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Dermatomyositis

A disease of connective tissue characterized by inflammation of the muscles and skin.

PubMed Health Glossary
(Source: Wiktionary)

About 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, accompanies, or follows progressive muscle weakness. The rash looks patchy, with purple or red discolorations, and characteristically develops on the eyelids and on muscles used to extend or straighten joints, including knuckles, elbows, knees, 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, a low-grade fever, inflamed lungs, and be sensitive to light such that the rash or muscle disease gets worse. 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....Read more about Dermatomyositis NIH - National Institute of Neurological Disorders and Stroke

What works? Research summarized

Evidence reviews

Drugs that suppress or modify the immune system for dermatomyositis and polymyositis

Dermatomyositis and polymyositis are long‐term inflammatory muscle diseases, causing muscle weakness and disability. For some reason, the body's immune system turns against its own muscles in an autoimmune response. Corticosteroids are the principal treatment but due to side effects, there is a need for additional treatment with drugs that suppress the immune system (immunosuppressants) or modify it (immunomodulatory therapies) to improve patient outcomes. For this review, an update of a review first published in 2005, we found ten randomised trials available, involving 258 participants.

Intravenous immunoglobulin therapy in adult patients with polymyositis/dermatomyositis: a systematic literature review

The objectives of this study are to review and summarize published information on the use, effectiveness, and adverse effects of intravenous immunoglobulin (IVIG) in patients with polymyositis (PM) or dermatomyositis (DM) and to search MEDLINE and CNKI (Chinese) databases from 1985 to 2011 to retrieve clinical research articles concerning IVIG in adult patients with PM/DM. Of the 14 articles selected, two were randomized controlled trials, nine prospective open studies, and three retrospective studies with a total of 308 adult patients. IVIG has been used successfully in the treatment of PM/DM. The standard dose is 2 g/kg, given in two to five individual daily doses. The course of IVIG treatment is usually 3~6 months. IVIG therapy seemed rarely employed as first-line therapy in PM/DM. In a double-blind study conducted in patients with refractory DM, IVIG combined with corticosteroid significantly improved muscle strength and decreased serum creatine kinase level, compared with placebo. The beneficial effect of IVIG in refractory, flare-up, rapidly progressive, or severe PM/DM has been documented in many open-label trials. IVIG was shown to be effective in most of PM/DM patients with lung involvement and esophageal involvement. In some patients, IVIG can lower the corticosteroid dose required for maintenance, demonstrating the most effective steroid-sparing effect. Adverse effects were generally tolerable. IVIG is effective in the treatment of adult patients with PM/DM and appears to be relatively well tolerated and safe. IVIG may be a good choice especially in patients with refractory, flare-up, rapidly progressive, or severe PM/DM, and can be tried in patients with a contraindication for corticosteroid.

New onset of dermatomyositis/polymyositis during anti-TNF-therapies: a systematic literature review

We performed a systematic search of databases from 1990 to 2013 to identify articles concerning the new onset of dermatomyositis/polymyositis (DM/PM) in patients treated with anti-TNF-α therapy. We retrieved 13 publications describing 20 patients where the new onset of DM/PM after anti-TNF-α therapy was recorded. 17 patients were affected by rheumatoid arthritis (RA), one by Crohn's disease, one by ankylosing spondilytis, and one by seronegative arthritis. In 91% of the cases antinuclear autoantibodies were detected after the introduction of anti-TNF-α therapy. In 6 patients antisynthetase antibodies were detected and other clinical findings as interstitial lung disease (ILD) were recorded. Improvement of DM/PM after anti-TNF suspension (with the concomitant use of other immunosuppressors) was recorded in 94% of cases. The emergence of DM/PM and antisynthetase syndrome seem to be associated with the use of anti-TNF-α agents, especially in patients with chronic inflammatory diseases (mainly RA) with positive autoantibodies before therapy initiation. In particular, physicians should pay attention to patients affected by RA with positive antisynthetase antibodies and/or history of ILD. In those cases, the use of the TNF-α blocking agents may trigger the onset of PM/DM or antisynthetase syndrome or may aggravate/trigger the lung disease.

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Summaries for consumers

Drugs that suppress or modify the immune system for dermatomyositis and polymyositis

Dermatomyositis and polymyositis are long‐term inflammatory muscle diseases, causing muscle weakness and disability. For some reason, the body's immune system turns against its own muscles in an autoimmune response. Corticosteroids are the principal treatment but due to side effects, there is a need for additional treatment with drugs that suppress the immune system (immunosuppressants) or modify it (immunomodulatory therapies) to improve patient outcomes. For this review, an update of a review first published in 2005, we found ten randomised trials available, involving 258 participants.

Strength training or comprehensive aerobic exercise training for muscle disease

Strength training, which is performed to improve muscle strength and muscle endurance, or aerobic exercise programmes, which are designed to improve cardiorespiratory endurance, might optimise physical fitness and prevent additional muscle wasting in people with muscle disease. However, people with muscle disease and some clinicians are still afraid of overuse and have a cautious approach to training. This updated review (most recent date of search 2 July 2012) included two eligible trials of strength training in people with facioscapulohumeral muscular dystrophy (FSHD) and myotonic dystrophy (101 participants), two trials of strength training combined with aerobic exercise in people with mitochondrial myopathy (18 participants) and myotonic dystrophy type I (35 participants) and one trial of aerobic exercise in people with polymyositis and dermatomyositis (14 participants). These trials showed that moderate‐intensity strength training in people with myotonic dystrophy or with FSHD, and aerobic exercise training in people with dermatomyositis or polymyositis appear not to harm muscles. Strength training combined with aerobic exercise appears to be safe in myotonic dystrophy type I and may be effective in increasing endurance in people with mitochondrial myopathy. Evidence suggests that strength training is not harmful in people in FSHD, myotonic dystrophy, mitochondrial disorders and dermatomyositis and polymyositis, but further research is needed to determine potential benefit.

Ketanserin is a drug that has been studied in the treatment of Raynaud's phenomenon and associated conditions. It is not widely used however.

Raynaud's phenomenon is a disease that causes decreased blood flow and circulation to the extremeties. Symptoms include discolouration, pain, and in some severe cases ulceration of the hands and feet. It is most often triggered by cold, stress, and emotional discomfort. Primary Raynaud's phenomenon has no underlying disease associated with it. Secondary Raynaud's phenomenon is most often associated with scleroderma, but may also be related to systemic lupus erythematosus, mixed connective tissue disease, Sjorgen's syndrome, dermatomyositis, or rheumatoid arthritis. Scleroderma is a connective tissue disease causing hardening and commonly affects the skin and internal organs such as the GI tract, lungs, kidney and heart.

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Terms to know

Inflammation
Redness, swelling, pain, and/or a feeling of heat in an area of the body. This is a protective reaction to injury, disease, or irritation of the tissues.
Muscles
Muscles function to produce force and motion. They are primarily responsible for maintaining and changing posture, locomotion, as well as movement of internal organs, such as the contraction of the heart and the movement of food through the digestive system.
Rash
Any change in the skin which affects its appearance or texture. A rash may be localized to one part of the body, or affect all the skin. Rashes may cause the skin to change color, itch, become warm, bumpy, dry, cracked or blistered, swell and may be painful.
Rheumatologist
Doctors who diagnose and treat diseases of the bones, joints, muscles, and tendons, including arthritis and collagen diseases.
Skin
The outer covering of the body that protects it from the environment.

More about Dermatomyositis

Photo of an adult woman

Also called: Wagner-Unverricht syndrome, DM

See Also: Polymyositis

Other terms to know: See all 5
Inflammation, Muscles, Rash

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