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. 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. CONCLUSION: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.

journal_name

BMC Gastroenterol

journal_title

BMC gastroenterology

authors

Pan A,Doyle T,Schlup M,Lubcke R,Schultz M

doi

10.1186/1471-230X-11-77

subject

Has Abstract

pub_date

2011-06-22 00:00:00

pages

77

issn

1471-230X

pii

1471-230X-11-77

journal_volume

11

pub_type

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