Wang Y C, Li J, Lin J Y
Tianjin Eye Hospital, Nankai University Affiliated Eye Hospital, Clinical College of Ophthalmology of Tianjin Medical University, Tianjin Eye Institute, Tianjin Key Laboratory of Ophthalmology and Visual Science, Tianjin 300020, China.
Zhonghua Yan Ke Za Zhi. 2022 Sep 11;58(9):682-687. doi: 10.3760/cma.j.cn112142-20220128-00037.
To investigate the clinicopathological features of adult orbital xanthogranulomatous disease (AOXGD) with lacrimal gland reactive lymphoid hyperplasia. Retrospective case series study. The clinical and pathological data of AOXGD cases diagnosed and treated in Tianjin Eye Hospital from January 2002 to December 2021 was reviewed, and the clinical characteristics, radiologic findings and pathological characteristics of periocular and lacrimal gland lesions of 5 cases were retrospectively analyzed. The expression of IgG4 and IgG protein in periocular and lacrimal gland lesions was detected by immunohistochemical staining, and the role of IgG4 in AOXGD was preliminarily studied. There were four females and one male with an average age of 53.8 years (39 to 77 years). Among the five AOXGD cases, there were three cases of adult-onset xanthogranuloma, one case of adult-onset asthma and periocular xanthogranuloma, and one case of necrobiotic xanthogranuloma. All cases involved both eyes. The swelling of eyelids was observed in five cases, and the yellow or pale yellow eyelid skin was found in two cases. Imaging examinations showed the tumor mainly involved the eyelids, subcutaneous tissues, anterior orbit and lacrimal gland. A large number of foam cells and typical Touton giant cells were found in the periorbital lesions, accompanied by different degrees of fibrosis. The fibrinoid necrosis was detected in one case of necrobiotic xanthogranuloma. The lacrimal gland lesions showed different types of reactive lymphoid hyperplasia, including IgG4-related disease in two cases, follicular lymphoid hyperplasia in two cases and focal lymphoid hyperplasia in one case. IgG4 levels of periorbital and lacrimal gland lesions were elevated in four cases. Asthma and elevated serum IgG4 were found in one case of adult-onset periocular xanthogranuloma. Three patients underwent surgical resection and adjuvant hormone or immunosuppressive therapy, and two patients underwent simple surgical resection. The patients were followed up for 1.5 to 10.0 years, one patient was lost, and four patients had no recurrence. AOXGD with lacrimal gland reactive lymphoid hyperplasia is a group of rare diseases. The periorbital lesions of that are characterized by proliferation of foamy histiocytes and Touton giant cells, and the lacrimal gland lesions of that manifest as IgG4-related disease in some cases.
探讨成人眼眶黄色肉芽肿性疾病（AOXGD）合并泪腺反应性淋巴样增生的临床病理特征。回顾性病例系列研究。回顾性分析2002年1月至2021年12月在天津眼科医院诊断和治疗的AOXGD病例的临床和病理资料，对5例患者眼周及泪腺病变的临床特征、影像学表现和病理特征进行分析。采用免疫组织化学染色检测眼周及泪腺病变中IgG4和IgG蛋白的表达，初步研究IgG4在AOXGD中的作用。5例患者中，女性4例，男性1例，平均年龄53.8岁（39～77岁）。5例AOXGD患者中，成人起病的黄色肉芽肿3例，成人起病的哮喘合并眼周黄色肉芽肿1例，渐进性坏死性黄色肉芽肿1例。所有病例均累及双眼。5例患者均有眼睑肿胀，2例可见黄色或淡黄色眼睑皮肤。影像学检查显示肿瘤主要累及眼睑、皮下组织、眶前部和泪腺。眼周病变中可见大量泡沫细胞和典型的杜顿巨细胞，伴有不同程度的纤维化。1例渐进性坏死性黄色肉芽肿可见纤维蛋白样坏死。泪腺病变表现为不同类型的反应性淋巴样增生，其中2例为IgG4相关疾病，2例为滤泡性淋巴样增生，1例为局灶性淋巴样增生。4例患者眼周及泪腺病变的IgG4水平升高。1例成人起病的眼周黄色肉芽肿患者伴有哮喘且血清IgG4升高。3例患者接受手术切除并辅助激素或免疫抑制治疗，2例患者仅接受手术切除。患者随访1.5～10.0年，1例失访，4例无复发。AOXGD合并泪腺反应性淋巴样增生是一组罕见疾病。其眼周病变以泡沫状组织细胞和杜顿巨细胞增生为特征，部分病例泪腺病变表现为IgG4相关疾病。
