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.^[1]The disease may progress to erythroderma with distinct areas of uninvolved skin (the so-called islands of sparing).

Griffiths divided PRP into the following five types^{[2, 3]}:

Classic adult (type I)
Atypical adult (type II)
Classic juvenile (type III)
Circumscribed juvenile (type IV)
Atypical juvenile (type V)

Subsequently, an HIV-associated type (type VI) was added to this classification system.^[4] A few reports have also described pityriasis rubra pilaris associated with underlying malignancy.^{[5, 6, 7]}

Next: Pathophysiology

Pathophysiology

The etiology of PRP is unknown. A familial form of the disease exists, with an autosomal dominant inheritance pattern. Type V PRP has been linked to mutations in the gene CARD14.^{[8, 9]}Most cases of PRP are sporadic, however. It has been hypothesized that PRP may be related to an abnormal immune response to an antigenic trigger. Case reports have described PRP occurring after streptococcal infections.^[10] PRP occurring after COVOD-19 vaccination has been reported, though the precise nature of the association remains to be defined.^[11]

Next: Pathophysiology

Epidemiology

The incidence of PRP has been reported to be 1 case in 3500-5000 patients presenting to dermatologic clinics in the United States. The familial form of PRP typically begins in early childhood and has an autosomal dominant inheritance pattern. The acquired form has a bimodal age distribution, with peaks in the first and fifth decades of life, but it can begin at any age. PRP occurs equally among men and women,^[12] and persons of any race can be affected.

Next: Pathophysiology

Prognosis

Each type of PRP 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 PRP can have painful and disabling palmoplantar keratoderma. Nail dystrophy and shedding may be present. However, most of the morbidity associated with PRP is associated with the erythroderma (see Complications).

Next: Pathophysiology

Patient Education

The PRP Alliance is a nonprofit patient advocacy organization whose stated mission is to advocate for the timely and accurate diagnosis of PRP, the implementation of more effective and accessible treatment options, and an increase in PRP-specific research.

Clinical Presentation

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Media Gallery

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

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#author-disclosures{display:block}#author-disclosures.modal-layer{position:relative}#author-disclosures .modal-content{max-height:none}#author-disclosures .close-modal{display:none}

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: Biogen US (Adjudicator for study entry cutaneous lupus erythematosus); Priovant (Adjudicator for entry into a dermatomyositis study); IQVIA (Serono - adjudicator for a study of cutaneous LE) <br/>Received honoraria from UpToDate for author/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 these trust accounts for: Stocks held in various trust accounts: Allergen; Amgen; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble;; Celgene; Gilead; CVS; Walgreens; Bristol-Myers Squibb.

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.

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Pityriasis Rubra Pilaris

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