Mechanisms of immunologic injury. Pemphigus and bullous pemphigoid

Our understanding of bullous skin disorders has increased enormously since the beginning of the century, when a patient with generalized blisters was firmly diagnosed as having pemphigus vulgaris only on his demise. Those fortunate patients that survived obviously did not have true pemphigus vulgaris, but likely had "pemphigus of the aged" or "bullous dermatitis herpetiformis." The key observations that defined these diseases more precisely were the careful histopathologic studies of Lever and the immunofluorescence microscopy studies of Beutner and Jordon²and Jordon et al.³The autoantibodies in pemphigus vulgaris and bullous pemphigoid (BP) that can be detected both bound in and around lesions and circulating in the serum samples of patients have proved to be not only crucial for the accurate diagnosis of these conditions, but appear to be of primary importance in the pathogenesis of the diseases as well. There is now substantial evidence that pemphigus autoantibodies