Disease Information for Dermatomyositis: Definition

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  • A subacute or chronic inflammatory disease of muscle and skin, marked by proximal muscle weakness and a characteristic skin rash; The illness occurs with approximately equal frequency in children and adults; The skin lesions usually take the form of a purplish rash (or less often an exfoliative dermatitis) involving the nose, cheeks, forehead, upper trunk, and arms; The disease is associated with a complement mediated intramuscular microangiopathy, leading to loss of capillaries, muscle ischemia, muscle-fiber necrosis, and perifascicular atrophy; The childhood form of this disease tends to evolve into a systemic vasculitis; Dermatomyositis may occur in association with malignant neoplasms-----(From Adams et al, Principles of Neurology, 6th ed, pp1405-6)----------

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