Bullous pemphigoid (BP) has a relatively higher incidence in Europe, females, older people and high-income level country, and prevalence of BP was found to be higher in Asia. The studies analyzed showed high heterogeneity thus necessitating caution when interpreting these findings.

• This systematic review and meta-analysis of observational studies was conducted to estimate the incidence and prevalence of BP.

• For BP, a global incidence of 0.0419 per 1,000 person-years was estimated.

• The incidence of BP was estimated to be 0.047 per 1,000 person-years, 0.0419 per 1,000 person-years, 0.0072 per 1,000 person-years, 0.003 per 1,000 person-years in North America, Europe, Asia and Africa, respectively.

• The incidence for females and males was 0.0202 per 1,000 person-years and 0.0181 per 1,000 person-years, respectively.

• For age groups < 50, 50-59, 60-69, 70-79, 80-89 and ≥90 years, the estimated incidence was 0.001 per 1,000 person-years, 0.002 per 1,000 person-years, 0.004 per 1,000 person-years; 0.007 per 1,000 person-years, 0.011 per 1,000 person-years, 0.017 per 1,000 person-years, respectively.

• Pooled clinic-based prevalence was estimated to be 0.79 %, and 1.13%, 0.21%, 0.13% for Asia, Africa and Europe.

• In this study, the global incidence as well as prevalence of BP was described in terms of spatial and population distributions and among various income levels.