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Dermatomyositis
Although dermatomyositis may occur at any age, it mostly affects adults in their late 40s to early 60s or children between 5 and 15 years of age. Women have it more often than men do. Dermatomyositis in children is distinct from the adult form. The disease usually develops over weeks or months. Dermatomyositis is a relatively rare disease marked by muscle weakness and a distinctive skin rash. Because of similarities in signs, symptoms and treatment, it's often discussed in conjunction with polymyositis. Both conditions fall into the category of inflammatory muscle diseases - myo means "muscles" in Greek; itis means "inflamed." Derma, which means "skin," implies the skin-related signs and symptoms that accompany the muscle inflammation. Globally, dermatomyositis and polymyositis together affect about 5 to 10 people out of 100,000. Other inflammatory muscle diseases include inclusion body myositis, which progresses more slowly than other forms; myositis associated with other connective tissue diseases, such as lupus or scleroderma; and myositis associated with cancer (malignancy). Periods of remission, during which signs and symptoms improve spontaneously, may occur. Treatment can improve your skin and your muscle strength and function. Signs and symptoms The most common signs and symptoms of dermatomyositis include:
The skin rash usually occurs at the same time as muscle weakness, but may precede it by a few weeks. Sometimes, the skin rash alone determines the diagnosis. In some children with dermatomyositis, the skin may become thick and hard in a way similar to scleroderma. When this happens, the condition is called sclerodermatomyositis. Weakness in muscles such as your hips and shoulders can lead to difficulty in getting out of chairs, climbing stairs, brushing your hair or working with your arms over your head. Weakness in your neck muscles can make it hard to hold your head up. Causes Dermatomyositis belongs to a group of conditions called inflammatory myopathies. Myopathies are diseases or abnormal conditions of the striated muscles that cover your skeleton. The cause of most inflammatory myopathies is unknown. Doctors suspect that these are autoimmune disorders, in which your body's immune system attacks itself. Infections caused by bacteria, parasites or viruses can cause inflammatory myopathies, but in most cases, doctors aren't able to identify an infection in dermatomyositis. Some doctors think certain people may have a genetic susceptibility to the disease. Typically, your immune system works to protect your healthy cells from attacks by foreign substances, such as bacteria and viruses. If you have dermatomyositis, an unknown cause seems to trigger your immune system to begin producing autoimmune antibodies (also called autoantibodies) — immune cells that attack the body's own tissues. Many people with dermatomyositis show a detectable level of autoantibodies in their blood. It's unclear whether these autoantibodies are involved in causing dermatomyositis.
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