What is Dermatomyositis?
Dermatomyositis is one of a group of muscle diseases known as the
inflammatory myopathies, which are characterized by chronic muscle inflammation
accompanied by muscle weakness. Dermatomyositis’ cardinal symptom is a skin rash
that precedes or accompanies progressive muscle weakness. 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. The rash sometimes occurs without obvious muscle involvement.
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.
Is there any treatment?
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. 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.
What is the prognosis?
Most cases of dermatomyositis respond to therapy. The disease is usually more
severe and resistant to therapy in individuals with cardiac or pulmonary
problems.
What research is being done?
The National Institute of Neurological Disorders and Stroke (NINDS) and other
institutes of the National Institutes of Health (NIH) conduct research relating
to dermatomyositis in laboratories at the NIH and support additional research
through grants to major medical institutions across the country. Currently
funded research is exploring patterns of gene expression among the inflammatory
myopathies, the role of viral infection as a precursor to the disorders, and the
safety and efficacy of various treatment regimens.
Organizations
Muscular Dystrophy Association
3300 East Sunrise Drive
Tucson, AZ 85718-3208
mda@mdausa.org
www.mda.org
Tel: 520.529.2000 800.344.4863
Fax: 520.529.5300
American Autoimmune Related Diseases Association
22100 Gratiot Avenue
Eastpointe, MI 48201-2227
aarda@aarda.org
www.aarda.org
Tel: 586.776.3900 800.598.4668
Fax: 586.776.3903
Myositis Association
1233 20th Street, NW, Suite 402
Washington, DC 20036
tma@myositis.org
www.myositis.org
Tel: 202.887.0088 800.821.7356
Fax: 202.466.8940
National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
National Institutes of Health, DHHS
31 Center Dr., Rm. 4C02 MSC 2350
Bethesda, MD 20892-2350
NIAMSinfo@mail.nih.gov
www.niams.nih.gov
Tel: 301.496.8190 877.22.NIAMS (226.4267)
National Organization for Rare Disorders (NORD)
P.O. Box 1968
(55 Kenosia Avenue)
Danbury, CT 06813-1968
orphan@rarediseases.org
www.rarediseases.org
Tel: 203.744.0100 Voice Mail 800.999.NORD (6673)
Fax: 203.798.2291
Source: National Institutes of Health; National Institute of
Neurological Disorders and Stroke
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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: 1/29/2009...#6003
