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Low sensitivity of type VII collagen enzyme-linked immunosorbent assay in epidermolysis bullosa acquisita:serration pattern analysis on skin biopsy is required for diagnosis

By J. B. Terra, M. F. Jonkman, G. F. H. Diercks and H. H. Pas


<p>BackgroundThe type VII collagen (coll VII) enzyme-linked immunosorbent assay (ELISA) has been reported to have high sensitivity (>93%) and specificity (>96%) for diagnosing epidermolysis bullosa acquisita (EBA) in patients who are seropositive on indirect immunofluorescence on salt-split skin (SSS).</p><p>ObjectivesTo investigate the added value of the coll VII ELISA in the laboratory diagnosis of SSS-positive and SSS-negative EBA and to correlate the ELISA index with disease episode.</p><p>MethodsThe coll VII ELISA was performed on banked sera of 28 patients with EBA: 15 SSS positive and 13 SSS negative. Sera from healthy blood donors (n=17) and patients with other autoimmune blistering diseases (n=29) served as controls. In four patients, the ELISA index was measured longitudinally. Serration pattern analysis by direct immunofluorescence has been prospectively performed since 2000 in 19 patients.</p><p>ResultsThe sensitivity in the SSS-positive group was 80% whereas it was 23% in the SSS-negative group. In the prospective EBA subset it was 45%. The sensitivity of u-serration pattern analysis on skin biopsy was 89%. Ten (53%) of these cases were seronegative with both ELISA and SSS, and would have been missed by serum analysis alone. Of the 46 control sera, one serum tested positive (specificity 978%). The coll VII ELISA correlated with disease activity over time in individual patients.</p><p>ConclusionsThe coll VII ELISA has limited added value in SSS-negative EBA cases. The ELISA test is valuable in differentiating EBA from antilaminin-332 mucous membrane pemphigoid and anti-p200 pemphigoid and in its ability to monitor patients with EBA serologically. U-serration pattern analysis on immunofluorescence skin biopsy is the gold standard for the diagnosis of EBA.</p>

Year: 2013
DOI identifier: 10.1111/bjd.12300
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