How does acantholysis occur in pemphigus vulgaris: a critical review.
Pemphigus vulgaris is a life-threatening autoimmune blistering disease targeting skin and mucous membranes, characterized by disruption of keratinocytes' adhesion termed acantholysis. Today multiple classes of targets are considered to play a role in the genesis of the acantholysis; of these, the classical pemphigus antigens, desmosomal cadherins (desmoglein 1 and 3) are the best characterized and considered as the most important. Additional antigens include the novel epithelial acetylcholine receptors (alpha9 and pemphaxin). Thus, acantholysis in pemphigus seems to result from a cooperative action of antibodies to different keratinocyte self-antigens, but the mechanisms by which epithelial cleft occurs are not yet clearly understood. In fact, the binding of the autoantibodies to these targets generates a plethora of biological effects due, on one hand, to their direct interference with adhesive function and, on the other, to more complex events involving intracellular pathways that modify proteases activity or calcium metabolism, leading to loss of cell-cell adhesion.
Lanza
Alessandro
A
Regional Center on Craniofacial Malformations, School of Medicine, II University of Naples, 80100 Naples, Italy. sandrolanza80@libero.it
Cirillo
Nicola
N
Femiano
Felice
F
Gombos
Fernando
F
D000051:Acantholysis / Q000276:immunology / Q000503:physiopathology*; D001323:Autoantibodies / Q000276:immunology; D001324:Autoantigens / Q000276:immunology; D015551:Autoimmunity; D006801:Humans; D015603:Keratinocytes / Q000276:immunology; D010392:Pemphigus / Q000276:immunology / Q000503:physiopathology*
D001323:Autoantibodies; D001324:Autoantigens