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26 April 2012 - Case of the Week #240
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Thanks to Dr. J. J. Sola, Clnica Universidad de Navarra, Pamplona (Spain), for contributing this case. To contribute a Case of the Week, follow the guidelines on our Case of the Week page.
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Case of the Week #240
A 46 year old woman had a chronic history of abdominal discomfort. Five years ago, an ileal biopsy showed non-specific chronic erosive inflammation, and Crohn disease was clinically diagnosed. After a CT scan showed "tumoral enlargement of the ileal wall", her terminal ileum was resected.
Top row: H&E; bottom row: trichrome
What is your diagnosis?
Small bowel diaphragm disease
Diaphragm disease was first described in 1988, based on a review of small bowel resections to assess whether they exhibited any pathology related to non steroidal anti-inflammatory drugs/NSAIDs (J Clin Pathol 1988;41:516). A characteristic finding was the segmentation of the ileum by the formation of incomplete mucosal diaphragms, defined as thin circumferential membranes resembling the plica circularis, and composed of mucosa and submucosa with accompanying fibrosis. Other common gross features included ulceration, strictures and perforation. Histologically, these specimens also show neuromuscular and vascular hamartoma-like changes, eosinophilic enteritis and acute inflammation (Am J Clin Pathol 2008;130:518).
The differential diagnosis includes cryptogenic multifocal ulcerous stenosing enteritis (CMUSE), defined as small intestinal strictures and ulcerations of unknown origin (World J Gastroenterol 2011;17:2873). It is characterized as an atypical vasculitis presenting with unexplained stricture and ulceration of the small bowel in young and middle-aged patients, but without systemic inflammation. Of note, CMUSE can be treated with steroids.
Patients with diaphragm disease are often treated with surgery (Colorectal Dis 2011 Aug 3 [Epub ahead of print]) or endoscopic balloon dilation (Gastrointest Endosc 2006;64:1014). Cessation of NSAID use is recommended.
In this case, after the pathological diagnosis, a history of chronic low-dose aspirin was discovered.
Nat Pernick, M.D., President
and Liz Parker, B.A., Associate Medical Editor
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