title: Prevalence of pemphigus and pemphigoid autoantibodies in the general population.
abstract: BACKGROUND
  Mucocutaneous blistering is characteristic of autoimmune bullous dermatoses (AIBD). Blisters are caused by autoantibodies directed against structural components of the skin. Hence, detection of specific autoantibodies has become a hallmark for AIBD diagnosis. Studies on prevalence of AIBD autoantibodies in healthy individuals yielded contradictory results.
  
  
  METHODS
  To clarify this, samples from 7063 blood donors were tested for presence of anti-BP180-NC16A, anti-BP230 and anti-Dsg1/3 IgG by indirect immunofluorescence (IF) microscopy using a biochip.
  
  
  RESULTS
  Cumulative prevalence of these autoantibodies was 0.9 % (CI: 0.7-1.1 %), with anti-BP180-NC16A IgG being most prevalent. Validation of IF findings using ELISA confirmed presence of autoantibodies in 7/15 (anti-Dsg1), 6/7 (anti-Dsg3), 35/37 (anti-BP180-NC16A) and 2/3 (anti-BP230) cases. Moreover, in 16 samples, anti-BP180-NC16A autoantibody concentrations exceeded the cut-off for the diagnosis of bullous pemphigoid. Interestingly, these anti-BP180-NC16A autoantibodies from healthy individuals formed immune complexes with recombinant antigen and dose-dependently activated neutrophils in vitro. However, fine-epitope mapping within NC16A showed a different binding pattern of anti-BP180-NC16A autoantibodies from healthy individuals compared to bullous pemphigoid patients, while IgG subclasses were identical.
  
  
  CONCLUSIONS
  Collectively, we here report a low prevalence of AIBD autoantibodies in a large cohort of healthy individuals. Furthermore, functional analysis shows differences between autoantibodies from healthy donors and AIBD patients.
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany. wiebke.jung@medizin.uni-luebeck.de.|Lübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, Germany. wiebke.jung@medizin.uni-luebeck.de.
authors.forename: Wiebke
authors.initials: W
authors.lastname: Prüßmann
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany. jasper.pruessmann@medizin.uni-luebeck.de.|Lübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, Germany. jasper.pruessmann@medizin.uni-luebeck.de.
authors.forename: Jasper
authors.initials: J
authors.lastname: Prüßmann
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany. hiroshi_koga@med.kurume-u.ac.jp.|Lübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, Germany. hiroshi_koga@med.kurume-u.ac.jp.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Koga
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany. Andreas.Recke@uksh.de.|Lübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, Germany. Andreas.Recke@uksh.de.
authors.forename: Andreas
authors.initials: A
authors.lastname: Recke
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany. hiriwata0927@yahoo.co.jp.|Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan. hiriwata0927@yahoo.co.jp.
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Iwata
authors.affiliation: Institute of Transfusion Medicine, University Hospital of Schleswig-Holstein, Lübeck and Kiel, Germany. david.juhl@uksh.de.
authors.forename: David
authors.initials: D
authors.lastname: Juhl
authors.affiliation: Institute of Transfusion Medicine, University Hospital of Schleswig-Holstein, Lübeck and Kiel, Germany. siegfried.goerg@uksh.de.
authors.forename: Siegfried
authors.initials: S
authors.lastname: Görg
authors.affiliation: Transfusion Medicine, Cellular Therapeutics and Hemostaseology, Clinics of the Ludwigs-Maximilians-University Munich, Munich, Germany. Reinhard.Henschler@med.uni-muenchen.de.
authors.forename: Reinhard
authors.initials: R
authors.lastname: Henschler
authors.affiliation: Department of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, Kurume, Japan. hashimot@med.kurume-u.ac.jp.
authors.forename: Takashi
authors.initials: T
authors.lastname: Hashimoto
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany. enno.schmidt@uksh.de.
authors.forename: Enno
authors.initials: E
authors.lastname: Schmidt
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany. detlef.zillikens@uksh.de.|Lübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, Germany. detlef.zillikens@uksh.de.
authors.forename: Detlef
authors.initials: D
authors.lastname: Zillikens
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany. saleh.ibrahim@uksh.de.|Lübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, Germany. saleh.ibrahim@uksh.de.
authors.forename: Saleh M
authors.initials: SM
authors.lastname: Ibrahim
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany. ralf.ludwig@uksh.de.|Lübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, Germany. ralf.ludwig@uksh.de.
authors.forename: Ralf J
authors.initials: RJ
authors.lastname: Ludwig
mesh_terms: D000328:Adult; D001323:Autoantibodies / Q000276:immunology*; D001324:Autoantigens / Q000276:immunology; D015551:Autoimmunity; D045744:Cell Line, Tumor; D002478:Cells, Cultured; D051182:Desmogleins / Q000276:immunology; D005260:Female; D006801:Humans; D008297:Male; D024041:Non-Fibrillar Collagens / Q000276:immunology; D010391:Pemphigoid, Bullous / Q000276:immunology*; D010392:Pemphigus / Q000276:immunology*; D015995:Prevalence; D000097805:Collagen Type XVII
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D051182:Desmogleins; D024041:Non-Fibrillar Collagens; D000097805:Collagen Type XVII