European Journal of Dermatology
MENUUpdate on the pathogenesis of squamous cell carcinoma development in recessive dystrophic epidermolysis bullosa Volume 25, supplement 1, April 2015
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3 The University of New South Wales,
Sydney, Australia
Sydney, Australia
* Reprints
- Key words: Collagen VII, high mortality, pathogenesis, RDEB, SCC, tumour microenvironment
- DOI : 10.1684/ejd.2015.2552
- Page(s) : 30-2
- Published in: 2015
Epidermolysis bullosa (EB) is a rare genetic blistering disorder which is characterized by skin fragility and recurrent blistering of the cutaneous and mucous membranes following minimal trauma. Its prevalence is estimated to be around 8.22 per million in the United States [1]. Recessive dystrophic epidermolysis bullosa (RDEB) is the most severe form of EB, caused by mutations in the collagen VII gene COL7A1. This causes alterations in the anchoring fibrils that attach the epidermal basement membrane [...]