JEADV clinical practice最新文献

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Itch improvement has a major and comparable effect on the Dermatology Life Quality Index in psoriasis and atopic dermatitis patients 瘙痒的改善对银屑病和特应性皮炎患者的皮肤病生活质量指数具有重要且可比的影响
JEADV clinical practice Pub Date : 2024-08-08 DOI: 10.1002/jvc2.509
Wolf-Henning Boehncke, Adam Reich, Sonja Ständer, Julio Hernandez, Can Mert, Susanne Grond, Maria Jose Rueda, Christopher Schuster, Gil Yosipovitch
{"title":"Itch improvement has a major and comparable effect on the Dermatology Life Quality Index in psoriasis and atopic dermatitis patients","authors":"Wolf-Henning Boehncke,&nbsp;Adam Reich,&nbsp;Sonja Ständer,&nbsp;Julio Hernandez,&nbsp;Can Mert,&nbsp;Susanne Grond,&nbsp;Maria Jose Rueda,&nbsp;Christopher Schuster,&nbsp;Gil Yosipovitch","doi":"10.1002/jvc2.509","DOIUrl":"10.1002/jvc2.509","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Itch is known to have a particularly high impact on psoriasis (PsO) and atopic dermatitis (AD) patients' quality of life. Although AD therapies have exhibited a high efficacy when it comes to itch control, itch control with PsO therapies is not as well documented.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>The aim of this post-hoc analysis is to better understand the impact of itch on the patients' quality of life in PsO as well as AD by providing a pairwise correlation between itch improvement and patients' quality of life and determine the predictive factors in patients achieving Dermatology Life Quality Index score of 0 or 1 [DLQI (0/1)].</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Three phase III clinical studies, one in PsO and two in AD, were assessed. Pairwise correlations between objective improvement of visible signs of disease, quality of life, and itch intensity were investigated at 16 weeks of treatment. Predictive analyses methods were applied on the data to assess the impact of clinical and itch improvement on the DLQI improvement.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>This study shows that change in itch from baseline in AD and PsO patients correlate to change in DLQI from baseline. Change in itch from baseline was found to be the most important factor in predicting DLQI (0/1).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>These results highlight the necessity to study itch in both PsO and AD clinical trials, and it is recommended that itch may be considered a coprimary or at minimum a secondary efficacy end-point in all such clinical studies.</p>\u0000 </section>\u0000 </div>","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"3 5","pages":"1509-1517"},"PeriodicalIF":0.0,"publicationDate":"2024-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.509","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141927095","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}
引用次数: 0
Access to information, sun protection and dermatological care in patients with xeroderma pigmentosum in Nepal 尼泊尔色素性干皮病患者获取信息、防晒和皮肤科护理的途径
JEADV clinical practice Pub Date : 2024-08-01 DOI: 10.1002/jvc2.494
Marie Fournier, Vilok Mishra, Upama Paudel, Anne Grange, Sudip Parajuli, Florent Grange
{"title":"Access to information, sun protection and dermatological care in patients with xeroderma pigmentosum in Nepal","authors":"Marie Fournier,&nbsp;Vilok Mishra,&nbsp;Upama Paudel,&nbsp;Anne Grange,&nbsp;Sudip Parajuli,&nbsp;Florent Grange","doi":"10.1002/jvc2.494","DOIUrl":"https://doi.org/10.1002/jvc2.494","url":null,"abstract":"&lt;p&gt;We previously highlighted the clinical and genetic characteristics of xeroderma pigmentosum (XP) in Nepal, dominated by a high frequency not related to consanguinity, the significant severity of the disease, the absence of neurological abnormalities and the homozygous specific XPC mutation c.1243 C &gt; T, p.R415X in 15/17 cases, suggesting a founder effect.&lt;span&gt;&lt;sup&gt;1&lt;/sup&gt;&lt;/span&gt;&lt;/p&gt;&lt;p&gt;Severe XP (particularly XPC in Nepal) frequently manifests early in childhood by multiple freckle-like spots and pre-cancerous lesions, followed by skin and/or eye cancers &lt;span&gt;&lt;sup&gt;2&lt;/sup&gt;&lt;/span&gt; (Figure 1). No curative treatment is available. Only extreme photoprotection can prevent skin cancers. Close follow-up by a dermatologist every 3 to 6 months is recommended.&lt;span&gt;&lt;sup&gt;2&lt;/sup&gt;&lt;/span&gt; Studies have shown that patients’ understanding oftheir disease and adherence to skin and eye protection are crucial, and that poor compliance and lack of knowledge and awareness may dramatically reduce lifespan.&lt;span&gt;&lt;sup&gt;3-5&lt;/sup&gt;&lt;/span&gt;&lt;/p&gt;&lt;p&gt;To complete and extend previous results in Nepal, we conducted a prospective, non-interventional study in the department of Dermatology and Venereology, Maharajgunj Medical Campus Tribhuvan University, a tertiary referral centre of the country. A standardised questionnaire (Table 1) was offered to patients (and/or parents for minors) consulting between August 2022 and April 2023, including questions about their lifestyle, medical history, knowledge of the disease, awareness of sun risk, means of photoprotection and access to medical care and dermatological follow-up. All participating patients and/or families gave their informed consent.&lt;/p&gt;&lt;p&gt;Thirty out of 80 patients seen at least once for XP were included, aged between 19 months and 58 years (median: 16.5 years). Half (&lt;i&gt;n&lt;/i&gt; = 15) had a family history of XP, with an average of two affected children per family. The median age at diagnosis was 4 years. Median time from diagnosis to start of follow-up was 4 years.&lt;/p&gt;&lt;p&gt;One-third (&lt;i&gt;n&lt;/i&gt; = 10) were followed by a dermatologist at least twice a year; the others were only followed once a year (&lt;i&gt;n&lt;/i&gt; = 11) or less (&lt;i&gt;n&lt;/i&gt; = 9).&lt;/p&gt;&lt;p&gt;Half of the patients had previous skin carcinomas removed, usually multiple (2 to &gt;10). Only 2/30 took vitamin D supplements. Eight patients or parents (27%) were unaware of the genetic/hereditary nature of XP. Nearly all (29/30) knew sun exposure could be deleterious, but 9/30 did not know what ultraviolet (UV) rays were and 10/30 identified direct outside exposure on sunny days as the only risk factor. None had an easy-to-use UV radiation metre. Some 13/30 reported outdoor activities during more than 8 h a week.&lt;/p&gt;&lt;p&gt;Worryingly, 8/27 patients of school age or older (30%) were not in education or employment. Six (20%) had a UV-resistant face mask, as recommended, but only two wore it every day while outside; the other four wore it rarely or never, being afraid of 'how others would look at","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"3 5","pages":"1677-1681"},"PeriodicalIF":0.0,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.494","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142762294","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}
引用次数: 0
Evaluation of public perception towards patients with hidradenitis suppurativa and atopic dermatitis 公众对化脓性皮炎和特应性皮炎患者认知的评价
JEADV clinical practice Pub Date : 2024-08-01 DOI: 10.1002/jvc2.489
Jonathan D. Greenzaid, Matthew C. Johnson, Steven R. Feldman
{"title":"Evaluation of public perception towards patients with hidradenitis suppurativa and atopic dermatitis","authors":"Jonathan D. Greenzaid,&nbsp;Matthew C. Johnson,&nbsp;Steven R. Feldman","doi":"10.1002/jvc2.489","DOIUrl":"https://doi.org/10.1002/jvc2.489","url":null,"abstract":"&lt;p&gt;Dear Editor,&lt;/p&gt;&lt;p&gt;Hidradenitis suppurativa (HS) and atopic dermatitis (AD) are chronic inflammatory cutaneous disorders associated with psychosocial distress. The stigma of HS and AD often creates a burden on patients, leading to a negative self-image and higher rates of anxiety and depression.&lt;span&gt;&lt;sup&gt;1-3&lt;/sup&gt;&lt;/span&gt; However, few studies investigate the general public's endorsement of stigmatizing attitudes towards people with cutaneous diseases.&lt;span&gt;&lt;sup&gt;4, 5&lt;/sup&gt;&lt;/span&gt; We aimed to analyze the perception towards individuals with HS and AD and evaluate factors leading to higher rates of stigmatization.&lt;/p&gt;&lt;p&gt;A 51-question survey was distributed to subjects over the age of 18 on the online survey recruitment platform Amazon Mechanical Turk (MTurk). To improve survey reliability, a series of four screening questions testing participants' attentiveness were randomly embedded throughout the survey. Patients were ineligible for the study if they answered one of the screening questions incorrectly. The survey displayed two images each of moderate-to-severe HS and AD and then examined participants' attitudes towards these images. The questions measuring stigma were separated into three categories based on previous studies&lt;span&gt;&lt;sup&gt;4, 5&lt;/sup&gt;&lt;/span&gt;: Desire to Social Distance, Stereotype Endorsement and Belief in AD Myths. Each question was scored on a 1–5 Likert scale, with 1 being the most stigmatizing attitude and 5 the least. Responses that were scored as a 1 or 2 were considered as the endorsement of stigmatizing attitudes when completing statistical analysis. Fisher's exact test was used to compare differences in attitudes towards patients with HS versus AD, and patients currently affected or with a history of HS/AD versus those with no history of HS/AD.&lt;/p&gt;&lt;p&gt;A total of 497 participants responded to the survey, of which 350 answered the screening questions correctly (70% response rate, Table 1). Participants reported not feeling comfortable hiring someone with HS and AD (17.4% vs. 18.3%, &lt;i&gt;p&lt;/i&gt; = 0.8), believing patients with HS and AD have poor hygiene (27.7% and 28.0%, &lt;i&gt;p&lt;/i&gt; = 0.9) and endorsed the myth that people with HS and AD are to blame for their condition (64.3% vs. 64.6%, &lt;i&gt;p&lt;/i&gt; = 1.0, Table 2). There was a minimal difference in the stigma towards individuals with HS versus AD. Survey respondents had more stigmatizing attitudes towards wanting to date an individual with AD compared to HS (mean Likert score 3.49 vs. 3.68, &lt;i&gt;p&lt;/i&gt; = 0.03), and this was the only statistically significant difference when comparing average Likert scores towards individuals with HS versus AD. Participants not currently affected or without a history of HS or AD had more stigmatizing attitudes compared to individuals with a history of HS or AD (&lt;i&gt;p&lt;/i&gt; &lt; 0.0001, Table 2).&lt;/p&gt;&lt;p&gt;Patients with HS and AD often experience reduced quality of life, which is in part due to the perceived stigma of having cutaneous disease. Our study is limited by","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"3 5","pages":"1682-1685"},"PeriodicalIF":0.0,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.489","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142762295","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}
引用次数: 0
Sjögren syndrome from a dermatological perspective: A retrospective study of 185 SSA-Ro positive patients 从皮肤科角度看斯约格伦综合征:185 例 SSA-Ro 阳性患者的回顾性研究
JEADV clinical practice Pub Date : 2024-07-19 DOI: 10.1002/jvc2.498
Fiona André, Martha Bujak, Nina Frischhut, Andreas Kronbichler, Norbert Sepp, Gudrun Ratzinger, Barbara Böckle
{"title":"Sjögren syndrome from a dermatological perspective: A retrospective study of 185 SSA-Ro positive patients","authors":"Fiona André,&nbsp;Martha Bujak,&nbsp;Nina Frischhut,&nbsp;Andreas Kronbichler,&nbsp;Norbert Sepp,&nbsp;Gudrun Ratzinger,&nbsp;Barbara Böckle","doi":"10.1002/jvc2.498","DOIUrl":"10.1002/jvc2.498","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Sjögren syndrome (SS) is a systemic autoimmune disease characterised by oral and ocular mucosal dryness.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>Our study focused on skin manifestations of SS. The differences between primary SS (pSS) and secondary SS (sSS) were investigated. Furthermore, we elucidated factors predicting internal organ involvement.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This was a single centre retrospective cohort study. We analysed clinical and laboratory data from 185 Ro-SSA positive SS patients between January 2000 and December 2016.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Skin manifestations were present in 74.9% (<i>n</i> = 137) of SS patients. Dermatitis and Lupus erythematosus (LE)-specific skin manifestations were present in 34.1% (<i>n</i> = 63) and 21.6% (<i>n</i> = 40) of SS patients respectively. 65.9% (<i>n</i> = 122) of SS patients had internal organ involvement, with 53% (<i>n</i> = 98) presenting neuropsychiatric manifestations. Several risk factors for internal organ involvement were identified: presence of myalgia/arthralgia (odds ratio [OR] = 3.05, confidence interval [CI] 1.29−7.22), fatigue (OR = 2.53, CI 1.11−5.73) and age older than 50 years (OR = 2.23, CI 1.01−4.93).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Internal organ involvement is frequent in SS, however there is no significant difference between pSS and sSS concerning its occurrence.</p>\u0000 </section>\u0000 </div>","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"3 5","pages":"1478-1486"},"PeriodicalIF":0.0,"publicationDate":"2024-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.498","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141823780","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}
引用次数: 0
Two pemphigoid cases with mucous membrane involvement successfully treated with baricitinib 巴利替尼成功治疗两例粘膜受累的丘疹性荨麻疹病例
JEADV clinical practice Pub Date : 2024-07-19 DOI: 10.1002/jvc2.511
Tamás Malkovics, Orsolya N. Horváth, Miklós Sárdy
{"title":"Two pemphigoid cases with mucous membrane involvement successfully treated with baricitinib","authors":"Tamás Malkovics,&nbsp;Orsolya N. Horváth,&nbsp;Miklós Sárdy","doi":"10.1002/jvc2.511","DOIUrl":"10.1002/jvc2.511","url":null,"abstract":"<p>Both bullous pemphigoid and mucous membrane pemphigoid are autoimmune bullous diseases that can be challenging to treat in individual cases. Janus kinase inhibitors have shown efficacy in several autoimmune and inflammatory diseases, although these drugs are not yet widely used in autoimmune bullous diseases. We report two recalcitrant cases of pemphigoid with mucous membrane involvement which were successfully treated with the JAK 1/2 inhibitor baricitinib after all other feasible therapeutic options had been exhausted.</p>","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"3 5","pages":"1656-1659"},"PeriodicalIF":0.0,"publicationDate":"2024-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.511","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141823934","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}
引用次数: 0
Rosacea fulminans: An anti-inflammatory-based therapeutic approach 红斑痤疮:基于抗炎的治疗方法
JEADV clinical practice Pub Date : 2024-07-14 DOI: 10.1002/jvc2.508
Gabi Handgretinger, Alexandra Saur, Isabel Wolff, Martin Schaller
{"title":"Rosacea fulminans: An anti-inflammatory-based therapeutic approach","authors":"Gabi Handgretinger,&nbsp;Alexandra Saur,&nbsp;Isabel Wolff,&nbsp;Martin Schaller","doi":"10.1002/jvc2.508","DOIUrl":"10.1002/jvc2.508","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>The treatment of rosacea fulminans still poses a significant challenge in daily clinical practice. The pronounced centrofacial inflammation accompanied by severe pustulation, pain and high psychological stress, mostly affecting young women, requires an appropriate fast-acting treatment regimen. A major difficulty here is that if isotretinoin is recommended for therapy, sufficiently reliable contraception is absolutely necessary and not always given. So far, alternative therapies have not been extensively studied.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>Our goal is to show the efficacy of a treatment regimen with macrolide antibiotics (azithromycin) without the need for initial systemic isotretinoin or corticosteroids.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We present six female patients in our outpatient clinic treated with a combination of azithromycin and topical ivermectin reaching rapid inflammation control.</p>\u0000 \u0000 <p>Subsequently, maintenance with ultra-low dose isotretinoin was initiated in 66% of cases. The patients were monitored for a median follow-up time of 15.5 months [1–51 months].</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>We can show rapid improvement of inflammation under antibiotic-treatment with azithromycin (500 mg 3x weekly for 4 weeks, 2x weekly for 4 weeks, 1x weekly 4 weeks) followed by ultra-low dose isotretinoin maintenance treatment with minimal side effects. Topical ivermectin was administered additionally.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Macrolides, in particular azithromycin, pose an alternative treatment method when retinoids are contraindicated extending the treatment regimen of rosacea fulminans. They are a fast-acting and safe alternative to retinoids without the need for initial contraception. The antibiotic-based approach is particularly suited to the control of inflammation.</p>\u0000 </section>\u0000 </div>","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"3 5","pages":"1596-1601"},"PeriodicalIF":0.0,"publicationDate":"2024-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.508","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141650460","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}
引用次数: 0
Koebnerization of Hailey–Hailey disease into chronic prurigo nodularis: Clearance of both entities under a naloxone therapy Koebnerization of Hailey-Hailey disease into chronic prurigo nodularis:在纳洛酮疗法下清除两种实体
JEADV clinical practice Pub Date : 2024-07-14 DOI: 10.1002/jvc2.510
Felix Witte, Dieter Metze, Sonja Ständer, Claudia Zeidler
{"title":"Koebnerization of Hailey–Hailey disease into chronic prurigo nodularis: Clearance of both entities under a naloxone therapy","authors":"Felix Witte,&nbsp;Dieter Metze,&nbsp;Sonja Ständer,&nbsp;Claudia Zeidler","doi":"10.1002/jvc2.510","DOIUrl":"10.1002/jvc2.510","url":null,"abstract":"<p>Hailey–Hailey disease (HHD) is a genetic skin disorder, accompanied by lesional pruritus and a relevant impact on quality of life of affected patients. Approved or even successful treatments are still lacking, highlighting the need for further insight in therapeutic options. µ-opioid receptor antagonists have recently been identified as a potentially successful therapy in HHD-patients, while already being used successfully in treating chronic pruritus. In our case of a patient with severe HHD and generalization of chronic pruritus, which led to koebnerization of HHD lesions into clinically healthy skin, intravenous naloxone treatment proved extraordinarily beneficial in improving both HHD and concomitant chronic pruritus.</p>","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"3 5","pages":"1653-1655"},"PeriodicalIF":0.0,"publicationDate":"2024-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.510","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141650371","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}
引用次数: 0
Annular Hailey–Hailey disease 环形海利-海利病
JEADV clinical practice Pub Date : 2024-07-12 DOI: 10.1002/jvc2.479
Ariane Fizazi, Olivier Dereure
{"title":"Annular Hailey–Hailey disease","authors":"Ariane Fizazi,&nbsp;Olivier Dereure","doi":"10.1002/jvc2.479","DOIUrl":"10.1002/jvc2.479","url":null,"abstract":"<p>Hailey–Hailey disease (HHD) is a genodermatosis that typically presents with crusted erosions located in skin folds and the perineum. We report two cases of a highly atypical form of HHD, characterised by inflammatory annular skin lesions on the trunk, isolated and sparing the folds in one patient. Diagnosis was confirmed by histology or genetic analysis. Clinicians should consider this exceptional subset of HHD when encountering annular lesions resistant to antifungals even when a relevant familial background is lacking.</p>","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"3 5","pages":"1638-1640"},"PeriodicalIF":0.0,"publicationDate":"2024-07-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.479","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141655253","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}
引用次数: 0
Recent changes in speckled pigmented lesion 斑点状色素病变的最新变化
JEADV clinical practice Pub Date : 2024-07-09 DOI: 10.1002/jvc2.506
Irina Nocivin, Rakesh Anand, Parastoo Babakinejad, Andrew Giles, Khushboo Sinha
{"title":"Recent changes in speckled pigmented lesion","authors":"Irina Nocivin,&nbsp;Rakesh Anand,&nbsp;Parastoo Babakinejad,&nbsp;Andrew Giles,&nbsp;Khushboo Sinha","doi":"10.1002/jvc2.506","DOIUrl":"10.1002/jvc2.506","url":null,"abstract":"&lt;p&gt;A 54-year-old Caucasian male (phototype III) was referred via the skin cancer screening pathway with a skin lesion present since infancy. He reported a 1-year history of change with no associated symptoms.&lt;/p&gt;&lt;p&gt;He did not report a prior family history of skin malignancy. He had a significant history of sun exposure as he worked in the Royal Navy in the past. He denied any associated symptoms.&lt;/p&gt;&lt;p&gt;On examination, there was a background 4.5 × 5 cm pigmented speckled lesion with a nodular component. Dermoscopy showed a large background light brown patch with a few scattered darker pigmented macular lesions with a reticular pattern. Additionally, noted a nodular component with darker coalescing irregular reticular pattern with associated structureless central zone (Figure 1). In view of the suspicious clinical and dermoscopic features, the lesion, both macular and nodular component was excised with a 2 mm margin (Figure 2).&lt;/p&gt;&lt;p&gt;Nevus spilus is a rare type of congenital lesion initially described in 1842 by Burkley.&lt;span&gt;&lt;sup&gt;1&lt;/sup&gt;&lt;/span&gt; It consists of a background patch of hyperpigmentation with darker speckled macules or papules. It is uncommon, found in 0.2%−2.3% of the population.&lt;span&gt;&lt;sup&gt;2&lt;/sup&gt;&lt;/span&gt;&lt;/p&gt;&lt;p&gt;In our case, we observed (Figure 1a,b) on dermoscopy two distinct features: an area of change within a pre-existing lesion with background pale brown patch and speckled pigmented macules and an area of recent change with and a nodular component with milky red areas and polymorphous atypical vascular structures with a pigmented patch showing peripheral dots and globules and brown structureless zones. These changes were highly suggestive of a malignant transformation.&lt;/p&gt;&lt;p&gt;Previously, NS was considered an innocuous pigmented lesion. However, the first case of a melanoma arising within a NS was reported in 1957 by Perkinson.&lt;span&gt;&lt;sup&gt;3&lt;/sup&gt;&lt;/span&gt; There have been approximately 30 cases published of this type of clinical presentation. Within the literature, most cases of malignant transformation were noted in Caucasian or Afro-Caribbean patients, with a slight female preponderance. Common sites of presentation were noted on the limbs (36%) and trunk (64%) and the mean age of diagnosis was 49 years.&lt;span&gt;&lt;sup&gt;4, 5&lt;/sup&gt;&lt;/span&gt;&lt;/p&gt;&lt;p&gt;NS generally presents as three sizes: small (&lt;1.5 cm), medium (1.5–19.9 cm) and large (&gt;20 cm), with rarer presentations being segmented and zosteriform.&lt;span&gt;&lt;sup&gt;4&lt;/sup&gt;&lt;/span&gt;&lt;/p&gt;&lt;p&gt;There is no clear consensus, as this presentation is not common, but it is thought that the risk of malignant transformation is around 0.13%–0.2%.&lt;span&gt;&lt;sup&gt;6&lt;/sup&gt;&lt;/span&gt;&lt;/p&gt;&lt;p&gt;However, once a melanoma arises, there is no clear difference in terms of management and prognosis when compared to melanomas which arise in other clinical circumstances. The prevalent practice of self-monitoring, photographic documentation, and long-term follow-up with dermoscopy is favoured.&lt;span&gt;&lt;sup&gt;7&lt;/sup&gt;&lt;/span&gt; Additionally, the biops","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"4 1","pages":"356-358"},"PeriodicalIF":0.0,"publicationDate":"2024-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.506","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141664078","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}
引用次数: 0
Association between pemphigus vulgaris and pyoderma gangrenosum 寻常天疱疮与脓皮病之间的联系
JEADV clinical practice Pub Date : 2024-07-08 DOI: 10.1002/jvc2.507
Ana M. Cano, Valentina Burbano, Ricardo A. Rueda
{"title":"Association between pemphigus vulgaris and pyoderma gangrenosum","authors":"Ana M. Cano,&nbsp;Valentina Burbano,&nbsp;Ricardo A. Rueda","doi":"10.1002/jvc2.507","DOIUrl":"10.1002/jvc2.507","url":null,"abstract":"<p>Pyoderma gangrenosum (PG) is a neutrophilic dermatosis associated with certain neoplasms, predominantly of haematological origin, and autoimmune diseases. Pemphigus vulgaris (PV) is a disease in which antibodies target crucial intercellular junction components. A few cases of PG associated with PV have been described. We present a woman with a high phototype presenting this rare association and highlight the diagnostic challenge of diagnosing rare diseases in patients with high phenotypes.</p>","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"3 3","pages":"902-905"},"PeriodicalIF":0.0,"publicationDate":"2024-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.507","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141667259","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}
引用次数: 0
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