Epidermolysis bullosa acquisita

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 anchor...

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Bibliographic Details
Published in:Journal of the European Academy of Dermatology and Venereology 2013-10, Vol.27 (10), p.1204-1213
Main Authors: Kim, J.H., Kim, S.-C.
Format: Article
Language:English
Subjects:
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
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Summary: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.
ISSN:0926-9959
1468-3083
DOI:10.1111/jdv.12096