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Apremilast in Chronic Graft-Versus-Host Disease: A Paediatric Case Report. 阿普司特治疗慢性移植物抗宿主病:儿科病例报告
IF 3.7 4区 医学 Q1 DERMATOLOGY Pub Date : 2024-11-15 DOI: 10.1093/ced/llae494
Tristán Gabriel Sobral-Costas, Rafael Escudero-Tornero, Rocío Maseda, Yasmina Mozo Del Castillo, Marta Feito-Rodríguez
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引用次数: 0
Real-world efficacy and safety of dupilumab for paediatric atopic dermatitis: a multicentre retrospective study. 杜匹单抗治疗儿童特应性皮炎的实际疗效和安全性:一项多中心回顾性研究。
IF 3.7 4区 医学 Q1 DERMATOLOGY Pub Date : 2024-11-14 DOI: 10.1093/ced/llae406
Eitan Pirov, Vered Molho-Pessach, Eran Cohen-Barak, Jen A Barak Levitt, Aviv Barzilai, Jonathan Bar, Yael Renert-Yuval, Efrat Bar-Ilan, Rivka Friedland, Shoshana Greenberger

Background: Real-world data regarding the use of dupilumab in children with atopic dermatitis (AD) are limited.

Objectives: To evaluate the real-world efficacy of dupilumab in children with moderate-to-severe AD over an extended follow-up period.

Methods: This was a retrospective study of patients (≤ 18 years) with moderate-to-severe AD treated with dupilumab in four Israeli tertiary centres. Efficacy and safety were assessed using descriptive statistics.

Results: In total, 230 patients were included in the analysis [age 9.9 years (SD 4.3), male/female 1 : 1 ratio)]. Of them, 59.6% (137/230) had ≥ 1 atopic comorbidity. The follow-up duration ranged from 2 to 248 weeks, with a median of 52 weeks (interquartile range 24-96). Within 12 weeks of treatment, 41.7% (68/163) of patients had reached Investigator Global Assessment 0-1. The mean body surface area was reduced from 58.0% (SD 20.5%) at baseline to 27.8% (SD 20.2%) at 12 weeks. The average Pruritus Numeric Rating Scale score was reduced from 7.9 (SD 2.2) at baseline to 2.3 (SD 2.8) at 12 weeks. Adverse events, in 210 patients, included conjunctivitis in 34 patients (16.2%), injection-site reactions in 11 patients (5.2%) and dupilumab-associated head and neck dermatitis in 6 patients (2.9%). Overall, 26 of 210 patients (12.3%) discontinued the treatment: 9 of the 26 patients (35%) because of adverse events and 15 patients (58%) because of inadequate efficacy. The overall probability of dupilumab survival at 52 weeks was 94.0%.

Conclusions: Real-world data presented here for 230 paediatric and adolescents with moderate-to-severe AD reinforce dupilumab's efficacy and safety and highlight dupilumab's high survival rate after 1 year of treatment in the paediatric population.

背景有关在特应性皮炎(AD)患儿中使用杜比鲁单抗的真实世界数据有限:评估杜必鲁单抗在中重度特应性皮炎儿童患者中的实际疗效:这是一项回顾性研究,研究对象是在以色列四家三级医疗中心接受过杜比鲁单抗治疗的中重度 AD 患者(≤ 18 岁)。研究采用描述性统计方法评估疗效和安全性:共有 230 名患者纳入分析[年龄 9.9 岁(标清 4.3),男女比例 1:1]。其中,59.6%(137/230)的患者有≥一种特应性合并症。随访时间从 2 周到 248 周不等,中位数为 52 周(四分位数间距为 24-96)。在治疗的 12 周内,41.7%(68/163)的患者达到了研究者总体评估 0-1。平均体表面积从基线时的58.0%(标准差20.5%)减少到12周时的27.8%(标准差20.2%)。瘙痒数值评定量表的平均得分从基线时的 7.9 分(标准差 2.2 分)降至 12 周时的 2.3 分(标准差 2.8 分)。210名患者中发生的不良反应包括:34名患者(16.2%)出现结膜炎,11名患者(5.2%)出现注射部位反应,6名患者(2.9%)出现杜匹单抗相关头颈部皮炎。总体而言,210 名患者中有 26 人(12.3%)中断了治疗:26 人中有 9 人(35%)是因为不良反应,15 人(58%)是因为疗效不佳。52周时,杜必鲁单抗的总体存活概率为94.0%:本文提供的 230 例中度至重度 AD 儿童和青少年患者的真实数据增强了杜比单抗的疗效和安全性,并强调了杜比单抗在儿童群体中治疗 1 年后的高存活率。
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引用次数: 0
Papular elastorrhexis- Recognizing an uncommon entity. 乳头状皲裂--认识一种不常见的病症。
IF 3.7 4区 医学 Q1 DERMATOLOGY Pub Date : 2024-11-12 DOI: 10.1093/ced/llae485
Aishwarya Muddebihal, Pooja Arora, Meenakshi Batrani
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引用次数: 0
Malignant Peripheral Nerve Sheath Tumour in the Setting of a Rare Germline BAP1 Disease Causing Variant presenting as a Metastatic Melanoma Mimic. 恶性外周神经鞘瘤是一种罕见的遗传性 BAP1 致病变异,表现为一种转移性黑色素瘤模拟体。
IF 3.7 4区 医学 Q1 DERMATOLOGY Pub Date : 2024-11-11 DOI: 10.1093/ced/llae462
Amy Cunliffe, Rand Hawari, Angana Mitra, Bipin Mathew, William Merchant, Hayley Smith, Andrew J Muinonen-Martin, Maulina Sharma
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引用次数: 0
Celiac risk doubles in patients with alopecia areata: a nationwide case -control study. 斑秃患者患乳糜泻的风险增加一倍:一项全国性病例对照研究。
IF 3.7 4区 医学 Q1 DERMATOLOGY Pub Date : 2024-11-09 DOI: 10.1093/ced/llae489
Yonit Wohl, Jacob Mashiah, Yarden Drutin, Amir Ben-Tov

Both alopecia areata (AA) and celiac disease are common immune-mediated diseases. Despite accumulating evidence of bi-directional associations between the two disorders, data remains unclear and inconsistent. In this study we explored the association between alopecia areata and celiac disease in a large representative population cohort. Patients with alopecia areata diagnosed during 2005-2019 were compared with age and gender matched healthy controls for celiac autoimmunity. A total of 33,401 patients with alopecia areata and 66,802 controls were included in the analysis. Overall, 754 patients (0.85) had celiac disease. Compared to controls, celiac prevalence increased twofold among AA patients (1.1% Vs 0.6%, Odds ratio (OR) 1.95, 95% Confidence Interval (CI) 1.69-2.25). Higher prevalence rates were observed among all age groups, with the highest risk in AA patients older than 40 years and older. Alopecia areata may be associated with a significant increased risk for celiac disease, suggesting early diagnostic and preventive measures.

斑秃(AA)和乳糜泻都是常见的免疫介导疾病。尽管有越来越多的证据表明这两种疾病之间存在双向关联,但相关数据仍不明确且不一致。在这项研究中,我们在一个大型代表性人群队列中探讨了斑秃与乳糜泻之间的关联。我们将 2005-2019 年期间确诊的斑秃患者与年龄和性别匹配的健康对照组进行了乳糜泻自身免疫性比较。共有 33,401 名秃发症患者和 66,802 名对照者参与了分析。总体而言,754 名患者(0.85%)患有乳糜泻。与对照组相比,AA 患者的乳糜泻发病率增加了两倍(1.1% 对 0.6%,比值比 (OR) 1.95,95% 置信区间 (CI) 1.69-2.25)。所有年龄组的患病率都较高,其中 40 岁以上的 AA 患者患病风险最高。斑秃可能与罹患乳糜泻的风险显著增加有关,建议尽早采取诊断和预防措施。
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引用次数: 0
Multiple purple coloured plaques in a dark skinned Asian paediatric patient. 一名皮肤黝黑的亚裔儿科患者身上出现多处紫色斑块。
IF 3.7 4区 医学 Q1 DERMATOLOGY Pub Date : 2024-11-08 DOI: 10.1093/ced/llae486
Christian Schembri, Yana Marie Dimech, Alexandra Betts, Nathalie Galea, Charles Joseph Borg, Liam Mercieca, Mark Anthony Aquilina, Adriana Warrington
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引用次数: 0
General dermatology and dermatology in primary health care. 普通皮肤病学和初级卫生保健中的皮肤病学。
IF 3.7 4区 医学 Q1 DERMATOLOGY Pub Date : 2024-11-08 DOI: 10.1093/ced/llae488
Tanvi Dev, Aneet Kaur, Chirantan S, Pradeep Kanumilli, Pallavi Pallavi
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引用次数: 0
Latent TB screening without chest x-ray: a single centre experience. 无需胸部 X 光检查的潜伏肺结核筛查:单个中心的经验。
IF 3.7 4区 医学 Q1 DERMATOLOGY Pub Date : 2024-11-08 DOI: 10.1093/ced/llae483
Emily K Pender, Rosalind Hughes, Brian Kirby
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引用次数: 0
Psychocutaneous Medicine: Current Global Understanding and Imperatives for Continuing Medical Education and Training Opportunities: A Systematic Review. 皮肤心理医学:当前全球对继续医学教育和培训机会的理解和要求:系统回顾。
IF 3.7 4区 医学 Q1 DERMATOLOGY Pub Date : 2024-11-08 DOI: 10.1093/ced/llae484
Isabella J Tan, Olivia M Katamanin, Jillian Barry, Mohammad Jafferany

The intersection of dermatology and psychiatry has gained significant attention in recent years. Psychocutaneous disease affect patient population, who have undiagnosed and undertreated ailments related to the body and mind. These patients suffer a reduce quality of life due to knowledge gaps and reduce awareness about psycho dermatology. This review aims to assess the integration of psychopharmacology topics in continuing medical education programs (CME) and its relevance in clinical practice, it's impact on healthcare professionals' quality of care, and existing knowledge gaps among trainees and young dermatologists. To identify data from inception up to December 2023, a systematic literature review and search was conducted following PRISMA guidelines. Pubmed, Cochrane, and Embase databases were searched using predefined terms including "continuing medical education", "dermatology", "psychology", "psychodermatology", "psychocutaneous", "awareness" and "practice patterns". Inclusion criteria comprised studies evaluating CME programs, clinical studies and reviews published in English language. Studies were excluded if they were single case reports. A total of 93 studies were identified and 12 were included in this review following screening, removal of duplicates, and application of inclusion criteria. Studies revealed lower level of confidence among professionals in handling psycho cutaneous conditions, limited awareness of available resources and high interest in CME programs. Initiatives like video mentoring and clinical skills lessons showed promise but are currently scarce. Examined data from selected studies demonstrate that current CME programs in psychodermatology lack depth, leaving practitioners feeling ill equipped and unaware of available resources. There is substantial global interest in enhancing these programs. Given the breadth of conditions covered, educating dermatologists is crucial for enhanced patient outcomes and satisfaction. Addressing resource scarcity and patient stigma is imperative to develop specialized programs. Improvement to education can drive stronger outcomes and holistic patient care. Future efforts should prioritize refining program strategies and conducting thorough assessments to maximize impact.

近年来,皮肤病学和精神病学的交叉研究获得了极大关注。皮肤精神疾病影响着那些与身体和精神有关的疾病得不到诊断和治疗的患者群体。由于对精神皮肤病的认识不足和意识淡薄,这些患者的生活质量下降。本综述旨在评估继续医学教育项目(CME)中精神药理学主题的整合情况及其与临床实践的相关性、对医护人员护理质量的影响,以及受训者和年轻皮肤科医生现有的知识差距。为了确定从开始到 2023 年 12 月的数据,我们按照 PRISMA 指南进行了系统的文献综述和检索。使用预先定义的术语(包括 "继续医学教育"、"皮肤病学"、"心理学"、"皮肤心理学"、"皮肤心理学"、"意识 "和 "实践模式")对 Pubmed、Cochrane 和 Embase 数据库进行了检索。纳入标准包括以英语发表的继续医学教育项目评估研究、临床研究和综述。如果研究为单一病例报告,则排除在外。经过筛选、去除重复内容并应用纳入标准后,共确定了 93 项研究,其中 12 项被纳入本综述。研究显示,专业人员对处理皮肤心理疾病的信心水平较低,对可用资源的认识有限,对继续医学教育项目的兴趣较高。视频指导和临床技能课程等举措显示了前景,但目前还很少见。从部分研究中获得的数据表明,目前的精神皮肤病学继续医学教育项目缺乏深度,使从业人员感到装备不足,并且不了解可用资源。全球都对加强这些项目表现出浓厚的兴趣。由于涉及的病症范围广泛,教育皮肤科医生对于提高患者疗效和满意度至关重要。解决资源稀缺和患者污名化问题是发展专业项目的当务之急。改善教育可提高疗效,为患者提供全面护理。未来的工作应优先考虑完善计划策略和进行全面评估,以最大限度地发挥影响。
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引用次数: 0
Preliminary assessment of the diagnostic accuracy of cutaneous T-cell lymphoma through deep sequencing of T-cell receptor gamma gene. 通过T细胞受体γ基因深度测序初步评估皮肤T细胞淋巴瘤的诊断准确性。
IF 3.7 4区 医学 Q1 DERMATOLOGY Pub Date : 2024-11-06 DOI: 10.1093/ced/llae413
Jin-Bon Hong, Tyng-Shiuan Hsieh, Tsen-Fang Tsai, Jau-Yu Liau, Hsien-Ching Chiu, Tung-Lung Lee, Tai-Chung Huang

Background: The diagnostic challenges in early mycosis fungoides (MF) and other cutaneous T-cell lymphomas (CTCL) persist despite advancements in molecular methods.

Aim: The study aims to provide a preliminary assessment of next-generation sequencing in analyzing T-cell receptor gamma (TRG) sequences for distinguishing CTCL from benign inflammatory disorders.

Methods: Skin samples from CTCL and benign inflammatory skin disorders proven clinicopathologically were assessed for TRG by NGS.

Results: Our study analyzed skin samples from a total of 36 subjects, comprising 22 cases of CTCL, including 14 MF and 8 other CTCLs, alongside 14 cases of benign inflammatory skin disorders. According to the LymphoTrack criteria, monoclonality was detected in 75.0 % of the overall 24 CTCL patients. Specifically, in MF cases, 10 out of 14 were identified as monoclonality, with all four non-monoclonal cases being in the patch stage. For the other CTCL, 6 out of 8 cases displayed monoclonality. Among the overall 22 CTCL patients, 11 had multiple biopsies, with 9 displaying the same dominant clone across different sites. Among the 14 benign cases, only the case with erythrodermic psoriasis exhibited monoclonality. Our decision tree analysis suggests that a high frequency of the most abundant clone, its ratio to the third most abundant clone, and TRG V-I segment usage are effective markers aiding in diagnosing CTCL.

Conclusion: Combination of the clone frequencies, and TRG V segment usage may enhance diagnosis for MF and other CTCLs, aiding in differentiating them from benign conditions. However, molecular diagnosis for patch-stage MF remains challenging.

背景:目的:本研究旨在对下一代测序在分析T细胞受体γ(TRG)序列以区分CTCL和良性炎症性疾病方面的应用进行初步评估:通过 NGS 评估经临床病理证实的 CTCL 和良性炎症性皮肤病的皮肤样本中的 TRG:我们的研究共分析了 36 例受试者的皮肤样本,其中包括 22 例 CTCL(包括 14 例 MF 和 8 例其他 CTCL)以及 14 例良性皮肤炎症性疾病。根据 LymphoTrack 标准,在全部 24 例 CTCL 患者中,75.0% 的患者被检测出单克隆性。具体来说,在中耳炎病例中,14 例中有 10 例被确定为单克隆病例,其中 4 例非单克隆病例均处于斑块期。在其他 CTCL 病例中,8 例中有 6 例表现为单克隆性。在所有 22 例 CTCL 患者中,有 11 例进行了多次活检,其中 9 例在不同部位显示出相同的优势克隆。在 14 例良性病例中,只有红皮病型银屑病病例表现出单克隆性。我们的决策树分析表明,最大量克隆的高频率、其与第三大量克隆的比率以及TRG V-I段的使用是诊断CTCL的有效标记:结论:结合克隆频率和TRG V段的使用可提高对中性粒细胞白血病和其他CTCL的诊断率,有助于将它们与良性疾病区分开来。然而,斑块期 MF 的分子诊断仍具有挑战性。
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Clinical and Experimental Dermatology
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