Article
Patients with recessive dystrophic epidermolysis bullosa develop squamous-cell carcinoma regardless of type VII collagen expression.
The Journal of investigative dermatology - 1 Oct 2007
Pourreyron Celine, Cox Georgie, Mao Xin, Volz Andreas, Baksh Nuzhat, Wong Tracy, Fassihi Hiva, Arita Ken, O'Toole Edel A, Ocampo-Candiani Jorge, Chen Mei, Hart Ian R, Bruckner-Tuderman Leena, Salas-Alanis Julio C, McGrath John A, Leigh Irene M, South Andrew P
Abstract excerpt
Recent data suggest that individuals with recessive dystrophic epidermolysis bullosa (RDEB) only develop squamous-cell carcinoma (SCC) in the presence of the NC1 domain of type VII collagen. This conclusion was based on experimental work in which cryosections of SCCs from 10 people with RDEB all showed positive type VII collagen immunostaining and observations in a murine model of SCC development in which tumors...
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