The Skin

Nelson Self Assessments website 17th Edition

Question . 1. A 10-day-old infant develops an annular, scaly, erythematous eruption

on the forehead. He was born at term after an uncomplicated delivery, with a past
medical history significant only for hyperbilirubinemia treated with phototherapy.
Results of a KOH examination are negative. His mother has intermittent episodes of
joint pain. The most appropriate next step in diagnosis is:

A. Liver function tests

B. Fungal culture
C. Skin biopsy
D. Autoimmune work-up
Explanation: This vasculitic-type rash is compatible with neonatal lupus.
Maternal antibodies cross the placenta (IgG) and produce autoimmune
phenomena in the baby. These include hemolytic anemia, thrombocytopenia,
neutropenia, cutaneous vasculitis, and congenital heart block. All
manifestations except heart block resolve with time. E would also be correct.
Neonatal lupus should be considered diagnostically based on the age at
presentation, morphology of rash, and history of UV light exposure. NLE
babies can have ECG abnormalities, but only 1 in 10 has both ECG changes
and a rash. Therefore, because a rash is present, the next best step
diagnostically would be serum assay for autoantibodies, which should be
diagnostic. (See Chapter 637 in Nelson Textbook of Pediatrics, 17th ed.)
E. Electrocardiogram

Question . 2. A 2-mo-old girl has a rapidly growing hemangioma involving the

perioral region bilaterally, with symmetric extension onto the chin and anterior neck.
She was born 8 wk premature with an otherwise benign medical history, although her
parents wonder if she may be developing asthma. The most appropriate next step in
patient management is:

A. Laser surgery
B. Skin biopsy
C. Reassurance that hemangiomas will go away on their own
D. Inhaler prescription
E. ENT evaluation
Explanation: A beard area hemangioma may herald a subglottic lesion that
could be life-threatening and may be the source of the perceived asthma
symptoms. (See Chapter 640 in Nelson Textbook of Pediatrics, 17th ed.)

The Skin - Nelson Self Assessments website 17th Edition 1

Question . 3. A previously healthy 1-yr-old boy presents to the emergency
department with seizures. He has had no recent illnesses or high fevers, and has had
no ingestion history and is taking no new medications. On examination he is alert,
interactive, and afebrile with normal vital signs. His skin examination is significant
for confetti-type white spots on his right lower extremity his parents state is a
"birthmark." The most likely diagnosis is:

A. Neurofibromatosis type 1
B. Tuberous sclerosis
Explanation: These hypopigmented macules resemble ash leaves and are best
identified with a Wood lamp. (See Chapter 643 in Nelson Pediatrics, 17th ed.)
C. Waardenburg syndrome
D. McCune-Albright syndrome
E. Vitiligo

Question . 4. A 6-mo-old infant is presented for evaluation of a tuft of thick black

hair located on the sacrum. The child's mother states that the lesion has been present
since birth and seems to be asymptomatic, and the child is otherwise healthy. The
most appropriate next step in management is:

A. Reassurance that the lesion will resolve spontaneously in time

B. MRI of the spinal cord
Explanation: Lumbar or sacral hair tufts or hemangiomas may be associated
with spinal dysraphisms, such as a tethered cord. In younger infants, an
ultrasound study is a useful examination, but as infants grow older, MRI is
indicated. (See Chapter 637 in Nelson Textbook of Pediatrics, 17th ed.)
C. Referral to a plastic surgeon for excision
D. Counseling of the parents regarding melanoma risk
E. Watchful waiting

Question . 5. Hypohidrotic ectodermal dysplasia is associated with all of the

following Except:

A. Partial or complete absence of sweat glands

B. Hypertrichosis
Explanation: Patients usually have hypotrichosis. Because of the inability to
sweat, these children often have many episodes of high fever; therefore, this
entity is included in the differential diagnosis of fever of unknown origin. (See
Chapter 639 in Nelson Textbook of Pediatrics, 17th ed.)
C. Anomalous detention
D. X-linked recessive inheritance
E. Mutations of ectodysplasin

The Skin - Nelson Self Assessments website 17th Edition 2

Question . 6. PHACES syndrome is associated with all of the following Except:

A. Phalangeal dysplasia
Explanation: PHACES is associated with posterior fossa defects such as
Dandy-Walker cysts or cerebellar hypoplasia. Strokes are a major source of
morbidity. (See Chapter 640 in Nelson Textbook of Pediatrics, 17th ed.)
B. Facial hemangiomas
C. Aneurysms and stroke
D. Coarctation of the aorta
E. Ocular anomalies
F. Sternal anomalies

Question . 7. Kasabach-Merritt syndrome is seen most often with:

A. Hemangiomas
B. Nevus flammeus
C. Tufted angioma
Explanation: This syndrome, associated with thrombocytopenia,
microangiopathic hemolytic anemia, and consumption coagulopathy, is due to
a tufted angioma or a kaposiform hemangioendothelioma. (See Chapter 640 in
Nelson Textbook of Pediatrics, 17th ed.)
D. Port-wine stain
E. Capillary hemangiomas

Question . 8. Nevus sebaceus (Jadassohn sebaceous nevus) is associated with all of

the following Except:

A. Yellow-orange-appearing plaque
B. Cardiac failure
Explanation: These initially benign-appearing plaques, usually seen on the
head or neck of infants, may develop into benign or malignant tumors in
adulthood. They should be resected before adulthood. (See Chapter 641 in
Nelson Textbook of Pediatrics, 17th ed.)
C. Risk of benign tumors
D. Risk of malignancy
E. Verrucous changes during adolescence

Question . 9. Recurrent erythema multiforme is associated with:

A. Human herpesvirus 6
B. Anxiety
C. Herpes simplex
Explanation: Both HSV herpes labialis and HSV herpes genitalis have been
implicated in the majority of cases of recurrent EM. (See Chapter 644 in
Nelson Textbook of Pediatrics, 17th ed.)
D. Psychosis
E. Mononucleosis

The Skin - Nelson Self Assessments website 17th Edition 3

Question . 10. Complications of Stevens-Johnson syndrome include all of the following Except:

A. Bullae and exfoliation

B. Corneal ulceration
C. Polyarthritis
D. Osteomyelitis
Explanation: Secondary local bacterial infections of the skin or sepsis may
occur. Antibiotics are indicated for proven infections or if there is a high index
of suspicion for infection. Steroids may increase the risk of infection.
Treatment for patients with Stevens-Johnson syndrome is similar to that for
patients with extensive burns. (See Chapter 644 in Nelson Pediatrics, 17th ed.)
E. Myocarditis
F. Hepatitis

Question . 11. A 15-yr-old girl who had new-onset seizures was started on
carbamazepine 4 wk ago. She now has a diffuse erythematous macular rash, fever,
lymphadenopathy, eosinophilic leukocytosis, and elevated values on liver function
tests. The most likely cause of these abnormalities is:

A. Erythema multiforme
B. Stevens-Johnson syndrome
C. Hypersensitivity syndrome
Explanation: The rash may look like any of the other choices (more often
choices B and E). However, hepatitis and lymphadenopathy are uncommon
findings with choice B or E. Hypersensitivity reactions to anticonvulsant drugs
occur 1-3 mo after initiation of therapy and probably represent a cell-mediated
immunologic reaction to the drug or a drug metabolite. (See Chapter 644)
D. Serum sickness
E. Toxic epidermal necrolysis

Question . 12. Onychomycosis is associated with all of the following Except:

A. T. rubrum invasive infection

B. T. mentagrophytes superficial infection
C. A good response to treatment with oral terbinafine
D. A good response to topical ketoconazole
Explanation: Topical therapy is of little value in fungal infections of the nail.
Prolonged oral therapy is needed to penetrate the growing nail, making it
resistant to fungal infections. (See Chapter 656 in Nelson Textbook of
Pediatrics, 17th ed.)
E. A good response to oral itraconazole

Question . 13. The treatment of choice for scabies is:

A. Crotamiton lotion
B. Permethrin 5% cream
Explanation: One percent lindane cream (or lotion) is also recommended but is
potentially neurotoxic. (See Chapter 658 in Nelson Pediatrics, 17th ed.)
C. Ultraviolet light
D. Salicylate baths
E. Topical gentamicin

The Skin - Nelson Self Assessments website 17th Edition 4

Question . 14. The treatment of choice for pediculosis capitis is:

A. Application of 0.5% malathion

Explanation: In addition, 1% permethrin cream is also a treatment of choice.
Malathion is not indicated in infants or neonates. Permethrin plus oral
trimethoprim-sulfamethoxazole is a promising therapy for hard-to-treat cases.
(See Chapter 658 in Nelson Textbook of Pediatrics, 17th ed.)
B. Application of 10% permethrin ointment
C. Brushing and combing
D. Hydrogen peroxide
E. Selenium-based shampoo

Question . 15. Giant congenital pigmented nevi are associated with all of the
following Except:

A. An incidence of less than 1 case in 20,000 births

B. Leptomeningeal involvement
C. Malignant melanoma
D. Hydrocephalus
E. Male predominance
Explanation: Giant pigmented nevi are difficult-to-manage lesions that affect
males and females with equal frequency. (See Chapter 641 in Nelson
Textbook of Pediatrics, 17th ed.)

Question . 16. Incontinentia pigmenti is associated with all of the following Except:

A. Lethality in females
Explanation: Incontinentia pigmenti is X-linked dominant and is lethal in
males during fetal life. (See Chapter 642 in Nelson Textbook of Pediatrics,
17th ed.)
B. Erythematous linear streaks and vesicles
C. Alopecia
D. Hypodontia
E. Microphthalmos
F. Seizures

Question . 17. Stevens-Johnson syndrome (erythema multiforme major) is associated

with all of the following Except:

A. A good response to prednisone

Explanation: There is no agreed-on indication for systemic steroids in Stevens-
Johnson syndrome. Topical steroids are indicated for ocular involvement to
prevent scarring. (See Chapter 644 in Nelson Textbook of Pediatrics, 17th ed.)
B. Involvement of two mucous membranes
C. Esophageal stricture
D. Corneal scarring
E. Infectious causes
F. Drug-related causes

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Question . 18. A well-appearing newborn has a lacy, reticulated, red and/or blue
cutaneous pattern over most of the body, which is prominent when the neonate is in a
cool environment. The correct diagnosis is:

A. Mongolian spots
B. Salmon patch
C. Cutis marmorata
Explanation: Cutis marmorata is an accentuated physiologic vasomotor
response that disappears with increasing age during the first year of life. (See
Chapter 637 in Nelson Textbook of Pediatrics, 17th ed.)
D. Harlequin color change
E. Erythema toxicum

Question . 19. A 2-day-old well-appearing full-term white neonate experiences

multiple firm, yellow-white, 1- to 2-mm papules or pustules with a surrounding
erythematous flare on the trunk. Wright stain of the lesions shows numerous
eosinophils. The most likely diagnosis is:

A. Erythema toxicum
Explanation: Erythema toxicum is a benign, self-limited, evanescent eruption
that occurs in approximately 50% of full-term infants; preterm infants are
affected less commonly. The lesions are firm, yellow-white, 1- to 2-mm
papules or pustules with a surrounding erythematous flare. Lesions may be
sparse or numerous and clustered in several sites or widely dispersed over
much of the body surface. Palms and soles are usually spared. Peak incidence
is on the second day of life, but new lesions may erupt during the first few
days as the rash waxes and wanes. (See Chapter 637 in Nelson Pedia, 17th ed.)
B. Pustular melanosis
C. Acropustulosis
D. Eosinophilic pustular folliculitis
E. Herpes simplex virus infection

Question . 20. The recommended therapy for port-wine nevus is:

A. Tattooing
B. Excision and skin grafting
C. Cryosurgery
D. Laser therapy
Explanation: The most effective treatment for port-wine stains is the
flashlamp-pumped-pulsed dye laser. This therapy is targeted at the lesion and
avoids thermal injury to the surrounding normal tissue. Alternative therapies
include masking with cosmetics, cryosurgery, excision, grafting, and tattooing.
(See Chapter 640 in Nelson Textbook of Pediatrics, 17th ed.)
E. Interferon-

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Question . 21. An 8-yr-old girl presents with large caf-au-lait spots with irregular
borders and precocious puberty. X-ray shows polyostotic fibrous dysplasia of bone.
The most likely diagnosis is:

A. Neurofibromatosis
B. McCune-Albright syndrome
Explanation: Large, often asymmetric caf-au-lait spots with irregular borders
are characteristic of McCune-Albright syndrome. This disorder also includes
polyostotic fibrous dysplasia of bone, leading to pathologic fractures;
precocious puberty; and numerous hyperfunctional endocrinopathies. (See
Chapter 642 in Nelson Textbook of Pediatrics, 17th ed.)
C. Tuberous sclerosis
D. Maffucci syndrome
E. Normal child

Question . 22. A black teenager presents with a sharply demarcated, dense, firm,
rubbery growth on the face at the site of a previous smaller laceration that was
incurred long ago. Which of the following is the most likely diagnosis?

A. Granuloma annulare
B. Keloid
Explanation: Keloids are usually induced by trauma and commonly follow ear
piercing, burns, scalds, and surgical procedures. Certain individuals, especially
blacks, seem predisposed to keloid formation. In both keloids and
hypertrophic scars, new collagen forms over a much longer period than in
wounds that heal normally. Unlike keloids, hypertrophic scars remain
confined to the site of injury and gradually involute over time. (See Chapter
649 in Nelson Textbook of Pediatrics, 17th ed.)
C. Necrobiosis lipoidica
D. Anetoderma
E. Mastocytosis

Question . 23. A young child has unusual skin hyperelasticity and joint hypermobility
of gradual onset. The skin snaps back into place when pulled. Which of the following
is the most likely diagnosis?

A. Cutis laxa
B. Ehlers-Danlos syndrome
Explanation: Ehlers-Danlos syndrome is a group of genetically heterogeneous
connective tissue disorders. Affected children appear normal at birth, but skin
hyperelasticity, fragility of the skin and blood vessels, and joint hypermobility
develop over time. The essential defect is a quantitative deficiency of
collagen. Ehlers-Danlos syndrome has been classified into 10 clinical forms.
(See Chapter 649 in Nelson Textbook of Pediatrics, 17th ed.)
C. Anetoderma
D. Pseudoxanthoma elasticum
E. Elastosis perforans serpingosa

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Question . 24. A child presents with multiple, discrete, skin-colored, 1- to 3-mm
dome-shaped smooth papules on the face with central umbilication. The most likely
diagnosis is:

A. Verruca vulgaris (common warts)

B. Verruca plana (flat warts)
C. Condylomata acuminata
D. Molluscum contagiosum
Explanation: The lesions of molluscum contagiosum are discrete, pearly, skin-
colored, dome-shaped, smooth papules that may range in size from 1 to 5 mm.
They typically have a central umbilication from which a plug of cheesy
material can be expressed. Papules of molluscum contagiosum may occur
anywhere on the body, but the face, eyelids, neck, axillae, and thighs are areas
of predilection. (See Chapter 657 in Nelson Textbook of Pediatrics, 17th ed.)
E. Keloid

Question . 25. Which of the following is the most serious adverse effect associated
with use of isotretinoin?
A. Hepatitis
B. Cardiomyopathy
C. Pseudotumor cerebri
D. Teratogenicity
Explanation: Isotretinoin has many adverse effects. It is teratogenic and is
contraindicated for use during pregnancy; pregnancy should be avoided for 1
mo after discontinuation of therapy. Most patients experience cheilitis, xerosis,
periodic epistaxis, and blepharoconjunctivitis. Increased serum triglyceride
and cholesterol levels are also common. Less common adverse effects include
arthralgias, myalgias, depression, temporary thinning of the hair, paronychia,
increased susceptibility to sunburn, formation of pyogenic granulomas, and
colonization of the skin with Staphylococcus aureus leading to impetigo,
secondarily infected dermatitis, and scalp folliculitis. (See Chapter 659 in
Nelson Textbook of Pediatrics, 17th ed.)
E. Carcinogenicity

Question . 26. A 2-yr-old child presents with symmetric psoriasiform skin lesions
distributed in the perioral, acral, and perineal areas and on the cheeks, knees, and
elbows. There is mild alopecia and a history of chronic diarrhea. The most likely
diagnosis is:
A. Psoriasis
B. Juvenile xanthogranuloma
C. Acrodermatitis enteropathica
Explanation: Acrodermatitis enteropathica is caused by an inability to absorb
sufficient zinc from the diet. Initial signs and symptoms occur during infancy
and consist of a rash in the perioral, acral, and perineal areas and on the
cheeks, knees, and elbows. There is often alopecia and chronic diarrhea. Some
patients with cystic fibrosis present with a similar rash. Biotinidase deficiency
should be in the differential diagnosis. Treatment of acrodermatitis
enteropathica is with oral zinc compounds. (See Chapter 661 in Nelson)
D. Scurvy
E. Pellagra

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