Dermatomyositis

Dermatomyositis is a rare, progressive autoimmune connective tissue disorder characterized by inflammatory and degenerative changes of the muscles and skin. It is believed to affect about one in 5,000 people. The onset of the disease typically occurs in children between the ages of 5 and 15 and in adults between the ages of 40 and 60, with women developing this condition more often than men. The cause of dermatomyositis is unknown.

Cutaneous or skin-predominant dermatomyositis causes progressive and painful skin abnormalities including a distinctive reddish-purple rash on the upper eyelids, across the cheeks and bridge of the nose in a "butterfly" distribution; scaling and degenerative changes of affected skin on the knuckles, elbows, knees, and other regions; and an abnormal accumulation of fluid in body tissues surrounding the eyes. Skin-predominant dermatomyositis results in severe morbidity, significantly impacts regular daily function and quality of life, can eventually lead to muscle or lung inflammation, and has been associated with an increased risk of cancer. There are currently no approved therapies for DM.


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