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.
{"title":"Recent progress of Stevens-Johnson syndrome/toxic epidermal necrolysis: Diagnosis criteria, pathogenesis and therapy.","authors":"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","doi":"10.1093/bjd/ljae321","DOIUrl":"https://doi.org/10.1093/bjd/ljae321","url":null,"abstract":"<p><p>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.</p>","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":"141981724","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}
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。
{"title":"Global, regional and national epidemiology of alopecia areata: a systematic review and modelling study.","authors":"Jae Joon Jeon, Seung-Won Jung, You Hyun Kim, Rosa Parisi, Ju Yeong Lee, Myung Ha Kim, Won-Soo Lee, Solam Lee","doi":"10.1093/bjd/ljae058","DOIUrl":"10.1093/bjd/ljae058","url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Objectives: </strong>To investigate systematically the global, regional and national incidence and prevalence of AA.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>","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":"139706143","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}
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.
{"title":"Increasing melanoma incidence with unchanged mortality: more sunshine, better treatment, increased diagnostic activity, overdiagnosis or lowered diagnostic threshold?","authors":"Jesper Bo Nielsen, Ivar Sønbø Kristiansen, Subash Thapa","doi":"10.1093/bjd/ljae175","DOIUrl":"10.1093/bjd/ljae175","url":null,"abstract":"<p><strong>Background: </strong>Increasing melanoma incidence with less increasing mortality is observed in several countries. This discrepancy is not well understood.</p><p><strong>Objectives: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>","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":"140854306","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}
{"title":"MiTES: itch or pain?","authors":"Cristina Has","doi":"10.1093/bjd/ljae198","DOIUrl":"10.1093/bjd/ljae198","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":"140911473","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}
{"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}
{"title":"Expanding the knowledge of tumour necrosis factor-α-induced gasdermin E-mediated pyroptosis in psoriasis.","authors":"Helen Vaher","doi":"10.1093/bjd/ljae223","DOIUrl":"10.1093/bjd/ljae223","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":"141080528","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}
{"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}
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}
{"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}
{"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}