Department of Plastic Surgery, Peking University Third Hospital, 49 North Garden Road, Haidian District, Beijing, 100191, China.
Department of Epidemiology and Biostatistics, School of Public Health, Peking University, 38 Xueyuan Road, Haidian District, Beijing, 100191, China.
Orphanet J Rare Dis. 2021 Mar 17;16(1):134. doi: 10.1186/s13023-021-01715-6.
Extramammary Paget's disease (EMPD) is an intraepithelial adenocarcinoma. The chronic relapsing clinical course and unbearable clinical symptoms of extramammary Paget's disease usually result in a markedly diminished quality of life. No national data are available on descriptive epidemiology of EMPD in China, the most populous country over the world. This population-based study aimed to estimate the prevalence and associated sex and age patterns of EMPD in China.
This study was conducted using data from China's Urban Employee Basic Medical Insurance and Urban Resident Basic Medical Insurance, covering approximately 0.43 billion Chinese urban residents in 2016. Patients with EMPD were identified based on the diagnostic names and codes in claim data.
A total of 53 males and 31 females with EMPD were found. The crude prevalence in 2016 was 0.04 per 100,000 population [95% confidence interval (CI) 0.02-0.06], ranging from 0.01 (95% CI 0.00-0.02) in North or Northeast China to 0.08 (95% CI 0.03-0.16) in Southwest China. The rate was higher in males (0.05, 95% CI 0.03-0.08) compared with females (0.03, 95% CI 0.02-0.05). The mean age of patients was 65.87 (standard deviation: 14.21) years, with the peak prevalence appeared in patients aged 70-79 (0.28, 95% CI 0.16-0.42).
The prevalence of EMPD was markedly lower than those in the United States and Europe, and varied across regions in China. Chinese patients were much younger, with significant male predominance. Further studies are warranted to examine potential pathophysiologic mechanism.
外阴 Paget 病(EMPD)是一种上皮内腺癌。外阴 Paget 病的慢性复发性临床病程和难以忍受的临床症状通常导致生活质量显著下降。在世界上人口最多的国家中国,尚无关于 EMPD 的描述性流行病学的全国数据。这项基于人群的研究旨在估计中国 EMPD 的患病率以及相关的性别和年龄模式。
本研究使用了中国城镇职工基本医疗保险和城镇居民基本医疗保险的数据,该数据涵盖了 2016 年约 4.3 亿中国城市居民。根据理赔数据中的诊断名称和代码,确定 EMPD 患者。
共发现 53 名男性和 31 名女性 EMPD 患者。2016 年的粗患病率为 0.04/10 万[95%置信区间(CI)0.02-0.06],从中国北部或东北部的 0.01(95%CI 0.00-0.02)到中国西南部的 0.08(95%CI 0.03-0.16)不等。男性(0.05,95%CI 0.03-0.08)的患病率高于女性(0.03,95%CI 0.02-0.05)。患者的平均年龄为 65.87 岁(标准差:14.21),70-79 岁患者的患病率最高(0.28,95%CI 0.16-0.42)。
EMPD 的患病率明显低于美国和欧洲,且在中国各地区之间存在差异。中国患者年龄更小,男性明显居多。需要进一步研究以探讨潜在的病理生理机制。