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巴泽克斯综合征(副肿瘤性肢端角化病):发病机制、临床表现及治疗方法的叙述性综述

Bazex Syndrome (Acrokeratosis Paraneoplastica): A Narrative Review of Pathogenesis, Clinical Manifestations, and Therapeutic Approaches.

作者信息

Shah Muhammad Hamza, Ferrazzano Carlo, Karthikeyan Anhukrisha, Hejazi Hamad, Bhattacharya Anushri, Andrew Awuah Wireko, Isik Arda

机构信息

Centre for Anatomy, Deanery of Biomedical Sciences, The University of Edinburgh, Edinburgh, GBR.

School of Medicine, Dentistry and Biomedical Sciences, Queen's University Belfast, Belfast, GBR.

出版信息

Cureus. 2023 Sep 16;15(9):e45368. doi: 10.7759/cureus.45368. eCollection 2023 Sep.

Abstract

Acrokeratosis paraneoplastica, also known as Bazex syndrome, is a relatively understudied yet clinically important dermatological condition that is inextricably associated with squamous cell carcinoma, predominantly of the upper respiratory and gastrointestinal tracts. Manifesting as unique psoriasis-like cutaneous lesions, Bazex syndrome functions as an early warning signal for underlying malignancies, underscoring the urgent need for its timely diagnosis and intervention. Notwithstanding its clinical relevance, the molecular and cellular mechanisms underpinning its pathogenesis are not fully understood. To address these gaps, this comprehensive literature review undertook a meticulous search across reputable databases such as EMBASE, MEDLINE/PubMed, and Web of Science. Our analysis ventures into multiple putative pathogenic pathways, including shifts in Th2 immune responses, aberrant secretion of growth factors, and immunological reactions to tumor-specific antigens. We also detail the clinical phenotypes of Bazex syndrome and their chronological linkage with the corresponding malignancies. Finally, the review evaluates the therapeutic effectiveness of various approaches, including but not limited to targeted cancer treatments, PUVA therapy, and topical corticosteroids. This synthesis aims to arm healthcare providers with a nuanced understanding of Bazex syndrome, facilitating more accurate diagnosis and tailored treatment paradigms.

摘要

副肿瘤性肢端角化病,又称巴泽克斯综合征,是一种研究相对较少但临床上很重要的皮肤病,与鳞状细胞癌密切相关,主要发生在上呼吸道和胃肠道。巴泽克斯综合征表现为独特的银屑病样皮肤病变,是潜在恶性肿瘤的早期预警信号,凸显了及时诊断和干预的迫切需求。尽管其具有临床相关性,但其发病机制的分子和细胞机制尚未完全明确。为了填补这些空白,本全面的文献综述在EMBASE、MEDLINE/PubMed和Web of Science等知名数据库中进行了细致的检索。我们的分析深入探讨了多种假定的致病途径,包括Th2免疫反应的变化、生长因子的异常分泌以及对肿瘤特异性抗原的免疫反应。我们还详细阐述了巴泽克斯综合征的临床表型及其与相应恶性肿瘤的时间关联。最后,本综述评估了包括但不限于靶向癌症治疗、补骨脂素紫外线A光化学疗法(PUVA疗法)和外用糖皮质激素等各种治疗方法的有效性。本综述旨在让医疗服务提供者对巴泽克斯综合征有更细致入微的了解,促进更准确的诊断和量身定制的治疗方案。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8d49/10505057/7e14bb376a22/cureus-0015-00000045368-i01.jpg

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