大疱性类天疱疮诊断和治疗的专家建议

中华皮肤科杂志 ›› 2016, Vol. 49 ›› Issue (6): 384-387.

大疱性类天疱疮诊断和治疗的专家建议

中国医师协会皮肤科医师分会自身免疫……¹,左亚刚²,晋红中²

1.
2. 中国医学科学院北京协和医学院北京协和医院

收稿日期:2015-11-30 修回日期:2016-01-14 发布日期:2016-05-31
通讯作者: 晋红中 E-mail:jinhongzhong@126.com.cn

Diagnosis and treatment of bullous pemphigoid: an expert proposal

,Ya-gang ZUO,

Received:2015-11-30 Revised:2016-01-14 Published:2016-05-31

摘要/Abstract

摘要：

大疱性类天疱疮（bullous pemphigoid，BP）是自身免疫性大疱性疾病，老年人多见，偶见于儿童和青少年。临床表现为紧张性水疱和大疱，尼氏征阴性，有不同程度的瘙痒，多数不伴黏膜损害。BP患者血清中产生针对皮肤基底膜带的循环自身抗体，包括抗表皮基底膜抗原230 000（BP230，BPAG1）、180 000（BP180，BPAG2）两种主要自身抗体，简称抗BP230和抗BP180。抗BP230的致病作用尚待进一步研究。抗BP180抗体可直接与皮肤基底膜的半桥粒蛋白BP180胞外区域的膜近端非胶原16A区域（BP180 NC16A）结合，激活补体，趋化炎症细胞到达炎症部位，释放蛋白水解酶，从而引起水疱和大疱［１］。此外，药物等因素可能与BP发病相关［2］……

中国医师协会皮肤科医师分会自身免疫…… 左亚刚晋红中. 大疱性类天疱疮诊断和治疗的专家建议[J]. 中华皮肤科杂志, 2016,49(6):384-387. doi:

Ya-gang ZUO. Diagnosis and treatment of bullous pemphigoid: an expert proposal[J]. Chinese Journal of Dermatology, 2016, 49(6): 384-387.doi:

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