Regional ischemic immune myopathy: A paraneoplastic dermatomyopathy

Chunyu Cai, Ali Alshehri, Rati Choksi, Alan Pestronk

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

Necrosis and regeneration of scattered muscle fibers are common features of many active acquired and immune myopathies. We studied a series of patients with acquired myopathies with an unusual pattern of regional, rather than scattered, muscle fiber necrosis and regeneration. Retrospective review of records of 7 patients with acquired myopathies having regional muscle fiber necrosis on muscle biopsy. Clinical features of patients included proximal symmetric weakness in arms and legs with a subacute onset (100%) beginning at ages between 41 and 92 years, with dysphagia (83%), myalgias (100%), skin rash (67%), and associated malignancy (71%). Serum creatine kinase was often very high (91,600 U/L) (83%). Survival was less than 1 year in 43%. Myopathology included a regional distribution with muscle fiber necrosis and capillary loss in the border zones between intermediatesized perimysial vessels, vascular pathology with damaged walls of intermediate-sized perimysial veins, and connective tissue with expression of the ischemia marker carbonic anhydrase IX but no mononuclear cell inflammatory foci. These data indicate that regional ischemic immune myopathies are likely caused by ischemia in border zones between damaged intermediate-sized perimysial blood vessels. Regional ischemic immune myopathies are a distinctive pathologic group of acquired, probably immune, noninflammatory dermatomyopathies with weakness and often a skin rash and systemic neoplasm.

Original languageEnglish (US)
Pages (from-to)1126-1133
Number of pages8
JournalJournal of Neuropathology and Experimental Neurology
Volume73
Issue number12
DOIs
StatePublished - Dec 8 2014

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Muscular Diseases
Necrosis
Muscles
Exanthema
Blood Vessels
Regeneration
Ischemia
Myalgia
Creatine Kinase
Deglutition Disorders
Connective Tissue
Veins
Neoplasms
Leg
Arm
Pathology
Biopsy
Survival
Serum

Keywords

  • Dermatomyositis
  • Immune
  • Ischemia
  • Myopathy
  • Vein

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neurology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

Cite this

Regional ischemic immune myopathy : A paraneoplastic dermatomyopathy. / Cai, Chunyu; Alshehri, Ali; Choksi, Rati; Pestronk, Alan.

In: Journal of Neuropathology and Experimental Neurology, Vol. 73, No. 12, 08.12.2014, p. 1126-1133.

Research output: Contribution to journalArticle

Cai, Chunyu ; Alshehri, Ali ; Choksi, Rati ; Pestronk, Alan. / Regional ischemic immune myopathy : A paraneoplastic dermatomyopathy. In: Journal of Neuropathology and Experimental Neurology. 2014 ; Vol. 73, No. 12. pp. 1126-1133.
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