嗜酸性血管中心性纤维变性

定义 英文原文（暂无中文）

A rare otorhinolaryngologic disease characterized by an indolent submucosal mass of variable size and extent, most commonly arising in the anterior nasal cavity, involving the nasal septum and lateral nasal wall, and potentially extending into the adjacent sinuses. Occurrence in the larynx and lower respiratory tract or the orbit is rare. Histological examination shows concentric angiocentric stromal fibrosis (onionskin fibrosis) and prominent eosinophils. Increased numbers of IgG4-positive plasma cells in the lesion may also be observed, in addition to elevated serum IgG4. Patients typically present with long-standing obstructive symptoms.

别名

lgG4相关嗜酸性血管中心性纤维变性

基本事实

遗传方式

不适用

发病年龄

成年期

患病率

<1 / 1 000 000

外部标识与链接