JEADV clinical practice最新文献

{"title":"Skin matrix meets immunomatrix—Implications for genetic and acquired diseases","authors":"Alexander Nyström,&nbsp;Gregor Conradt,&nbsp;Saskia Lehr,&nbsp;Dimitra Kiritsi","doi":"10.1002/jvc2.532","DOIUrl":"https://doi.org/10.1002/jvc2.532","url":null,"abstract":"<p>An extracellular matrix (ECM) is essential for multicellular life. Apart from being a scaffold, it is an actively signalling unit, orchestrating homo- and heterocellular communication to uphold tissue homeostasis or elicit an appropriate regenerative response after injury. The skin as a barrier organ meeting unremittent physical biological and chemical challenges is dependent on both a specialized ECM and attentive yet balanced immune surveillance. Intriguingly, skin-like ECM composites occur in primary and secondary lymphoid organs. Evolutionary, the expansion of the ECM coincides with development of adaptive immunity. Studies of acquired and genetic skin diseases suggest that the skin and lymphoid ECMs are essential, emerging, but yet-under-appreciated, gatekeepers of dermal immune homeostasis. Here, we summarize knowledge of the dermal and skin-distal lymphoid ECM as a mediator of skin immune homeostasis. We argue that increased awareness of the lymphoid-ECM as a potential regulator of skin immunity will increase our understanding of diseases linked to skin inflammation and allow for improved treatment options of them.</p>","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"3 5","pages":"1399-1409"},"PeriodicalIF":0.0,"publicationDate":"2024-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.532","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142762653","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"No need for a p value. Comments on analytical decisions in “Rosacea fulminas: An anti-inflammatory-based therapeutic approach”","authors":"Yung Gonzaga,&nbsp;Ana Luísa Sampaio","doi":"10.1002/jvc2.533","DOIUrl":"https://doi.org/10.1002/jvc2.533","url":null,"abstract":"<p>We read with interest the study by Handgretinger and colleagues entitled <i>Rosacea Fulminans: An Anti-inflammatory-based Therapeutic Approach</i>,<span>¹</span> and although the topic addressed is of great clinical relevance, the presentation of the results, particularly concerning the choice of the statistical test, warrants discussion.</p><p>The study tracked the evolution of the skin condition in six6 patients diagnosed with Rosacea fulminans before and after treatment with azithromycin. According to the methodological section, a <i>t</i> test for independent samples was used to compare the before and after treatment. This choice seems inappropriate to us, as it overlooks some fundamental assumptions in selecting statistical tests that assess the association between two variables.<span>^{2, 3}</span></p><p>The first issue concerns the independence between observations: The ‘before’ and ‘after’ measurements for the same individual are dependent, as they are related to the same subject. The independent samples <i>t</i> test assumes that the observations in each group are independent, which is not the case here.</p><p>The second issue concerns the nature of the variable being studied. The Clinical Erythema Assessment (CEA), for example, is an ordinal categorical variable. For instance, on a scale from 0 to 4, as in this case, the differences between ‘0 and 1’ may not represent the same quantitative change as a difference between ‘3 and 4.’ The <i>t</i> test assumes that the data are numbers, meaning that the differences between the values are consistent and quantifiable. For example, the difference between ages 25 and 20 is the same as the difference between 37 and 32, which is 5 years. The <i>t</i> test works with numerical variables, making it inappropriate for dealing with categorical variables, even if they are represented by numbers and have a natural order.</p><p>The third issue pertains to the necessity of a statistical test at all. In this regard, we would like to emphasize that the reason for writing this letter is not merely to criticize the choice of the statistical test, but to highlight that the value of a study like this should not rely on statistical significance. It is much more related to the descriptive and visual nature capable of creating a clear sensorial perception of clinical benefit and generating a relevant hypothesis to be explored more thoroughly in future studies.<span>⁴</span> In this regard, in our opinion, the study by Handgretinger and colleagues achieves the goal. It is a beautiful and illustrative case series that does not need <i>p</i> values to demonstrate its importance.</p><p>Yung Gonzaga conceived and wrote the article. Ana Luísa Sampaio wrote and reviewed the article.</p><p>The authors declare no conflict of interest.</p><p>Not applicable.</p>","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"3 5","pages":"1711-1712"},"PeriodicalIF":0.0,"publicationDate":"2024-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.533","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142762654","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Extensive polymorphic eruption and lymphocytosis in a 56-year-old male—A difficult diagnosis!","authors":"Berbie Byrne,&nbsp;Marion Leahy,&nbsp;Kevin Molloy,&nbsp;Mary Laing","doi":"10.1002/jvc2.484","DOIUrl":"https://doi.org/10.1002/jvc2.484","url":null,"abstract":"&lt;p&gt;A 56-year-old male presented with a 5-month history of a pruritic, papular truncal rash with partial response to oral steroids. He reported no systemic symptoms. Initial differential diagnosis included dermatitis herpetiformis, drug-induced rash, a viral exanthem and lymphomatoid papulosis. Multiple skin biopsies were inconclusive, demonstrating a normal epidermis with mixed B-cell and T-cell dermal lymphohistiocytoc infiltrate. A skin biopsy for direct immunofluorescence was negative.&lt;/p&gt;&lt;p&gt;Laboratory investigations revealed mild iron deficient anaemia and CT Thorax Abdomen Pelvis (CT TAP) and colonoscopy confirmed diverticulosis. The patient responded minimally to oral doxycycline, topical clobetasol proprionate and antihistamines.&lt;/p&gt;&lt;p&gt;After 18 months he developed rapid extensive skin involvement with atypical targetoid polymorphic plaques on his limbs, pink coalescing urticated plaques with a geographical border on his trunk (Figure 1a), and ecchymosis of the anterior shoulders (Figure 1b). Fixed, annular, scaly plaques were evident on the dorsum of his hands (Figure 2a) and bilateral thighs (Figure 2b). This was associated with severe itch, fatigue and dyspnoea. Investigations revealed a lymphocytosis (44.7 × 10̂&lt;sup&gt;9&lt;/sup&gt;/L) thrombocytopenia (72 × 10̂&lt;sup&gt;9&lt;/sup&gt;/L), anaemia (Hb 9.4 × 10̂&lt;sup&gt;9&lt;/sup&gt;/L) and atypical lymphocytes on blood film. CT imaging confirmed widespread lymphadenopathy.&lt;/p&gt;&lt;p&gt;A skin biopsy was done (Figure 3a,b).&lt;/p&gt;&lt;p&gt;The rapid deterioration in our patient after 18 months with extensive cutaneous involvement, in association with systemic symptoms, widespread lymphadenopathy and lymphocytosis progressed the differential diagnosis to include Sezary syndrome or transformed mycosis fungoides (T-MF). Clinically the patient was not erythrodermic and did not meet Sezary syndrome diagnostic criteria&lt;span&gt;&lt;sup&gt;4&lt;/sup&gt;&lt;/span&gt; and no Sezary cells were identified in the skin, blood, or lymph nodes. Regarding T-MF, the patient did not progress through the patch-plaque stages of MF and skin biopsy did not reveal features of MF such as epidermotropism or pautrier microabscess. There was a patchy expression of CD30+ cells but significantly less than 25% of the infiltrate required to meet the diagnostic criteria of T-MF.&lt;span&gt;&lt;sup&gt;5&lt;/sup&gt;&lt;/span&gt;&lt;/p&gt;&lt;p&gt;The atypical T-cell population and T-cell clonality identified on multiple BM, LN and skin biopsies resulted in a suspected diagnosis of leukemic dissemination of peripheral T-cell lymphoma not otherwise specified (PTCL NOS), but formal classification remained challenging. PTCL NOS is a heterogeneous group of aggressive nodal and extranodal T-cell lymphomas that are not characterized by any known clinicopathological criteria.&lt;span&gt;&lt;sup&gt;6&lt;/sup&gt;&lt;/span&gt;&lt;/p&gt;&lt;p&gt;Following expert hematopathology review the histology, molecular results and immunophenotypic studies were repeated. Among the BM, LN and skin specimens three TFH markers, namely CD10, BCL6 and PD1, were collectively identified th","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"3 5","pages":"1745-1748"},"PeriodicalIF":0.0,"publicationDate":"2024-08-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.484","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142762723","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A case of mogamulizumab-induced vitiligo in a patient with mycosis fungoides","authors":"Charissa N. Obeng-Nyarko,&nbsp;Ciara G. Robinson,&nbsp;Anna R. Axelson","doi":"10.1002/jvc2.523","DOIUrl":"https://doi.org/10.1002/jvc2.523","url":null,"abstract":"&lt;p&gt;Vitiligo is an acquired disorder of pigmentation characterized by the development of asymptomatic, well-defined, depigmented macules and/or patches on the skin. The pathogenesis involves an interplay of genetic, environmental triggers, autoimmunity and oxidative stress mechanisms, thus leading to chemokine-driven melanocyte destruction by autoreactive cytotoxic CD8+ T lymphocytes.&lt;span&gt;&lt;sup&gt;1&lt;/sup&gt;&lt;/span&gt;&lt;/p&gt;&lt;p&gt;Drug-induced vitiligo has been associated with biologic medications, including monoclonal antibodies, TNF-⍺ inhibitors, and interleukin inhibitors. Vitiligo-like depigmentation has also been reported in patients on immune checkpoint inhibitors.&lt;span&gt;&lt;sup&gt;2&lt;/sup&gt;&lt;/span&gt; Mogamulizumab is an anti-CC chemokine receptor-4 (CCR4) monoclonal antibody used to treat recalcitrant mycosis fungoides (MF) and Sézary syndrome (SS). The most common dermatologic adverse event is the ‘mogamulizumab-associated’ rash characterized by papules/plaques, folliculotropic MF-like plaques and cutaneous granulomatous drug eruption.&lt;span&gt;&lt;sup&gt;3, 4&lt;/sup&gt;&lt;/span&gt; Alopecia areata universalis&lt;span&gt;&lt;sup&gt;5&lt;/sup&gt;&lt;/span&gt; and a few cases of vitiligo have been reported.&lt;span&gt;&lt;sup&gt;6, 7&lt;/sup&gt;&lt;/span&gt; In this case report, we add to the existing literature by presenting another case of mogamulizumab-induced vitiligo.&lt;/p&gt;&lt;p&gt;An 83-year-old African American male with a history of hypertension and stage IV-A2 MF with leukaemic disease and lymph node involvement presented to the dermatology clinic for follow-up visit and complaint of skin discoloration. The patient's initial peripheral blood smear revealed 60% atypical lymphocytes with cerebriform morphology. Flow cytometry findings revealed CD4:CD8 ratio of 48:1, and CD4+ population was divided into: 89% CD7− and 91% CD26−. His skin disease was initially controlled on topical corticosteroids; however, he began to have progression of his MF as evidenced by erythroderma, persistent skin erosions, new lymphadenopathy, and multiple hospitalizations for superinfection. Repeat biopsies were consistent with MF and negative for infection. Due to progression and lack of adequate disease control on topical corticosteroids and pulse dexamethasone, mogamulizumab was recommended and subsequently initiated. Approximately 6 months following the initial dose of mogamulizumab, the patient developed depigmented macules and patches on his bilateral upper extremities that slowly progressed in a symmetrical, generalized distribution consistent with non-segmental vitiligo (Figures 1a,b; 2a,b; and 3). The patient's vitiligo was not affecting him psychosocially, and he was not interested in pursuing treatment.&lt;/p&gt;&lt;p&gt;In this report, we present the case of a patient who developed vitiligo 6 months following the initiation of mogamulizumab. In Algarni et al.'s report of three cases, patients presented with well-demarcated hypopigmented patches ranging from acrofacial to scalp, truncal and upper extremity distribution from 6 to 8 months after initial treatment ","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"3 5","pages":"1705-1707"},"PeriodicalIF":0.0,"publicationDate":"2024-08-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.523","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142762724","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A toolkit to facilitate skin research participation in underrepresented ethnic populations: A co-designed, mixed methods refinement exercise","authors":"S. P. Choy,&nbsp;M. Naveed,&nbsp;J. Prasad,&nbsp;K. Quadry,&nbsp;L. Moorhead,&nbsp;C. H. Smith,&nbsp;S. K. Mahil","doi":"10.1002/jvc2.516","DOIUrl":"https://doi.org/10.1002/jvc2.516","url":null,"abstract":"&lt;div&gt;\u0000 \u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Background&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;Inclusive, generalisable research is vital to inform evidence-based patient care. However, people from ethnic minority backgrounds remain underrepresented in research, increasing health disparities in under-served communities.&lt;/p&gt;\u0000 &lt;/section&gt;\u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Objectives&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;Codevelop a toolkit to increase the representation of people from ethnic minority groups in skin research.&lt;/p&gt;\u0000 &lt;/section&gt;\u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Methods&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;Our four-phase approach comprised: (1) focus group discussions with individuals from ethnic minority groups with skin diseases to identify barriers and enablers to participate in skin research, (2) a narrative literature review, (3) development of a skin research inclusion toolkit, (4) dissemination of findings.&lt;/p&gt;\u0000 &lt;/section&gt;\u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Results&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;Focus group discussions (phase 1) identified a positive value model (belief in the value of research) and inclusive recruitment strategies (e.g. strong patient–recruiter relationships) as enablers to participation. Barriers included mistrust in research (e.g. poor information on personal data use) and social stigma of skin disease. Our narrative literature review (phase 2) reinforced these themes. Social stigma may accentuate feelings of shame or embarrassment associated with a skin condition. Mistrust is accentuated by a lack of information or understanding about research processes. Understanding distinct motivators for research participation across ethnic groups may help to cultivate a positive value model. Inclusive recruitment strategies should be codeveloped with populations of interest and culturally competent research teams to build lasting partnerships.&lt;/p&gt;\u0000 \u0000 &lt;p&gt;Phases 1-2 informed a skin research inclusion toolkit (phase 3), which recommends researchers formulate (i) an inclusion plan during study design (e.g. consider differences in pathophysiology of skin diseases across ethnic groups), (ii) inclusive enrolment strategies (e.g. skin-of-colour education to clinicians and patients to address disease-associated stigma, codevelop study materials including translations) and (iii) retention strategies (e.g. time or travel reimbursement, timely feedback of findings). In phase 4, findings were disseminated to focus group participants.&lt;/p&gt;\u0000 &lt;/section&gt;\u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Conclusions&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;Our co-designed toolkit has the potential to improve ethnic diversity in skin research cohorts to enable more representati","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"3 5","pages":"1548-1557"},"PeriodicalIF":0.0,"publicationDate":"2024-08-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.516","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142762607","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Differences in frequency of vulvar dermatoses according to ethnicity: A study of a cohort of patients in a large diverse UK teaching hospital","authors":"Majeeda Patel,&nbsp;Jade Simpson,&nbsp;Beth Stuart,&nbsp;Sujatha Thamban,&nbsp;Arucha Ekeowa-Anderson","doi":"10.1002/jvc2.529","DOIUrl":"https://doi.org/10.1002/jvc2.529","url":null,"abstract":"&lt;p&gt;The National Health Service Hospital Trust in East London, where we are based, serves an ethnically diverse population. The population of England and Wales as a total are 82% White, with the remaining 18% from Black, Asian, Mixed or Other ethnic groups. Tower Hamlets Borough, where one of our hospitals is based, had a population mix 41.8% Asian, 39.3% White and 7.4% Black in the 2021 UK census.&lt;span&gt;&lt;sup&gt;1&lt;/sup&gt;&lt;/span&gt; There is little data on the prevalence of different vulvar dermatoses in different ethnic groups. Lichen sclerosus is a common vulvar dermatosis in Caucasian women but we wanted to investigate the case mix across different ethnicities. We collected information on diagnosis and ethnicity in the population of patients attending the Vulvar Clinics at our hospitals over a total of 6 months in 2022 and 2023. The months were split to avoid duplication of patients and only data from individual patients was analysed. Diagnosis was made by a consultant dermatologist with subspecialty expertise in vulvar disease, sometimes in joint consultation with a consultant gynaecologist.&lt;/p&gt;&lt;p&gt;Data from 246 individual patients was collected. UK census data is collected into five ethnic groups as follows:—‘White’, ‘Asian or Asian British’, ‘Black or Black British, Caribbean or African’, ‘Mixed/Multiple’ and lastly ‘Other’ ethnic group.&lt;/p&gt;&lt;p&gt;The majority of patients seen were White (128/246) with 83 Asian, 17 Black, 15 Mixed/Multiple and 3 from ‘Other’ ethnic groups. Within the Asian group, the most common subdivisions included Asian Bangladeshi (38/83), Asian Indian (12), Asian Pakistani (14) and Asian other/non stated (19).&lt;/p&gt;&lt;p&gt;The mean age of patients in the three largest cohorts was 61 years—White, 46.2 years—Asian and 52.9 years—Black.&lt;/p&gt;&lt;p&gt;The five most common diagnoses varied according to ethnicity and are tabulated below (Table 1).&lt;/p&gt;&lt;p&gt;Lichen sclerosus was the most frequently diagnosed vulvar dermatosis in all ethnic groups except Asian. The mean age of patients of Asian ethnicity was also lower than the other groups. In Asian patients, lichen simplex was the most frequent diagnosis made. Further analysing the data, &lt;i&gt;χ&lt;/i&gt;&lt;sup&gt;2&lt;/sup&gt; test was performed on the 225 patients with diagnoses of either lichen sclerosus or lichen simplex (Table 2). There were statistically significant differences in patients receiving each diagnosis; according to ethnicity.&lt;/p&gt;&lt;p&gt;Controlling for age, the differences seen in each ethnic group were not statistically significant for lichen sclerosus. Results for lichen simplex were, although confidence intervals were wide. The increased odds of a lichen simplex diagnosis was 20.2 times higher in Asian patients (95% confidence interval [CI]: 4.48, 91.41; &lt;i&gt;p&lt;/i&gt; &lt; 0.001) and 11.71 times higher in Black patients (95% CI: 1.77, 77.59; &lt;i&gt;p&lt;/i&gt; = 0.011) compared with White.&lt;/p&gt;&lt;p&gt;These results suggest lichen simplex of the vulva is more frequently the diagnosis behind a vulval presentation in Asian and Black pa","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"3 5","pages":"1708-1710"},"PeriodicalIF":0.0,"publicationDate":"2024-08-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.529","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142762679","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cutaneous presentations of systemic sclerosis in skin of colour: A systematic review","authors":"Alicia Podwojniak,&nbsp;Isabella J. Tan,&nbsp;Aliza Leiter,&nbsp;Hira Ghani,&nbsp;Bernard A. Cohen","doi":"10.1002/jvc2.527","DOIUrl":"https://doi.org/10.1002/jvc2.527","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 <p>Systemic sclerosis (SSc), a rheumatologic autoimmune disease, can present diversely, especially in patients with skin of colour (SOC). Differences in clinical manifestations among SOC individuals compared to predominantly White populations require a comprehensive examination to optimize diagnosis and treatment. This systematic review aims to explore the differences in SSc manifestations among individuals with SOC, identifying variations in disease severity, clinical features and psychosocial impacts. A systematic review of the literature was conducted following PRISMA guidelines. Databases searched included PubMed, SCOPUS, Cochrane and Web of Science, focusing on studies published within the last 10 years. Nine studies were selected and qualitatively analysed due to the heterogeneity of study types. Key findings highlight significant variations in disease severity and cutaneous clinical features among SOC cohorts compared to predominantly White populations. Raynaud's phenomenon was prevalent across all ethnicities; however, SOC groups exhibited fewer hallmark features of scleroderma, such as telangiectasias, calcinosis and digital swelling, which complicates diagnosis. SOC individuals showed a higher propensity for severe pulmonary complications and increased mortality rates. Atypical cutaneous presentations in SOC were associated with disease progression. Psychosocial impacts were more pronounced in SOC individuals, with greater awareness and dissatisfaction over cutaneous symptoms. Recognizing the atypical presentations of SSc in SOC is critical for early diagnosis and optimizing patient outcomes. The study underscores the need for further research to determine if observed differences are due to variations in prevalence, genetic factors or insufficient training in recognizing cutaneous manifestations in SOC. Enhanced awareness and targeted training for healthcare providers are essential to address these diagnostic challenges and improve care for SOC patients with SSc.</p>\u0000 </section>\u0000 </div>","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"3 5","pages":"1420-1429"},"PeriodicalIF":0.0,"publicationDate":"2024-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.527","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142762718","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Posttransplantation course after allogeneic stem cell transplantation in a patient with advanced mycosis fungoides—Balancing relapse and rejection","authors":"Inga Hansen-Abeck,&nbsp;Chiara L. Blomen,&nbsp;Finn Abeck,&nbsp;Leopold Torster,&nbsp;Stefan W. Schneider,&nbsp;Nina Booken","doi":"10.1002/jvc2.514","DOIUrl":"https://doi.org/10.1002/jvc2.514","url":null,"abstract":"<p>Treatment of advanced cutaneous T-cell lymphoma (CTCL) can be challenging. To date, the only potentially curative treatment option for advanced CTCL is allogeneic hematopoietic stem cell transplantation. We report on a patient with mycosis fungoides who received allogeneic hematopoietic stem cell transplantation due to rapid progression of the disease. Eight months after transplantation, relapse as well as chronic graft-versus-host disease occurred. Therefore, we initiated different treatment modalities, including bexarotene, extracorporeal photopheresis, topical treatment as well as the Janus-kinase-inhibitor Ruxolitinib. Even though the patient experienced high morbidity due to the allogeneic hematopoietic stem cell transplantation, 3 years after he is still alive and reports good health-related quality of life. With this case, we aim to demonstrate that the posttransplant course can be difficult, balancing between relapse and graft-versus-host disease. Nevertheless, patients can benefit in terms of survival and their health-related quality of life. Therefore, allogeneic hematopoietic stem cell transplantation should be considered as a treatment option, especially for patients with advanced CTCL and poor prognosis.</p>","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"3 5","pages":"1660-1664"},"PeriodicalIF":0.0,"publicationDate":"2024-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.514","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142762755","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Dupilumab-related ocular surface disorders in a paediatric cohort with atopic dermatitis, treated in a tertiary paediatric hospital in London","authors":"Anjali Rampersad,&nbsp;Karolina Gholam,&nbsp;Lea Solman,&nbsp;Natalia Cartledge,&nbsp;Sri Gore,&nbsp;Gabriela Petrof","doi":"10.1002/jvc2.528","DOIUrl":"https://doi.org/10.1002/jvc2.528","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Dupilumab is the first human monoclonal antibody approved for the treatment of moderate–severe atopic dermatitis (AD) in children from 6 years of age. Real-world studies reviewing dupilumab-related ocular surface disorders (DROSD) in the paediatric population are needed to detail ophthalmological findings.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>This cross-sectional observational study aimed to review the incidence, features, and ophthalmological findings of DROSD in a real-world paediatric cohort, treated with dupilumab for AD.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Pharmacy and electronic medical records were used to identify all patients prescribed dupilumab for AD from April 2019 to July 2022. Data, including demographics, ocular signs and symptoms, severity and treatments, were collected and analysed.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>We identified 46 patients, with a median age of treatment initiation of 12 years (range 9–14 years). Twelve patients (26.1%) developed DROSD. Mean time to development of DROSD was 4.1 months (±2.4 months, 95% confidence interval). Two patients (16.7%) with DROSD had severe eye findings including peripheral corneal opacification, one of which had reported only asymptomatic red eyes. Ocular signs included 91.7% (11/12) bulbar conjunctival injection, 41.7% (5/12) follicle involvement (limbal or forniceal), 25% (3/12) diffuse tarsal-conjunctival thickening, 16.7% (2/12) featureless thickening, 16.7% (2/12) peripheral corneal opacification, 8.3% (1/12) cicatrisation and 8.3% (1/12) periorbital skin changes. No patients ceased treatment due to DROSD.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>This study had a higher incidence of DROSD, compared to other real-world paediatric studies but had a similar incidence to real-world adult studies. Patient-reported eye symptoms did not always correlate to severity of DROSD. Larger paediatric studies looking at the incidence and long-term outcome of DROSD are needed.</p>\u0000 </section>\u0000 </div>","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"3 5","pages":"1558-1563"},"PeriodicalIF":0.0,"publicationDate":"2024-08-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.528","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142762639","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Investigating Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) reported in association with HIV antiretroviral therapy: An analysis of the FDA Adverse Event Reporting System","authors":"Bianca E. Ituarte,&nbsp;Mitchell A. Taylor,&nbsp;Erin X. Wei,&nbsp;Jennifer Adams","doi":"10.1002/jvc2.513","DOIUrl":"https://doi.org/10.1002/jvc2.513","url":null,"abstract":"&lt;p&gt;Antiretroviral medications used to treat and prevent human immunodeficiency virus (HIV) are the cornerstone in management and should be initiated as early as possible.&lt;span&gt;&lt;sup&gt;1&lt;/sup&gt;&lt;/span&gt; These medications are widely regarded as efficacious, with benefits outweighing potential risks and contributing to longer, healthier lives for patients. Previous literature has described nevirapine and raltegravir, two commonly prescribed medications, as associated with the development of Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS);&lt;span&gt;&lt;sup&gt;2, 3&lt;/sup&gt;&lt;/span&gt; however, little is known regarding reports of cutaneous drug eruptions among other antiretrovirals. This study seeks to provide a classification of reported events of DRESS in relation to the use of antiretrovirals for HIV prevention and treatment.&lt;/p&gt;&lt;p&gt;The FDA Adverse Event Reporting System (FAERS) Public Dashboard was used to extract all available data through 31 December 2023 for the analysis of reports of DRESS in relation to 40 different HIV antiretroviral medications. These medications were categorized into seven groups: combination antiretrovirals (cARTs), nucleoside reverse transcriptase inhibitors (NRTIs), non-NRTIs (NNRTIs), entry inhibitors (EIs), protease inhibitors (PIs), integrase inhibitors (INSTIs), and boosting agents (BAs). A full description of each medication included can be found in Table 1. To ensure broad data collection, both brand and generic names were included for each unique medication. The frequencies of both DRESS and total cutaneous-specific adverse events were recorded in Figure 1.&lt;/p&gt;&lt;p&gt;Among the 40 unique antiretroviral medications analysed, there were 30,353 cutaneous-specific adverse events. Among these, 1295 (4.3%) were cases of DRESS. Six relatively new medications (with brand name—drug class) that had no reported cases at the time of data collection were as follows: dolutegravir/lamivudine (Dovato—cART), doravirine/lamivudine/tenofovir disoproxil (Delstrigo—cART), darunavir/cobicistat/emtricitabine/tenofovir alafenamide (Symtuza—cART), atazanavir/cobicistat (Evotaz—PI), nelfinavir (Viracept—PI), and ibalizumab-uiyk (Trogarzo—EI). Individual antiretrovirals most reported in association with DRESS were raltegravir (Isentriss—INSTI) (15.0% of DRESS cases, &lt;i&gt;n&lt;/i&gt; = 194), ritonavir (Norvir—BA) (10.5%, &lt;i&gt;n&lt;/i&gt; = 136), atazanavir (Reyataz—PI) (10.2%, &lt;i&gt;n&lt;/i&gt; = 132), lopinavir/ritonavir (Kaletra—PI) (8.3%, &lt;i&gt;n&lt;/i&gt; = 107), and emtricitabine/tenofovir (Truvada—cART) (7.8%, &lt;i&gt;n&lt;/i&gt; = 101). Nevirapine (Viramune—NNRTI) had 71 reported cases (5.5%). Among medication classes, the most reported in association with DRESS was PI (27.8%, &lt;i&gt;n&lt;/i&gt; = 359), followed by cART (20.3%, &lt;i&gt;n&lt;/i&gt; = 263) and NRTIs (12.4%, &lt;i&gt;n&lt;/i&gt; = 160). The newest class of antiretroviral therapy, the EIs, was the lowest reported class, with 10 total cases at the time of data collection.&lt;/p&gt;&lt;p&gt;This study provides a collection of reported cases of DRESS associated with antiretrov","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"3 5","pages":"1701-1704"},"PeriodicalIF":0.0,"publicationDate":"2024-08-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.513","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142762792","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}