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Description
  • Dystrophic epiclermolysis bullosa (DEB) is a group of hereditary blistering diseases with tissue separation under the basement membrane at the level of anchoring fibrils. DEB pruriginosa subtype (DEB-Pr) is characterized by fragile skin, blisters, pruritic lichcnoid papules on the shins and toenail dystrophy; some cases have a very late onset [1]. All DEB are caused by mutations in the COL7A1 gene encoding the alpha-1 chain of collagen VII of anchoring fibrils. Acquired epidermolysis bullosa (EBA) is an autoimmune bullous disease with formation of autoanti-bodies against collagen VII. We report a dominant DEB-Pr (DDEB-Pr) with features of EBA.
  • Dystrophic epiclermolysis bullosa (DEB) is a group of hereditary blistering diseases with tissue separation under the basement membrane at the level of anchoring fibrils. DEB pruriginosa subtype (DEB-Pr) is characterized by fragile skin, blisters, pruritic lichcnoid papules on the shins and toenail dystrophy; some cases have a very late onset [1]. All DEB are caused by mutations in the COL7A1 gene encoding the alpha-1 chain of collagen VII of anchoring fibrils. Acquired epidermolysis bullosa (EBA) is an autoimmune bullous disease with formation of autoanti-bodies against collagen VII. We report a dominant DEB-Pr (DDEB-Pr) with features of EBA. (en)
Title
  • Dystrophic epidermolysis bullosa pruriginosa with autoantibodies against collagen VII
  • Dystrophic epidermolysis bullosa pruriginosa with autoantibodies against collagen VII (en)
skos:prefLabel
  • Dystrophic epidermolysis bullosa pruriginosa with autoantibodies against collagen VII
  • Dystrophic epidermolysis bullosa pruriginosa with autoantibodies against collagen VII (en)
skos:notation
  • RIV/00159816:_____/12:#0001039!RIV13-MZ0-00159816
http://linked.open...avai/predkladatel
http://linked.open...avai/riv/aktivita
http://linked.open...avai/riv/aktivity
  • I, V
http://linked.open...iv/cisloPeriodika
  • 4
http://linked.open...vai/riv/dodaniDat
http://linked.open...aciTvurceVysledku
http://linked.open.../riv/druhVysledku
http://linked.open...iv/duvernostUdaju
http://linked.open...titaPredkladatele
http://linked.open...dnocenehoVysledku
  • 132605
http://linked.open...ai/riv/idVysledku
  • RIV/00159816:_____/12:#0001039
http://linked.open...riv/jazykVysledku
http://linked.open.../riv/klicovaSlova
  • atrophic skin disease, blister, clinical feature, COL7A1 gene, disease duration, epidermolysis bullosa (en)
http://linked.open.../riv/klicoveSlovo
http://linked.open...odStatuVydavatele
  • FR - Francouzská republika
http://linked.open...ontrolniKodProRIV
  • [BB829E522D80]
http://linked.open...i/riv/nazevZdroje
  • European Journal of Dermatology
http://linked.open...in/vavai/riv/obor
http://linked.open...ichTvurcuVysledku
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http://linked.open...UplatneniVysledku
http://linked.open...v/svazekPeriodika
  • 22
http://linked.open...iv/tvurceVysledku
  • Jedličková, Hana
http://linked.open...ain/vavai/riv/wos
  • 000308371200018
issn
  • 1167-1122
number of pages
http://bibframe.org/vocab/doi
  • 10.1684/ejd.2012.1709
is http://linked.open...avai/riv/vysledek of
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