What is dermatomyositis?

When the inflammatory muscle disease polymyositis affects the skin, it is called dermatomyositis. In this condition, the eyes can be surrounded by a violet discoloration with swelling. There may be scaly, red skin over the knuckles, elbows, and knees. In addition, a red rash can occur on the face, neck, and upper chest. Hard lumps of calcium deposits can develop in the fatty layer of the skin.

What causes dermatomyositis?

In people with polymyositis and dermatomyositis, the body’s immune system stops working well and begins attacking healthy tissues. Factors triggering this process are not known.

What are the symptoms of dermatomyositis?

Symptoms of dermatomyositis can vary a great deal from patient to patient. Some people may have the disease for months or years before seeing it. Muscle weakness may arise at the same time as the rash, or it may occur weeks, months, or years later. Some other common symptoms include:

  • Reddish or bluish-purple patches, mostly on areas exposed to the sun
  • Purple spots on bony prominences, especially the knuckles
  • Discoloration with swelling around the eyes
  • Ragged cuticles and prominent blood vessels on nail folds
  • A red rash on the face, neck, shoulders, upper chest and elbows

How is dermatomyositis diagnosed?

The diagnosis of dermatomyositis is usually confirmed by the following tests:

  • Blood tests to detect increased amounts of muscle enzymes such as creatine kinase (CK) and sometimes lactic dehydrogenase (LDH)
  • Blood tests to detect autoantibodies (antibodies that react with cells, tissues, or native proteins of the individual in which the antibodies are produced)
  • Skin biopsy of the rash
  • Biopsy of an affected muscle
  • Electromyography (EMG) testing
  • Magnetic resonance imaging (MRI) scan of muscles

How is dermatomyositis treated?

Doctors usually begin treatment of dermatomyositis with steroid drugs such as prednisone. Immunosuppressants may be helpful for patients who are not helped by prednisone. These include:

  • Methotrexate (brand names Rheumatrex® and Trexall®)
  • Azathioprine (brand name Imuran® and Azasan®)
  • Cyclophosphamide (brand name Cytoxan®)
  • Chlorambucil (brand name Leukeran®)
  • Cyclosporine (brand name Sandimmune®, Gengraf®, and Neoral®)
  • Tacrolimus (brand name Astagraf XL®, Hecoria®, Prograf®)
  • Mycophenolate (brand name CellCept®, Myfortic®)
  • Rituximab (brand name Rituxan®)

Further treatment with intravenous immunoglobulin (IVIG), used to slow down the autoimmune process, has been shown to be effective and safe. Physical therapy can preserve muscle function and prevent muscle wasting.

For more information, please see the following:

Muscular Dystrophy Association - USA
National Office
222 S. Riverside Plaza, Suite 1500
Chicago, IL 60606
mda@mdausa.org
www.mda.org
Toll free: 800-572-1717

National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) Information Clearinghouse
National Institutes of Health
1 AMS Circle
Bethesda, MD 20892-3675
Email: NIAMSinfo@mail.nih.gov
www.niams.nih.gov
Tel: 301-495-4484
Toll free: 877-22-NIAMS (877-226-4267)

Myositis Association
1737 King Street
Suite 600
Alexandria, VA 22314
Tel: 703-299-4850 (DC Area)
Toll free: 800-821-7356
Fax: 703-535-6752
Email: tma@myositis.org
www.myositis.org

References

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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 9/22/2015…#15701