Author: Vanessa Ngan, Staff Writer, 2003.
Dermatomyositis is a rare acquired muscle disease that is accompanied by a rash. It is one of a group of muscle diseases called inflammatory myopathies.
Dermatomyositis may affect people of any race, age or sex, although it is twice as common in women than in men. The onset of the disease is most common in those aged 50–70 years.
Factors that may play a part in its development are listed below.
Dermatomyositis is characterised by cutaneous features and myositis.
In many patients, the first sign of dermatomyositis is the presence of a symptomless, itchy or burning rash. The rash often, but not always, develops before the muscle weakness.
Muscle weakness may arise at the same time as the dermatomyositis rash, or it may occur weeks, months or years later. Proximal muscles are affected, that is, those closest to the trunk (upper arms, thighs). The first indication of myositis is when the following everyday movements become difficult.
Occasionally the affected muscles ache and become tender to touch.
Calcinosis affects some people, especially children and adolescents
Some patients have swollen joints (arthritis) and Raynaud phenomenon (this term refers to fingers that go very white and stiff in cold conditions, then purple as they warm again).
The diagnosis of dermatomyositis is usually confirmed by the following tests.
In those over 60, full body examination and testing are recommended, looking for underlying cancer.
The primary aim of treatment is to control the skin disease and the muscle disease. An oral corticosteroid such as prednisone in moderate to high dose is the mainstay of medical therapy and is given to slow down the rate of disease progression. Immunosuppressive or cytotoxic drugs may also be used including methotrexate, azathioprine, cyclophosphamide, ciclosporin, mycophenolate, high dose intravenous immunoglobulin and experimentally, biologics such as rituximab. Other important measures in the management of dermatomyositis include:
Most patients will require treatment throughout their lifetime, but dermatomyositis completely resolves in about one-in-five patients. Patients who have disease affecting their heart or lungs, or who also have an underlying cancer, do less well and may ultimately die from their disease.
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