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A pediatric case of SAPHO syndrome without palmoplantar pustulosis.
Pub Date : 2025-01-28 DOI: 10.1111/1346-8138.17646
Masato Ishikawa, Toshiyuki Yamamoto
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引用次数: 0
Predictive factors for treatment responses to baricitinib in severe alopecia areata: A retrospective, multivariate analysis of 70 cases from a single center.
Pub Date : 2025-01-27 DOI: 10.1111/1346-8138.17641
Moyuka Wada-Irimada, Takehiro Takahashi, Mana Sekine, Toshiki Okazaki, Takuya Takahashi, Tomoko Chiba, Emi Yamazaki, Kosuke Shido, Toshiya Takahashi, Masato Mizuashi, Yoshihide Asano

Alopecia areata (AA) is a chronic, autoimmune skin disease characterized by non-scarring hair loss. Baricitinib, a Janus kinase inhibitor (JAKi), prevents hair loss and promotes hair regrowth by inhibiting the inflammatory Janus kinase-signal transducer and activator of transcription (JAK-STAT) signaling pathway involved in cytotoxic T cell responses targeting hair follicles. The introduction of JAKi has transformed treatment against severe AA. However, treatment responses to JAKi are highly variable among patients, and the predictors of responsiveness remain insufficiently elucidated. This study aimed to identify independent predictive factors for the efficacy of baricitinib in patients with severe AA using multivariate analyses. A retrospective study was conducted on 70 severe AA patients who started baricitinib treatment at Tohoku University Hospital between July 2022 and August 2023. The primary outcome was the percentage of patients achieving a Severity of Alopecia Tool (SALT) score of ≤20 after 9 months of baricitinib treatment. Multivariate analysis assessed potential predictors of baricitinib treatment responses, including AA type, sex, age, disease duration, history of atopic dermatitis, intravenous methylprednisolone pulse (IVMP) therapy, and Clinician-Reported Outcome (ClinRO) measures for eyebrows and eyelashes. Achievement of a SALT score of ≤20 and SALT score improvement rates were used as objective variables in the multivariate analyses. Among the 70 patients completing 9 months of baricitinib treatment, 41% achieved a SALT score of ≤20. Multivariate analyses identified several independent predictors for positive outcomes, including shorter disease duration (≤4 years), history of IVMP, therapy SALT score of ≤95 at baricitinib initiation, and female sex. Further, we found differential response patterns based on AA type and sex. Specifically, AA type significantly influenced treatment responses, with ophiasis alopecia (OA) associated with the poorest improvement rate. In summary, the response to baricitinib in AA is significantly influenced by sex, AA type, disease duration, history of IVMP, and pre-treatment SALT score.

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引用次数: 0
Repurposing diacerein for the treatment of chronic wounds in recessive-dystrophic epidermolysis bullosa patients by modulating matrix metalloproteinase-9 expression.
Pub Date : 2025-01-24 DOI: 10.1111/1346-8138.17621
Sonja Dorfer, Michael Ablinger, Monika Wimmer, Julia I Hummel, Selma Ibrahimpašić, Anja Diem, Martin Laimer, Stefanie Gruner, Josefina Piñón Hofbauer, Christina Guttmann-Gruber, Ulrich Koller, Iris K Gratz, Johann W Bauer, Roland Zauner, Verena Wally

Recessive dystrophic epidermolysis bullosa (RDEB) is caused by mutations in COL7A1, leading to loss or dysfunction of type-VII collagen (C7), a protein essential for skin stability. Clinically, patients suffer from severe skin blistering, chronic or recurrent wounds, and scarring, which predispose to early onset of aggressive squamous cell carcinoma. Previous studies showed that RDEB-keratinocytes (RDEB-KC) express high levels of matrix-metalloproteinase 9 (MMP-9), a molecule known to play a crucial role in wound chronification if dysregulated. We investigated the potential of diacerein, a small molecule that interferes with the MMP-9 regulatory pathway, to improve wound healing in a 5-year old RDEB patient presenting with chronic, generalized skin involvement unresponsive to previous treatment approaches. Upon 4 weeks of topical therapy applied to the patient's back, parents reported a nearly complete wound closure and a significant increase in quality of life. We also provide evidence that diacerein treatment of patient keratinocytes results in a downregulation of MMP-9 expression, accompanied by a reduction in their ability to degrade a fibrinogen matrix. These data characterize diacerein as a potential candidate for improving wound healing in RDEB through its impact on inflammatory as well as epithelial cells.

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引用次数: 0
A case of localized scleroderma with variable response to excimer light therapy. 准分子光治疗对局部硬皮病的可变反应1例。
Pub Date : 2025-01-21 DOI: 10.1111/1346-8138.17635
Kyoko Kimura, Takeshi Fukumoto, Akiharu Kubo
{"title":"A case of localized scleroderma with variable response to excimer light therapy.","authors":"Kyoko Kimura, Takeshi Fukumoto, Akiharu Kubo","doi":"10.1111/1346-8138.17635","DOIUrl":"https://doi.org/10.1111/1346-8138.17635","url":null,"abstract":"","PeriodicalId":94236,"journal":{"name":"The Journal of dermatology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-01-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143019166","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Long-term follow-up of sweat-gland carcinoma with neuroendocrine differentiation (SCAND) with sentinel lymph node metastases. 前哨淋巴结转移伴神经内分泌分化(SCAND)的汗腺癌的长期随访。
Pub Date : 2025-01-21 DOI: 10.1111/1346-8138.17620
Masahito Yasuda, Setsuko Sasahira, Etsuko Okada, Atsushi Tamura, Osamu Ishikawa, Keisuke Goto, Yoji Kukita, Keiichiro Honma, Sei-Ichiro Motegi
{"title":"Long-term follow-up of sweat-gland carcinoma with neuroendocrine differentiation (SCAND) with sentinel lymph node metastases.","authors":"Masahito Yasuda, Setsuko Sasahira, Etsuko Okada, Atsushi Tamura, Osamu Ishikawa, Keisuke Goto, Yoji Kukita, Keiichiro Honma, Sei-Ichiro Motegi","doi":"10.1111/1346-8138.17620","DOIUrl":"https://doi.org/10.1111/1346-8138.17620","url":null,"abstract":"","PeriodicalId":94236,"journal":{"name":"The Journal of dermatology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-01-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143019301","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A case of psoriasiform drug eruption revealed by a drug-induced lymphocyte stimulation test. 药物性淋巴细胞刺激试验显示银屑病样药疹1例。
Pub Date : 2025-01-21 DOI: 10.1111/1346-8138.17638
Satoshi Takeuchi, Yasuko Shiomichi, Takeshi Nakahara
{"title":"A case of psoriasiform drug eruption revealed by a drug-induced lymphocyte stimulation test.","authors":"Satoshi Takeuchi, Yasuko Shiomichi, Takeshi Nakahara","doi":"10.1111/1346-8138.17638","DOIUrl":"https://doi.org/10.1111/1346-8138.17638","url":null,"abstract":"","PeriodicalId":94236,"journal":{"name":"The Journal of dermatology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-01-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143019208","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Buruli ulcer with eccrine squamous syringometaplasia mimicking squamous cell carcinoma. 布鲁里溃疡伴酷似鳞状细胞癌的胰腺鳞状淋巴管化生。
Pub Date : 2025-01-21 DOI: 10.1111/1346-8138.17637
Yoshimichi Kobayashi, Toshifumi Takahashi, Yasuaki Ikuno, Hiraku Kokubu, Akihiko Yamaguchi, Yoshihiko Hoshino, Akiko Arakawa, Noriki Fujimoto
{"title":"Buruli ulcer with eccrine squamous syringometaplasia mimicking squamous cell carcinoma.","authors":"Yoshimichi Kobayashi, Toshifumi Takahashi, Yasuaki Ikuno, Hiraku Kokubu, Akihiko Yamaguchi, Yoshihiko Hoshino, Akiko Arakawa, Noriki Fujimoto","doi":"10.1111/1346-8138.17637","DOIUrl":"https://doi.org/10.1111/1346-8138.17637","url":null,"abstract":"","PeriodicalId":94236,"journal":{"name":"The Journal of dermatology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-01-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143019281","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A Japanese case of Papillon-Lefèvre syndrome diagnosed by pyogenic liver abscess. 日本1例以化脓性肝脓肿诊断为左侧乳头状瘤综合征。
Pub Date : 2025-01-20 DOI: 10.1111/1346-8138.17631
Akari Terada, Takaya Murai, Eijiro Akasaka
{"title":"A Japanese case of Papillon-Lefèvre syndrome diagnosed by pyogenic liver abscess.","authors":"Akari Terada, Takaya Murai, Eijiro Akasaka","doi":"10.1111/1346-8138.17631","DOIUrl":"https://doi.org/10.1111/1346-8138.17631","url":null,"abstract":"","PeriodicalId":94236,"journal":{"name":"The Journal of dermatology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143019278","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Identification of key malodor components in onychomycosis: A pilot study. 甲真菌病中关键恶臭成分的鉴定:一项初步研究。
Pub Date : 2025-01-20 DOI: 10.1111/1346-8138.17633
Keiko Sakamoto, Masako Katsuyama, Chieko Okamura, Tetsuya Honda
{"title":"Identification of key malodor components in onychomycosis: A pilot study.","authors":"Keiko Sakamoto, Masako Katsuyama, Chieko Okamura, Tetsuya Honda","doi":"10.1111/1346-8138.17633","DOIUrl":"https://doi.org/10.1111/1346-8138.17633","url":null,"abstract":"","PeriodicalId":94236,"journal":{"name":"The Journal of dermatology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143019299","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Clinical patterns of vitiligo in Japan: A descriptive study using the JMDC claims database. 日本白癜风的临床模式:使用JMDC索赔数据库的描述性研究。
Pub Date : 2025-01-20 DOI: 10.1111/1346-8138.17627
Atsushi Tanemura, Yue Ma, Masayo Sakaki-Yumoto, Shintaro Hiro, Tomohiro Hirose, Tamio Suzuki

Vitiligo is a chronic autoimmune disorder that profoundly impacts patients' quality of life. Real-world data on vitiligo in Japan are limited. This descriptive, cross-sectional study used a claims database to evaluate vitiligo prevalence, patient demographics, treatments, and comorbidities in Japanese patients with vitiligo. Patients with claims for a vitiligo diagnosis in the JMDC database from January 2010 to December 2022 were included. Annual vitiligo prevalence, comorbidities, treatments, and medical facility visits were analyzed. Of 16 947 087 patients in the database during the 13-year analysis period, a total of 26 358 patients (0.16%, 95% confidence interval 0.15-0.16) had a diagnosis of vitiligo. The standardized prevalence of vitiligo by sex and age in Japan remained generally consistent between 2010 (0.051%) and 2022 (0.056%). Atopic dermatitis was the most prevalent comorbidity. Comorbid atopic dermatitis prevalence increased between 2010 (21.8%) and 2022 (34.0%), and was highest among children aged 5-9 years. Other common comorbidities in 2022 included hypertension (10.4%), dyslipidemia (8.0%), anxiety disorder (7.4%), and psoriasis (7.0%). Topical corticosteroids were the most commonly used treatment throughout the period analyzed. Between 2010 and 2022, topical corticosteroid use decreased from 75.1% to 66.9%, and the use of narrowband ultraviolet B procedures increased from 19.2% to 28.1%. Mean duration of care was 12.9 months (standard deviation 20.5 months) and the median total number of outpatient medical facility visits was 3.0 (interquartile range 1.0-12.0). Key limitations include age and occupational biases in the JMDC database and potential misclassification of comorbidities due to off-label treatment coding. Despite limitations in using a claims database, this study demonstrates consistent vitiligo prevalence in Japan, a high comorbidity burden, and evolving treatment patterns. Findings may guide clinical practice and treatment guidelines to improve management of vitiligo in Japanese patients.

白癜风是一种慢性自身免疫性疾病,严重影响患者的生活质量。日本白癜风的真实数据有限。这项描述性横断面研究使用索赔数据库来评估日本白癜风患者的白癜风患病率、患者人口统计、治疗和合并症。2010年1月至2022年12月在JMDC数据库中被诊断为白癜风的患者被纳入研究。分析白癜风年度患病率、合并症、治疗和医疗机构就诊情况。在13年的分析期间,数据库中的16 947 087例患者中,共有26 358例(0.16%,95%可信区间0.15-0.16)被诊断为白癜风。日本按性别和年龄划分的白癜风标准化患病率在2010年(0.051%)和2022年(0.056%)之间基本保持一致。特应性皮炎是最常见的合并症。共病性特应性皮炎患病率在2010年(21.8%)至2022年(34.0%)期间有所增加,其中5-9岁儿童患病率最高。2022年的其他常见合并症包括高血压(10.4%)、血脂异常(8.0%)、焦虑症(7.4%)和牛皮癣(7.0%)。在整个分析期间,外用皮质类固醇是最常用的治疗方法。2010年至2022年间,局部皮质类固醇的使用从75.1%下降到66.9%,窄带紫外线B手术的使用从19.2%上升到28.1%。平均护理时间为12.9个月(标准差为20.5个月),门诊总次数中位数为3.0次(四分位数间距为1.0-12.0)。主要的限制包括JMDC数据库中的年龄和职业偏差,以及由于标签外治疗编码而导致的合并症的潜在错误分类。尽管在使用索赔数据库方面存在局限性,但本研究表明,白癜风在日本的患病率是一致的,合并症负担高,治疗模式也在不断发展。研究结果可以指导临床实践和治疗指南,以改善日本白癜风患者的管理。
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引用次数: 0
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The Journal of dermatology
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