MD TP67: Experimental mouse model for Pemphigus vulgaris

Pemphigus vulgaris is an autoimmune skin disease characterized by intraepithelial blisters. Keratinocytes in the epidermis of the skin and mucous membranes lose their cell-cell adhesion due to autoantibodies against desmoglein (dsg) 1 and dsg 3.

By injecting a single chain variable fragment (scFv) against dsg 1and dsg 3 in an ex vivo skin organ culture in human and murine skin we could mimic the intraepidermal split formation. Next, we want to establish an adult mouse model with mucocutaneous skin lesions. Furthermore, we will test various novel inhibitory compounds next to standard glucocorticoid treatment thought to improve the disease outcome of pemphigus vulgaris.