Pityriasis Rubra Pilaris
Pityriasis rubra pilaris (PRP) was first described in 1828 by Tarral and was named by Besnier in 1889. It is a chronic papulosquamous disorder of unknown etiology characterized by reddish orange scaly plaques, palmoplantar keratoderma, and keratotic follicular papules. The disease may progress to erythroderma with distinct areas of uninvolved skin, the so-called islands of sparing.
Griffiths divided pityriasis rubra pilaris into 5 categories: classic adult type, atypical adult type, classic juvenile type, circumscribed juvenile type, and atypical juvenile type. [1, 2] More recently, an HIV-associated type has been added to this classification system. [3, 4, 5, 6]
The etiology is unknown. A familial form of the disease exists, with an autosomal dominant inheritance pattern. Type V pityriasis rubra pilaris has been linked to mutations in the gene, CARD. [9, 10, 11] Most cases of pityriasis rubra pilaris are sporadic, however.  One hypothesis is that pityriasis rubra pilaris may be related to an abnormal immune response to an antigenic trigger. Case reports have described pityriasis rubra pilaris occurring after streptococcal infections. 
The incidence of pityriasis rubra pilaris has been reported to be 1 case in 3500-5000 patients presenting to dermatologic clinics.
Persons of any race can be affected.
Pityriasis rubra pilaris occurs equally among men and women. 
The familial form of pityriasis rubra pilaris typically begins in early childhood and has an autosomal dominant inheritance pattern.
The acquired form of pityriasis rubra pilaris has a bimodal age distribution, with peaks in the first and fifth decades of life, but it can begin at any age.
Each type of pityriasis rubra pilaris has its own prognosis. In general, the familial form of the disease may be persistent throughout life, and the acquired form of the disease may resolve spontaneously within 1-3 years. Patients with pityriasis rubra pilaris can have painful and disabling palmoplantar keratoderma. Nail dystrophy and shedding may be present. However, most of the morbidity associated with pityriasis rubra pilaris is associated with the erythroderma (see Complications).
The prpAlliance (http://prpalliance.com/) is a nonprofit, patient advocacy organization. Their Web site is currently offline, but it directs the reader to the PRP Community on RareConnect (see https://www.rareconnect.org/en/community/pityriasis-rubra-pilaris). Additionally, there is a PRP Facebook Support Group, founded in 2013, for patients and caregivers.
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Philip D Shenefelt, MD, MS Professor, Department of Dermatology and Cutaneous Surgery, University of South Florida College of Medicine; Past Chief, Section of Dermatology, James A Haley Veteran Affairs Medical Center
Philip D Shenefelt, MD, MS is a member of the following medical societies: American Academy of Dermatology, Florida Medical Association, Noah Worcester Dermatological Society, Society for Clinical and Experimental Hypnosis, American Contact Dermatitis Society, American Association for Physician Leadership, American Medical Association, American Society of Clinical Hypnosis
Disclosure: Nothing to disclose.
Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
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Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine
Disclosure: Received income in an amount equal to or greater than $250 from: Lilly; Amgen <br/>Received honoraria from UpToDate for author/editor; Received honoraria from JAMA Dermatology for associate editor; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for i inherited these trust accounts; for: Allergen; Celgene; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen.
Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.
Andrea Leigh Zaenglein, MD Professor of Dermatology and Pediatrics, Department of Dermatology, Hershey Medical Center, Pennsylvania State University College of Medicine
Disclosure: Received consulting fee from Galderma for consulting; Received consulting fee from Valeant for consulting; Received consulting fee from Promius for consulting; Received consulting fee from Anacor for consulting; Received grant/research funds from Stiefel for investigator; Received grant/research funds from Astellas for investigator; Received grant/research funds from Ranbaxy for other; Received consulting fee from Ranbaxy for consulting.
The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Margaret H. Rinker, MD, to the development and writing of this article.
Pityriasis Rubra Pilaris
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