Skin

Question 1

Scalp lesion

Answer 1

Aplasia Cutis Congenita

• congenital absence of skin
• single or multiple, usually scalp involved

Question 2

Young child with short, deformed right arm and large, red, scaly skin patches

Answer 2

CHILD syndrome

congenital hemidysplasia with ichthyosiform erythroderma

Question 3

What is this?

Answer 3

Ichthyosis:

Thickened lamellar hyperkeratosis, reduced/absent granular layer

Question 4

What is this?

Answer 4

Fetal Harlequin Ichthyosis

Question 5

Infant encased in keratinous membrane sheds in 1-2 weeks

Answer 5

Collodion Baby

Question 6

17 yo w/vesicles in neck, groin & axillae

Answer 6

Hailey-Hailey disease

Question 7

What is this?

Answer 7

Restrictive dermopathy

Histology: thick, hyperkeratotic epidermis, flat rete-ridges, poorly developed adnexal structures

Question 8

What are features of focal dermal hypoplasia

Answer 8

Thin dermis w/ thin fibrils of collagen admixed with fat

X-linked dominant

Question 9

A neonate with diffuse skin blistering, respiratory failure and GI bleeding

Answer 9

Epidermolysis bullosa, junctional type - note cleavage above basement membrane

Question 10

Child with vesiculobullous acral and perioral lesions, diarrhea and alopecia

Answer 10

Acrodermatitis enteropathica - intraepidermal vesicles with psorisiform hyperplasia

Question 11

Granular IgA at tips of dermal papillae

vs

Linear IgA at basement membrane

Answer 11

Dermatitis herpetiformis - gluten, scalp & extensor surfaces

vs

Linear IgA bullous dermatosis - no gluten ass’n; tense bullae lower trunk

Both w/bullae containing neutrophils & eos

Question 12

Erythema toxicum neonatorum

Answer 12

Eosinophils in pilosebacceous units

Self-limited macular-pustular rash

Question 13

Name 2 transient dermatoses in black infants/neonates

Answer 13

Acropustulosis of infancy

Transient neonatal pustular melanosis

Pustules in both contain neutrophils

Question 14

Most common skin infection in children

Answer 14

Impetigo (staph aureus)

Question 15

Dermatitis caused by Group A strep

Answer 15

Ecthyma - ulcerative dermatitis w/dense neutrophils

Question 16

Cutaneous ulcers w/ scant neutrophils in an immunecompromised child

Answer 16

Ecthyma gangrenosum - pseudomonas; necrotizing vasculitis at base of ulcers

Question 17

Continuous granular deposits of IgG & C3 at DE junction

Answer 17

Lupus erythematousus

Question 18

Syndrome: multiple epidermal inclusion cysts

Answer 18

Gardner

Question 19

What is this?

Answer 19

Steatocystoma: note corrugated epithelium and sebaceous units

Question 20

What is this?

Answer 20

Myofibroma/myofibromatosis

• note central hemangiopericytoma-like pattern and peripheral celular proliferation of fibroblasts/myofibroblasts

Question 21

Negative for virus. What is this?

Answer 21

Urticaria pigmentosa - diffuse mast cell induced maculaopapular rash

Question 22

What is Darier sign?

Answer 22

Marked focal redness over area of trauma/scratching 2* degranulation of mast cells in systemic mastocytosis

Question 23

Name the tumors most commonly associated with this presentation

Answer 23

Blueberry muffin baby:

Neuroblastoma, leukemia, choriocarcinoma, rhabdomyosarcoma

Question 24

Name the dermal manifestations of tuberous sclerosis

Answer 24

Ashleaf spot, angiofibroma, fibroma, shagreen patch