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Pityriasis rubra pilaris

Andreas Katsambas, MD
Clio Dessinioti, MD
Section Editor
Kristina Callis Duffin, MD
Deputy Editor
Abena O Ofori, MD


Pityriasis rubra pilaris (PRP) is a rare papulosquamous inflammatory dermatosis of unknown origin and considerable clinical heterogeneity (table 1). The cardinal clinical features of PRP are hyperkeratotic follicular papules, orange-red scaling plaques, and palmoplantar hyperkeratosis (picture 1A-F). Well-demarcated areas of spared skin (also known as "islands of sparing") are a frequent finding in patients with generalized involvement. Erythroderma may occur as a complication of PRP.

The treatment of PRP can be challenging. Systemic retinoids and methotrexate are the most commonly employed therapies, and limited data suggest that biologic TNF-alpha inhibitors may be effective. Multiple other therapies have also been utilized for PRP.

The pathogenesis, diagnosis, and treatment of PRP will be reviewed here.


A disorder with features consistent with pityriasis rubra pilaris (PRP) was first reported in 1828 as a variant of psoriasis, and in 1856, "pityriasis pilaris" was identified as an independent disease [1]. The term "pityriasis rubra pilaris" was coined in 1889, and remains the accepted terminology for this disease [2].


The incidence and prevalence of pityriasis rubra pilaris (PRP) is not precisely known. The disease has been estimated to account for 1 in 5000 new patients who present with skin disease in Great Britain [3]. PRP occurs in individuals of all racial backgrounds and appears to affect both genders equally [3].


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Literature review current through: Sep 2016. | This topic last updated: Apr 6, 2016.
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