Cases reported "Behcet Syndrome"

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1/4. Behcet's disease complicated by pylephlebitis and hepatic abscesses.

    A 22 year old man presented with fever, abdominal pain, weight loss and diarrhea. Past medical history revealed recurrent aseptic meningitis, uveitis, and erythema nodosum. Further inquiry unveiled a prominent history of oral aphthous ulcers; all features of Behcet's disease. Imaging revealed mesenteric arteritis and pylephlebitis, septic thrombophlebitis of the portal vein, a previously unrecognized complication of Behcet's disease, with multiple intrahepatic abscesses. Portal venography demonstrated an extensively diseased, expanded, and obstructed portal venous system. blood cultures and portal vein aspirate yielded polymicrobial flora. Percutaneous intraportal thrombolytic therapy and mechanical thrombectomy were attempted to restore flow to the portal venous system. This distinctly rare manifestation of Behcet's disease, pylephlebitis, may result from ischemic injury and structural compromise of the bowel mucosa, resulting from underlying vasculitis.
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2/4. Lethal fulminate S. aureus sepsis in M. Behcet overnight cold exposure.

    Whether patients with Behcet's disease (BD) and immunosuppressive therapy are generally prone to acquire severe infectious diseases is unknown. A 48-year-old man under corticosteroids and azathioprine for BD since 1995 was admitted because of a transitory ischemic attack. Between the third and fourth hospital day he was accidentally locked up, insufficiently dressed, in the hospital's chapel over night. On the following day, he developed fever and deteriorating consciousness until he became comatose. CT scans of the brain were normal and there was only a slight pleocytosis. Despite adequate therapy, the patient's condition further deteriorated such that he died. Responsible for his decline was a fulminate sepsis, diagnosed upon fever, increased c-reactive protein, thrombocyte decline, multi-organ failure, rhabdomyolysis, growth of S. aureus on blood culture, and autopsy. patients with BD and immunosuppressive therapy, may be more vulnerable to infections and may develop lethal overwhelming sepsis already after overnight cold exposure.
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3/4. Arterial aneurysms in Behcet's disease.

    Six cases of arterial aneurysms, due to "Behcet's disease" located in various arteries are presented. Surgical reconstruction included: Resection of the lesions and replacement with corresponding grafts with satisfactory results. In spite of the macroscopic similarity of the aneurysms found in "Behcet's disease" as compared to those of mycotic origin, the presence of any microorganism could not be detected in various cultures or pathological specimens. Meticulous diagnosis on the nature of the aneurysm, close follow-up and careful postoperative anticoagulant treatment, is imperative for satisfactory immediate and late results.
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4/4. Treatment of colitis in Behcet's disease with thalidomide.

    A 40-year-old Turkish man on busulphan treatment for chronic myeloid leukaemia was hospitalized with steroid resistant (up to 100 mg prednisolone/24 h for over 4 weeks) colitis. On the basis of concomitant orogenital ulceration and unusual, deep punched out ulcers in the colon and smaller ulcers in the terminal ileum, Behcet's disease of the bowel, manifesting as ileocolitis, was diagnosed. Leukaemic infiltration, Crohn's colitis, neutropenic colitis and infectious colitis were excluded by histology and cultures. Treatment with colchicine 2 mg/day improved the orogenital ulcers but with only minor improvement of the colitis. thalidomide treatment (400 mg/day), however, dramatically improved the colitis within 7 days: fever and diarrhoea disappearing. colonoscopy after 4 weeks of treatment revealed previously giant ulcers practically healed.
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