Dermatomyositis is one of a group of acquired muscle diseases called inflammatory myopathies (disorder of muscle tissue or muscles), which are characterized by chronic muscle inflammation accompanied by muscle weakness. The cardinal symptom is a skin rash that precedes or accompanies progressive muscle weakness. Dermatomyositis may occur at any age, but is most common in adults in their late 40s to early 60s, or children between 5 and 15 years of age. 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 cause of dermatomyositis is unknown.

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Symptoms

The most common signs and symptoms of dermatomyositis include:

  • Skin changes. A violet-colored or dusky red rash develops, most commonly on your face and eyelids and on knuckles, elbows, knees, chest and back. The rash, which can be itchy and painful, is often the first sign of dermatomyositis.
  • Muscle weakness. Progressive muscle weakness involves the muscles closest to the trunk, such as those in your hips, thighs, shoulders, upper arms and neck. The weakness affects both the left and right sides of your body, and tends to gradually worsen.

Causes

The cause of dermatomyositis is unknown, but the disease has much in common with autoimmune disorders, in which your immune system mistakenly attacks your body tissues.

Small blood vessels in muscular tissue are particularly affected in dermatomyositis. Inflammatory cells surround the blood vessels and eventually lead to destruction of muscle fibers.

Diagnosis

If your doctor suspects you have dermatomyositis, he or she might suggest some of the following tests:

  • Blood analysis. A blood test will let your doctor know if you have elevated levels of muscle enzymes, such as creatine kinase (CK) and aldolase. Increased CK and aldolase levels can indicate muscle damage. A blood test can also detect autoantibodies associated with different symptoms of dermatomyositis, which can help in determining the best medication and treatment.
  • Chest X-ray. This simple test can check for signs of the type of lung damage that sometimes occurs with dermatomyositis.
  • Electromyography. A doctor with specialized training inserts a thin needle electrode through the skin into the muscle to be tested. Electrical activity is measured as you relax or tighten the muscle, and changes in the pattern of electrical activity can confirm a muscle disease. The doctor can determine which muscles are affected.
  • MRI. A scanner creates cross-sectional images of your muscles from data generated by a powerful magnetic field and radio waves. Unlike a muscle biopsy, an MRI can assess inflammation over a large area of muscle.
  • Skin or muscle biopsy. A small piece of skin or muscle is removed for laboratory analysis. A skin sample can help confirm the diagnosis of dermatomyositis. A muscle biopsy might reveal inflammation in your muscles or other problems, such as damage or infection. If the skin biopsy confirms the diagnosis, a muscle biopsy might not be necessary.
 Treatment

There’s no cure for dermatomyositis, but treatment can improve your skin and your muscle strength and function. The earlier treatment is started in the course of dermatomyositis, the more effective it is.

Medications

Medications used to treat dermatomyositis include:

  • Corticosteroids. Drugs such as prednisone can control dermatomyositis symptoms quickly. But prolonged use can have serious side effects. So your doctor, after prescribing a relatively high dose to control your symptoms, might gradually reduce as your symptoms improve.
  • Corticosteroid-sparing agents. When used with a corticosteroid, these drugs can decrease the dose and side effects of the corticosteroid. The two most common medications for dermatomyositis are azathioprine (Azasan, Imuran) and methotrexate (Trexall).
  • Rituximab (Rituxan). More commonly used to treat rheumatoid arthritis, rituximab is an option if initial therapies don’t adequately control your symptoms.
  • Antimalarial medications. For a persistent rash, your doctor might prescribe an antimalarial medication, such as hydroxychloroquine (Plaquenil).
  • Sunscreens. Protecting your skin from sun exposure by applying sunscreen and wearing protective clothing and hats is important for managing the rash of dermatomyositis.

Therapy

Depending on the severity of your symptoms, your doctor might suggest:

  • Physical therapy. A physical therapist can show you exercises to help maintain and improve your strength and flexibility and advise you about an appropriate level of activity.
  • Speech therapy. If your swallowing muscles are affected, speech therapy can help you learn how to compensate for those changes.
  • Dietetic assessment. Later in the course of dermatomyositis, chewing and swallowing can become more difficult. A registered dietitian can teach you how to prepare easy-to-eat foods.

Surgical and other procedures

  • Intravenous immunoglobulin (IVIg). IVIg is a purified blood product that contains healthy antibodies from thousands of blood donors. These antibodies can block the damaging antibodies that attack muscle and skin in dermatomyositis. Given as an infusion through a vein, IVIg treatments are expensive and might need to be repeated regularly for the effects to continue.
  • Surgery. Surgery might be an option to remove painful calcium deposits and prevent recurrent skin infections.

Complications

Possible complications of dermatomyositis include:

  • Difficulty swallowing. If the muscles in your esophagus are affected, you can have problems swallowing (dysphagia), which can cause weight loss and malnutrition.
  • Aspiration pneumonia. Difficulty swallowing can also cause you to breathe food or liquids, including saliva, into your lungs (aspiration).
  • Breathing problems. If the condition affects your chest muscles, you might have breathing problems, such as shortness of breath.
  • Calcium deposits. These can occur in your muscles, skin and connective tissues (calcinosis) as the disease progresses. These deposits are more common in children with dermatomyositis and develop earlier in the course of the disease.

Associated conditions

Dermatomyositis may cause other conditions or put you at higher risk of developing them, including:

  • Raynaud’s phenomenon. This condition causes your fingers, toes, cheeks, nose and ears to turn pale when exposed to cold temperatures.
  • Other connective tissue diseases. Other conditions, such as lupus, rheumatoid arthritis, scleroderma and Sjogren’s syndrome, can occur with dermatomyositis (overlap syndromes).
  • Cardiovascular disease. Dermatomyositis can cause heart muscle inflammation (myocarditis). In a small number of people who have dermatomyositis, congestive heart failure and heart arrhythmias develop.
  • Lung disease. Interstitial lung disease can occur with dermatomyositis. Interstitial lung disease refers to a group of disorders that cause scarring (fibrosis) of lung tissue, making the lungs stiff and inelastic. Signs and symptoms include a dry cough and shortness of breath.
  • Cancer. Dermatomyositis in adults has been linked to an increased likelihood of developing cancer, particularly of the cervix, lungs, pancreas, breasts, ovaries and gastrointestinal tract. Risk of cancer increases with age, although it appears to level off three years or so after a diagnosis of dermatomyositis. Dermatomyositis can also develop after you receive a diagnosis of cancer.

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