Understanding Dermatomyositis (DM): Causes, Symptoms, and Management

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Dermatomyositis (DM)

by Alike Medical Team ∙ Updated on June 13, 2023

Additional names

This group contains additional names: - Juvenile dermatomyositis

General

Dermatomyositis (DM) is a rheumatological chronic disorder which affects the skin and the muscles. It is a rare inflammatory disease characterized by muscle weakness and a skin rash. This condition usually appears in children between 5-15 years of age or in adults in their 40s-60s. Females are more likely to have DM than males. The cause of the disease is unknown but it seems to be autoimmune. It has a connection to environmental factors such as viral infections, sun exposure, certain medication and smoking. DM is linked to other conditions, and DM patients are prone to other conditions such as Raynaud's phenomenon, lung diseases, cardiovascular disease, lupus, rheumatoid arthritis, scleroderma, and Sjorgen’s syndrome. Complications may include breathing problems, difficulty swallowing, aspiration pneumonia and calcium deposits.

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Signs & symptoms

Symptoms include skin changes such as a red or purple rash, mostly on the face, knuckles, elbows, knees, chest and back.The typical rash called heliotrope rash, occurring around the eyes and upper chest (V-sign). Another form the rash takes is called Gottron's sign which are slightly raised papules that erupt on any of the finger joints and other bony prominence. The rash can be painful and itchy, and many times can be the first sign of the disorder. Also, a progressive muscle weakness is present in the muscles close to the trunk such as the hips, thighs, shoulders, upper arms and neck.

Diagnosis

If dermatomyositis is suspected due to findings in medical history and physical examination, other tests may be done. Blood analysis can look for high muscle enzymes (CPK, aldolase, pyruvate transaminase and lactate dehydrogenase). 80% will also be positive for antinuclear antibodies (ANA), other antibodies important to check are aminoacyl-tRNA synthetases, Jo-1, SRP, and anti-Mi-2 antibodies. Electric signaling in muscle tests can look for signs of muscle weakness. Muscle biopsy may look for white blood cells or dying muscle cells. A typical rash must also be present.

Treatment

There is no cure for dermatomyositis, therefore most of the treatment is symptomatic. The gold standard medication is corticosteroids. Immunosuppressant drugs (methotrexate, azathioprine) are given to those who do not respond to steroids to reduce inflammation. Topical steroids can be given to the rash. Other lines of treatment may include antimalarial medication and rituximab. Physical therapy is recommended for regaining muscle strength and range of motion.

Note

☝ We provide information on prescription and over-the-counter medicines, diagnosis, procedures and lab tests. This material is provided for educational purposes only and is not medical advice, diagnosis or treatment.

National Institutes of Health ∙ World Health Organization ∙ MedlinePluse ∙ Centers for Disease Control and Prevention

☝ All information has been reviewed by certified physicians from Alike

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