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- W2318083610 abstract "Background: Pityriasis rubra pilaris (PRP) is a rare dermatosis of unknown etiology. Most cases of PRP are sporadic; however, rare cases of familial PRP have been reported. Objectives: To present a case of PRP inherited in an autosomal dominant (AD) fashion and to evaluate the current literature on familial PRP and formulate a comprehensive, up-to-date summary of this rare condition. Methods: PubMed was used to conduct a search for articles pertaining to familial PRP published through May 2011. Results: The first documented case was published in 1910, and 36 subsequent familial cases of PRP have been reported. Familial PRP typically presents very early in childhood, has a gradual onset, and persists throughout life. Given the rarity of this subtype, determining the best therapy has been a challenge. In the pediatric population, a conservative treatment approach, including topical therapy, is frequently used, whereas systemic treatments are reserved for patients with a severe disease that is refractory to therapy. Conclusion: Rare cases of PRP inherited in an AD fashion have been described and tend to have a chronic clinical course and are treatment refractory. Therefore, the awareness of familial PRP is important for early and accurate diagnosis and administration of appropriate therapy." @default.
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- W2318083610 date "2013-07-01" @default.
- W2318083610 modified "2023-10-14" @default.
- W2318083610 title "Familial Pityriasis Rubra Pilaris: Case Report and Review" @default.
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- W2318083610 doi "https://doi.org/10.2310/7750.2012.12018" @default.
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