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Epidermolysis bullosa acquisita

Authors
 J.H. Kim  ;  S.-C. Kim 
Citation
 Journal of the European Academy of Dermatology and Venereology, Vol.27(10) : 1204-1213, 2013 
Journal Title
 Journal of the European Academy of Dermatology and Venereology 
ISSN
 0926-9959 
Issue Date
2013
MeSH
Adrenal Cortex Hormones/therapeutic use ; Animals ; Antibodies, Monoclonal, Murine-Derived/therapeutic use ; Autoantibodies/metabolism ; Autoimmune Diseases/diagnosis* ; Autoimmune Diseases/drug therapy* ; Autoimmune Diseases/etiology ; Colchicine/therapeutic use ; Collagen Type VII/immunology ; Dapsone/therapeutic use ; Disease Models, Animal ; Epidermolysis Bullosa Acquisita/diagnosis* ; Epidermolysis Bullosa Acquisita/drug therapy* ; Epidermolysis Bullosa Acquisita/etiology ; Humans ; Immunoglobulins, Intravenous/therapeutic use ; Mice ; Rituximab
Keywords
Adrenal Cortex Hormones/therapeutic use ; Animals ; Antibodies, Monoclonal, Murine-Derived/therapeutic use ; Autoantibodies/metabolism ; Autoimmune Diseases/diagnosis* ; Autoimmune Diseases/drug therapy* ; Autoimmune Diseases/etiology ; Colchicine/therapeutic use ; Collagen Type VII/immunology ; Dapsone/therapeutic use ; Disease Models, Animal ; Epidermolysis Bullosa Acquisita/diagnosis* ; Epidermolysis Bullosa Acquisita/drug therapy* ; Epidermolysis Bullosa Acquisita/etiology ; Humans ; Immunoglobulins, Intravenous/therapeutic use ; Mice ; Rituximab
Abstract
Epidermolysis bullosa acquisita (EBA) is a chronic autoimmune subepidermal bullous disease with clinical features similar to the genetic form of dystrophic epidermolysis bullosa. EBA is characterized by the presence of autoantibodies against type VII collagen which is a major component of the anchoring fibrils at the dermal-epidermal junction. EBA can be divided into two main clinical types; mechanobullous and inflammatory EBA. Mechanobullous EBA, referred to as classic EBA, presents with skin fragility, blisters and dystrophic changes on trauma-prone areas. Inflammatory EBA resembles other autoimmune subepidermal bullous diseases. Compelling evidence from mouse models supports a pathogenic role of autoantibodies against type VII collagen in EBA. Treatment of EBA is often unsatisfactory. The most widely used systemic treatment is corticosteroids. Colchicine and dapsone have been reported to be good treatment modalities when combined with corticosteroids. Some intractable cases of EBA have successfully been treated with intravenous immunoglobulin or rituximab.
Full Text
http://onlinelibrary.wiley.com/doi/10.1111/jdv.12096/abstract
DOI
10.1111/jdv.12096
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Dermatology (피부과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Soo Chan(김수찬) ORCID logo https://orcid.org/0000-0002-2327-4755
Kim, Jong Hoon(김종훈) ORCID logo https://orcid.org/0000-0002-3385-8180
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/89090
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