Successful Treatment of Intravenous Immunoglobulins in a Patient with Intractable Epidermolysis Bullosa Acquisita with Autoantibodies to Type VII Collagen and Laminin Alpha-3
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چکیده
Copyright: © 2013 Osada SI, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Successful Treatment of Intravenous Immunoglobulins in a Patient with Intractable Epidermolysis Bullosa Acquisita with Autoantibodies to Type VII Collagen and Laminin Alpha-3
منابع مشابه
The epidermolysis bullosa acquisita antigen (type VII collagen) is present in human colon and patients with crohn's disease have autoantibodies to type VII collagen.
Epidermolysis bullosa acquisita is an autoimmune blistering disease of the skin characterized by IgG autoantibodies against type VII collagen. Systemic diseases are often associated with epidermolysis bullosa acquisita, Crohn's disease being the most frequent. This study sought to determine if type VII collagen, the epidermolysis bullosa acquisita autoantigen, was present in normal human colon ...
متن کاملEpidermolysis bullosa acquisita antigen is the globular carboxyl terminus of type VII procollagen.
Epidermolysis bullosa acquisita (EBA) is a severe, chronic blistering disease of the skin. EBA patients have circulating and tissue-bound autoantibodies to a large (Mr = 290,000) macromolecule that is localized within the basement membrane zone between the epidermis and dermis of skin, the site of blister formation. The "EBA antigen" is known to be distinct from laminin, heparan sulfate proteog...
متن کاملA novel deletion and two recurrent substitutions on type VII collagen gene in seven Iranian patients with epidermolysis bullosa
Objective(s): Epidermolysis bullosa is one of the most important series of mechano-bullous heritable skin disorders which is categorized into four major types according to the layer that bullae forms within basement membrane zone. In dystrophic form of the disease, blisters are made in the sublamina densa zone, at the level of type VII collagen protein which produce anchoring fibrils. Type VII ...
متن کاملT cells are required for the production of blister-inducing autoantibodies in experimental epidermolysis bullosa acquisita.
Epidermolysis bullosa acquisita is a prototypical organ-specific autoimmune disease caused by autoantibodies against type VII collagen of the dermal-epidermal junction. Although mechanisms of autoantibody-induced blister formation were extensively characterized, the initiation of autoantibody production in autoimmune blistering diseases is still poorly defined. In the current study, we addresse...
متن کاملChildhood epidermolysis bullosa acquisita with autoantibodies against the non-collagenous 1 and 2 domains of type VII collagen: a case report and a review of the literature
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 Author(s) Mayuzumi, M.; Akiyama, M.; Nishie, W.; Ukae, S.; Abe, M.; Sawamura, D.; Hashimoto, T.; Shimizu, H. Citation British Journal of Dermatology, 155(5): 1048-1052 Issue Date 2006-11 Doc URL http://hdl.handle.net/2115/...
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