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Pityriasis rubra pilaris is a rare chronic disease that causes hyperkeratotic yellowing of the palms and soles and red follicular papules that merge to form red-orange scaling plaques and confluent areas of erythema with islands of normal skin between lesions.

Pityriasis Rubra Pilaris (Hyperkeratotic Palms)

Pityriasis Rubra Pilaris (Scaling Plaques)

The cause of pityriasis rubra pilaris is unknown.

The 2 most common forms of the disease are

• Juvenile classic (characterized by autosomal dominant inheritance and childhood onset)
• Adult classic (characterized by no apparent inheritance and adult onset)

Atypical forms exist in both age groups. Sunlight can trigger a flare.

Diagnosis is by clinical appearance and may be supported by biopsy. Differential diagnosis includes seborrheic dermatitis (in children) and psoriasis when disease occurs on the scalp, elbows, and knees.

Treatment is exceedingly difficult and empiric. Disease may be ameliorated but almost never cured; classic forms of the disease resolve slowly over 3 yr, whereas nonclassic forms persist. Scaling may be improved with emollients or 12% lactic acid under occlusive dressing, followed by topical corticosteroids. Oral vitamin A may be effective. Oral retinoids or methotrexate Some Trade Names
RHEUMATREX
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is an option when a patient is resistant to topical treatment.

Last full review/revision August 2009 by Peter C. Schalock, MD

Content last modified August 2009