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Pityrisis rubra pilaris as a systemic disease

par Kamarachev, Jivko;Grozdev, Ivan ;Darlenski, Razvigor;Tsankov, N.
Référence Clinics in dermatology
Publication Publié, 2019-06-01

Article révisé par les pairs

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Résumé :

Pityriasis rubra pilaris (PRP) is a rare inflammatory skin disorder of unknown etiology, initially described in 1835. It is characterized by keratotic follicular papules, well-demarcated salmon-colored erythematous scaly plaques interspersed with distinct islands of uninvolved skin, and palmoplantar keratoderma. Is PRP a systemic disease? Skin is mainly affected in PRP. Despite its clinical heterogeneity, PRP could be associated with a variety of rheumatologic, infectious, neoplastic, and other extracutaneous manifestations. We accept the hypothesis of not only an association but also a causative relation between skin and systemic manifestations with possible common underlying pathomechanisms such as systemic immunologic processes and superantigen mimicry.