Merck Manual

Please confirm that you are a health care professional

Loading

Pityriasis Rubra Pilaris

By

Shinjita Das

, MD, Harvard Medical School

Last full review/revision Apr 2020| Content last modified Apr 2020
Click here for Patient Education
NOTE: This is the Professional Version. CONSUMERS: Click here for the Consumer Version
Topic Resources

Pityriasis rubra pilaris is a rare chronic disorder that causes hyperkeratotic yellowing of the skin, including the trunk, extremities, and, particularly, the palms and soles. Red follicular papules typically merge to form red-orange scaling plaques and confluent areas of erythema with islands of normal skin between lesions. Treatment is aimed at relieving symptoms.

The cause of pityriasis rubra pilaris is unknown.

The 2 most common forms of the disorder are

  • Juvenile classic (characterized by autosomal dominant inheritance and childhood onset)

  • Adult classic (characterized by no apparent inheritance and adult onset)

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

Diagnosis

  • Clinical evaluation

  • Sometimes biopsy

Diagnosis of pityriasis rubra pilaris is by clinical appearance and may be supported by biopsy.

Biopsy is done when the clinical diagnosis is unclear (eg, when the patient is erythrodermic at presentation).

Differential diagnosis of pityriasis rubra pilaris includes the following:

Treatment

  • Symptom relief (eg, with emollients, topical lactic acid and topical corticosteroids, or oral retinoids)

Treatment of pityriasis rubra pilaris is exceedingly difficult and empiric. The disorder may be ameliorated but almost never cured; classic forms of the disorder resolve slowly over 3 years, whereas nonclassic forms persist. Scaling may be reduced with emollients or 12% lactic acid under occlusive dressing, followed by topical corticosteroids. Oral vitamin A may be effective. Oral acitretin (a retinoid) or methotrexate is an option when a patient is resistant to topical treatment.

Phototherapy, immunomodulatory agents (biologics), cyclosporine, mycophenolate mofetil, azathioprine, and corticosteroids have also been used (1).

Treatment reference

  • 1. Eastham AB, Femia A, Qureshi A, et al: Treatment options for pityriasis rubra pilaris including biologic agents: A retrospective analysis from an academic medical center. JAMA Dermatol 150(1):92–94, 2014. doi:10.1001/jamadermatol.2013.4773.

Click here for Patient Education
NOTE: This is the Professional Version. CONSUMERS: Click here for the Consumer Version
Professionals also read

Also of Interest

SOCIAL MEDIA

TOP