title: Meeting Report of the Pathogenesis of Pemphigus and Pemphigoid Meeting in Munich, September 2016.
abstract: Autoimmune blistering diseases are a heterogeneous group of about a dozen complex disorders that are characterized by intraepidermal (pemphigus) and subepidermal blistering (pemphigoid diseases and dermatitis herpetiformis). The Pathogenesis of Pemphigus and Pemphigoid Meeting, organized by the Departments of Dermatology in Lübeck and Marburg and the Institute of Anatomy and Cell Biology, Munich, was held in September 2016 in Munich. The meeting brought together basic scientists and clinicians from all continents dedicating their work to autoimmune blistering diseases. Considerable advances have been made in describing incidences and prevalences of these diseases and linking comorbidities with autoantibody reactivities and clinical variants, for example, dipeptidyl peptidase-IV inhibitor-associated noninflammatory bullous pemphigoid. Although new entities are still being described, diagnosis of most autoimmune blistering diseases can now be achieved using standardized and widely available serological test systems. Various experimental mouse models of pemphigus and pemphigoid disease are increasingly being used to understand mechanisms of central and peripheral tolerance and to evaluate more specific treatment approaches for these disorders, such as molecules that target autoreactive T and B cells and anti-inflammatory mediators, that is, dimethyl fumarate, phosphodiesterase 4, and leukotriene B4 inhibitors in pemphigoid disorders, and chimeric antigen receptor T cells in pemphigus. Very recent experimental data about the immunopathology and the determinants of autoantibody formation and keratinocyte susceptibility in pemphigus were discussed. With regard to cellular mechanisms leading to the loss of cell-cell adhesion, new ideas were shared in the field of signal transduction. Major steps were taken to put the various partly contradictory and controversial findings about the effects of pemphigus autoantibodies and other inflammatory mediators into perspective and broaden our view of the complex pathophysiology of this disease. Finally, two investigator-initiated multicenter trials highlighted doxycycline and dapsone as valuable medications in the treatment of bullous pemphigoid.
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany; Lübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, Germany. Electronic address: enno.schmidt@uksh.de.
authors.forename: Enno
authors.initials: E
authors.lastname: Schmidt
authors.affiliation: Institute of Anatomy and Cell Biology, Ludwig-Maximilians-Universität, Munich, Germany.
authors.forename: Volker
authors.initials: V
authors.lastname: Spindler
authors.affiliation: Department of Dermatology, Philipps University, Marburg, Germany.
authors.forename: Rüdiger
authors.initials: R
authors.lastname: Eming
authors.affiliation: Department of Dermatology, Keio University, Tokyo, Japan.
authors.forename: Masayuki
authors.initials: M
authors.lastname: Amagai
authors.affiliation: Department of Dermatology, University of Reims, Reims, France.
authors.forename: Frank
authors.initials: F
authors.lastname: Antonicelli
authors.affiliation: Max Planck Institute for Evolutionary Biology, Plön, and Institute for Experimental Medicine, University of Kiel, Kiel, Germany.
authors.forename: John F
authors.initials: JF
authors.lastname: Baines
authors.affiliation: Max Planck Institute for Evolutionary Biology, Plön, and Institute for Experimental Medicine, University of Kiel, Kiel, Germany.
authors.forename: Meriem
authors.initials: M
authors.lastname: Belheouane
authors.affiliation: Department of Dermatology, University of Reims, Reims, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Bernard
authors.affiliation: Department of Dermatology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.
authors.forename: Luca
authors.initials: L
authors.lastname: Borradori
authors.affiliation: Department of Dermatology, University of Florence, Florence, Italy.
authors.forename: Marzia
authors.initials: M
authors.lastname: Caproni
authors.affiliation: Molecular and Cell Biology Laboratory, Istituto Dermopatico dell'Immacolata (IDI)-IRCCS FLMM, Rome, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Di Zenzo
authors.affiliation: Institute for Immunology and Departments of Dermatology and Biological Chemistry, University of California, Irvine, California, USA.
authors.forename: Sergei
authors.initials: S
authors.lastname: Grando
authors.affiliation: Department of Dermatology, University Hospitals of Leicester, Leicester, UK.
authors.forename: Karen
authors.initials: K
authors.lastname: Harman
authors.affiliation: Department of Dermatology, University Medical Centre Groningen, University of Groningen, Groningen, The Netherlands.
authors.forename: Marcel F
authors.initials: MF
authors.lastname: Jonkman
authors.affiliation: Department of Dermatology, Kurume University School of Medicine, Kurume, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Koga
authors.affiliation: Lübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, Germany.
authors.forename: Ralf J
authors.initials: RJ
authors.lastname: Ludwig
authors.affiliation: Departments of Cell Biology and Dermatology, Emory University, Atlanta, Georgia, USA.
authors.forename: Andrew P
authors.initials: AP
authors.lastname: Kowalczyk
authors.affiliation: Department of Dermatology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland; Institute of Animal Pathology, University of Bern, Switzerland.
authors.forename: Eliane J
authors.initials: EJ
authors.lastname: Müller
authors.affiliation: Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
authors.forename: Wataru
authors.initials: W
authors.lastname: Nishie
authors.affiliation: Department of Dermatology, University Medical Centre Groningen, University of Groningen, Groningen, The Netherlands.
authors.forename: Hendri
authors.initials: H
authors.lastname: Pas
authors.affiliation: Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
authors.forename: Aimee S
authors.initials: AS
authors.lastname: Payne
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany.
authors.forename: Christian D
authors.initials: CD
authors.lastname: Sadik
authors.affiliation: Department of Psychiatry, Helsinki University Hospital, Helsinki, Finland.
authors.forename: Allan
authors.initials: A
authors.lastname: Seppänen
authors.affiliation: Mucosal and Salivary Biology, Dental Institute, King's College London & St John's Institute of Dermatology, Guy's and St Thomas' NHS Foundation Trust, London, UK.
authors.forename: Jane
authors.initials: J
authors.lastname: Setterfield
authors.affiliation: Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Shimizu
authors.affiliation: Department of Dermatology, Jacobs School of Medicine and Biomedical Sciences, University of Buffalo, Buffalo, New York, USA.
authors.forename: Animesh A
authors.initials: AA
authors.lastname: Sinha
authors.affiliation: Department of Dermatology, Tel Aviv Medical Center, Tel Aviv, Israel.
authors.forename: Eli
authors.initials: E
authors.lastname: Sprecher
authors.affiliation: Department of Dermatology, University Hospitals Erlangen, Erlangen, Germany.
authors.forename: Michael
authors.initials: M
authors.lastname: Sticherling
authors.affiliation: Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
authors.forename: Hideyuki
authors.initials: H
authors.lastname: Ujiie
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany.
authors.forename: Detlef
authors.initials: D
authors.lastname: Zillikens
authors.affiliation: Department of Dermatology, Philipps University, Marburg, Germany.
authors.forename: Michael
authors.initials: M
authors.lastname: Hertl
authors.affiliation: Institute of Anatomy and Cell Biology, Ludwig-Maximilians-Universität, Munich, Germany.
authors.forename: Jens
authors.initials: J
authors.lastname: Waschke
mesh_terms: D000818:Animals; D001323:Autoantibodies / Q000276:immunology; D001327:Autoimmune Diseases / Q000503:physiopathology / Q000628:therapy; D032921:Consensus; D005260:Female; D005858:Germany; D006801:Humans; D008297:Male; D051379:Mice; D010391:Pemphigoid, Bullous / Q000276:immunology* / Q000503:physiopathology* / Q000628:therapy; D010392:Pemphigus / Q000276:immunology* / Q000503:physiopathology* / Q000628:therapy; D011379:Prognosis; D018570:Risk Assessment
chemical_list: D001323:Autoantibodies