Dermatomyositis is an autoimmune disease that manifests with a rash and muscle weakness. Despite these prosaic symptoms, persons who are not treated for the condition have a very poor prognosis. The disease has only been treatable in modern times with steroids, chemotherapy agents and immunoglobulin.
The causes of the condition are poorly understood. Because it often manifests after a pregnancy, it was thought to have been caused by an autoimmune reaction to fetal cells that continued until the antibodies started attacking the cells in the skin and muscles. In most cases, the immune response spreads to other connective tissues. However, in some patients, the condition can be triggered by an infection.
Dermatomyositis is difficult to diagnose. Only a muscle biopsy will provide a definitive answer. The disease may present with mild or severe symptoms, including pain. In most cases, the symptoms typical of dermatomyositis are usually associated with autoimmune disorders that affect the lymphocytes. In addition, in about 10% of cases, the condition presents with no skin or muscle symptoms whatsoever.
Prognosis is good with early treatment, but even with treatment, the survival rate after ten years is still only about 45%. Some famous people who were eventually killed by the disease were opera star Maria Callas, actor Laurence Olivier, football running back Ricky Bell, and humanist, physician and humourist Rob Buckman.