Arterial thrombosis leading to intestinal infarction in a patient with Beh?et's disease associated with protein C deficiency

Beh?et's disease may be a possible cause of both occlusive and aneurysmal arterial involvement as well as recurrent venous thrombosis. A case of Beh?et's disease complicated with vascular involvement leading to intestinal infarction is presented. A 41-yr-old man suffering from Beh?et's disease for 15 yr presented with a 2-day history of severe abdominal pain and bloody diarrhea. Intestinal infarction secondary to thrombosis of the superior mesenteric artery had been diagnosed during surgical exploration 3 yr previously. He was started on anticoagulation with nutritional support. The patient was readmitted with severe diarrhea and malabsorption symptoms 3 yr after intestinal resection. A thrombus located in the posterior wall of the infrarenal portion of aorta was detected by aortography and ultrasonography. Although thrombosis is a relatively common complication of Beh?et's disease caused by vasculitis, protein C deficiency, which is a pertinent laboratory finding in this case, might be a secondary factor in the thrombotic event. This is the first case reported of mesenteric artery thrombosis leading to bowel infarction and abdominal aorta thrombosis associated with protein C deficiency.