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Weber-Christian disease associated with multiple perforations of the ileum and colon
H Kiguchi1, K Kasahara, M Mochizuki
1Department of Surgery, Jichi Medical School, Tochigi, Japan.
Abstract:
Panperitonitis developed in a 57-yr-old Japanese man during the course of Weber-Christian disease (WCD). Emergent operation disclosed three perforations of the ascending colon and two of the distal ileum. Histological study of the surgical specimen showed that all perforations were located at the centers of acute subserosal panniculitic foci. No vasculitis was discerned. In the previously reported three cases of WCD with intestinal perforation, perforation was solitary and seen in the small intestine. The cause of perforation was considered to be vasculitis in two cases. In the remaining one, panniculitis around the perforation was in chronic granulomatous stage. This was the first case of WCD in which acute subserosal panniculitis caused multiple ileocolonic perforations.
Insights
Weber-Christian disease (WCD) can cause acute subserosal panniculitis leading to multiple ileocolonic perforations. This rare presentation differs from previously reported WCD intestinal perforation cases.
Area of Science:
- Gastroenterology
- Pathology
Background:
- Weber-Christian disease (WCD) is a rare lipodystrophy characterized by recurrent, sterile, subcutaneous adipose tissue inflammation.
- Intestinal involvement in WCD is uncommon, with previous reports of perforation primarily involving the small intestine and often attributed to vasculitis.
Observation:
- A 57-year-old Japanese man with known Weber-Christian disease developed panperitonitis.
- Emergency surgery revealed multiple perforations: three in the ascending colon and two in the distal ileum.
- Histological examination of the perforations showed they were centered within acute subserosal panniculitic foci, with no evidence of vasculitis.
Findings:
- This case represents the first documented instance of Weber-Christian disease presenting with acute subserosal panniculitis causing multiple ileocolonic perforations.
- Unlike prior cases, the perforations were multiple and located in both the large and small intestines.
- The etiology of perforation in this patient was directly linked to acute panniculitis, not vasculitis or chronic granulomatous inflammation.
Implications:
- Highlights the potential for acute panniculitis in WCD to cause severe, multifocal gastrointestinal complications.
- Challenges the prevailing understanding of WCD-associated intestinal perforations, suggesting panniculitis itself can be the direct cause.
- Underscores the importance of considering WCD in the differential diagnosis of unexplained intestinal perforations, particularly when panniculitis is evident.