Therapeutics of xeroderma pigmentosum: A PRISMA-compliant systematic review

نویسندگان

چکیده

Xeroderma pigmentosum is a rare hereditary autosomal recessive genodermatosis. At present, there are many treatment options for xeroderma pigmentosum, covering medical/procedural, surgical and combined modalities. However, the quality of these interventions has not been assessed. Our study aimed to perform systematic review literature regarding pigmentosum. Multiple medical databases were accessed with Medical Subject Headings terms; “xeroderma pigmentosum,” “therapeutics” “surgical procedures, operative” from January 2000 April 2019, including articles published in Portuguese, Spanish English (PROSPERO-CRD42018114858). Two hundred ninety-eight studies found researched, which, after applying inclusion criteria, only 33 remained. The complete read by three authors, having found: 16 reported medical/procedural 17 treatments. Only one clinical presented good level evidence (EL: 2): randomized trial using T4 endonuclease V (T4N5) liposome lotion which reduced development skin lesions patients Amongst modalities, all low 4). Three illustrative cases also presented, emphasize multiple number times that modalities may be required patients. therapeutic both surgical, scientific did allow meta-analysis. More studies, better needed.

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Xeroderma pigmentosum: a review and case series.

Xeroderma pigmentosa (XP) is a condition inherited as an autosomal recessive trait and is characterized by photosensitivity, pigmentary changes, premature skin ageing and malignant tumour development resulting from the defect in DNA repair. The management of complications of XP, especially orofacial tumours entails an enormous surgical challenge to the clinicians. We present five cases of XP.

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Xeroderma pigmentosum (XP) is defined by extreme sensitivity to sunlight, resulting in sunburn, pigment changes in the skin and a greatly elevated incidence of skin cancers. It is a rare autosomal recessive disorder and has been found in all continents and racial groups. Estimated incidences vary from 1 in 20, 000 in Japan to 1 in 250, 000 in the USA, and approximately 2.3 per million live birt...

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ژورنال

عنوان ژورنال: Indian Journal of Dermatology, Venereology and Leprology

سال: 2021

ISSN: ['0378-6323', '0973-3922']

DOI: https://doi.org/10.25259/ijdvl_431_19