TY - JOUR
T1 - Meeting Report of the Pathogenesis of Pemphigus and Pemphigoid Meeting in Munich, September 2016
AU - Schmidt, Enno
AU - Spindler, Volker
AU - Eming, Rüdiger
AU - Amagai, Masayuki
AU - Antonicelli, Frank
AU - Baines, John F.
AU - Belheouane, Meriem
AU - Bernard, Philippe
AU - Borradori, Luca
AU - Caproni, Marzia
AU - Di Zenzo, Giovanni
AU - Grando, Sergei
AU - Harman, Karen
AU - Jonkman, Marcel F.
AU - Koga, Hiroshi
AU - Ludwig, Ralf J.
AU - Kowalczyk, Andrew P.
AU - Müller, Eliane J.
AU - Nishie, Wataru
AU - Pas, Hendri
AU - Payne, Aimee S.
AU - Sadik, Christian D.
AU - Seppänen, Allan
AU - Setterfield, Jane
AU - Shimizu, Hiroshi
AU - Sinha, Animesh A.
AU - Sprecher, Eli
AU - Sticherling, Michael
AU - Ujiie, Hideyuki
AU - Zillikens, Detlef
AU - Hertl, Michael
AU - Waschke, Jens
PY - 2017/6/1
Y1 - 2017/6/1
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=85019921840&partnerID=8YFLogxK
U2 - 10.1016/j.jid.2017.01.028
DO - 10.1016/j.jid.2017.01.028
M3 - Journal articles
C2 - 28390814
AN - SCOPUS:85019921840
SN - 0022-202X
VL - 137
SP - 1199
EP - 1203
JO - Journal of Investigative Dermatology
JF - Journal of Investigative Dermatology
IS - 6
ER -