Pemphigus family is a group of autoimmune blistering diseases.
Pemphigus vulgaris, the prototype of the pemphigus family, is a serious, acute or chronic, bullous, autoimmune disease of skin and mucous membranes that is often fatal unless treated with immunosuppressive agents.
A loss of normal cell-to-cell adhesion in the epidermis occurs as a result of circulating antibodies of the IgG class;
These antibodies bind to cell surface glycoproteins of the epidermis and induce acantholysis, probably by the activation of serine proteases;
The superficial subtypes of pemphigus are associated with autoantibodies to desmoglein 1, a 160 kD transmembrane desmosomal component;
The deep subtypes of pemphigus are associated with autoantibodies to desmoglein 3, a 130 kd transmembrane desmosomal component, and to desmoglein 1.