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中国临床药理学与治疗学 ›› 2022, Vol. 27 ›› Issue (10): 1197-1200.doi: 10.12092/j.issn.1009-2501.2022.10.015

• 综述与讲座 • 上一篇    下一篇

黏膜性类天疱疮治疗研究进展

卢晓红,何彩凤,慈超,袁涛   

  1. 皖南医学院弋矶山医院皮肤性病科,芜湖 241000,安徽
  • 收稿日期:2022-04-13 修回日期:2022-10-13 出版日期:2022-10-27 发布日期:2022-11-14
  • 通讯作者: 何彩凤,女,博士,副主任医师,研究方向:遗传性皮肤病、性传播疾病。 E-mail: caifenghe@ 126.com
  • 作者简介:卢晓红,女,硕士,医师,研究方向:遗传性皮肤病。 E-mail: wdm151963@163.com
  • 基金资助:
    2020年度安徽省教育厅高等学校省级质量工程一般教学改革项目(2020yjxm2114);2021年度皖南医学院重点科研项目(WK2021ZF02)

Progress in the treatment of mucosal pemphigoid

LU Xiaohong, HE Caifeng, Ci Chao, YUAN Tao   

  1. Department of Dermatology and Venereology, Yijishan Hospital of Wannan Medical College, Wuhu 241000, Anhui, China
  • Received:2022-04-13 Revised:2022-10-13 Online:2022-10-27 Published:2022-11-14

摘要: 黏膜性类天疱疮是一种罕见的表皮下免疫性大疱病,主要累及口腔、眼睛等黏膜,具有形成瘢痕的风险,严重者可危及生命,早诊断早治疗可避免严重并发症的发生。轻症患者,可使用氨苯砜或局部外用糖皮质激素;重症患者,系统使用糖皮质激素,联合使用免疫抑制剂,包括环磷酰胺、硫唑嘌呤、甲氨蝶呤及霉酚酸酯有助于提高临床疗效,降低糖皮质激素使用剂量。上述治疗均无效的病情顽固者,可选择生物制剂(包括利妥昔单抗、依那西普、英夫里昔单抗和巴西替尼)、静脉注射人免疫球蛋白(IVIG)等疗法。部分已形成黏膜瘢痕的患者,可考虑手术或激光治疗改善症状。一些免疫抑制剂和生物制剂治疗黏膜性类天疱疮的临床疗效还有待较大样本量的临床试验进一步验证。

关键词: 黏膜性类天疱疮, 药物治疗, 外科手术

Abstract: Mucosal pemphigoid is a rare subepidermal immune bullous disease, mainly involving the oral cavity, eyes and other mucous membranes, with the risk of scarring, which can be life-threatening in severe cases. For patients with mucosal pemphigoid, early diagnosis and early treatment can avoid serious complications. For mild patients, dapsone or topical glucocorticoids can be used; for severe patients, combination of immunosup-pressants helps to improve clinical efficacy and reduce the dosage of glucocorticoid, including cyclophosphamide, azathioprine, methotrexate, and mycophenolic acid esters. For some refractory cases without any response to the treatments of dapsone, glucocorticoids and immunosuppressants, biological agents (including rituximab, etanercept, infliximab, and braziltinib), intravenous immune globulin and other therapies can be selected. For some patients with mucosal scarring, surgery or laser therapy can be considered to improve symptoms. However, it is necessary to further demonstrate the clinical efficacy of some immunosuppressants and biologic agents on mucosal pemphigoid in a lot of larger clinical trials. 

Key words: mucosal pemphigoid, drug therapy, surgery

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