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Serological diagnostics in the detection of IgG autoantibodies against human collagen VII in epidermolysis bullosa acquisita: a multicentre analysis
Author(s) -
Schmidt T.,
Hoch M.,
Lotfi Jad S.S.,
Solimani F.,
Di Zenzo G.,
Marzano A.V.,
Goebeler M.,
Cozzani E.,
Kern J.S.,
Sitaru C.,
Lakoš Jukić I.,
Sárdy M.,
Uzun S.,
Jedlickova H.,
Gläser R.,
Kaneda M.,
Eming R.,
Göpel G.,
Ishii N.,
Greene B.,
Hashimoto T.,
Hertl M.
Publication year - 2017
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/bjd.15800
Subject(s) - iif , epidermolysis bullosa acquisita , autoantibody , medicine , bullous pemphigoid , pemphigoid , mucocutaneous zone , anchoring fibrils , immunology , immunofluorescence , serology , autoimmune disease , dermatology , epidermolysis bullosa , pathology , antibody , disease , basement membrane
Summary Background Epidermolysis bullosa acquisita ( EBA ) is a rare, potentially devastating autoimmune disease of the skin. IgG autoantibodies directed against type VII collagen (Col7), the major component of anchoring fibrils, induce skin fragility leading to cutaneous and mucocutaneous blister formation, which is mostly of a scarring phenotype. Thus, powerful and reproducible diagnostic assays are critical to establish the diagnosis of EBA early to avoid irreversible sequelae. Objectives The present international, retrospective multicentre study included a large cohort of patients with EBA and evaluated the diagnostic power of four different diagnostic assays for the detection of anti‐Col7 IgG autoantibodies. Methods Overall, 95 EBA sera and 200 control sera consisting of 100 bullous pemphigoid sera, 50 pemphigus vulgaris sera and 50 sera of healthy controls were tested for anti‐Col7 IgG autoantibodies using indirect immunofluorescence ( IIF ), two commercial enzyme‐linked immunosorbent assay ( ELISA ) systems and Western blot ( WB ) analysis. EBA sera were taken from patients with positive direct immunofluorescence and IgG reactivity in at least one of the immunoserological assays ( IIF , ELISA , WB ). Results A Col7‐ NC 1/ NC 2 ELISA ( MBL , Nagoya, Japan) showed the highest sensitivity (97·9%), followed by a Col7‐ NC 1 ELISA (Euroimmun, Lübeck, Germany) (89·5%), WB with Col7‐ NC 1 (85·3%), and IIF on saline‐split human skin (74·7%). The specificities of both ELISA systems were comparable ( NC 1 98·7%, NC 1/ NC 2 99·3%). Furthermore, WB was more sensitive than IIF , which was more specific. Conclusions The two commercially available ELISA systems allow for a highly sensitive and specific diagnosis of EBA . The sensitivity of the Col7‐ NC 1/ NC 2 ELISA is significantly higher compared with the ELISA based on the Col7‐ NC 1 domain only.