Zhang Jiawen, Zhang Chengfeng, Xu Zhongyi, Liang Jun, Zhu Qinyuan
Department of Dermatology, Huashan Hospital, Fudan University, 12 Middle Wulumuqi Road, Shanghai, China.
Inflamm Res. 2025 Jun 3;74(1):89. doi: 10.1007/s00011-025-02059-8.
Erythrodermic pemphigus foliaceus (EPF) is a severe, often refractory autoimmune disease.
We report successful treatment of refractory EPF in a 59-year-old male using low-dose rituximab (RTX) and intravenous immunoglobulin (IVIG).
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.
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.
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。
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。
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。
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。