Juvenile pityriasis rubra pilaris in Chang Gung Memorial Hospital, Taipei and Linkou: A retrospective study in the past ten years

Pityriasis rubra pilaris (PRP) is an uncommon dermatosis in childhood. Only a few long-term studies to evaluate the course and prognosis of PRP in children have been done. In this study, we retrospectively reviewed the clinical manifestation, family history, course of the disease and response to treatment in 18 patients with PRP ages 18 years old or younger. These patients were seen at Chang Gung Memorial Hospital, Taipei and Linkou, between 1997 and 2006. Of the 18 patients, 11 were male and 7 were female. The most common sites of involvement were the palms and soles, knees, elbows, and buttock. According to Griffith's classification, 4 patients were classified as type III PRP, and the other 14 patients were classified as type IV PRP. In our studies, we suggested that palmoplantar involvement could be a diagnostic feature in juvenile PRP and buttocks could be another common site of involvement in type IV PRP. All patients were treated in an outpatient setting with topical agents. In addition to topical treatment, a few patients took acitretin for short durations. In our studies, there is no conclusion on the treatment of choice for juvenile PRP, but generally speaking, the prognosis of juvenile PRP is good.