MULTIFOCAL STENOSING ULCERATIONS OF THE SMALL-INTESTINE REVEALING VASCULITIS ASSOCIATED WITH C2 DEFICIENCY

A patient with cryptogenic multifocal ulcerous stenosing enteritis cha racterized by repeated bouts of intestinal obstruction, ulcerative ste nosis of the small bowel relapsing after surgical resection, and stero id sensitivity is described. Fourteen strictures of the jejunum were f ound at laparotomy. Despite resection, abdominal pain persisted. Stero id therapy was effective but led to dependence. In our patient, crypto genic multifocal ulcerous stenosing enteritis was associated with feve r, asthma, Raynaud's phenomenon, sicca syndrome, heterozygous type I C 2 deficiency (28-base pair gene deletion), stenosis, and aneurysms on selective mesenteric angiography. It is hypothesized that cryptogenic multifocal ulcerous stenosing enteritis might be related to a particul ar form of polyarteritis nodosa with mainly intestinal expression or t o a yet unclassified independent vasculitis.