Their estimated incidence is two new cases/million inhabitants/year in central Europe. They present chronic evolution, with significant morbidity and mortality, as well as an important impairment in quality of life. They originate from the production of pathogenic autoantibodies (usually of the IgG class) directed against different proteins of desmosomes (desmogleins). The union of these autoantibodies to the components of the desmosomes compromises intraepidermal adhesion, leading to acantholysis and formation of vesicles, blisters, and erosions on the skin and/or mucous membranes.
Different types of pemphigus have been identified based on the clinical and histopathological characteristics, as well as on the specific antigens against which the autoantibodies are produced. The main forms are pemphigus vulgaris (PV) and pemphigus foliaceus (PF), but in the last decades non-classical forms of pemphigus have also been described: paraneoplastic pemphigus, pemphigus herpetiformis, and IgA pemphigus.