EC Gastroenterology and Digestive System

Case Report Volume 11 Issue 5 - 2024

Case Study and Bibliographic Review of Erdheim-Chester Disease

Abderrazzak Ajertil1*, Hakima Rhou2 and Mohammed Cherkaoui Malki1

1Department of Radiology, Cheikh Zayd International University Hospital, UIASS, Rabat, Morocco
2Department of Nephrology, Cheikh Zayd International University Hospital, UIASS, Rabat, Morocco
*Corresponding Author: Abderrazzak Ajertil, Radiology Department, Cheikh Zayd International University Hospital, UIASS, Rabat, Morocco.
Received: April 17, 2024; Published: May 06, 2024




Erdheim-Chester Disease (ECD) is a rare form of non-Langerhans cell histiocytosis, characterized by xanthogranulomatous infiltration of foamy histiocytes surrounded by fibrosis. The disease can manifest asymptomatically or as a potentially severe multisystemic disease, with a predominance of involvement in the skeletal system. Immunohistochemistry reveals cell positivity for CD68, negative CD1a, and negative S100, with an absence of Birbeck granules. We present the case of a 66-year-old man with neurological, cardiac, renal, peritoneal symptoms, and cutaneous signs.

 Keywords: Erdheim-Chester Disease; Histiocytosis; Hairy Kidney Sign; Renal Insufficiency; Enrobed Aorta Sign

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Abderrazzak Ajertil., et al. “Case Study and Bibliographic Review of Erdheim-Chester Disease".  11.5 (2024): 01-05.

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