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Pityriasis Rubra Pilaris

By

Shinjita Das

, MD, Harvard Medical School

Last full review/revision Aug 2021| Content last modified Aug 2021
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Topic Resources

Pityriasis rubra pilaris is a rare chronic skin disorder that causes thickening and yellowing of the skin, including the palms and soles, and red, raised bumps. The bumps may merge together to form red-orange, scaly patches (plaques) with areas of normal skin in-between.

The cause of pityriasis rubra pilaris is unknown.

The two most common forms of the disorder are

  • Juvenile classic

  • Adult classic

The juvenile classic form of pityriasis rubra pilaris is inherited and begins in childhood. The adult classic form of pityriasis rubra pilaris does not seem to be inherited and begins in adulthood.

Other nonclassic forms exist in both age groups. Sunlight, human immunodeficiency virus (HIV) infection or another infection, minor trauma, or an autoimmune disorder may trigger a flare-up.

Symptoms of Pityriasis Rubra Pilaris

Symptoms of pityriasis rubra pilaris include pink, red, or orange-red scaly patches that can develop on any part of the body and are usually itchy. The skin can become thick and yellow.

Diagnosis of Pityriasis Rubra Pilaris

Treatment of Pityriasis Rubra Pilaris

  • Drugs applied to the skin

  • Drugs taken by mouth

  • Phototherapy

Treatment of pityriasis rubra pilaris is very difficult. Symptoms of the disorder may be lessened, but the disorder itself can almost never be cured. Classic forms of the disorder go away slowly over 3 years, whereas nonclassic forms last much longer.

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