Generalized Schamberg's disease treated with PUVA in a child

Chronic pigmented purpuric dermatitis includes various entities seen above all in adults, although they may occasionally appear in children. The various therapies available are generally unsuccessful. We report the case of a child who responded dramatically to PUVA therapy. A 10-year-old Caucasian boy of phototype III was evaluated for a one-month history of progressive eruption on the upper extremities, followed by spread to the trunk and the lower extremities. On examination, he had generalized red-yellow lesions with "cayenne-pepper" spots. Extracutaneous examination showed no abnormal features. A skin biopsy showed a superficial, perivascular mononuclear inflammatory infiltrate with extravasations of red blood cells. The laboratory findings were normal. PUVA therapy was given three times weekly. After 4 weeks of PUVA (21 J/cm2) the lesions cleared up. The patient was still free of lesions after 3-years of follow up. The clinical and histological findings in our case were consistent with Schamberg's purpura, a rare disease but nevertheless the most common form of pigmented purpura in children. Schamberg's purpura in children is a chronic disease that can persist for up to 7 years in the absence of treatment, although spontaneous remission may occur within 1 to 4 years. Phototherapy with PUVA and UVB-TL01 has been shown to be efficacious in various forms of pigmented purpuric dermatosis in adults, but only in isolated cases. To our knowledge, this is the first pediatric case of successful PUVA therapy in this disease.