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What Are Dermatomyositis and Polymyositis?

Dermatomyositis and polymyositis are rare diseases that cause inflammation and weakness of muscles and some skin tissues. They are also known as “idiopathic inflammatory myopathies.” They affect both adults (usually ages 45 to 60) and children (usually ages 10 to 15), more females than males.

What Causes Dermatomyositis and Polymyositis?

The cause is unknown, but they are thought to be autoimmune disorders. In autoimmune disorders, the immune system attacks the body’s own tissues. Small blood vessels in muscles and some skin tissues get damaged, which weakens muscle fibers and makes them break down. The disorders aren’t contagious or passed from parents to children.

What Are the Symptoms of Dermatomyositis and Polymyositis?

Symptoms of dermatomyositis are a rash (on the face, chest, back, elbows, knees, and knuckles) and weakness of neck, shoulder, upper arm, hip, and thigh muscles. People may have trouble getting out of chairs, climbing stairs, lifting things, or reaching over their heads. Sometimes, trouble swallowing, sore muscles, tiredness, fever, hard bumps under the skin, and weight loss occur. The disease may also affect the lungs, heart, or gut. No rash occurs in polymyositis, but other symptoms are the same.

How Are Dermatomyositis and Polymyositis Diagnosed?

The health care provider makes a diagnosis based on your history, physical examination, blood tests, electromyography, and muscle biopsy. Blood tests can show muscle breakdown, inflammation, or antibodies. Electromyography measures how muscles are working. In a biopsy, the doctor removes a small piece of muscle that is sent for study.

How Are Dermatomyositis and Polymyositis Treated?

Treatments may help the rash and muscle strength improve, but some muscles may be permanently weak. However, treatment may last for months or even years. Sometimes, the disease goes away on its own.

Corticosteroid drugs, especially prednisone, are the main treatment for inflammation. If corticosteroids don’t work, immunosuppressant drugs and immunoglobulins may be tried. Skin cream (such as prednisone and tacrolimus) may be used for the rash.

Muscle exercises are an important part of treatment. They’re for flexibility and help strengthen muscles. A physical therapist can create the right exercise program.

DOs and DON’Ts in Managing Dermatomyositis and Polymyositis:

  • DO get treatment as soon as possible. Early treatment gets better results.
  • DO tell your health care provider if you have medicine side effects.
  • DO see your health care provider right away if you have trouble swallowing or shortness of breath.
  • DO keep active. This will help keep your muscles from getting weaker. Do your physical therapy exercises.
  • DO cover your skin or wear strong sunscreen when you go out.
  • DO remember that treatments may be needed for years. You may need frequent blood tests to monitor your disease and side-effects of the medications. About half of people have a remission and stop therapy within 5 years of diagnosis. The others will have active disease that needs ongoing treatment or inactive disease with permanent muscle weakness.
  • DO understand that the disease may be related to an increased risk of cancers of the colon, breast, and ovary.
  • DO call your health care provider if you have increasing muscle weakness.
  • DON’T push your body too hard. Learn to pace yourself. Rest when you’re tired.
  • DON’T miss follow-up appointments.
  • DON’T stop taking medicines without talking to your health care provider. Prednisone, if used for a long time, must be tapered off rather than stopped suddenly.
FOR MORE INFORMATION

Contact the following sources:

  • The Myositis Association
    Tel: (202) 887-0088, (800) 821-7356
    Website: http://www.myositis.org
  • National Organization for Rare Disorders
    Tel: (800) 999-6673, (203) 744-0100
    Website: http://www.rarediseases.org
  • National Institute of Arthritis and Musculoskeletal and Skin Diseases
    Tel: (301) 496-8188
    Website: http://www.niams.nih.gov

Copyright © 2016 by Saunders, an imprint of Elsevier, Inc.

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