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Pityriasis Rubra Pilaris

  • Author: Philip D Shenefelt, MD, MS; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Feb 25, 2016
 

Background

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]

A few reports have also described pityriasis rubra pilaris associated with underlying malignancy.[7, 8]

Other Medscape pityriasis articles include Dermatologic Manifestations of Pityriasis Alba, Pityriasis Lichenoides, Pityriasis Rosea, and Pityriasis Rotunda.

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Pathophysiology

The etiology is unknown. A familial form of the disease exists, with an autosomal dominant inheritance pattern. Recently, the inherited form of PRP was linked to mutations in the gene, CARD.[9, 10] Most cases of PRP are sporadic, however.[11] 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.[12]

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Epidemiology

US frequency

The incidence of pityriasis rubra pilaris has been reported to be 1 case in 3500-5000 patients presenting to dermatologic clinics.

Race

Persons of any race can be affected.

Sex

Pityriasis rubra pilaris occurs equally among men and women.[13]

Age

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.

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Contributor Information and Disclosures
Author

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.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: XOMA; Biogen/IDEC; Novartis; Janssen Biotech, Abbvie, CSL pharma<br/>Received honoraria from UpToDate for author/editor; Received honoraria from JAMA Dermatology for associate editor and intermittent author; 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: Celgene; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen.

Chief Editor

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.

Additional Contributors

Andrea Leigh Zaenglein, MD Professor of Dermatology and Pediatrics, Department of Dermatology, Hershey Medical Center, Pennsylvania State University College of Medicine

Andrea Leigh Zaenglein, MD is a member of the following medical societies: American Academy of Dermatology, Society for Pediatric Dermatology

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.

Acknowledgements

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.

References
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Reddish orange plaques on the trunk.
Follicular hyperkeratosis seen on the dorsal aspect of the proximal phalanges.
Plantar keratoderma with an orange hue on the soles.
Palmar keratoderma with an orange hue on the palms.
 
 
 
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