Life-threatening bullous dermatoses: Pemphigus vulgaris.
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.
Ruocco
Eleonora
E
Department of Dermatology, Second University of Naples, Italy.
Baroni
Adone
A
Wolf
Ronni
R
Ruocco
Vincenzo
V
D001327:Autoimmune Diseases / Q000503:physiopathology; D016638:Critical Illness; D006801:Humans; D010392:Pemphigus / Q000503:physiopathology*