Prevalence of extramammary Paget\u2019s disease in urban China: a population-based study

Shilu Yin,Guozhen Liu,Jinxi Wang,Zhenmin Zhao,Siyan Zhan,Lu Xu,Jingnan Feng,Lili Liu,Pei Gao,Shengfeng Wang

doi:10.1186/s13023-021-01715-6

Abstract

BackgroundExtramammary 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.MethodsThis 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.ResultsA 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).ConclusionsThe 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.

Highlights

Extramammary Paget’s disease (EMPD) is an intraepithelial adenocarcinoma
Prevalence The 2016 prevalence of EMPD in urban China in 2016 was 0.04 per 100,000 population
In terms of the age trend of EMPD prevalence, the prevalence reached a peak at 70–79 years old (0.28, 95% confidence interval (CI) 0.16–0.42) (Fig. 1)

Summary

Introduction

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. Extramammary Paget’s disease (EMPD) is an intraepithelial adenocarcinoma which predominantly affects areas with a high apocrine gland concentration, including the vulva, perineal, perianal, scrotal, penile and axillae skin [1,2,3]. Epidemiological information of EMPD was limited, especially in the developing countries including China.

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Results

Discussion

Conclusion