Autoimmune bullous disorders are a group of severe skin diseases characterized clinically by blisters and erosions of skin and/or mucous membranes. A hallmark of these disorders is the presence of IgG and occasionally IgA autoantibodies that target distinct adhesion structures of the epidermis, dermoepidermal basement membrane, and anchoring fibrils of the dermis. This Review focuses on the potential role of autoreactive T cells in the pathogenesis of these disorders. Pemphigus vulgaris (PV) and bullous pemphigoid (BP) are the best-characterized bullous disorders with regard to pathogenesis and T cell involvement. Activation of autoreactive T cells in PV and BP is restricted by distinct HLA class II alleles that are prevalent in individuals with these disorders. Autoreactive T cells are not only present in patients but can also be detected in healthy individuals. Recently, a subset of autoreactive T cells with remarkable regulatory function was identified in healthy individuals and to a much lesser extent in patients with PV, suggesting that the occurrence of autoimmune bullous disorders may be linked to a dysfunction of Tregs.
Michael Hertl, Rüdiger Eming, Christian Veldman
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Clinical & Experimental Immunology | 2013 |
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2009 | |
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2009 | |
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Experimental Dermatology | 2008 |
B-Cell Depletion Immunotherapy in Pemphigus: Effects on Cellular and Humoral Immune Responses
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Rituximab Immunotherapy in Pemphigus: Therapeutic Effects Beyond B-Cell Depletion
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Clinical and Molecular Characterization of Autoimmune Bullous Diseases
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Journal of Investigative Dermatology | 2008 |
Experimental Extracorporeal Photopheresis Inhibits the Sensitization and Effector Phases of Contact Hypersensitivity via Two Mechanisms: Generation of IL-10 and Induction of Regulatory T Cells
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Detection of low avidity desmoglein 3-reactive T cells in pemphigus vulgaris using HLA-DRβ1⁎0402 tetramers
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Clinical Immunology | 2007 |
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Nothing but skin and bone
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