title: Comparison of 3 type VII collagen (C7) assays for serologic diagnosis of epidermolysis bullosa acquisita (EBA).
abstract: BACKGROUND
  Serologic diagnosis of epidermolysis bullosa acquisita (EBA) relies on the detection of circulating autoantibodies to type VII collagen (C7).
  
  
  OBJECTIVE
  We sought to compare the diagnostic performances of a commercialized enzyme-linked immunosorbent assay (ELISA) using C7 noncollagenous (NC) domains (C7-NC1/NC2 ELISA) and indirect immunofluorescence (IIF) biochip test on NC1-C7-expressing transfected cells (IIFT), with a full-length-C7 ELISA developed in our laboratory.
  
  
  METHODS
  C7-NC1/NC2 ELISA, IIFT, and full-length-C7 ELISA were run on 77 nonselected consecutive EBA sera.
  
  
  RESULTS
  C7-NC1/NC2 ELISA, IIFT, and full-length-C7 ELISA were positive, respectively, for: 30%, 27%, and 65% of the 77 sera; 43%, 32%, and 80% of 44 sera labeling the salt-split-skin (SSS) floor (F) by IIF (SSS/F(+)); 9%, 22%, and 47% of 32 SSS/F(-) sera; 28%, 28%, and 58% of classic EBA; 41%, 41%, and 82% of inflammatory EBA; and 18%, 0%, and 55% of mucous-membrane-predominant EBA. Significant differences for all sera were found between: the 2 ELISAs for the 77 sera, SSS/F(+) and SSS/F(-) sera, and IIFT versus full-length-C7 ELISA.
  
  
  CONCLUSIONS
  The retrospective design was a limitation.
  
  
  CONCLUSIONS
  C7-NC1/NC2 ELISA and IIFT sensitivities for serologic diagnoses of EBA were low. Full-length-C7 ELISA was significantly more sensitive and could serve as a reference test.
authors.affiliation: Department of Dermatology, APHP, Avicenne Hospital, Bobigny, France.
authors.forename: Vannina
authors.initials: V
authors.lastname: Seta
authors.affiliation: Department of Immunology, Assistance Publique des Hôpitaux de Paris (APHP), Saint-Louis Hospital, Paris, France.
authors.forename: Françoise
authors.initials: F
authors.lastname: Aucouturier
authors.affiliation: Institut National de la Santé et de la Recherche Médicale (INSERM), Unité Mixte de Recherche (UMR) 1163, Institut Imagine, Paris, France; Université Paris Descartes-Sorbonne Paris Cité, Paris, France.
authors.forename: Jonathan
authors.initials: J
authors.lastname: Bonnefoy
authors.affiliation: Department of Dermatology, APHP, Avicenne Hospital, Bobigny, France.
authors.forename: Christelle
authors.initials: C
authors.lastname: Le Roux-Villet
authors.affiliation: INSERM, U563, Purpan Hospital, Toulouse, France.
authors.forename: Valérie
authors.initials: V
authors.lastname: Pendaries
authors.affiliation: Department of Dermatology, APHP, Avicenne Hospital, Bobigny, France.
authors.forename: Marina
authors.initials: M
authors.lastname: Alexandre
authors.affiliation: Department of Immunology, APHP, Bichat Hospital, Paris, France.
authors.forename: Sabine
authors.initials: S
authors.lastname: Grootenboer-Mignot
authors.affiliation: Department of Histology, Unité de Formation et de Recherche (UFR) Léonard de Vinci, University Paris 13, Bobigny, France.
authors.forename: Michel
authors.initials: M
authors.lastname: Heller
authors.affiliation: Department of Histology, Unité de Formation et de Recherche (UFR) Léonard de Vinci, University Paris 13, Bobigny, France.
authors.forename: Nicole
authors.initials: N
authors.lastname: Lièvre
authors.affiliation: Department of Dermatology, APHP, Avicenne Hospital, Bobigny, France.
authors.forename: Liliane
authors.initials: L
authors.lastname: Laroche
authors.affiliation: Department of Dermatology, APHP, Avicenne Hospital, Bobigny, France.
authors.forename: Frédéric
authors.initials: F
authors.lastname: Caux
authors.affiliation: Institut National de la Santé et de la Recherche Médicale (INSERM), Unité Mixte de Recherche (UMR) 1163, Institut Imagine, Paris, France; Université Paris Descartes-Sorbonne Paris Cité, Paris, France.
authors.forename: Matthias
authors.initials: M
authors.lastname: Titeux
authors.affiliation: Institut National de la Santé et de la Recherche Médicale (INSERM), Unité Mixte de Recherche (UMR) 1163, Institut Imagine, Paris, France; Université Paris Descartes-Sorbonne Paris Cité, Paris, France; Department of Genetics, APHP, Necker-Enfants Malades Hospital, Paris, France.
authors.forename: Alain
authors.initials: A
authors.lastname: Hovnanian
authors.affiliation: Department of Dermatology, APHP, Avicenne Hospital, Bobigny, France; Department of Histology, Unité de Formation et de Recherche (UFR) Léonard de Vinci, University Paris 13, Bobigny, France; Department of Pathology, APHP, Avicenne Hospital, Bobigny, France. Electronic address: catherine.prost@aphp.fr.
authors.forename: Catherine
authors.initials: C
authors.lastname: Prost-Squarcioni
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D019540:Area Under Curve; D001323:Autoantibodies / Q000097:blood*; D002648:Child; D002675:Child, Preschool; D024143:Collagen Type VII / Q000276:immunology*; D004797:Enzyme-Linked Immunosorbent Assay / Q000379:methods; D016107:Epidermolysis Bullosa Acquisita / Q000097:blood* / Q000175:diagnosis*; D005260:Female; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D008297:Male; D008875:Middle Aged; D040081:Protein Array Analysis; D012372:ROC Curve; D012189:Retrospective Studies; D012698:Serologic Tests / Q000379:methods*; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D024143:Collagen Type VII
keywords: autoimmune bullous disease; biochip; enzyme-linked immunosorbent assay; epidermolysis bullosa acquisita; indirect immunofluorescence; sensitivity; serologic diagnosis; type VII collagen