Hailey-Hailey disease successfully treated with naloxone: 2 case reports and Review of the literature on efficacy of opioid receptor antagonist in Hailey-Hailey disease patients.

Junyou Zheng, Zhimin Duan, Beilei Xu, Hao Song, Jianbing Wu, Fang Fang, Nan Sheng, Chengrang Li
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Abstract

Background: Hailey-Hailey disease (HHD), a genetic blistering disease, is caused by a mutation in a calcium transporter protein in the Golgi apparatus encoded by the ATP2C1 gene. Clinically, HHD is characterized by flaccid vesicles, blisters, erosions, fissures, and maceration mainly in intertriginous regions. Some patients remain refractory to conventional treatments. Previously, a series of reports have confirmed naltrexone as an effective option for those patients. However, in China, naltrexone is unavailable in some hospitals and unaffordable for some patients.

Objective: To confirm naloxone as a treatment option for HHD, and assess the efficacy rate and safety of naltrexone for patients with HHD.

Methods: Two patients with biopsy-proven HHD received naloxone (2 mg/d, via intravenous infusion). We followed up with the two patients, assessing the change of skin lesions and obtaining photographs. We searched the PubMed databases using the keywords 'Hailey-Hailey disease' or 'benign familial pemphigus', and 'naltrexone' or 'naloxone', and reviewed English publications of reports and analyzed the efficacy and safety of naltrexone.

Results: Two patients prescribed naloxone showed completely remission in two weeks without any adverse reactions. The total remission rate of naltrexone for HHD is approximately 80%, without severe adverse effects.

Conclusion: Naltrexone is effective and safe in the treatment of HHD. Naloxone, an analog of naltrexone, can also effectively and safely treat HHD, potentially offering a new therapeutic option for patients with refractory HHD.

纳洛酮成功治疗哈雷-哈雷病2例报告及阿片受体拮抗剂治疗哈雷-哈雷病疗效文献综述
背景:海利-海利病(HHD)是一种遗传性水疱病,由ATP2C1基因编码的高尔基体中钙转运蛋白突变引起。在临床上,HHD主要表现为松弛的囊泡、水疱、糜烂、裂隙和浸渍,主要发生在三节间区。一些病人对常规治疗仍然难以治愈。此前,一系列的报道已经证实纳曲酮是这些患者的有效选择。然而,在中国,一些医院没有纳曲酮,一些患者负担不起。目的:确认纳曲酮作为HHD的治疗选择,并评价纳曲酮治疗HHD患者的有效性和安全性。方法:2例活检证实的HHD患者接受纳洛酮(2mg /d,静脉滴注)治疗。我们对两名患者进行随访,评估皮肤病变的变化并拍摄照片。我们检索PubMed数据库,检索关键词为“海利-海利病”或“良性家族性天疱疮”,检索关键词为“纳曲酮”或“纳洛酮”,查阅英文文献报道,分析纳曲酮的疗效和安全性。结果:2例患者服用纳洛酮后2周内病情完全缓解,无不良反应。纳曲酮治疗HHD的总缓解率约为80%,无严重不良反应。结论:纳曲酮治疗HHD有效、安全。纳洛酮,纳曲酮的类似物,也可以有效和安全地治疗HHD,可能为难治性HHD患者提供新的治疗选择。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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