Cases reported "Behcet Syndrome"

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1/36. Superimposed primary chancre in a patient with Adamantiades-Behcet's disease.

    Adamantiades-Behcet's disease was diagnosed in a 42 year old Turkish patient with recurrent oral aphthae, genital ulcerations, papules, and sterile pustules, histologically presenting as cutaneous vasculitis, and intermittent arthritis with joint effusion particularly of the knees. Six months after initial improvement under treatment with colchicine 2 mg/day, a solitary genital ulcer with enlarged inguinal lymph nodes appeared and persisted for 7 weeks despite the continuation of colchicine treatment and the introduction of clindamycin 2 mg/day intravenously. The unusual persistence of the ulcer and the failure of clindamycin therapy led to further differential diagnostic considerations and the identification of primary syphilis. The genital lesion healed 4 weeks after initiation of treatment with tetracycline 2 mg/day by mouth for 15 days.
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2/36. Encapsulated necrosis associated with Behcet's syndrome.

    We reported a case of a 45-year-old woman with encapsulated necrosis associated with Behcet's syndrome. The lesions were characterized by multiple, small subcutaneous nodules on the extremities. Histologically, a nodule consisted of degenerative adipocytes encapsulated by fibrous tissue. A membranocystic lesion was also observed in the cavity of the nodule. The patient had a long-term history of Behcet's syndrome and suffered from erythematous subcutaneous nodules, resembling erythema nodosum on the extremities with a tendency to recurrence. These observations indicate that Behcet's syndrome could be implicated in the development of encapsulated necrosis as an underlying disease.
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3/36. An adult case with an abnormal right ventricular structure causing intraventricular pressure gradient and a history of aphthous stomatitis and thrombophlebitis.

    We report a 50-year-old man with a right ventricular structure causing an intraventricular pressure gradient. He had been diagnosed as vasculo-Behcet with a history of aphthous stomatitis and thrombophlebitis. He had also been suffering from atrial flutter and mild right-side heart failure. echocardiography showed that there was an abnormal structure attached to the right ventricular free wall and protruding into the cavity, and that it caused the pressure gradient estimated to be approximately 19 mmHg. Chest X-ray computed tomography demonstrated that the structure was partially calcified. magnetic resonance imaging depicted the structure separating the right ventricle into two chambers. Angiographic study revealed a markedly enlarged right atrium and a filling defect at the mid-portion of the right ventricle, which divided the right ventricular cavity into two parts. Hemodynamic study showed a slightly elevated right atrial pressure (mean 7 mmHg) and a peak-to-peak intraventricular pressure difference of 18 mmHg in the right ventricle. The diastolic pressure tracing of the right ventricular low pressure chamber showed a 'dip and plateau' pattern. Although the pathological features of the abnormal right ventricular structure in this case were not fully clarified, abnormal muscle bundle and/or endocardial fibrosis, which were reported to be associated with Behcet's disease, may have contributed to its generation.
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4/36. Subpleural lung involvement in Behcet's disease: first localization of a systemic entity.

    Behcet's disease (BD) is a chronic multisystem vasculitis, affecting many organs and the vascular system, of unknown aetiology. Eyes, skin, joints, the oral cavity, the central nervous system, and, less frequently, heart, lung, kidney, the genital system and the gastrointestinal tract can be involved. Intrathoracic manifestations of BD consist mainly of thromboembolism of the superior vena cava and/or other mediastinal veins; aneurysms of the aorta and pulmonary arteries; pulmonary infarct and haemorrhage; pleural effusion; and, rarely, myocardial and/or hilar lymphoid involvement. In the present case, the patient presented with BD with an asymptomatic subpleural lung mass and bilateral pulmonary artery enlargement. The patient was treated with a combination of surgical and medical therapy with complete resolution of the lung involvement and without any parenchymal relapses after an 8-month follow-up.
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5/36. 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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6/36. Bilateral subclavian arterial aneurysm and ruptured abdominal aorta pseudoaneurysm in Behcet's disease.

    Behcet's disease is characterized by recurrent ulcers of the mouth and genitalia and relapsing iritis. It is recognized as a chronic multisystem disease affecting the skin, mucous membranes, eye, joints, central nervous system, and blood vessels. About 8% of the patients with Behcet's disease have severe vascular complications such as arterial aneurysm and occlusion. In our patient, there was a massive, painful, pulsatile mass on the clavicle on the right side of neck. A left subclavian artery aneurysm mass was observed on the left apex on a chest X-ray. Through angiography, a lobular giant saccular aneurysm on the proximal side of the right subclavian artery, giant aneurysm on the left subclavian artery, and occlusion on the left subclavian-axillary artery were observed. We treated first the right and then the left subclavian arterial aneurysm with a two-stage operation. The aneurysms were resected and polytetrafluoroethylene (PTFE) graft interposition was performed. Control angiography was performed 6 months postoperatively. Both grafts were open and there was no anastomotic aneurysm. The patient was reoperated on for a ruptured abdominal aorta pseudoaneurysm 13 months after the first operation. The aortic defect was repaired using a Dacron patch.
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7/36. diabetes insipidus as a consequence of neurologic involvement in Behcet's syndrome.

    OBJECTIVE: To describe a case of central diabetes insipidus that was associated with Behcet's syndrome. methods: We present a case report, including clinical, laboratory, and radiologic data. The pertinent literature is reviewed relative to diabetes insipidus and Behcet's syndrome, and a discussion about a possible association is presented. RESULTS: A 32-year-old man presented with diplopia and severe headaches and was found to have transverse sinus thrombosis. He reported having recurrent mouth and genital ulcers for 3 months before the current consultation. On the basis of the clinical picture, Behcet's syndrome was diagnosed. During hospitalization of the patient, polyuria and polydipsia developed, along with hypernatremia and hypotonic urine, indicative of diabetes insipidus. After desmopressin treatment was initiated, considerable improvement in clinical status and laboratory data was evident and persisted during follow-up. CONCLUSION: Behcet's syndrome, probably through vasculitic mechanisms, may result in central diabetes insipidus. This case report emphasizes that Behcet's syndrome should be considered in the differential diagnosis of diabetes insipidus.
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8/36. Endovascular stent-grafting for recurrent aneurysm in Behcet's disease.

    Arterial aneurysms represent a severe complication of Behcet's disease. A 42-year-old woman with Behcet's disease had a recurrence of an aneurysm after two surgical repair attempts using grafts. A covered stent-graft was implanted in her iliac external artery to occlude the neck of the aneurysm at the anastomosis of the bypass graft to her external iliac artery. The procedure reduced the size of the aneurysm by allowing the formation of a thrombus within its cavity. The implantation of an endovascular stent-graft may be a sound alternative to surgical repair for aneurysms associated with Behcet's disease.
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9/36. Transformation of a ruptured giant pulmonary artery aneurysm into an air cavity after transcatheter embolization in a Behcet's patient.

    pulmonary artery aneurysms due to Behcet's disease are mainly seen in young males and very rarely in females. To our knowledge there are only 10 cases reported in the related literature. Emergent transcatheter embolization was performed in a female patient with a known history of Behcet's disease in whom massive hemoptysis developed because of rupture of a giant pulmonary artery aneurysm. At 6-month follow-up, transformation of the aneurysm sac into an air cavity was detected. To our knowledge, such a transformation has never been reported in the literature before. Embolization of the pulmonary artery aneurysm and the mechanism of cavity transformation are reviewed and discussed.
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10/36. Encapsulated triamcinolone cyst after subtenon injection.

    The authors report the formation of a subtenon cyst following subtenon triamcinolone injection for chronic recurrent uveitis in Behcet's disease. The steroid was inadvertently placed superficially in the anterior subtenon space and was encapsulated eventually to present as a subtenon cyst. The episode of uveitis recurrence, which did not regress, required treatment with a repeat posterior subtenon injection 2 months later. The cyst was removed 4.5 months after the initial subtenon injection, when the patient underwent a trabeculectomy for refractory glaucoma. Histological examination of the cyst revealed a fibrous encapsulated cavity filled with small birefringent crystals, consistent with an encapsulated triamcinolone collection. Fibrous encapsulation of triamcinolone crystals can arise after a superficially placed anterior subtenon injection and this may impede the absorption of the corticosteroid and hamper its effectiveness in treating ocular inflammatory diseases.
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