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Clinician's Ability to Identify Non-Melanoma Skin Cancer on 3D-Total Body Photography Sectors that Were Initially Identified during In-Person Skin Examination with Dermoscopy. 临床医生在3D至Metal身体摄影部门识别非羊毛瘤皮肤癌症的能力,这些部门最初是在使用皮肤镜进行的住院皮肤检查中识别的。
IF 3.4 3区 医学 Q2 DERMATOLOGY Pub Date : 2024-01-01 Epub Date: 2023-11-06 DOI: 10.1159/000535031
Sarah Hobelsberger, Julian Steininger, Jörg Laske, Katja Berndt, Friedegund Meier, Stefan Beissert, Frank Friedrich Gellrich

Introduction: Non-melanoma skin cancer (NMSC) is a cause of significant morbidity and mortality in high-risk individuals. Total body photography (TBP) is currently used to monitor melanocytic lesions in patients with high risk for melanoma. The authors examined if three-dimensional (3D)-TBP could be useful for diagnosis of NMSC.

Methods: Patients (n = 129; 52 female, 77 male) with lesions suspicious for NMSC who had not yet had a biopsy underwent clinical examination followed by examination of each lesion with 3D-TBP Vectra®WB360 (Canfield Scientific, Parsippany, NJ, USA) and dermoscopy.

Results: The 129 patients had a total of 182 lesions. Histological examination was performed for 158 lesions; the diagnoses included basal cell carcinoma (BCC; n = 107), squamous cell carcinoma (SCC; n = 27), in-situ SCC (n = 15). Lesions were located in the head/neck region (n = 138), trunk (n = 21), and limbs (n = 23). Of the 182 lesions examined, 12 were not visible on 3D-TBP; reasons for not being visible included location under hair and on septal of nose. Two lesions appeared only as erythema in 3D-TBP but were clearly identifiable on conventional photographs. Sensitivity of 3D-TBP was lower than that of dermoscopy for BCC (73% vs. 79%, p = 0.327), higher for SCC (81% vs. 74%, p = 0.727), and lower for in-situ SCC (0% vs. 33%, p = 125). Specificity of 3D-TBP was lower than that of dermoscopy for BCC (77% vs. 82%, 0.581), lower for SCC (75% vs. 84%, p = 0.063), and higher for in-situ SCC (97% vs. 94%, p = 0.344). Diagnostic accuracy of 3D-TBP was lower than that of dermoscopy for BCC (75% vs. 80%), lower for SCC (76% vs. 82%), and lower for in-situ SCC (88% vs. 89%). Lesion location was not associated with diagnostic confidence in dermoscopy (p = 0.152) or 3D-TBP (p = 0.353). If only lesions with high confidence were included in the calculation, diagnostic accuracy increased for BCC (n = 27; sensitivity 85%, specificity 85%, diagnostic accuracy 85%), SCC (n = 10; sensitivity 90%, specificity 80%, diagnostic accuracy 83%), and for in-situ SCC (n = 2; sensitivity 0%, specificity 100%, diagnostic accuracy 95%).

Conclusion: Diagnostic accuracy appears to be slightly lower for 3D-TBP in comparison to dermoscopy. However, there is no statistically significant difference in the sensitivity and specificity of 3D-TBP and dermoscopy for NMSC. Diagnostic accuracy increases, if only lesions with high confidence are included in the calculation. Further studies are necessary to determine if 3D-TBP can improve management of NMSC.

简介:癌症(NMSC)是高风险人群中发病率和死亡率较高的原因。全身摄影(TBP)目前用于监测黑色素瘤高危患者的黑色素细胞病变。作者检查了三维(3D)-TBP是否有助于诊断NMSC。方法:对尚未进行活检的可疑NMSC病变患者(n=129;52名女性,77名男性)进行临床检查,然后用3D-TBP(Vectra®WB360(Canfield Scientific,Parsippany,NJ,USA)和皮镜检查每个病变。结果:129例患者共有182个病灶。对158个病灶进行了组织学检查;诊断包括基底细胞癌(BCC;n=107)、鳞状细胞癌(SCC;n=27)、原位SCC(n=15)。病变位于头部/颈部(n=138)、躯干(n=21)和四肢(n=23)。在182个检查的病变中,12个在3D-TBP上不可见;不可见的原因包括位于头发下面和鼻中隔上。两处病变在3D-TBP中仅表现为红斑,但在常规照片上可清楚识别。3D-TBP对基底细胞癌的敏感性低于皮肤镜检查(73%对79%,p=0.327),对SCC的敏感性高于皮肤镜(81%对74%,p=0.727),对原位SCC的敏感性较低(0%对33%,p=125)。3D-TBP对基底细胞癌的特异性低于皮镜检查(77%vs.82%,0.581),对SCC的特异性较低(75%vs.84%,p=0.063),对原位SCC的特异性较高(97%vs.94%,p=0.344)。病变位置与皮镜检查(p=0.152)或3D-TBP(p=0.353)的诊断置信度无关。如果仅将高置信度的病变纳入计算,则BCC的诊断准确度增加(n=27;敏感性85%,特异性85%,诊断准确度85%),SCC(n=10;敏感性90%,特异性80%,诊断准确率83%)和原位SCC(n=2;敏感性0%,特异性100%,诊断准确度95%)。结论:与皮肤镜检查相比,3D-TBP的诊断准确率似乎略低。然而,3D-TBP和皮肤镜检查对NMSC的敏感性和特异性没有统计学上的显著差异。如果计算中只包括具有高置信度的病变,则诊断准确性会提高。需要进一步的研究来确定3D-TBP是否可以改善NMSC的管理。
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引用次数: 0
Rare MED12L Variants Are Associated with Susceptibility to Guttate Psoriasis in the Han Chinese Population. 罕见的 MED12L 变异与中国汉族人群肠槽型银屑病的易感性有关。
IF 3 3区 医学 Q2 DERMATOLOGY Pub Date : 2024-01-01 Epub Date: 2024-05-10 DOI: 10.1159/000538805
Kejia Wu, Wanrong Wang, Qianhui Cheng, Duncheng Xiao, Yunxiao Li, Mengyun Chen, Xiaodong Zheng

Introduction: According to the common disease/rare variant hypothesis, it is important to study the role of rare variants in complex diseases. The association of rare variants with psoriasis has been demonstrated, but the association between rare variants and specific clinical subtypes of psoriasis has not been investigated.

Methods: Gene-based and gene-level meta-analyses were performed on data extracted from our previous study data sets (2,483 patients with guttate psoriasis and 8,292 patients with non-guttate psoriasis) for genotyping. Then, haplotype analysis was performed for rare loss-of-function variants located in MED12L, and protein function prediction was performed for MED12L. Gene-based analysis at each stage had a moderate significance threshold (p < 0.05). A χ2 test was then conducted on the three potential genes, and the merged gene-based analysis was used to confirm the results. We also conducted association analysis and meta-analysis for functional variants located on the identified gene.

Results: Through these gene-level analyses, we determined that MED12L is a guttate psoriasis susceptibility gene (p = 9.99 × 10-5), and the single-nucleotide polymorphism with the strongest association was rs199780529 (p_combine = 1 × 10-3, p_meta = 2 × 10-3).

Conclusions: In our study, a guttate psoriasis-specific subtype-associated susceptibility gene was confirmed in a Chinese Han population. These findings contribute to a better genetic understanding of different subtypes of psoriasis.

简介根据常见疾病/罕见变异(CDRV)假说,研究罕见变异在复杂疾病中的作用非常重要。罕见变异与银屑病的关系已经得到证实,但罕见变异与银屑病特定临床亚型之间的关系尚未得到研究:方法: 从我们之前的研究数据集(2,483 名鳞屑型银屑病患者和 8,292 名非鳞屑型银屑病患者)中提取的数据进行基因分型,并在此基础上进行基因水平的荟萃分析。然后,对位于 MED12L 的罕见功能缺失变异进行了单倍型分析,并对 MED12L 进行了蛋白质功能预测。每个阶段的基因分析都有一个中等显著性阈值(P < 0.05)。然后对三个潜在基因进行卡方检验,并利用合并的基于基因的分析来确认结果。我们还对已确定基因上的功能变异进行了关联分析和荟萃分析:通过这些基因水平的分析,我们确定 MED12L 是肠槽型银屑病的易感基因(P=9.99x10-5),而关联性最强的单核苷酸多态性(SNP)是 rs199780529(P_combine=1x10-3,P_meta=2x10-3):结论:我们的研究在中国汉族人群中证实了一种肠槽型银屑病特异亚型相关易感基因。这些发现有助于更好地从遗传学角度理解银屑病的不同亚型。
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引用次数: 0
Tattoo-Associated Skin Reactions: A Danish Population-Based Survey in 5,914 Tattooed Individuals. 与纹身有关的皮肤反应:一项针对 5914 名纹身者的丹麦人口调查。
IF 3.4 3区 医学 Q2 DERMATOLOGY Pub Date : 2024-01-01 Epub Date: 2023-12-11 DOI: 10.1159/000535536
Karina Friis, Anne Marie Ladehoff Thomsen, Jørn Olsen, Mikael Rahbek Rørth, Jørgen Serup

Background: In Europe, Australia, and the USA, the estimated overall prevalence of tattooing is around 10-20%. Tattoo ink often comprises harmful chemicals and epidemiological studies on adverse effects of tattoos are lacking.

Objectives: We aimed to estimate the prevalence of tattoo-associated skin reactions in the general Danish population and describe individuals with tattoo-associated skin reactions by socio-demographic factors and tattoo characteristics.

Methods: The study was based on respondents aged 16 years or older from a population-based 2021 survey entitled "How are you?" conducted in the Central Denmark Region (n = 33,925). Logistic regression was used to characterise individuals with tattoo-associated skin reactions by socio-demographic factors (gender, age, educational level, and ethnic background). Also, the relationship between size, age and colour of the tattoo, and tattoo-associated skin reactions was studied. Model 1 was adjusted for all socio-demographic variables (gender, age, educational level, and ethnic background); model 2, for all socio-demographic variables and tattoo characteristics (size, age, and colour).

Results: In total, 21.1% reported that they had at least one tattoo, 10.2% hereof reported that they had experienced tattoo-associated skin reactions (itching, pain, inflammation, and swelling) beyond the first 3 weeks after the tattoo was made. Lower age (16-44 years) (adjusted odds ratio (AOR) ≥1.75), larger tattoos (AOR ≥1.61) and having had tattoos for more than 10 years (AOR = 2.92, 95% confidence interval 1.45-5.88) increased the odds of tattoo-associated skin reactions. In general, tattooed individuals with colours other than black had higher odds of tattoo-associated skin reactions.

Conclusion: Among participants with at least one tattoo, 10.2% had experienced tattoo-associated skin reactions beyond the first 3 weeks after their tattoo was made. This finding highlights the need for safer tattoo inks to prevent the adverse health problems experienced by many individuals with tattoos.

背景 在欧洲、澳大利亚和美国,纹身的总体流行率估计约为 10-20%。纹身墨水通常含有有害化学物质,而有关纹身不良影响的流行病学研究却十分缺乏。目的 我们旨在估算纹身相关皮肤反应在丹麦普通人群中的流行率,并根据社会人口因素和纹身特征描述纹身相关皮肤反应患者的情况。方法 该研究以丹麦中部地区 2021 年进行的一项名为 "您好吗?"的人口调查中 16 岁或以上的受访者(n = 33,925 人)为基础。研究采用逻辑回归法,根据社会人口学因素(性别、年龄、教育水平和种族背景)对有纹身相关皮肤反应的个体进行特征描述。此外,还研究了纹身的大小、年龄和颜色与纹身相关皮肤反应之间的关系。模型 1 对所有社会人口变量(性别、年龄、教育水平和种族背景)进行了调整;模型 2 对所有社会人口变量和纹身特征(大小、年龄和颜色)进行了调整。结果 共有 21.1%的人表示他们至少有一个纹身,其中 10.2%的人表示在纹身后的头三周内出现过与纹身相关的皮肤反应(瘙痒、疼痛、发炎和肿胀)。年龄越小(16-44 岁)(调整赔率 (AOR) ≥ 1.75)、纹身面积越大(AOR ≥ 1.61)、纹身时间超过十年(AOR = 2.92,95% 置信区间 (CI) 1.45-5.88),发生纹身相关皮肤反应的几率就越大。一般来说,除黑色外,其他颜色的纹身者发生纹身相关皮肤反应的几率更高。结论 在至少有一处纹身的参与者中,10.2%的人在纹身后的头三周内出现过纹身相关皮肤反应。这一发现突出表明,有必要使用更安全的纹身墨水,以防止许多纹身者出现不良健康问题。
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引用次数: 0
The Agreement between Consumer-Driven Self-Assessment of Psoriasis Severity and Physician-Assessed Severity Based on Patient-Taken Photographs Is Weak: A Cross-Sectional Study. 消费者对银屑病严重程度的自我评估与医生根据患者拍摄的照片对严重程度的评估之间的一致性较弱:横断面研究。
IF 3.4 3区 医学 Q2 DERMATOLOGY Pub Date : 2024-01-01 Epub Date: 2024-01-10 DOI: 10.1159/000536175
Zarqa Ali, Ali Al-Mousawi, Benóný Þór Björnsson, Alexander Egeberg, Christian Riemer, Simon Francis Thomsen

Introduction: Digital advancements have given access to huge amounts of real-world data (RWD) widely used for dermatological research.

Objectives: The objective of this study was to investigate the agreement between consumer-driven self-assessed psoriasis severity and physician-assessed severity based on photographs.

Methods: Customer IDs in the NØIE database (Danish skincare company) from 2009 to 2022 with a smartphone photograph of psoriasis vulgaris on the body and a corresponding completed questionnaire were included. Smartphone photographs were evaluated by a physician-assessing erythema, induration, and scaling on a scale from 0 to 4 based on Psoriasis Area Severity Index (PASI). Self-assessment was done on a scale from 0 to 10 and converted to 0-4 scale (0 converted to 0; 1-3 to 1; 4-6 to 2; 7-8 to 3; and 9-10 to 4). Intraclass correlation coefficients with 95% confidence intervals (CIs) were calculated.

Results: In total, 187 patients (63% women) with mean age of 38 years were included. Self-assessment scores were higher than physicians' assessment scores for all groups, and scaling was closest to the physicians' assessment, while erythema and induration had a greater distance between the physicians' and patients' assessment. The correlation between self-assessed and physician-assessed psoriasis severity for all patients was 0.23 (95% CI: 0.0-0.92); 0.34 (95% CI: 0.0-0.95) for chronic patients; and 0.09 (-0.01 to 0.82) for non-chronic patients. The agreement was better for men (0.53 [-0.02 to 0.98]) than for women (0.12 [-0.01 to 0.84]).

Conclusion: There was weak agreement between self-assessed psoriasis severity and photographically assessed severity by the physician. Consumer-driven RWD should be interpreted with caution.

简介:数字技术的进步使人们能够获得大量真实世界数据(RWD),这些数据被广泛用于皮肤病研究:数字技术的进步使人们可以获得大量真实世界数据(RWD),这些数据被广泛用于皮肤病研究:调查消费者自我评估的银屑病严重程度与医生根据照片评估的严重程度之间的一致性:方法:纳入 Nøie 数据库(丹麦护肤品公司)中 2009 年至 2022 年使用智能手机拍摄的身体寻常型银屑病照片和相应填写的调查问卷的客户 ID。智能手机照片由一名医生进行评估,根据银屑病面积严重程度指数(PASI),以 0-4 分来评估红斑、压痕和鳞屑。自我评估采用 0-10 分制,并转换为 0-4 分制(0 分制转换为 0;1-3 分制转换为 1;4-6 分制转换为 2;7-8 分制转换为 3;9-10 分制转换为 4)。计算了带 95% 置信区间 (CI) 的类内相关系数 (ICC):共纳入 187 名患者(63% 为女性),平均年龄为 38 岁。在所有组别中,自我评估得分均高于医生评估得分,鳞屑与医生评估最接近,而红斑和压痕则与医生和患者的评估相差较大。所有患者的自我评估与医生评估的银屑病严重程度之间的相关性为 0.23(95% CI 0.0-0.92);慢性患者的相关性为 0.34(95% CI 0.0-0.95),非慢性患者的相关性为 0.09(-0.01-0.82)。男性的一致性为 0.53(-0.02-0.98),高于女性的 0.12(-0.01-0.84):结论:自我评估的银屑病严重程度与医生通过照片评估的严重程度之间的一致性较弱。消费者驱动的 RWD 应谨慎解释。
{"title":"The Agreement between Consumer-Driven Self-Assessment of Psoriasis Severity and Physician-Assessed Severity Based on Patient-Taken Photographs Is Weak: A Cross-Sectional Study.","authors":"Zarqa Ali, Ali Al-Mousawi, Benóný Þór Björnsson, Alexander Egeberg, Christian Riemer, Simon Francis Thomsen","doi":"10.1159/000536175","DOIUrl":"10.1159/000536175","url":null,"abstract":"<p><strong>Introduction: </strong>Digital advancements have given access to huge amounts of real-world data (RWD) widely used for dermatological research.</p><p><strong>Objectives: </strong>The objective of this study was to investigate the agreement between consumer-driven self-assessed psoriasis severity and physician-assessed severity based on photographs.</p><p><strong>Methods: </strong>Customer IDs in the NØIE database (Danish skincare company) from 2009 to 2022 with a smartphone photograph of psoriasis vulgaris on the body and a corresponding completed questionnaire were included. Smartphone photographs were evaluated by a physician-assessing erythema, induration, and scaling on a scale from 0 to 4 based on Psoriasis Area Severity Index (PASI). Self-assessment was done on a scale from 0 to 10 and converted to 0-4 scale (0 converted to 0; 1-3 to 1; 4-6 to 2; 7-8 to 3; and 9-10 to 4). Intraclass correlation coefficients with 95% confidence intervals (CIs) were calculated.</p><p><strong>Results: </strong>In total, 187 patients (63% women) with mean age of 38 years were included. Self-assessment scores were higher than physicians' assessment scores for all groups, and scaling was closest to the physicians' assessment, while erythema and induration had a greater distance between the physicians' and patients' assessment. The correlation between self-assessed and physician-assessed psoriasis severity for all patients was 0.23 (95% CI: 0.0-0.92); 0.34 (95% CI: 0.0-0.95) for chronic patients; and 0.09 (-0.01 to 0.82) for non-chronic patients. The agreement was better for men (0.53 [-0.02 to 0.98]) than for women (0.12 [-0.01 to 0.84]).</p><p><strong>Conclusion: </strong>There was weak agreement between self-assessed psoriasis severity and photographically assessed severity by the physician. Consumer-driven RWD should be interpreted with caution.</p>","PeriodicalId":11185,"journal":{"name":"Dermatology","volume":" ","pages":"362-368"},"PeriodicalIF":3.4,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11168445/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139416648","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Dermatofibrosarcoma Protuberans: A Study of 148 Patients. 原发性皮肤纤维肉瘤。对 148 名患者的研究。
IF 3 3区 医学 Q2 DERMATOLOGY Pub Date : 2024-01-01 Epub Date: 2024-01-16 DOI: 10.1159/000536172
Joaquim Marcoval, Carlos Moreno-Vílchez, Clara Torrecilla-Vall-Llosera, Clara Muntaner-Virgili, Diana Pérez Sidelnikova, Xavier Sanjuán, Rosa Maria Penín

Introduction: Dermatofibrosarcoma protuberans (DFSP) is the most common sarcoma of the skin. Although distant metastases are infrequent, DFSP is highly aggressive locally with frequent local recurrences. It has been reported that the presence within the tumour of areas histopathologically mimicking fibrosarcoma may increase the risk of recurrence.

Objective: The objective of this study was to review the clinical features of our patients with DFSP and the factors associated with recurrence of the tumour, focussing on the presence of fibrosarcomatous areas.

Methods: Retrospective study of patients with DFSP diagnosed in 1990-2021 in a tertiary university hospital. The medical records were reviewed to obtain the following data: age, sex, tumour location, diameter, evolution time, presence of fibrosarcomatous areas, development of recurrence, and follow-up. Factors possibly associated with disease-free survival were analysed with Kaplan-Meier method and multivariate Cox regression.

Results: 148 patients (74 women/74 men, mean age 46.28 years, SD 14.431) were included in the study. Tumours involved the head and neck in 15 cases, thorax in 31, abdomen in 16, upper back in 43, lower back in 10, upper extremities in 10, and lower extremities in 23. Fibrosarcoma-like areas were observed in 16 tumours (10.81%). In 17 patients (11.49%), recurrences were observed (13 local recurrences, 3 lung metastasis, and 1 local recurrence with lung metastasis). Fibrosarcomatous DFSP recurred more frequently than classic DFSP (50% vs. 6.82%, respectively), and its disease-free survival was significantly lower (p < 0.001). In multivariate Cox regression, the presence of fibrosarcomatous areas was the only factor influencing disease-free survival.

Conclusions: It is important to identify the fibrosarcomatous variant since it recurs more frequently and has lower recurrence-free survival. Distant metastases, mainly in the lung, are also more frequent in fibrosarcomatous DFSP.

简介皮纤维肉瘤(DFSP)是最常见的皮肤肉瘤。虽然远处转移并不常见,但皮纤维肉瘤在局部的侵袭性很强,经常会出现局部复发。有报道称,肿瘤内存在组织病理学上类似纤维肉瘤的区域可能会增加复发风险:回顾 DFSP 患者的临床特征以及与肿瘤复发相关的因素,重点关注纤维肉瘤区域的存在:方法:对一家三级大学医院1990-2021年确诊的DFSP患者进行回顾性研究。回顾病历以获取以下数据:年龄、性别、肿瘤位置、直径、演变时间、是否存在纤维肉瘤区、复发情况和随访情况。采用卡普兰-梅耶法和多变量考克斯回归法分析了可能与无病生存率相关的因素:研究共纳入 148 名患者(74 名女性/74 名男性,平均年龄 46.28 岁,SD 14.431)。肿瘤累及头颈部15例,胸部31例,腹部16例,上背部43例,下背部10例,上肢10例,下肢23例。在 16 例肿瘤(10.81%)中观察到纤维肉瘤样区域。在 17 名患者(11.49%)中观察到复发(13 例局部复发,3 例肺转移,1 例局部复发伴肺转移)。纤维肉瘤型 DFSP 的复发率高于典型 DFSP(分别为 50%和 6.82%),且无病生存率明显较低(p结论:鉴别纤维肉瘤变体非常重要,因为其复发率更高,无复发生存率更低。纤维肉瘤型DFSP的远处转移(主要在肺部)也更为常见。
{"title":"Dermatofibrosarcoma Protuberans: A Study of 148 Patients.","authors":"Joaquim Marcoval, Carlos Moreno-Vílchez, Clara Torrecilla-Vall-Llosera, Clara Muntaner-Virgili, Diana Pérez Sidelnikova, Xavier Sanjuán, Rosa Maria Penín","doi":"10.1159/000536172","DOIUrl":"10.1159/000536172","url":null,"abstract":"<p><strong>Introduction: </strong>Dermatofibrosarcoma protuberans (DFSP) is the most common sarcoma of the skin. Although distant metastases are infrequent, DFSP is highly aggressive locally with frequent local recurrences. It has been reported that the presence within the tumour of areas histopathologically mimicking fibrosarcoma may increase the risk of recurrence.</p><p><strong>Objective: </strong>The objective of this study was to review the clinical features of our patients with DFSP and the factors associated with recurrence of the tumour, focussing on the presence of fibrosarcomatous areas.</p><p><strong>Methods: </strong>Retrospective study of patients with DFSP diagnosed in 1990-2021 in a tertiary university hospital. The medical records were reviewed to obtain the following data: age, sex, tumour location, diameter, evolution time, presence of fibrosarcomatous areas, development of recurrence, and follow-up. Factors possibly associated with disease-free survival were analysed with Kaplan-Meier method and multivariate Cox regression.</p><p><strong>Results: </strong>148 patients (74 women/74 men, mean age 46.28 years, SD 14.431) were included in the study. Tumours involved the head and neck in 15 cases, thorax in 31, abdomen in 16, upper back in 43, lower back in 10, upper extremities in 10, and lower extremities in 23. Fibrosarcoma-like areas were observed in 16 tumours (10.81%). In 17 patients (11.49%), recurrences were observed (13 local recurrences, 3 lung metastasis, and 1 local recurrence with lung metastasis). Fibrosarcomatous DFSP recurred more frequently than classic DFSP (50% vs. 6.82%, respectively), and its disease-free survival was significantly lower (p &lt; 0.001). In multivariate Cox regression, the presence of fibrosarcomatous areas was the only factor influencing disease-free survival.</p><p><strong>Conclusions: </strong>It is important to identify the fibrosarcomatous variant since it recurs more frequently and has lower recurrence-free survival. Distant metastases, mainly in the lung, are also more frequent in fibrosarcomatous DFSP.</p>","PeriodicalId":11185,"journal":{"name":"Dermatology","volume":" ","pages":"487-493"},"PeriodicalIF":3.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11168446/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139477903","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
A Non-Interventional, Multicenter Study to Characterize the Socio-Demographics, Clinical Characteristics, and Management of Generalized Pustular Psoriasis Patients in Spain: IMPULSE Study. 一项非干预性多中心研究,旨在了解西班牙泛发性脓疱型银屑病 (GPP) 患者的社会人口统计学特征、临床特征和管理情况:IMPULSE 研究。
IF 3 3区 医学 Q2 DERMATOLOGY Pub Date : 2024-01-01 Epub Date: 2024-07-17 DOI: 10.1159/000540019
Lluís Puig, Rosa Izu Belloso, Raquel Rivera-Díaz, Jordi Mollet Sánchez, Lourdes Rodríguez Fernández-Freire, Antonio Sahuquillo-Torralba, Ricardo Ruiz-Villaverde

Introduction: Generalized pustular psoriasis (GPP) is a chronic, rare, and potentially life-threatening skin condition characterized by flares comprising widespread sterile pustules and systemic inflammation. Both the rarity and heterogeneity of the disease have made GPP classification and standardization of clinical criteria challenging. Before the approval of spesolimab (IL-36R antibody) in 2022, there were no approved treatments in the USA or Europe for GPP flares. Treatment for GPP has amounted to off-label use of medicines approved to treat plaque psoriasis. Our aim was to describe the sociodemographics, clinical characteristics, and treatment patterns of patients with GPP in Spain.

Methods: Non-interventional, descriptive, multi-center, retrospective chart review of patients diagnosed with GPP in Spain.

Results: 56 patients (50% women) were included, with a mean (standard deviation, SD) age at diagnosis of 53.7 (20.5) and a mean (SD) time of follow-up of 3.7 (3.1) years. In 80% of patients, GPP diagnosis was associated with a flare and 67.3% had known risk factors for GPP (such as previous diagnosis or family history of plaque psoriasis, comorbidities, smoking or stress). Hypertension and plaque psoriasis were the most frequent comorbidities (44.6% each). The number of GPP flares per patient-year was 0.55 with (range 0-4) a mean (SD) body surface area involvement of 21.3% (19.1). The most frequent manifestations of GPP flares were pustules (88.5%), erythema (76.9%), and scaling (76.9%). Additionally, 65.4% of patients had plaque psoriasis, 53.8% had unspecified skin lesions, and 30.8% experienced pain. The treatments used for GPP flares were off-label conventional systemic drugs (75%), mostly corticosteroids, cyclosporine, and acitretin. In the periods between flares, off-label biologics were used in 56.5% of patients. During the study period, 9 patients (16.1%) had at least one complication and 5 of them required hospitalization.

Conclusion: This is the first multicenter study in Spanish GPP patients. Most patients were in their fifties, with personal or family history of plaque psoriasis, stress, smoking and a wide range of comorbidities and complications. Even though the number of flares per patient/year was 0.55, there was variability between patients. Both off-label conventional systemics and off-label biologics were used for flare management without a clear treatment pattern.

背景:泛发性脓疱型银屑病(GPP)是一种慢性、罕见且可能危及生命的皮肤病,其特征是由广泛的无菌脓疱和全身炎症组成的复发。这种疾病的罕见性和异质性使 GPP 的分类和临床标准的标准化面临挑战。在 2022 年斯派索利单抗(IL-36R 抗体)获批之前,美国和欧洲都没有获批治疗 GPP 病发的药物。对 GPP 的治疗相当于在标签外使用已批准用于治疗斑块状银屑病的药物。我们的目的是描述西班牙GPP患者的社会人口学、临床特征和治疗模式:方法:对西班牙确诊的 GPP 患者进行非干预性、描述性、多中心、回顾性病历审查:共纳入 56 名患者(50% 为女性),确诊时的平均(标清)年龄为 53.7(20.5)岁,平均(标清)随访时间为 3.7(3.1)年。在 80% 的患者中,GPP 的诊断与复发有关,67.3% 的患者有 GPP 的已知风险因素(如既往诊断或斑块状银屑病家族史、合并症、吸烟或压力)。高血压和斑块状银屑病是最常见的合并症(各占 44.6%)。每名患者每年的 GPP 复发次数为 0.55 次(0-4 次不等),平均(标清)受累体表面积为 21.3% (19.1)。GPP复发的最常见表现是脓疱(88.5%)、红斑(76.9%)和脱屑(76.9%)。此外,65.4%的患者有斑块状银屑病,53.8%的患者有不明皮损,30.8%的患者有疼痛感。GPP复发时使用的治疗方法是标签外常规全身用药(75%),主要是皮质类固醇激素、环孢素和阿西曲汀。在两次复发之间,56.5%的患者使用了标签外生物制剂。在研究期间,9 名患者(16.1%)至少出现了一种并发症,其中 5 人需要住院治疗:这是首个针对西班牙GPP患者的多中心研究。大多数患者年龄在 50 岁左右,有斑块状银屑病个人或家族史,精神压力大,吸烟,有多种合并症和并发症。尽管每位患者每年复发的次数为 0.55 次,但不同患者的情况各不相同。标示外的传统系统药物和标示外的生物制剂都被用于治疗复发,但没有明确的治疗模式。
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引用次数: 0
Associations of Digital Ulcers in Patients with Systemic Sclerosis: An 8-Year Retrospective Study. 系统性硬化症患者数字溃疡的相关性:一项为期 8 年的回顾性研究。
IF 3.4 3区 医学 Q2 DERMATOLOGY Pub Date : 2024-01-01 Epub Date: 2024-03-21 DOI: 10.1159/000536030
Miaomiao Chi, Qi An, Xiuyuan Feng, Lan He, Ying Pan

Introduction: This study aimed to investigate the associations of digital ulcers (DUs) in patients with systemic sclerosis (SSc).

Methods: This retrospective study investigated the demographic characteristics, specific autoantibodies, organ involvement, and laboratory tests in patients with SSc from our hospital.

Results: This study enrolled 144 patients with SSc. The DU+ group consisted of 15 (10.4%) patients. Patients with SSc having DUs have longer disease duration, higher fibrinogen, higher fibrin degradation product, and lower cholesterol. None of the patients used cholesterol-lowering drugs before onset of DUs. The study also demonstrated a higher prevalence of anti-dsDNA and anti-histone antibodies in patients with SSc with DUs. Anti-dsDNA antibody is a specific antibody for SLE with a specificity of 96-99%. A total of 86.1% (124/144) of patients suffered from diffuse cutaneous SSc, and 28.5% (41/144) of patients suffered from overlap syndrome.

Conclusion: Our study indicated that patients with SSc with fibrinogen of >2.895 g/L (p = 0.043) and cholesterol of <3.340 mmol/L (p = 0.036), which is equal to 129.258 mg/dL, are at high risk of developing DUs.

导言本研究旨在探讨系统性硬化症(SSc)患者数字溃疡(DUs)的相关性:这项回顾性研究调查了我院SSc患者的人口统计学特征、特异性自身抗体、器官受累情况和实验室检查结果:本研究共纳入 144 名 SSc 患者。DU+组有15名患者(10.4%)。有DU的SSc患者病程较长,纤维蛋白原较高,纤维蛋白降解产物较高,胆固醇较低。这些患者在出现 DUs 前均未使用降胆固醇药物。该研究还表明,在有DUs的SSc患者中,抗dsDNA抗体和抗组蛋白抗体的发病率较高。抗dsDNA抗体是系统性红斑狼疮的特异性抗体,特异性高达96%-99%。共有86.1%(124/144)的患者患有弥漫性皮肤SSc,28.5%(41/144)的患者患有重叠综合征:我们的研究表明,纤维蛋白原>2.895 g/L (P = 0.043)和胆固醇>2.895 g/L (P = 0.043)的SSc患者,其血浆中的纤维蛋白原和胆固醇含量均高于正常人。
{"title":"Associations of Digital Ulcers in Patients with Systemic Sclerosis: An 8-Year Retrospective Study.","authors":"Miaomiao Chi, Qi An, Xiuyuan Feng, Lan He, Ying Pan","doi":"10.1159/000536030","DOIUrl":"10.1159/000536030","url":null,"abstract":"<p><strong>Introduction: </strong>This study aimed to investigate the associations of digital ulcers (DUs) in patients with systemic sclerosis (SSc).</p><p><strong>Methods: </strong>This retrospective study investigated the demographic characteristics, specific autoantibodies, organ involvement, and laboratory tests in patients with SSc from our hospital.</p><p><strong>Results: </strong>This study enrolled 144 patients with SSc. The DU+ group consisted of 15 (10.4%) patients. Patients with SSc having DUs have longer disease duration, higher fibrinogen, higher fibrin degradation product, and lower cholesterol. None of the patients used cholesterol-lowering drugs before onset of DUs. The study also demonstrated a higher prevalence of anti-dsDNA and anti-histone antibodies in patients with SSc with DUs. Anti-dsDNA antibody is a specific antibody for SLE with a specificity of 96-99%. A total of 86.1% (124/144) of patients suffered from diffuse cutaneous SSc, and 28.5% (41/144) of patients suffered from overlap syndrome.</p><p><strong>Conclusion: </strong>Our study indicated that patients with SSc with fibrinogen of &gt;2.895 g/L (p = 0.043) and cholesterol of &lt;3.340 mmol/L (p = 0.036), which is equal to 129.258 mg/dL, are at high risk of developing DUs.</p>","PeriodicalId":11185,"journal":{"name":"Dermatology","volume":" ","pages":"387-396"},"PeriodicalIF":3.4,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140038936","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Dermoscopy of Lentiginous Melanomas and Equivocal Benign Pigmented Macules of the Scalp: A Case-Control Multicentric Study. 皮肤镜检查透镜状黑色素瘤和模棱两可的良性头皮色素斑:一项病例对照多中心研究。
IF 3.4 3区 医学 Q2 DERMATOLOGY Pub Date : 2024-01-01 Epub Date: 2023-11-30 DOI: 10.1159/000535030
Amanda Regio Pereira, Sergio Hirata, Paweł Pietkiewicz, Scott W Menzies, Gabriella Brancaccio, Helena Collgros, Giuseppe Argenziano, Serigne N Lo, Tasnia Ahmed, Riccardo Pampena, Caterina Longo, Pascale Guitera

Introduction: Although the dermoscopic features of facial lentiginous melanomas (LM), including lentigo maligna and lentigo maligna melanoma, have been extensively studied, the literature about those located on the scalp is scarce. This study aims to describe the dermoscopic features of scalp LM and assess the diagnostic accuracy of dermoscopy to discriminate them from equivocal benign pigmented macules.

Methods: Consecutive cases of scalp LM and histopathology-proven benign but clinically equivocal pigmented macules (actinic keratoses, solar lentigos, seborrhoeic keratoses, and lichen planus-like keratoses) from four referral centres were included. Dermoscopic features were analysed by two blinded experts. The diagnostic performance of a predictive model was assessed.

Results: 56 LM and 44 controls were included. Multiple features previously described for facial and extrafacial LM were frequently identified in both groups. Expert's sensitivity to diagnose scalp LM was 76.8% (63.6-87.0) and 78.6% (65.6-88.4), with specificity of 54.5% (38.9-69.6) and 56.8% (41.0-71.7), and fair agreement (kappa coefficient 0.248). The strongest independent predictors of malignancy were (OR, 95% CI) chaos of colour (15.43, 1.48-160.3), pigmented reticular lines (14.96, 1.68-132.9), increased density of vascular network (3.45, 1.09-10.92), and perifollicular grey circles (2.89, 0.96-8.67). The predictive model achieved 85.7% (73.8-93.6) sensitivity, 61.4% (45.5-75.6) specificity, and 81.5 (73.0-90.0) area under curve to discriminate benign and malignant lesions. A diagnostic flowchart was proposed, which should improve the diagnostic performance of dermoscopy.

Conclusion: Both facial and extrafacial dermoscopic patterns can be identified in scalp LM, with considerable overlap with benign pigmented macules, leading to low specificity and interobserver agreement on dermoscopy.

虽然面部黄斑黑色素瘤(LM)的皮肤镜特征已被广泛研究,包括恶性黄斑和恶性黄斑黑色素瘤,但关于其位于头皮的文献很少。本研究旨在描述头皮LM的皮肤镜特征,并评估皮肤镜诊断的准确性,以区分它们与模棱两可的良性色素斑。方法:连续4个转诊中心的头皮LM和组织病理学证实的良性但临床上模棱两可的色素斑(光化性角化病、太阳色斑、脂溢性角化病和扁平苔藓样角化病)病例。两位盲法专家对皮肤镜特征进行了分析。评估了预测模型的诊断性能。结果:LM 56例,对照组44例。先前描述的面部和面外LM的多种特征在两组中都经常被发现。专家诊断头皮LM的敏感性分别为76.8%(63.6 ~ 87.0)和78.6%(65.6 ~ 88.4),特异性分别为54.5%(38.9 ~ 69.6)和56.8%(41.0 ~ 71.7),一致性较好(kappa系数0.248)。恶性肿瘤最强的独立预测因子是(OR, 95%CI)颜色混乱(15.43,1.48-160.3);色素网状线(14.96,1.68-132.9),血管网密度增加(3.45,1.09-10.92),滤泡周围灰圈(2.89,0.96-8.67)。预测模型区分良恶性病变的敏感性为85.7%(73.8 ~ 93.6),特异性为61.4%(45.5 ~ 75.6),曲线下面积为81.5(73.0 ~ 90.0)。提出了一种诊断流程,以提高皮肤镜的诊断性能。结论:头皮LM的面部和面外皮肤镜模式均可识别,与良性色素斑有相当大的重叠,导致皮肤镜下特异性较低,观察者之间的一致性较差。
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引用次数: 0
Viewpoint on the Evaluation of Severity and Treatment Effects in Mild Hidradenitis Suppurativa: The Cumulative IHS4. 关于轻度化脓性扁平苔癣严重程度和治疗效果评估的观点:累积性 IHS4 (IHS4-C)。
IF 3 3区 医学 Q2 DERMATOLOGY Pub Date : 2024-01-01 Epub Date: 2023-12-20 DOI: 10.1159/000535867
Hessel H van der Zee, Johanna C van Huijstee, Kelsey R van Straalen, Gregor B E Jemec, Christos C Zouboulis, Thrasyvoulos Tzellos, Errol P Prens
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引用次数: 0
Mortality and Autopsy Findings in Patients with Pyoderma Gangrenosum: A Multi-Institutional Series. 脓皮病患者的死亡率和尸检结果:多机构系列研究
IF 3.4 3区 医学 Q2 DERMATOLOGY Pub Date : 2024-01-01 Epub Date: 2024-01-06 DOI: 10.1159/000536145
Hadir Shakshouk, Mallory deCampos-Stairiker, Shannon Kody, Peter Stenzel, Eric Xia, Arash Mostaghimi, Bicong Wu, Michi M Shinohara, Angelo V Marzano, Samantha Polly, Anthony P Fernandez, Alex G Ortega-Loayza

Introduction: Pyoderma gangrenosum (PG) is a rare ulcerative skin condition with an increased risk of mortality compared to the general population. The causes of this increased risk are not well understood. Misdiagnosis is common in PG, and many studies are limited by the inclusion of misdiagnosed cases. The goal of this study was to review autopsy findings, identify causes of death, and identify factors that may worsen outcomes among deceased patients confirmed to have PG.

Methods: Data was retrospectively reviewed from the electronic medical records at five academic hospitals. A search was conducted for deceased patients with a diagnosis of PG who had an autopsy performed between 2010 and 2020. We report a descriptive analysis of 11 patients and their clinical characteristics, causes of death, and autopsy findings.

Results: The average age of death was 62.9 years. Seven patients had at least one underlying condition known to be associated with PG including inflammatory bowel disease, inflammatory arthritis, or a hematologic disorder. The most common cause of death was infection (n = 6, 54.5%), followed by pulmonary embolism (n = 3, 27.3%), and myelodysplastic syndrome (n = 2, 18.2%). Six patients (54.5%) were taking systemic steroids at the time of death.

Conclusion: The development of PG may shorten life expectancy among those with underlying conditions associated with PG, and common treatments for PG may contribute to the risk of fatal complications. Awareness of the risk of infection, thrombosis, and malignancy among those with PG is necessary for proper management. Further research is needed to explore the relationship between PG and thromboembolism.

简介坏疽性脓皮病(PG)是一种罕见的溃疡性皮肤病,与普通人群相比,其死亡风险更高。造成这种风险增加的原因尚不十分清楚。误诊在脓皮病中很常见,许多研究都因纳入误诊病例而受到限制。本研究的目的是回顾尸检结果,确定死亡原因,并找出在确认患有脓皮病的死亡患者中可能会恶化预后的因素:方法:对五家学术医院的电子病历数据进行回顾性审查。方法:我们从五家学术医院的电子病历中回顾性地查阅了数据,并搜索了在 2010 年至 2020 年间接受尸检的确诊为 PG 的已故患者。我们报告了对11名患者及其临床特征、死亡原因和尸检结果的描述性分析:平均死亡年龄为 62.9 岁。7名患者至少患有一种已知与PG相关的基础疾病,包括炎症性肠病、炎症性关节炎或血液病。最常见的死因是感染(6例,54.5%),其次是肺栓塞(3例,27.3%)和骨髓增生异常综合征(2例,18.2%)。6名患者(54.5%)死亡时正在服用全身类固醇:结论:PG的发生可能会缩短与PG相关的潜在疾病患者的预期寿命,而PG的常见治疗方法可能会增加致命并发症的风险。为了进行适当的治疗,有必要了解 PG 患者感染、血栓形成和恶性肿瘤的风险。需要进一步研究探讨脓皮病与血栓栓塞之间的关系。
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