title: Erdheim Chester - a rare disease with unique endoscopic features.
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.
authors.affiliation: 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.
authors.forename: Gil
authors.initials: G
authors.lastname: Ben-Yaakov
authors.affiliation: 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.
authors.forename: Daniela
authors.initials: D
authors.lastname: Munteanu
authors.affiliation: 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.
authors.forename: Ignacio
authors.initials: I
authors.lastname: Sztarkier
authors.affiliation: 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.
authors.forename: Alexander
authors.initials: A
authors.lastname: Fich
authors.affiliation: 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.
authors.forename: Doron
authors.initials: D
authors.lastname: Schwartz
mesh_terms: D000328:Adult; D015415:Biomarkers / Q000032:analysis; D001706:Biopsy; D003106:Colon / Q000737:chemistry / Q000187:drug effects / Q000473:pathology*; D003113:Colonoscopy; D003967:Diarrhea / Q000209:etiology; D004359:Drug Therapy, Combination; D031249:Erdheim-Chester Disease / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D005221:Fatigue; D005260:Female; D005773:Gastroscopy; D006801:Humans; D007150:Immunohistochemistry; D011141:Polyuria / Q000209:etiology; D011237:Predictive Value of Tests; D011241:Prednisone / Q000627:therapeutic use; D013270:Stomach / Q000737:chemistry / Q000187:drug effects / Q000473:pathology*; D016896:Treatment Outcome; D014747:Vinblastine / Q000627:therapeutic use; D015431:Weight Loss
chemical_list: D015415:Biomarkers; D014747:Vinblastine; D011241:Prednisone
keywords: Colon; Erdheim chester; Histiocytes; Lamina propria; Stomach