Antibody transfer model of mucous membrane pemphigoid

Mucous membrane pemphigoid (MMP) is together with pemphigus vulgaris the second most frequent autoimmune blistering diseases next to bullous pemphigoid. MMP is clinically characterized by predominant involvement of mucosal tissues, most frequently the mouth followed by conjunctivae, nasal and genital mucosa, and, less often, larynx, trachea and esophagus. In about one third of MMP patients, mild skin lesions can be found (Lancet, 2013). BP180 (type XVII collagen) and laminin 332 are the main autoantigens of MMP. Laminin 332 is a heterotrimer composed of an alpha3, beta3, and gamma2 chain and, like BP180, a structural protein of the dermal-epidermal junction (Exp Dermatol, 2017).

By the repeated injection of anti-murine alpha3 laminin IgG in adult mice major clinical and immunopathological features of the human disease were replicated, i.e. (i) deposits of IgG and C3 along the dermal-epidermal junction, (ii) subepidermal splitting occurring in the lower lamina lucida, (iii) oral and conjunctival erosions, and (iv) skin lesions (J Invest Dermatol, 2017).

Subsequent analyses have shown that clinical disease depends on