国际眼科纵览 ›› 2021, Vol. 45 ›› Issue (2): 165-171.doi: 10.3760/cma.j.issn.1673-5803.2021.02.014

• 综述 • 上一篇    下一篇

眼眶渐进性坏死性黄色肉芽肿的研究进展

孙梅  马建民   

  1. 首都医科大学附属北京同仁医院  北京同仁眼科中心  眼科学与视觉科学北京市重点实验室 100730
  • 收稿日期:2020-07-23 出版日期:2021-04-22 发布日期:2021-04-28
  • 通讯作者: 马建民, Email:jmma@sina.com
  • 基金资助:
    北京市自然科学基金(7182038);北京市“登峰”人才培养计划(DFL20190201)

Progress of study on orbitalnecrobiotic xanthogranuloma

Sun Mei, Ma Jianmin   

  1. Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University ; Beijing Key Laboratory of Ophthalmology & Vision Science, Beijing 100730, China
  • Received:2020-07-23 Online:2021-04-22 Published:2021-04-28
  • Contact: Ma Jianmin, Email: jmma@sina.com
  • Supported by:
    Beijing Natural Science Foundation of China (7182038); Beijing Ascension Talent Training Program (DFL20190201) 

摘要: 渐进性坏死性黄色肉芽肿(necrobiotic xanthogranuloma,NXG)是一种罕见的慢性进展性多器官疾病,属于成人眼眶黄色肉芽肿病的一个分型。典型表现是眼眶周围多发的硬化性黄褐色斑块或结节,可有萎缩、溃疡和毛细血管扩张。NXG主要影响皮肤和皮下组织,亦可累及身体其他系统和内脏器官,还可引起严重的眼部并发症。目前病因及发病机制未明,组织病理学特征是大面积的变性坏死胶原伴随肉芽肿性炎症,可见胆固醇结晶、Touton巨细胞、泡沫细胞以及异物巨细胞浸润。通常预后良好,但目前尚无根治性治疗方案。最常用烷基化剂进行化学治疗,在伴发其他系统疾病时可与全身性糖皮质激素联合应用。局灶性糖皮质激素注射、放射治疗和手术切除可用于局部和轻微病变。其他治疗方式还包括干扰素、血浆置换、免疫球蛋白静脉注射等,但疗效各不相同。(国际眼科纵览,2021, 45:165-171)


关键词: 渐进性坏死性黄色肉芽肿/诊断;渐进性坏死性黄色肉芽肿/治疗;眼眶 

Abstract: Necrobiotic xanthogranuloma (NXG) is a rare chronic progressive multiple organ disease, and it is a subtype of adults’ yellow orbital granuloma. The typical clinical manifestations of NXG are multiple sclerosing yellow-brown plaques or nodules around the orbit, and the lesions may have atrophy, ulcer and telangiectasia. NXG mainly affects the skin and subcutaneous tissues. It can also affect other body systems and internal organs, also causing serious complications of eye. At present, the etiology and pathogenesis of NXG are unknown. The most prominent histopathological features of NXG is large areas of denatured collagen with granulomatous inflammation, and cholesterol crystal Touton giant cell, foam cells and foreign body giant cell can be seen in the lesion specimen. The prognosis of NXG is usually good, but there is no radical treatment. Chemotherapy with alkylating agents is most commonly used, and corticosteroids combined with chemotherapy can be used to treat NXG with other systemic diseases. Focal glucocorticoid injection, radiotherapy, and surgical excision may be used for local and minor lesions. Other treatments include interferon, plasmapheresis, and intravenous immunoglobulin and so on, but the results vary. (Int Rev Ophthalmol, 2021, 45: 165-171)


Key words: necrobiotic xanthogranuloma/ diagnosis, necrobiotic xanthogranuloma /treatment, orbit ,