29 - Pityriasis Rubra Pilaris Flashcards
Most cases of PRP are (familial/sporadic)
Sporadic
Etiology of PRP
Unknown
Approximately _____% of PRP are familial
6.5
Familial PRP is linked to _____ mutations
CARD14
Types of PRP
I - Classic adult II - Atypical adult III - Classic juvenile IV - Circumscribed juvenile V - Atypical juvenile VI - HIV- associated
Most common subtype and occurs in more than 50% of all cases
I - Classic adult
Hallmark of PRP
Nappes claires - sharply demarcated islands of unaffected skin
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
I - Classic adult
Ichthyosiform scaling, areas of follicular hyperkeratosis, sparseness of the scalp hair; long duration of more than 20 years
II - Atypical adult
Atypical adult vs classic adult
Atypical adult - cephalocaudal progression is missing; less tendency to develop erythroderma
Clinical counterpart of type I PRP in children; onset usually between the ages of 5 and 10 years
III - Classic juvenile
Well-demarcated hyperkeratotic erythematous plaques on the elbows and knees; usually manifests in prepubertal children and adults; do not progress to widespread erythema
IV - Circumscribed juvenile
Characterized by an early age of onset and chronic course; hyperkeratotic follicular lesions and scleroderma-like features affecting the hands and feet
V - atypical juvenile
Most patients of the familial PRP belong to this category
V - atypical juvenile
Additional manifestations of HIV-associated PRP
Acne conglobata
Hidradenitis suppurativa
Lichen spinulosus
PRP vs psoriasis
PRP:
Affects the face
Typical salmon-colored appearance
Classical islands of healthy skin over the trunk
Distinct areas of follicular hyperkeratosis
Waxy palmoplantar keratoderma
First line therapy for PRP
Oral retinoids
Second most used drug for PRP
Methotrexate
