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Pityriasis rubra pilaris

Definition

Pityriasis rubra pilaris (PRP) is a rare skin disorder that causes inflammation and scaling (exfoliation) of the skin.

Alternative Names

PRP; Pityriasis pilaris; Lichen ruber acuminatus; Devergie disease

Causes

There are several subtypes of PRP. The cause is unknown, although genetic factors and an abnormal immune response may be involved. One subtype is associated with HIV/AIDS.

Symptoms

PRP is a chronic skin condition in which orange or salmon-colored scaly patches with thick skin develop on the hands and feet.

The scaly areas may cover much of the body. Small islands of normal skin (called islands of sparing) are seen within the areas of the scaly skin. The scaly areas may be itchy. There may be changes in the nails.

PRP can be severe. Although it's not life threatening, PRP can greatly reduce quality of life and limit activities of daily living.

Exams and Tests

The health care provider will examine your skin. Diagnosis is usually made by presence of the unique skin lesions. (A lesion is an abnormal area on the skin). The provider may take samples (biopsies) of the affected skin to confirm the diagnosis and rule out conditions that may look like PRP.

Treatment

Topical creams containing urea, lactic acid, retinoids, and steroids may help. More commonly, treatment includes pills taken by mouth such as isotretinoin, acitretin, or methotrexate. Exposure to ultraviolet light (light therapy) may also help. Drugs that affect the body's immune system are currently being studied and may be effective for PRP.

Support Groups

These resources can provide more information on PRP:

When to Contact a Medical Professional

Call your provider if you develop symptoms of PRP. Also call if you have the disorder and symptoms worsen.

References

Eastham AB, Femia AN, Qureshi A, Vleugels RA. Treatment options for pityriasis rubra pilaris including biologic agents: a retrospective analysis from an academic medical center. JAMA Dermatol. 2014;150(1):92-94. PMID: 23986433 www.ncbi.nlm.nih.gov/pubmed/23986433.

James WD, Berger TG, Elston DM. Pityriasis rosea, pityriasis rubra pilaris, and other papulosquamous and hyperkeratotic diseases. In: James WD, Berger TG, Elston DM, eds. Andrews' Diseases of the Skin: Clinical Dermatology. 12th ed. Philadelphia, PA: Elsevier; 2016:chap 11.

Tobin AM, Kirby B. Pityriasis rubra pilaris. In: Lebwohl MG, Heymann WR, Berth-Jones J, Coulson I, eds. Treatment of Skin Disease: Comprehensive Therapeutic Strategies. 4th ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 187.

Review Date:5/2/2017
Reviewed By:David L. Swanson, MD, Vice Chair of Medical Dermatology, Associate Professor of Dermatology, Mayo Medical School, Scottsdale, AZ. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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Health Outcome Data

No data available for this condition/procedure.

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