Fibrosis as a Risk Factor for Cutaneous Squamous Cell Carcinoma in Recessive Dystrophic Epidermolysis Bullosa: A Systematic Review.

IF 0.4 Q4 PEDIATRICS Journal of pediatric genetics Pub Date : 2023-02-24 eCollection Date: 2023-06-01 DOI:10.1055/s-0043-1763257

Brenda Lamônica Rodrigues de Azevedo, Gabriel Marim Roni, Rosalie Matuk Fuentes Torrelio, Letícia Nogueira da Gama-de-Souza

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Abstract

Recessive dystrophic epidermolysis bullosa (RDEB) is a severe subtype of epidermolysis bullosa caused by changes in collagen VII with a high risk of early development of cutaneous squamous cell carcinoma (cSCC). This review aimed to discuss the relationship between the recurrent healing process, the appearance of fibrosis, and malignant epithelial transformation in RDEB. We searched PubMed, the Regional Portal of the Virtual Health Library, and Embase for articles on the relationship between blistering, recurrent scarring, and fibrosis in the context of cSCC and RDEB. That alterations of collagen VII result in blister formation, scar deficiency associated with inflammation, and increased expression of transforming growth factor β. These events promote the differentiation of myofibroblasts and the expression of profibrotic proteins, leading to structural changes and the establishment of a microenvironment favorable to carcinogenesis. Patients with RDEB and areas of recurrent scarring and fibrosis may be more prone to the development of cSCC.

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纤维化是隐性萎缩性表皮松解症皮肤鳞状细胞癌的危险因素：系统综述。

隐性萎缩性大疱性表皮松解症（RDEB）是大疱性表皮松解症的一种严重亚型，由胶原蛋白Ⅶ的变化引起，早期发展为皮肤鳞状细胞癌（cSCC）的风险很高。本综述旨在讨论 RDEB 的复发性愈合过程、纤维化的出现和恶性上皮转化之间的关系。我们在 PubMed、虚拟健康图书馆区域门户网站和 Embase 中检索了有关 cSCC 和 RDEB 中水疱、复发性瘢痕和纤维化之间关系的文章。胶原蛋白 VII 的改变会导致水疱形成、与炎症相关的瘢痕缺失以及转化生长因子 β 的表达增加。这些事件会促进肌成纤维细胞的分化和促纤维化蛋白的表达，从而导致结构改变并建立起有利于癌变的微环境。患有 RDEB 和复发性瘢痕和纤维化区域的患者可能更容易发展为 cSCC。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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Journal of pediatric genetics PEDIATRICS-

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期刊介绍： The Journal of Pediatric Genetics is an English multidisciplinary peer-reviewed international journal publishing articles on all aspects of genetics in childhood and of the genetics of experimental models. These topics include clinical genetics, molecular genetics, biochemical genetics, medical genetics, dysmorphology, teratology, genetic counselling, genetic engineering, formal genetics, neuropsychiatric genetics, behavioral genetics, community genetics, cytogenetics, hereditary or syndromic cancer genetics, genetic mapping, reproductive genetics, fetal pathology and prenatal diagnosis, multiple congenital anomaly syndromes, and molecular embryology of birth defects. Journal of Pediatric Genetics provides an in-depth update on new subjects and current comprehensive coverage of the latest techniques used in the diagnosis of childhood genetics. Journal of Pediatric Genetics encourages submissions from all authors throughout the world. The following articles will be considered for publication: editorials, original and review articles, short report, rapid communications, case reports, letters to the editor, and book reviews. The aim of the journal is to share and disseminate knowledge between all disciplines in the field of pediatric genetics. This journal is a publication of the World Pediatric Society: http://www.worldpediatricsociety.org/ The Journal of Pediatric Genetics is available in print and online. Articles published ahead of print are available via the eFirst service on the Thieme E-Journals platform.