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首页> 外文期刊>Modern rheumatology >A case of Beh?et's disease with widespread perforating enteric ulcers preceded by a long history of peripheral gangrene
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A case of Beh?et's disease with widespread perforating enteric ulcers preceded by a long history of peripheral gangrene

机译：一例伴有广泛性肠溃疡的白塞氏病，伴有长期的坏疽病史

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摘要

Patients suffering from Beh?et's disease (BD) with intestinal involvement often have an atypical disease course. The disease in many patients does not fully meet the diagnostic criteria defined by the International Study Group for Beh?et's Disease, and instead such patients are diagnosed as having an incomplete type of BD according to the criteria of the BD Research Committee of Japan. The patient reported here developed uveitis and gangrene of the extremities as the initial symptoms and was treated with corticosteroid. After 16 years, the gangrene relapsed and multiple deep ulcers suddenly developed in the gastrointestinal tract, with oral ulcer and uveitis. The intestinal perforation was acute, progressive, severe, and extensive. With a diagnosis of incomplete BD with intestinal involvement, she was treated with high-dose glucocorticoid. However, uncontrollable gastrointestinal bleeding led to her death. Histopathological examination revealed that she suffered from intestinal and vascular BD, although gangrene of the extremities has rarely been reported as a manifestation of vascular BD. Thus, the disease course of this patient was characterized by the reappearance of peripheral gangrene after a long interval that preceded the devastating intestinal lesions.
机译：患有肠病的贝叶氏病（BD）的患者通常具有非典型的病程。在许多患者中，该疾病并未完全满足国际行为研究小组所定义的诊断标准，而是根据日本BD研究委员会的标准将此类患者诊断为BD类型不完整。该患者报告这里发展为葡萄膜炎和四肢坏疽为最初症状，并接受了皮质类固醇激素治疗。 16年后，坏疽复发，胃肠道突然出现多处深溃疡，并伴有口腔溃疡和葡萄膜炎。肠穿孔是急性的，进行性的，严重的和广泛的。由于诊断出肠道受累不完全BD，她接受了大剂量糖皮质激素治疗。但是，无法控制的胃肠道出血导致她死亡。组织病理学检查显示她患有肠和血管性BD，尽管很少报道四肢坏疽是血管性BD的表现。因此，该患者的病程的特征是在毁灭性肠损害之前的很长一段时间后，周围坏疽再次出现。

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