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
Introduction: 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.
Methods: 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.
Results: 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.
Conclusion: 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.
{"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}
Emmy C Crüts, Myrthe M G Moermans, Myrurgia Abdul Hamid, Patty J Nelemans, Klara Mosterd
Background: 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.
Summary: 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).
Key messages: 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.
{"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}
Zarqa Ali, Johan Anker Chrom Allerup, Anders Daniel Andersen, Justin M Ko, John R Zibert, Simon Francis Thomsen
Introduction: Atopic dermatitis (AD) severity is traditionally evaluated during in-office consultations; however, this does not provide continuous monitoring and any intermittent flare/remission cycles are usually not recorded. The aim was to apply smartphone technology to evaluate AD severity, to explore if severity based on highly frequent sampling of photographs is associated with patient reported outcomes, and to investigate disease fluctuations and trigger associations based on passively collected environmental data.
Methods: In this 12-week decentralized observational study, adult patients with AD were recruited online and used a tailored remote clinical trial platform app to perform all study tasks including capturing photographs and completing the Patient-Oriented Eczema Measure (POEM) weekly. AD severity was assessed based on photographs using the Eczema Area and Severity Index (EASI) and Scoring Atopic Dermatitis (SCORAD). Geographical location collected in the app was used to retrieve data on ambient temperature and carbon monoxide (CO), a common air pollutant.
Results: A total of 42 patients (35 women) were recruited online. A total of 712 photographs were taken, with an average of 17 photographs per participant. Photographic SCORAD (r = 0.450) and EASI (r = 0.206) were significantly associated with subjective severity POEM. Patients experiencing AD fluctuation (n = 10) based on SCORAD had significantly higher risk of also having a psychiatric disorder (60 vs. 17%, p = 0.008). Anxiety was significantly associated with disease fluctuation based on EASI (40 vs. 7%, p = 0.01), and a tendency was observed for depression (40 vs. 13%, p = 0.06). Decreasing temperature was significantly associated with higher POEM (estimate -0.18, p = 0.012) and EASI score (estimate -0.14, p = 0.007), but not with SCORAD. High levels of CO were significantly associated with higher SCORAD (estimate 15.9, p < 0.001).
Conclusion: In this small study with a predominance of young adults, primarily women, we were able to recruit patients and monitor AD entirely remotely via smartphone-enabled photographic assessments; patients reported outcomes and passively collected environmental data without physical contact between patient and investigator.
{"title":"Using Smartphone Data to Frequently Measure Atopic Dermatitis Severity and Understand Triggers: A Decentralized 12-Week Observational Study.","authors":"Zarqa Ali, Johan Anker Chrom Allerup, Anders Daniel Andersen, Justin M Ko, John R Zibert, Simon Francis Thomsen","doi":"10.1159/000542771","DOIUrl":"10.1159/000542771","url":null,"abstract":"<p><strong>Introduction: </strong>Atopic dermatitis (AD) severity is traditionally evaluated during in-office consultations; however, this does not provide continuous monitoring and any intermittent flare/remission cycles are usually not recorded. The aim was to apply smartphone technology to evaluate AD severity, to explore if severity based on highly frequent sampling of photographs is associated with patient reported outcomes, and to investigate disease fluctuations and trigger associations based on passively collected environmental data.</p><p><strong>Methods: </strong>In this 12-week decentralized observational study, adult patients with AD were recruited online and used a tailored remote clinical trial platform app to perform all study tasks including capturing photographs and completing the Patient-Oriented Eczema Measure (POEM) weekly. AD severity was assessed based on photographs using the Eczema Area and Severity Index (EASI) and Scoring Atopic Dermatitis (SCORAD). Geographical location collected in the app was used to retrieve data on ambient temperature and carbon monoxide (CO), a common air pollutant.</p><p><strong>Results: </strong>A total of 42 patients (35 women) were recruited online. A total of 712 photographs were taken, with an average of 17 photographs per participant. Photographic SCORAD (r = 0.450) and EASI (r = 0.206) were significantly associated with subjective severity POEM. Patients experiencing AD fluctuation (n = 10) based on SCORAD had significantly higher risk of also having a psychiatric disorder (60 vs. 17%, p = 0.008). Anxiety was significantly associated with disease fluctuation based on EASI (40 vs. 7%, p = 0.01), and a tendency was observed for depression (40 vs. 13%, p = 0.06). Decreasing temperature was significantly associated with higher POEM (estimate -0.18, p = 0.012) and EASI score (estimate -0.14, p = 0.007), but not with SCORAD. High levels of CO were significantly associated with higher SCORAD (estimate 15.9, p < 0.001).</p><p><strong>Conclusion: </strong>In this small study with a predominance of young adults, primarily women, we were able to recruit patients and monitor AD entirely remotely via smartphone-enabled photographic assessments; patients reported outcomes and passively collected environmental data without physical contact between patient and investigator.</p>","PeriodicalId":11185,"journal":{"name":"Dermatology","volume":" ","pages":"1-11"},"PeriodicalIF":3.0,"publicationDate":"2024-11-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142715657","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}
Introduction: The natural history, the progression of a disease process in an individual over time, has not yet been fully elucidated in hidradenitis suppurativa (HS). In this large multicenter study, we aimed to investigate the natural history of HS and its gender differences.
Methods: This cross-sectional study included 827 patients. The chronological order of the clinical manifestations for each patient was recorded retrospectively. Sociodemographic characteristics, triggering factors, clinical, treatment, and prognostic features were also evaluated.
Results: The mean age of disease onset was significantly younger in women than in men (22.42 ± 9.28 vs. 27.06 ± 20.56, p < 0.001) and those with a family history (p < 0.0001). The mean disease duration was 91.17 ± 83.64 months. The most common symptom was purulent discharge (81%). The mean duration of abscess was shorter in women than in men (3.11 ± 2.65 vs. 3.75 ± 3.75, p = 0.01). The axilla was the most common onset area followed by the inguinal and gluteal regions. The disease ran a more severe course in men. Abscess/inflammatory nodule was defined in the last 6 months in 88.6% of the patients; however, the first 5 years of the disease were the most active disease period in 67.5% of the patients. Multivariate analysis revealed being male, older age, family history, involvement of the axillary, inguinal, and perianal regions independently associated with HS severity. While antibiotic use was the most important factor in improving the disease symptoms, stress was the most common aggravating factor. Biological therapy in men, laser epilation and pregnancy in women were significant alleviating factors, whereas weight gain was a more common aggravating factor for women.
Conclusion: HS shows a relentlessly progressive course with inflammatory attacks, but the first years of the disease are the most active period. This study confirms that environmental and hormonal factors may play an important role in the disease course, probably with other endogenous or exogenous factors.
导言:化脓性扁桃体炎(HS)的自然病史,即疾病在个体身上随时间推移而发展的过程,尚未完全阐明。在这项大型多中心研究中,我们旨在调查化脓性扁桃体炎的自然病史及其性别差异:这项横断面研究包括 827 名患者。回顾性记录了每位患者临床表现的时间顺序。研究还评估了社会人口学特征、诱发因素、临床、治疗和预后特征:结果:女性的平均发病年龄明显小于男性(22.42±9.28 vs. 27.06±20.56,p):HS的病程呈无情的进行性发展,并伴有炎症发作,但疾病的最初几年是最活跃的时期。这项研究证实,环境和激素因素可能在病程中起着重要作用,可能还有其他内源性或外源性因素。
{"title":"Clinical Features and Natural Course of Hidradenitis Suppurativa in Turkey: A Multicenter Study.","authors":"Erkan Alpsoy, Bilge Fettahlıoglu Karaman, Duriye Deniz Demirseren, S Levent Cınar, Nida Kacar, Aylin Türel Ermertcan, Emel Bulbul Baskan, Derya Ucmak, Kifayet Mammadli, Fadime Kılınc, Serkan Yazici, Selami Aykut Temiz, Tugba Özkök Akbulut, Arzu Ataseven, Aysun Şikar Aktürk, Hayriye Sarıcaoğlu, Meltem Türkmen, Fatmagül Gülbaşaran, Burhan Engin, Hatice Kaya Özden, Koray Durmaz, Müge Güler Özden, Hilal Özdemir, Bengü Çevirgen Cemil, Sezgi Sarıkaya Solak, Serap Günes Bilgili, İbrahim Halil Yavuz, Göknur Özaydın Yavuz, Münevver Güven, Algün Polat Ekinci, Tuğba Atcı, Didem Didar Balci, Aylin Oztürk, İlknur Kıvanç Altunay, Ezgi Özkur, Ece Ugurer, Ayse Serap Karadag, Göknur Kalkan, Sevilay Kılıc, Bilgen Erdoğan, Savas Yayli, Leyla Baykal Selçuk, Levent Dönmez, Aslı Bilgic","doi":"10.1159/000542670","DOIUrl":"10.1159/000542670","url":null,"abstract":"<p><strong>Introduction: </strong>The natural history, the progression of a disease process in an individual over time, has not yet been fully elucidated in hidradenitis suppurativa (HS). In this large multicenter study, we aimed to investigate the natural history of HS and its gender differences.</p><p><strong>Methods: </strong>This cross-sectional study included 827 patients. The chronological order of the clinical manifestations for each patient was recorded retrospectively. Sociodemographic characteristics, triggering factors, clinical, treatment, and prognostic features were also evaluated.</p><p><strong>Results: </strong>The mean age of disease onset was significantly younger in women than in men (22.42 ± 9.28 vs. 27.06 ± 20.56, p < 0.001) and those with a family history (p < 0.0001). The mean disease duration was 91.17 ± 83.64 months. The most common symptom was purulent discharge (81%). The mean duration of abscess was shorter in women than in men (3.11 ± 2.65 vs. 3.75 ± 3.75, p = 0.01). The axilla was the most common onset area followed by the inguinal and gluteal regions. The disease ran a more severe course in men. Abscess/inflammatory nodule was defined in the last 6 months in 88.6% of the patients; however, the first 5 years of the disease were the most active disease period in 67.5% of the patients. Multivariate analysis revealed being male, older age, family history, involvement of the axillary, inguinal, and perianal regions independently associated with HS severity. While antibiotic use was the most important factor in improving the disease symptoms, stress was the most common aggravating factor. Biological therapy in men, laser epilation and pregnancy in women were significant alleviating factors, whereas weight gain was a more common aggravating factor for women.</p><p><strong>Conclusion: </strong>HS shows a relentlessly progressive course with inflammatory attacks, but the first years of the disease are the most active period. This study confirms that environmental and hormonal factors may play an important role in the disease course, probably with other endogenous or exogenous factors.</p>","PeriodicalId":11185,"journal":{"name":"Dermatology","volume":" ","pages":"1-11"},"PeriodicalIF":3.0,"publicationDate":"2024-11-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142686324","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}
Rewan M Abdelwahab, Nessa Aghazadeh Mohandesi, Charles D Sturgis, Afsaneh Alavi
Introduction: Squamous metaplasia of lactiferous duct (SMOLD), also known as Zuska's disease, is an uncommon, recurrent inflammatory fistulizing disease of the breast that strongly correlates with smoking in premenopausal patients. Clinical and imaging findings may overlap with other breast conditions. SMOLD is well recognized by breast pathologists; however, the dermatology literature on this condition remains scarce.
Methods: In this retrospective study, we reviewed 29 patients with SMOLD diagnosed at Mayo Clinic.
Results: The mean age of the patient cohort is 50.3 with a range of 30 to 81 years. One patient (3.7%) had hidradenitis suppurativa of the retroareolar area. Patient smoking history demonstrated prior/current smokers of 37.9% (11/29), lifetime nonsmokers with significant secondhand exposure 6.9% (2/29), and unknown smoking status 3.4% (1/29). One patient had a personal history of invasive ductal carcinoma, and 10.3% (3/29) had a history of breast cancer in a first-degree relative. The clinical presentation of the patient cohort includes areolar papules, nodules, and draining tract/fistula 13.7% (4/29); pustular cyst/abscess on the breast 13.7% (4/29); breast mass 3.4% (1/29); pain breast discomfort/pain 13.7% (4/29); nipple retraction 3.4% (1/29); and asymptomatic with nipple calcifications on mammogram 3.4% (1/29). A total of 77.8% (7/9) of patients with bacterial cultures demonstrated polymicrobial growth. Overall, 37.9% (11/29) of patients received at least one round of antibiotic therapy. In total, 27.6% (8/29) of patients underwent invasive intervention. Staphylococcus, Streptococcus, and Cutibacterium species were the most frequent causes of infection in our patient cohort.
Conclusions: We confirm previous findings of strong association between SMOLD and current/former smoking status and a potential, novel correlation between extensive secondhand exposure and SMOLD development. While both medical and surgical interventions are employed in patient management, many patients ultimately require complete excision of the involved duct(s). Dermatologists should consider SMOLD in the differential diagnosis of patients presenting with breast abscess, fistulizing tracts with mass, and breast pain.
{"title":"Squamous Metaplasia of Lactiferous Ducts (Zuska's Disease) of the Breast: Clinical and Histopathologic Manifestations.","authors":"Rewan M Abdelwahab, Nessa Aghazadeh Mohandesi, Charles D Sturgis, Afsaneh Alavi","doi":"10.1159/000542622","DOIUrl":"10.1159/000542622","url":null,"abstract":"<p><strong>Introduction: </strong>Squamous metaplasia of lactiferous duct (SMOLD), also known as Zuska's disease, is an uncommon, recurrent inflammatory fistulizing disease of the breast that strongly correlates with smoking in premenopausal patients. Clinical and imaging findings may overlap with other breast conditions. SMOLD is well recognized by breast pathologists; however, the dermatology literature on this condition remains scarce.</p><p><strong>Methods: </strong>In this retrospective study, we reviewed 29 patients with SMOLD diagnosed at Mayo Clinic.</p><p><strong>Results: </strong>The mean age of the patient cohort is 50.3 with a range of 30 to 81 years. One patient (3.7%) had hidradenitis suppurativa of the retroareolar area. Patient smoking history demonstrated prior/current smokers of 37.9% (11/29), lifetime nonsmokers with significant secondhand exposure 6.9% (2/29), and unknown smoking status 3.4% (1/29). One patient had a personal history of invasive ductal carcinoma, and 10.3% (3/29) had a history of breast cancer in a first-degree relative. The clinical presentation of the patient cohort includes areolar papules, nodules, and draining tract/fistula 13.7% (4/29); pustular cyst/abscess on the breast 13.7% (4/29); breast mass 3.4% (1/29); pain breast discomfort/pain 13.7% (4/29); nipple retraction 3.4% (1/29); and asymptomatic with nipple calcifications on mammogram 3.4% (1/29). A total of 77.8% (7/9) of patients with bacterial cultures demonstrated polymicrobial growth. Overall, 37.9% (11/29) of patients received at least one round of antibiotic therapy. In total, 27.6% (8/29) of patients underwent invasive intervention. Staphylococcus, Streptococcus, and Cutibacterium species were the most frequent causes of infection in our patient cohort.</p><p><strong>Conclusions: </strong>We confirm previous findings of strong association between SMOLD and current/former smoking status and a potential, novel correlation between extensive secondhand exposure and SMOLD development. While both medical and surgical interventions are employed in patient management, many patients ultimately require complete excision of the involved duct(s). Dermatologists should consider SMOLD in the differential diagnosis of patients presenting with breast abscess, fistulizing tracts with mass, and breast pain.</p>","PeriodicalId":11185,"journal":{"name":"Dermatology","volume":" ","pages":"1-6"},"PeriodicalIF":3.0,"publicationDate":"2024-11-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142616507","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}
John R Ingram, Vincenzo Bettoli, Jasmine I Espy, Georgios Kokolakis, Antonio Martorell, Axel P Villani, Hayley Wallinger, Isabel Truman, Emily Coak, Torben Kasparek, Elisa Muscianisi, Craig Richardson, Alexa B Kimball
Introduction: Hidradenitis suppurativa (HS) is a debilitating, inflammatory skin disorder. Treatment strategies in patients with HS are challenging; real-world evidence in a HS population is warranted for greater disease understanding. The objective of this analysis was to describe real-world treatment patterns and treatment satisfaction in patients with HS.
Methods: This was a cross-sectional market research survey with retrospective data collection in patients with HS from the USA and five European countries (France, Germany, Italy, Spain, and the UK) between November 2020 and April 2021, using physician- and patient-reported surveys. Eligible physicians were general dermatologists actively managing patients with HS; dermatologists were required to have consulted with ≥2 patients with HS in the previous 12 months. Adult (≥18 years) and adolescent (10-17 years) HS patients visiting a participating dermatologist were included. Outcomes included treatment patterns, flare status, treatments prescribed in response to flares, previous surgeries, barriers to biologics, and patient- and physician-reported satisfaction with the disease control provided by treatment.
Results: Survey data from 1,787 patients were collected from 312 dermatologists. The most frequently prescribed treatments were topicals, oral antibiotics, and antiseptic washes/creams at diagnosis and sampling. At sampling, biologics were more frequently prescribed in patients with more severe disease (prescribed in 26.6%, 31.0%, and 52.4% of patients with mild, moderate, and severe disease, respectively); oral antibiotics (48.8%), topicals (37.4%), and biologics (34.3%) were the most frequently prescribed treatment classes in response to a flare. Of patients currently not receiving a biologic, dermatologists reported that 18.9% of patients' condition warranted their use. Approximately one quarter of dermatologists (24.5%) and patients (27.4%) were not satisfied with current treatment; of patients who were dissatisfied, 12.8% reported they would never raise their dissatisfaction with their doctor.
Conclusion: These real-world data suggest a high disease burden and potential undertreatment in patients with HS. Patients received multiple treatments, and a notable proportion underwent surgery. Robustly integrating the patient voice in HS treatment decisions may lead to better outcomes and improved treatment satisfaction.
{"title":"Treatment Satisfaction in Patients with Hidradenitis Suppurativa: A Real-World Survey from the EU5 and USA.","authors":"John R Ingram, Vincenzo Bettoli, Jasmine I Espy, Georgios Kokolakis, Antonio Martorell, Axel P Villani, Hayley Wallinger, Isabel Truman, Emily Coak, Torben Kasparek, Elisa Muscianisi, Craig Richardson, Alexa B Kimball","doi":"10.1159/000542343","DOIUrl":"10.1159/000542343","url":null,"abstract":"<p><strong>Introduction: </strong>Hidradenitis suppurativa (HS) is a debilitating, inflammatory skin disorder. Treatment strategies in patients with HS are challenging; real-world evidence in a HS population is warranted for greater disease understanding. The objective of this analysis was to describe real-world treatment patterns and treatment satisfaction in patients with HS.</p><p><strong>Methods: </strong>This was a cross-sectional market research survey with retrospective data collection in patients with HS from the USA and five European countries (France, Germany, Italy, Spain, and the UK) between November 2020 and April 2021, using physician- and patient-reported surveys. Eligible physicians were general dermatologists actively managing patients with HS; dermatologists were required to have consulted with ≥2 patients with HS in the previous 12 months. Adult (≥18 years) and adolescent (10-17 years) HS patients visiting a participating dermatologist were included. Outcomes included treatment patterns, flare status, treatments prescribed in response to flares, previous surgeries, barriers to biologics, and patient- and physician-reported satisfaction with the disease control provided by treatment.</p><p><strong>Results: </strong>Survey data from 1,787 patients were collected from 312 dermatologists. The most frequently prescribed treatments were topicals, oral antibiotics, and antiseptic washes/creams at diagnosis and sampling. At sampling, biologics were more frequently prescribed in patients with more severe disease (prescribed in 26.6%, 31.0%, and 52.4% of patients with mild, moderate, and severe disease, respectively); oral antibiotics (48.8%), topicals (37.4%), and biologics (34.3%) were the most frequently prescribed treatment classes in response to a flare. Of patients currently not receiving a biologic, dermatologists reported that 18.9% of patients' condition warranted their use. Approximately one quarter of dermatologists (24.5%) and patients (27.4%) were not satisfied with current treatment; of patients who were dissatisfied, 12.8% reported they would never raise their dissatisfaction with their doctor.</p><p><strong>Conclusion: </strong>These real-world data suggest a high disease burden and potential undertreatment in patients with HS. Patients received multiple treatments, and a notable proportion underwent surgery. Robustly integrating the patient voice in HS treatment decisions may lead to better outcomes and improved treatment satisfaction.</p>","PeriodicalId":11185,"journal":{"name":"Dermatology","volume":" ","pages":"1-13"},"PeriodicalIF":3.0,"publicationDate":"2024-11-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142581484","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}
Aviël Ragamin, Karlijn F van Halewijn, Renske Schappin, Willemijn C A M Witkam, Nicolette J T Arends, Jaap Hoogeterp, Arthur M Bohnen, Gijs Elshout, Marie L A Schuttelaar, Suzanne G M A Pasmans
Introduction: Various healthcare professionals (HCPs) deliver care for patients with atopic dermatitis (AD). Although pivotal, management strategies and the relation with corticophobia among HCPs have not been investigated. This study aimed to investigate management strategies for AD and its relation with corticophobia among HCPs.
Methods: Dutch general practitioners (GPs), youth healthcare physicians (YHPs), pediatricians, dermatologists, pharmacists, and pharmacy assistants participated in a survey on management strategies and corticophobia. The Topical Corticosteroid Phobia questionnaire for professionals (TOPICOP-P) was used to measure attitudes toward topical corticosteroids (TCSs). Higher scores reflect a more negative attitude.
Results: A total of 407 HCPs (124 GPs, 33 YHPs, 51 pediatricians, 56 dermatologists, 58 pharmacists, and 85 pharmacy assistants) participated. Compared to dermatologists, other HCPs showed greater reluctance to TCS. This difference was highlighted by the finding that half of GPs reported to prescribed only TCS of mild potency for infants with severe AD, while few dermatologists (9%) reported a similar approach. Dermatologists had lowest TOPICOP-P scores (median: 19, IQR: 12-28). GPs and pharmacy assistants had highest scores (GPs median: 36, IQR: 31-44, pharmacy assistants: median: 36, IQR: 31-42). More corticophobia was significantly associated with prescription of a lower TC potency class in prescribing HCPs (B -0.04, 95% CI: -0.07 to 0.01, p = 0.01), and a trend was found between more corticophobia and longer perceived durability of one TCS tube.
Conclusions: This study shows the differences in management of AD and reluctance toward TCS in HCPs. Furthermore, corticophobia among HCPs and its influence on the selection of TCS potency class and recommendations were demonstrated. To reduce corticophobia and improve care for AD, more education is needed.
背景:许多医疗保健专业人员(HCPs)为特应性皮炎(AD)患者提供护理服务。尽管管理策略至关重要,但尚未对医护人员的管理策略及其与恐皮质症的关系进行调查。本研究旨在调查特应性皮炎的管理策略及其与卫生保健人员恐皮质症的关系:荷兰全科医生(GPs)、青年保健医生(YHPs)、儿科医生、皮肤科医生、药剂师和药剂助理参与了一项关于管理策略和皮质恐惧症的调查。专业人员外用皮质类固醇恐惧症问卷(TOPICOP-P)用于测量对外用皮质类固醇(TCS)的态度。得分越高,说明态度越消极:共有 407 名高级保健医生(124 名全科医生、33 名青年保健医生、51 名儿科医生、56 名皮肤科医生、58 名药剂师和 85 名药房助理)参加了调查。与皮肤科医生相比,其他高级保健医生更不愿意使用TCS,这体现在全科医生(50%)与皮肤科医生(9%)相比,只为患有严重AD的婴儿开具温和药效的TCS处方。皮肤科医生的 TOPICOP-P 得分最低(中位数:19,IQR:12-28)。全科医生和药房助理的得分最高(全科医生中位数:36,IQR:31-44;药房助理中位数:36,IQR:31-42)。更多的皮质恐惧症与处方较低的三氯化碳效力等级明显相关(B -0.04,95%CI:-0.07-0.01,P=0.01),并且发现更多的皮质恐惧症与一个三氯化碳管的感知持久性更长之间存在趋势:本研究显示了HCP在管理AD和不愿使用TCS方面的差异。此外,HCPs 中的皮质恐惧症及其对 AD 管理的影响也得到了证实。为了减少恐皮质症并改善对 AD 的护理,需要开展更多的教育。
{"title":"Management Strategies and Corticophobia among Healthcare Professionals Involved in the Care for Atopic Dermatitis: A Dutch Survey.","authors":"Aviël Ragamin, Karlijn F van Halewijn, Renske Schappin, Willemijn C A M Witkam, Nicolette J T Arends, Jaap Hoogeterp, Arthur M Bohnen, Gijs Elshout, Marie L A Schuttelaar, Suzanne G M A Pasmans","doi":"10.1159/000542421","DOIUrl":"10.1159/000542421","url":null,"abstract":"<p><strong>Introduction: </strong>Various healthcare professionals (HCPs) deliver care for patients with atopic dermatitis (AD). Although pivotal, management strategies and the relation with corticophobia among HCPs have not been investigated. This study aimed to investigate management strategies for AD and its relation with corticophobia among HCPs.</p><p><strong>Methods: </strong>Dutch general practitioners (GPs), youth healthcare physicians (YHPs), pediatricians, dermatologists, pharmacists, and pharmacy assistants participated in a survey on management strategies and corticophobia. The Topical Corticosteroid Phobia questionnaire for professionals (TOPICOP-P) was used to measure attitudes toward topical corticosteroids (TCSs). Higher scores reflect a more negative attitude.</p><p><strong>Results: </strong>A total of 407 HCPs (124 GPs, 33 YHPs, 51 pediatricians, 56 dermatologists, 58 pharmacists, and 85 pharmacy assistants) participated. Compared to dermatologists, other HCPs showed greater reluctance to TCS. This difference was highlighted by the finding that half of GPs reported to prescribed only TCS of mild potency for infants with severe AD, while few dermatologists (9%) reported a similar approach. Dermatologists had lowest TOPICOP-P scores (median: 19, IQR: 12-28). GPs and pharmacy assistants had highest scores (GPs median: 36, IQR: 31-44, pharmacy assistants: median: 36, IQR: 31-42). More corticophobia was significantly associated with prescription of a lower TC potency class in prescribing HCPs (B -0.04, 95% CI: -0.07 to 0.01, p = 0.01), and a trend was found between more corticophobia and longer perceived durability of one TCS tube.</p><p><strong>Conclusions: </strong>This study shows the differences in management of AD and reluctance toward TCS in HCPs. Furthermore, corticophobia among HCPs and its influence on the selection of TCS potency class and recommendations were demonstrated. To reduce corticophobia and improve care for AD, more education is needed.</p>","PeriodicalId":11185,"journal":{"name":"Dermatology","volume":" ","pages":"1-12"},"PeriodicalIF":3.0,"publicationDate":"2024-11-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142575394","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}
Ahmed Samaouel Chehad, Samira Zobiri, Dorra Bouazzi, Cecilia E Medianfar, Robin Christensen, Gregor B E Jemec, Amina Serradj
Introduction Although there has been an expansion of knowledge on hidradenitis suppurativa (HS), data about the disease is largely based on Western population and no relevant African or Asian studies are available. Methods We conducted a descriptive, cross-sectional, multicenter study, as part of GHiSA (Global HS Atlas) initiative, to assess the epidemiologic profile of HS in Algerian population. Healthy adults accompanying patients undergoing care in a non-dermatological wards were approached and invited to complete a self-administered questionnaire. Subsequently, a clinical assessment was performed by an in-person dermatologists for all screen-positive participants and ten percent of the screen-negative ones. Results A total of 1434 participants were included in this study. The prevalence of HS among Algerian adults was 0.78%. Compared to non HS group, no significant difference was found regarding gender, age, body mass index and smoker status. Both the sensitivity (100%) and the specificity (97%) of the HS screening questionnaire were excellent. Conclusion The prevalence of HS in Algeria is very close to that of Australia (0.8%) and Europe (0.7%) and almost the same prevalence found by Ghanaian study (other GHiSA study from Africa). The results of this study demonstrate also the reliability and validity of GHiSA questionnaire as HS data collection instrument.
{"title":"Hidradenitis suppurativa prevalence in Algeria: A multicenter cross-sectional study.","authors":"Ahmed Samaouel Chehad, Samira Zobiri, Dorra Bouazzi, Cecilia E Medianfar, Robin Christensen, Gregor B E Jemec, Amina Serradj","doi":"10.1159/000539599","DOIUrl":"https://doi.org/10.1159/000539599","url":null,"abstract":"<p><p>Introduction Although there has been an expansion of knowledge on hidradenitis suppurativa (HS), data about the disease is largely based on Western population and no relevant African or Asian studies are available. Methods We conducted a descriptive, cross-sectional, multicenter study, as part of GHiSA (Global HS Atlas) initiative, to assess the epidemiologic profile of HS in Algerian population. Healthy adults accompanying patients undergoing care in a non-dermatological wards were approached and invited to complete a self-administered questionnaire. Subsequently, a clinical assessment was performed by an in-person dermatologists for all screen-positive participants and ten percent of the screen-negative ones. Results A total of 1434 participants were included in this study. The prevalence of HS among Algerian adults was 0.78%. Compared to non HS group, no significant difference was found regarding gender, age, body mass index and smoker status. Both the sensitivity (100%) and the specificity (97%) of the HS screening questionnaire were excellent. Conclusion The prevalence of HS in Algeria is very close to that of Australia (0.8%) and Europe (0.7%) and almost the same prevalence found by Ghanaian study (other GHiSA study from Africa). The results of this study demonstrate also the reliability and validity of GHiSA questionnaire as HS data collection instrument.</p>","PeriodicalId":11185,"journal":{"name":"Dermatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141199430","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}
Ahmed Samaouel Chehad, Samira Zobiri, Dorra Bouazzi, Cecilia E Medianfar, Robin Christensen, Gregor B E Jemec, Amina Serradj
Introduction Although there has been an expansion of knowledge on hidradenitis suppurativa (HS), data about the disease is largely based on Western population and no relevant African or Asian studies are available. Methods We conducted a descriptive, cross-sectional, multicenter study, as part of GHiSA (Global HS Atlas) initiative, to assess the epidemiologic profile of HS in Algerian population. Healthy adults accompanying patients undergoing care in a non-dermatological wards were approached and invited to complete a self-administered questionnaire. Subsequently, a clinical assessment was performed by an in-person dermatologists for all screen-positive participants and ten percent of the screen-negative ones. Results A total of 1434 participants were included in this study. The prevalence of HS among Algerian adults was 0.78%. Compared to non HS group, no significant difference was found regarding gender, age, body mass index and smoker status. Both the sensitivity (100%) and the specificity (97%) of the HS screening questionnaire were excellent. Conclusion The prevalence of HS in Algeria is very close to that of Australia (0.8%) and Europe (0.7%) and almost the same prevalence found by Ghanaian study (other GHiSA study from Africa). The results of this study demonstrate also the reliability and validity of GHiSA questionnaire as HS data collection instrument.
{"title":"Hidradenitis suppurativa prevalence in Algeria: A multicenter cross-sectional study.","authors":"Ahmed Samaouel Chehad, Samira Zobiri, Dorra Bouazzi, Cecilia E Medianfar, Robin Christensen, Gregor B E Jemec, Amina Serradj","doi":"10.1159/000539599","DOIUrl":"https://doi.org/10.1159/000539599","url":null,"abstract":"<p><p>Introduction Although there has been an expansion of knowledge on hidradenitis suppurativa (HS), data about the disease is largely based on Western population and no relevant African or Asian studies are available. Methods We conducted a descriptive, cross-sectional, multicenter study, as part of GHiSA (Global HS Atlas) initiative, to assess the epidemiologic profile of HS in Algerian population. Healthy adults accompanying patients undergoing care in a non-dermatological wards were approached and invited to complete a self-administered questionnaire. Subsequently, a clinical assessment was performed by an in-person dermatologists for all screen-positive participants and ten percent of the screen-negative ones. Results A total of 1434 participants were included in this study. The prevalence of HS among Algerian adults was 0.78%. Compared to non HS group, no significant difference was found regarding gender, age, body mass index and smoker status. Both the sensitivity (100%) and the specificity (97%) of the HS screening questionnaire were excellent. Conclusion The prevalence of HS in Algeria is very close to that of Australia (0.8%) and Europe (0.7%) and almost the same prevalence found by Ghanaian study (other GHiSA study from Africa). The results of this study demonstrate also the reliability and validity of GHiSA questionnaire as HS data collection instrument.</p>","PeriodicalId":11185,"journal":{"name":"Dermatology","volume":" ","pages":""},"PeriodicalIF":3.0,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142460188","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}
Pub Date : 2024-03-01Epub Date: 2023-07-25DOI: 10.1177/17585732231190011
Michel Pj van den Bekerom, Huub H de Klerk, Roger van Riet
Osteochondritis dissecans of the capitellum is debilitating and is a potentially sports career-ending injury in a young and athletic population. Osteochondritis dissecans typically occurs in patients between the ages of 10 and 24 years, and boys are more commonly affected than girls. Conventional radiographs have low diagnostic accuracy, and magnetic resonance imaging (with or without contrast) or computed tomography may aid in accurate diagnosis. The primary indication for non-operative treatment is the presence of an intact cartilage cap on magnetic resonance imaging, indicating a "stable lesion." However, if operative treatment is necessary, various surgical procedures are available when operative treatment for an osteochondritis dissecans of the capitellum is considered, including open or arthroscopic removal of loose bodies, with or without microfracturing, fragment fixation, osteochondral autograft transplantation, and osteochondral allograft transplantation. The decision-making process for selecting the appropriate treatment considers factors such as the patient's characteristics, functional limitations, and lesion morphology.
{"title":"Update in diagnosis, treatment, and prevention of osteochondritis dissecans of the capitellum.","authors":"Michel Pj van den Bekerom, Huub H de Klerk, Roger van Riet","doi":"10.1177/17585732231190011","DOIUrl":"10.1177/17585732231190011","url":null,"abstract":"<p><p>Osteochondritis dissecans of the capitellum is debilitating and is a potentially sports career-ending injury in a young and athletic population. Osteochondritis dissecans typically occurs in patients between the ages of 10 and 24 years, and boys are more commonly affected than girls. Conventional radiographs have low diagnostic accuracy, and magnetic resonance imaging (with or without contrast) or computed tomography may aid in accurate diagnosis. The primary indication for non-operative treatment is the presence of an intact cartilage cap on magnetic resonance imaging, indicating a \"stable lesion.\" However, if operative treatment is necessary, various surgical procedures are available when operative treatment for an osteochondritis dissecans of the capitellum is considered, including open or arthroscopic removal of loose bodies, with or without microfracturing, fragment fixation, osteochondral autograft transplantation, and osteochondral allograft transplantation. The decision-making process for selecting the appropriate treatment considers factors such as the patient's characteristics, functional limitations, and lesion morphology.</p>","PeriodicalId":11185,"journal":{"name":"Dermatology","volume":"118 1","pages":"24-34"},"PeriodicalIF":3.0,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10901169/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86855911","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}