Pub Date : 2024-08-10eCollection Date: 2024-10-01DOI: 10.1002/ski2.436
Inês P Amaral, Ivânia Soares, Madalena P Correia, Catarina Correia, Pedro Vasconcelos, Luis S Almeida, Paulo Filipe
Cylindromas are rare, benign adnexal neoplasms primarily found on the scalp and face, with a higher incidence in women and typically manifesting in the second or third decade of life. These tumours can present as single or multiple nodules, with the latter form often linked to CYLD gene mutations, particularly in the context of CYLD cutaneous syndrome. Here, we report a case of a 61-year-old male presenting with multiple cylindromas of the scalp, prompting genetic testing that revealed a novel pathogenic variant in the CYLD gene.
{"title":"Novel CYLD pathogenic variant associated with multiple cylindromas of the scalp-A case report.","authors":"Inês P Amaral, Ivânia Soares, Madalena P Correia, Catarina Correia, Pedro Vasconcelos, Luis S Almeida, Paulo Filipe","doi":"10.1002/ski2.436","DOIUrl":"10.1002/ski2.436","url":null,"abstract":"<p><p>Cylindromas are rare, benign adnexal neoplasms primarily found on the scalp and face, with a higher incidence in women and typically manifesting in the second or third decade of life. These tumours can present as single or multiple nodules, with the latter form often linked to CYLD gene mutations, particularly in the context of CYLD cutaneous syndrome. Here, we report a case of a 61-year-old male presenting with multiple cylindromas of the scalp, prompting genetic testing that revealed a novel pathogenic variant in the CYLD gene.</p>","PeriodicalId":74804,"journal":{"name":"Skin health and disease","volume":"4 5","pages":"e436"},"PeriodicalIF":0.0,"publicationDate":"2024-08-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11442051/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142362542","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Tyler T. Nguyen, Stephanie M. Cohen, Katharina S. Shaw, Fatma Dedeoglu, R. A. Vleugels, Ingrid M. Ganske
Diagnosis of craniofacial morphea (CM) relies upon clinical examination of progressive craniofacial changes. We assess the utility of 3D stereophotogrammetry in documenting asymmetry of the face compared to clinical notetaking. This retrospective study of 3D images and clinical documentation included 32 patients (mean age 15.7 years) with CM. A panel of specialists identified additional areas of asymmetry (those highlighted in 3D photographs that were not noted in clinical documentation) and categorised them as likely, ambiguously or unlikely related to CM. 28 patients (87.5%) had asymmetries noted on 3D photos that were not documented in clinical notes. In 46.4% of them, additional areas were deemed consistent with CM. In the remaining patients, additional asymmetries were ambiguous (42.9%) or not thought to be related to CM (10.7%). Our results suggest that adjunctive use of 3D stereophotogrammetry enhances the documentation of CM at discrete clinical time points and therefore could be a better comparative reference during later re‐examination.
颅面病变(CM)的诊断依赖于颅面进行性变化的临床检查。与临床记录相比,我们评估了三维立体摄影测量在记录面部不对称方面的实用性。这项三维图像和临床记录的回顾性研究包括 32 名 CM 患者(平均年龄 15.7 岁)。专家小组确定了额外的不对称区域(三维照片中突出显示但临床记录中未注意到的区域),并将其分为可能、模糊或不可能与 CM 有关。有 28 名患者(87.5%)在三维照片中发现了不对称的部位,但临床记录中并未提及。其中 46.4% 的患者的其他部位被认为与 CM 一致。在其余患者中,额外的不对称部位不明确(42.9%)或被认为与 CM 无关(10.7%)。我们的研究结果表明,辅助使用三维立体摄影测量法可增强在离散临床时间点上对 CM 的记录,因此可作为日后复查时更好的对比参考。
{"title":"Comparison of 3D facial photographs and clinical documentation in patients with craniofacial morphea","authors":"Tyler T. Nguyen, Stephanie M. Cohen, Katharina S. Shaw, Fatma Dedeoglu, R. A. Vleugels, Ingrid M. Ganske","doi":"10.1002/ski2.438","DOIUrl":"https://doi.org/10.1002/ski2.438","url":null,"abstract":"Diagnosis of craniofacial morphea (CM) relies upon clinical examination of progressive craniofacial changes. We assess the utility of 3D stereophotogrammetry in documenting asymmetry of the face compared to clinical notetaking. This retrospective study of 3D images and clinical documentation included 32 patients (mean age 15.7 years) with CM. A panel of specialists identified additional areas of asymmetry (those highlighted in 3D photographs that were not noted in clinical documentation) and categorised them as likely, ambiguously or unlikely related to CM. 28 patients (87.5%) had asymmetries noted on 3D photos that were not documented in clinical notes. In 46.4% of them, additional areas were deemed consistent with CM. In the remaining patients, additional asymmetries were ambiguous (42.9%) or not thought to be related to CM (10.7%). Our results suggest that adjunctive use of 3D stereophotogrammetry enhances the documentation of CM at discrete clinical time points and therefore could be a better comparative reference during later re‐examination.","PeriodicalId":74804,"journal":{"name":"Skin health and disease","volume":"5 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-08-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141920118","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}
Rory Sheppard, W. K. Gan, Gladys L. Onambele‐Pearson, Helen S. Young
Patients with psoriasis are less physically active compared to age‐matched controls, due to psoriasis‐specific barriers, which significantly limits their ability to benefit from health‐promoting levels of physical activity (PA). In addition, long‐term health outcomes for people with psoriasis are poor and include depression, metabolic syndrome and cardiovascular disease (CVD); presenting a significant challenge to healthcare services.We designed a PA intervention in partnership with patients with psoriasis hypothesising this may have therapeutic utility in the management of psoriasis.Participants with chronic plaque psoriasis were recruited to a single‐centre, 20‐week, prospective cohort study. A wrist‐worn accelerometer (GENEActiv Original; Activinsights Ltd) and a hip‐worn pedometer (Onwalk 900; Decathlon Group) were used objectively measure levels of PA. Our 10‐week PA intervention comprised twice weekly 60‐min walks within three different greenspaces in Greater Manchester, each led by a Sports and Exercise Scientist to deliver a pre‐specified volume/dose of activity. During weeks‐11–20 of the study, participants followed independent activities. Clinical evaluation, including assessment of psoriasis severity, cardiometabolic parameters, psychological wellbeing and functional capacity was made at baseline, week‐10 and ‐20.Sixteen patients with psoriasis completed the study. We observed significantly reduced Psoriasis Area and Severity Index at week‐10 (p = 0.01) and ‐20 (p = 0.001) compared to baseline, with 50% of participants achieving PASI‐50 at week‐20. Dermatology Life Quality Index (DLQI) was significantly reduced at week‐20 (p = 0.04), compared to baseline. Significant reduction in blood pressure at week‐10 (systolic: −7.4 mmHg, p = 0.002; diastolic: −4.2 mmHg, p = 0.03) and ‐20 (systolic: −8.8 mmHg, p = 0.001; diastolic: 4.1 mmHg, p = 0.008) was observed and pulse wave velocity was significantly reduced by week‐20 (p = 0.02), suggesting improvement in cardiovascular health. Despite high prevalence of anxiety and depression at baseline, we documented a significant improvement in wellbeing and psychological health. Functional capacity was significantly enhanced following completion of the study.Increasing PA constitutes a promising therapeutic intervention in the management of psoriasis. Evaluation of our intervention in a clinical trial would help determine clinical utility and establish PA guidelines for patients with psoriasis.
{"title":"Increased physical activity promotes skin clearance, improves cardiovascular and psychological health, and increases functional capacity in patients with psoriasis","authors":"Rory Sheppard, W. K. Gan, Gladys L. Onambele‐Pearson, Helen S. Young","doi":"10.1002/ski2.426","DOIUrl":"https://doi.org/10.1002/ski2.426","url":null,"abstract":"Patients with psoriasis are less physically active compared to age‐matched controls, due to psoriasis‐specific barriers, which significantly limits their ability to benefit from health‐promoting levels of physical activity (PA). In addition, long‐term health outcomes for people with psoriasis are poor and include depression, metabolic syndrome and cardiovascular disease (CVD); presenting a significant challenge to healthcare services.We designed a PA intervention in partnership with patients with psoriasis hypothesising this may have therapeutic utility in the management of psoriasis.Participants with chronic plaque psoriasis were recruited to a single‐centre, 20‐week, prospective cohort study. A wrist‐worn accelerometer (GENEActiv Original; Activinsights Ltd) and a hip‐worn pedometer (Onwalk 900; Decathlon Group) were used objectively measure levels of PA. Our 10‐week PA intervention comprised twice weekly 60‐min walks within three different greenspaces in Greater Manchester, each led by a Sports and Exercise Scientist to deliver a pre‐specified volume/dose of activity. During weeks‐11–20 of the study, participants followed independent activities. Clinical evaluation, including assessment of psoriasis severity, cardiometabolic parameters, psychological wellbeing and functional capacity was made at baseline, week‐10 and ‐20.Sixteen patients with psoriasis completed the study. We observed significantly reduced Psoriasis Area and Severity Index at week‐10 (p = 0.01) and ‐20 (p = 0.001) compared to baseline, with 50% of participants achieving PASI‐50 at week‐20. Dermatology Life Quality Index (DLQI) was significantly reduced at week‐20 (p = 0.04), compared to baseline. Significant reduction in blood pressure at week‐10 (systolic: −7.4 mmHg, p = 0.002; diastolic: −4.2 mmHg, p = 0.03) and ‐20 (systolic: −8.8 mmHg, p = 0.001; diastolic: 4.1 mmHg, p = 0.008) was observed and pulse wave velocity was significantly reduced by week‐20 (p = 0.02), suggesting improvement in cardiovascular health. Despite high prevalence of anxiety and depression at baseline, we documented a significant improvement in wellbeing and psychological health. Functional capacity was significantly enhanced following completion of the study.Increasing PA constitutes a promising therapeutic intervention in the management of psoriasis. Evaluation of our intervention in a clinical trial would help determine clinical utility and establish PA guidelines for patients with psoriasis.","PeriodicalId":74804,"journal":{"name":"Skin health and disease","volume":"30 12","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-08-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141923109","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}
Pub Date : 2024-08-07eCollection Date: 2024-12-01DOI: 10.1002/ski2.404
John E Harris, Amit G Pandya, Mark Lebwohl, Iltefat H Hamzavi, Pearl Grimes, Alice B Gottlieb, Howard L Sofen, Angela Y Moore, Mingyue Wang, Deanna Kornacki, Kathleen Butler, David Rosmarin
{"title":"Safety and efficacy of ruxolitinib cream for the treatment of vitiligo: A randomised controlled trial secondary analysis at 3 years.","authors":"John E Harris, Amit G Pandya, Mark Lebwohl, Iltefat H Hamzavi, Pearl Grimes, Alice B Gottlieb, Howard L Sofen, Angela Y Moore, Mingyue Wang, Deanna Kornacki, Kathleen Butler, David Rosmarin","doi":"10.1002/ski2.404","DOIUrl":"https://doi.org/10.1002/ski2.404","url":null,"abstract":"","PeriodicalId":74804,"journal":{"name":"Skin health and disease","volume":"4 6","pages":"e404"},"PeriodicalIF":0.0,"publicationDate":"2024-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11608864/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142775321","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-08-07eCollection Date: 2024-12-01DOI: 10.1002/ski2.432
Hamisha Salih, Cristina Psomadakis, Susannah M C George
Sunscreens are topical formulations incorporating filters that protect our skin against ultraviolet radiation (UVR) emitted by the sun. Sunscreen use has been increasingly encouraged to protect against sunburn, skin cancer and photoaging that can occur because of prolonged and cumulative sun exposure. However, sunscreens and their constituent UVR filters have been purported to be problematic themselves. In this narrative review, we will describe the history of sunscreens, types of UVR filters and how sunscreens are classified and rated. We will also explore some of the controversies regarding sunscreens, including concerns about their safety and environmental impact. Awareness of these potential consequences is paramount to the process of informed decision-making.
{"title":"Sunscreens: A narrative review.","authors":"Hamisha Salih, Cristina Psomadakis, Susannah M C George","doi":"10.1002/ski2.432","DOIUrl":"https://doi.org/10.1002/ski2.432","url":null,"abstract":"<p><p>Sunscreens are topical formulations incorporating filters that protect our skin against ultraviolet radiation (UVR) emitted by the sun. Sunscreen use has been increasingly encouraged to protect against sunburn, skin cancer and photoaging that can occur because of prolonged and cumulative sun exposure. However, sunscreens and their constituent UVR filters have been purported to be problematic themselves. In this narrative review, we will describe the history of sunscreens, types of UVR filters and how sunscreens are classified and rated. We will also explore some of the controversies regarding sunscreens, including concerns about their safety and environmental impact. Awareness of these potential consequences is paramount to the process of informed decision-making.</p>","PeriodicalId":74804,"journal":{"name":"Skin health and disease","volume":"4 6","pages":"e432"},"PeriodicalIF":0.0,"publicationDate":"2024-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11608887/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142775331","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-08-07eCollection Date: 2024-12-01DOI: 10.1002/ski2.437
Ivan Rodriguez, Karen Lam, Simone Montgomery, Shanice McKenzie, Scott Worswick
Acute cutaneous presentations in immunocompromised patients demand careful and thorough evaluation. Here, we present the case of a 26-year-old female with acute myeloid leukaemia undergoing salvage chemotherapy, who developed an acutely expanding subcutaneous mass starting on the frontotemporal scalp to the angle of the jaw. Histopathologic evaluation was consistent with sclerosing fat necrosis with overlying psoriasiform spongiotic dermatitis. Tissue cultures revealed multi-drug resistant Pseudomonas aeruginosa. Given these findings, the patient was diagnosed with infectious panniculitis. This case underscores the necessity of comprehensive diagnostic approaches and multidisciplinary collaboration in managing unusual cutaneous presentations in immunocompromised individuals.
{"title":"Expanding subcutaneous mass on the scalp of an immunocompromised woman.","authors":"Ivan Rodriguez, Karen Lam, Simone Montgomery, Shanice McKenzie, Scott Worswick","doi":"10.1002/ski2.437","DOIUrl":"https://doi.org/10.1002/ski2.437","url":null,"abstract":"<p><p>Acute cutaneous presentations in immunocompromised patients demand careful and thorough evaluation. Here, we present the case of a 26-year-old female with acute myeloid leukaemia undergoing salvage chemotherapy, who developed an acutely expanding subcutaneous mass starting on the frontotemporal scalp to the angle of the jaw. Histopathologic evaluation was consistent with sclerosing fat necrosis with overlying psoriasiform spongiotic dermatitis. Tissue cultures revealed multi-drug resistant Pseudomonas aeruginosa. Given these findings, the patient was diagnosed with infectious panniculitis. This case underscores the necessity of comprehensive diagnostic approaches and multidisciplinary collaboration in managing unusual cutaneous presentations in immunocompromised individuals.</p>","PeriodicalId":74804,"journal":{"name":"Skin health and disease","volume":"4 6","pages":"e437"},"PeriodicalIF":0.0,"publicationDate":"2024-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11608867/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142775378","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-08-05eCollection Date: 2024-10-01DOI: 10.1002/ski2.433
Emmanuel Edwar Siddig, Ayman Ahmed
Botryomycosis of the tongue is a rare chronic bacterial infection that presents as nodular masses, mimicking other infectious or neoplastic conditions such as mycetoma. A case of an 80-year-old male was presented with painless swelling on the right lateral side of his tongue to the outpatient clinic. Biopsy and microbiological investigations revealed an unexpected Staphylococcus aureus-related botryomycosis. This case highlights the diagnostic challenge for unusual clinical presentations of bacterial infections. Healthcare providers in countries endemic with diseases that manifest similarly should investigate thoroughly to ensure a positive clinical outcome through early diagnosis and effective case management.
{"title":"A diagnostic challenge of tongue botryomycosis miming mycetoma-A case report.","authors":"Emmanuel Edwar Siddig, Ayman Ahmed","doi":"10.1002/ski2.433","DOIUrl":"10.1002/ski2.433","url":null,"abstract":"<p><p>Botryomycosis of the tongue is a rare chronic bacterial infection that presents as nodular masses, mimicking other infectious or neoplastic conditions such as mycetoma. A case of an 80-year-old male was presented with painless swelling on the right lateral side of his tongue to the outpatient clinic. Biopsy and microbiological investigations revealed an unexpected <i>Staphylococcus aureus</i>-related botryomycosis. This case highlights the diagnostic challenge for unusual clinical presentations of bacterial infections. Healthcare providers in countries endemic with diseases that manifest similarly should investigate thoroughly to ensure a positive clinical outcome through early diagnosis and effective case management.</p>","PeriodicalId":74804,"journal":{"name":"Skin health and disease","volume":"4 5","pages":"e433"},"PeriodicalIF":0.0,"publicationDate":"2024-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11442049/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142362518","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
A 32-year-old male presented with diffuse plaques accompanied by multiple large, painful swellings predominantly distributed over the trunk, face and both upper and lower extremities for 11 years. There was no family history of similar complaints. The histopathological examination (HPE), supplemented with special stains confirmed the diagnosis of cutaneous leiomyoma (CL). The immunohistochemical analysis showed a strong expression of smooth muscle actin. The final diagnosis of sporadic diffuse multiple cutaneous leiomyomatosis was made. This presentation of CL is uncommon and resembled plexiform neurofibroma. The clinical diagnosis of this presentation was challenging. Therefore, HPE combined with special stains aided in confirming this surgically challenging rare tumour. In resource-poor settings regular screening is required for early detection of renal cancer and other associated complications.
{"title":"A sporadic diffuse multiple cutaneous leiomyomatosis mimicking plexiform neurofibromatosis: A surgical challenge.","authors":"Gajanand M Antakanavar, Bijaylaxmi Sahoo, Aneet Kaur, Ishu Ghiloria, Ajay Jangid, Bhavishya Shetty","doi":"10.1002/ski2.414","DOIUrl":"10.1002/ski2.414","url":null,"abstract":"<p><p>A 32-year-old male presented with diffuse plaques accompanied by multiple large, painful swellings predominantly distributed over the trunk, face and both upper and lower extremities for 11 years. There was no family history of similar complaints. The histopathological examination (HPE), supplemented with special stains confirmed the diagnosis of cutaneous leiomyoma (CL). The immunohistochemical analysis showed a strong expression of smooth muscle actin. The final diagnosis of sporadic diffuse multiple cutaneous leiomyomatosis was made. This presentation of CL is uncommon and resembled plexiform neurofibroma. The clinical diagnosis of this presentation was challenging. Therefore, HPE combined with special stains aided in confirming this surgically challenging rare tumour. In resource-poor settings regular screening is required for early detection of renal cancer and other associated complications.</p>","PeriodicalId":74804,"journal":{"name":"Skin health and disease","volume":"4 5","pages":"e414"},"PeriodicalIF":0.0,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11442042/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142362519","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Angry back","authors":"R.W.D. Ottevanger, Roel E. Genders","doi":"10.1002/ski2.428","DOIUrl":"https://doi.org/10.1002/ski2.428","url":null,"abstract":"","PeriodicalId":74804,"journal":{"name":"Skin health and disease","volume":"29 25","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141806021","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}
G. Pathak, Kush Patel, Christopher Wachuku, T. Truong, Priya Agarwal, Babar K. Rao
Pemphigus represents a spectrum of autoimmune‐mediated blistering diseases associated with high morbidity, mortality and reduced quality of life (QoL). Despite an increase in pemphigus clinical trials, the varied instrument measurements of disease severity and QoL outcomes make comparisons between studies challenging. This study aimed to evaluate trends in the use of disease severity and QoL outcome measurements in pemphigus clinical trials. A review of pemphigus clinical trials was conducted using the PubMed, Embase, Cochrane Reviews and ClinicalTrials.gov databases up until September 2023. Only pemphigus randomized clinical trials that assessed at least one disease severity and/or QoL outcome were included. Overall, 53 clinical trials were eligible for this review. All clinical trials evaluated a disease severity outcome, with the Pemphigus Disease Area Index being the most used validated questionnaire (28.3% of trials) and more popular after 2015 (47.8% of trials since). The autoimmune bullous skin disorder intensity score (7.6%) and visual analogue measurements (7.6%) have fallen out of favour. Most studies now include lab parameters (56.5% of trials after 2015), with anti‐desmoglein 1 and 3 antibody levels (30.2%), immunoglobulins (IgG and/or IgM and IgA) (11.3%), and anti‐drug antibody levels (7.6%) being frequently evaluated. A small portion of trials evaluated QoL (26.5% of studies), with the autoimmune bullous quality of life being the most common (15.1%), however QoL utilization as an outcome measure has been increasing since 2015 (61.1% of trials since). Standardising the use of validated outcome measurements allows for better data interpretation, comparability and clinical application of results.
丘疹性荨麻疹是一种由自身免疫介导的水疱性疾病,具有发病率高、死亡率高和生活质量(QoL)下降的特点。尽管丘疹性荨麻疹的临床试验越来越多,但由于疾病严重程度和 QoL 结果的测量工具各不相同,因此对不同研究进行比较具有挑战性。本研究旨在评估丘疹性荨麻疹临床试验中疾病严重程度和 QoL 结果测量方法的使用趋势。我们使用 PubMed、Embase、Cochrane Reviews 和 ClinicalTrials.gov 数据库对截至 2023 年 9 月的丘疹性荨麻疹临床试验进行了回顾。仅纳入了评估至少一种疾病严重程度和/或 QoL 结果的丘疹性荨麻疹随机临床试验。共有 53 项临床试验符合本综述的要求。所有临床试验都评估了疾病严重程度结果,其中丘疹性荨麻疹疾病面积指数是使用最多的有效问卷(占试验的28.3%),在2015年后更受欢迎(占此后试验的47.8%)。自身免疫性大疱性皮肤病强度评分(7.6%)和视觉模拟测量(7.6%)已不再受欢迎。目前,大多数研究都包括实验室参数(2015年后的试验占56.5%),其中抗去疱疹蛋白1和3抗体水平(30.2%)、免疫球蛋白(IgG和/或IgM和IgA)(11.3%)和抗药物抗体水平(7.6%)是经常评估的项目。一小部分试验对生活质量进行了评估(占研究的26.5%),其中自身免疫性牛皮癣患者的生活质量是最常见的(15.1%),但自2015年以来,将生活质量作为结果测量指标的情况一直在增加(自2015年以来占试验的61.1%)。规范使用经过验证的结果测量指标可以更好地解释数据,提高结果的可比性和临床应用。
{"title":"Trends in disease severity and quality of life outcome measures in pemphigus clinical trials: A scoping review","authors":"G. Pathak, Kush Patel, Christopher Wachuku, T. Truong, Priya Agarwal, Babar K. Rao","doi":"10.1002/ski2.429","DOIUrl":"https://doi.org/10.1002/ski2.429","url":null,"abstract":"Pemphigus represents a spectrum of autoimmune‐mediated blistering diseases associated with high morbidity, mortality and reduced quality of life (QoL). Despite an increase in pemphigus clinical trials, the varied instrument measurements of disease severity and QoL outcomes make comparisons between studies challenging. This study aimed to evaluate trends in the use of disease severity and QoL outcome measurements in pemphigus clinical trials. A review of pemphigus clinical trials was conducted using the PubMed, Embase, Cochrane Reviews and ClinicalTrials.gov databases up until September 2023. Only pemphigus randomized clinical trials that assessed at least one disease severity and/or QoL outcome were included. Overall, 53 clinical trials were eligible for this review. All clinical trials evaluated a disease severity outcome, with the Pemphigus Disease Area Index being the most used validated questionnaire (28.3% of trials) and more popular after 2015 (47.8% of trials since). The autoimmune bullous skin disorder intensity score (7.6%) and visual analogue measurements (7.6%) have fallen out of favour. Most studies now include lab parameters (56.5% of trials after 2015), with anti‐desmoglein 1 and 3 antibody levels (30.2%), immunoglobulins (IgG and/or IgM and IgA) (11.3%), and anti‐drug antibody levels (7.6%) being frequently evaluated. A small portion of trials evaluated QoL (26.5% of studies), with the autoimmune bullous quality of life being the most common (15.1%), however QoL utilization as an outcome measure has been increasing since 2015 (61.1% of trials since). Standardising the use of validated outcome measurements allows for better data interpretation, comparability and clinical application of results.","PeriodicalId":74804,"journal":{"name":"Skin health and disease","volume":"58 8","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141807091","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}