Necrotizing myopathy with pipestem capillaries and minimal cellular infiltration: a case associated with cutaneous signs of dermatomyositis

Neurology. 1996 May;46(5):1448-51. doi: 10.1212/wnl.46.5.1448.

Abstract

In 1991, Emslie-Smith and Engel described a distinct form of idiopathic inflammatory myopathy they called "necrotizing myopathy with pipestem capillaries, microvascular deposition of the complement membrane attack complex (MAC) and minimal cellular infiltration." We describe a patient with exercise-dependent painful myopathy related to a necrotizing myopathy with pipestem capillaries in whom mild cutaneous signs of dermatomyositis were detected 7 years after onset and who subsequently developed multiple cerebral infarcts.

Publication types

  • Case Reports

MeSH terms

  • Capillaries / pathology*
  • Capillaries / ultrastructure
  • Cerebral Infarction
  • Dermatomyositis / pathology*
  • Dermatomyositis / physiopathology
  • Electromyography
  • Endothelium, Vascular / pathology
  • Endothelium, Vascular / ultrastructure
  • Follow-Up Studies
  • Humans
  • Inflammation
  • Macrophages / pathology
  • Male
  • Microscopy, Electron
  • Middle Aged
  • Muscle Fibers, Skeletal / pathology
  • Muscle Fibers, Skeletal / ultrastructure
  • Muscle, Skeletal / pathology*
  • Muscle, Skeletal / ultrastructure
  • Necrosis
  • Pain
  • Vascular Diseases / pathology*