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Polymyositis & Dermatomyositis Information

Dermatomyositis (DM) is a connective-tissue disease related to Polymyositis (PM) that is characterized by inflammation of the muscles and the skin. The cause is unknown, but it may result from either a viral infection or an autoimmune reaction. Some cases of dermatomyositis actually "overlap" (are combined with) another autoimmune disease such as lupus, scleroderma, or vasculitis. Because of the link between DM and autoimmune disease, doctors and patients suspecting DM may find it helpful to run an ANA - antinuclear antibody - test, which in cases of a lupus-like nature may be positive (usually from 1:160 to 1:640, with normal ranges at 1:40 and below).

Some cases of DM are a paraneoplastic phenomenon, indicating the presence of cancer. In cases involving cancer, the cancer is usually pre-existent, with removal of the cancer resulting in remission of the DM. The onset of a rash in patients with pre-existing myositis requires investigation of the neoplastic possibility.

Before the advent of modern treatments such as: prednisone, IVIG, plasmapheresis, chemotherapies, and other drugs, the mortality rate was very high with these conditions (up to 75% of patients died in cases that involved the lungs). 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 main symptoms include skin rash and symmetric proximal muscle weakness which may be accompanied by pain. The pain may resemble the type experienced after strenuous exercise. Some DM patients have little pain, while in others (esp. in JDM), the pain may be severe. It is important to remember that this condition varies from person to person in many ways.

Skin findings occur in DM but not PM and are generally present at diagnosis. Gottron's sign is an erythematous, scaly eruption occurring in symmetric fashion over the MCP and interphalangeal joints (can mimic psoriasis). Heliotrope rash is a violaceous eruption on the upper eyelids, often with swelling (most specific, though uncommon). Shawl (or V-) sign is a diffuse, flat, erythematous lesion over the chest and shoulders or in a "V" over the anterior neck and chest, worsened with UV light. Erythroderma is a flat, erythematous lesion similar to the shawl sign but located in other areas, such as the malar region and the forehead. Periungual telangiectasias and erythema occur.

Mechanic's hands (also in PM) refers to rough, cracked skin at the tips and lateral aspects of the fingers forming irregular dirty-appearing lines that resemble those seen in a laborer (this is also associated with the anti-synthetase syndrome).See: sclerodactyly. Psoriaform changes in the scalp can occur. Centripetal flagellate erythema comprises linear, violaceous streaks on the trunk (possibly caused by itching pruritic skin). Calcinosis cutis (deposition of calcium in the skin) is usually seen in juvenile DM, not adult DM. Dysphagia (difficulty swallowing) is another feature, occurring in as much as 33% of cases.

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