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Abstract
We report a retrospective study of 14 patients with pityriasis rubra pilaris (PRP) referred to our department from 1973 to 1997. Four of them were children. The purpose of this study was to determine their response to several treatments in order to be able to define the best management of PRP. Nine patients were treated with retinoids (etretinate or acitretin) for an average period of 18.8 months. This treatment achieved in seven of them a partial or complete clearing without major side-effects. All five patients treated with methotrexate were adults. The mean duration of treatment was 12.1 months. All the methotrexate-treated patients improved, even though two of them had not responded to retinoids. The other therapies, including systemic steroids, topical treatments and psoralens-UVA therapy, produced inconsistent results. Our results suggest that retinoids should be considered a first-line treatment at an initial dosage of 0.5 mg/kg per day. Methotrexate has to be the second-line treatment, at an initial dosage between IS and 25 mg/week, in cases of inefficacy of retinoids, or the first alternative treatment when retinoids are contraindicated, especially in older patients.