Antibody transfer model of bullous pemphigoid

The repeated injection of anti-murine NC15A BP180 (type XVII collagen) IgG in adult mice results in a clinical and immunopathological phenotype that mirrors the human disease. Deposits of IgG and C3 along the dermal-epidermal junction are found in perilesional skin biopsies, subepidermal blistering and a mixed inflammatory infiltrate are found in the upper dermis, and erosions and erythema develop preferentially around the snout, ears, and neck (Am J Pathol, 2014).

Subsequent analyses have shown that clinical disease depends on

• FcγRIII (Am J Pathol, 2014)
• FcγRIV (Am J Pathol, 2014)
• an intact sugar moiety at Asp 297 of the Fc-portion (Am J Pathol, 2014)
• C5 (Front Immunol, 2018)
• C5aR1 (Front Immunol, 2018)
• C5aR2 (Front Immunol, 2018)
• IL-17A (J Autoimmun, 2019)

The following therapeutic interventions led to disease improvement:

• Enzymatic autoantibody glycan hydrolysis (Am J Pathol, 2014)
• Inhibition of FcγRIV (Am J Pathol, 2014)
• C5 (Front Immunol, 2018)
• IL-17A (J Autoimmun, 2019)