[D-penicillamine in treatment of scleroderma "en coup de sabre"].

Autor: van Bergen BH; Universitätsklinik für Hautkrankheiten, Katholische Universität, Nijmegen., van Dooren-Greebe RJ, Fiselier TJ, Koopman RJ
Jazyk: němčina
Zdroj: Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete [Hautarzt] 1997 Jan; Vol. 48 (1), pp. 42-4.
DOI: 10.1007/s001050050545
Abstrakt: A ten year old boy with linear erythema above the right eyebrow and a groove in the underlying skull bone is described. Histological examination of a biopsy revealed a spotty, periadnexal, perivascular, lymphocytic infiltrate. The papillary dermis was almost absent and the collagen fibres were slightly thickened. Based on the clinical appearance and the histology a diagnosis of linear scleroderma (en coup de sabre) was 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 months, 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-penicillamine rather than to the natural course of the disease.
Databáze: MEDLINE