Abstract

Dermatomyositis (DM) is an idiopathic inflammatory myopathy traditionally characterized by a progressive, symmetrical proximal muscle weakness and pathognomonic or characteristic cutaneous manifestations. In some patients with DM, cutaneous disease exists in the absence of objective evidence of muscle inflammation, and these cases are referred to as amyopathic DM. Management of DM begins with careful investigation for the presence of muscle disease or of additional systemic involvement, particularly of the pulmonary, cardiac or gastrointestinal systems, and for the possibility of an accompanying malignancy. The presence of muscle or systemic involvement or the presence of malignancy alters the initial approach to management. Muscle disease and systemic involvement can be refractory and may require multiple sequential therapeutic interventions or, at times, combinations of therapies. In addition, even after these disease components are controlled, the cutaneous disease of DM can be particularly challenging...

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