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Epidermolysis bullosa acquisita associated with epidermal-binding circulating antibodies

Lookup NU author(s): Professor Peter Farr

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Abstract

Epidermolysis bullosa acquisita (EBA) is a rare, immunobullous disease, characterized by circulating and tissue-bound antibodies against type VII collagen (C7) of anchoring fibrils in the cutaneous basement membrane zone, These antibodies localize to the dermal aspect of salt-split skin on indirect and direct immunofluorescence (IMF), We report two patients with clinical features of EBA, in whom circulating IgG antibodies bound to the epidermal aspect of salt-split skin, In both patients direct IMF of salt-split perilesional skin revealed dermal IgG deposits, and direct immunogold immunoelectron microscopy showed antibody deposits in the region of anchoring fibrils, Their serum failed to react with epidermal or dermal extracts on Western immunoblotting. Epidermal-binding antibodies have not been reported previously in association with EBA, and the IMF findings in these cases suggest the development of autoantibodies to additional epidermal-associated antigens, Target antigen heterogeneity has been reported in most other immunobullous diseases, and may be a hitherto unrecognized feature of EBA.

Publication metadata

Author(s): Wakelin SH, Bhogal B, Black MM, Allen JM, Wojnarowska F, Hashimoto T, Farr PM, Swain AF

Publication type: Article

Publication status: Published

Journal: British Journal of Dermatology

Year: 1997

Volume: 136

Issue: 4

Pages: 604-609

ISSN (print): 0007-0963

ISSN (electronic): 1365-2133

URL: http://dx.doi.org/10.1046/j.1365-2133.1997.d01-1245.x

DOI: 10.1046/j.1365-2133.1997.d01-1245.x

PubMed id: 9155969

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