Cases reported "Behcet Syndrome"

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1/77. A case of pediatric Behcet's disease with intestinal involvement.

    We report a 6-year-old girl with recurrent oral aphthae, genital and perianal ulcers, and folliculitis-like erythema followed by intestinal symptoms. No ocular lesions were found. Her ailment was diagnosed as incomplete Behcet's disease with intestinal involvement. According to the literature, pediatric Behcet's disease is characterized by a low incidence of ocular lesions and a high incidence of intestinal involvement, as exhibited in this case. As intestinal lesions in pediatric Behcet's disease are often life-threatening, a barium enema must be given to exclude the possibility of intestinal involvement when pediatric patients with Behcet's disease complain of abdominal pain.
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2/77. Behcet's disease and pheochromocytoma.

    A 46-year-old black female with a history of Behcet's disease was admitted with a diagnosis of abdominal pain. She had complained of palpitations, diaphoresis, anxiety, and fatigue for years which had been variously attributed to the perimenopause and her Behcet's. During her hospital evaluation, she had an episode of supraventricular tachycardia associated with severely elevated blood pressure. Subsequent evaluation revealed a large pararenal mass which was found to be a pheochromocytoma. Search of six databases revealed this to be only the second case report in the English literature of a pheochromocytoma in association with Behcet's disease.
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3/77. Gastrointestinal manifestations of Behcet's disease.

    Behcet's disease (BD) is a multisystem, chronic, relapsing vasculitis of unknown origin that affects nearly all organs and systems. While recurrent oral ulcerations are a "sine qua non" of BD, the frequency of extra-oral parts of the gastrointestinal involvement varies widely in different countries. The most frequent extra-oral sites of gastrointestinal involvement are the ileocecal region and the colon. The liver (except with budd-chiari syndrome), pancreas, and spleen are rarely involved. The symptoms associated with these extra-oral manifestations of BD are abdominal pain, nausea, vomiting, diarrhea with or without blood, and constipation. The lesions typically are resistant to medical treatment and frequently recur with surgical treatment. We review the literature regarding the gastrointestinal and hepatobiliary systems in BD. Also, we present a patient who had BD complicated with radiologically-proven hepatic veins involvement (budd-chiari syndrome) and complete occlusion of hepatic portion of inferior vena cava and who had a good response to colchicine and penicillin treatment.
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4/77. Neuro-Behcet's syndrome in a patient not fulfilling criteria for Behcet's disease: clinical features and value of brain imaging.

    central nervous system involvement is rarely an initial presenting manifestation of Behcet's disease (BD). We report the case of a 33-year-old man with recurrent attacks of fever, oral mucosal ulcers, deep venous thrombosis, diplopia, vertigo and headache. Sequential brain magnetic resonance imaging (MRI) scans showed fluctuating lesions of the brain stem, mesencephalon and thalamus. F-18-fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) revealed hypometabolism at the parieto-occipital cortex at both sides and the brain stem. Treatment with prednisone and cyclosporine A led to a complete remission and normalisation of MRI and FDG-PET lesions. The present case illustrates the difficulty in the differential diagnosis of early neuro-BD.
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5/77. Treatment of intestinal Behcet's syndrome with chimeric tumour necrosis factor alpha antibody.

    Few patients with Behcet's syndrome have gastrointestinal ulceration. Such patients are difficult to treat and have a higher mortality. Faced with refractory symptoms in two patients with intestinal Behcet's, we used the tumour necrosis factor alpha (TNF-alpha) monoclonal antibody infliximab to induce remission. Both women (one aged 27 years, the other 30 years) presented with orogenital ulceration, pustular rash, abdominal pain, bloody diarrhoea due to colonic ulceration, weight loss, and synovitis. One had thrombophlebitis, digital vasculitis, perianal fistula, and paracolic abscess; the other had conjunctivitis and an ulcer in the natal cleft. Treatment with prednisolone, methyl prednisolone, and thalidomide in one and prednisolone, colchicine, and cyclosporin in the other was ineffective. After full discussion, infliximab (3 mg/kg, dose reduced because of recent sepsis in one, and 5 mg/kg in the other) was administered. Within 10 days the ulcers healed, with resolution of bloody diarrhoea and all extraintestinal manifestations. A second infusion of infliximab was necessary eight weeks later in one case, followed by sustained (>15 months) remission on low dose thalidomide. Remission was initially sustained for 12 months in the other but thalidomide had to be stopped due to intolerance, and a good response to retreatment lasted only 12 weeks without immunosuppression, before a third infusion. The cause of Behcet's syndrome is unknown but peripheral blood CD45 gammadelta T cells in Behcet's produce >50-fold more TNF-alpha than controls when stimulated with phorbol myristate acetate and anti-CD3. Infliximab could have a role for inducing remission in Behcet's syndrome.
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6/77. Recurrent giant longitudinal duodenal ulcer with massive hemorrhage in a helicobacter pylori-negative patient.

    A 67-year-old man, in whom a linear ulcer running from the duodenal bulb to the descending part had been noted 3 years previously, was admitted to our hospital because of abdominal pain and melena. duodenoscopy revealed a bleeding giant longitudinal ulcer, which was more extensive than before. Tests for Helicobacter pylori (Hp) were negative. The ulcer was cured by endoscopic hemostasis and repeated blood transfusions. attention must be paid to Hp-negative post-bulbar duodenal ulcers because of the frequent complications including hemorrhage.
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7/77. Massive thrombosis after central venous catheterization in a patient with previously undiagnosed Behcet's disease.

    Thrombosis is an important complication of central venous catheterization. Among the many intrinsic and extrinsic factors, the patient's medical disease can play a role in thrombogenesis. Behcet's disease (BD), classified as a vasculitis, is a multisystem disease involving the small blood vessels. It is often difficult to recognize and diagnose the disease. A 24-yr-old female patient showed massive central venous thrombosis which caused superior vena cava syndrome after subclavian vein catheterization. Twenty days after catheterization, the patient exhibited swelling of the face, neck, and both upper extremities. Despite thrombectomy and continuous anticoagulation therapy, her facial and upper extremity swelling reappeared and follow-up chest computed tomography (CT) showed the recurrent thrombosis in the same central veins previously affected. A diagnosis of BD was then made. Following steroid therapy, neither clinical symptoms nor CT findings suggestive of central venous thrombosis were observed during the subsequent 6-months of follow-up period. This case emphasizes that central venous catheterization in a patient with BD should be performed with great caution.
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keywords = chest
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8/77. 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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9/77. optic nerve involvement in neuro-Behcet's disease.

    BACKGROUND: To report the ocular manifestations of neuro-Behcet's disease. CASES: A 34-year-old woman had recurrent meningitis. She developed diplopia, headache, and ataxia, and was diagnosed as having neuro-Behcet's disease. OBSERVATIONS: Imaging revealed two infarct foci in the transitional midbrain and pons. After treatment with prednisolone and colchicine, the diplopia resolved. Two years later, a scotoma developed in the right eye, in which the best-corrected visual acuity was 4/200. Papillitis and a prepapillary vitreous opacity were seen in the right fundus. These findings disappeared 11 days after subconjunctival steroid injections and increased colchicine. Her vision gradually improved to 20/20 two months later. CONCLUSION: Neuro-Behcet's disease may manifest with transient optic neuritis and prepapillary vitreous opacity.
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keywords = headache
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10/77. A case of Behcet's disease accompanied by colitis with longitudinal ulcers and granuloma.

    A 37-year-old female presenting with oral and genital ulcers and erythema nodosum on both arms was diagnosed as having Behcet's disease. The symptoms resolved spontaneously. However, she was admitted to our hospital (Keio University Hospital) several months later because of fever, aphthous ulcers of the oral cavity, lower abdominal pain and frequent diarrhea. A colonoscopic examination revealed multiple ulcers including longitudinal ulcers in the ascending and transverse colon, and histological examination of biopsied specimens demonstrated non-caseating epithelioid granuloma. Treatment with prednisolone and 5-aminosalicylic acid was started, and the patient responded well clinically. One month later, a repeated colonoscopy confirmed that the lesions including longitudinal ulcers had disappeared. In this report, we describe our experience of this rare case of Behcet's disease concomitant with colonic longitudinal ulcers and epithelioid granuloma, and discuss the difficulties in making a differential diagnosis, primarily with regard to Crohn's disease.
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