Some epidermolysis bullosa acquisita sera react with epitopes within the triple-helical collagenous domain as indicated by immunoelectron microscopy.

Full Abstract

BACKGROUND: Epidermolysis bullosa acquisita (EBA) autoantibodies recognize epitopes predominantly within the N-terminal noncollagenous (NC)-1 domain of type VII collagen. Recently, some EBA cases with reactivity to other domains, i.e. the triple-helical (T-H) collagenous domain and the NC-2 domain, have been reported. OBJECTIVES: To investigate the ultrastructural localization of epitopes for sera from five patients with EBA that were unreactive by immunoblotting with the NC-1, NC-2 and collagenous domains of type VII collagen. METHODS: Immunogold postembedding indirect immunoelectron microscopy was performed using normal human skin and type VII collagen-deficient skin as substrates. RESULTS: Postembedding indirect immunoelectron microscopy revealed that the five EBA sera showed immunoreactivity in the dermis, mainly located 0-400 nm below the lamina densa. IgG labelling was not observed in type VII collagen-deficient skin from a patient with recessive dystrophic epidermolysis bullosa. The distribution histogram found in this study was different from those of sera that reacted with the NC-1 and/or NC-2 domains, and was similar to those of sera reacting with the T-H collagenous domain. CONCLUSIONS: Our results suggest that epitopes within the T-H collagenous domain of type VII collagen are recognized by IgG antibodies from some EBA sera. These antibodies appear to be found in patients with inflammatory-type EBA.

Author information

Author/s: Ishii, N (N); Yoshida, M (M); Ishida-Yamamoto, A (A); Fritsch, A (A); Elfert, S (S); Bruckner-Tuderman, L (L); Hashimoto, T (T);

Affiliation: Department of Dermatology, Kurume University School of Medicine, 67 Asahimachi, Kurume, Fukuoka 830-0011, Japan. norito(-atsign-)med.kurume-u.ac.jp

Journal and publication information

Publication Type: Journal Article; Research Support, Non-U.S. Gov't

Journal: The British journal of dermatology (Br J Dermatol), published in England. (Language: eng)

Reference: 2009-May; vol 160 (issue 5) : pp 1090-3

Dates: Created 2009/05/07; Completed 2009/07/15;

PMID: 19067699, status: MEDLINE (last retrieval date: 7/24/2009, IMS Date: )

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