Case Report
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World J Gastroenterol. Jul 7, 2014; 20(25): 8309-8311
Published online Jul 7, 2014. doi: 10.3748/wjg.v20.i25.8309
Erdheim chester - A rare disease with unique endoscopic features
Gil Ben-yaakov, Daniela Munteanu, Ignacio Sztarkier, Alexander Fich, Doron Schwartz
Gil Ben-yaakov, Daniela Munteanu, Alexander Fich, Doron Schwartz, Institute of Gastroenterology and Hepatology, Soroka University Medical Center, Faculty of Health Science Ben Gurion University of the Negev, Beer sheva 84101, Israel
Ignacio Sztarkier, Institute of Pathology, Soroka University Medical Center, Faculty of Health Science, Ben Gurion University of the Negev, Beer sheva 84101, Israel
Author contributions: Ben-yaakov G and Munteanu D were the patient endoscopist; Schwartz D was senior endoscopist and case manager; Fich A was the case counselor; Sztarkier I was involved in the case as pathologist.
Correspondence to: Gil Ben-yaakov, MD, Institute of Gastroenterology and Hepatology, Soroka University Medical Center, Faculty of Health Science Ben Gurion University of the Negev, Beer sheva 84101, Israel. gilbenyakov@gmail.com
Telephone: +972-528-433388 Fax: +972-86-233083
Received: August 3, 2013
Revised: September 23, 2013
Accepted: October 19, 2013
Published online: July 7, 2014
Abstract

Erdheim-Chester disease (ECD) is a rare inflammatory syndrome in which systemic infiltration of non-Langerhans cell histiocytes occurs in different sites. Both the etiology and pathophysiology of ECD are unknown, but CD68 positive CD 1a/S100 negative cells are characteristic. The presentation of ECD differs according to the involved organs. This case report describes a patient with ECD and the gastrointestinal manifestations and unique endoscopic appearance as seen in gastroscopy and colonoscopy with histological proof of histiocyte infiltration of the lamina propria. The clinical and endoscopic findings of this unique case, to our knowledge, were never described before, so were the features of the gastrointestinal involvement in this disease.

Keywords: Erdheim chester, Histiocytes, Lamina propria, Colon, Stomach

Core tip: This case report describes a patient with Erdheim-Chester disease with gastrointestinal manifestations and unique endoscopic appearance as seen both in gastroscopy and colonoscopy with histological proof of histiocyte infiltration of the lamina propria. The clinical and endoscopic findings of this unique case, to the best of our knowledge, were never described before, so were the features of the gastrointestinal involvement in this disease.