title: Unusual manifestation of Erdheim-Chester disease.
abstract: BACKGROUND
  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.
  
  
  METHODS
  This case report describes the case of a 69-year-old man who presented otherwise well to the gastroenterology department with unspecific abdominal symptoms, nausea, vomiting and weight loss. ECD involving the gastrointestinal tract was confirmed clinically, radiologically and histologically.
  
  
  CONCLUSIONS
  Gastroenterological manifestation of ECD is rare but should be considered in the differential diagnosis in patients presenting with evidence of multi-organ disease and typical radiological features of Erdheim-Chester disease elsewhere.
authors.affiliation: Gastroenterology Unit, Southern District Health Board, Dunedin, New Zealand.
authors.forename: Antony
authors.initials: A
authors.lastname: Pan
authors.forename: Terence
authors.initials: T
authors.lastname: Doyle
authors.forename: Martin
authors.initials: M
authors.lastname: Schlup
authors.forename: Ralf
authors.initials: R
authors.lastname: Lubcke
authors.forename: Michael
authors.initials: M
authors.lastname: Schultz
mesh_terms: D000368:Aged; D003937:Diagnosis, Differential; D031249:Erdheim-Chester Disease / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality; D005767:Gastrointestinal Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality; D006801:Humans; D006854:Hydrocortisone / Q000008:administration & dosage; D008279:Magnetic Resonance Imaging; D008297:Male; D011241:Prednisone / Q000008:administration & dosage; D014057:Tomography, X-Ray Computed
chemical_list: D011241:Prednisone; D006854:Hydrocortisone