¹Department of Dermatology and Cutaneous Biology, Jefferson Medical College, and Jefferson Institute of Molecular Medicine, Thomas Jefferson University, Philadelphia, Pennsylvania, USA

Correspondence: Dr Ulrich Rodeck, Jefferson Institute of Molecular Medicine, Thomas Jefferson University, 233 S. 10th Street, Philadelphia, Pennsylvania 19107, USA. E-mail: Ulrich.Rodeck@mail.tju.edu

Abstract

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.