RDB — Summary

From the Editor…

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Summary

Pityriasis rubra pilaris (PRP) is a group of rare skin disorders that cause inflammation and shedding of the skin.  The name means scaling (pityriasis), redness (rubra) and involvement of the hair follicles (pilaris).  (1)

Typically, PRP appears first as a small spot somewhere on the body and then spreads elsewhere. (2)

It will impact different parts of the body in different ways for unpredictable periods of time. (2) The inflammation may cover the entire body or just parts of the body such as the elbows and knees, palms and soles. (3) The disease may progress and leave distinct areas of uninvolved skin, the so-called islands of sparing. (4)

The disorder may start in childhood or adulthood. It affects males and females equally. (5)

There are several types of PRP, which are classified based on age of onset, body areas affected, and whether other associated conditions are present. PRP is usually sporadic (occurring randomly) but some forms may be inherited. (6)

With an estimated 800-plus “active” patients in the U.S. and less than 1,900 in Europe, PRP is an ultra-rare skin disorder. The rarity of PRP notwithstanding, the signs and symptoms of PRP often mimic those of eczema (31.6 million patients) and psoriasis (8 million patients) (7) (8)

The treatment of PRP typically involves a combination of systemic and topical therapies combined. Topical therapies can help with the symptoms and may be enough for people with mild PRP. Topical treatments are usually combined with systemic therapy for PRP that affects a large part of the body. Most people need systemic therapy to control the condition.

Currently there are no treatments approved by the US Food and Drug Administration (FDA) or the European Medicines Agency (EMA) for use in PRP. (9)

PRP patients and their caregivers quickly learn that every case of PRP is unique. (1)

(1) British Association of Dermatologists. Pityriasis rubra pilaris leaflet. http://www.bad.org.uk/shared/get-file.ashx?id=116&itemtype=document  Updated May 2016. Accessed August 7, 2017.

(2) PRP Survival Guide. Chapter 1 — The Basics.  http://prpsurvivalguide.org/2017/05/19/yayaya/?trashed=1&ids=3 Accessed August 7, 2017.

(3) DermNet New Zealand. Pityriasis rubra pilaris.  www.dermnetnz.org/topics/pityriasis-rubra-pilaris/ Updated October, 2015. Accessed August 7, 2017.

(4)  Medscape.com. Pityriasis rubra pilaris. http://reference.medscape.com/article/1107742-overview Updated April 17, 2017. Accessed August 7, 2017.

(5) Healthline.com. Pityriasis rubra pilaris.  http://www.healthline.com/health/pityriasis-rubra-pilaris#Introduction1 Reviewed June 9, 2016. Accessed August 7, 2017.

(6)  Genetic and Rare Disease Information Center. Pityriasis rubra pilaris. https://rarediseases.info.nih.gov/diseases/7401/pityriasis-rubra-pilaris Updated August 1, 2017. Accessed August 7, 2017.

(7)  National Eczema Association. Eczema Facts. https://nationaleczema.org/research/eczema-facts/ Accessed August 7, 2017.

(8) National Psoriasis Foundation. About the National Psoriasis Foundation. https://www.psoriasis.org/about-us Accessed August 7, 2017.

(9) Genetic and Rare Diseases Information Center. Pityriasis rubra pilaris. https://rarediseases.info.nih.gov/diseases/7401/pityriasis-rubra-pilaris#ref_7262 Accessed August 7, 2017

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2017 NORD PRP Report Revision