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      Perspective From the 5th International Pemphigus and Pemphigoid Foundation Scientific Conference

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          The 5th Scientific Conference of the International Pemphigus and Pemphigoid Foundation (IPPF), “Pemphigus and Pemphigoid: A New Era of Clinical and Translational Science” was held in Orlando, Florida, on May 15–16, 2018. Scientific sessions covered recent, ongoing, and future clinical trials in pemphigus and bullous pemphigoid, disease activity and quality of life instruments, and the IPPF Natural History Study. Furthermore, the meeting provided an opportunity to hear firsthand from patients, investigators, and industry about their experience enrolling for clinical trials.

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          Most cited references 14

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          Reengineering chimeric antigen receptor T cells for targeted therapy of autoimmune disease

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            First-line rituximab combined with short-term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): a prospective, multicentre, parallel-group, open-label randomised trial.

            High doses of corticosteroids are considered the standard treatment for pemphigus. Because long-term corticosteroid treatment can cause severe and even life-threatening side-effects in patients with this disease, we assessed whether first-line use of rituximab as adjuvant therapy could improve the proportion of patients achieving complete remission off-therapy, compared with corticosteroid treatment alone, while decreasing treatment side-effects of corticosteroids.
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              In vitro and in vivo models to investigate the pathomechanisms and novel treatments for pemphigoid diseases.

              Pemphigoid diseases (PD) are a subgroup of rare acute or chronic autoimmune skin disorders characterized and caused by autoantibodies directed against distinct structural components of the dermal-epidermal junction. Binding of autoantibodies to their targets leads to the formation of blisters and erosions in patients. PDs comprise eight disorders for which the molecular target antigens have been identified. First, we review the available in vitro and ex vivo models for analysis of distinct aspects of the pathogenesis of PDs. This includes the binding of autoantibodies to skin sections, the analysis of blister formation capability and skin complement activation as well as investigation of neutrophil and keratinocyte activation. In addition to this, several animal models of PD have been developed during the last decades. These animal models have greatly contributed to our current understanding of the pathogenesis of PDs. We summarize spontaneously arising PD in animals and the induction of PD by transfer of (auto)antibodies, transfer of (auto)-antigen-specific lymphocytes and by immunization. In combined use, these models allow dissecting all aspects of PD pathogenesis, for example loss of tolerance, autoantibody production and inflammatory skin processes that lead to blister formation. Overall, we aimed to foster translational biomedical research, to deepen our understanding of PD pathogenesis and to develop novel treatments for patients suffering from these life-threatening and difficult-to-treat autoimmune diseases.

                Author and article information

                Front Med (Lausanne)
                Front Med (Lausanne)
                Front. Med.
                Frontiers in Medicine
                Frontiers Media S.A.
                08 November 2018
                : 5
                1Department of Dermatology, University of Pennsylvania , Philadelphia, PA, United States
                2Corporal Michael J. Crescenz VAMC , Philadelphia, PA, United States
                3Department of Dermatology, Duke University , Durham, NC, United States
                4Department of Dermatology and Allergology, Philipps-Universität Marburg , Marburg, Germany
                5Department of Dermatology, University of Iowa , Iowa City, IA, United States
                6Orphan Disease Center, Perelman School of Medicine, University of Pennsylvania , Philadelphia, PA, United States
                7Centre of Evidence Based Dermatology, University of Nottingham , Nottingham, United Kingdom
                8Department of Dermatology, University Medical Center Groningen, University of Groningen , Groningen, Netherlands
                9Department of Dermatology, University Hospitals Cleveland Medical Center, Case Western Reserve University , Cleveland, OH, United States
                10Department of Dermatology, Lübeck Institute of Experimental Dermatology, University of Lübeck , Lübeck, Germany
                11Department of Dermatology, University of New South Wales , Sydney, NSW, Australia
                12Department of Dermatology, Rouen University Hospital , Rouen, France
                13Program for Clinical Research, Department of Dermatology, University of California, San Francisco , San Francisco, CA, United States
                14Department of Dermatology, Allergy, and Venereology, University of Lübeck , Lübeck, Germany
                15Department of Dermatology, Keio University , Tokyo, Japan
                16International Pemphigus and Pemphigoid Foundation , Sacramento, CA, United States
                Author notes

                Edited by: Sergei Grando, University of California, Irvine, United States

                Reviewed by: Takashi Hashimoto, Graduate School of Medicine, Osaka University, Japan; Oleg E. Akilov, University of Pittsburgh, United States

                *Correspondence: Aimee S. Payne aimee.payne@

                This article was submitted to Dermatology, a section of the journal Frontiers in Medicine

                Copyright © 2018 Lee, Werth, Hall, Eming, Fairley, Fajgenbaum, Harman, Jonkman, Korman, Ludwig, Murrell, Musette, Naik, Sadik, Yamagami, Yale and Payne.

                This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 16, Pages: 5, Words: 4269

                leukotriene, fumarate, t-cell, eotaxin, fcrn, btk, rituximab, doxycycline


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