Unusual manifestation of Erdheim-Chester disease
Open Access
- 22 June 2011
- journal article
- case report
- Published by Springer Nature in BMC Gastroenterology
- Vol. 11 (1) , 77
- https://doi.org/10.1186/1471-230x-11-77
Abstract
Erdheim-Chester disease (ECD) is a rare multisystem non-Langerhans cell histiocytosis that is characterized histologically by xanthogranulomatous infiltrates and radiologically by symmetrical sclerosis of long bones. The xanthomatous process is characterized by prominent foamy histiocytes staining positive for CD68, occasionally for PS100 and negative for S100 and CD1a. Gastroenterological involvement is exceedingly rare.Keywords
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