Pemphigus and pemphigoid are considered to be pro-totypic bullous disorders based on their well-characterized immune pathogenesis. Apart from pemphigus and BP, there is only circumstantial evidence that auto-reactive T cells are present and involved in the patho-genesis of the autoimmune bullous disorders epider-molysis bullosa acquisita and dermatitis herpetiformis. In PV and BP, autoreactive CD4+ T lymphocytes that are presumably crucial in initiating the autoimmune response recognize distinct epitopes of the extracellular portions of Dsg3 and BP180, components of desmo-somal and hemidesmosomal adhesion complexes of human skin, respectively. Dsg3- and BP180-reactive T cells produce T-helper 2 (Th2) cytokines, such as IL-4,
Fig. 8.3. Schematic overview of the immune pathogenesis of pemphigus vulgaris. Pemphigus vulgaris (PV) is the prototype of an autoanti-body-mediated immunobul-lous skin disorder and is characterized by a loss of intraepidermal adhesion primarily caused by IgG auto-antibodies specific for des-moglein (Dsg)3 and Dsg1, components ofthe desmo-somal adhesion complex of epidermal keratinocytes that are connected to the keratin cytoskeleton through interaction with the intracellular plaque proteins plakoglobin (PG) and desmoplakin (DP) (inset). IgG production by the autoreactive B cells is presumably regulated by Dsg3- and Dsg1-reactive Th1 and Th2 cells
IL-5 and IL-13, and presumably foster the production of autoantibodies of the Th2-dependent IgG4 subtype which are preferentially seen in active stages of these disorders (Fig. 8.3).
Rituximab (Anti-CD20 Monoclonal Antibody) in the Treatment of Autoimmune Bullous Skin Disorders 8.2.1
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