Abstract
Pemphigus vulgaris (PV) is a complex, multifactorial, and polygenic disease, whose exact pathogenesis is difficult to pinpoint. Research aimed at further elucidating associated epidemiologic risk factors is hampered by its rare disease status. Further, a lack of centralization and standardization of available data makes the practical application of this information challenging. We comprehensively reviewed 90 PV articles from 26 different countries for parameters including age, sex, ethnicity, incidence, prevalence, and HLA allele association. Analysis of the aggregated data revealed several trends including linkage disequilibrium of HLA DRB1*0402 and DQB1*0302 alleles in a geographic distribution centered in Europe, with migratory spread into North and South America. Analysis of the geographic distribution of alleles also shows HLA DQB1*0503 in linkage disequilibrium with DRB1*1404 and DRB1*1401 clustering mainly in Europe, the Middle East, and Asian countries. HLA allele DRB1*04 and DRB1*14 were shown to be linked to the Hispanic population. HLA allele DRB1*0804 is demonstrated to be highly associated with Brazilian and Egyptian descent. The highest incidence of PV was found in Iran with 5 per 100,000 people per year. The lowest incidence was found in Germany with 0.035 per 100,000 people per year. The highest incidences are geographically distributed around the Mediterranean and Middle East. Reported prevalances ranged from 0.38 per 100,000 people in Bulgaria to 30 per 100,000 people in Iran. The mean age of patients ranged from 36.5 years in Kuwait to 71 years in the United Kingdom, with an overall average mean age of 49.5 years across all studies. Female-to-male ratios ranged from 0.46 to 4.4, with a mean ratio of 1.8 across all studies analyzed. Overall, our analysis reveals global trends in the demographics and inheritance of PV that provide a more detailed insight into disease parameters to help inform future studies aimed at unraveling this disease’s complex pathogenesis.
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