Pityriasis rubra pilaris (PRP) is a rare papulosquamous disorder with a clinically heterogeneous presentation. It is characterized by widespread follicular keratotic papules, diffuse erythema with classic islands of sparing, and palmoplantar keratoderma. PRP has been classified into 6 subsets, types I through VI, differentiated by age at onset, disease duration, and clinical features.
1-3 Familial cases of PRP have been associated with germline gain-of-function variations (formerly
mutations) in the caspase recruitment domain family, member 14 (
CARD14).
4 PRP symptoms have major effects on quality of life,
5 and more than half of patients diagnosed with PRP report comorbid depressive symptoms.
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