dermatomyosiitista

Dermatomyositis is a rare idiopathic inflammatory myopathy characterized by inflammation of the muscles and skin. The exact cause is unknown but is thought to involve a combination of genetic predisposition, environmental factors, and immune system dysfunction. It can affect individuals of any age but is more common in women and typically appears in adulthood, though a juvenile form also exists.

The hallmark symptoms include muscle weakness, often affecting muscles closest to the trunk, such as the shoulders, hips, and thighs. This weakness can make everyday activities like climbing stairs, standing up from a chair, or lifting objects difficult. Skin manifestations are also prominent and can precede or accompany muscle symptoms. These include a characteristic heliotrope rash, a purplish discoloration around the eyes, often accompanied by edema, and Gottron's papules, which are reddish-purple, scaly bumps over the knuckles. Other skin findings may include a malar rash on the face, a V-neck rash on the chest, and cuticular changes.

Diagnosis involves a combination of clinical evaluation, blood tests (including muscle enzymes and autoantibodies), electromyography (EMG) to assess muscle electrical activity, and muscle biopsy to examine for inflammation and damage. Skin biopsy may also be performed.

Treatment aims to reduce inflammation, improve muscle strength, and manage skin symptoms. It typically involves corticosteroids as a first-line therapy, often supplemented with immunosuppressive agents such as methotrexate or azathioprine for more severe or refractory cases. Intravenous immunoglobulin (IVIG) is another option, particularly for rapidly progressive disease or those unresponsive to other treatments. Physical therapy is crucial for maintaining muscle function and preventing contractures. Prognosis varies, with many patients experiencing significant improvement, though some may have long-term muscle weakness or complications.