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.
|Translated title of the contribution||Linear scleroderma 'en coup de sabre': Description of a case and review of the literature|
|Number of pages||3|
|Journal||Giornale Italiano di Dermatologia e Venereologia|
|State||Published - Jan 1 2000|
- Calcitriol therapeutic use
- Scleroderma, circums ribed
ASJC Scopus subject areas