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Autoreactive T cells in the immune pathogenesis of pemphigus vulgaris
Author(s) -
Amber Kyle T.,
Staropoli Patrick,
Shiman Michael I.,
Elgart George W.,
Hertl Michael
Publication year - 2013
Publication title -
experimental dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.108
H-Index - 96
eISSN - 1600-0625
pISSN - 0906-6705
DOI - 10.1111/exd.12229
Subject(s) - pemphigus vulgaris , immunology , acantholysis , autoantibody , immune system , pemphigus , medicine , antigen , desmoglein 3 , antibody , autoimmune disease , immunosuppression
Pemphigus vulgaris is a life‐threatening autoimmune blistering disease caused by anti‐desmoglein I g G autoantibodies that finally lead to acantholysis presenting clinically as progressive blistering. Whilst the production of pathogenic antibodies is key to the development of pemphigus vulgaris, many immunological steps are required prior to autoantibody induction. We review advances in the understanding of these immunologic processes with a focus on human leucocyte antigen polymorphisms and antigen recognition, epitope spreading, central and peripheral tolerance, T helper differentiation, induction of pro‐ and anti‐inflammatory cytokines and T ‐cell regulation of B cells. Targeting autoaggressive T cells as regulators and stimulators of B ‐cell antibody production should allow for more specific therapeutic immune interventions, avoiding the global immunosuppression seen with many commonly used immunosuppressants in pemphigus vulgaris.