title: Urban legends: pemphigus vulgaris.
abstract: Pemphigus vulgaris (PV) is the most common type of pemphigus. PV pathogenesis is still debated, and treatment remains challenging. We investigated five controversial topics: (1) What are the target antigens in PV? (2) Do desmogleins adequately address PV pathophysiology? (3) How does acantholysis occur in PV? (4) Is PV still a lethal disease? (5) What is the role of rituximab (RTX) in PV treatment? Results from extensive literature searches suggested the following: (1) Target antigens of PV include a variety of molecules and receptors that are not physically compartmentalized within the epidermis. (2) PV is caused by a variety of autoantibodies to keratinocyte self-antigens, which concur to cause blistering by acting synergistically. (3) The concept of apoptolysis distinguishes the unique mechanism of autoantibody-induced keratinocyte damage in PV from other known forms of cell death. (4) PV remains potentially life-threatening largely because of treatment side effects, but it is uncertain which therapies carry the highest likelihood of lethal risk. (5) RTX is a very promising treatment option in patients with widespread recalcitrant or life-threatening PV. RTX's cost is an issue, its long-term side effects are still unknown, and randomized controlled trials are needed to establish the optimal dosing regimen.
authors.affiliation: Department of Oral and Dental Sciences, University of Bristol, Bristol, UK. n.cirillo@bristol.ac.uk
authors.forename: N
authors.initials: N
authors.lastname: Cirillo
authors.forename: E
authors.initials: E
authors.lastname: Cozzani
authors.forename: M
authors.initials: M
authors.lastname: Carrozzo
authors.forename: S A
authors.initials: SA
authors.lastname: Grando
mesh_terms: D000051:Acantholysis / Q000503:physiopathology; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D001324:Autoantigens / Q000502:physiology; D015815:Cell Adhesion Molecules / Q000502:physiology; D051182:Desmogleins / Q000502:physiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D010392:Pemphigus / Q000188:drug therapy / Q000276:immunology / Q000401:mortality / Q000503:physiopathology; D011494:Protein Kinases / Q000378:metabolism; D000069283:Rituximab; D014481:United States / Q000453:epidemiology
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D001324:Autoantigens; D015815:Cell Adhesion Molecules; D051182:Desmogleins; D007166:Immunosuppressive Agents; D000069283:Rituximab; D011494:Protein Kinases