The presence of other autoantibodies may interfere with the classical pathophysiological process of BP (12, 16, 17, 24, 33–36, 46). In BP patients without mucosal involvement, the serum concentration of anti-BP180 NC16A autoantibodies was well correlated with both the skin erythema/urticaria and the blisters/erosions BPDAI scores, which is in line with the fact that anti-BP180 autoantibody binding onto their target induced an inflammatory response leading to blister formation (14–18). By contrast, the correlation between anti-BP180 autoantibody titer and the skin blisters/erosions BPDAI score was not evidenced for BP patients with mucosal involvement. Of note, the BPDAI investigated the presence of both blisters and erosions. Thus, considering that the number of daily new blisters only displayed a higher tendency in BP patients with mucosal involvement, the highly significant difference in the BPDAI scores associated to blisters and erosions lesions may be related to the presence of erosions rather than to new blisters. This implies that the mechanisms leading to skin blisters/erosions and maybe to mucosal involvement too, rely on either different or additional trigger than anti-BP180-NC16A autoantibodies in BP patients with mucosal lesion. It was proposed that differential epitope recognition of BP180 could be associated with distinct clinical severity (5). However, additional studies are necessary to define the panel of autoantibodies associated with BP with mucosal lesions, to decipher the pathogenic role of these autoantibodies as well the immunologic mechanisms associated to their production.