In univariate analysis, clinical features associated with mucosal involvement at baseline were a number of daily new blisters >10 (P = 0.02), a higher total, skin and blister/erosion BPDAI (P = 0.004, P = 0.02, P = 0.001, respectively), and a severe disease according BPDAI (P = 0.04). The absence of anti-BP230 autoantibody also showed a tendency of association with a mucosal involvement (P = 0.08). By contrast, univariate analysis revealed no relationship between anti-BP180 autoantibody serum concentration and mucosal involvement. In multivariate analysis, the absence of serum anti-BP230 autoantibody was the only factor independently associated with mucosal involvement (OR 7.8; 95% CI, 3.1–19.6) (P < 0.0001).