CLINICAL CASES   

Giornale Italiano di Dermatologia e Venereologia 2000 August;135(4):487-9

Copyright © 2000 EDIZIONI MINERVA MEDICA

language: Italian

Linear scleroderma “en coup de sabre”: description of a case and review of the literature

Tosti A., Iorizzo M., Piraccini B. M., Lorenzi S., Vincenzi C.

Università degli Studi - Bologna Dipartimento di Medicina Clinica Specialistica e Sperimentale Sezione di Clinica Dermatologica (Direttore: Prof. C. Varotti)

Scleroderma “en coup de sabre” is a form of linear scleroderma characterized by a sclerotic band involving the face and the scalp with an area of cicatricial alopecia. It is a typical childhood disease affecting most commonly caucasian females. The cause remains unknown and the pathogenesis is still uncertain. It seems that a genetical predisposition maybe at the basis of this disease and that, a trauma or an autoimmune activation, can provoke clinical manifestations. The current theories regarding the pathogenesis of linear scleroderma, from the immune disorders to vascular abnormalities, are described. A 20-year-old patient affected by linear scleroderma “en coup de sabre”, who was successfully treated with oral calcitriol for 8 months, is presented.