眼眶渐进性坏死性黄色肉芽肿的研究进展

Progress of study on orbitalnecrobiotic xanthogranuloma

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。

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.