Immune cellular regulation on autoantibody production in pemphigus

Pemphigus is an autoantibody-mediated blistering disease in the skin and mucous membranes. The autoantibodies primarily target desmoglein (Dsg)1 or/and Dsg3, transmembrane glycoproteins of skin epidermal cells, leading to loss of desmosome adhesion and acantholysis through signaling dependent and independent pathways. Thus, the pemphigus autoantibodies themselves and immune processes, particularly cellular regulation of antibody production, remain as interesting topics in probing pemphigus pathogenesis. In this review, we focus on current advances regarding how the pemphigus antibody production is highly regulated by the key immune effectors including T cells, B cells and their secreted cytokines. Specifically targeting these immune effectors involved in the pemphigus autoantibody production should provide better therapeutic options for disease treatment of pemphigus.