
Erdheim-Chester Disease: A Case Report and Review of the Literature
Author(s) -
Hema Merai,
David Collas,
Ashish Bhagat,
Uday Mandalia
Publication year - 2020
Publication title -
journal of clinical imaging science
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.279
H-Index - 18
eISSN - 2156-7514
pISSN - 2156-5597
DOI - 10.25259/jcis_68_2020
Subject(s) - medicine , erdheim–chester disease , disease , pathology , histiocytosis
Erdheim-Chester disease (ECD) is a rare form of non-Langerhans’ cell histiocytosis characterized by xanthogranulomatous infiltration of foamy histiocytes surrounded by fibrosis. ECD may be asymptomatic or present as a multi-systemic disease with life-threatening manifestations, most commonly involving the skeletal system. Immunohistochemical staining demonstrates cells that are CD68+, CD1a–, and S100– with an absence of Birbeck granules. We report a case of a 69-year old male patient who presented with neurological symptoms – eventually thought to be separate to his diagnosis of ECD. It represents the ability to diagnose ECD based just on radiological findings in an otherwise asymptomatic individual.