Orbital Necrobiotic Xanthogranuloma Associated With Systemic IgG4 Disease

Orbital Necrobiotic Xanthogranuloma Associated With Systemic IgG4 Disease
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与全身性 IgG4 疾病相关的眼眶坏死性黄色肉芽肿

DOI:
10.3109/09273948.2010.490629
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发表时间:
2010
影响因子:
3.3
通讯作者:
C. Eberhart
C. Eberhart
中科院分区:
医学4区
文献类型:
--
作者:
Kamaljeet Singh;K. Rajan;C. Eberhart

文献摘要

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目的:作者描述了2例眼眶黄色肉芽肿性疾病伴IgG4阳性浆细胞增多的病例,并观察了其他类型的眼眶炎症中的IgG4。方法:对18例眼眶炎症进行免疫组织化学染色,其中慢性泪腺炎(n = )10例,坏死性黄色肉芽肿(n = 2),黄色肉芽肿(n = 1),特发性眼眶炎症/假瘤(n = 4),真菌感染(n = 1)。结果:1例出现坏死性黄色肉芽肿的患者有全身性IgG4病的症状。他的眼眶病变显示IgG4阳性浆细胞数量增加(55%)。另一例无全身症状的眼眶黄色肉芽肿性病变也含有较高比例的IgG4阳性浆细胞(80%)。仅1例慢性泪腺炎有明显的IgG4阳性浆细胞(平均17个/HPF)。结论:在非硬化性眼眶炎性病变中,IgG4阳性浆细胞相对较少。然而,全身性疾病IgG4可能与眼眶坏死性黄色肉芽肿有关。
Purpose: The authors describe 2 cases of orbital xanthogranulomatous disease associated with an increase in IgG4-positive plasma cells, and also examine IgG4 in other types of orbital inflammation. Methods: Immunohistochemistry for total IgG and IgG4 was performed in 18 cases of orbital inflammation, including chronic dacryoadenitis (n = 10), necrobiotic xanthogranuloma (n = 2), xanthogranuloma (n = 1), idiopathic orbital inflammation/pseudotumor (n = 4), and fungal infection (n = 1). Results: One patient presenting with necrobiotic xanthogranuloma had signs of systemic IgG4 disease. His orbital lesion showed an elevated number of IgG4 positive plasma cells (55%). An orbital xanthogranulomatous lesion in a second patient lacking systemic symptoms also contained a high percentage of IgG4-positive plasma cells (80%). Only 1 case of chronic dacryoadenitis contained prominent IgG4-positive plasma cells (mean 17/hpf). Conclusions: IgG4-positive plasma cells are relatively rare in nonsclerosing orbital inflammatory lesions. However, systemic disease IgG4 can be associated with necrobiotic xanthogranuloma of the orbit.