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.
Life-threatening bullous dermatoses: Pemphigus vulgaris
RUOCCO, Eleonora;BARONI, Adone;RUOCCO, Vincenzo
2005
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.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
