Dermatomyositis (DM)
16:52 Posted by Ahmed Khalil
| What is it? |
| Dermatomyositis’ cardinal symptom is a skin rash that precedes or accompanies progressive muscle weakness such as a disorder called polymyositis, which affects the muscles, causing inflammation and weakness. Dermatomyositis includes these symptoms, as well as skin inflammation. Dermatomyositis may be associated with collagen-vascular or autoimmune diseases, such as lupus. |
Who gets it? |
| Dermatomyositis can affect any age group, but is more likely to occur in middle-aged adults. In children, it usually appears between the ages of 5 and 15. It is twice as common in women. |
| What causes it? |
| The cause of dermatomyositis is unknown. It may be related to an autoimmune reaction, or may be triggered by a virus or cancer. |
| What are the symptoms? |
| Symptoms of dermatomyositis usually begin gradually, with muscle weakness, joint pain, fever, weight loss, and fatigue. Children may experience a more rapid onset. Muscle weakness usually affects the upper arms, neck, and legs, on both sides of the body. Some people experience joint and muscle pain, and some have difficulty swallowing. As the disease progresses, the muscles begin to waste away (atrophy) and permanently shorten (contracture). A grayish-red rash appears at the same time as the muscle weakness. The rash commonly appears on the face and includes a purplish swelling, called a heliotrope, around the eyes. There may be a shawl-like rash over the neck, shoulders, and upper chest and back. Another rash often appears around the joints, especially around the fingers. This rash may be smooth or have scaly patches, and is called Gottron’s sign. When the rash fades, it leaves behind pigmented patches of skin. Some people with dermatomyositis develop a rash without muscle weakness. This is called dermatomyositis sine myositis. Others may also have Raynaud’s phenomenon, in which the small arteries in the fingers and toes constrict, causing the skin to look pale or bluish, usually after exposure to cold. People with dermatomyositis are more likely to develop malignant cancers. |
| How is it diagnosed? |
| Dermatomyositis is diagnosed through a thorough physical exam and the characteristic symptoms. Your doctor will order diagnostic tests to check levels of certain enzymes in your bloodstream. He or she will also take a small sample of tissue from the affected muscle, called a muscle biopsy, to check for abnormalities. A test called electromyography will reveal any abnormal electrical activity in the muscles. Magnetic resonance imaging (MRI) is also helpful in revealing muscle inflammation. Your doctor will order blood tests and other cancer screening tests to check for malignancies. |
| What is the treatment? |
| There is no cure for dermatomyositis, but the symptoms can be treated. Options include medication, physical therapy, exercise, heat therapy (including microwave and ultrasound), orthotics and assistive devices, and rest. The standard treatment for dermatomyositis is a corticosteroid drug, given either in pill form or intravenously. Immunosuppressant drugs, such as azathioprine and methotrexate, may reduce inflammation in people who do not respond well to prednisone. Periodic treatment using intravenous immunoglobulin can also improve recovery. Other immunosuppressive agents used to treat the inflammation associated with dermatomyositis include cyclosporine A, cyclophosphamide, and tacrolimus. Physical therapy is usually recommended to prevent muscle atrophy and to regain muscle strength and range of motion. Many individuals with dermatomyositis may need a topical ointment, such as topical corticosteroids, for their skin disorder. They should wear a high-protection sunscreen and protective clothing. Surgery may be required to remove calcium deposits that cause nerve pain and recurrent infections. |
| Self-care tips |
| While there is no known way to prevent dermatomyositis, you can find relief from symptoms and possible remission by following your doctor’s treatment recommendations. It is very important to reduce your physical activity until your muscles have had the opportunity to recover. |
| What research is being done? |
| The National Institute of Neurological Disorders and Stroke (NINDS) and other institutes of the National Institutes of Health (NIH) conduct research relating to dermatomyositis in laboratories at the NIH and support additional research through grants to major medical institutions across the country. Currently funded research is exploring patterns of gene expression among the inflammatory myopathies, the role of viral infection as a precursor to the disorders, and the safety and efficacy of various treatment regimens. |
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