Definition: 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)
coord IM with PARANEOPLASTIC SYNDROMES (IM) if pertinent
Other names Polymyositis-Dermatomyositis; Polymyositis-Dermatomyositides; Polymyositis Dermatomyositis; Dermatopolymyositides; Dermatomyositides, Childhood Type; Dermatomyositides, Adult Type; Dermatomyositides; Childhood Type Dermatomyositis; Childhood Type Dermatomyositides; Adult Type Dermatomyositis; Adult Type Dermatomyositides; Dermatopolymyositis; Dermatomyositis, Childhood Type; Dermatomyositis, Adult Type
