Division of Plastic Surgery, Federal University of Alagoas, Maceió, Brazil.
Division of Plastic Surgery, Federal University of São Paulo, São Paulo, SP, Brazil.
Indian J Dermatol Venereol Leprol. 2021 Mar-Apr;87(2):176-189. doi: 10.25259/IJDVL_431_19.
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 a systematic review of the literature regarding the treatment of xeroderma pigmentosum. Multiple medical databases were accessed with the Medical Subject Headings terms; "xeroderma pigmentosum," "therapeutics" and "surgical procedures, operative" from January 2000 to April 2019, including articles published in Portuguese, Spanish and English (PROSPERO-CRD42018114858). Two hundred and ninety-eight studies were found in the databases researched, of which, after applying the inclusion criteria, only 33 studies remained. The 33 complete articles were read by three of the authors, having been found: 16 reported medical/procedural and 17 reported surgical treatments. Only one clinical study presented a good level of evidence (EL: 2): a randomized clinical trial using a T4 endonuclease V (T4N5) liposome lotion which reduced the development of skin lesions in patients with xeroderma pigmentosum. Amongst surgical modalities, all studies presented low evidence level (EL: 4). Three illustrative cases are also presented, to emphasize the multiple number of times that surgical modalities may be required in these patients. The therapeutic modalities, both clinical and surgical, for xeroderma pigmentosum presented a low level of scientific evidence which did not allow meta-analysis. More therapeutic studies, both clinical and surgical, with better scientific evidence are needed.
着色性干皮病是一种罕见的遗传性常染色体隐性遗传皮肤病。目前，有许多治疗着色性干皮病的方法，包括医疗/程序、手术和联合治疗。然而，这些干预措施的质量尚未得到评估。我们的研究旨在对有关着色性干皮病治疗的文献进行系统评价。使用医学主题词（“着色性干皮病”、“治疗”和“手术程序、手术”）从 2000 年 1 月至 2019 年 4 月，在多个医学数据库中进行了检索，包括发表在葡萄牙语、西班牙语和英语中的文章（PROSPERO-CRD42018114858）。在研究的数据库中发现了 298 项研究，其中，在应用纳入标准后，只有 33 项研究仍然存在。这 33 篇完整的文章由三位作者阅读，发现：16 篇报告了医疗/程序治疗，17 篇报告了手术治疗。只有一项临床研究具有良好的证据水平（EL：2）：使用 T4 内切核酸酶 V（T4N5）脂质体洗剂的随机临床试验减少了着色性干皮病患者皮肤病变的发展。在手术方式中，所有研究的证据水平均较低（EL：4）。还介绍了三个说明性病例，以强调这些患者可能需要多次手术方式。着色性干皮病的治疗方法，包括临床和手术治疗，科学证据水平较低，不允许进行荟萃分析。需要更多具有更好科学证据的临床和手术治疗研究。
