Cerebral Microvascular Erdheim-Chester Disease: A Perivascular Hematopoietic Vasculopathy

Cerebrovasc Dis. 2021;50(6):746-751. doi: 10.1159/000516803. Epub 2021 Jul 6.

Abstract

Erdheim-Chester disease (ECD) is a rare and elusive hematopoietic malignancy that may involve the nervous system in various ways. Cerebrovascular ECD involves the perivascular infiltration and compromise of any cervicocranial vessel by transformed proliferating histiocytes. Presented is the novel case of a patient with pathologically proven perivascular microangiopathy, manifesting in multifaceted fashion with ischemia, hemorrhage, mass lesions, and edema.

Keywords: Autopsy; Cerebral vasculature; Erdheim-Chester disease; Hemorrhage; Infarction; Microvascular infiltrates; Perivascular microangiopathy.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cerebral Small Vessel Diseases* / diagnosis
  • Erdheim-Chester Disease* / diagnosis
  • Histiocytes / pathology
  • Humans