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Validity of diagnosis codes to identify a dermatomyositis cohort from electronic health records.
IF 3 3区 医学
Dermatology Pub Date : 2025-01-25 DOI: 10.1159/000543803
Alex Silberzweig, Andrew Strunk, Elizabeth Flatley, Anthony P Fernandez, Amit Garg
{"title":"Validity of diagnosis codes to identify a dermatomyositis cohort from electronic health records.","authors":"Alex Silberzweig, Andrew Strunk, Elizabeth Flatley, Anthony P Fernandez, Amit Garg","doi":"10.1159/000543803","DOIUrl":"https://doi.org/10.1159/000543803","url":null,"abstract":"<p><strong>Introduction: </strong>Dermatomyositis (DM) is an uncommon idiopathic inflammatory myopathy resulting in characteristic patterns of cutaneous lesions and myositis. Observational evidence related to the disease is limited by small case cohorts. We aimed to evaluate the validity of specialist-specific diagnostic coding for DM in an outpatient clinical database.</p><p><strong>Methods: </strong>Adults having an outpatient encounter between January 1, 2010-June 30, 2023 at a United States regional health system with ICD-9/-10 coding for DM were identified. We randomly sampled 156 patients with > 1 code applied by a dermatologist or rheumatologist. The primary reference standard for case adjudication was a confirmed diagnosis of DM by the treating physician in the medical chart. Fulfilment of the 2017 European Alliance of Associations for Rheumatology/American College of Rheumatology (EULAR/ACR) criteria for \"probable\" or \"definite\" DM was used as a secondary, more stringent reference standard. Positive predictive values (PPVs) for several case definitions were calculated with 95% confidence intervals.</p><p><strong>Results: </strong>Among eligible patients, median age was 51.5 years, and 81% were female. Using the treating physician's diagnosis as reference, PPVs of > 1 and > 2 codes applied by a dermatologist were 93.2% (95% CI 82.0%-98.3%) and 96.4% (82.2%-99.8%), respectively. The PPVs of > 1 and > 2 codes from a rheumatologist were 82.0% (77.1%-86.9%) and 85.8% (80.6%-91.1%), respectively. At least one and at least two codes from a rheumatologist or dermatologist had PPVs of 82.1% (77.3%-86.8%) and 85.7% (80.7%-90.8%), respectively. The rate of confirmed cases based on EULAR/ACR criteria ranged from 44.9% to 57.1%.</p><p><strong>Conclusion: </strong>All tested algorithms yielded an accurate case cohort with high PPV. Studies prioritizing sensitivity may use > 1 code by dermatology or rheumatology to identify patients with DM.</p>","PeriodicalId":11185,"journal":{"name":"Dermatology","volume":" ","pages":"1-9"},"PeriodicalIF":3.0,"publicationDate":"2025-01-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143045512","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
Tattoo ink products and pigments 2018-19 upfront the new EUREACH regulation: Danish golden benchmark Study of 108 studios and 39687 clients tattooed with inks from 109720 ink bottles. 纹身油墨产品和颜料2018-19年前期EUREACH新法规:丹麦黄金基准对108个工作室和39687个客户进行了研究,他们使用了109720个墨水瓶中的墨水。
IF 3 3区 医学
Dermatology Pub Date : 2025-01-10 DOI: 10.1159/000543455
Jørgen Vedelskov Serup, Bjørn Christian Severin, Esben Hammershøy
{"title":"Tattoo ink products and pigments 2018-19 upfront the new EUREACH regulation: Danish golden benchmark Study of 108 studios and 39687 clients tattooed with inks from 109720 ink bottles.","authors":"Jørgen Vedelskov Serup, Bjørn Christian Severin, Esben Hammershøy","doi":"10.1159/000543455","DOIUrl":"https://doi.org/10.1159/000543455","url":null,"abstract":"<p><strong>Background: </strong>The new EU regulation on tattoo inks in force January 2022 in a hitherto unregulated market marks a historical change.</p><p><strong>Objective: </strong>Mapping of the thousands of tattoo inks de facto used in studios before the new EU regulation and establish a historical reference to tattoo customer exposure, ink toxicology assessment, clinical complications, and the impact on tattooing businesses.</p><p><strong>Method: </strong>A tattooist-operated electronic system (InkBase) for ink registration required by law is used in Denmark since 2018. A local database in studios refers to a central database. Clients, sessions, ink bottles, brand name, and pigment color index (CI) are registered. Person's data protection is respected. Tracing harmful inks is possible, with public warning.</p><p><strong>Results: </strong>Registrations from 108 studios employing about 700 tattooists were collected from March 2018 through 2019. 39687 clients were tattooed in 50604 sessions, using colors from 109720 ink bottles. 10833 were CI-labelled identifying the pigment. 98.1% of inks originated from USA. Detailed statistics on inks and pigments used are provided as a benchmark showing the spontaneous use and preference of \"old\" tattoo inks before the EU regulation compulsory to member states introduced dramatic restrictions difficult to follow.</p><p><strong>Conclusions: </strong>Denmark can, having detailed ink registration enforced by law and having a commonly used electronic registration system reporting to a central server, function as an index country in future surveillance of use of tattoo inks in studios, toxicology aspects and the impact of regulatory intervention on the tattooing industry, with a large sample of data collected in 2018-19. Wordcount 249.</p>","PeriodicalId":11185,"journal":{"name":"Dermatology","volume":" ","pages":"1-21"},"PeriodicalIF":3.0,"publicationDate":"2025-01-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142970090","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
Development and External Validation of the Hidradenitis Suppurativa Cutaneous Abscess Prediction Score-2 (HSCAPS-2): A Clinical Decision Support Tool for Diagnosis of Hidradenitis Suppurativa over Cutaneous Abscess. 化脓性汗腺炎皮肤脓肿预测评分-2 (HSCAPS-2)的开发和外部验证:化脓性汗腺炎皮肤脓肿诊断的临床决策支持工具。
IF 3 3区 医学
Dermatology Pub Date : 2025-01-09 DOI: 10.1159/000543130
Amit Garg, Jonathan Koptyev, Melissa Butt, Joslyn Kirby, Andrew Strunk
{"title":"Development and External Validation of the Hidradenitis Suppurativa Cutaneous Abscess Prediction Score-2 (HSCAPS-2): A Clinical Decision Support Tool for Diagnosis of Hidradenitis Suppurativa over Cutaneous Abscess.","authors":"Amit Garg, Jonathan Koptyev, Melissa Butt, Joslyn Kirby, Andrew Strunk","doi":"10.1159/000543130","DOIUrl":"https://doi.org/10.1159/000543130","url":null,"abstract":"<p><strong>Introduction: </strong>Patients with hidradenitis suppurativa (HS) experience a 10-year diagnosis delay, on average. Accordingly, time to diagnosis represents one of the greatest unmet needs in HS, which to date has not been adequately addressed. A general lack of awareness about HS in the medical community and a notable heterogeneity in clinical presentation, which is most often confused with cutaneous abscess (CA), forms the basis of poor disease recognition and diagnosis delay. Our objective was to develop and validate a prediction model for diagnosis of HS versus site-specific cutaneous abscess (CA).</p><p><strong>Methods: </strong>We performed a cross-sectional study in which the model was developed using a large clinical database and externally validated using a sample of clinical records at Penn State Health. Prediction model discrimination and calibration were evaluated using the c-statistic, calibration intercept/slope, and a flexible calibration curve.</p><p><strong>Results: </strong>Variable selection identified 15 predictors, 7 of which remained after model simplification. The following characteristics were predictive of HS relative to site-specific CA: female sex, increasing age up to 44 years, African American race, race other than White or African American, increasing body mass index, polycystic ovarian syndrome, and acne. The c-statistics of the 7-variable model in validation was 0.77 (95% CI 0.73-0.80). Calibration intercept and slope were 0.29 (95% CI 0.14, 0.43) and 1.09 (95% CI 0.90, 1.28).</p><p><strong>Conclusion: </strong>Clinical characteristics can predict diagnosis of HS over CA in practice without reliance on a specialty-specific examination to identify disease and potentially reduce diagnosis delay.</p>","PeriodicalId":11185,"journal":{"name":"Dermatology","volume":" ","pages":"1-14"},"PeriodicalIF":3.0,"publicationDate":"2025-01-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142946209","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
Exploring Health Literacy among Adults with Hidradenitis Suppurativa. 探讨成人化脓性汗腺炎患者的健康素养。
IF 3 3区 医学
Dermatology Pub Date : 2025-01-03 DOI: 10.1159/000543286
Nicole D K Koerts, Barbara Horváth
{"title":"Exploring Health Literacy among Adults with Hidradenitis Suppurativa.","authors":"Nicole D K Koerts, Barbara Horváth","doi":"10.1159/000543286","DOIUrl":"https://doi.org/10.1159/000543286","url":null,"abstract":"<p><strong>Background: </strong>Health literacy plays an important role in the management of chronic and debilitating skin diseases like hidradenitis suppurativa (HS). Adequate health literacy empowers patients to understand their disease, manage it effectively, and make informed decisions about their health. Exploring the interplay between health literacy and HS is essential to improve healthcare outcomes in this population.</p><p><strong>Objectives: </strong>This study aims to assess the prevalence of limited health literacy among HS patients and its associated factors which has never been studied before.</p><p><strong>Methods: </strong>In this epidemiological cross-sectional study data were collected through a population-wide survey-based study within the Lifelines Cohort Study in the Netherlands. Health literacy of participants with HS were compared to non-HS controls. Health literacy was measured using six validated questions covering functional, communicative, and critical health literacy. Associations between the characteristics of the HS group and limited health literacy were examined.</p><p><strong>Results: </strong>Out of 56,084 adult respondents, 1,156 participants with HS were identified. The prevalence of limited functional health literacy was 24.5% in the non-HS group and 26.4% in the HS group. Our findings indicate that limited health literacy among HS patients is associated with higher BMI (26.9 vs 25.8) [OR 0.969, 95% CI 0.941-0.998], lower education level (34.5% vs 19.9%) [OR 0.495, 95% CI 0.350-0.701], lower socioeconomic status (-0.68 vs -0.58) [OR 1.194, 95% CI 1.029-1.386]), and more severe disease stage according to the Hurley stage (33.0% vs. 25.8%) [OR 1.400, 95% CI 1.005-1.952].</p><p><strong>Conclusions: </strong>Our study highlights the importance of addressing health literacy in HS patients, given the high prevalence of limited health literacy among this group and associations with higher BMI and more severe disease stage. Limited health literacy may contribute to poorer health outcomes, suboptimal healthcare utilization, elevated healthcare costs, and health disparities. Targeted interventions to improve health literacy could enhance care quality and outcomes for HS patients.</p>","PeriodicalId":11185,"journal":{"name":"Dermatology","volume":" ","pages":"1-20"},"PeriodicalIF":3.0,"publicationDate":"2025-01-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142930877","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
Questionnaire-Based Global Prevalence of Hidradenitis Suppurativa: A Systematic Review and Meta-Analysis. 基于问卷调查的全球化脓性汗腺炎患病率:系统回顾和荟萃分析。
IF 3 3区 医学
Dermatology Pub Date : 2024-12-23 DOI: 10.1159/000537920
Jawad Ahmad Zahid, Mattias A S Henning, Dorra Bouazzi, Gregor B E Jemec
{"title":"Questionnaire-Based Global Prevalence of Hidradenitis Suppurativa: A Systematic Review and Meta-Analysis.","authors":"Jawad Ahmad Zahid, Mattias A S Henning, Dorra Bouazzi, Gregor B E Jemec","doi":"10.1159/000537920","DOIUrl":"https://doi.org/10.1159/000537920","url":null,"abstract":"<p><strong>Background: </strong>Hidradenitis suppurativa (HS), a chronic inflammatory skin disease affecting intertriginous areas, presents a recurring and debilitating challenge. Even though recent efforts have been made to estimate the overall HS prevalence, variations in screening modalities and missing data from the Global South warrant further investigation. Understanding the HS prevalence is crucial for treatment approaches and pathogenesis. Thus, we aimed to estimate a global HS prevalence based on studies using homogeneous validated questions.</p><p><strong>Methods: </strong>This systematic review was prospectively registered on PROSPERO and adhered to Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Searches in PubMed, Embase, and CINAHL were performed on August 9, 2023. Original reports assessing the HS prevalence in adults using the following two questions were included: \"Do you have recurrent boils of the skin?\" and \"Have you for the past 6 months had 2 or more boils/abscesses in any of the below locations: in the axilla, in the groin, around your genitals, on the buttocks, several locations i.e., first the buttocks then the axilla, etc.?\" A pooled prevalence with 95% confidence interval (CI) was calculated with a random-effects model.</p><p><strong>Results: </strong>Eight studies qualified for inclusion, of which the prevalence estimate ranged from 0.7% to 6.4%. The meta-analysis encompassing 49,971 participants revealed a global HS prevalence of 2.5% (95% CI, 1.8-3.5%).</p><p><strong>Conclusion: </strong>This meta-analysis suggests a global HS prevalence of 2.5% using a validated questionnaire. Due to heterogeneity and sparse data from the Global South, this estimate should be interpreted with caution.</p>","PeriodicalId":11185,"journal":{"name":"Dermatology","volume":" ","pages":"1-10"},"PeriodicalIF":3.0,"publicationDate":"2024-12-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142881504","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
The Risk of Hospital Admission for an Acute-Onset Psychiatric Disorder in Adolescents and Adults Treated with Isotretinoin: A French, Nationwide, Population-Based, Case-Time-Control Study. 接受异维甲酸治疗的青少年和成人急性发作精神障碍的住院风险:一项法国全国性、基于人群的病例-时间对照研究
IF 3 3区 医学
Dermatology Pub Date : 2024-12-21 DOI: 10.1159/000542626
Catherine Droitcourt, Sandrine Kerbrat, Maxime Raby, Claire Laurent, David Travers, Frédéric Balusson, Emmanuel Oger, Alain Dupuy
{"title":"The Risk of Hospital Admission for an Acute-Onset Psychiatric Disorder in Adolescents and Adults Treated with Isotretinoin: A French, Nationwide, Population-Based, Case-Time-Control Study.","authors":"Catherine Droitcourt, Sandrine Kerbrat, Maxime Raby, Claire Laurent, David Travers, Frédéric Balusson, Emmanuel Oger, Alain Dupuy","doi":"10.1159/000542626","DOIUrl":"10.1159/000542626","url":null,"abstract":"<p><strong>Introduction: </strong>Oral isotretinoin is the only effective treatment for severe acne without an alternative. Isotretinoin has been linked to the occurrence of acute psychiatric disorders outside suicidal behaviors. There are few large-scale epidemiological studies in this area, and the putative associations are unclear. Our objective was to determine whether adolescents and young adults have an elevated risk of acute-onset psychiatric disorder requiring hospital treatment within 2 months of starting isotretinoin treatment.</p><p><strong>Methods: </strong>Our data source was the French national health insurance database (Système National des Données de Santé, SNDS), 2010-2015. We performed a case-time-control study nested in an exhaustive, nationwide cohort of all French adolescents and young adults aged 10-25 years treated with isotretinoin. The outcome was an acute-onset psychiatric disorder requiring hospitalization (including anxiety, depressive, mood, adjustment, and psychotic disorders). A conditional logistic model was used to estimate odds ratios (ORs) with their 95% confidence interval (CI) for acute psychiatric events.</p><p><strong>Results: </strong>2,284 acute-onset psychiatric disorder requiring hospitalization were recorded for the study population of 262,786 patients. Among the patients with at least one psychiatric event, 88 had started taking isotretinoin in the risk period (0-2 months before the date of the event), versus 81 in the reference period (2-4 months before the event). A comparison with the 383 and 355 time-trend matched controls who started taking isotretinoin in the risk and reference periods, respectively, yielded a case-time-control OR (95% CI) of 1.01 (0.72-1.41).</p><p><strong>Conclusion: </strong>Psychiatric events managed outside the hospital system were not recorded. Our findings are reassuring for clinicians concerning the risk of severe acute-onset psychiatric events after isotretinoin initiation.</p>","PeriodicalId":11185,"journal":{"name":"Dermatology","volume":" ","pages":"1-11"},"PeriodicalIF":3.0,"publicationDate":"2024-12-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142876477","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
Prevalence of Hidradenitis Suppurativa in Xi'an, China: A Single Center Epidemiological Study. 西安市化脓性汗腺炎流行病学单中心研究
IF 3 3区 医学
Dermatology Pub Date : 2024-12-20 DOI: 10.1159/000539019
Danchen Hu, Dorra Bouazzi, Cecilia E Medianfar, Robin Christensen, Gregor B E Jemec, Songmei Geng
{"title":"Prevalence of Hidradenitis Suppurativa in Xi'an, China: A Single Center Epidemiological Study.","authors":"Danchen Hu, Dorra Bouazzi, Cecilia E Medianfar, Robin Christensen, Gregor B E Jemec, Songmei Geng","doi":"10.1159/000539019","DOIUrl":"https://doi.org/10.1159/000539019","url":null,"abstract":"<p><strong>Background: </strong>Hidradenitis suppurativa (HS), also known as acne inversa, is a chronic, recurrent, and inflammatory disease. HS is fairly rare among the population. This study was a part of the Global Hidradenitis Suppurativa Atlas (GHiSA) initiative, aiming to establish the prevalence of HS, based on a questionnaire and subsequent clinical examination targeting healthy adults accompanying patients undergoing care in a tertiary hospital.</p><p><strong>Objective and methods: </strong>This study was a monocenter study in tertiary care in, The Second Affiliated Hospital, Xi'an Jiaotong University, Xi'an, China, and was conducted by the Department of Dermatology. Healthy adults accompanying a patient to the outpatient clinic or hospitalized (Department of Dermatology excluded) were approached and surveyed after agreeing to participation. The data were collected by residents and students and the questionnaire was translated into Chinese.</p><p><strong>Results: </strong>A total of 552 Chinese participants were eligible for inclusion, consisting of 246 males and 306 females. A total of 515 participants were screened negative through the questionnaire. Twenty-two of the participants screened positive. Of these, four participants who were highly suspected to have HS declined a physical exam. The possibility of HS was ruled out by the collection of detailed medical histories of other participants (n = 16) screened positive. The confirmed prevalence of HS in the remaining 552 participants was 0.36% (95% confidence interval 0.10%-1.31%). Two participants, one male and one female, respectively, were diagnosed with HS. There were no significant differences in the median age, BMI and the percentage of smokers between the control group and the HS group.</p><p><strong>Conclusion: </strong>The estimated adult HS prevalence rate in Xi'an, China is 0.36%.</p>","PeriodicalId":11185,"journal":{"name":"Dermatology","volume":" ","pages":"1-6"},"PeriodicalIF":3.0,"publicationDate":"2024-12-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142876451","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
Erythema nodosum in sarcoidosis. Relationship with extrathoracic involvement and prognostic significance. 结节性红斑。与胸外受累的关系及预后意义。
IF 3 3区 医学
Dermatology Pub Date : 2024-12-19 DOI: 10.1159/000542407
Joaquim Marcoval, Adriana Iriarte, Gemma Rocamora Blanch, Juan Mañá
{"title":"Erythema nodosum in sarcoidosis. Relationship with extrathoracic involvement and prognostic significance.","authors":"Joaquim Marcoval, Adriana Iriarte, Gemma Rocamora Blanch, Juan Mañá","doi":"10.1159/000542407","DOIUrl":"https://doi.org/10.1159/000542407","url":null,"abstract":"<p><strong>Introduction: </strong>Only a few studies analysed the prognosis significance of erythema nodosum (EN) in sarcoidosis. Our objective was to analyze the prevalence of EN in sarcoidosis, its possible association of EN with extrathoracic sarcoidosis, and its prognostic significance.</p><p><strong>Methods: </strong>Retrospective study including patients diagnosed with sarcoidosis at Bellvitge University Hospital, Barcelona, Spain, between 1980 and 2017. Patients were followed up prospectively at the sarcoid clinic of the hospital. Patients' medical records were reviewed to obtain the clinical data.</p><p><strong>Results: </strong>728 patients (494 women and 234 men, mean age 43.45 years SD 13,833) were diagnosed with sarcoidosis. Radiological stages were 0 in 68 patients (9.34%), 1 in 434 (59.62%), 2 in 167 (22.94%), 3 in 45 (6.18%) and 4 in 14 (1.92%). Pulmonary fibrosis at 5 years of follow up was documented in 44 patients. EN was diagnosed in 269 (37%). In 265 cases (98.5%), EN lesions were present at the onset of sarcoidosis and mostly corresponded to radiological stage 1 at diagnosis (82.52%). Among patients with EN, 3/269 had pulmonary fibrosis at 5 years of follow-up (p<0.001), sarcoidosis activity persist for more than 5 years in 28/269 (p<0.001), and oral corticosteroids were required in 48/269 patients (p<0.001). Patients with EN had a low incidence of severe extrapulmonary involvement.</p><p><strong>Conclusion: </strong>EN was observed in 37% of sarcoidosis patients, usually at the onset of the disease. Patients with EN-associated sarcoidosis have mainly radiological stages 1 at diagnosis, and are at lower risk of chronicity, pulmonary fibrosis, and severe forms of extrathoracic organ involvement.</p>","PeriodicalId":11185,"journal":{"name":"Dermatology","volume":" ","pages":"1-10"},"PeriodicalIF":3.0,"publicationDate":"2024-12-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142863582","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
Ehlers-Danlos Syndromes and Related Disorders: Diagnostic Challenges and the Need for an Interdisciplinary Patient Care in Germany. 埃勒-丹洛斯综合征和相关疾病:诊断挑战和需要跨学科的病人护理在德国。
IF 3 3区 医学
Dermatology Pub Date : 2024-12-10 DOI: 10.1159/000542026
Nikolaus Kernich, Franziska Peters, Julia Schreml, Oliver Semler, Manuel Koch, Eckhard Schönau, Michael Huntgeburth, Peer Eysel, Thomas Krieg, Esther von Stebut-Borschitz, Iliana Tantcheva-Poór
{"title":"Ehlers-Danlos Syndromes and Related Disorders: Diagnostic Challenges and the Need for an Interdisciplinary Patient Care in Germany.","authors":"Nikolaus Kernich, Franziska Peters, Julia Schreml, Oliver Semler, Manuel Koch, Eckhard Schönau, Michael Huntgeburth, Peer Eysel, Thomas Krieg, Esther von Stebut-Borschitz, Iliana Tantcheva-Poór","doi":"10.1159/000542026","DOIUrl":"10.1159/000542026","url":null,"abstract":"<p><strong>Introduction: </strong>Ehlers-Danlos syndromes (EDS) represent a group of heritable connective tissue disorders characterized by skin hyperelasticity, joint hypermobility and generalized tissue fragility. Many patients remain undiagnosed years after initial symptoms and an accurate diagnosis is difficult despite all efforts. Currently, Germany lacks a patient registry and a specialized EDS centre.</p><p><strong>Methods: </strong>In early 2020, a dermatological-orthopaedic EDS outpatient service was established at the University Hospital of Cologne. Medical records of all patients presenting in 2020 were retrospectively analysed.</p><p><strong>Results: </strong>Forty-three adults were examined. Fifteen patients were diagnosed with EDS (different types), 13 with hypermobility spectrum disorder, and 1 with likely Loeys-Dietz syndrome (LDS) based on patient history and a suspicious variant in the gene TGFBR1. Excluding hypermobile EDS (6 patients), molecular confirmation was achieved in a total of 4 of 9 patients. The combination of symptomatic generalized hypermobility and skin manifestations was diagnostic in more than two-thirds of the EDS patients. Arterial involvement (aneurysms, dissection and rupture) and distinctive cutaneous signs (thin translucent skin with haematomas) indicated vascular EDS and LDS in altogether 3 patients.</p><p><strong>Conclusion: </strong>With the present analysis, we discuss our diagnostic approach in patients with a suspected diagnosis of EDS in order to raise awareness of this rare group of genodermatoses and review recent developments in EDS nosology.</p>","PeriodicalId":11185,"journal":{"name":"Dermatology","volume":" ","pages":"1-9"},"PeriodicalIF":3.0,"publicationDate":"2024-12-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142806473","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
Perineural Invasion for Risk Stratification in Cutaneous Squamous Cell Carcinoma: A Scoping Review. 用于皮肤鳞状细胞癌风险分层的神经周围侵犯:范围界定综述。
IF 3 3区 医学
Dermatology Pub Date : 2024-11-27 DOI: 10.1159/000542772
Emmy C Crüts, Myrthe M G Moermans, Myrurgia Abdul Hamid, Patty J Nelemans, Klara Mosterd
{"title":"Perineural Invasion for Risk Stratification in Cutaneous Squamous Cell Carcinoma: A Scoping Review.","authors":"Emmy C Crüts, Myrthe M G Moermans, Myrurgia Abdul Hamid, Patty J Nelemans, Klara Mosterd","doi":"10.1159/000542772","DOIUrl":"10.1159/000542772","url":null,"abstract":"<p><strong>Background: </strong>Microscopic perineural invasion (mPNI) is a histopathological characteristic that can be found in cutaneous squamous cell carcinoma (cSCC). In the eighth edition of the American Joint Committee on Cancer (AJCC), mPNI defined as the involvement of nerves ≥0.1 mm and nerves deeper than the dermis is included in risk stratification of cSCC. The question remains whether other mPNI features are important for optimal cSCC staging. We aimed to summarize the evidence from published studies on the independent association between various mPNI features and the risk of recurrence, metastasis and disease-specific death in patients with cSCC.</p><p><strong>Summary: </strong>Embase, PubMed, and Web of Science were searched from January 2023 to February 2024 to identify studies that reported the prognostic impact of mPNI features in patients ≥18 years with histopathologically verified cSCC. Data on study and tumour characteristics were extracted. Nineteen studies met the inclusion criteria and evaluated one or more mPNI features in cSCC including nerve diameter, the extent of mPNI, the number of affected nerves, and depth of mPNI. Two studies provided evidence that \"mPNI ≥0.1 mm\" and \"mPNI deeper than the dermis\" are significantly and independently associated with poor prognosis after correction for other mPNI features and high-risk factors. One of these studies additionally identified \"involvement of ≥3 nerves\" as an independent and significant predictor of higher risk of local recurrence (HR, 2.17; 95% CI: 1.03-4.56; p = 0.04).</p><p><strong>Key messages: </strong>Besides \"nerve diameter of ≥0.1 mm\" and \"depth of mPNI involvement,\" \"involvement of multiple nerves\" was found to be an independent risk factor for poor prognosis and should also be considered for appropriate risk stratification.</p>","PeriodicalId":11185,"journal":{"name":"Dermatology","volume":" ","pages":"1-7"},"PeriodicalIF":3.0,"publicationDate":"2024-11-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142738733","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}
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