眶内罕见的硬性炎性病变：独特病例报告。
A rare lumpy inflammatory lesion of the orbit: a unique case report.
机构信息
Beijing Institute of Ophthalmology, Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, 100730, China.
Pathology Department, Beijing Tongren Hospital, Capital Medical University, Beijing, 100730, China.
出版信息
Diagn Pathol. 2023 Feb 23;18(1):30. doi: 10.1186/s13000-023-01316-w.
BACKGROUND
Eosinophilic angiocentric fibrosis (EAF) is a rare inflammatory lesion, especially in orbit. EAF is believed to be related to IgG4-related disease (IgG4-RD), but the clinical manifestations of systemic involvement are relatively rare and easy to be confused with tumors or other inflammatory diseases. Histopathological examination is the most important way of its diagnosis and differentiation.
CASE PRESENTATION
We presented a 55-year-old female patient presented with recurrent swelling of the right lower eyelid for more than 2 months. The pathological diagnosis was EAF. Positive immunostaining for IgG, CD34, κ, and λ, while negative immunostaining for IgG4.
CONCLUSIONS
Complete surgical resection is the preferred treatment, histopathological examination is the main diagnostic standard.
背景
嗜酸性血管中心性纤维化为一种罕见的炎症性病变，尤其是在眼眶中。嗜酸性血管中心性纤维化被认为与 IgG4 相关疾病（IgG4-RD）有关，但全身受累的临床表现相对少见，容易与肿瘤或其他炎症性疾病混淆。组织病理学检查是其诊断和鉴别诊断的最重要方法。
病例介绍
我们报告了一例 55 岁女性患者，因右眼下眼睑反复肿胀超过 2 个月就诊。病理诊断为嗜酸性血管中心性纤维化。免疫组化 IgG、CD34、κ 和 λ 阳性，而 IgG4 阴性。
结论
完全手术切除是首选治疗方法，组织病理学检查是主要的诊断标准。
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