National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Linear scleroderma


Other Names:
Scleroderma, linear; En coup de sabre
Categories:
This disease is grouped under:
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 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".[1] 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.[2]
Last updated: 2/10/2016

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • The Scleroderma Clinical Trials Consortium is an international organization of scleroderma clinical researchers. The consortium Web site contains a listing of active scleroderma trials, past copies of the Scleroderma Care and Research journal, and a tool for finding your nearest member institution. 

Patient Registry

  • A registry supports research by collecting of information about patients that share something in common, such as being diagnosed with Linear scleroderma. The type of data collected can vary from registry to registry and is based on the goals and purpose of that registry. Some registries collect contact information while others collect more detailed medical information. Learn more about registries.

    Registries for Linear scleroderma:
    CONQUER Registry?
     

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

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

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  1. Denton CP. Overview and classification of scleroderma disorders. UpToDate. 2016; 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.