Abstract

DERMATOMYOSITIS is an inf lammatory disease of muscle present ing as a syndrome of proximal muscle weakness accompanied by an ery thematous skin rash. Classical his topathologic features on muscle biopsy, as given in order of f requency by Pearson, 1 are degenera t ion , regenerat ion and necrosis o f muscle fibers, in f l ammatory infiltration, var ia t ion in fiber size, and intersti t ial fibrosis. The under lying lesion in some chi ldhood cases, however, appears to be ischemia caused by a vasculopathy of small veins, arteries, and capillaries. ~-4 The finding by Whi t ake r and EngeP of vessel wall deposits of IgM together with a variable deposi t ion of IgG and C3 in n ine of 11 muscle biopsies f rom cases of ch i ldhood dermatomyosi t i s suggests that the vascular lesions migh t be caused by the deposi t ion o f autoant ibodies or i m m u n e complexes, and therefore would be amenable to p lasmapheres is therapy.

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