29 - Pityriasis Rubra Pilaris

Question 1

Most cases of PRP are (familial/sporadic)

Answer 1

Sporadic

Question 2

Etiology of PRP

Answer 2

Unknown

Question 3

Approximately _____% of PRP are familial

Answer 3

6.5

Question 4

Familial PRP is linked to _____ mutations

Answer 4

CARD14

Question 5

Types of PRP

Answer 5

I - Classic adult
II - Atypical adult
III - Classic juvenile
IV - Circumscribed juvenile
V - Atypical juvenile
VI - HIV- associated

Question 6

Most common subtype and occurs in more than 50% of all cases

Answer 6

I - Classic adult

Question 7

Hallmark of PRP

Answer 7

Nappes claires - sharply demarcated islands of unaffected skin

Question 8

Erythematous macules forming patches and with follicular hyperkeratotic papules on the upper half of the body; generalized erythroderma over a period of 2-3 months

Answer 8

I - Classic adult

Question 9

Ichthyosiform scaling, areas of follicular hyperkeratosis, sparseness of the scalp hair; long duration of more than 20 years

Answer 9

II - Atypical adult

Question 10

Atypical adult vs classic adult

Answer 10

Atypical adult - cephalocaudal progression is missing; less tendency to develop erythroderma

Question 11

Clinical counterpart of type I PRP in children; onset usually between the ages of 5 and 10 years

Answer 11

III - Classic juvenile

Question 12

Well-demarcated hyperkeratotic erythematous plaques on the elbows and knees; usually manifests in prepubertal children and adults; do not progress to widespread erythema

Answer 12

IV - Circumscribed juvenile

Question 13

Characterized by an early age of onset and chronic course; hyperkeratotic follicular lesions and scleroderma-like features affecting the hands and feet

Answer 13

V - atypical juvenile

Question 14

Most patients of the familial PRP belong to this category

Answer 14

V - atypical juvenile

Question 15

Additional manifestations of HIV-associated PRP

Answer 15

Acne conglobata
Hidradenitis suppurativa
Lichen spinulosus

Question 16

PRP vs psoriasis

Answer 16

PRP:
Affects the face
Typical salmon-colored appearance
Classical islands of healthy skin over the trunk
Distinct areas of follicular hyperkeratosis
Waxy palmoplantar keratoderma

Question 17

First line therapy for PRP

Answer 17

Oral retinoids

Question 18

Second most used drug for PRP

Answer 18

Methotrexate