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Review

Epidermolysis Bullosa-Associated Squamous Cell Carcinoma: From Pathogenesis to Therapeutic Perspectives

1
Genetics and Rare Diseases Research Division, Bambino Gesù Children’s Hospital, IRCCS, viale di San Paolo 15, 00146 Rome, Italy
2
Laboratory of Molecular and Cell Biology, IDI-IRCCS, via Monti di Creta 104, 00167 Rome, Italy
*
Author to whom correspondence should be addressed.
Int. J. Mol. Sci. 2019, 20(22), 5707; https://doi.org/10.3390/ijms20225707
Received: 12 October 2019 / Revised: 9 November 2019 / Accepted: 11 November 2019 / Published: 14 November 2019
(This article belongs to the Special Issue Skin Cancer: From Pathophysiology to Novel Therapeutic Approaches)
Epidermolysis bullosa (EB) is a heterogeneous group of inherited skin disorders determined by mutations in genes encoding for structural components of the cutaneous basement membrane zone. Disease hallmarks are skin fragility and unremitting blistering. The most disabling EB (sub)types show defective wound healing, fibrosis and inflammation at lesional skin. These features expose patients to serious disease complications, including the development of cutaneous squamous cell carcinomas (SCCs). Almost all subjects affected with the severe recessive dystrophic EB (RDEB) subtype suffer from early and extremely aggressive SCCs (RDEB-SCC), which represent the first cause of death in these patients. The genetic determinants of RDEB-SCC do not exhaustively explain its unique behavior as compared to low-risk, ultraviolet-induced SCCs in the general population. On the other hand, a growing body of evidence points to the key role of tumor microenvironment in initiation, progression and spreading of RDEB-SCC, as well as of other, less-investigated, EB-related SCCs (EB-SCCs). Here, we discuss the recent advances in understanding the complex series of molecular events (i.e., fibrotic, inflammatory, and immune processes) contributing to SCC development in EB patients, cross-compare tumor features in the different EB subtypes and report the most promising therapeutic approaches to counteract or delay EB-SCCs. View Full-Text
Keywords: cancer; wound-healing; basement membrane zone; extracellular matrix; fibrosis; inflammation; immunity; collagen VII; laminin-332; kindlin-1 cancer; wound-healing; basement membrane zone; extracellular matrix; fibrosis; inflammation; immunity; collagen VII; laminin-332; kindlin-1
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MDPI and ACS Style

Condorelli, A.G.; Dellambra, E.; Logli, E.; Zambruno, G.; Castiglia, D. Epidermolysis Bullosa-Associated Squamous Cell Carcinoma: From Pathogenesis to Therapeutic Perspectives. Int. J. Mol. Sci. 2019, 20, 5707. https://doi.org/10.3390/ijms20225707

AMA Style

Condorelli AG, Dellambra E, Logli E, Zambruno G, Castiglia D. Epidermolysis Bullosa-Associated Squamous Cell Carcinoma: From Pathogenesis to Therapeutic Perspectives. International Journal of Molecular Sciences. 2019; 20(22):5707. https://doi.org/10.3390/ijms20225707

Chicago/Turabian Style

Condorelli, Angelo G.; Dellambra, Elena; Logli, Elena; Zambruno, Giovanna; Castiglia, Daniele. 2019. "Epidermolysis Bullosa-Associated Squamous Cell Carcinoma: From Pathogenesis to Therapeutic Perspectives" Int. J. Mol. Sci. 20, no. 22: 5707. https://doi.org/10.3390/ijms20225707

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