PEMPHIGUS - FROM IMMUNOFLUORESCENCE TO MOLECULAR-BIOLOGY

Since the discovery of autoantibodies in patients with pemphigus, pemp higus has been intensively studied by dermatologists and cutaneous or cellular biologists by means of various techniques including immunoflu orescence, immunoelectron microscopy, immunoprecipitation, immunoblott ing, and molecular biology. In this article, up-dated topics on pemphi gus obtained by each individual technique are reviewed. In the course of immunofluorescence studies on unusual cases of blistering diseases, a new entity characterized by immunoglobulin A (IgA)-type autoantibod ies directed against keratinocyte cell surfaces has been discovered. I mmunoelectron microscopy using low temperature post-embedding gold lab eling enabled us to quantitate binding sites of pemphigus autoantibodi es within desmosomes at different levels of epidermis. Immunoprecipita tion and immunoblot analyses allowed us to characterize antigen comple xes in paraneoplastic pemphigus. Finally, approaches using molecular b iology not only have given us a fundamental insight that pemphigus aut oantigen is a cadherin-type cell adhesion molecule both in pemphigus v ulgaris and pemphigus foliaceus, but also provided tools to develop no vel diagnostic and therapeutic strategies.