title: Novel ELISA systems for antibodies to desmoglein 1 and 3: correlation of disease activity with serum autoantibody levels in individual pemphigus patients.
abstract: Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are intraepidermal blistering skin diseases. PV is characterised by autoantibodies directed against desmoglein (Dsg) 3 and in patients with the mucocutaneous variant also against Dsg 1, whereas in PF, only Dsg 1 is targeted. Here, ectodomains of Dsg 3 and Dsg 1 were recombinantly expressed in a human cell line (HEK293) and applied as authentic solid phases in ELISA test systems. Autoantibodies against Dsg 3 and/or Dsg 1 could be detected in 71 (100%) of 71 PV sera and against Dsg 1 in 48 (96%) of 50 PF sera. Control sera showed reactivity with Dsg 3 and Dsg 1 in 0.2% and 0.7%, respectively, of 401 healthy blood donors and in 2.1% of 48 randomly selected patients with bullous pemphigoid. No reactivity with Dsg 1 and 3 was detected in 21 patients with linear IgA disease. For both pemphigus variants, a statistically significant correlation between clinical severity and autoantibody levels was observed as demonstrated for 10 PV and 5 PF patients. In conclusion, the use of the ectodomains of Dsg 3 and 1 as target antigens expressed in a human cell line resulted in sensitive and specific ELISA systems for both diagnosis and monitoring of PV and PF.
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany. enno.schmidt@uk-sh.de
authors.forename: Enno
authors.initials: E
authors.lastname: Schmidt
authors.forename: Cornelia
authors.initials: C
authors.lastname: Dähnrich
authors.forename: Anke
authors.initials: A
authors.lastname: Rosemann
authors.forename: Christian
authors.initials: C
authors.lastname: Probst
authors.forename: Lars
authors.initials: L
authors.lastname: Komorowski
authors.forename: Sandra
authors.initials: S
authors.lastname: Saschenbrecker
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Schlumberger
authors.forename: Winfried
authors.initials: W
authors.lastname: Stöcker
authors.forename: Takashi
authors.initials: T
authors.lastname: Hashimoto
authors.forename: Eva-Bettina
authors.initials: EB
authors.lastname: Bröcker
authors.forename: Andreas
authors.initials: A
authors.lastname: Recke
authors.forename: Christian
authors.initials: C
authors.lastname: Rose
authors.forename: Detlef
authors.initials: D
authors.lastname: Zillikens
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood / Q000276:immunology*; D051183:Desmoglein 1 / Q000235:genetics / Q000276:immunology*; D051184:Desmoglein 3 / Q000235:genetics / Q000276:immunology*; D004797:Enzyme-Linked Immunosorbent Assay / Q000379:methods; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000276:immunology; D008297:Male; D008875:Middle Aged; D010391:Pemphigoid, Bullous / Q000276:immunology; D010392:Pemphigus / Q000175:diagnosis* / Q000276:immunology; D012372:ROC Curve; D011994:Recombinant Proteins / Q000276:immunology; D012680:Sensitivity and Specificity; D055815:Young Adult
chemical_list: D001323:Autoantibodies; C495297:DSG1 protein, human; C071894:DSG3 protein, human; D051183:Desmoglein 1; D051184:Desmoglein 3; D007074:Immunoglobulin G; D011994:Recombinant Proteins