Dermatomyositis

Overview

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.

Symptoms and Causes

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.

Diagnosis and Tests

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.

Management and Treatment

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.

Resources

Where can I learn more about dermatomyositis?

For more information, please see the following:

Muscular Dystrophy Association - USA
National Office
161 N. Clark, Suite 3550
Chicago, IL 60601
www.mda.org
Toll free: 800.572.1717

National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) Information Clearinghouse
National Institutes of Health
Bethesda, MD 20892-3675
Phone: 301.495.4484
Toll free: 877.226.4267 (877.22.NIAMS)
TTY: 301.565.2966
Fax: 301.718.6366
Email: ++NIAMSinfo@mail.nih.gov++
Website: ++https://www.niams.nih.gov++

Myositis Association
1940 Duke St., Suite 200
Alexandria, VA 22314
Toll free: 800.821.7356
Email: tma@myositis.org
www.myositis.org

Last reviewed by a Cleveland Clinic medical professional on 04/02/2019.

References

  • Hellmann DB, Imboden JB, Jr.. Rheumatologic & Immunologic Disorders. In: Papadakis MA, McPhee SJ, Rabow MW. eds. Current Medical Diagnosis & Treatment 2015. New York, NY: McGraw-Hill; 2014.
  • Usatine RP, Smith MA, Chumley HS, Mayeaux EJ, Jr. Chapter 181. Dermatomyositis. In: Usatine RP, Smith MA, Chumley HS, Mayeaux EJ, Jr. eds. The Color Atlas of Family Medicine, 2e. New York, NY: McGraw-Hill; 2013.
  • Genetic and Rare Diseases Information Center. Dermatomyositis. Accessed 4/3/2019.
  • American Osteopathic College of Dermatologists. Dermatomyositis. Accessed 4/3/2019.

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Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy

Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy