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Pityriasis rubra pilaris

Definition

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

Causes

There are many different types of pityriasis rubra pilaris. The cause is unknown, although genetic factors, an abnormal immune response, or vitamin A deficiency may be involved.

Symptoms

Pityriasis rubra pilaris is a chronic (life-long) skin condition in which orange-red scaly patches, and thick skin develop on the hands and feet.

The scaly areas cover much of the body. Small islands of normal skin (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.

Exams and Tests

The health care provider will examine your skin. Diagnosis is usually made because of the unique skin lesions. (A lesion is an abnormal area on the skin).

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.

When to Contact a Medical Professional

Call your health care provider if you develop symptoms of pityriasis rubra pilaris. Also call if you have the disorder and symptoms worsen.

References

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: 4/14/2015
Reviewed By: Kevin Berman, MD, PhD, Atlanta Center for Dermatologic Disease, Atlanta, GA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
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