Schematic representation of the attachment complexes at the dermal-epidermal basement membrane zone, which form a continuous network of interacting proteins necessary for stable association of epidermis and dermis at the dermal-epidermal junction. Note the presence of hemidesmosomal components, plectin, α6β4 integrin, and the type XVII collagen/the 180 kD bullous pemphigoid antigen 2 (BPAG2). The critical role of the hemidesmosomal proteins in the integrity of the skin is indicated by mutations in the corresponding genes that result in dermal-epidermal separation. (Adapted from Pulkkinen L, Uitto J. Hemidesmosomal variants of epidermolysis bullosa. Mutations in the alpha6beta4 integrin and the 180-kD bullous pemphigoid antigen/type XVII collagen genes. Exp Dermatol 1998;7:46–64., with permission)