Urban legends: pemphigus vulgaris

摘要: Pemphigus vulgaris (PV) is the most common type of pemphigus. PV pathogenesis still debated, and treatment remains challenging. We investigated five controversial topics: (1) What are target antigens in PV? (2) Do desmogleins adequately address pathophysiology? (3) How does acantholysis occur (4) Is a lethal disease? (5) role rituximab (RTX) treatment? Results from extensive literature searches suggested following: Target include variety molecules receptors that not physically compartmentalized within epidermis. caused by autoantibodies to keratinocyte self-antigens, which concur cause blistering acting synergistically. The concept apoptolysis distinguishes unique mechanism autoantibody-induced damage other known forms cell death. potentially life-threatening largely because side effects, but it uncertain therapies carry highest likelihood risk. RTX very promising option patients with widespread recalcitrant or PV. RTX's cost an issue, its long-term effects unknown, randomized controlled trials needed establish optimal dosing regimen.