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autoantibody; chimeric antigen receptor; desmoglein antibody; fumaric acid dimethyl ester; leukotriene B4 receptor antagonist; pemphigus antibody; phosphodiesterase IV inhibitor; autoantibody, blister; bullous pemphigoid; Conference Paper; dermatology; diagnostic procedure; experimental mouse; human; immunopathogenesis; immunoreactivity; incidence; medical society; nonhuman; pathogenesis; pemphigus; prevalence; priority journal; scientist; serology; treatment indication; animal; Autoimmune Diseases; consensus; female; Germany; immunology; male; mouse; pathophysiology; Pemphigoid, Bullous; pemphigus; prognosis; risk assessment, Animals; Autoantibodies; Autoimmune Diseases; Consensus; Female; Germany; Humans; Male; Mice; Pemphigoid, Bullous; Pemphigus; Prognosis; Risk Assessment
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. © 2017 The Authors
