Journal of Dermatology最新文献

{"title":"Red Yet Joyful: Finkelstein Disease","authors":"Inês Tribolet-Abreu, Madalena Pupo-Correia, Filipe Silva-Monteiro, Gustavo Almeida-Silva, Sónia Fernandes","doi":"10.1111/1346-8138.17739","DOIUrl":"https://doi.org/10.1111/1346-8138.17739","url":null,"abstract":"","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"52 5","pages":"e332-e333"},"PeriodicalIF":2.9,"publicationDate":"2025-04-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143914577","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cutaneous manifestations of WHIM syndrome","authors":"Rutha Adhanom,&nbsp;Caleb Kim,&nbsp;Jennifer Strong,&nbsp;Sophia Martinez,&nbsp;Heidi H. Kong,&nbsp;Isaac Brownell,&nbsp;Philip M. Murphy,&nbsp;David H. McDermott,&nbsp;Leslie Castelo-Soccio","doi":"10.1111/1346-8138.17733","DOIUrl":"10.1111/1346-8138.17733","url":null,"abstract":"<p>Warts, Hypogammaglobulinemia, Infections, and Myelokathexis (WHIM) syndrome is a rare immunodeficiency caused by gain-of-function mutations in the chemokine receptor CXCR4. While human papillomavirus (HPV) skin infection (warts) is the dermatological hallmark of the disease, individuals with WHIM have high rates of other skin manifestations that may aid early diagnosis and management. This study was a retrospective review of medical records from a United States National Institutes of Health natural history cohort of patients with WHIM syndrome seen between 2005 and 2024, including a cross-sectional analysis of cutaneous manifestations and CXCR4 variants. The cohort compromised 45 patients with genetically confirmed WHIM syndrome, 16 men and 29 women, with a mean age of 33.3 years (range, 0–69 years) and mean age at diagnosis of 20.4 years (range, 0–59 years). The cohort exhibited a range of skin manifestations which included cutaneous infections with HPV in 34 (76%) patients, bacteria in 32 (71%) patients, other viruses in 27 (60%) patients, and fungi in 25 (56%) patients. Inflammatory conditions included six (13%) patients with seborrheic dermatitis, five (11%) with contact dermatitis, four (9%) with psoriasis, three (7%) with nummular eczema, and 13 (29%) with other eczematous dermatitis. Despite the young median age, seven (16%) patients had skin cancer. All seven patients had <i>CXCR4</i> truncation mutations, while those with a missense mutation (E343K) generally had fewer skin manifestations. Our study found that WHIM syndrome is associated with diverse infectious, inflammatory, and neoplastic skin conditions beyond HPV skin infection.</p>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"52 5","pages":"917-921"},"PeriodicalIF":2.9,"publicationDate":"2025-04-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/1346-8138.17733","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143813275","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Author Guidelines","authors":"","doi":"10.1111/1346-8138.17729","DOIUrl":"https://doi.org/10.1111/1346-8138.17729","url":null,"abstract":"","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"52 4","pages":"e326-e329"},"PeriodicalIF":2.9,"publicationDate":"2025-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/1346-8138.17729","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143793356","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Safety and effectiveness of secukinumab in Japanese patients with generalized pustular psoriasis: A post-marketing surveillance","authors":"Ayako Fujishige,&nbsp;Noriko Seko","doi":"10.1111/1346-8138.17648","DOIUrl":"10.1111/1346-8138.17648","url":null,"abstract":"<p>Secukinumab is one of the human monoclonal antibodies recommended in the Japanese guidelines for patients with psoriasis, but few case reports and clinical studies on secukinumab for pustular psoriasis are available because of the rarity of the disease. This was an open-label, multicenter, uncontrolled, single-arm, prospective observational surveillance conducted in a clinical practice setting to evaluate the safety and effectiveness of secukinumab in Japanese patients with generalized pustular psoriasis (GPP). Patients were monitored for 1 year after starting secukinumab and followed up for an additional 2 years. Of 99 patients from 71 sites, 95 were included in safety and 82 in effectiveness analysis. The mean (standard deviation) observation period was 346.2 (64.87) days, and 91.58% of patients were observed over 52 weeks. Adverse events, serious adverse events, and adverse reactions were reported in 51.58%, 12.63%, and 35.79% of patients, respectively. Safety evaluations showed no significant difference in the incidence of events based on the history of biologics The proportion of patients with either “complete response” or “partial response” was ~90% from week 2 and remained stable until week 52. The proportion of patients with “remission (no symptom)” in the Japanese Dermatological Association total score increased from week 4 (22.22%) to week 52 (47.83%). The mean Psoriasis Area and Severity Index score decreased from week 1 (17.26) to week 16 (1.18), with the mean percentage change decreasing from −28.07% to −90.18%. The mean Dermatology Life Quality Index (DLQI) total score decreased from 8.7 at the start of secukinumab treatment to 1.9 at week 52. At week 52, the proportion of patients with DLQI total score of 0/1 was 57.14%. No new safety signals for secukinumab in long-term treatment were observed from this surveillance, and no additional measures needed to be taken. Moreover, secukinumab showed sustained effectiveness in patients with GPP in Japan.</p>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"52 5","pages":"773-786"},"PeriodicalIF":2.9,"publicationDate":"2025-04-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/1346-8138.17648","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143775211","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Disease features, treatments, and burden of palmoplantar pustulosis in Korea: The EPPPIK study","authors":"Seong Jin Jo,&nbsp;Byung Soo Kim,&nbsp;Dong Hyun Kim,&nbsp;Jong Hoon Kim,&nbsp;Chun Wook Park,&nbsp;Sang Wook Son,&nbsp;Jiyoung Ahn,&nbsp;Ji Yeoun Lee,&nbsp;Ki-Heon Jeong,&nbsp;Youngdoe Kim,&nbsp;Jihye An,&nbsp;Chul Jong Park,&nbsp;Sang Woong Youn","doi":"10.1111/1346-8138.17702","DOIUrl":"10.1111/1346-8138.17702","url":null,"abstract":"<p>Palmoplantar pustulosis (PPP) is a chronic skin disease characterized by erythema, pustules, and desquamation of the palms and soles. PPP epidemiological data are scarce. Understanding disease characteristic is essential for effective management and to reduce burden. The objective of the study was to describe disease characteristics of PPP in Korea, including demographics, disease burden, and current clinical practice. This was a cross-sectional, multicenter, noninterventional study conducted among 20 sites in Korea. Patients (aged ≥19 years) with a confirmed PPP diagnosis were examined and interviewed, collecting clinical and patient-reported outcomes in 1 day. A total of 379 patients with PPP (mean age, 51.4 years; 39.3% male) were enrolled. The mean age at diagnosis was 47.9 years, and the mean duration of PPP was 3.2 years. Mean±standard deviation of Palmoplantar Pustulosis Area and Severity Index (PPPASI) score was 10.3±9.0; 33.8% of patients scored ≥12. PPPASI score was significantly higher with younger age (<i>p</i> = 0.0001) and nail involvement (<i>p</i> = 0.0002). Mean Dermatology Life Quality Index (DLQI) score was 12.0, and mean EuroQoL 5-Dimension Health Questionnaire (EQ-5D) score was 62.4 ± 19. Patients with PPPASI ≥12 had significantly higher DLQI (14.1 ± 7.3 vs 10.9 ± 7.6; <i>p</i> &lt; 0.0001) and lower EQ-5D (57.9 ± 18.1 vs 64.7 ± 19.2; <i>p</i> = 0.002) scores. The most common treatments in patients with PPPASI &lt;12 were topicals (50.6%), conventional therapies (34.7%), and retinoids (32.7%); the most common treatments with PPPASI ≥12 were conventional therapies (37.5%), topicals (34.4%), and retinoids (29.7%). Conventional therapy users were “more satisfied” than nonusers (49.6% vs 39.0%; <i>p</i> = 0.05), with a numerical difference observed for biologics (41.8% vs 63.1%; <i>p</i> = 0.07). PPP is a debilitating disease that significantly diminishes overall quality of life. Despite most patients receiving treatment, PPP burden persists. Developing treatment guidelines and identifying more targeted and effective treatments would help alleviate symptoms, reduce severity, and improve overall quality of life.</p>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"52 5","pages":"802-811"},"PeriodicalIF":2.9,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/1346-8138.17702","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143756907","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The required absolute PASI score to achieve DLQI remission","authors":"Emi Nishida,&nbsp;Akimichi Morita","doi":"10.1111/1346-8138.17723","DOIUrl":"10.1111/1346-8138.17723","url":null,"abstract":"<p>Psoriasis is a chronic inflammatory skin disease that significantly impacts patients' quality of life (QoL). While the Psoriasis Area and Severity Index (PASI) has traditionally been used to assess disease severity and treatment response, achieving substantial improvement in QoL has become an increasingly important therapeutic goal. Recent advances in biologic therapies have enabled higher rates of PASI 90 and PASI 100 responses; however, PASI 75 is no longer considered sufficient for optimal patient outcomes. Calculating PASI improvement rates in daily practice remains challenging, shifting focus toward absolute PASI values as a practical indicator of disease control. In this study, we analyzed 235 psoriasis patients treated with biologics at Nagoya City University Hospital, evaluating 3526 data points collected over a maximum of 288 weeks. We found that PASI positively correlated with DLQI (<i>r</i> = 0.5696, <i>p</i> &lt; 0.001), and that an absolute PASI score of ≤2.2 was associated with DLQI remission (AUC = 0.8140). Notably, 82.6% of patients achieving PASI 100 also achieved DLQI 0/1 status. However, 1.0% of patients with a PASI score of 0 still reported a DLQI ≥10, suggesting that factors beyond skin lesions, such as stigma or residual damage, may contribute to impaired QoL. These findings underscore the importance of evaluating absolute PASI values to guide treatment decisions and achieve DLQI remission. Additionally, the psychosocial burden of psoriasis must be addressed to ensure comprehensive care and sustained improvements in QoL.</p>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"52 5","pages":"947-949"},"PeriodicalIF":2.9,"publicationDate":"2025-03-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/1346-8138.17723","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143712657","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Lichen nitidus on the penis","authors":"Teng Liu,&nbsp;Jianmin Chang","doi":"10.1111/1346-8138.17719","DOIUrl":"10.1111/1346-8138.17719","url":null,"abstract":"","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"52 5","pages":"e330-e331"},"PeriodicalIF":2.9,"publicationDate":"2025-03-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143712643","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Incidence of the Stevens-Johnson syndrome spectrum and its patterns in urban China","authors":"Chaiquan Li,&nbsp;Mali Dai,&nbsp;Qinyi Gu,&nbsp;Ke Lu,&nbsp;Chaoxiang Ge,&nbsp;Xiaofei Liu,&nbsp;Zhiming Li,&nbsp;Jingying Wang,&nbsp;Jinxi Wang,&nbsp;Chen Wei,&nbsp;Xun Tang,&nbsp;Qinxiang Zheng,&nbsp;Pei Gao,&nbsp;Wei Chen","doi":"10.1111/1346-8138.17692","DOIUrl":"10.1111/1346-8138.17692","url":null,"abstract":"<p>Stevens-Johnson syndrome (SJS) spectrum is a series of rare, but life-threatening mucocutaneous hypersensitivity reactions. However, the epidemiological evidence of it is limited, especially in developing countries. The shape associations with age and its seasonal pattern are still uncertain. Therefore, this study aimed to describe the nationwide incidence of SJS spectrum, its associations with age and season, and its subsequent diagnosed diseases across urban China. By using national health insurance datasets from 2013 to 2017, this retrospective cohort study estimated the incidence of SJS spectrum and its associations in 23 Chinese provinces. SJS spectrum was defined to include SJS and/or toxic epidermal necrolysis (ICD-10 code: L51.1–2). First, age-specific incidence rates were estimated in each province and then pooled using a random-effects model. Relative risks (RR) of SJS spectrum in different seasons were estimated by Poisson regression. Diseases diagnosed within 6 months following SJS occurrence were described. In total, 9308 patients were identified (50.17% women, median age 45). The average standardized incidence rate of SJS spectrum was 3.43 (95% confidence interval [CI] 3.04–3.84) per million person-years, which increased by 0.57 (95% CI 0.29–0.84) per million person-years. Incidence rates were significantly higher among children age &lt;10 years (5.48, 95% CI 4.02–7.09, <i>p</i> = 0.0036) and adults age ≥ 40 (5.50, 95% CI 4.35–6.78, <i>p</i> = 0.00085) than the group aged 10–40 years (3.12, 95% CI 2.29–4.07). The risk of SJS spectrum was highest in summer (vs autumn: RR = 1.83, 95% CI 1.72–1.93). The most frequently diagnosed disease within 6 months after the onset of SJS was respiratory disease (43.8%). In conclusion, this study provides evidence on the epidemiology of SJS spectrum in developing countries and supports the two-peak distribution of its incidence across age. Its high occurrence in summer needs further confirmation.</p>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"52 5","pages":"897-906"},"PeriodicalIF":2.9,"publicationDate":"2025-03-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143694969","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Development of Japanese versions of the Autoimmune Bullous Disease Quality of Life and Treatment of Autoimmune Bullous Disease Quality of Life questionnaires","authors":"Chika Tanemura,&nbsp;Maya Nunotani,&nbsp;Kyoko Kawabata,&nbsp;Yuki Morooka,&nbsp;Jun Yamagami,&nbsp;Risa Kakuta,&nbsp;Yasuko Saito,&nbsp;Yuichi Kurihara,&nbsp;Hayato Takahashi,&nbsp;Norito Ishii,&nbsp;Hiroshi Koga,&nbsp;Takekuni Nakama,&nbsp;Daisuke Hayashi,&nbsp;Sho Hiroyasu,&nbsp;Chiharu Tateishi,&nbsp;Daisuke Tsuruta,&nbsp;Dedee F. Murrell,&nbsp;Takashi Hashimoto","doi":"10.1111/1346-8138.17707","DOIUrl":"10.1111/1346-8138.17707","url":null,"abstract":"<p>Patients with autoimmune bullous disease have their quality of life (QOL) affected by both the disease and its treatment burden. While QOL assessment is clinically important, it is often hindered by limited time in clinical practice, highlighting the need for accurate and efficient QOL evaluation tools. However, no validated QOL questionnaires are currently available in Japan. This study evaluated the validity and reliability of the Japanese versions of the Autoimmune Bullous Disease Quality of Life (ABQOL) and Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) questionnaires, as well as their practical application in clinical settings. The original questionnaires were forward and back-translated into Japanese by certified translators according to established guidelines, then their validity and reliability were evaluated using data from 147 patients with autoimmune bullous diseases. Validity was evaluated via confirmatory and exploratory factor analyses, cross-cultural validation, hypothesis testing, and convergent validity. Reliability was evaluated via test–retest and internal consistency. Although confirmatory factor analysis showed a weak fit and factor structures slightly differed from the original versions, internal consistency was cross-culturally valid. Also, the Japanese version cohort showed lower mean scores and better QOL outcomes compared with other language versions for similar cohorts. Hypothesis testing revealed a significant positive correlation between ABQOL scores and subjective disease severity; TABQOL scores were significantly correlated with steroid dosage. The mucosal subscale of the ABQOL showed a significant difference based on mucosal lesion status. Bland–Altman plots confirmed approximate agreement between the two sets of measurements: Cronbach's alpha coefficients were 0.872 for ABQOL and 0.903 for TABQOL, verifying reliability. Finally, an expert panel reviewed and agreed on the target population, timing, methods for using the scales, and considerations for scale evaluation. The Japanese versions of the ABQOL and TABQOL are expected to be implemented in clinical practice as reliable and validated tools in Japan.</p>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"52 5","pages":"812-830"},"PeriodicalIF":2.9,"publicationDate":"2025-03-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/1346-8138.17707","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143665777","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A case of pseudoangiomatous spindle-cell lipoma with unique MRI manifestations corresponding to distinct histological features","authors":"Maho Matsuo,&nbsp;Hiroki Kato,&nbsp;Kazuhiro Kobayashi,&nbsp;Miki Nagai,&nbsp;Hiroaki Iwata","doi":"10.1111/1346-8138.17701","DOIUrl":"10.1111/1346-8138.17701","url":null,"abstract":"<p>A 50-year-old man presented with a subcutaneous mass on the posterior neck. A skin biopsy suggested lipoma, leading to observation. Over the next 5 years, the tumor gradually enlarged. Clinical evaluation found the tumor to be a soft, mobile mass of 10 × 7 cm. Magnetic resonance imaging showed the lesion to be cystic, with hyperintense mural nodules on T2-weighted sequences, and with scattered, hyperintense foci indicative of fatty components on T1-weighted imaging. Fat-suppressed contrast-enhanced T1-weighted imaging revealed moderately enhanced mural nodules. Surgical resection revealed a well-circumscribed cystic mass containing fluid, surrounded by a fibrous capsule. We suspected spindle cell lipoma associated with fluid and performed a marginal resection. Gross examination showed a heterogeneous mixture of reddish-brown and yellowish zones interspersed with fissures. Histological analysis revealed a leaf-like or reticular pattern, with slender, spindle-shaped cells and ropey collagens within a mucinous matrix, along with mature adipocytes. There were many irregular, branching, cleft-like vascular spaces lined by flattened cells. Immunohistochemical staining revealed tumor cells positive for CD34 and negative for retinoblastoma protein. Luminal endothelial-like cells showed positivity for D2-40, CD31, and erythroblastosis transformation-specific regulated gene 1. Based on these findings, the diagnosis of pseudoangiomatous spindle-cell lipoma was confirmed, with the luminal endothelial-like cells exhibiting characteristics of lymphatic endothelium.</p>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"52 5","pages":"943-946"},"PeriodicalIF":2.9,"publicationDate":"2025-03-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143652964","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}