Dermatomyositis

Medically Reviewed By William C. Lloyd III, MD, FACS
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What is dermatomyositis?

Dermatomyositis is an inflammatory condition that affects the muscles and is characterized by muscle pain and skin rashes. It may result in chronic inflammation of your muscles and skin, which causes muscle pain, weakness, atrophy (reduction in size), and dysfunction. In addition, a purple or dark red rash may occur anywhere on the skin, but most typically appears on the eyelids and in places on the skin where a muscle covers a joint, such as the elbows, knees, knuckles and toes.

The exact cause of dermatomyositis is not clear. It is thought to have a genetic link or possibly be related to a previous infection. It is also similar to autoimmune diseases because the immune system targets the muscle and skin tissues. Studies have shown that people who have dermatomyositis usually have elevated muscle enzymes and elevated autoantibody levels. Dermatomyositis occurs most often in women who are 40 to 60 and in children who are 5 to 15. The disease is less common in men than in women. (Source: PubMed).

The symptoms of dermatomyositis typically develop slowly, possibly over weeks or several months. Once you develop dermatomyositis, you may have periods of remission followed by flare-ups of symptoms.

The most common symptom of dermatomyositis is a skin rash that is a dark reddish or purple color and appears on your skin in places where muscles are involved in joint movement. Muscle symptoms occur when blood vessels in the muscle fibers become inflamed, causing muscle damage, which results in muscle weakness, atrophy, and pain. Fortunately, the symptoms of dermatomyositis can be treated successfully. The most common treatment is immunosuppressant medications, such as corticosteroids, that will suppress the immune system response and relieve pain. Support therapies include physical therapy to help strengthen affected muscles and speech therapy when muscles involving speech are affected.

In some cases, dermatomyositis can be associated with serious conditions or symptoms. Seek immediate medical care (call 911) for serious symptoms, such as profound weakness, inability to urinate, severe muscle pain, difficulty breathing, or chest pain.

Seek prompt medical care if you are being treated for dermatomyositis but mild symptoms recur or are persistent.

What are the symptoms of dermatomyositis?

Dermatomyositis causes inflammation and weakness in the muscles and produces characteristic skin rashes. Symptoms can vary in intensity among individuals and may occur daily or only occasionally. However, at any time, symptoms may become severe.

Common symptoms of dermatomyositis

You may experience dermatomyositis symptoms daily or only occasionally. At times, any of these symptoms can be severe:

Serious symptoms that might indicate a life-threatening condition

In some cases, dermatomyositis can be associated with serious symptoms. Seek immediate medical care (call 911) if you, or someone you are with, have any of these life-threatening symptoms including:

  • Chest pain or pressure
  • Difficulty breathing or rapid breathing
  • Inability to urinate
  • Profound muscle weakness
  • Severe muscle pain

What causes dermatomyositis?

The cause of dermatomyositis is poorly understood. It may be related to an infection. It is also thought that there is a genetic association with the development of dermatomyositis. Dermatomyositis is similar to autoimmune diseases because the immune system targets the muscle and skin tissues. Once you develop dermatomyositis, you may have periods of remission followed by flare-ups of symptoms.

What are the risk factors for dermatomyositis?

A number of factors increase the risk of developing dermatomyositis. Not all people with risk factors will get dermatomyositis. Risk factors include:

    • Autoimmune disorders
    • Bacterial, parasitic or viral infections
    • Elevated autoantibody levels
    • Family history of dermatomyositis
    • Inflammatory conditions

    How is dermatomyositis treated?

    Treatment for dermatomyositis begins with seeking medical care from your health care provider. To determine whether you have dermatomyositis, you will be asked questions about your symptoms and when they occur. You may be asked to undergo diagnostic testing.

    Treatments for dermatomyositis

    Dermatomyositis requires specific regimens to treat and manage symptoms. There is no cure for the condition. Due to the inflammatory characteristics of this disorder, corticosteroid medications are used to decrease the immune system response. These medications are the most common treatment for symptoms associated with dermatomyositis, and they are effective in reducing inflammation, redness and pain.

    Treatment options include:

    • Antimalarial medications, including hydroxychloroquine (Plaquenil)
    • Corticosteroids to reduce inflammation
    • Immunosuppressant medications such as cyclophosphamide (Cytoxan)
    • Intravenous immunoglobulins to reduce the immune response
    • Pain relieving medications such as ibuprofen (Advil, Motrin) or acetaminophen (Tylenol)
    • Steroid-sparing agents (alternative drugs that modify the disease course) such as azathioprine (Imuran)

    Overall, treatment should include a lifestyle plan to optimize health and reduce symptoms. A well-balanced nutrition plan, regular physical activity, and routine testing to monitor disease are all important treatment options for people with dermatomyositis. Other types of treatment include physical therapy, speech therapy, and dietary counseling.

    What you can do to improve your dermatomyositis

    In addition to following your health care provider’s instructions and taking all medication as prescribed, you can improve your symptoms by:

    • Ensuring adequate hydration by drinking plenty of water and fluids
    • Following a balanced nutrition plan
    • Getting plenty of rest
    • Having routine follow-up appointments with your healthcare provider
    • Maintaining a healthy body weight

    What are the potential complications of dermatomyositis?

    You can help minimize your risk of serious complications by following the treatment plan you and your health care professional design specifically for you. Complications of dermatomyositis include:

    • Calcinosis (calcium deposits beneath the skin)
    • Increased risk of cancer
    • Lung disease
    • Myocarditis (infection of the middle layer of the heart wall)
    • Pulmonary aspiration (inhaling blood, vomited material or other substances into lungs)
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    Medical Reviewer: William C. Lloyd III, MD, FACS
    Last Review Date: 2020 Aug 20
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    THIS TOOL DOES NOT PROVIDE MEDICAL ADVICE. It is intended for informational purposes only. It is not a substitute for professional medical advice, diagnosis or treatment. Never ignore professional medical advice in seeking treatment because of something you have read on the site. If you think you may have a medical emergency, immediately call your doctor or dial 911.
    1. Dermatomyositis. PubMed Health, a service of the NLM from the NIH. http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001842/
    2. NINDS dermatomyositis information page. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/dermatomyositis/dermatomyositis.htm
    3. Callen JP, Wortmann RL. Dermatomyositis. Clin Dermatol 2006; 24:363