Journal of the European Academy of Dermatology and Venereology最新文献

{"title":"Darier disease: Golden era of discovery and global collaborations","authors":"Sofia Labbouz, Roni P. Dodiuk-Gad","doi":"10.1111/jdv.20641","DOIUrl":"https://doi.org/10.1111/jdv.20641","url":null,"abstract":"<p>My mentee and I were pleased to read the comprehensive review by Ettinger et al.<span><sup>1</sup></span> on Darier disease (DD), a rare and complex genodermatosis. This article provides a valuable synthesis of current knowledge. Even after 15 years of studying and treating over 250 patients, some spanning four generations within the same family, I continue to encounter the profound clinical and therapeutic complexities of Darier disease.</p><p>We would like to highlight a few key topics discussed in the review.</p><p>DD has traditionally been viewed as a skin disease, but its systemic nature is now well recognized. Extracutaneous manifestations include neuropsychiatric disorders, learning disabilities, salivary gland obstructions and ocular abnormalities. The authors rightly emphasize the importance of early screening and multidisciplinary management to improve patient outcomes.</p><p>In 1999, Professor Alain Hovnanian's group identified ATP2A2 as the defective gene in DD.<span><sup>2</sup></span> This breakthrough revealed that mutations impair calcium homeostasis, disrupting keratinocyte adhesion and differentiation. This discovery has transformed diagnostic strategies and research directions. We wish to emphasize that genetic counselling, including ATP2A2 gene analysis and prenatal diagnostic options, should be offered to all patients.</p><p>The heterogeneity of DD lesions necessitates a standardized classification system. It is important to distinguish between classical and non-classical lesions. Classical DD lesions are common and disease-defining, including keratotic papules, pits, wart-like lesions and nail abnormalities. Non-classical DD lesions are rarer, affecting only a subset of patients, and include acral keratoderma, leucodermic macules, giant comedones, keloid-like vegetations and acral hemorrhagic blisters<span><sup>3</sup></span> (Figure 1). This distinction may serve as the basis for a formal disease classification: classic DD (only classical lesions) and non-classical DD (at least one non-classical lesion). Distinguishing transient from persistent lesions is crucial for accurate treatment assessment. Recent findings link persistent lesions to second-hit somatic ATP2A2 mutations.<span><sup>4</sup></span>\u0000 </p><p>DD is associated with a dysbiotic skin microbiome, with <i>Staphylococcus</i> species predominance.<span><sup>5</sup></span> In my experience, bleach baths are highly effective in reducing bacterial colonization, preventing infections and minimizing odour. This simple, low-cost intervention should be more widely recommended.</p><p>The review highlights the inflammatory component of DD, particularly the IL-17A/IL-23A axis, shifting the focus beyond keratinocyte dysfunction. Monoclonal antibodies targeting IL-17A, IL-23A, IL-4 and IL-13 have shown promising results in case reports. However, as the authors rightfully stated, there is a lack of randomized placebo-controlled trials owing to the limited number o","PeriodicalId":17351,"journal":{"name":"Journal of the European Academy of Dermatology and Venereology","volume":"39 5","pages":"883-884"},"PeriodicalIF":8.4,"publicationDate":"2025-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/jdv.20641","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143871524","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Epidermolytic ichthyosis: New insights and ongoing challenges","authors":"J. Mazereeuw-Hautier","doi":"10.1111/jdv.20636","DOIUrl":"https://doi.org/10.1111/jdv.20636","url":null,"abstract":"<p>I read with great interest the paper by Frommherz et al.<span><sup>1</sup></span> published in this issue. The study provides a focused analysis of epidermolytic ichthyosis (EI)—a well-defined subgroup of congenital ichthyosis (CI)—which minimizes heterogeneity bias and includes a large patient cohort despite the disease's rarity.</p><p>It highlights that, similar to other forms of CI, the burden of EI is significant—particularly due to itch, pain and the high cost of skin care—and provides new insights into genotype–phenotype correlations. Moreover, this study provides us with the opportunity to discuss future research directions.</p><p>The authors demonstrated a significant impact on quality of life (QOL) in EI patients. Although the DLQI, a widely used generic dermatology tool, was used in this study, adopting a validated ichthyosis-specific measure such as IQOL-32<span><sup>2</sup></span> may more precisely capture the disease impact; however, this score remains unavailable for paediatric use.</p><p>Regarding severity assessment, although the Ichthyosis Area Severity Index (IASI) is validated and user-friendly, it does not account for other anomalies that impact disease severity. Therefore, the authors modified the IASI to include palmoplantar involvement. This logical modification underscores the need for scoring systems that capture all manifestations, but a revised score requires further validation. The authors also used the Investigator's Global Assessment (IGA 0–4), a score commonly applied in dermatology, particularly in atopic dermatitis. However, for its use in CI, a clear definition of each IGA category (clear, almost clear, mild, moderate and severe) would be necessary, along with inter-individual validation to ensure reproducibility.</p><p>Finally, their three-level classification of localized, intermediate and severe aligns with approaches in other genodermatoses but requires broader consensus and validation in EI.</p><p>This series showed that patients with <i>KRT1</i> variants tended to be more severe. Future multicentre investigations pooling international cohorts could illuminate additional genetic modulators—such as the microbiome or inflammatory cytokines—that drive clinical variability.</p><p>As highlighted in the paper, there is no highly effective therapy available (gold standard: emollients and oral retinoids, as described in the updated international guidelines<span><sup>3</sup></span>). The use of biologics has recently proven disappointing. Our large international retrospective series on biologics in CI revealed that these agents may improve outcomes in only a subset of patients, with good responses observed primarily in erythrodermic forms, and not in EI.<span><sup>4</sup></span> Future therapeutic approaches should ideally be targeted and based on the underlying functional pathways. To address this need, the Reclassifying Epidermal Differentiation Disorders Initiative task force brought together experts and patie","PeriodicalId":17351,"journal":{"name":"Journal of the European Academy of Dermatology and Venereology","volume":"39 5","pages":"893-894"},"PeriodicalIF":8.4,"publicationDate":"2025-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/jdv.20636","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143871464","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Consensus on core domains for hand eczema trials: Signs, symptoms, control and quality of life","authors":"Henriette Rönsch,&nbsp;Karl Philipp Drewitz,&nbsp;Amber Reck Atwater,&nbsp;Detlef Becker,&nbsp;Philipp Bentz,&nbsp;Richard Brans,&nbsp;Tricia Chong,&nbsp;Heinrich Dickel,&nbsp;Peter Elsner,&nbsp;Ana M. Giménez-Arnau,&nbsp;Fabrizio Guarneri,&nbsp;María Graciela Guzmán Perera,&nbsp;Sarah Ibrahim,&nbsp;Dimitra Koumaki,&nbsp;Jamie Koelbel,&nbsp;Francesca Larese Filon,&nbsp;Suzana Ljubojević Hadžavdić,&nbsp;Laura Loman,&nbsp;Mihaly Matura,&nbsp;Sonja Molin,&nbsp;Robert Ofenloch,&nbsp;Katharina Piontek,&nbsp;Radoslaw Spiewak,&nbsp;Anne Strunk,&nbsp;Margo Reeder,&nbsp;David Reissig,&nbsp;Thomas Rustemeyer,&nbsp;Marie-Louise Schuttelaar,&nbsp;Dagmar Simon,&nbsp;Manon Sloot,&nbsp;Markus F. C. Steiner,&nbsp;Saïda Tongalaza,&nbsp;Skaidra Valiukevičienė,&nbsp;Maurice Waitek,&nbsp;Elke Weisshaar,&nbsp;Stefan Wöhrl,&nbsp;Doreen Wolff,&nbsp;Andrea Bauer,&nbsp;Christian Apfelbacher","doi":"10.1111/jdv.20671","DOIUrl":"10.1111/jdv.20671","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Hand eczema (HE) is a common and complex skin disease. A uniform set of core outcomes and related measures for use in clinical trials is lacking, making it difficult to compare results across HE studies.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>To reach consensus on a set of core domains and subdomains that should be measured in future therapeutic HE trials.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>In 2024, we conducted a two-round online Delphi (eDelphi) survey among international HE experts, including physicians, patients and their relatives, researchers and industry representatives. A domain/subdomain was included in the core set when ≥80% of participants rated is as ‘critically important’; 50% agreement or less resulted in its exclusion. Results from 50% to 80% were deemed controversial and subject for further discussion. During a hybrid consensus meeting, the stakeholders reviewed, completed and, if necessary, revised the preliminary eDelphi consensus.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>In the first and second round of the eDelphi, 208 and 134 persons, respectively, participated. Forty participants from 18 countries attended the consensus meeting. Consensus was reached to include the core domains ‘signs of HE’ (with five core subdomains), ‘symptoms of HE’ (two subdomains), ‘HE-related quality of life’ (four subdomains) and ‘HE control over time’ (four subdomains). The subdomains ‘desquamation/scaling’ and ‘emotional impact/mental health’ remained controversial. Consensus was reached that the domains ‘skin barrier function’ and ‘patient-reported treatment experience’ and 28 subdomains should not be part of the core outcome set.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>To produce comparable and meaningful results, future trials evaluating the effectiveness of HE treatments should measure signs and symptoms of HE, HE-related quality of life and HE control over time as core outcome domains. The next step of the HE core outcome set initiative (HECOS) is to identify appropriate measurement instruments.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17351,"journal":{"name":"Journal of the European Academy of Dermatology and Venereology","volume":"39 9","pages":"1588-1599"},"PeriodicalIF":8.0,"publicationDate":"2025-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/jdv.20671","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144017266","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Who treats matters: Elevating LGBTQI dermatology","authors":"Tiago R. Matos,&nbsp;Myrto Trakatelli","doi":"10.1111/jdv.20635","DOIUrl":"https://doi.org/10.1111/jdv.20635","url":null,"abstract":"&lt;p&gt;The LGBTQI community faces unique health challenges, particularly in dermatovenereology. LGBTQI patients often experience discrimination, refusal of healthcare or other negative healthcare experiences that may deter them from seeking professional care in the future.&lt;span&gt;&lt;sup&gt;1&lt;/sup&gt;&lt;/span&gt;\u0000 &lt;/p&gt;&lt;p&gt;Dermatology is one of the least ethnically and racially diverse specialties, second only to orthopaedic surgery—at least according to the only study we found based on US data.&lt;span&gt;&lt;sup&gt;2&lt;/sup&gt;&lt;/span&gt; This lack of diversity may contribute to the dermatologic community's limited awareness of the nuances faced by minority groups. This disparity has been highlighted by recent acknowledgements of gaps in medical education curricula regarding skin of colour.&lt;span&gt;&lt;sup&gt;3&lt;/sup&gt;&lt;/span&gt; Additionally, 47% of dermatologists and residents report feeling insufficiently trained to diagnose diseases in skin of colour.&lt;span&gt;&lt;sup&gt;4&lt;/sup&gt;&lt;/span&gt;\u0000 &lt;/p&gt;&lt;p&gt;Recognizing the sensitivity and controversy surrounding diversity and inclusion studies in medicine—especially given our own unsuccessful attempts to conduct dermatology diversity studies in Europe due to insufficient peer support—we deeply appreciate the significance of the work by Tsentemeidou et al.&lt;span&gt;&lt;sup&gt;5&lt;/sup&gt;&lt;/span&gt;\u0000 &lt;/p&gt;&lt;p&gt;The study, the first of its kind in Europe, assessed 74 dermatologists-venereologists using the LGBT-Development of Clinical Skills Scale (DOCSS). Participants rated themselves moderately prepared to handle LGBT-specific dermatologic and venereological concerns, though there remains room for improvement. Specialists aged 45 and over reported less training and more conservative views regarding LGBT morality than their younger colleagues. This generational gap underscores the need for targeted continuing medical education to ensure all healthcare providers offer equitable and informed care.&lt;/p&gt;&lt;p&gt;Comparing these findings with scarce publications from other countries, it becomes evident that dermatovenereologists across the globe are more aware of attitudes and beliefs than they are prepared to handle real medical situations, emphasizing how LGBTQI-oriented education remains a global need in dermatology training. While Greece, a country generally considered to be both conservative and religious, demonstrates promise, bridging knowledge gaps and fostering inclusivity must continue to be priorities.&lt;/p&gt;&lt;p&gt;At a time when diversity, equity and inclusion initiatives face growing criticism, it is crucial to recognize their impact on patient care. Studies show that race-concordant visits improve patient satisfaction and perceptions of physician engagement. Minority physicians are more likely to serve their own communities, work in underserved areas and care for disadvantaged patients. In dermatology, increasing underrepresented physician representation could help reduce disparities in access to care and address the specialty's geographic maldistribution.&lt;/p&gt;&lt;p&gt;Inte","PeriodicalId":17351,"journal":{"name":"Journal of the European Academy of Dermatology and Venereology","volume":"39 5","pages":"897-898"},"PeriodicalIF":8.4,"publicationDate":"2025-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/jdv.20635","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143871853","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Emergency for dermatologic wards?","authors":"Falk Ochsendorf","doi":"10.1111/jdv.20619","DOIUrl":"https://doi.org/10.1111/jdv.20619","url":null,"abstract":"<p>In this respect, the above-mentioned work<span>¹</span> encourages numerous further investigations to clarify the open questions mentioned. Above all, however, it is a wake-up call for all hospitals with dermatology departments to review how patients with acute dermatological conditions are directed from the emergency department to the wards providing care. This is the only way to ensure that these patients receive optimal medical and nursing care on a dermatology ward.</p><p>None.</p>","PeriodicalId":17351,"journal":{"name":"Journal of the European Academy of Dermatology and Venereology","volume":"39 5","pages":"889-890"},"PeriodicalIF":8.4,"publicationDate":"2025-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/jdv.20619","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143871624","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Surgical outcomes in patients treated with deroofing for hidradenitis suppurativa.","authors":"Meerab Majeed, Zeena Kailani, Mohannad Abu-Hilal","doi":"10.1111/jdv.20704","DOIUrl":"https://doi.org/10.1111/jdv.20704","url":null,"abstract":"","PeriodicalId":17351,"journal":{"name":"Journal of the European Academy of Dermatology and Venereology","volume":" ","pages":""},"PeriodicalIF":8.4,"publicationDate":"2025-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143971174","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Editor’s Picks May 2025","authors":"","doi":"10.1111/jdv.20642","DOIUrl":"https://doi.org/10.1111/jdv.20642","url":null,"abstract":"<p></p><p>Carle Paul</p><p>Hidradenitis suppurativa (HS) represents one of the most burdensome skin diseases and has long been unrecognized and neglected. Zouboulis et al. updated comprehensive management guidelines that will elevate the standard of care in HS. The holistic combination of medical and surgical treatment should help patients experience relief from the enormous impact of the disease. The approval of biological agents over the past few years is transforming patient care and will help modify the disease course (as in the Figure 1 algorithm). We are living a time of new hope in HS.</p><p>Zouboulis, CC, Bechara, FG, Benhadou, F et al. European S2k guidelines for hidradenitis suppurativa/acne inversa part 2: treatment. <i>J Eur Acad Dermatol Venereol</i> 2025; <b>39</b>: 899–941. https://doi.org/10.1111/jdv.20472.</p><p>As we face a shortage of dermatologists in many countries, the exact role of dermatologists in hospitals is frequently challenged based on the public perception of dermatology as a superficial, frivolous specialty. In this analysis from a Swiss University hospital, Cazzaniga et al. show increased marginalization of dermatologists in managing dermatological emergencies (Figure 2), with only 2% of total hospitalized patients admitted to a dermatology ward. These important data reveal the opinion others may have on the specialty and call for action to maintain our expertise in treating patients with dermatological emergencies.</p><p>Cazzaniga, S, Heidemeyer, K, Zahn, CA et al. Dermatological emergencies and determinants of hospitalization in Switzerland: a retrospective study. <i>J Eur Acad Dermatol Venereol</i> 2025; <b>39</b>: 1001–1010. https://doi.org/10.1111/jdv.20176.</p><p>Multidisciplinary care of patients always surpasses individual approaches. Scleromyxedema, a life-threatening condition, is a rare form of sclerodermic disorder associated with monoclonal gammopathy. High-dose IV immunoglobulins are the recommended treatment for scleromyxedema; however, some patients require long-term maintenance, or relapse while still on therapy.</p><p>In this clinical report from dermatologists and haematologists, Theves et al. show that the combination of IV Immunoglobulins and plasma-cell directed treatment with various agents is able to induce long-term remission of scleromyxedema (Figure 3). Indeed, seeing a disease from various angles can lead to medical improvement and tangible patient benefits.</p><p>Theves, F, Lahuna, C, Mahévas, T et al. Plasma cell-directed therapies induce profound clinical and durable responses in patients with severe or relapsed/refractory scleromyxedema. <i>J Eur Acad Dermatol Venereol</i> 2025; <b>39</b>: 1011–1016. https://doi.org/10.1111/jdv.20257.</p><p>Darier disease has long been a difficult-to-treat genodermatosis. Ettinger et al. have summarized the current understanding of Darier disease pathophysiology focusing on chronic skin inflammation (Figure 4) and the potential role of targeting Th1","PeriodicalId":17351,"journal":{"name":"Journal of the European Academy of Dermatology and Venereology","volume":"39 5","pages":"881-882"},"PeriodicalIF":8.4,"publicationDate":"2025-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/jdv.20642","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143871466","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Topical minocycline foam: A new option for acne treatment?","authors":"Evgenia Makrantonaki","doi":"10.1111/jdv.20637","DOIUrl":"https://doi.org/10.1111/jdv.20637","url":null,"abstract":"<p>Acne vulgaris (AV) is a chronic inflammatory disorder of the pilosebaceous unit and is one of the most common dermatological conditions worldwide, affecting an estimated 650 million people.<span>^{1, 2}</span> Abnormalities in several processes (inflammation, sebum production, sebocyte differentiation and <i>P. acnes</i> proliferation) may contribute to the development of acne; hence, most patients require a multi-pronged treatment regimen.<span>^{1, 2}</span> However, the complexity of these regimens can often lead to non-adherence, which is a key factor in treatment failure. Acne is known to lead to scarring and post-inflammatory hyperpigmentation, which can subsequently impact a patient's quality of life. It is therefore vital that treatment is initiated early. While systemic antibiotics, particularly tetracyclines, have historically been a cornerstone of treatment for moderate-to-severe AV, concerns regarding antibiotic resistance and systemic adverse effects (e.g. gastrointestinal, nervous system and liver toxicity) underscore the need for alternative treatment strategies.</p><p>In this context, the study by Le et al.<span>³</span> evaluating the efficacy and safety of topical minocycline foam (FMX101 4%) in Chinese patients with moderate-to-severe facial AV offers a promising therapeutic approach. This phase 3, multi-centre, randomized, double-blind, vehicle-controlled study demonstrated that FMX101 4% significantly reduced the inflammatory lesion count (ILC) at Week 12 compared with vehicle foam (ILC = −21.0 vs. −12.3, <i>p</i> &lt; 0.001). In addition, the Investigator's Global Assessment (IGA) success rate was significantly higher in the treatment group (8.06% vs. 0%, <i>p</i> = 0.002), supporting the clinical efficacy of the formulation. The study also confirmed a favourable safety profile, with treatment-emergent adverse events (TEAEs) predominantly mild to moderate and comparable between the two groups. No treatment-emergent serious adverse events were reported, further demonstrating FMX101 4% as a well-tolerated option.</p><p>The present findings are consistent with those of prior studies conducted outside China, wherein FMX101 4% demonstrated significant efficacy and safety benefits.<span>^{4, 5}</span> However, variations in IGA success rates across studies underscore the necessity for standardized assessment protocols to minimize subjective bias. A notable advantage of FMX101 4% is its capacity to deliver minocycline directly to the pilosebaceous unit while minimizing systemic absorption, thereby reducing the risk of antibiotic resistance and systemic toxicity. This characteristic is of particular relevance in the context of long-term AV management, where the sustained efficacy and tolerability of the treatment are of paramount importance. The findings of the study support the integration of FMX101 4% into clinical practice as an effective and safer alternative to systemic tetracyclines, espec","PeriodicalId":17351,"journal":{"name":"Journal of the European Academy of Dermatology and Venereology","volume":"39 5","pages":"885-886"},"PeriodicalIF":8.4,"publicationDate":"2025-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/jdv.20637","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143871622","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clonal disorders of clinical significance. A concept with important therapeutic implications","authors":"Dan Lipsker","doi":"10.1111/jdv.20598","DOIUrl":"https://doi.org/10.1111/jdv.20598","url":null,"abstract":"&lt;p&gt;Several diseases result from the biological activity of molecules synthesized by cells in various parts of the body. For example, when the cells that synthesize these molecules are those of a solid cancer, leading to various manifestations at distant sites, without a direct link to the tumour, it is referred to as a paraneoplastic syndrome.&lt;/p&gt;&lt;p&gt;Such distant manifestations can also occur with plasma cell dyscrasias. Plasma cells synthesize immunoglobulins. A clonal proliferation of plasma cells synthesizes monoclonal immunoglobulins, either as part of a well-defined hemopathy such as myeloma (IgG, IgA), Waldenström's disease (IgM) or in the context of a monoclonal gammopathy of undetermined significance (MGUS). In 2006, Merlini and Stone introduced the term ‘dangerous small B-cell clones’ stating that ‘even a small clone, as seen in MGUS, may synthesize a noxious monoclonal protein that can be responsible for devastating systemic organ damage dominating the clinical presentation, altering the natural history, and determining the prognosis’.&lt;span&gt;&lt;sup&gt;1&lt;/sup&gt;&lt;/span&gt; This publication paved the way for redefining some cases of MGUS as monoclonal gammopathy of renal significance,&lt;span&gt;&lt;sup&gt;2&lt;/sup&gt;&lt;/span&gt; monoclonal gammopathy of cutaneous significance&lt;span&gt;&lt;sup&gt;3&lt;/sup&gt;&lt;/span&gt; or the generic term monoclonal gammopathy of clinical significance, which includes neurologic to thrombotic manifestations.&lt;/p&gt;&lt;p&gt;The article by Theves et al.&lt;span&gt;&lt;sup&gt;4&lt;/sup&gt;&lt;/span&gt; published in this issue of the &lt;i&gt;Journal&lt;/i&gt; perfectly illustrates this concept. The authors show that targeting plasma cells in patients with scleromyxedema can induce durable responses. Scleromyxedema is a mucinosis characterized by a generalized papular and sclerodermoid eruption that usually occurs in association with a monoclonal gammopathy. It can be an extremely serious disease due to dermato-neuro syndrome, which can be fatal, or because of severe cardiac involvement. Its treatment is challenging. The authors demonstrate that targeting plasma cells yields excellent therapeutic results, supporting the fact that scleromyxedema belongs nosologically to the group of monoclonal gammopathy of cutaneous/clinical significance.&lt;/p&gt;&lt;p&gt;I now wish to extend this concept to all clonal pathologies and introduce the concept of &lt;i&gt;clonal disorders of clinical significance&lt;/i&gt;. Merlini referred to ‘dangerous small B clones’, but there are also dangerous small T clones. We followed up a patient with hypereosinophilic syndrome. She had many manifestations related to hypereosinophilia, including urticarial plaques, widespread red and scaly itchy lesions, enlarged lymph nodes, and a deterioration of her general condition. Treatments targeting hypereosinophilia, such as steroids, interferon or mepolizumab, did not control the clinical manifestations. She had a CD3− CD4+ T-cell clone. We postulated that this T-cell clone was responsible for the secretion of cytokines, such as IL-5, which caused eosinophilia ","PeriodicalId":17351,"journal":{"name":"Journal of the European Academy of Dermatology and Venereology","volume":"39 5","pages":"891-892"},"PeriodicalIF":8.4,"publicationDate":"2025-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/jdv.20598","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143871463","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Interdisciplinary care in immune-mediated inflammatory diseases with skin manifestations within the WHO's people-centred healthcare framework","authors":"Rachel Sommer,&nbsp;Matthias Augustin","doi":"10.1111/jdv.20640","DOIUrl":"https://doi.org/10.1111/jdv.20640","url":null,"abstract":"&lt;p&gt;Immune-mediated inflammatory diseases with skin manifestations (skIMIDs), including chronic inflammatory skin diseases such as psoriasis, are largely systemic conditions. They do not only affect the skin but also can involve the joints, vessels, soft tissues, organs and the psyche and thus require interdisciplinary care, IC (such as dermatologists, rheumatologists, internists, GPs and psychologists). The prevalence of such conditions is estimated to be up to 7% of the Western population.&lt;span&gt;&lt;sup&gt;1&lt;/sup&gt;&lt;/span&gt; As part of a holistic approach to people-centred care as promoted by the World Health Organization,&lt;span&gt;&lt;sup&gt;2&lt;/sup&gt;&lt;/span&gt; not only physician-reported outcomes but also patient-reported outcomes (PRO) are fundamental for clinical decision-making. PROs typically include quality of life but can also encompass a variety of other constructs, such as treatment satisfaction or well-being. PROs are increasingly used to evaluate new therapies and to support the approval and reimbursement of pharmaceuticals. With regard to interdisciplinary treatment and management of patients with IMIDs, a recently published study has examined patient's experiences and perspectives.&lt;span&gt;&lt;sup&gt;3&lt;/sup&gt;&lt;/span&gt; It has been shown that IC models offer advantages for patients with multiple inflammatory diseases compared with conventional care. Patients reported improvements in various aspects of quality of life, including acceptance, optimism, disease understanding, personal development and better disease management.&lt;span&gt;&lt;sup&gt;3&lt;/sup&gt;&lt;/span&gt; In order to implement IC models in the long term, it is important, alongside medical and financial indicators, to be able to demonstrate benefits for both patients and providers. Van den Steen et al.&lt;span&gt;&lt;sup&gt;4&lt;/sup&gt;&lt;/span&gt; have made a significant contribution to this with their systematic review on the evaluation of IC in skIMIDs from patients and health care provider's perspective. Their systematic literature review—including 21 studies—highlighted several advantages of IC from both patients and HCP perspectives. However, the authors found that not only the diseases covered in the reported ICs are heterogeneous but also the measurement methods and instruments used for evaluation, which complicates a sound assessment of IC in skIMIDs. The development and use of standardized core outcome sets for evaluating IC could help address this challenge. A key finding of this systematic review is that tailored local IC models show better outcomes than broader regional IC models. As with usual care, outcomes seem to depend greatly on the involvement and enthusiasm of individual HCPs. This is a crucial factor that, despite all the known benefits of IC compared to usual care, is essential for the long-term and successful implementation. Therefore, in medical education and training, it is essential to emphasize the importance of IC in the management of complex chronic diseases. It is crucial to convey the benefits of IC in order to foster u","PeriodicalId":17351,"journal":{"name":"Journal of the European Academy of Dermatology and Venereology","volume":"39 5","pages":"887-888"},"PeriodicalIF":8.4,"publicationDate":"2025-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/jdv.20640","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143871623","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}