A 2 year-old has a high fever for three days, as the fever breaks, a generalized rash appears on the trunk. The most likely diagnosis is: 5Roseola
Roseola typically appears in toddlers. A high fever followed by a generalized maculopapular rash is characteristic for this HHV6 infection.
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What is the most likely diagnosis? 1Papular acrodermatitis of childhood
2Mucocutaneous lymph node syndrome
3German measles
4Letterer-Siwe disease
5Exanthem subitum
Q/Q(M)-475959Report a Problem
What is the most likely diagnosis? 1Papular 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.
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Ankyloblepharon filiforme adnatum is seen with a defect in: 1Plakophilin
2Plakoglobin
3Desmoglein
4C-kit
5p63
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Ankyloblepharon filiforme adnatum is seen with a defect in: 5p63
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.
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Which of the following is characteristic of Wooly Hair Nevus: 1Typically occurs in the 5th decade
2Ocular abnormalities may be associated
3There are no nevi associated with this hair abnormality
4Sponteneous improvement never occurs
5It is hereditary
Q/Q(M)-478697Report a Problem
Which of the following is characteristic of Wooly Hair Nevus: 5It 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)-478697Report a Problem
Which of the following should be the next step in the management of this patient? 1Barium swallow
2spine x-ray
3Cardiac evaluation
4Administration of oral antibiotics
5Pulmonary function studies
Q/Q(M)-477846Report a Problem
Which of the following should be the next step in the management of this patient? 3Cardiac 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 cleft 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 involvement and appropriate imaging and evaluation is mandated. Systemic steroids at high doses (5 mg/kg) are usually administered. If caught early, the sequelae of PHACES syndrome can be minimized.
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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? 1Corneal opacities
2Cryptorchidism
3Ectropion
4Prolonged maternal labor
5Neurologic abnormality
Q/Q(M)-474934Report 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? 3Ectropion
X-linked ichthyosis is characterized by small, dark, firmly adherent scales accentuated on the sides of the neck and trunk. The face, palms, soles, antecubital and popliteal flexures are generally spared. Associated extracutaneous findings include corneal opacities (50%), undescended testes (20%), and prolonged maternal labor (usual). Neurological or mental retardation 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.
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What is the best test to confirm a diagnosis? 1DsDNA antibody
2Anti Ro antibody
3Biopsy
4KOH
5Gram stain
Q/Q(M)-476258Report a Problem
What is the best test to confirm a diagnosis? 4KOH
The picture depicts tinea faceii. A KOH exam should be performed to look for hyphae.
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What is the best treatment option? 1Oral cephalexin
2Oral acyclovir
3Observation
4Topical tretinoin
5Topical ketoconazole
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What is the best treatment option? 4Topical tretinoin
The lesions shown in the photo are comedones of infantile acne. Topical tretinoin would be the best treatment option.
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A young boy presents with a port-wine stain, a Mongolian spot, and a nevus spilus. Which phakomatosis pigmentovascularis does he have? 1Type I
2Type II
3Type III
4Type IV
5Type V
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A young boy presents with a port-wine stain, a Mongolian spot, and a nevus spilus. Which phakomatosis pigmentovascularis does he have? 4Type IV
This patient has type IV phakomatosis pigmentovascularis characterized by 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 a nevus amenicus. Phakomatosis pigmentovascularis is thought to be caused by the "twin spot" phenomenon.
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Which of the following is the most common complication associated with cutis marmorata telangectatica congenita? 1Seizure disorder
2Atrial septal defect
3Systemic lupus erythematosus
4Hypercalcemia
5Limb hypertrophy or atrophy
Q/Q(M)-473676Report a Problem
Which of the following is the most common complication associated with cutis marmorata telangectatica congenita? 5Limb hypertrophy or atrophy
Hypertrophy or atrophy of the affected limb is the most likely consequence of cutis marmorata telangectatica congenita. Orthopedic evaluation should be a part of the patients routine management.
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Juvenile self-healing papular mucinosis is associated with which of the findings? 1Arthralgias
2Cataracts
3Type I diabetes
4Calcinosis cutis
5Xeropthalmia
Q/Q(M)-482478Report a Problem
Juvenile self-healing papular mucinosis is associated with which of the findings? 1Arthralgias
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 children. Self-healing papular mucinosis is associated with systemic findings during acute stages, including fever, arthralgias, weakness, and often carpal tunnel syndrome. A handful of cases have been reported in adults, although systemic symptoms are not typically found in this population.
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Which of the following syndromes is associated with development of giant cell epulides? 1Cherubism
2Gardner syndrome
3Cowden syndrome
4Multiple hamartoma syndrome
5CHILD syndrome
Q/Q(M)-482834Report a Problem
Which of the following syndromes is associated with development of giant cell epulides? 1Cherubism
Cherubism is an autosomally-dominant inherited disorder characterized by asymptomatic fibro-osseous 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 individuals 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 similarity to giant cell tumor of the tendon sheath.
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What is the most likely diagnosis? 1Psoriasis
2Langerhans cell histiocytosis
3Seborrheic dermatitis
4Contact dermatitis
5Perianal streptococcal disease
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What is the most likely diagnosis? 1Psoriasis
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.
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Neonatal acne is associated with species of which organism: 1Staphylococcus
2Propionibacterium
3Candida
4Malassezia
5Streptococcus
Q/Q(M)-475879Report a Problem
Neonatal acne is associated with species of which organism: 4Malassezia
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 of age, and persisting for up to 8 months. As these lesions are self-resolving, no treatment is necessary, 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 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 affected patients, though their exact role in unclear.
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Most common location of cutaneous lesions in neonatal lupus erythrematosus is 1Cheeks
2Nose
3Scalp
4Perioral
5Periorbital
Q/Q(M)-482134Report a Problem
Most common location of cutaneous lesions in neonatal lupus erythrematosus is 5Periorbital
neonatal lupus erythrematosus(NLE) is a form of subacute cutaneous lupus erythrematosus(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.
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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? 1Hypertension
2Hypercalcemia
3Hypoglycemia
4Tachycardia
5Hypernatremia
Q/Q(M)-482515Report 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? 3Hypoglycemia
In the treatment of infantile hemangiomas with propranolol, the most common serious adverse effects are bradycardia and hypotension. Other 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 risk for high-output cardiac compromise.
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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? 1Granuloma gluteale infantum
2Langerhans cell histiocytosisc.
3Seborrheic dermatitis
4Biotin deficiency
5Psoriasis
Q/Q(M)-473683Report 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? 1Granuloma gluteale infantum
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.
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The Carney complex is associated with a defect in: 1PRKAR1A
2LYST
3PTEN
4MASH2
5MLH1
Q/Q(M)-473700Report a Problem
The Carney complex is associated with a defect in: 1PRKAR1A
A PRKAR1A gene defect is associated with the Carney complex.
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Koplik spots typically appear: 1Before the exanthem
2At the same time as the exanthem
31 week after the exanthem
46 weeks after the exanthem
58 weeks after the exanthem
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oplik spots typically appear: 1Before the exanthem
The enanthem of measles precedes the morbilliform eruption.
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What is the diagnosis? 1Transient neonatal pustular melanosis
2Acropustulosis of infancy
3Neonatal cphalic pustulosis
4Miliaria
5Erythema toxicum neonatorum
Q/Q(M)-476868Report a Problem
What is the diagnosis? 3Neonatal cphalic pustulosis
In neonatal cephalic pustulosis (neonatal acne), papules and pustules are present but comedones are absent. Spontaneous remission occurs.
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What is the most likely diagnosis? 1Psoriasis
2Langerhans cell histiocytosis
3Granuloma gluteale infantum
4Contact dermatitis
5Perianal streptococcal disease
Q/Q(M)-476438Report a Problem
What is the most likely diagnosis? 5Perianal streptococcal disease
The bright red erythema of perianal streptococcal disease can also involve the creases of the groin and axillae.
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Hemangiomas of infancy are more likely to occur: 1a. In males
2b. In Asians
3c. In children who are large for gestational age
4d. In multiple gestation
5e. In post term infants
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Hemangiomas of infancy are more likely to occur: 4d. 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
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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 laboratory values is likely to be abnormal? 1Hematocrit
2Calcium
3Platelet count
4ALT
5Alkaline phosphatase
Q/Q(M)-473679Report 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 laboratory values is likely to be abnormal? 5Alkaline phosphatase
The most likely diagnosis is acrodermatitis enteropathica. Alkaline phosphatase is a zinc dependant enzyme that is decreased in response to low serum zinc levels.
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Trichothiodystrophy classically includes which of the following findings: 1Trichostasis
2Trichoschisis
3Pili torti
4Trichorrhexis invaginata
5Trichorrhexis nodosa
Q/Q(M)-476262Report a Problem
Trichothiodystrophy classically includes which of the following findings: 2Trichoschisis
Trichothiodystrophy or PIBIDS is associated with the tiger-tail abnormality of trichoschisis.
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Slapped cheeks followed by a lacy eruption on extremities: 1ssDNA virus
2dsDNA virus
3ssRNA virus
4dsRNA virus
5Streptococcus
Q/Q(M)-473692Report a Problem
Slapped cheeks followed by a lacy eruption on extremities: 1ssDNA virus
The erythematous eruption on the cheeks precedes the generalized lacy rash of Fifth disease. The causative agent is parvovirus B19, a single stranded DNA virus.
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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? 1Bullous Dermolysis of the Newborn
2Neonatal Pemphigus
3Linear IgA Bullous Dermatosis
4Junctional epidermolysis bullosa (Herlitz)
5Epidermolysis bullosa simplex
Q/Q(M)-477809Report 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? 3Linear IgA Bullous Dermatosis
Linear IgA Bullous Dermatosis is due to a target 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 in intracytoplasmic packaging or in the transport of type VII collagen within basal keratinocytes. Neonatal pemphigus is due to maternal transfer of IgG antibodies reacting 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.
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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? 1Muckle-Wells syndrome
2Deficiency of the interleukin-1-receptor antagonist (DIRA)
3Familial Mediterranean fever
4Hyper-IgD syndrome
5PAPA syndrome
Q/Q(M)-482549Report 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? 2Deficiency 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 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 the CD2 binding protein 1 (CD2BP1).
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Which of the following disorders is more likely to occur in children with chronic fecal incontinence? 1Langerhans cell histiocytosis
2Perianal pseudoverrucous papules and nodules
3Perianal streptococcal disease
4Granuloma gluteale infantum
5Seborrheic dermatitis
Q/Q(M)-473685Report a Problem
Which of the following disorders is more likely to occur in children with chronic fecal incontinence? 2Perianal pseudoverrucous papules and nodules
The warty papules of perianal pseudoverrucous papules and nodules are seen more commonly in the setting of chronic fecal incontinence.
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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? 1Tinea facei
2Acne vulgaris
3Keratosis pilaris
4Pityrosporum Folliculitis
5Diagnosis not listed
Q/Q(M)-482890Report 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? 4Pityrosporum Folliculitis
It presents as 1-2mm pruritic monomorphic papules and pustules on chest, back, upper arms, sometimes face. The presence of pruritus may help to distinguish this entity from acne. It has been associated with oral cortico-steroid use, diabetes and other immune suppressed states.
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Which of the following diseases with immunodeficiency has an increased risk of lymphoreticular malignancy? 1Wiskott-Aldrich syndrome
2Chronic granulomatous disease
3Job syndrome
4Severe combined immunodeficiency syndrome
5Leiners disease
Q/Q(M)-477213Report a Problem
Which of the following diseases with immunodeficiency has an increased risk of lymphoreticular malignancy? 1Wiskott-Aldrich syndrome
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 increased risk for lymphoreticular malignancy (20%).
Q/Q(M)-477213Report a Problem
The standard of care of patients with acute Kawasakis disease is: 1Prednisone
2Supportive care
3Aspirin and IVIG
4Penicillin
5Acetaminophen and IVIG
Q/Q(M)-477150Report a Problem
The standard of care of patients with acute Kawasakis disease is: 3Aspirin 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 cavity, acute cervical lymphadenopathy. Fever must be present, lasting more than 5 days. Treatment is aimed to prevent coronary aneurysms and myocardial infarction. Treatment for acute Kawasaki disease is intravenous immunoglobulin 2 g/kg over 10-12 hours and aspirin therapy.
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