The present review focuses on BP180, the main autoantigen of the DEJ, and highlights the fine autoantibody specificities against this autoantigen targeted in four PD, i.e. bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), linear IgA dermatosis, and pemphigoid gestationis (16, 17). This evidence concerns the gene COL17A1 and linear IgA Dermatosis.