Detection of anti-type VII collagen IgE antibodies in epidermolysis bullosa acquisita.

Abstract

Epidermolysis bullosa acquisita (EBA) is a rare pemphigoid disease involving autoantibodies to type VII collagen (COL7), a major structural component of anchoring fibrils. IgE autoantibodies to type XVII collagen (BP180) have been identified in bullous pemphigoid (BP), the prototype of pemphigoid diseases. Although the pathogenic relevance of IgG anti-COL7 has been investigated, that of IgE in EBA remains unclear. To reveal the presence and pathogenic relevance of IgE anti-COL7 in EBA. We examined IgE antibodies in 109 patients with EBA by indirect immunofluorescence (IIF) and enzyme-linked immunosorbent assay (ELISA). IIF with normal human skin revealed IgE reactivity in the basement membrane zone in 29 (26·6%) cases. To verify whether the IgE antibodies were specific to COL7, we performed IIF with 21 clearly positive cases and the skin of a patient with dystrophic EBA, which does not involve COL7. All cases showed negative results, indicating that IgE antibodies were specific to COL7. In a modified IgG COL7 ELISA for IgE, 16 (14·7%) cases were positive (three and 13 cases were negative and positive on IIF, respectively). We compared anti-COL7 IgG and IgE, and found a weak but significant correlation (r = 0·459, P < 0·001). EBA is clinically divided into a mechanobullous (MB; noninflammatory) type and an inflammatory (INF) type resembling BP. Of the IIF-positive cases, 11 of 30 (37%) had INF and nine of 48 (19%) had MB. This study is the first to demonstrate the presence of circulating anti-COL7 IgE in patients with EBA, which may correlate with the clinical phenotype.