title: Life-threatening bullous dermatoses: Pemphigus vulgaris.
abstract: Pemphigus vulgaris (PV) is a rare autoimmune bullous dermatosis with a high mortality rate if untreated. The disease results from autoimmunity to normal components of keratinocyte cell membrane (desmogleins 3 and 1) belonging to the cadherin supergene family. Standard therapy for PV is based on a combined administration of high-dosed glucocorticoids and immunosuppressive drugs. In patients with severe, life-threatening, or recalcitrant PV, stronger therapeutic options should be considered, such as 'pulse-therapy' with discontinuous intravenous infusion of megadoses of immunosuppressive drugs over a short-time, plasmapheresis, and extracorporeal immunoadsorption of pathogenic autoantibodies using the extracellular domain of the PV main antigen (desmoglein 3) produced by baculovirus or, more recently, a tryptophan-linked polyvinyl alcohol adsorber.
authors.affiliation: Department of Dermatology, Second University of Naples, Italy.
authors.forename: Eleonora
authors.initials: E
authors.lastname: Ruocco
authors.forename: Adone
authors.initials: A
authors.lastname: Baroni
authors.forename: Ronni
authors.initials: R
authors.lastname: Wolf
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Ruocco
mesh_terms: D001327:Autoimmune Diseases / Q000503:physiopathology; D016638:Critical Illness; D006801:Humans; D010392:Pemphigus / Q000503:physiopathology*