Pemphigus vulgaris is an autoimmune blistering disease of the skin and mucous membrane. It is mediated by autoantibody against desmoglein 3, an intracellular adhesion glycoprotein that forms a part of desmosome. Loss of tolerance to this autoantigen happens for a still unknown reason. However, this disease has a very strong association with some human leukocyte antigen molecules whose structure favors the binding of certain peptide of desmoglein 3. The mechanism of the action of autoantibody has turned out to be more complicated than it was thought, and it is not well established yet. Nevertheless, in the past few years, important discoveries have been made that point to a role of autoantibodies in staining different signaling cascades that lead to loss of adhesion between keratinocyte and death of these cells resulting in acantholysis as histologic manifestation and blister as clinical manifestation.