Pemphigus vulgaris (PV) is the most frequent condition inside the pemphigus group, especially in Europe and North America. It is the prototype of a genetically mediated disease. The HLA alleles associated with PV are well-known. Recent studies found an association between disease severity and some HLA DRB1 and DQB1 alleles. The author of this book discusses the management of PV that should be started with an evaluation of disease severity.