The pooled ORs and 95% CIs indicated that four HLA variants were associated with a significantly increased frequency in pemphigus patients: HLA-DRB1*04 (genotype: OR 4.86; 95% CI 3.61–6.54; p < 0.00001; allele: OR 4.18; 95% CI 3.14–5.56; p < 0.00001), HLA-DRB1*14 (genotype: OR 4.81; 95% CI 2.88–8.05; p < 0.00001; allele: OR 6.14; 95% CI 4.98–7.58), HLA-DQB1*03 (genotype: OR 2.77; 95% CI 1.56–4.92; p = 0.0005; allele: OR 1.99; 95% CI 1.39–2.83; p = 0.0002) and HLA-DQB1*05 (genotype: OR 4.3; 95% CI 2.53–7.28; p < 0.00001; allele: OR 3.04; 95% CI 2.10–4.41; p < 0.00001). Here, HLA-DRB1 is linked to pemphigus.