Open AccessCerebral Microvascular Erdheim-Chester Disease: A Perivascular Hematopoietic Vasculopathy
Author(s) -
Michael Moussouttas,
Shanu F. Roemer,
Dennis W. Dickson
Publication year - 2021
Publication title -
cerebrovascular diseases
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.221
H-Index - 104
eISSN - 1421-9786
pISSN - 1015-9770
DOI - 10.1159/000516803
Subject(s) - medicine , erdheim–chester disease , pathology , perivascular space , microangiopathy , thrombotic microangiopathy , histiocyte , infiltration (hvac) , ischemia , malignancy , disease , histiocytosis , cardiology , physics , thermodynamics , diabetes mellitus , endocrinology
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.