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Pityriasis Rubra Pilaris
Pityriasis rubra pilaris is a rare chronic disorder 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.
The cause of pityriasis rubra pilaris is unknown.
The 2 most common forms of the disorder are
Atypical (nonclassic) forms exist in both age groups. Sunlight, HIV or another infection, minor trauma, or an autoimmune disorder may trigger a flare-up.
Treatment is exceedingly difficult and empiric. The disorder may be ameliorated but almost never cured; classic forms of the disorder resolve slowly over 3 yr, 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. Other less common oral drugs include cyclosporine, azathioprine, and corticosteroids.
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