Extramammary Paget’s disease (EMPD) is subclinical in extent and multifocal in nature. There is no global consensus for treatment, so its management represents a challenge in clinical practice. Therefore, we conducted a systematic review through the main electronic databases to assess the effectiveness of topical imiquimod in cutaneous EMPD and to discuss its management. Finally, 24 studies involving a total of 233 EMPD patients treated with topical imiquimod were selected. The topical imiquimod response rate was 67%, and the complete response (CR) rate was 48%. Patients were treated with a three–four times a week regimen in most cases, ranging between 2 to 52 weeks. In addition, imiquimod was applied as an adjunctive treatment in 21 patients, achieving a CR rate of 71%. Consequently, imiquimod therapy could achieve a good response ratio as a first-line treatment, as adjuvant and neo-adjuvant therapy, and as a treatment for recurrent disease. The heterogeneity between studies and the lack of a control arm made it impossible to conduct a meta-analysis. To improve the quality of evidence on EMPD, multicenter studies are essential to collect a larger number of patients and, consequently, obtain high-quality evidence to standardize treatment. The Prospero registration number is CRD42023447443.
乳房外佩吉特病(EMPD)在范围上呈亚临床状态,在本质上呈多灶性。目前全球尚无统一的治疗共识,因此其临床管理颇具挑战。为此,我们通过主要电子数据库进行系统综述,以评估局部使用咪喹莫特治疗皮肤EMPD的疗效并探讨其治疗方案。最终共纳入24项研究,涵盖233例接受局部咪喹莫特治疗的EMPD患者。局部咪喹莫特治疗的总缓解率为67%,完全缓解(CR)率为48%。多数患者采用每周3-4次的治疗方案,疗程介于2至52周之间。此外,21例患者将咪喹莫特作为辅助治疗,完全缓解率达71%。由此可见,咪喹莫特疗法作为一线治疗、辅助及新辅助治疗、以及复发性疾病的治疗方案均可获得良好缓解率。由于研究间存在异质性且缺乏对照组,无法进行荟萃分析。为提高EMPD相关证据质量,亟需开展多中心研究以纳入更多病例,从而获得高质量证据以规范治疗方案。本研究已在PROSPERO平台注册,注册号为CRD42023447443。
Topical Imiquimod in Primary Cutaneous Extramammary Paget’s Disease: A Systematic Review