Pediatric Diseases and ConditionsJuvenile Dermatomyositis
Topic Index - Pediatric Arthritis and Other Rheumatic Diseases
Dermatomyositis is a rare disease marked by increasing muscle weakness, skin rash and inflammation, and swelling of the eyelids.
Dermatomyositis is one of a group of muscle diseases that cause muscle swelling, or inflammatory myopathies (myos comes from the Greek word for muscle). It's different from other muscle diseases because it also causes skin changes. Some people with the disease also have a connective tissue disorder, such as lupus or rheumatoid arthritis.
Dermatomyositis can occur at any age, but the most common time of diagnosis is between ages 50 and 70. Women are twice as likely as men to be diagnosed with this disease.
The exact cause is not known, but possible causes include:
Abnormal genes you are born with
Cancer, especially in older people
Autoimmune disease. This is a disease that causes your body's immune system, or natural defense system, to turn against you.
An infection, medication, or another exposure in your environment that triggers the disease
The signs and symptoms of dermatomyositis are caused by swelling and inflammation in the blood vessels that supply your skin and muscles.
Dermatoyositis is the term used to describe both muscle and skin symptoms. Some people have muscle symptoms without skin symptoms, and this is called polymyositis. Others have skin symptoms without muscle symptoms, a condition called amyopathic dermatomyositis.
You could have any of these signs and symptoms if you have dermatomyositis:
Red to purple rash on sun-exposed areas that may be painful or itchy
Red to purple swelling of the upper eyelids called heliotrope
Red to purple spots on the knuckles, elbows, knees, and toes called Gottron's papules
Raynaud's phenomenon, in which small joints may become white, stiff, and painful in cold conditions and improve when warmed
Scaly, rough, dry skin, which can lead to hair thinning
Swollen, red areas around the fingernails
Hard lumps under the skin caused by calcium deposits called calcinosis
Muscle weakness in the neck, hip, back, and shoulders
Difficulty swallowing and voice changes
Tiredness, fever, and weight loss
Trouble rising from a chair or getting out of bed because of muscle weakness
Sometimes the muscle weakness also spreads to the heart, GI tract, and lungs and can cause breathing difficulties and coughing. Adults may develop a low-grade fever, along with lung inflammation and sensitivity to light.
A full history and physical should be done to look for any associated underlying disease, such as cancer. Here are some common diagnostic tests for dermatomyositis:
Blood tests may be done to look for signs of muscle inflammation and abnormal proteins called antibodies, which form in autoimmune disease. The most common blood tests include muscle enzyme creatine kinase and the antinuclear antibody.
Electromyelogram, or EMG, may be done to detect abnormal electrical activity in affected muscles.
MRI scan may be done to look for inflammation.
Skin or muscle biopsy may be done to look for tissue changes caused by dermatomyositis.
Dermatomyositis can't be cured, but treatment helps control the signs and symptoms of the disease for most people. There is no single best treatment for dermatomyositis—you may need more than one kind of treatment, and your treatment may need to be changed over time.
Common treatments include:
Physical therapy. You may need special exercises to stretch and strengthen your muscles. As part of your therapy, orthotics or assistive devices may be recommended.
Skin treatment. You may need to avoid sun exposure and wear sunscreen to help prevent skin rashes. Your doctor can treat itchy skin rashes with antihistamine drugs or with anti-inflammatory steroid creams that are applied to the skin.
Anti-inflammatory medications. Steroid drugs, or corticosteroids, that block inflammation may be given by mouth or directly into the bloodstream.
Immunosuppressive drugs. If steroid drugs are not working for you, other options include drugs that block or slow down, or suppress, your body's immune system. These include the drugs azathioprine, methotrexate, cyclosporine A, cyclophosphamide, and tacrolimus.
Immunoglobulin. If you have not responded to other treatments, these drugs may be given directly into your bloodstream. Immunoglobulins are donated blood products that may boost your body's immune system.
Surgery. Surgery may be done to remove the calcinosis, or calcium deposits, under the skin if they become painful or infected.
If you have dermatomyositis, you may need to be treated for the rest of your life. It's important to learn as much as you can about your disease and work closely with your doctor. Researchers are actively studying causes and treatments for dermatomyositis. It is likely that early diagnosis and treatment for this disease will continue to improve over time.