Dermatomyositis is an inflammatory skin myopathy, characterized by proximal skeletal muscle weakness as well as muscle inflammation.

There are also a variety of skin manifestations, including Gottrons sign, a heliotropic rash and the shawl sign. This condition also presents with many GI abnormalities: uncoordinated swallowing, uncoordinated esophageal peristalsis, hiatal hernia with reflux & stricture formation. Severe dysphagia occurs in 5-30% of these patients and while it can be debilitating, the use of plasma exchange has been found to improve such symptoms in these patients. In fact, intravenous immunoglobulin (IVIG)was found to have high efficacy in the treatment of refractory idiopathic myopathies.

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Cherin P, Auperin I, Bussel A, Pourrat J, Herson S

Plasma exchange in polymyositis and dermatomyositis: a multicenter study of 57 cases. Clin Exp Rheumatol 1995;13:270-1.

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de Merieux P, Verity MA, Clements PJ, Paulus HE

Esophageal abnormalities and dysphagia in polymyositis and dermatomyositis. Arthritis Rheum 1983;26:961-8

1. Dalakas MC, Hohlfeld R. Polymyositis and dermatomyositis. Lancet 2003; 362:971.

1. Plotz PH, Dalakas M, Leff RL, et al. Current concepts in the idiopathic inflammatory myopathies: polymyositis, dermatomyositis, and related disorders. Ann Intern Med 1989; 111:143.