Linear Scleroderma in Children

BACKGROUND: Linear scleroderma is a cutaneous disease of unknown etiology. It most often affects children, who develop band-like lesions in an asymmetric distribution. The literature is reviewed as to the diagnosis, pathogenesis, and treatment of linear scleroderma. CASE REPORT: Six new cases of linear scleroderma in children are reported. Their ages ranged from 6 to 17 years, and five were girls. A 17-year-old girl had en coup de sabre. Multiple treatment modalities were used. All laboratory testing was negative. CONCLUSIONS: Linear scleroderma is a clinical diagnosis. The pathogenesis remains unknown. A consistently effective therapy has not been found, although diphenylhydantoin and ticlopidine hydrochloride should be evaluated in clinical trials.