Unusual manifestation of Erdheim-Chester disease
| Autor: | R. Lübcke, Terence Doyle, Antony Pan, Martin Schlup, Michael Schultz |
|---|---|
| Jazyk: | angličtina |
| Předmět: |
Male
Erdheim-Chester Disease Pathology medicine.medical_specialty Hydrocortisone Gastrointestinal Diseases Case Report Retroperitoneal fibrosis Diagnosis Differential Prednisone Internal medicine medicine Humans lcsh:RC799-869 Aged CD68 business.industry Gastroenterology General Medicine Hepatology medicine.disease Magnetic Resonance Imaging Histiocytosis Erdheim–Chester disease Vomiting lcsh:Diseases of the digestive system. Gastroenterology medicine.symptom Differential diagnosis Tomography X-Ray Computed business medicine.drug |
| Zdroj: | BMC Gastroenterology, Vol 11, Iss 1, p 77 (2011) BMC Gastroenterology |
| ISSN: | 1471-230X |
| DOI: | 10.1186/1471-230x-11-77 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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