Recessive dystrophic epidermolysis bullosa-associated squamous-cell carcinoma: An enigmatic entity with complex pathogenesis

[摘要] Expression of either collagen VII or the noncollagenous ( NC1) fragment derived from it has been suggested to be indispensable for the development of squamous-cell carcinomas ( SCCs) in patients affected by recessive dystrophic epidermolysis bullosa ( RDEB). This view is challenged here by the observation that SCCs do develop in RDEB patients lacking expression of collagen VII altogether. The aggressive behavior of RDEB- associated SCCs remains unexplained.

[发布日期] 2007-10-01