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A case of IgA vasculitis with Koebner phenomenon of the nose 一例 IgA 血管炎合并鼻腔柯布纳现象的病例
Pub Date : 2024-09-13 DOI: 10.1111/1346-8138.17458
Taiki Nakai, Chikane Maeda, Issei Kido, Kimiko Nakajima, Yuki Osakabe, Tatsuki Matsumoto, Yuki Ogasawara, Yusuke Oki, Kozo Nakai
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引用次数: 0
Psoriasis treatment and biologic switching: The association with clinical characteristics and laboratory biomarkers over a 13‐year retrospective study 银屑病治疗与生物制剂转换:13 年回顾性研究与临床特征和实验室生物标志物的关系
Pub Date : 2024-09-13 DOI: 10.1111/1346-8138.17465
Asumi Koyama, Lixin Li, Toyoki Yamamoto, Haruka Taira, Eiki Sugimoto, Yukiko Ito, Yuka Mizuno, Kentaro Awaji, Shoko Tateishi, Hiroko Kanda, Shinichi Sato, Sayaka Shibata
The advent of biologics has greatly improved patient outcomes, yet some patients are compelled to switch therapies. Predicting these therapeutic failures is important; however, the factors associated with switching biologics have not been fully explored. This study examined patterns and determinants of biologics switching in psoriasis treatment retrospectively over 13 years. We focused on the association between clinical characteristics, basal laboratory data, and frequency of biologics switching. The findings revealed that elevated Psoriasis Area Severity Index scores and the presence of arthritis were observed in patients who experienced two or more treatment switches compared with those without treatment switches. Moreover, neutrophil to lymphocyte ratio was associated with higher biologics switching rates, indicating that systemic inflammation significantly impacts treatment adherence. A treatment approach, taking into account both the clinical presentation and inflammatory biomarkers, may be important for optimizing patient management in psoriasis.
生物制剂的出现大大改善了患者的治疗效果,但有些患者不得不更换疗法。预测这些治疗失败非常重要;然而,与转换生物制剂相关的因素尚未得到充分探讨。本研究回顾性研究了 13 年来银屑病治疗中生物制剂转换的模式和决定因素。我们重点研究了临床特征、基础实验室数据和生物制剂转换频率之间的关联。研究结果表明,与未更换治疗方案的患者相比,更换过两次或两次以上治疗方案的患者的银屑病面积严重性指数评分升高,并伴有关节炎。此外,中性粒细胞与淋巴细胞比率与较高的生物制剂转换率相关,表明全身炎症对治疗依从性有重大影响。同时考虑临床表现和炎症生物标志物的治疗方法可能对优化银屑病患者的管理非常重要。
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引用次数: 0
A case of solitary encapsulated angiolymphoid hyperplasia with eosinophilia in the hypodermis of possible lymph node origin 一例真皮下伴有嗜酸性粒细胞增多的单发包裹性血管淋巴样增生症,可能源自淋巴结
Pub Date : 2024-09-13 DOI: 10.1111/1346-8138.17462
Keisuke Ueda, Noriko Kato, Hirofumi Niwa, Kazuhiro Kobayashi, Hiroaki Iwata
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引用次数: 0
A case of familial progressive hyperpigmentation with or without hypopigmentation presenting with hypopigmented striae along the lines of Blaschko 一例伴有或不伴有色素减退的家族性进行性色素沉着症,表现为沿布拉什科纹的色素减退性条纹
Pub Date : 2024-09-13 DOI: 10.1111/1346-8138.17459
Tokimasa Hida, Masashi Idogawa, Aki Ishikawa, Masae Okura, Satoru Sasaki, Takashi Tokino, Hisashi Uhara
Familial progressive hyperpigmentation with or without hypopigmentation (FPHH) is an autosomal dominant disorder characterized by widespread skin hyperpigmentation, café‐au‐lait spots, and hypopigmented circular macules, resulting from KITLG variants. KITLG, expressed by keratinocytes, binds to KIT on melanocytes, stimulating melanogenesis. Disturbances in the KITLG‐KIT interaction result in diffuse hyperpigmentation in FPHH. However, the mechanisms behind hypopigmented macule formation remain unclear. This report presents a unique FPHH case in a patient with a novel KITLG mutation (Ser78Leu). Notably, the patient showed multiple hypopigmented macules and striae along the lines of Blaschko. Digital polymerase chain reaction analysis of the DNA from skin and blood tissues indicated a copy‐neutral loss of heterozygosity at the KITLG locus, only in the hypopigmented macule. These findings suggest that the hypopigmented macules might result from revertant mosaicism. Conversely, café‐au‐lait spots do not follow the lines of Blaschko and can superimpose on the hypopigmented striae, indicating a distinct pathogenesis. This case contributes to the understanding of the genetic mechanisms in FPHH.
家族性进行性色素沉着伴或不伴色素减退(FPHH)是一种常染色体显性遗传病,其特征是广泛的皮肤色素沉着、咖啡斑和色素减退性圆形斑丘疹,由 KITLG 变体引起。KITLG 由角质形成细胞表达,能与黑色素细胞上的 KIT 结合,刺激黑色素生成。KITLG-KIT 相互作用的紊乱会导致 FPHH 中弥漫性色素沉着。然而,色素沉着斑形成背后的机制仍不清楚。本报告介绍了一名患有新型 KITLG 突变(Ser78Leu)患者的独特 FPHH 病例。值得注意的是,该患者出现了多个色素减退性黄斑和沿布拉氏线的条纹。对来自皮肤和血液组织的 DNA 进行的数字聚合酶链反应分析表明,KITLG 基因座的杂合性缺失为拷贝中性,仅存在于色素减退斑中。这些发现表明,色素沉着斑可能是由回复嵌合引起的。相反,咖啡色斑并不遵循布拉什科线,而且可以叠加在色素减退斑上,这表明其发病机制不同。本病例有助于人们了解 FPHH 的遗传机制。
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引用次数: 0
Clinical and laboratory features between anti‐TIF1γ dermatomyositis with and without malignancy: 37 case series and a review 抗TIF1γ皮肌炎伴有和不伴有恶性肿瘤的临床和实验室特征:37个病例系列和综述
Pub Date : 2024-09-11 DOI: 10.1111/1346-8138.17426
Ke‐yun Tang, Han‐lin Zhang, Xin‐yi Zhang, Hong‐zhong Jin
We aimed to analyze the clinical profile and malignancy indicators in dermatomyositis (DM) with anti‐transcriptional intermediary factor 1 antibody (anti‐TIF1γ‐Ab). A comparison was made between clinical information of anti‐TIF1γ DM patients with and without malignancy. Additionally, a review of the literature on anti‐TIF1γ DM and malignancy was conducted by searching PubMed and EMBASE databases. In our cohort of 37 patients, 27.0% (10/37) developed malignancy. The timeframe during which these 10 patients developed malignancy ranged from 21 months prior to the diagnosis of DM to 36 months following the diagnosis of DM. Specifically, one patient was diagnosed with breast cancer at the age of 36. Comparing the groups with and without malignancy, we found that age over 65 years (40% vs 7.4%, P = 0.035), a shorter duration from the onset of symptoms to the diagnosis of DM (2.5 vs 10 months, P = 0.003), and higher erythrocyte sedimentation rate (ESR) levels (23 vs 10 mm/h, P = 0.048) were found to be associated with an increased risk of malignancy. Conversely, the presence of Gottron's papules (63% vs 20%, P = 0.029) may suggest a lower likelihood of malignancy. The literature review revealed that the prevalence of myositis‐associated malignancy was 40.7% (340/836), with variations ranging from 19% to 82.9% across different series. In summary, factors such as age over 65 years, a shorter duration between symptom onset and diagnosis of DM, and elevated ESR levels may indicate an increased risk of malignancy in anti‐TIF1γ DM patients.
我们旨在分析皮肌炎(DM)患者抗转录中间因子1抗体(抗TIF1γ-Ab)的临床概况和恶性肿瘤指标。比较了有恶性肿瘤和无恶性肿瘤的抗TIF1γ DM患者的临床信息。此外,还通过检索 PubMed 和 EMBASE 数据库,对抗 TIF1γ DM 和恶性肿瘤的相关文献进行了综述。在我们的 37 例患者中,27.0%(10/37)发生了恶性肿瘤。这10名患者罹患恶性肿瘤的时间范围从DM诊断前的21个月到DM诊断后的36个月不等。其中,一名患者在 36 岁时被诊断出患有乳腺癌。比较有恶性肿瘤和没有恶性肿瘤的组别,我们发现年龄超过 65 岁(40% vs 7.4%,P = 0.035)、从出现症状到确诊为 DM 的时间较短(2.5 vs 10 个月,P = 0.003)、红细胞沉降率(ESR)水平较高(23 vs 10 mm/h,P = 0.048)与恶性肿瘤风险增加有关。相反,出现戈特龙丘疹(63% 对 20%,P = 0.029)可能表明恶性肿瘤的可能性较低。文献综述显示,肌炎相关恶性肿瘤的发病率为 40.7%(340/836),不同系列的发病率从 19% 到 82.9% 不等。总之,年龄超过65岁、从症状出现到诊断为DM的时间较短、ESR水平升高等因素可能预示着抗TIF1γ DM患者发生恶性肿瘤的风险增加。
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引用次数: 0
Analysis of disease burden in patients with hereditary angioedema from Japan by patient‐reported outcomes 通过患者报告结果分析日本遗传性血管性水肿患者的疾病负担
Pub Date : 2024-09-11 DOI: 10.1111/1346-8138.17421
Michihiro Hide, Miwa Kishimoto, Ippei Kotera, Akinori Oh, Yoichi Inoue, Beverley Anne Yamamoto, Shinichi Noto
Hereditary angioedema (HAE) symptoms can vary greatly. Disease burden evaluation is essential for providing adequate treatments for patients. Patient‐reported outcome measures (PROMs), including the 12‐Item Short Form Health Survey (SF‐12), the Angioedema Quality of Life (AE‐QoL), the Hospital Anxiety and Depression Scale (HADS), and the Work Productivity and Activity Impairment: Specific Health Problem (WPAI:SHP) questionnaires, were collected in 2021, before modern medications for long‐term prophylaxis (LTP) of HAE were licensed in Japan. Patients also reported their HAE attack frequency as “annual” (several attacks annually), “monthly” (several attacks monthly) or “weekly” (several attacks weekly). Multiple linear regression analyses were conducted on the relationship between independent parameters (sex, age, attack frequency, HAE type, and HADS scores) and dependent parameters (AE‐QoL and SF‐12 scores). Fifty‐four patients reported PROMs. All PROMs showed substantial health‐related quality of life (HRQoL) impairment. Overall, the higher the attack frequencies, the greater the reported impairment in the PROMs tended to be. In multiple linear regression analyses, higher AE‐QoL Fatigue/Mood and Fears/Shame domain scores (greater impairment) were associated with higher HADS anxiety subscale scores; higher AE‐QoL total scores (greater HRQoL impairment) and lower SF‐12 Physical and Mental Health Composite scores (greater general health impairment) were associated with higher HADS depression subscale scores. Patients with monthly or weekly HAE attacks reported numerically low absenteeism and numerically high presenteeism and work productivity loss as measured by the WPAI:SHP questionnaire. In this study, conducted before modern LTP options were available in Japan, patients with HAE reported notable impairment in HRQoL and work productivity. Weekly or monthly HAE attack frequencies were associated with a high disease burden. Furthermore, a substantial number of patients reported notable fatigue/mood impairment as measured by the AE‐QoL and depression as measured by the HADS regardless of attack frequency. These results provide a basis for future studies evaluating the effect of LTP on the clinical manifestations and HRQoL in patients with HAE.
遗传性血管性水肿(HAE)的症状千差万别。疾病负担评估对于为患者提供适当的治疗至关重要。患者报告的结果测量(PROMs)包括 12 项简表健康调查(SF-12)、血管性水肿生活质量(AE-QoL)、医院焦虑抑郁量表(HADS)以及工作效率和活动障碍:这些问卷是在2021年收集的,当时日本还没有获得用于HAE长期预防(LTP)的现代药物许可。患者还以 "年"(每年发作数次)、"月"(每月发作数次)或 "周"(每周发作数次)为单位报告其HAE发作频率。对自变量(性别、年龄、发作频率、HAE 类型和 HADS 评分)与因变量(AE-QoL 和 SF-12 评分)之间的关系进行了多元线性回归分析。54 名患者报告了 PROMs。所有 PROM 均显示与健康相关的生活质量(HRQoL)严重受损。总体而言,发作频率越高,所报告的 PROMs 受损程度往往越大。在多元线性回归分析中,较高的 AE-QoL 疲劳/情绪和恐惧/羞耻领域得分(损伤程度更大)与较高的 HADS 焦虑分量表得分相关;较高的 AE-QoL 总分(较高的 HRQoL 损伤程度)和较低的 SF-12 身心健康综合得分(较高的一般健康损伤程度)与较高的 HADS 抑郁分量表得分相关。根据WPAI:SHP问卷调查,每月或每周HAE发作的患者缺勤率较低,旷工率和工作效率损失较高。在这项研究中,HAE 患者的 HRQoL 和工作效率明显受损。HAE 每周或每月的发作频率与较高的疾病负担有关。此外,无论发作频率如何,相当多的患者都报告了明显的疲劳/情绪损害(用 AE-QoL 测量)和抑郁(用 HADS 测量)。这些结果为今后评估 LTP 对 HAE 患者临床表现和 HRQoL 的影响的研究提供了依据。
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引用次数: 0
HPV 51‐associated inguinal SCC on an atopic dermatitis patient treated with cyclosporine 一名接受环孢素治疗的特应性皮炎患者腹股沟 SCC 与 HPV 51 相关
Pub Date : 2024-09-10 DOI: 10.1111/1346-8138.17454
Tomotaka Sato, Manabu Nakazono, Keita Ohyachi, Aya Okaniwa, Hisashi Kobayashi, Kazuto Yamazaki
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引用次数: 0
Exploratory study on short‐term administration of oral fosravuconazole for tinea pedis 关于短期口服福斯拉康唑治疗足癣的探索性研究
Pub Date : 2024-09-10 DOI: 10.1111/1346-8138.17455
Yuichiro Tsunemi, Wataru Naka
We investigated the clinical efficacy of short‐term, oral fosravuconazole (F‐RVCZ) therapy for tinea pedis, commonly known as athlete's foot. F‐RVCZ (equivalent to 100 mg ravuconazole) was administered orally once daily for 1 week for interdigital and vesicular tinea pedis and for 4 weeks for hyperkeratotic tinea pedis. Efficacy was evaluated based on mycological efficacy and clinical symptoms at Weeks 1, 4, and 8 for interdigital and vesicular tinea pedis and at Weeks 4, 8, and 12 for hyperkeratotic tinea pedis. Efficacy was confirmed at the end of treatment. Therapeutic efficacy increased over time from the end of treatment for both types of tinea pedis. All adverse drug reactions (ADRs) were within expectations and there were no cases of discontinuation due to ADRs or serious ADRs. Short‐term oral F‐RVCZ therapy is expected to be as effective or more effective than terbinafine and itraconazole, which have already been approved in Japan and may be a useful option for the treatment of tinea pedis.
我们研究了短期口服福斯拉康唑(F-RVCZ)治疗脚癣(俗称香港脚)的临床疗效。F-RVCZ(相当于 100 毫克雷武康唑)每天口服一次,治疗趾间癣和水泡型足癣 1 周,治疗角化过度型足癣 4 周。根据霉菌学疗效和临床症状,在第 1、4 和 8 周对足间癣和水泡型足癣进行疗效评估,在第 4、8 和 12 周对角化过度型足癣进行疗效评估。疗效在治疗结束时得到确认。两种类型的足癣疗效均在治疗结束后随时间推移而增加。所有药物不良反应(ADR)均在预期范围内,没有因药物不良反应或严重药物不良反应而停药的病例。预计短期口服 F-RVCZ 的疗效与特比萘芬和伊曲康唑相同或更佳,这两种药物已在日本获得批准,可能是治疗足癣的有效选择。
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引用次数: 0
Coexistence of Basan syndrome and cutaneous basal cell carcinoma: Genetic and clinical perspectives 巴桑综合征与皮肤基底细胞癌并存:遗传与临床视角
Pub Date : 2024-09-10 DOI: 10.1111/1346-8138.17443
Zhijie Luo, Yansi Lyu, Wenwu Dong, Zhongxi Mei, Huijun Wang, Fang Yang
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引用次数: 0
Association of hidradenitis suppurativa (HS) with waist circumference: A bidirectional two-sample Mendelian randomization study of HS with metabolic syndrome. 化脓性扁桃体炎(HS)与腰围的关系:一项关于化脓性扁桃体炎与代谢综合征的双向双样本孟德尔随机研究。
Pub Date : 2024-09-04 DOI: 10.1111/1346-8138.17436
Huaiyu Wang, Baofeng Wu, Min Luo, Yue Han, Jinhua Chen, Jingjing Liu, Lihang Lin, Xuemin Xiao

Observational studies have suggested an associations between hidradenitis suppurativa (HS) and metabolic syndrome (MetS) and its components. However, it remains unclear whether the relationship is causal or not. Our study aimed to investigate the causal association of HS with MetS and its components. We performed a bidirectional, two-sample Mendelian randomization study using summary-level data from the most comprehensive genome-wide association studies of HS (n = 362 071), MetS (n = 291 107), waist circumference (n = 462 166), hypertension (n = 463 010) fasting blood glucose (FBG, n = 200 622), triglycerides (n = 441 016), and high-density lipoprotein cholesterol (HDL-C, n = 403 943). Genetic instrumental variables were constructed by identifying single nucleotide polymorphisms associated with the corresponding factors. The random-effects inverse-variance weighted method was applied as the primary method. The results showed that genetically predicted HS was positively associated with waist circumference risk in both directions. High waist circumference increased the risk of HS (odds ratio [OR] 4.147; 95% confidence interval [CI] 2.610-6.590; p = 1.746 × 10-9). In addition, HS was also affected by waist circumference (OR 1.009; 95% CI 1.006-1.012; p = 3.08 × 10-7). No causal relationships were found between HS and MetS or its components other than waist circumference. The findings highlight the importance of early intervention for obesity in HS patients. Further studies are needed to determine the pathophysiology of HS associated with MetS and its components.

观察性研究表明,化脓性扁桃体炎(HS)与代谢综合征(MetS)及其组成部分之间存在关联。然而,这种关系是否是因果关系仍不清楚。我们的研究旨在调查 HS 与 MetS 及其组成部分之间的因果关系。我们使用最全面的全基因组关联研究的汇总数据,对 HS(n = 362 071)、MetS(n = 291 107)、腰围(n = 462 166)、高血压(n = 463 010)、空腹血糖(FBG,n = 200 622)、甘油三酯(n = 441 016)和高密度脂蛋白胆固醇(HDL-C,n = 403 943)进行了双向、双样本孟德尔随机化研究。通过识别与相应因子相关的单核苷酸多态性,构建了遗传工具变量。采用随机效应逆方差加权法作为主要方法。结果显示,遗传预测的 HS 与腰围风险呈双向正相关。高腰围会增加罹患 HS 的风险(几率比 [OR] 4.147;95% 置信区间 [CI]2.610-6.590;P = 1.746 × 10-9)。此外,腰围也会影响 HS(OR 1.009;95% CI 1.006-1.012;P = 3.08 × 10-7)。除腰围外,未发现 HS 与 MetS 或其组成部分之间存在因果关系。研究结果凸显了对 HS 患者肥胖进行早期干预的重要性。要确定HS与MetS及其组成部分之间的病理生理学关系,还需要进一步的研究。
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引用次数: 0
期刊
The Journal of dermatology
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