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Pityriasis Rubra Pilaris


Shinjita Das

, MD, Harvard Medical School

Last review/revision Aug 2021 | Modified Sep 2022
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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.

Manifestations of Pityriasis Rubra Pilaris

Diagnosis of Pityriasis Rubra Pilaris

  • 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 of Pityriasis Rubra Pilaris

  • 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.

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

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