Human pemphigus autoantibodies are pathogenic to squamous epithelium

Abstract

In 1957, Witbesky et al. put forward several criteria that ideally should be fulfilled in order to prove the pathogenic role of an autoantibody in a putative autoimmune disease. There can now be very little doubt of the autoimmune nature of this disease and of the primary role of autoantibodies in its pathogenesis. The evidence that supports the concept that pemphigus autoantibodies are of primary pathogenic importance in the disease is as follows: IgG class autoantibodies can be found both circulating in the serum and bound to the epithelial cell surfaces in and around lesions in patients with pemphigus. These autoantibodies, purified from the serum of pemphigus patients, can induce acantholytic lesions typical of pemphigus both in experimental animals (neonatal mice) and in human and murine epidermal cell cultures. These autoantibodies react with a specific antigen of the epidermal cell. This purified antigen has been used to immunize rabbits and the resulting antibodies are capable of inducing pemphigus-like lesions in neonatal mice.