Over the last several decades, advances in the understanding of the pathogenesis of autoimmune blistering diseases has resulted in significant improvements in diagnosis and management. These improvements include new diagnostic assays and therapies targeted at specific disease mediators. Furthermore, the abundance of new therapies in clinic trials for autoimmune blistering diseases will translate to an enhanced therapeutic armamentarium for clinicians. The aim of this article is to review new developments in the understanding of autoimmune blistering diseases and to summarize advancements in their diagnosis and management.