Dermatomyositis
Dermatomyositis is a disease that involves muscle inflammation and a skin rash. Polymyositis is a similar inflammatory condition that also involves muscle weakness, swelling, tenderness, and tissue damage but no skin rash. Both are part of a larger group of diseases called myopathies, more specifically inflammatory myopathies.
Red, thickened, scaly skin over the knuckles (Gottron sign) associated with dermatomyositis, an autoimmune disease of the skin and muscles.
This violet-colored inflammation (erythema) over the knuckles is caused by Dermatomyositis. Other skin conditions produce more redness, while the color of this lesion is violet. There may also be inflammation in the muscle tissue.
This photograph demonstrates the sign heliotrope eyelids in which the eyelids develop a brown (violaceous - rather than red) color. Heliotrope eyelids and Gottron's papules on the knuckles are characteristic findings in dermatomyositis.
The appearance of purple (violaceous) plaques on the knees may be associated with dermatomyositis. Typically, most lesions associated with other diseases are red (erythematous).
This is Gottron sign, seen in dermatomyositis (an inflammatory disease of the muscles and skin). Violet-colored inflammation over the knuckles is an important diagnostic finding in dermatomyositis, since other skin conditions produce more redness.
Dermatomyositis, a connective tissue disease, typically produces a reddish-purple (violaceous) rash. The rash is named after the tendency of plants to grow toward the sun (heliotropic) and is characteristic of dermatomyositis.
Causes
The cause of dermatomyositis is unknown. Experts think it may be due to a viral infection of the muscles or a problem with the body's immune system. It may also occur in people who have cancer in the abdomen, lung, or other parts of the body.
Anyone can develop this condition. It most often occurs in children age 5 to 15 and adults age 40 to 60. It affects women more often than men.
Symptoms
Symptoms may include:
- Muscle weakness, stiffness, or soreness
- Problems swallowing
- Purple color to the upper eyelids
- Purple-red skin rash
- Shortness of breath
- Difficulty swallowing
The muscle weakness may come on suddenly or develop slowly over weeks or months. You may have trouble raising your arms over your head, getting up from a sitting position, and climbing stairs.
The rash may appear on your face, knuckles, neck, shoulders, upper chest, and back.
Exams and Tests
The health care provider will do a physical exam. Tests may include:
- Bloods test to check levels of muscle enzymes called creatine phosphokinase and aldolase
- Blood tests for autoimmune diseases
- Electrocardiogram (ECG)
- Electromyography (EMG)
- Magnetic resonance imaging (MRI)
- Muscle biopsy
- Skin biopsy
- Screening tests for cancer
- Chest x-ray and CT scan of the chest
- Lung function tests
- Swallowing study
- Myositis specific and associated autoantibodies
Treatment
The main treatment is the use of corticosteroid medicines. The dose of medicine is slowly tapered off as muscle strength improves. This takes about 4 to 6 weeks. You may stay on a low dose of a corticosteroid medicine after that.
Medicines to suppress the immune system may be used to replace the corticosteroids. These drugs may include azathioprine, methotrexate or mycophenolate.
Treatments that may be tried when disease that remains active in spite of these medicines are:
- Intravenous gamma globulin
- Biologic drugs
When your muscles get stronger, your provider may tell you to slowly cut back on your doses. Many people with this condition must take a medicine called prednisone for the rest of their lives.
If a tumor is causing the condition, the muscle weakness and rash may get better when the tumor is removed.
Outlook (Prognosis)
It is important to be followed by a health care provider when you have dermatomyositis. Severe illness can lead to disability and can sometimes result in death.
Symptoms may go away completely in some people, such as children.
The condition may be fatal in adults due to:
- Severe muscle weakness
- Malnutrition
- Pneumonia
- Lung failure
The major causes of death with this condition are cancer and lung disease.
People with lung disease with the anti-MDA-5 antibody have a poor prognosis in spite of current treatment.
Possible Complications
Complications may include:
- Lung disease
- Acute renal failure
- Cancer (malignancy)
- Inflammation of the heart
- Joint pain
When to Contact a Medical Professional
Contact your provider if you have muscle weakness or other symptoms of this condition.
References
Aggarwal R, Rider LG, Ruperto N, et al. 2016 American College of Rheumatology/European League against rheumatism criteria for minimal, moderate, and major clinical response in adult dermatomyositis and polymyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. Arthritis Rheumatol. 2017;69(5):898-910. PMID: 28382787
Dalakas MC. Inflammatory muscle diseases. N Engl J Med. 2015;373(4):393-394. PMID: 26200989
Nagaraju K, Aggarwal R, Lundberg IE. Inflammatory diseases of muscle and other myopathies. In: Firestein GS, Budd RC, Gabriel SE, Koretzky GA, McInnes IB, O'Dell JR, eds. Firestein & Kelley's Textbook of Rheumatology. 11th ed. Philadelphia, PA: Elsevier; 2021:chap 90.
National Organization for Rare Disorders website. Dermatomyositis.
Version Info
Last reviewed on: 1/25/2023
Reviewed by: Neil J. Gonter, MD, Assistant Professor of Medicine, Columbia University, NY and private practice specializing in Rheumatology at Rheumatology Associates of North Jersey, Teaneck, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
