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低剂量利妥昔单抗联合静脉注射免疫球蛋白成功治疗难治性落叶型天疱疮。

Successful treatment of refractory erythrodermic pemphigus foliaceus with low-dose rituximab and intravenous immunoglobulin.

作者信息

Zhang Jiawen, Zhang Chengfeng, Xu Zhongyi, Liang Jun, Zhu Qinyuan

机构信息

Department of Dermatology, Huashan Hospital, Fudan University, 12 Middle Wulumuqi Road, Shanghai, China.

出版信息

Inflamm Res. 2025 Jun 3;74(1):89. doi: 10.1007/s00011-025-02059-8.

Abstract

BACKGROUND

Erythrodermic pemphigus foliaceus (EPF) is a severe, often refractory autoimmune disease.

METHOD

We report successful treatment of refractory EPF in a 59-year-old male using low-dose rituximab (RTX) and intravenous immunoglobulin (IVIG).

RESULTS

Initial high-dose corticosteroids and IVIG failed, complicated by infections. A modified regimen of RTX (0.5 g) and IVIG (0.4 g/kg/day for three days) every two weeks for two cycles induced remission, with a mild relapse at six months successfully retreated. No infections occurred during 15-month follow-up.

CONCLUSION

This combined therapy using low-dose RTX and IVIG may be a safe and effective option for refractory EPF, potentially minimizing infection risk associated with standard RTX dosing. Further studies are warranted.

摘要

背景

红皮病型落叶型天疱疮(EPF)是一种严重的、通常难治的自身免疫性疾病。

方法

我们报告了一名59岁男性难治性EPF患者使用低剂量利妥昔单抗(RTX)和静脉注射免疫球蛋白(IVIG)成功治疗的病例。

结果

初始高剂量皮质类固醇和IVIG治疗失败,并伴有感染并发症。采用改良方案,每两周给予RTX(0.5 g)和IVIG(0.4 g/kg/天,共三天),共两个周期,诱导病情缓解,六个月时出现轻度复发,成功再次治疗。在15个月的随访期间未发生感染。

结论

这种低剂量RTX和IVIG联合治疗可能是难治性EPF的一种安全有效的选择,有可能将与标准RTX给药相关的感染风险降至最低。有必要进行进一步研究。

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