Autoreactive T cells in the immune pathogenesis of pemphigus vulgaris

Authors

  • Kyle T. Amber,

    Corresponding author
    1. Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, FL, USA
    • Correspondence: Kyle T. Amber, BS, Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, 10660 SW 75th Ave, Miami, FL 33156, USA, Tel.: 305-609-2110, Fax: 305-663-0844, e-mail: KAmber@med.miami.edu

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  • Patrick Staropoli,

    1. Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, FL, USA
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  • Michael I. Shiman,

    1. Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, FL, USA
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  • George W. Elgart,

    1. Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, FL, USA
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  • Michael Hertl

    1. Department of Dermatology and Allergology, Philipps-Universität, Marburg, Germany
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Abstract

Pemphigus vulgaris is a life-threatening autoimmune blistering disease caused by anti-desmoglein IgG 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.

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