UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

1. 水疱性類天疱瘡および粘膜類天疱瘡の疫学および病因 epidemiology and pathogenesis of bullous pemphigoid and mucous membrane pemphigoid
2. 表皮水疱症の疫学、病因、分類、および臨床的特徴 epidemiology pathogenesis classification and clinical features of epidermolysis bullosa

English Journal

Anti-collagen XVII single-chain Fv antibody blocks the autoimmune reaction mediated by pathogenic autoantibodies in bullous pemphigoid.

Wu Y, Sun NN, Dang EL, Jin L, Liu ZF, Zhang W, Yang LT, Wang G.SourceDepartment of Dermatology, Xijing Hospital, Fourth Military Medical University, 127 Changlexi Road, Xi'an 710032, China.
Journal of dermatological science.J Dermatol Sci.2013 Oct;72(1):25-31. doi: 10.1016/j.jdermsci.2013.05.010. Epub 2013 Jun 13.
BACKGROUND: Pathogenic autoantibodies in bullous pemphigoid (BP) recognize the non-collagenous 16A domain (NC16A) of collagen XVII (COL17), a hemidesmosomal component at the skin membrane. This immune inflammation involves activation of the complement cascade via the classical pathway. With similar
PMID 23827201

Michael M, Begum R, Fong K, Pourreyrone C, South AP, McGrath JA, Parsons M.Source1] Randall Division of Cell and Molecular Biophysics, King's College London, Guy's Campus, London, UK [2] St Johns Institute of Dermatology, King's College London, Guys Campus, London, UK [3] Division of Molecular Cell Biology, Institute for Molecular Bioscience, The University of Queensland, St Lucia, Brisbane, Queensland, Australia.
The Journal of investigative dermatology.J Invest Dermatol.2013 Sep 11. doi: 10.1038/jid.2013.382. [Epub ahead of print]
Bullous pemphigoid antigen 1 (BPAG1-e, also known as BP230) is a member of the plakin family of hemidesmosome cytoskeletal linker proteins that is encoded by an isoform of the dystonin (DST) gene. Recently, we reported two unrelated families with homozygous nonsense mutations in this DST isoform tha
PMID 24025550

Phenotypic spectrum of epidermolysis bullosa associated with α6β4 integrin mutations.

Schumann H, Kiritsi D, Pigors M, Hausser I, Kohlhase J, Peters J, Ott H, Hyla-Klekot L, Gacka E, Sieron AL, Valari M, Bruckner-Tuderman L, Has C.SourceDepartment of Dermatology, University Medical Center Freiburg, Hauptstr 7, 79104 Freiburg, Germany.
The British journal of dermatology.Br J Dermatol.2013 Jul;169(1):115-24. doi: 10.1111/bjd.12317.
BACKGROUND: Integrin α6β4 is a transmembrane receptor and a key component of the hemidesmosome anchoring complex. It is involved in cell-matrix adhesion and signalling in various tissues. Mutations in the ITGA6 and ITGB4 genes coding for α6β4 integrin compromise dermal-epidermal adhesion and are
PMID 23496044