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Pityriasis rubra pilaris

ORPHA:2897· ICD-10 L44.0

Definition

Pityriasis rubra pilaris is a rare chronic papulosquamous disorder of unknown etiology characterized by small follicular papules, scaly red-orange patches, and palmoplantar hyperkeratosis, which may progress to plaques or erythroderma. Although most of the cases are sporadic and acquired, a familial form of the disease exists.

Prevalence
1-9 / 1 000 000
Inheritance
Autosomal dominant, Not applicable
Age of onset
All ages