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Childhood epidermolysis bullosa acquisita with autoantibodies against the noncollagenous 1 and 2 domains of type VII collagen : case report and review of the literature

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Title: Childhood epidermolysis bullosa acquisita with autoantibodies against the noncollagenous 1 and 2 domains of type VII collagen : case report and review of the literature
Authors: Mayuzumi, M. Browse this author
Akiyama, M. Browse this author
Nishie, W. Browse this author
Ukae, S. Browse this author
Abe, M. Browse this author
Sawamura, D. Browse this author
Hashimoto, T. Browse this author
Shimizu, H. Browse this author →KAKEN DB
Keywords: EBA
epitope
immunoblot
immunofluorescence
inflammatory phenotype
Issue Date: Nov-2006
Publisher: Blackwell Publishing
Journal Title: British Journal of Dermatology
Volume: 155
Issue: 5
Start Page: 1048
End Page: 1052
Publisher DOI: 10.1111/j.1365-2133.2006.07443.x
PMID: 17034540
Abstract: Epidermolysis bullosa acquisita (EBA) is an acquired subepidermal bullous disease characterized by IgG autoantibodies to type VII collagen, a major component of anchoring fibrils. Most patients with EBA are adult and develop autoantibodies to the noncollagenous (NC) 1 domain of type VII collagen. We describe a 4-year-old Japanese boy presenting pruritic vesicles and tense blisters over his whole body. Immunofluorescence studies revealed linear IgG/C3 deposits along the dermal–epidermal junction of the patient's skin, and circulating IgG autoantibodies mapping to the dermal side of 1 M NaCl-split skin. By immunoblotting analysis using dermal extracts as a substrate, the patient's IgG antibodies labelled a 290-kDa protein corresponding to type VII collagen. Immunoblotting studies using recombinant proteins demonstrated that the patient's circulating autoantibodies recognized not only the NC1 but also the NC2 domain of type VII procollagen. Review of the previously reported cases and the present case suggested that patients with EBA with autoantibodies to regions other than the NC1 domain are all children younger than 10 years of age with clinical features of an inflammatory phenotype.
Rights: The definitive version is available at www.blackwell-synergy.com
Type: article (author version)
URI: http://hdl.handle.net/2115/30232
Appears in Collections:北海道大学病院 (Hokkaido University Hospital) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)

Submitter: 秋山 真志

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