[Scleroderma in children].
Fifteen children, ten with localized forms of scleroderma and five with progressive systemic sclerosis (PSS), were reviewed. Age of onset was 2 to 11 years. Mean follow-up was 3.4 years. Localized forms were: "en coup de sabre" in two cases, lineal scleroderma of extremities in five, morphoea in one, and combined lesions in two patients. None of them had systemic symptoms, although two had transient arthritis. Affected skin became atrophic in three cases, "En coup de sabre" lesions were removed surgically in two cases, and there were not recurrences. The other patients continued the same or worse. All five patients with PSS showed extracutaneous symptoms. Three of them with acrosclerosis, Raynaud's phenomenon and dysphagia. One patient died because of pulmonary fibrosis in less than a year. Disease remained unchanged in the other patients. The course of disease does not seems to be affected by treatment with several drugs (asiaticoside, colchicine, diphenyldantoin).
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D012594:Scleroderma, Localized / Q000188:drug therapy / Q000473:pathology; D012595:Scleroderma, Systemic / Q000188:drug therapy / Q000473:pathology; D012867:Skin / Q000473:pathology
Esclerodermia en niños.