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Journal of the American Academy of Dermatology
Volume 74, n° 6
pages 1166-1172 (juin 2016)
Doi : 10.1016/j.jaad.2016.01.005
accepted : 1 January 2016
Original Articles

Comparison of 3 type VII collagen (C7) assays for serologic diagnosis of epidermolysis bullosa acquisita (EBA)

Vannina Seta a, Françoise Aucouturier, PhD b, Jonathan Bonnefoy, PhD c, d, Christelle Le Roux-Villet, MD a, Valérie Pendaries, PhD e, Marina Alexandre, MD a, Sabine Grootenboer-Mignot, MD, PhD f, Michel Heller, PhD g, Nicole Lièvre g, Liliane Laroche, MD, PhD a, Frédéric Caux, MD, PhD a, Matthias Titeux, PhD c, d, Alain Hovnanian, MD, PhD c, d, h, Catherine Prost-Squarcioni, MD, PhD a, g, i,
a Department of Dermatology, APHP, Avicenne Hospital, Bobigny, France 
i Department of Pathology, APHP, Avicenne Hospital, Bobigny, France 
b Department of Immunology, Assistance Publique des Hôpitaux de Paris (APHP), Saint-Louis Hospital, Paris, France 
c Institut National de la Santé et de la Recherche Médicale (INSERM), Unité Mixte de Recherche (UMR) 1163, Institut Imagine, Paris, France 
d Université Paris Descartes–Sorbonne Paris Cité, Paris, France 
e INSERM, U563, Purpan Hospital, Toulouse, France 
f Department of Immunology, APHP, Bichat Hospital, Paris, France 
g Department of Histology, Unité de Formation et de Recherche (UFR) Léonard de Vinci, University Paris 13, Bobigny, France 
h Department of Genetics, APHP, Necker–Enfants Malades Hospital, Paris, France 

Reprint requests: Catherine Prost-Squarcioni, MD, PhD, Department of Dermatology, APHP, Avicenne Hospital, 125, route de Stalingrad, 93009 Bobigny Cedex, France.Department of DermatologyAPHPAvicenne Hospital125, route de StalingradBobigny Cedex93009France

Serologic diagnosis of epidermolysis bullosa acquisita (EBA) relies on the detection of circulating autoantibodies to type VII collagen (C7).


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.


C7-NC1/NC2 ELISA, IIFT, and full-length–C7 ELISA were run on 77 nonselected consecutive EBA sera.


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.


The retrospective design was a limitation.


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.

The full text of this article is available in PDF format.

Key words : autoimmune bullous disease, biochip, enzyme-linked immunosorbent assay, epidermolysis bullosa acquisita, indirect immunofluorescence, sensitivity, serologic diagnosis, type VII collagen

Abbreviations used : AFz, AIBD, AU, BP, C7, cEBA, EBA, ELISA, iEBA, IEM, IIF, IIFT, MMp, NC, NHS, SSS, SSS/F+, SSS/F

 Drs Prost-Squarcioni and Hovnanian have equally contributed to this work and share last authorship.
 Supported by GENEGRAFT FP17 European Project and Dystrophic Epidermolysis Bullosa Research Association–France.
 Conflicts of interest: None declared.

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