Childhood 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

نویسندگان

  • M. MAYUZUMI
  • M. AKIYAMA
  • W. NISHIE
  • S. UKAE
  • M. ABE
  • D. SAWAMURA
  • T. HASHIMOTO
  • H. SHIMIZU
  • Masashi Akiyama
چکیده

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/2115/30232 Right The definitive version is available at www.blackwell-synergy.com

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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.

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 whol...

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Blister-inducing antibodies target multiple epitopes on collagen VII in mice

Epidermolysis bullosa acquisita (EBA) is an autoimmune subepidermal blistering disease of mucous membranes and the skin caused by autoantibodies against collagen VII. In silico and wet laboratory epitope mapping studies revealed numerous distinct epitopes recognized by EBA patients' autoantibodies within the non-collagenous (NC)1 and NC2 domains of collagen VII. However, the distribution of pat...

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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 ...

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T 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...

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A 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 ...

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تاریخ انتشار 2017