Xeroderma Pigmentosum: General Aspects and Management

Xeroderma Pigmentosum (XP) is a rare genetic syndrome with a defective DNA nucleotide excision repair. It is characterized by (i) an extreme sensitivity to ultraviolet (UV)-induced damages in the skin and eyes; (ii) high risk to develop multiple skin tumours; and (iii) neurologic alterations in the...

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Autores principales:	Monica Piccione, Anna Belloni Fortina, Giulia Ferri, Gloria Andolina, Lorenzo Beretta, Andrea Cividini, Emanuele De Marni, Francesca Caroppo, Ugo Citernesi, Rosa Di Liddo
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	xeroderma pigmentosum nucleotide excision repair personalized medicine liposomes Dimericine Medicine R
Acceso en línea:	https://doaj.org/article/7636c1034cd84c288f3151df693a97c6
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spelling	oai:doaj.org-article:7636c1034cd84c288f3151df693a97c62021-11-25T18:07:33ZXeroderma Pigmentosum: General Aspects and Management10.3390/jpm111111462075-4426https://doaj.org/article/7636c1034cd84c288f3151df693a97c62021-11-01T00:00:00Zhttps://www.mdpi.com/2075-4426/11/11/1146https://doaj.org/toc/2075-4426Xeroderma Pigmentosum (XP) is a rare genetic syndrome with a defective DNA nucleotide excision repair. It is characterized by (i) an extreme sensitivity to ultraviolet (UV)-induced damages in the skin and eyes; (ii) high risk to develop multiple skin tumours; and (iii) neurologic alterations in the most severe form. To date, the management of XP patients consists of (i) early diagnosis; (ii) a long-life protection from ultraviolet radiation, including avoidance of unnecessary UV exposure, wearing UV blocking clothing, and use of topical sunscreens; and (iii) surgical resections of skin cancers. No curative treatment is available at present. Thus, in the last decade, in order to prevent or delay the progression of the clinical signs of XP, numerous strategies have been proposed and tested, in some cases, with adverse effects. The present review provides an overview of the molecular mechanisms featuring the development of XP and highlights both advantages and disadvantages of the clinical approaches developed throughout the years. The intention of the authors is to sensitize scientists to the crucial aspects of the pathology that could be differently targeted. In this context, the exploration of the process underlining the conception of liposomal nanocarriers is reported to focus the attention on the potentialities of liposomal technology to optimize the administration of chemoprotective agents in XP patients.Monica PiccioneAnna Belloni FortinaGiulia FerriGloria AndolinaLorenzo BerettaAndrea CividiniEmanuele De MarniFrancesca CaroppoUgo CiternesiRosa Di LiddoMDPI AGarticlexeroderma pigmentosumnucleotide excision repairpersonalized medicineliposomesDimericineMedicineRENJournal of Personalized Medicine, Vol 11, Iss 1146, p 1146 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	xeroderma pigmentosum nucleotide excision repair personalized medicine liposomes Dimericine Medicine R
spellingShingle	xeroderma pigmentosum nucleotide excision repair personalized medicine liposomes Dimericine Medicine R Monica Piccione Anna Belloni Fortina Giulia Ferri Gloria Andolina Lorenzo Beretta Andrea Cividini Emanuele De Marni Francesca Caroppo Ugo Citernesi Rosa Di Liddo Xeroderma Pigmentosum: General Aspects and Management
description	Xeroderma Pigmentosum (XP) is a rare genetic syndrome with a defective DNA nucleotide excision repair. It is characterized by (i) an extreme sensitivity to ultraviolet (UV)-induced damages in the skin and eyes; (ii) high risk to develop multiple skin tumours; and (iii) neurologic alterations in the most severe form. To date, the management of XP patients consists of (i) early diagnosis; (ii) a long-life protection from ultraviolet radiation, including avoidance of unnecessary UV exposure, wearing UV blocking clothing, and use of topical sunscreens; and (iii) surgical resections of skin cancers. No curative treatment is available at present. Thus, in the last decade, in order to prevent or delay the progression of the clinical signs of XP, numerous strategies have been proposed and tested, in some cases, with adverse effects. The present review provides an overview of the molecular mechanisms featuring the development of XP and highlights both advantages and disadvantages of the clinical approaches developed throughout the years. The intention of the authors is to sensitize scientists to the crucial aspects of the pathology that could be differently targeted. In this context, the exploration of the process underlining the conception of liposomal nanocarriers is reported to focus the attention on the potentialities of liposomal technology to optimize the administration of chemoprotective agents in XP patients.
format	article
author	Monica Piccione Anna Belloni Fortina Giulia Ferri Gloria Andolina Lorenzo Beretta Andrea Cividini Emanuele De Marni Francesca Caroppo Ugo Citernesi Rosa Di Liddo
author_facet	Monica Piccione Anna Belloni Fortina Giulia Ferri Gloria Andolina Lorenzo Beretta Andrea Cividini Emanuele De Marni Francesca Caroppo Ugo Citernesi Rosa Di Liddo
author_sort	Monica Piccione
title	Xeroderma Pigmentosum: General Aspects and Management
title_short	Xeroderma Pigmentosum: General Aspects and Management
title_full	Xeroderma Pigmentosum: General Aspects and Management
title_fullStr	Xeroderma Pigmentosum: General Aspects and Management
title_full_unstemmed	Xeroderma Pigmentosum: General Aspects and Management
title_sort	xeroderma pigmentosum: general aspects and management
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/7636c1034cd84c288f3151df693a97c6
work_keys_str_mv	AT monicapiccione xerodermapigmentosumgeneralaspectsandmanagement AT annabellonifortina xerodermapigmentosumgeneralaspectsandmanagement AT giuliaferri xerodermapigmentosumgeneralaspectsandmanagement AT gloriaandolina xerodermapigmentosumgeneralaspectsandmanagement AT lorenzoberetta xerodermapigmentosumgeneralaspectsandmanagement AT andreacividini xerodermapigmentosumgeneralaspectsandmanagement AT emanueledemarni xerodermapigmentosumgeneralaspectsandmanagement AT francescacaroppo xerodermapigmentosumgeneralaspectsandmanagement AT ugociternesi xerodermapigmentosumgeneralaspectsandmanagement AT rosadiliddo xerodermapigmentosumgeneralaspectsandmanagement
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Xeroderma Pigmentosum: General Aspects and Management

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