β2-microglobulin–deficient Mice Are Resistant to Bullous Pemphigoid

β2-microglobulin–deficient Mice Are Resistant to Bullous Pemphigoid

Recent understanding of the mechanism of immunoglobulin G (IgG) catabolism has yielded new insight into antibody-mediated diseases. We proposed that beta2-microglobulin (beta2m)-deficient mice have been protected from systemic lupus erythematosis (SLE)-like syndromes because they lack the beta2m-associated IgG protection receptor (FcRn) and therefore catabolize IgG, including pathogenic IgG aut...

Gelatinase B–deficient Mice Are Resistant to Experimental Bullous Pemphigoid

Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease characterized by deposition of autoantibodies at the basement membrane zone. In an experimental BP model in mice, the subepidermal blistering is mediated by antibodies directed against the hemidesmosomal protein BP180 (collagen XVII, BPAG2), and depends on complement activation and neutrophil infiltration. Gelatinase B is ...

Bullous pemphigoid

Bullous pemphigoid

Disease summary: Bullous Pemphigoid is an acquired, chronic, blistering autoimmune subepidermal bullous disease in which autoantibodies are directed against component of basement membrane zone of the skin [1]. It is characterized by formation of cutaneous bullae on the skin and mucous membrane. The pathogenesis involves migration of inflammatory cells into subepithilial tissues due to activatio...

Bullous pemphigoid.

B ullous pemphigoid is an autoimmune disease, which means that the cells in the body that normally fight infection attack the body instead. The body’s immune system is confused and makes an antibody (type of protein used to fight infection) that targets a part of the skin that normally holds it together. The attack on the skin causes blisters (firm, fluid-filled bubbles on the skin) to form. Th...