The Global Incidence Rate of Pemphigus Vulgaris: A Systematic Review and Meta-Analysis

Background: Pemphigus vulgaris is a life-threatening autoimmune bullous disease characterized by flaccid blister formation. As there has been no macroscopic assessment of epidemiological characteristics, its disease burden in the general population remains unknown. Objectives: The aim of this study was to assess the global incidence rate of pemphigus vulgaris in the general population. Methods: The search was conducted in databases including Medline, Embase, Web of Science, and the Cochrane Library from inception to May 1, 2022. We included original studies that either reported the incidence of pemphigus vulgaris or provided raw data for calculating. Studies based on a specific population instead of the general population were excluded. Individual studies were summarized using random-effects mode. The pooled incidence rate of pemphigus vulgaris among the general population and subgroups was obtained. Heterogeneity (I2 statistic) was assessed with the χ2 test on Cochrane’s Q statistic. Results: Twenty-nine studies were eligible for final analysis, and the pooled incidence rate of pemphigus vulgaris was 2.83 per million person-years (95% CI, 2.14–3.61). The incidence rate was similar between men and women and remained stable in the past half-century. Southern Asia showed the highest rate among subcontinents that had more than one study conducted as 4.94 per million person-years (95% CI, 2.55–8.10). Economic levels do not seem to have any bearing on incidence. Conclusions: Despite the substantial heterogeneity among studies, this meta-analysis revealed the worldwide incidence rate of pemphigus vulgaris for the first time and may assist in assessing global disease burden and promoting health policy.