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Dermatomyositis

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

Dermatomyositis affects adults and children alike. In adults, dermatomyositis usually occurs from the late 40s to early 60s. In children, the disease 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 remission — when symptoms improve spontaneously — may occur. Treatment can clear the skin rash and help you regain muscle strength and function.

Symptoms Causes Complications

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 areas around your nails, knuckles, elbows, knees, chest and back. The rash, which can be patchy with bluish-purple discolorations, 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.

The exact cause of dermatomyositis is unknown, but the disease shares many characteristics with autoimmune disorders, in which your immune system mistakenly attacks your own body tissues.

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

Possible complications of dermatomyositis include:

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

Associated conditions

Dermatomyositis may cause other conditions or put you at higher risk of developing them. These conditions include:

  • Raynaud's phenomenon. This is a condition in which your fingers, toes, cheeks, nose and ears 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 in combination with dermatomyositis.
  • Cardiovascular disease. Dermatomyositis may cause the muscular walls of your heart to become inflamed (myocarditis). In a small number of people who have dermatomyositis, congestive heart failure and heart arrhythmias may develop.
  • Lung disease. A condition called interstitial lung disease may 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. The diagnosis of cancer may also happen before you develop dermatomyositis.
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