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Dermatomyositis
Dermatomyositis is a progressive connective tissue disorder characterized by inflammatory and degenerative changes of the muscles and skin. Associated symptoms and physical findings may vary widely from case to case. Muscle abnormalities may begin with aches and weakness of the muscles of the trunk, upper arms, hips, and thighs (proximal muscles). Muscles may be stiff, sore, and tender and, eventually, show signs of degeneration (atrophy). Affected individuals may experience difficulty in performing certain functions, such as raising their arms and/or climbing stairs. In addition, affected individuals may experience speech and swallowing difficulties.
Skin abnormalities associated with dermatomyositis often include a distinctive reddish-purple rash (heliotrope rash) on the upper eyelids, across the cheeks and bridge of the nose in a "butterfly" distribution, the forehead, or additional skin regions; scaling and degenerative (atrophic) changes of affected skin on the extending surfaces of the knuckles, elbows, knees, and/or other regions (Gottron's sign); an abnormal accumulation of fluid (edema) in body tissues surrounding the eyes; and/or other features.
The symptoms of childhood dermatomyositis are similar to those associated with the adult form of the disorder. However, onset is usually more sudden. In addition, abnormal accumulations of calcium deposits (calcifications) in muscle and skin tissues as well as involvement of the digestive (gastrointestinal [GI]) tract are more common in the childhood form of dermatomyositis.
Although the exact cause of dermatomyositis is not known, it is thought to be an autoimmune disorder.
This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html
The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.
It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report
This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.
For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org
Last Updated: 7/23/2007
Copyright 1986, 1990, 1992, 1993, 1994, 1995, 1996, 1997, 2001, 2002, 2003, 2004, 2005, 2007 National Organization for Rare Disorders, Inc.
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