Cases reported "Behcet Syndrome"

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1/39. thalidomide responsiveness in an infant with Behcet's syndrome.

    BACKGROUND: Behcet's syndrome is a chronic relapsing vasculitis characterized by aphthous stomatitis, genital ulcerations, and uveitis. The disease is a multisystem illness, which may involve the skin, joints, gastrointestinal tract, blood vessels, central nervous system, and other organs. CASE STUDY: We report a case of an infant with features of Behcet's syndrome who did not respond to conventional immunosuppression with high-dose corticosteroids and cytotoxic agents. thalidomide, a potent immune response-modifying drug, was used with marked improvement and resolution of symptoms. CONCLUSION: This finding supports a trial of thalidomide treatment in patients with Behcet's syndrome who are unresponsive to other forms of immunosuppressive therapy or develop undesirable side effects.
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2/39. Cardiac and great vessel thrombosis in Behcet's disease.

    Behcet's disease (BD) is a chronic relapsing systemic vasculitis in which orogenital ulceration is a prominent feature. The disease affects many systems and causes hypercoagulability. We present a 27-year-old male patient who exhibited widespread great vessel thrombosis including right atrial and ventricular thrombi in the setting of right-sided infectious endocarditis and orogenital aphthous ulcerations and erythema nodosum due to BD. We reviewed the enigmatic prothrombotic state of BD, and discuss our prior experiences in this field.
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keywords = vessel
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3/39. Total occlusion of inferior vena cava in a patient with antiphospholipid antibody syndrome associated with behcet's disease.

    Behcet's disease frequently involves the venous system, usually affecting small vessels, but sometimes large vessels such as the vena cava. Antiphospholipid antibody syndrome is associated with an increased incidence of arterial and venous thrombosis. A 29-year-old male with Behcet's disease developed bilateral leg edema secondary to thrombotic occlusion of the inferior vena cava. Laboratory tests revealed positive antiphospholipid antibodies and lupus anticoagulant. Treatment with steroid and warfarin subsequent to intravenous administration of uro-kinase resulted in improvement of symptoms. The association of antiphospholipid antibody syndrome and Behcet's disease may have caused the total thrombotic occlusion of the vena cava in this case.
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4/39. 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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5/39. Opacification of a hydrophilic acrylic intraocular lens with exacerbation of Behcet's uveitis.

    Behcet's disease is 1 of the most common causes of uveitis in the Eastern world. Its common ocular complications are uveitis, cataract, and obliteration of retinal vessels. phacoemulsification with intraocular lens (IOL) implantation in patients with Behcet's disease is known to be a safe procedure. We managed a patient with Behcet's disease who had aggravated uveitis and opacification of a hydrophilic acrylic IOL (ACRL-C160, Ophthalmed) 4 months after cataract surgery. Recalcitrant uveitis despite maximum tolerable medication and IOL opacification with vitreous opacity necessitated an IOL exchange and trans pars plana vitrectomy. After the procedure, the eye became quiescent. However, the visual acuity was 20/200 because of the obliteration of retinal vessels.
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keywords = vessel
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6/39. 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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7/39. Neuro-Behcet's disease manifesting as a neoplasm-like lesion--case report.

    A 50-year-old man presented with neuro-Behcet's disease (NBD) manifesting as a large neoplasm-like lesion affecting the brainstem, basal ganglia, and white matter of the cerebral hemisphere. He had no history of disease except for psychoneurosis. On admission, neurological examination found left hemiparesis and dysarthria. Magnetic resonance (MR) imaging showed multiple small ring-like enhancement in the basal ganglia, brainstem, and deep white matter. biopsy of the mass was performed. Histological examination revealed invasion of inflammatory cells in the white matter, especially around the blood vessels. After the brain biopsy, the patient developed oral aphthae, genital ulcers, and skin eruptions, which are indicative of Behcet's disease. MR imaging after three courses of steroid pulse therapy revealed that the edematous lesion had become smaller with minimum midline shift. NBD should be considered in the differential diagnosis of lesions with multiple ring-like enhancement extending from the basal ganglia to the brainstem, because dermatological manifestations are sometimes obscured during periods of remission.
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8/39. Synovial histology in three Behcet's disease patients with orthopedic surgery.

    Specimens of synovial tissues from 5 affected joints of 3 patients with Behcet's disease were available for histopathological examination. All specimens were infiltrated by lymphocytes and neutrophils, and exhibited marked vascularity and infiltration of lymphoid cells among the vessels. Marked plasma cell infiltration and lymphoid follicle formation were found in one synovial tissue sample. There was no evidence of infection or vasculitis. These findings suggest that the histopathological characteristics of synovial tissue in Behcet's disease may have a wide range, some of which may even resemble the synovial tissue of rheumatoid arthritis.
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keywords = vessel
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9/39. Adamantiades-Behcet's disease with inner ear involvement.

    Adamantiades-Behcet's disease is a chronic recurrent inflammatory disorder involving the small and large vessels. Typical loci of manifestations are the mucous membranes, skin and eyes, as well as the joints and central nervous system. Other organs are not commonly involved. We present two patients, one with ocular and the other with mucocutaneous manifestation of Adamantiades-Behcet's disease. In addition, the first patient reported three episodes of sudden hearing loss while under immunosuppressive therapy for his eye involvement. The second, therapy-naive patient complained of tinnitus in his left ear. Careful examination revealed vestibular involvement in the first patient and retrocochlear involvement in the second. Inner ear involvement is an uncommon manifestation of Adamantiades-Behcet's disease. In case of relevant signs or history, such as hearing disturbance, tinnitus and/or vertigo, patients should be examined for inner ear involvement.
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keywords = vessel
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10/39. Behcet's disease involving the breast.

    Behcet's disease is a vasculitis of unknown origin that was traditionally defined by oral and genital ulcers and uveitis. We describe a case of a patient with a diagnosis of Behcet's syndrome who presented a palpable lesion in the right breast with inflammatory signs. X-ray findings posed a differential diagnosis between tumoral and inflammatory pathology. The pathological findings confirmed a small-vessel vasculitis. We found two reports of breast involvement by this disease in the literature. Our patient was studied by mammogram and sonogram which together with clinical history are important to prevent delay in diagnosis and unnecessary therapeutic procedures.
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keywords = vessel
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