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Pityriasis Rubra Pilaris

By

Shinjita Das

, MD, Harvard Medical School

Reviewed/Revised Sep 2023
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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

  • Medications applied to the skin (topical), taken by mouth (oral), or given by injection

  • 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.

Acitretin or methotrexate taken by mouth is an option when treatments applied to the skin are not helping.

Etanercept, ustekinumab, and secukinumab are biologic agents that are given by injection. They may also be used if the skin does not improve with the topical or oral therapies.

Phototherapy Phototherapy Psoriasis is a chronic, recurring disease that causes one or more raised, red patches that have silvery scales and a distinct border between the patch and normal skin. A problem with the immune... read more Phototherapy (exposure to ultraviolet light), vitamin A, and medications that weaken the immune system, such as cyclosporine, mycophenolate mofetil, azathioprine, and corticosteroids taken by mouth, have also been used.

Drugs Mentioned In This Article

Generic Name Select Brand Names
Soriatane
Jylamvo, Otrexup, Rasuvo, RediTrex, Rheumatrex, Trexall, Xatmep
Enbrel
Stelara
Cosentyx
A Mulsin, Aquasol A, Dofsol-A
Cequa, Gengraf , Neoral, Restasis, Sandimmune, SangCya, Verkazia, Vevye
CellCept, Myfortic
Azasan, Imuran
NOTE: This is the Consumer Version. DOCTORS: VIEW PROFESSIONAL VERSION
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