Chapter 29 :: Pityriasis Rubra Pilaris

Question 1

Erythroderma with islands of normal skin (“nappes claires”), follicular hyperkeratosis, waxy diffuse palmoplantar keratoderma

Classic adult

Answer 1

PRP Type I

Question 2

Combination of follicular hyperkeratosis and ichthyosiform lesions on the legs, sparse scalp hair

Atypical adult

Answer 2

PRP Type II

Question 3

Similar to type I but appears in years 1 or 2 of life

Answer 3

PRP Type III

Question 4

Prepubertal children; well demarcated scaly, erythematous plaques on the elbows and knees, resembling localized psoriasis

Circumscribed juvenile

Answer 4

PRP Type IV

Question 5

Begins in first few years, accounts for most familial cases; follicular hyperkeratosis, scleroderma-like appearance of the hands and feet

Atypical juvenile, associated with CARD14

Little to no tendency to resolve spontaneously

Answer 5

PRP Type V

Question 6

Similar to type I with variable beginning; associated with acne conglobata, hidradenitis suppurativa and lichen spinulosus

Answer 6

PRP Type VI

Question 7

First Line systemic treatment

Answer 7

Retinoids (0.5-0.75 mg/kg acitretin/day)

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