Pathogenic Autoantibody Derived from Regulatory T Cell‒Deficient Scurfy Mice Targets Type VII Collagen and Leads to Epidermolysis Bullosa Acquisita‒Like Blistering Disease.

Autoimmune blistering diseases (AIBDs) are severe human autoimmune diseases affecting the skin and mucous membranes (Witte et al., 2018). Patients with AIBDs develop pathogenic autoantibodies targeting structural skin proteins and causing intraepidermal blisters to form as in pemphigus vulgaris or at the basement membrane zone (BMZ) as in pemphigoid diseases (Schmidt and Zillikens, 2011). For many AIBDs, the events after autoantibody binding have been elucidated. However, early events in the pathogenic cascade leading to autoantibody production are still not fully understood.