Introduction

Over the centuries blistering skin disorders have been described under a variety of terms, such as pemphigus, plyctainia, phlyzakion. However, it is only about 40 years since Lever (1956), on the basis of distinctive clinical and his-tological features, recognized bullous pemphigoid as a distinct disorder within the large group of blistering disorders, including the pemphigus group. One milestone in the evolution of our understanding of bullous pemphigoid was the demonstration by Jordon et al. (Jordon et al. 1967) that the disease was associated with in vivo bound and circulating autoantibodies directed against the basement membrane zone of stratified epithelia. Today, BP has emerged as an example of organ-specific autoimmune disease and it represents the most frequent autoimmune blistering disorder.

In this review, we will discuss the clinical and immunopathological features of BP, its differential diagnoses and therapeutic options. We will also focus on recent progress in our understanding of the pathophysiology of this disorder and on the role of targeted autoantigens in the maintenance of epithelial-stromal adhesion.

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