NORD gratefully acknowledges Kristina Bundra, Pharm. D, NORD Editorial Intern, and Chester V. Oddis, MD, Professor of Medicine, University of Pittsburgh School of Medicine, for assistance in the preparation of this report.
Synonyms of Dermatomyositis
- Childhood dermatomyositis
- Idiopathic inflammatory myopathy
Subdivisions of Dermatomyositis
- Adult Dermatomyositis
- Dermatomyositis sine myositis
- Juvenile (Childhood) Dermatomyositis (JDMS)
Dermatomyositis is a type of inflammatory myopathy characterized by inflammatory and degenerative changes of the muscles and skin. Associated symptoms and physical findings may vary widely from case to case as patients may present differently. 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, 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 or develop speech and swallowing difficulties.
Skin abnormalities associated with dermatomyositis often include a distinctive reddish-purple rash (heliotrope rash) on the upper eyelid or across the cheeks and bridge of the nose in a "butterfly" distribution and on the forehead and scalp. Other characteristic rashes include scaling and redness of the knuckles, elbows, knees, and/or other extensor regions (Gottron papules and sign); an abnormal accumulation of fluid (edema) in body tissues surrounding the eyes; and/or other features.
The symptoms of childhood (juvenile) dermatomyositis (JDM) 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 JDM.
The inflammatory myopathies are a group of diseases that involve chronic muscle inflammation and weakness. They are thought to be autoimmune diseases, meaning the body’s natural defenses (antibodies, lymphocytes, etc.) against invading organisms suddenly begin to attack perfectly healthy tissue for unknown reasons, leading to inflammation or swelling.
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