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Dermatomyositis

Overview

Dermatomyositis (dur-muh-toe-my-uh-SY-tis) is an uncommon inflammatory disease marked by muscle weakness and a distinctive skin rash.

The condition can affect adults and children. In adults, dermatomyositis usually occurs from the late 40s to early 60s. In children, it most often appears between 5 and 15 years of age. Dermatomyositis affects more females than males.

There's no cure for dermatomyositis, but periods of symptom improvement (remission) can occur. Treatment can clear the skin rash and help you regain muscle strength and function.

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.

When to see a doctor

Seek medical attention if you develop muscle weakness or an unexplained rash.

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.

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.

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.

Preparing for an appointment

You're likely to first see your family doctor, who might refer you to a doctor who specializes in the treatment of arthritis and other diseases of the joints, muscles and bones (rheumatologist) or to a doctor who specializes in disorders of the nervous system (neurologist).

What you can do

Make a list that includes:

  • Detailed descriptions of your symptoms and when they began
  • Information about medical problems you've had, and those of your parents or siblings
  • All the medications and dietary supplements you take and their doses
  • Questions to ask the doctor

Take a family member or friend along, if possible, to help you remember the information you're given.

For dermatomyositis, questions to ask your doctor include:

  • What is likely causing my symptoms?
  • What are other possible causes?
  • Are my symptoms likely to change over time?
  • What tests might I need? Are special preparations required?
  • Are treatments available for my condition? What treatments do you recommend?
  • I have other medical conditions. How can I best manage them together?
  • Do you have brochures or other printed material I can have? What websites do you recommend?

Don't hesitate to ask other questions.

What to expect from your doctor

Your doctor will likely ask you questions, such as:

  • Did your condition develop gradually or come on suddenly?
  • Are you easily fatigued during waking hours?
  • Does your condition limit your activities?
  • Has anyone in your family been diagnosed with a condition that affects the muscles?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?

Lifestyle and home remedies

With dermatomyositis, areas affected by your rash are more sensitive to the sun. Wear protective clothing or high-protection sunscreen when you go outside.

Coping and support

Living with a chronic autoimmune disease can make you wonder whether you're up to the challenge. To help you cope, try supplementing your medical care with the following:

  • Know your illness. Read all you can about dermatomyositis and other muscle and autoimmune disorders. Talk to people who have a similar condition. Don't be afraid to ask your doctor questions concerning your illness, diagnosis or treatment plan.
  • Be a part of your medical team. Consider yourself, your doctor and other medical experts involved in your care as a united front in the fight against your disease. Following the treatment plan you agreed to is vital. Keep your doctor updated on any new signs or symptoms you develop.
  • Get active. A regular exercise routine can help you maintain and build your muscle strength. Be sure to get a detailed plan and recommendations from your doctor or physical therapist before starting an exercise program.
  • Rest when you're tired. Don't wait until you're exhausted. This will only set you back further as your body tries to recuperate. Learning to pace yourself can help you maintain a consistent level of energy, accomplish just as much and feel better emotionally.
  • Acknowledge your emotions. Denial, anger and frustration are normal when dealing with an illness. Things don't seem normal or fair and likely seem out of your control. Feelings of fear and isolation are common, so stay close to your family and friends. Try to maintain your daily routine as best you can and don't neglect doing things you enjoy. Many people find support groups helpful.

Last updated: August 1st, 2017

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