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氨苯砜在天疱疮和类天疱疮治疗中的应用:其长期被忽视的疗效的重新发现

Dapsone in the Management of Pemphigus and Pemphigoid: Rediscovery of its Long-Lost Efficacy.

作者信息

Alkeraye Salim, AlZamil Lama R, Alenazi Suha

机构信息

Department of Dermatology, College of Medicine, King Saud University, Riyadh, SAU.

出版信息

Cureus. 2020 Jun 24;12(6):e8805. doi: 10.7759/cureus.8805.

Abstract

Introduction Autoimmune mucocutaneous blistering dermatoses (AMCBD) are a group of disorders characterized by the production of autoantibodies that target specific adhesion molecules of the skin and/or mucous membranes. As a result, there is blister formation on the skin with or without mucous membrane involvement. Systemic corticosteroids have been used as the mainstay of treatment in AMCBD. However, due to the debilitating side effects associated with their use, there is significant morbidity and mortality, especially on the fragile elderly patients. Although the efficacy of dapsone in the treatment of AMCBD was identified decades ago, few recent studies shed light on that. Hence, further studies are needed to evaluate the efficacy of dapsone as a single agent in maintaining disease remission in patients with AMCBD. Materials and methods An observational retrospective study was performed. Patients with a known diagnosis of bullous pemphigoid (BP) or pemphigus vulgaris (PV) who are treated with dapsone with or without low-dose systemic corticosteroids were included in the study, and their medical files were reviewed.  Results A total of seven patients were included (three males and four females). All patients showed a satisfactory response to dapsone, achieving disease remission in a short period of time with no serious side effects necessitating treatment cessation. Conclusions Our findings support that dapsone may have a corticosteroid-sparing effect in the management of AMCBD. Further studies are warranted to confirm our findings.

摘要

引言 自身免疫性黏膜皮肤水疱性皮肤病(AMCBD)是一组以产生针对皮肤和/或黏膜特定黏附分子的自身抗体为特征的疾病。因此,皮肤会出现水疱,可伴有或不伴有黏膜受累。全身用皮质类固醇一直是AMCBD治疗的主要手段。然而,由于使用皮质类固醇会带来使人虚弱的副作用,存在显著的发病率和死亡率,尤其是对脆弱的老年患者。尽管数十年前就已确定氨苯砜在治疗AMCBD方面的疗效,但近期相关研究却很少。因此,需要进一步研究来评估氨苯砜作为单一药物维持AMCBD患者疾病缓解的疗效。

材料与方法 进行了一项观察性回顾性研究。纳入了已知诊断为大疱性类天疱疮(BP)或寻常型天疱疮(PV)且接受氨苯砜治疗(无论是否联合小剂量全身用皮质类固醇)的患者,并查阅了他们的病历。

结果 共纳入7例患者(3例男性和4例女性)。所有患者对氨苯砜均表现出满意的反应,在短时间内实现了疾病缓解,且无需要停药的严重副作用。

结论 我们的研究结果支持氨苯砜在AMCBD管理中可能具有皮质类固醇节省作用。有必要进行进一步研究以证实我们的研究结果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/db1a/7381850/21722df494a6/cureus-0012-00000008805-i01.jpg

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