Dermatomyositis (DM) is a connective-tissue disease related to polymyositis (PM) that is characterized by inflammation of the muscles and the skin.
Dermatomyositis is a type of autoimmune connective tissue disease. It is related to polymyositis and inclusion body myositis.
There is a form of this disorder that strikes children, known as juvenile dermatomyositis(JDM). For the most part Juvenile dermatomyositis is the same as the adult form, but the relationship with cancer is far lower, or non-existent.
Before the advent of modern treatments such as prednisone, Intravenous immunoglobulin, plasmapheresis, chemotherapies, and other drugs, prognosis was poor. Now, in the 21st century, there are numerous treatments and immune-modulating drugs. Fortunately, over 90% of patients today will do well for many years, with remission being a possibility. However, it is still important that treatment begin as soon as possible.
The mechanism of Dermatomyositis is conjectured to be complement-mediated damage of microscopic vessels with muscle atrophy and lymphocytic inflammation secondary to tissue ischemia.
This disease is incurable. Medications to help relieve symptoms include:
- Mycophenolate (CellCept / Myfortic)
- Intravenous immunoglobulin
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