Format

Send to:

Choose Destination
See comment in PubMed Commons below
J Am Acad Dermatol. 2013 Mar;68(3):e89-95. doi: 10.1016/j.jaad.2011.12.032. Epub 2012 Feb 16.

Sensitive and specific assays for routine serological diagnosis of epidermolysis bullosa acquisita.

Author information

  • 1Institute of Experimental Immunology, EUROIMMUN AG, Luebeck, Germany.

Abstract

BACKGROUND:

Epidermolysis bullosa acquisita (EBA) is a severe autoimmune subepidermal blistering disease characterized by autoantibodies against the N-terminal collagenous domain (NC1) of type VII collagen (Col VII).

OBJECTIVE:

Development of reliable assays for the detection of anti-Col VII-NC1 antibodies.

METHODS:

NC1 was expressed in human HEK293 cells and used as target antigen in an enzyme-linked immunosorbent assay (ELISA) and in an immunofluorescence assay (IFA). These two assays were probed in a large cohort of patients with EBA (n = 73), bullous pemphigoid (BP, n = 72), anti-p200 pemphigoid (n = 24), anti-laminin 332 mucous membrane pemphigoid (MMP, n = 15), pemphigus vulgaris (PV, n = 24), and healthy control subjects (n = 254).

RESULTS:

The cut-off for the ELISA was optimized for accuracy by receiver-operating characteristics (area under the curve [AUC] = 0.9952). IgG reactivity against NC1 was detected in 69 of 73 EBA (94.5%) and 5 control sera (2 healthy controls and 3 BP patients), resulting in a specificity of 98.7%. The IFA showed a sensitivity of 91.8% and specificity of 99.8%. Reproducibility of the ELISA was demonstrated by an intra-class correlation coefficient of 0.97. IgG subclass analyses by ELISA revealed IgG1, IgG2, IgG3, and IgG4 anti-NC1 reactivity in 83.6%, 85.3%, 37.7%, and 83.6% of EBA sera, respectively.

LIMITATIONS:

The novel assays were not evaluated prospectively and their use in monitoring serum levels during the disease course was not tested.

CONCLUSION:

The two assays are highly specific and sensitive to diagnose EBA. Their diagnostic competence was demonstrated in a large cohort of well-characterized EBA sera.

Copyright © 2012 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.

PMID:
22341608
[PubMed - indexed for MEDLINE]
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Elsevier Science
    Loading ...
    Write to the Help Desk