A woman in her 60s with rheumatoid arthritis was admitted with fever and abdominal pain. Laparoscopic examination with the differential diagnosis of peritoneal neoplasm and infection revealed granulomatous phlebitis in the resected greater omentum. Amorphous eosinophilic deposits observed in the resected tissue exhibited focal, weak positivity for Congo red but were strongly positive for thioflavin S, confirming their focal amyloid properties. Marked degeneration of elastic fibers was also evident. Electron microscopy revealed deposits around the affected elastic fibers. Immunohistochemistry revealed the deposition of epidermal growth factor-containing fibulin-like extracellular matrix protein 1 (EFEMP1) along with T-cell-predominant lymphocytic inflammation. The definitive diagnosis was granulomatous enterocolic lymphocytic phlebitis (ELP) associated with EFEMP1 deposition exhibiting focal amyloid properties (EFEMP1/AEFEMP1), supported by proteomics analysis. This type of vasculitis is similar to amyloid-β-related angiitis of the central nervous system. Thus, we speculate that granulomatous ELP also results from an immune response that recognizes EFEMP1/AEFEMP1 deposits as foreign material and attempts to remove them. Confirmation of EFEMP1/AEFEMP1 deposition with Congo red staining is challenging, particularly in the presence of inflammation, and warrants comprehensive evaluation.

中文翻译：

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与含有表皮生长因子的纤维蛋白样细胞外基质蛋白 1 沉积和局灶性淀粉样蛋白特性相关的小肠结肠肉芽肿性静脉炎：病例报告

一名 60 多岁的女性患有类风湿性关节炎，因发烧和腹痛入院。腹腔镜检查与腹膜肿瘤和感染的鉴别诊断显示，切除的大网膜存在肉芽肿性静脉炎。在切除的组织中观察到的无定形嗜酸性粒细胞沉积物表现出局灶性的刚果红弱阳性，但硫黄素 S 呈强阳性，证实了其局灶性淀粉样蛋白特性。弹性纤维也明显变性。电子显微镜显示受影响的弹性纤维周围有沉积物。免疫组织化学显示含有表皮生长因子的纤维蛋白样细胞外基质蛋白 1 (EFEMP1) 沉积以及 T 细胞为主的淋巴细胞炎症。最终诊断为肉芽肿性小肠结肠淋巴细胞性静脉炎 (ELP)，与 EFEMP1 沉积相关，表现出局灶性淀粉样蛋白特性 (EFEMP1/AEFEMP1)，并得到蛋白质组学分析的支持。这种类型的血管炎类似于中枢神经系统的淀粉样蛋白-β相关血管炎。因此，我们推测肉芽肿性 ELP 也是由免疫反应引起的，该免疫反应将 EFEMP1/AEFEMP1 沉积物识别为异物并试图将其去除。用刚果红染色确认 EFEMP1/AEFEMP1 沉积具有挑战性，特别是在存在炎症的情况下，需要进行全面评估。