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

Title: [D-penicillamine in treatment of scleroderma "en coup de sabre"].
Transliterated Title: D-Penizillamin in der Behandlung von Sklerodermie "en coup de sabre".
Authors: van Bergen BH; Universitätsklinik für Hautkrankheiten, Katholische Universität, Nijmegen.; van Dooren-Greebe RJ; Fiselier TJ; Koopman RJ
Source: Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete [Hautarzt] 1997 Jan; Vol. 48 (1), pp. 42-4.
Publication Type: Case Reports; English Abstract; Journal Article
Language: German
Journal Info: Publisher: Springer Verlag Country of Publication: Germany NLM ID: 0372755 Publication Model: Print Cited Medium: Print ISSN: 0017-8470 (Print) Linking ISSN: 00178470 NLM ISO Abbreviation: Hautarzt Subsets: MEDLINE
Imprint Name(s): Original Publication: Berlin : Springer Verlag
MeSH Terms: Antirheumatic Agents/*administration & dosage ; Penicillamine/*administration & dosage ; Scleroderma, Localized/*drug therapy; Scleroderma, Localized/diagnosis ; Adolescent ; Follow-Up Studies ; Forehead ; Humans ; Male
Abstract: 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.
Substance Nomenclature: 0 (Antirheumatic Agents); GNN1DV99GX (Penicillamine)
Entry Date(s): Date Created: 19970101 Date Completed: 19970430 Latest Revision: 20190920
Update Code: 20260130
DOI: 10.1007/s001050050545
PMID: 9132387
Database: MEDLINE

Case Reports; English Abstract; Journal Article