European Journal of Dermatology最新文献

{"title":"Symmetrical acral keratoderma associated with new variants in the filaggrin gene.","authors":"Wanting Luo, Qiaoyun Lu, Yangyang Jiang, Wenwu Dong, Zhimiao Lin, Huijun Wang, Fang Yang","doi":"10.1684/ejd.2024.4740","DOIUrl":"10.1684/ejd.2024.4740","url":null,"abstract":"<p><p>Symmetrical acral keratoderma (SAK) is a rare skin disorder with symmetric hyperkeratotic patches on the acral regions. Variants in the filaggrin gene (FLG) have been associated with SAK since 2020. To explore the clinical and genetic basis in six patients with SAK. Whole-exome sequencing, direct sequencing, and prediction of protein structure and function were performed. In this study, we identified two novel variants, c.3320del and c.4909del, and seven previously reported variants, c.3099C>G, c.4544C>A, c.6950_6957del, c.7264G>T, c.7945del, c.8117C>G, c.12064A>T. The findings of this study bolster the existing evidence implicating FLG variants in SAK, introducing two novel variants to the database of FLG variants associated with the condition.</p>","PeriodicalId":11968,"journal":{"name":"European Journal of Dermatology","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142079804","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Mogamulizumab for Sézary syndrome: long-term remission with associated autoimmune haemolytic anaemia.","authors":"Ana Maria Lé, Rita Peixeiro, Catarina Lau, Maria Dos Anjos Teixeira, Renata Cabral, Iolanda Fernandes","doi":"10.1684/ejd.2024.4744","DOIUrl":"10.1684/ejd.2024.4744","url":null,"abstract":"","PeriodicalId":11968,"journal":{"name":"European Journal of Dermatology","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142079795","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Challenging treatment of keloid scars: a case report.","authors":"Rauno J Harvima, Ilkka T Harvima","doi":"10.1684/ejd.2024.4728","DOIUrl":"10.1684/ejd.2024.4728","url":null,"abstract":"","PeriodicalId":11968,"journal":{"name":"European Journal of Dermatology","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142079785","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hydroelectrolytic and nutritional needs of infants with epidermolysis bullosa during the first month of life.","authors":"Hélène Schieber, Smail Hadj-Rabia, Agnès Giuseppi, Silvia Iacobelli, Frédérique Quetin, Elsa Kermorvant-Duchemin, Christine Bodemer, Alexandre Lapillonne","doi":"10.1684/ejd.2024.4736","DOIUrl":"https://doi.org/10.1684/ejd.2024.4736","url":null,"abstract":"<p><p>Dehydration and malnutrition are common in infants with severe epidermolysis bullosa (EB), but their nutritional needs have been poorly studied. The principal aim was to assess the nutritional status, fluid and electrolyte balance, and nutritional intake of newborns with EB during the first month of life and estimate their needs during this period. This was a retrospective study over an eight-year period. Inclusion criteria were neonates with confirmed EB admitted to our neonatal referral unit during the first month of life. Exclusion criteria were hospitalisations <7 days. Twenty-seven patients with EB (mean [min-max] gestational age = 39 weeks [33; 41]; birth weight = 2986 g [1982; 4150]), were included. Four patients (15%) had hyponatraemia < 135 mmol/L at admission (age at admission = 4.8 days +/- 2.6 [2; 7]). Sixteen patients (59%) had a sodium deficit -requiring fluid and sodium intake well above recommendations from the World Health Organisation (WHO). The risk of hyponatraemia was significantly higher in infants with the greatest body surface area affected but did not appear to be related to EB subtype. Caloric and protein intake were well above the WHO's recommendations, preventing acquired growth restriction. The rate of sodium deficit in neonates with EB is high and related to the significance of skin exudate. The administration of nutrient intake greater than that recommended helps to prevent acquired growth restriction. We propose recommendations for nutritional intake and monitoring in neonates with EB in the first month of life.</p>","PeriodicalId":11968,"journal":{"name":"European Journal of Dermatology","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142079790","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Effect of Th2 cytokines on ceramide subclasses based on a model of reconstructed human epidermis.","authors":"Joudi Bakar, Rime Michael-Jubeli, Michel Salmon, Céline Lancelot, Arlette Baillet-Guffroy, Ali Tfayli","doi":"10.1684/ejd.2024.4737","DOIUrl":"https://doi.org/10.1684/ejd.2024.4737","url":null,"abstract":"<p><p>Atopic dermatitis (AD) is associated with chronic inflammation and an altered skin barrier. Lipids of the stratum corneum of AD patients are known to differ substantially in composition from those of healthy subjects. A reconstructed human epidermis (RHE) model has been developed in vitro in order to mimic the characteristics of AD. In this study, using this model, we compared lipid profile modifications between control RHE and RHE treated with Th2 cytokines in order to mimic AD. We focused particularly on the lipid profile of the ceramide subclasses: non-hydroxy sphingosine (NS) and esterified ω-hydroxy sphingosine (EOS), which have been reported to be clearly modified in atopic skin. RHE lipids were extracted and analysed using high-performance liquid chromatography coupled to high-resolution mass spectrometry. The following lipid profile changes were observed in Th2-cytokine-treated RHE: (i) an increase in ceramide NS composed of an unsaturated fatty acid chain; (ii) an increase in saturated ceramide NS with small total carbon content (≤40 carbon atoms), whereas NS with a higher total carbon content (≥42 carbon atoms) was decreased; and (iii) a decrease in ceramide EOS. These results are in accordance with reported lipid profiles of human atopic skin in vivo. Moreover, the in vitro model represents a useful tool to better understand the pathogenesis of AD which may be used for future screening of new effective treatments.</p>","PeriodicalId":11968,"journal":{"name":"European Journal of Dermatology","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142079788","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Non-melanoma skin cancer burden in Spain: a nationwide analysis of incidence trends (1990-2019).","authors":"Lucía Cayuela, José-Juan Pereyra-Rodríguez, Juan-Carlos Hernández-Rodríguez, Mercedes Sendín-Martín, Aurelio Cayuela","doi":"10.1684/ejd.2024.4732","DOIUrl":"https://doi.org/10.1684/ejd.2024.4732","url":null,"abstract":"<p><p>Non-melanoma skin cancer (NMSC) is the most -common skin cancer in Spain, yet national data on its incidence trends are limited. To analyse the trends in NMSC incidence in Spain from 1990 to 2019, examining variations by sex, age, period, and birth cohort. Data on NMSC incidence was sourced from the Global Health Data Exchange. Age-standardized incidence rates (ASIRs) were calculated using the direct method. Trends and average annual percentage changes were identified using Joinpoint regression analysis. Age-period-cohort analysis was applied to assess age-specific, period-specific, and cohort-specific relative risks. From 1990 to 2019, Spain reported 2,302,399 NMSC cases. ASIRs significantly declined post-2005, with men exhibiting slightly higher rates than women. Joinpoint analysis revealed distinct trends between genders, with men experiencing an initial rise followed by a decline, while women exhibited periods of increase interspersed with decline. APC analysis showed a net decrease in age-adjusted NMSC rates for both sexes. Local drift analysis showed a downward trend in most age groups, indicating a broad decrease at the population level. However, no decrease was observed in young men (20-24 years). Both sexes showed an increased risk of NMSC between 1990 and 2002, followed by a decrease. In particular, those born at the beginning of the 21st century showed a significant decrease in NMSC risk compared with earlier cohorts, suggesting a possible cohort effect. A comprehensive analysis of NMSC trends in Spain highlights the need for ongoing research and interventions to address the evolving burden.</p>","PeriodicalId":11968,"journal":{"name":"European Journal of Dermatology","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142079797","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Steroid-resistant Vogt-Koyanagi-Harada-like uveitis due to BRAF/MEK inhibitor, successfully treated with adalimumab.","authors":"Manami Takahashi-Watanabe, Taku Fujimura, Ryo Amagai, Emi Yamazaki, Erika Tamabuchi, Saaya Akai, Yumi Kambayashi, Akira Hashimoto, Kazuki Hashimoto, Yoshihide Asano","doi":"10.1684/ejd.2024.4730","DOIUrl":"10.1684/ejd.2024.4730","url":null,"abstract":"","PeriodicalId":11968,"journal":{"name":"European Journal of Dermatology","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142079802","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Lasthenie de Ferjol syndrome associated with skin picking disorder.","authors":"Evelyne Harkemanne, Axel De Greef, Elie Cogan, Marie Baeck","doi":"10.1684/ejd.2024.4725","DOIUrl":"https://doi.org/10.1684/ejd.2024.4725","url":null,"abstract":"","PeriodicalId":11968,"journal":{"name":"European Journal of Dermatology","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142079794","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"An oil-in-water emulsion containing a combination of ginger extract and synthetic cannabidiol with potent in vitro anti-inflammatory effects alleviates symptoms of atopic dermatitis in a clinical trial.","authors":"Christine Neubauer, Martin Kragl, Theodor Braun, Przemyslaw Filipek, Ehsan Bonyadi Rad, Julia Grander, Nicole Mähler, Kathrin Moritz, Stefan Hoch, Ramona Ziegler, Stanislav Andreev, Hubert Steindl, Thomas Jakschitz, Andreas Koeberle, Günther Bonn, Michael Soeberdt, Christoph Abels","doi":"10.1684/ejd.2024.4738","DOIUrl":"10.1684/ejd.2024.4738","url":null,"abstract":"<p><p>Atopic dermatitis (AD) is a highly prevalent chronic skin disease. Anti-inflammatory and antipruritic emollients (emollients plus) with excellent cosmetic properties may alleviate AD-related symptoms and reduce the number of exacerbations. To screen for herbal extracts with potent anti-inflammatory and antioxidative potential in human skin cell cultures. Ginger extract and synthetic cannabidiol (CBD) were identified and combined in the cosmetic product BNO 3731, which was evaluated in a randomized clinical trial. Preclinical: anti-inflammatory effects of ginger extract, synthetic CBD and a combination thereof were evaluated in human skin cell cultures by analysing nuclear factor κB activation, release of inflammatory cytokines and endocannabinoid production. Clinical: BNO 3731 was studied in a clinical trial comprising 44 AD patients (adults and children) and compared to a benchmark product over a treatment duration of five days. Symptom severity was evaluated by objective and subjective dermatological assessments as well as physiological skin parameters. Itch intensity was assessed using a numerical rating scale (NRS-11). Preclinical: Ginger extract and synthetic CBD exhibited potent anti-inflammatory and antioxidative effects in vitro which were associated with elevated concentrations of the endocannabinoid, anandamide. Clinical: BNO 3731 significantly alleviated symptoms of AD and improved physiological skin parameters. Itch intensity decreased significantly by 55%, and in 75% of subjects, itch improved ≥2 points on the NRS-11 scale. No adverse events were reported. BNO 3731, containing a unique synergistic combination of ginger extract and synthetic CBD, is an effective and safe treatment option for dry and eczema-prone skin, providing rapid and substantial relief of pruritus.</p>","PeriodicalId":11968,"journal":{"name":"European Journal of Dermatology","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142079783","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"IgG4-related disease developing during the course of cutaneous plasmacytosis.","authors":"Makoto Inaoki, Chihiro Nishijima, Kiyoki Kitagawa, Takeshi Echigo, Kazuyoshi Katayanagi, Kiyohiro Tsutsui","doi":"10.1684/ejd.2024.4735","DOIUrl":"10.1684/ejd.2024.4735","url":null,"abstract":"","PeriodicalId":11968,"journal":{"name":"European Journal of Dermatology","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142079791","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}