By Disease Name > Pityriasis Rubra Pilaris

Pityriasis Rubra Pilaris

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•comprises more than one entity; acquired and familial forms (autosomal dominant)
•= a group of chronic disorders
•reddish-orange; islands of sparing
•palmoplantar keratoderma; keratototic follicular papules
•nails – thickened, brittle (rarely if ever pit)

 

•histology:   alternating vertical and horizontal parakeratosis
•prognosis – classic adult type 80% clear by 3 years
•treatment:  high dose vitamin A;  (Shupak says – Accutane 2mg/kg/day X 6 months); Soriatane; etanercept
•psoriasis-like;  ddx – psoriasis, phrynoderma, SCLE

 

 

Griffiths classification type I-V:

•three juvenile onset forms with poor prognosis for involution

 

type I        adult onset, classical

•~ orderly cephalocaudal progression
•spontaneous resolution in 80% of patients in 1 to 3 years
•best prognosis