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Diseases

Genetic and Rare Diseases Information Center (GARD)

Linear scleroderma


Other Names for this Disease
  • Scleroderma, linear
  • En coup de sabre
Related Diseases
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Overview

Linear scleroderma  is one sub-type of 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 predominantly on one side of the body.The face or scalp lesion is accompanied by abnormalities of the muscles, fat tissue and also the skull. In the case of facial involvement, some strips developing on the forehead may be hollow and lead to an appearance termed "en coup de sabre".[1] Because of the impact on growth, localized scleroderma can result in major facial or limb asymmetry, flexion contractures, and disability. Raynaud's phenomenon is usually absent. The exact cause is still unknown but is related to an autoimmune reaction which leads to localized overproduction of collagen. Management is symptomatic and includes immunosupressant medication. Physical therapy may be helpful to limit skin indurations and muscle retraction.[2]
Last updated: 2/10/2016

References

  1. Denton CP. Overview and classification of scleroderma disorders. UpToDate. January 9, 2015; http://www.uptodate.com/contents/overview-and-classification-of-scleroderma-disorders.
  2. Localized scleroderma. Orphanet. July, 2010; http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=12000.
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In Depth Information

  • 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.

Selected Full-Text Journal Articles

Other Names for this Disease
  • Scleroderma, linear
  • En coup de sabre
Related Diseases
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.