Other Names for this Disease
- Scleroderma, linear
- En coup de sabre
localized scleroderma, most commonly occurring in childhood. It is characterized by abnormalities of the skin and subcutaneous tissues that often follow a dermatomal distribution and that are found on one side of the body. Besides the lesion in the face or scalp there are also abnormalities of the muscles, fat tissue and skull. When the face is affected, some strips located on the forehead may be hollow and lead to an appearance termed "en coup de sabre". In most cases, Raynaud's phenomenon is absent. The exact cause is still unknown but may be related to an autoimmune reaction resulting in too much collagen. Management is symptomatic and includes immunosupressant medication. Physical therapy is helpful for the muscle retraction problems.Linear scleroderma is one sub-type of
Last updated: 2/10/2016
- Denton CP. Overview and classification of scleroderma disorders. UpToDate. January 9, 2015; http://www.uptodate.com/contents/overview-and-classification-of-scleroderma-disorders.
- Localized scleroderma. Orphanet. July, 2010; http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=12000.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Linear scleroderma. Click on the link to view a sample search on this topic.
- Select volumes of the Scleroderma Care and Research Journal can be viewed by visiting the Scleroderma Clinical Trials Consortium Web site. Click on the link above to learn more.