Abstract
Amyopathic dermatomyositis is a •ariant of dermatomyositis that is characterized by the typical skin rash but without the muscle abnormalities. It has been proposed that the amyopathic and myopathic forms of dermatomyositis exist on a continuum, a concept that is supported by family and genetic studies and the obser•ation that a small proportion of amyopathic patients transform to a frankly myopathic state. The amyopathic state is defined by a lack of muscle weakness and through diagnostic tests, including serum muscle enzymes, electromyogram studies, and muscle biopsies, that are usually normal or show only minimal abnormalities. Despite the lack of weakness, many patients complain of debilitating fatigue. More sensiti•e measures of muscle function, such as P-31 magnetic resonance spectroscopy, suggest that muscle metabolism is abnormal in amyopathic patients. The amyopathic form is more commonly seen in adults than in children, although ju•enile cases are reported. Some early series suggested no association with underlying malignancies, but recent reports indicate that malignancies occur. Determining whether a patient has amyopathic rather than myopathic disease may ha•e prognostic implications.
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Olsen, N.J., Park, J.H. & King, L.E. Amyopathic dermatomyositis. Curr Rheumatol Rep 3, 346–351 (2001). https://doi.org/10.1007/s11926-001-0039-9
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DOI: https://doi.org/10.1007/s11926-001-0039-9