D-PENICILLAMIN IN THE TREATMENT OF SCLERO DERMA EN COUP DE SABRE

A ten year old boy with linear erythema above the right eyebrow and a groove in the underlying skull bone is described. Histological examina tion of a biopsy revealed a spotty, periadnexal, perivascular, lymphoc ytic infiltrate. The papillary dermis was almost absent and the collag en fibres were slightly thickened. Based on the clinical appearance an d the histology a diagnosis of linear scleroderma (en coup de sabre) w as made. As the signs and symptoms were progressive, treatment with D- penicillamine was started. Subsequently the erythema disappeared. The shallow groove in the skull bone remained palpable. After thirteen mon ths, treatment was stopped. The patient is currently free of signs and symptoms after a follow-up period of three and a half years. We feel that the strong clinical improvement is due to treatment with D-penici llamine rather than to the natural course of the disease.