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Genetic and Rare Diseases Information Center (GARD)

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Your Question

My mother has been diagnosed with dermatomyositis. She has not responded to the treatments she has received. Can you provide information about the symptoms, treatment, and prognosis for this condition?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is dermatomyositis?

Dermatomyositis is one of a group of acquired muscle diseases called inflammatory myopathies (disorder of muscle tissue or muscles), which are characterized by chronic muscle inflammation accompanied by muscle weakness. The cardinal symptom is a skin rash that precedes or accompanies progressive muscle weakness.[1] Dermatomyositis may occur at any age, but is most common in adults in their late 40s to early 60s, or children between 5 and 15 years of age.[2] 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.[1] The cause of dermatomyositis is unknown.[3]

Last updated: 8/26/2013

What symptoms are commonly associated with dermatomyositis? 

The signs and symptoms of dermatomyositis may appear suddenly or develop gradually, over weeks or months.[2][3] The cardinal symptom of dermatomyositis is a skin rash that precedes or accompanies progressive muscle weakness.[1]  The rash looks patchy, with bluish-purple or red discolorations, and characteristically develops on the eyelids and on muscles used to extend or straighten joints, including knuckles, elbows, heels, and toes.  Red rashes may also occur on the face, neck, shoulders, upper chest, back, and other locations, and there may be swelling in the affected areas.[1][3] The rash sometimes occurs without obvious muscle involvement.[1]   

Adults with dermatomyositis may experience weight loss or a low-grade fever, have inflamed lungs, and be sensitive to light.  Children and adults with dermatomyositis may develop calcium deposits, which appear as hard bumps under the skin or in the muscle (called calcinosis). Calcinosis most often occurs 1-3 years after the disease begins.  These deposits are seen more often in children with dermatomyositis than in adults.  In some cases of dermatomyositis, distal muscles (muscles located away from the trunk of the body, such as those in the forearms and around the ankles and wrists) may be affected as the disease progresses.  Dermatomyositis may be associated with collagen-vascular or autoimmune diseases, such as lupus[1]

Last updated: 8/26/2013

What causes dermatomyositis?

The cause of this disorder is unknown. It is theorized that an autoimmune reaction (reactions caused by an immune response against the body's own tissues) or a viral infection of the skeletal muscle may cause the disease.[3] In addition, some doctors think certain people may have a genetic susceptibility to the disease.[2]
Last updated: 8/26/2013

How is dermatomyositis treated?

While there is no cure for dermatomyositis, 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.[1]
Last updated: 8/26/2013

What is the prognosis for individuals with dermatomyositis?

Most cases of dermatomyositis respond to therapy. Some people may recover and have symptoms completely disappear. This is more common in children. In adults, death may result from severe and prolonged muscle weakness, malnutrition, pneumonia, or lung failure. The outcome is usually worse if the heart or lungs are involved. [1][3]
Last updated: 8/26/2013