隐性营养不良性大疱性表皮松解症相关鳞状细胞癌：一种发病机制复杂的神秘疾病。

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

作者信息

Rodeck Ulrich, Uitto Jouni

机构信息

Department of Dermatology and Cutaneous Biology, Jefferson Medical College, and Jefferson Institute of Molecular Medicine, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA.

出版信息

J Invest Dermatol. 2007 Oct;127(10):2295-6. doi: 10.1038/sj.jid.5700939.

DOI:10.1038/sj.jid.5700939

PMID:17853916

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.

摘要

对于患有隐性营养不良性大疱性表皮松解症（RDEB）的患者，有人提出胶原蛋白VII或其衍生的非胶原蛋白（NC1）片段的表达对于鳞状细胞癌（SCC）的发展必不可少。然而，此处观察到完全缺乏胶原蛋白VII表达的RDEB患者确实会发生SCC，这一观点受到了挑战。RDEB相关SCC的侵袭性仍无法解释。