Microsoft word - the skin.doc

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.) 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 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 Question . 6. PHACES syndrome is associated with all of the following Except:
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.) The Skin - Nelson Self Assessments website 17th Edition 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.) 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.) 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 Question . 14. The treatment of choice for pediculosis capitis is:
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:
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:
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 The Skin - Nelson Self Assessments website 17th Edition 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:
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.) The Skin - Nelson Self Assessments website 17th Edition Question . 21. An 8-yr-old girl presents with large café-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 café-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?
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?
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 The Skin - Nelson Self Assessments website 17th Edition 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.) 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.) 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) The Skin - Nelson Self Assessments website 17th Edition

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