Karincaoglu, Yelda2024-08-042024-08-0420082717-63982651-5164https://hdl.handle.net/11616/94647Pemphigus, a group of bullous diseases affecting the oral mucosa and the skin, is caused by antibody-mediated autoimmune reaction to desmogleins (Dsg), desmosomal transmembrane glycoproteins, leading to acantholysis. Pemphigus has a worldwide distribution but the incidence in patients of Jewish origin is higher. The disease has a peak incidence of occurrence between the 4th and 6th decades. While various environmental factors have been implicated as triggering agents, HLA association is probably the most important predisposing factor Pemphigus, is caused by antibody-mediated autoimmune reaction to desmosomal cadherins, Dsg1, and Dsg3. Recent molecular studies have shown that acantholysis can occur also in the presence of antibodies against 9 alpha nicotinic acetylcholine receptor. Pemphigus is currently divided into three distinct varieties, i.e., pemphigus vulgaris (PV), pemphigus foliaceus (PF) and other variants of pemphigus, depending on clinical features, the level of separation in the epidermis, and immunologic characteristics of auto-antigens. Blistering pathogenesis differ for each of the types of pemphigus. PV is characterized by IgG autoantibodies against Dsg 3, whereas the target of PF is Dsgl, although about 50% of PV patients also have Dsg1 autoantibodies. Lesion distribution is related to the location of the antigen (Dgs 3 and/or Dgs 1) in the epithelium and specific autoantibody production. This article reviews the epidemiology and pathogenesis of pemphigus. (Turkderm 2008; 42 Suppl 1: 1-4)trinfo:eu-repo/semantics/closedAccessPemphigusepidemiologypathogenesisArticle42142-s2.0-51549086293Q4WOS:000261615400001N/A