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Recent progress of Stevens-Johnson syndrome/toxic epidermal necrolysis: Diagnosis criteria, pathogenesis and therapy. 史蒂文斯-约翰逊综合征/中毒性表皮坏死症的最新进展:诊断标准、发病机制和治疗。
IF 11 1区 医学 Q1 DERMATOLOGY Pub Date : 2024-08-14 DOI: 10.1093/bjd/ljae321
Natsumi Hama, Shigeki Aoki, Chun-Bing Chen, Akito Hasegawa, Youichi Ogawa, Marc Vocanson, Hideo Asada, Chia-Yu Chu, Cheng-Che E Lan, Roni P Dodiuk-Gad, Toshiharu Fujiyama, Tyng-Shiuan Hsieh, Kousei Ito, Elina Jerschow, Yoshiko Mizukawa, Saeko Nakajima, Kazutoshi Nakamura, Jean-François Nicolas, Takashi K Satoh, Tatsuo Shiohara, Hayato Takahashi, Mikiko Tohyama, Takahiro Ueda, Katsuaki Ura, Hideaki Watanabe, Yukie Yamaguchi, Thierry M Nordmann, Wen-Hung Chung, Dean Naisbitt, Carlo Pincelli, Werner J Pichler, Lars E French, Elizabeth Phillips, Riichiro Abe

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) represent a severe spectrum of rare mucocutaneous reactions, primarily drug-induced and characterized by significant morbidity and mortality. These conditions manifest through extensive skin detachment, distinguishing them from other generalized skin eruptions. The rarity and severity of SJS/TEN underscore the importance of accurate diagnostic criteria and effective treatments, which are currently lacking consensus. This review proposes new diagnostic criteria to improve specificity and global applicability. Recent advancements in understanding the immunopathogenesis of SJS/TEN are explored, emphasizing the role of drug-specific T cell responses and HLA polymorphisms in disease onset. The review also addresses current therapeutic approaches, including controversies surrounding the use of immunosuppressive agents and the emerging role of TNF-α inhibitors. Novel therapeutic strategies targeting specific pathogenic mechanisms, such as necroptosis and specific immune cell pathways, are discussed. Furthermore, the development of new drugs based on these insights, including targeted monoclonal antibodies and inhibitors, are examined. The review concludes by advocating for more robust and coordinated efforts across multidisciplinary medical fields to develop effective treatments and diagnostic tools for SJS/TEN, with the aim of improving patient outcomes and understanding of the disease and its mechanisms.

史蒂文斯-约翰逊综合征(SJS)和中毒性表皮坏死溶解症(TEN)是一种严重的罕见皮肤粘膜反应,主要由药物引起,发病率和死亡率都很高。这些病症表现为大面积皮肤脱落,有别于其他全身性皮肤糜烂。SJS/TEN 的罕见性和严重性凸显了准确诊断标准和有效治疗的重要性,而目前对此还缺乏共识。本综述提出了新的诊断标准,以提高特异性和全球适用性。本综述探讨了在了解 SJS/TEN 免疫发病机制方面的最新进展,强调了药物特异性 T 细胞反应和 HLA 多态性在发病中的作用。综述还探讨了当前的治疗方法,包括围绕使用免疫抑制剂的争议和 TNF-α 抑制剂的新作用。还讨论了针对特定致病机制(如坏死和特定免疫细胞通路)的新型治疗策略。此外,还探讨了基于这些见解的新药开发,包括靶向单克隆抗体和抑制剂。综述最后提倡在多学科医学领域开展更有力、更协调的工作,开发针对 SJS/TEN 的有效治疗方法和诊断工具,以改善患者的治疗效果,加深对该疾病及其机制的了解。
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引用次数: 0
Global, regional and national epidemiology of alopecia areata: a systematic review and modelling study. 脱发症的全球、地区和国家流行病学:系统回顾与模型研究》。
IF 11 1区 医学 Q1 DERMATOLOGY Pub Date : 2024-08-14 DOI: 10.1093/bjd/ljae058
Jae Joon Jeon, Seung-Won Jung, You Hyun Kim, Rosa Parisi, Ju Yeong Lee, Myung Ha Kim, Won-Soo Lee, Solam Lee

Background: Alopecia areata (AA) is a chronic autoimmune disease that leads to a high psychiatric, economic and systemic disease burden. A comprehensive understanding of AA epidemiology is essential for evaluating healthcare source utilization; however, a systematic approach to summarizing epidemiological data on AA is lacking.

Objectives: To investigate systematically the global, regional and national incidence and prevalence of AA.

Methods: A structured search was conducted using the databases MEDLINE, Embase, Cochrane Library, Web of Science, SciELO and Korean Journal Database from their date of inception to 4 October 2023. Studies that reported the prevalence or incidence of AA were included. We used a Bayesian hierarchical linear mixed model to analyse prevalence estimates. The primary outcomes of our study were the global, regional and national prevalence of physician-diagnosed AA for the overall population, for adults and for children. The incidence data were summarized descriptively.

Results: In total, 88 studies from 28 countries were included in the analysis. The reported incidence of AA tended to be higher in adults aged 19-50 years, and this trend was consistent with its estimated prevalence. The reported prevalence in overall populations tended to be higher in men vs. women. The estimated lifetime prevalence rate of AA was 0.10% [95% credible interval (CrI) 0.03-0.39] in the general population worldwide, 0.12% (95% CrI 0.02-0.52) in adults and 0.03% (95% CrI 0.01-0.12) in children. The estimated prevalence of AA was highest in the Asian region and lowest in the African region.

Conclusions: In this study, 48% of the Global Burden of Disease regions had insufficient data on the prevalence or incidence of AA. Further studies are needed to provide epidemiological information on middle- and low-income countries. Our study may serve as a crucial reference in terms of healthcare policy decisions.

背景:斑秃(AA)是一种慢性自身免疫性疾病,对精神、经济和全身性疾病造成沉重负担。全面了解 AA 的流行病学对评估医疗资源的利用情况至关重要;然而,目前还缺乏系统的方法来总结 AA 的流行病学数据:系统调查全球、地区和国家 AA 发病率和流行率:方法:使用 Ovid MEDLINE、EMBASE、Cochrane Library、Web of Science、SciELO 和韩国期刊数据库进行结构化检索。我们纳入了报告 AA 患病率或发病率的研究。我们采用贝叶斯分层线性混合模型来分析患病率估计值。我们研究的主要结果是医生诊断的 AA 在全球、地区和国家范围内的总体患病率、成人患病率和儿童患病率。我们还对发病率数据进行了描述性总结:共有来自 28 个国家的 88 项研究被纳入分析。据报告,19-50 岁成年人的脱发发病率较高,这一趋势与估计的发病率一致。与女性相比,男性在总人口中的报告发病率往往更高。据估计,AA 在全球总人口中的终生患病率为 0.10%(95% 可信区间,0.03%-0.39%),在成人中为 0.12%(95% 可信区间,0.02%-0.52%),在儿童中为 0.03%(95% 可信区间,0.01%-0.12%)。亚洲地区的估计发病率最高,非洲地区最低:在这项研究中,全球疾病负担地区中有 48% 的地区没有足够的数据报告 AA 的流行率或发病率。需要进一步开展研究,提供中低收入国家的流行病学信息。我们的研究可作为医疗保健政策决策的重要参考。
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引用次数: 0
Increasing melanoma incidence with unchanged mortality: more sunshine, better treatment, increased diagnostic activity, overdiagnosis or lowered diagnostic threshold? 黑色素瘤发病率上升而死亡率不变:更多的阳光、更好的治疗、诊断活动增加、过度诊断还是诊断阈值降低?
IF 11 1区 医学 Q1 DERMATOLOGY Pub Date : 2024-08-14 DOI: 10.1093/bjd/ljae175
Jesper Bo Nielsen, Ivar Sønbø Kristiansen, Subash Thapa

Background: Increasing melanoma incidence with less increasing mortality is observed in several countries. This discrepancy is not well understood.

Objectives: In this study, our aim was to discuss factors [ultraviolet radiation (UVR) exposure, melanoma treatment, diagnostic activity, overdiagnosis, pathologists' diagnostic threshold and clinicians' propensity to remove suspect skin lesions] that might influence melanoma incidence and mortality in Denmark.

Methods: This was a register study with the number of melanocyte-related lesions and melanoma mortality based on comprehensive national pathology and mortality databases for the period 1999-2019. We investigated melanocyte-related diagnoses and mortality in a population of 5.5 million with a national healthcare system. Age-adjusted melanoma mortality and age-adjusted incidence of benign naevi, atypical lesion, or melanoma in situ and of invasive melanoma were computed for data analysis.

Results: In total, 1 434 798 biopsies were taken from 704 682 individuals (65% female). The mean age at biopsy was 39.8 years in males and 37.6 in females. In males and females, the incidence of invasive melanoma increased by 87% during the period 1999-2011. During the subsequent period it increased by 9% in males but remained unchanged in females. The incidence of melanoma in situ increased by 476% in males and 357% in females during the study period, while the increases for atypical melanocytic lesions were 1928% and 1686%, respectively. Biopsy rates increased by 153% in males and 118% in females from 1999 through 2011 but fell by 20% in males and 22% in females during the subsequent period. Mortality varied slightly from year to year without any significant time trend for males or females. We identified no evidence of increased UVR exposure over the latest 30 years in Denmark. Immunotherapy of advanced melanoma was introduced in Denmark in 2010 and came into general use in 2014.

Conclusions: Comprehensive national data demonstrate increasing melanoma incidence correlated with increasing biopsy rates, but with no change in mortality. Previously suggested explanations for such a trend are a lowered threshold of melanoma diagnosis among pathologists, increased diagnostic activity in the presence of overdiagnosis and improved melanoma treatment. Because the study is observational and we have more explanatory factors than outcomes, the findings do not warrant conclusions about causal relationships.

背景:在一些国家,黑色素瘤的发病率不断上升,但死亡率却在下降。目的:我们的研究旨在讨论黑色素瘤的发病因素(紫外线照射、黑色素瘤治疗、诊断活动、过度诊断、病理学家):本研究旨在讨论可能影响丹麦黑色素瘤发病率和死亡率的因素(紫外线照射、黑色素瘤治疗、诊断活动、过度诊断、病理学家的诊断阈值以及临床医生切除可疑皮损的倾向):这是一项登记研究,根据 1999-2019 年间全面的国家病理学和死亡率数据库,对黑色素细胞相关病变的数量和黑色素瘤死亡率进行了研究。我们调查了拥有全国医疗保健系统的 550 万人口中与黑色素细胞相关的诊断和死亡率。我们计算了经年龄调整的黑色素瘤死亡率和经年龄调整的良性痣、非典型病变或原位黑色素瘤以及浸润性黑色素瘤的发病率,并进行了数据分析:共从 704,682 人(65% 为女性)身上提取了 1,434,798 份活检样本。男性活组织检查时的平均年龄为 39.8 岁,女性为 37.6 岁。在 1999-2011 年期间,男性和女性的浸润性黑色素瘤发病率增加了 87%。在随后的时间里,男性的发病率增加了9%,但女性的发病率保持不变。在研究期间,男性原位黑色素瘤的发病率增加了476%,女性增加了357%,而非典型黑色素细胞病变的发病率分别增加了1928%和1686%。从1999年到2011年,活检率男性增加了153%,女性增加了118%,但在随后的研究期间,活检率男性下降了20%,女性下降了22%。死亡率每年略有不同,男性和女性的死亡率没有明显的时间趋势。晚期黑色素瘤的免疫疗法于2010年引入丹麦,并于2014年开始普遍使用:综合全国数据显示,黑色素瘤发病率的上升与活检率的上升相关,但死亡率没有变化。以前曾有人提出过这种趋势的解释,即病理学家对黑色素瘤诊断的门槛降低了,诊断活动在过度诊断的情况下增加了,以及黑色素瘤的治疗得到了改善。由于这项研究是观察性的,而且我们的解释因素多于结果,因此研究结果并不能作为因果关系结论的依据。
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引用次数: 0
MiTES: itch or pain? MiTES:痒或痛。
IF 11 1区 医学 Q1 DERMATOLOGY Pub Date : 2024-08-14 DOI: 10.1093/bjd/ljae198
Cristina Has
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引用次数: 0
Ecological evidence for melanoma overdiagnosis in Denmark. 丹麦黑色素瘤过度诊断的生态学证据。
IF 11 1区 医学 Q1 DERMATOLOGY Pub Date : 2024-08-14 DOI: 10.1093/bjd/ljae206
Catherine M Olsen
{"title":"Ecological evidence for melanoma overdiagnosis in Denmark.","authors":"Catherine M Olsen","doi":"10.1093/bjd/ljae206","DOIUrl":"10.1093/bjd/ljae206","url":null,"abstract":"","PeriodicalId":9238,"journal":{"name":"British Journal of Dermatology","volume":null,"pages":null},"PeriodicalIF":11.0,"publicationDate":"2024-08-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140944019","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Expanding the knowledge of tumour necrosis factor-α-induced gasdermin E-mediated pyroptosis in psoriasis. 扩展有关 TNF-α 在银屑病中诱导 GSDME 介导的热蛋白沉积的知识。
IF 11 1区 医学 Q1 DERMATOLOGY Pub Date : 2024-08-14 DOI: 10.1093/bjd/ljae223
Helen Vaher
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引用次数: 0
Correction to: Loss-of-function variants in GLMN are associated with generalized skin hyperpigmentation with or without glomuvenous malformation. 更正为GLMN的功能缺失变体与伴有或不伴有葡萄胎畸形的全身性皮肤色素沉着有关。
IF 11 1区 医学 Q1 DERMATOLOGY Pub Date : 2024-08-14 DOI: 10.1093/bjd/ljae212
{"title":"Correction to: Loss-of-function variants in GLMN are associated with generalized skin hyperpigmentation with or without glomuvenous malformation.","authors":"","doi":"10.1093/bjd/ljae212","DOIUrl":"10.1093/bjd/ljae212","url":null,"abstract":"","PeriodicalId":9238,"journal":{"name":"British Journal of Dermatology","volume":null,"pages":null},"PeriodicalIF":11.0,"publicationDate":"2024-08-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141080491","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
De novo autoimmune connective tissue disease and mortality in patients treated with anti-programmed death receptor-1 and anti-programmed death-ligand 1 therapy: a population-level cohort study. 抗PD-1和抗PD-L1疗法患者的新发自身免疫性结缔组织疾病和死亡率:一项人群队列研究。
IF 11 1区 医学 Q1 DERMATOLOGY Pub Date : 2024-08-14 DOI: 10.1093/bjd/ljae184
Kimberly Tang, Ahmad Rajeh, Katharina S Shaw, Nga Nguyen, Guihong Wan, Kimberly B Hashemi, Rochelle L Castillo, Shawn G Kwatra, Nicole R LeBoeuf, Ruth Ann Vleugels, Yevgeniy R Semenov
{"title":"De novo autoimmune connective tissue disease and mortality in patients treated with anti-programmed death receptor-1 and anti-programmed death-ligand 1 therapy: a population-level cohort study.","authors":"Kimberly Tang, Ahmad Rajeh, Katharina S Shaw, Nga Nguyen, Guihong Wan, Kimberly B Hashemi, Rochelle L Castillo, Shawn G Kwatra, Nicole R LeBoeuf, Ruth Ann Vleugels, Yevgeniy R Semenov","doi":"10.1093/bjd/ljae184","DOIUrl":"10.1093/bjd/ljae184","url":null,"abstract":"","PeriodicalId":9238,"journal":{"name":"British Journal of Dermatology","volume":null,"pages":null},"PeriodicalIF":11.0,"publicationDate":"2024-08-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11324069/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140850627","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The pleiotropic role of interleukin-13 in the pathogenesis of atopic dermatitis. IL-13在注意力缺失症发病机制中的多向作用。
IF 11 1区 医学 Q1 DERMATOLOGY Pub Date : 2024-08-14 DOI: 10.1093/bjd/ljae174
Coco Dekkers, Marjolein de Bruin-Weller
{"title":"The pleiotropic role of interleukin-13 in the pathogenesis of atopic dermatitis.","authors":"Coco Dekkers, Marjolein de Bruin-Weller","doi":"10.1093/bjd/ljae174","DOIUrl":"10.1093/bjd/ljae174","url":null,"abstract":"","PeriodicalId":9238,"journal":{"name":"British Journal of Dermatology","volume":null,"pages":null},"PeriodicalIF":11.0,"publicationDate":"2024-08-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140849245","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The interferon story continues: EORTC CLTG study explores pegylated interferon α-2a's role in treating mycosis fungoides/Sézary syndrome. 干扰素的故事仍在继续:EORTC CLTG 研究探讨聚乙二醇干扰素 Alpha-2α 在治疗真菌病/塞扎里综合征中的作用。
IF 11 1区 医学 Q1 DERMATOLOGY Pub Date : 2024-08-14 DOI: 10.1093/bjd/ljae188
Rudolf Stadler
{"title":"The interferon story continues: EORTC CLTG study explores pegylated interferon α-2a's role in treating mycosis fungoides/Sézary syndrome.","authors":"Rudolf Stadler","doi":"10.1093/bjd/ljae188","DOIUrl":"10.1093/bjd/ljae188","url":null,"abstract":"","PeriodicalId":9238,"journal":{"name":"British Journal of Dermatology","volume":null,"pages":null},"PeriodicalIF":11.0,"publicationDate":"2024-08-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140853543","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
期刊
British Journal of Dermatology
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