Open Access Case report

Unusual manifestation of Erdheim-Chester disease

Antony Pan1, Terence Doyle23, Martin Schlup13, Ralf Lubcke1 and Michael Schultz13*

Author Affiliations

1 Gastroenterology Unit, Southern District Health Board, Dunedin, New Zealand

2 Department of Radiology, Southern District Health Board, Dunedin, New Zealand

3 Department of Medicine, University of Otago, Dunedin, New Zealand

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BMC Gastroenterology 2011, 11:77  doi:10.1186/1471-230X-11-77

Published: 22 June 2011



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.

Case Presentation

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.


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.