Journal of Dermatology最新文献

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Novel POLR3A Gene Mutation Results in Wiedemann-Rautenstrauch Syndrome With Striking Cutis Laxa and Myelofibrosis 新的POLR3A基因突变导致Wiedemann-Rautenstrauch综合征伴显著皮肤松弛和骨髓纤维化。
IF 2.7 3区 医学
Journal of Dermatology Pub Date : 2026-01-18 DOI: 10.1111/1346-8138.70119
Weiyi Xiang, Hongjie Luo, Deyu Song, Xueyu Zhang, Xian Jiang
{"title":"Novel POLR3A Gene Mutation Results in Wiedemann-Rautenstrauch Syndrome With Striking Cutis Laxa and Myelofibrosis","authors":"Weiyi Xiang,&nbsp;Hongjie Luo,&nbsp;Deyu Song,&nbsp;Xueyu Zhang,&nbsp;Xian Jiang","doi":"10.1111/1346-8138.70119","DOIUrl":"10.1111/1346-8138.70119","url":null,"abstract":"<div>\u0000 \u0000 <p>Wiedemann-Rautenstrauch syndrome is an extremely rare autosomal recessive progeroid disorder closely linked to mutations in POLR3A. Here, we report a case of a 4-year-old female patient carrying a novel compound-heterozygous variant in POLR3A. In addition to the classic Wiedemann-Rautenstrauch syndrome features—progressive diffuse alopecia, growth retardation, and abnormal white matter development—the patient presented with severe anemia and skin laxity, phenotypes not previously described in Wiedemann-Rautenstrauch syndrome. RT-qPCR analysis of skin tissue demonstrated a significant downregulation of <i>POLR3A</i> mRNA expression (<i>p</i> &lt; 0.01). To our knowledge, this is the first report implicating an intronic POLR3A variant in Wiedemann-Rautenstrauch syndrome in the Chinese population, expanding both the mutational and phenotypic spectra of the disorder and underscoring its clinical heterogeneity.</p>\u0000 </div>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"53 2","pages":"357-361"},"PeriodicalIF":2.7,"publicationDate":"2026-01-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146000285","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}
引用次数: 0
Guidelines for the Management of Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis 2025 Supplement 史蒂文斯-约翰逊综合征和中毒性表皮坏死松解症管理指南2025补充。
IF 2.7 3区 医学
Journal of Dermatology Pub Date : 2026-01-08 DOI: 10.1111/1346-8138.70136
Hideaki Watanabe, Youichi Ogawa, Yukie Yamaguchi, Saeko Nakajima, Yoshiko Mizukawa, Taisei Mushiroda, Hayato Takahashi, Toshiharu Fujiyama, Chie Sotozono, Yoshiko Kaneko, Akito Hasegawa, Kae Kobayashi, Yuma Sunaga, Yuko Watanabe, Takashi Nomura, Kenji Kabashima, Ayano Inui, Mikiya Fujieda, Mayumi Ueta, Natsumi Hama, Mikiko Tohyama, Riichiro Abe
{"title":"Guidelines for the Management of Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis 2025 Supplement","authors":"Hideaki Watanabe,&nbsp;Youichi Ogawa,&nbsp;Yukie Yamaguchi,&nbsp;Saeko Nakajima,&nbsp;Yoshiko Mizukawa,&nbsp;Taisei Mushiroda,&nbsp;Hayato Takahashi,&nbsp;Toshiharu Fujiyama,&nbsp;Chie Sotozono,&nbsp;Yoshiko Kaneko,&nbsp;Akito Hasegawa,&nbsp;Kae Kobayashi,&nbsp;Yuma Sunaga,&nbsp;Yuko Watanabe,&nbsp;Takashi Nomura,&nbsp;Kenji Kabashima,&nbsp;Ayano Inui,&nbsp;Mikiya Fujieda,&nbsp;Mayumi Ueta,&nbsp;Natsumi Hama,&nbsp;Mikiko Tohyama,&nbsp;Riichiro Abe","doi":"10.1111/1346-8138.70136","DOIUrl":"10.1111/1346-8138.70136","url":null,"abstract":"&lt;p&gt;Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are characterized by high fever, body malaise, and multiple erythema, erosions, and blisters on the skin and mucocutaneous junction. They are considered life-threatening and intractable diseases that leave sequelae, including blindness and respiratory disorders. The diagnostic criteria for SJS/TEN were developed in 2005 by the Japanese Ministry of Health, Labour and Welfare Research Group on Severe Erythema Multiforme in Japan. To further understand the actual clinical scenario, the first epidemiological survey targeting dermatologists' full-time facilities nationwide was conducted from 2005 to 2007 and subsequently published. In 2015, the Japanese guidelines for the management of SJS and TEN were established. The second national survey for SJS/TEN, conducted from 2016 to 2018, identified vascular disease, malignancy, and diabetes mellitus as risk factors for SJS/TEN development. Moreover, they also identified sepsis and diabetes as risk factors for death and sepsis, respectively. In this survey, the mortality rates of both SJS and TEN were higher than those recorded in the first national survey.&lt;/p&gt;&lt;p&gt;Based on these results, we revised the existing guidelines by incorporating recent knowledge on the pathogenesis, medical complications, treatment, and prognosis prediction of SJS/TEN with the aim of saving the lives of patients with SJS/TEN in the future. This addendum provides standard guidelines at this point. However, several of these guidelines are based on the committee's opinions, because clinical studies with high-level evidence are limited due to the rarity of these diseases, and their pathogeneses are often unclear. In actual practice, the treatment approach must consider each patient's situation, and the guidelines are not intended to force treatment selection or limit a physician's discretion.&lt;/p&gt;&lt;p&gt;Levels of Evidence Classification:&lt;/p&gt;&lt;p&gt;I: Systematic review/meta-analysis.&lt;/p&gt;&lt;p&gt;II: One or more randomized controlled trials.&lt;/p&gt;&lt;p&gt;III: Nonrandomized controlled trials.&lt;/p&gt;&lt;p&gt;IVa: Epidemiological studies, such as cohort studies.&lt;/p&gt;&lt;p&gt;IVb: Epidemiological studies, such as case–control and cross-sectional studies.&lt;/p&gt;&lt;p&gt;V: Descriptive studies, including case reports and case series studies.&lt;/p&gt;&lt;p&gt;VI: Personal opinion.&lt;/p&gt;&lt;p&gt;Recommendation Statement: Keratinocyte death leads to the formation of blisters and erosions. The death of these cells is mediated via different types of regulated cell death processes, including apoptosis and necroptosis.&lt;/p&gt;&lt;p&gt;Explanation&lt;/p&gt;&lt;p&gt;The culprit drug is recognized as a foreign antigen in individuals with specific human leukocyte antigen (HLA) alleles. Subsequently, activated T cells and natural killer (NK) cells release soluble and cytotoxic molecules, resulting in the death of epidermal keratinocytes. Death of epidermal keratinocytes is mediated by different types of regulated cell death processes, such as apoptosis and necroptosis. Ap","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"53 2","pages":"e88-e101"},"PeriodicalIF":2.7,"publicationDate":"2026-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/1346-8138.70136","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145919492","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}
引用次数: 0
Real-World Safety Profile of Spesolimab in Generalized Pustular Psoriasis: Insights From Japan as Part of a Multinational Expanded Access Program (EAP) Spesolimab治疗广泛性脓疱性银屑病的实际安全性:来自日本的跨国扩展准入计划(EAP)的见解
IF 2.7 3区 医学
Journal of Dermatology Pub Date : 2025-12-30 DOI: 10.1111/1346-8138.70122
Akimichi Morita, Yayoi Tada, Yuichiro Tsunemi, Mayumi Komine, Takuro Kanekura, Shinichi Imafuku, Toshiya Takahashi, Xuemei Ding, Nichiren Pillai, Morihisa Saitoh, Rafael Sani Simoes, Nora Pöntynen, Keiichi Yamanaka
{"title":"Real-World Safety Profile of Spesolimab in Generalized Pustular Psoriasis: Insights From Japan as Part of a Multinational Expanded Access Program (EAP)","authors":"Akimichi Morita,&nbsp;Yayoi Tada,&nbsp;Yuichiro Tsunemi,&nbsp;Mayumi Komine,&nbsp;Takuro Kanekura,&nbsp;Shinichi Imafuku,&nbsp;Toshiya Takahashi,&nbsp;Xuemei Ding,&nbsp;Nichiren Pillai,&nbsp;Morihisa Saitoh,&nbsp;Rafael Sani Simoes,&nbsp;Nora Pöntynen,&nbsp;Keiichi Yamanaka","doi":"10.1111/1346-8138.70122","DOIUrl":"10.1111/1346-8138.70122","url":null,"abstract":"<p>Generalized pustular psoriasis (GPP) is a heterogeneous, systemic neutrophilic inflammatory disease, characterized by chronic symptoms and recurrent flares, which can be potentially life-threatening. Spesolimab, an interleukin-36 receptor antagonist, has been approved to treat GPP flares in many countries including Japan. An expanded access program (EAP) in Japan provided early access to spesolimab for patients aged 18–75 years unable to participate in a clinical trial with no other treatment options. Patients received a single dose of 900 mg intravenous spesolimab for flare treatment, with an optional second dose after 1 week if symptoms persisted. Safety was monitored for 16 weeks post-treatment. Eleven patients (54.5% female; 51 years mean age; 26.7 kg/m<sup>2</sup> mean body mass index) received 23 doses of intravenous spesolimab. Nine patients (81.8%) were diagnosed with GPP for &gt; 5 years. Ten patients (90.9%) had ≥ 1 baseline medical condition. All patients used ≥ 1 concomitant medication prior to or during the spesolimab treatment period, most commonly immunosuppressants and non-steroidal anti-inflammatory agents. Seven patients (63.6%) experienced treatment-emergent adverse events, all of mild or moderate intensity, including skin and subcutaneous tissue disorders, general disorders and administration site conditions. No adverse event led to treatment discontinuation or death. A potential hypersensitivity event (face edema) resolved without intervention and was not considered treatment related. Spesolimab was well tolerated in a heterogeneous patient population with GPP, including those with comorbidities and concomitant medication use. The safety profile of spesolimab aligned with the EFFISAYIL 1 clinical trial results.</p>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"53 2","pages":"351-356"},"PeriodicalIF":2.7,"publicationDate":"2025-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12877984/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145866973","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}
引用次数: 0
Risk Factors for Microscopic Margin Positivity in Basal Cell Carcinoma Excision With a 2-mm Surgical Margin: A Japanese Retrospective Study 2毫米手术切缘基底细胞癌显微切缘阳性的危险因素:一项日本回顾性研究。
IF 2.7 3区 医学
Journal of Dermatology Pub Date : 2025-12-21 DOI: 10.1111/1346-8138.70118
Kaori Kyono, Eiki Tsushima, Satoshi Urushidate, Shintaro Goto, Yui Akemoto, Toshiaki Suzuki, Tadashi Yoshizawa, Yoshihiro Sowa, Kotaro Yoshimura, Akira Kurose
{"title":"Risk Factors for Microscopic Margin Positivity in Basal Cell Carcinoma Excision With a 2-mm Surgical Margin: A Japanese Retrospective Study","authors":"Kaori Kyono,&nbsp;Eiki Tsushima,&nbsp;Satoshi Urushidate,&nbsp;Shintaro Goto,&nbsp;Yui Akemoto,&nbsp;Toshiaki Suzuki,&nbsp;Tadashi Yoshizawa,&nbsp;Yoshihiro Sowa,&nbsp;Kotaro Yoshimura,&nbsp;Akira Kurose","doi":"10.1111/1346-8138.70118","DOIUrl":"10.1111/1346-8138.70118","url":null,"abstract":"<div>\u0000 \u0000 <p>Basal cell carcinoma is a common locally invasive and destructive skin cancer, if inadequately treated. Although topical noninvasive treatment has been reported, surgical excision is currently the gold standard. Surgical excision is performed with a predetermined surgical margin. There is worldwide debate on the optimal margin. Some guidelines recommend surgical margins &gt; 4 mm; however, Asians reports suggest a narrow surgical margin may be suitable. We previously reported that a 2-mm margin is acceptable for specific tumors. We aimed to clarify whether a 2-mm margin is suitable for all lesions and the differences in local recurrence between surgical procedures (one-step or two-step surgery). We retrospectively reviewed the data of 112 patients who underwent surgical excision with predetermined 2-mm or &gt; 2-mm margins. Pathological reports and surgical procedure databases were collected and statistically analyzed to clarify topical factors for microscopic margin clearance. We compared differences between macroscopic peripheral margins and the distance of microscopic margins and analyzed associations between surgical procedure and local recurrence. Aggressive type was significantly associated with poorly-defined borders (<i>p</i> &lt; 0.01), and microscopic excisional margin positivity was correlated with tumor thickness (<i>p</i> &lt; 0.01). The correlation coefficient between tumor size and tumor thickness was 0.321 and the <i>p</i>-value was 0.0005, indicating a weak correlation between a larger tumor size and a greater tumor thickness. The macroscopic surgical margin was significantly associated with the microscopic peripheral margin (<i>p</i> &lt; 0.001). Tumor size was significantly associated with tumor thickness, and aggressive type tumors were significantly poorly defined. Additionally, tumor size, tumor thickness, and tumor subtype aggressiveness were the strongest risk factors for microscopic margin positivity. For adequate treatment, poorly defined tumors, wider tumors, and aggressive tumor types were carefully treated, and confirmation after excision with a 2-mm margin by two-step surgery or accompanied with frozen section estimation. For well-pigmented and well-defined basal cell carcinomas, a 2-mm margin may be more suitable than for the two types requiring careful treatment.</p>\u0000 </div>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"53 2","pages":"295-303"},"PeriodicalIF":2.7,"publicationDate":"2025-12-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145807182","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}
引用次数: 0
The Predictive Value of Inflammatory Markers in the Severity and Mortality Risk of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis 炎症标志物对Stevens-Johnson综合征/中毒性表皮坏死松解严重程度和死亡风险的预测价值
IF 2.7 3区 医学
Journal of Dermatology Pub Date : 2025-12-19 DOI: 10.1111/1346-8138.70116
Xinjian Fan, Rui Liu, Xianwei Cao, Zhibin Zhang
{"title":"The Predictive Value of Inflammatory Markers in the Severity and Mortality Risk of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis","authors":"Xinjian Fan,&nbsp;Rui Liu,&nbsp;Xianwei Cao,&nbsp;Zhibin Zhang","doi":"10.1111/1346-8138.70116","DOIUrl":"10.1111/1346-8138.70116","url":null,"abstract":"<div>\u0000 \u0000 <p>Red blood cell distribution width-to-platelet ratio (RPR), red blood cell distribution width-to-hemoglobin ratio (RHR), red blood cell distribution width-to-albumin ratio (RAR), and prognostic nutritional index (PNI) have been implicated in disease severity and prognosis. However, their role in predicting severity and mortality in Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) remains unclear. This study aimed to investigate the association between RPR, RHR, RAR, PNI and both disease severity and mortality in SJS/TEN patients. In this retrospective study, clinical data from 111 SJS/TEN patients were analyzed. RPR and other laboratory parameters were compared between survivors and non-survivors. The associations of RHR, PNI, RAR, and RPR with SJS/TEN severity were evaluated using Spearman or Pearson correlation analyses. Patients were categorized into survivor and non-survivor groups. Univariate and multivariate logistic regression analyses were employed to examine the correlations between these markers and mortality. The predictive performance of these indicators for SJS/TEN mortality was assessed using receiver operating characteristic (ROC) curve analysis. Correlation analysis revealed that RAR and RHR were positively correlated with SCORTEN, Re-SCORTRN, ABCD-10 and CRISTEN scores (<i>p</i> &lt; 0.05), whereas PNI was negatively correlated (<i>p</i> &lt; 0.05). Correlation analysis revealed that RPR was positively correlated with Re-SCORTRN, ABCD-10 and CRISTEN scores (<i>p</i> &lt; 0.05). RAR, RPR, and RHR were identified as risk factors for mortality in SJS/TEN, while elevated PNI was a protective factor. The optimal cutoff values for RHR and RPR in predicting mortality were 0.13 (sensitivity 75%; specificity 73.6%) and 0.07 (sensitivity 90%; specificity 48.4%), respectively. These findings highlight the potential clinical utility of RHR (AUC = 0.841) and RAR (AUC = 0.729) as meaningful biomarkers for assessing SJS/TEN severity and associated mortality.</p>\u0000 </div>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"53 2","pages":"288-294"},"PeriodicalIF":2.7,"publicationDate":"2025-12-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145795782","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}
引用次数: 0
Immunoprofiling Reveals GATA3 as a Prognostic Marker in Transformed Mycosis Fungoides 免疫分析显示GATA3是转化性蕈样真菌病的预后标志物。
IF 2.7 3区 医学
Journal of Dermatology Pub Date : 2025-12-18 DOI: 10.1111/1346-8138.70070
Bruno de Castro e Souza, Denis Miyashiro, Jade Cury-Martins, Neusa Yuriko Sakai Valente, Luiz Fernando Ferraz da Silva, José Antonio Sanches
{"title":"Immunoprofiling Reveals GATA3 as a Prognostic Marker in Transformed Mycosis Fungoides","authors":"Bruno de Castro e Souza,&nbsp;Denis Miyashiro,&nbsp;Jade Cury-Martins,&nbsp;Neusa Yuriko Sakai Valente,&nbsp;Luiz Fernando Ferraz da Silva,&nbsp;José Antonio Sanches","doi":"10.1111/1346-8138.70070","DOIUrl":"10.1111/1346-8138.70070","url":null,"abstract":"<p>Transformed mycosis fungoides (TMF) is a rare, aggressive variant of cutaneous T-cell lymphoma characterized by the presence of large neoplastic cells and poor clinical outcomes. A retrospective cohort of 22 TMF patients was analyzed using immunohistochemistry on formalin-fixed, paraffin-embedded (FFPE) tissue for GATA3 (<i>n</i> = 20), T-bet (<i>n</i> = 22), and STAT3 (<i>n</i> = 22). Expression was quantified by image analysis integrated optical density per total area (IOD/area), standardized by <i>z</i>-score and correlated with survival. Seventeen patients with complete data underwent unsupervised clustering (<i>k</i>-means) and principal component analysis (PCA) based on marker expression profiles. High GATA3 expression was strongly associated with worse prognosis (median OS: 8.6 months vs. 41.7 months, <i>p</i> = 0.00094). T-bet and STAT3 expressions showed no significant individual association with survival. Clustering analysis revealed three distinct immunoprofiles: (1) low expression of all markers (intermediate survival, 28.1 months), (2) high STAT3 and T-bet expressions with intermediate GATA3 expression (longest survival, 53.1 months), and (3) high GATA3 expression with low STAT3 and T-bet expressions (poorest survival, 9.5 months). GATA3 is a robust prognostic marker in TMF, identifying patients with particularly poor outcomes. Its elevated expression delineates a Th2-skewed, immunosuppressive phenotype that may inhibit Th1/Th17 pathways via transcriptional repression. Integrative profiling reveals immunobiological subgroups with divergent prognoses, supporting GATA3 as a potential tool for risk stratification and a candidate for targeted intervention in TMF.</p>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"53 2","pages":"304-308"},"PeriodicalIF":2.7,"publicationDate":"2025-12-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12877973/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145784121","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}
引用次数: 0
A Pediatric Case of Wells Syndrome Presenting With Recurrent Annular Erythema and Fever Successfully Treated With Baricitinib 以反复出现的环状红斑和发热为表现的威尔斯综合征患儿一例,Baricitinib成功治疗。
IF 2.7 3区 医学
Journal of Dermatology Pub Date : 2025-12-17 DOI: 10.1111/1346-8138.70106
Risa Yamada, Hisamu Tanaka, Kentaro Yamamura, Tomoko Yoshida, Teruki Dainichi
{"title":"A Pediatric Case of Wells Syndrome Presenting With Recurrent Annular Erythema and Fever Successfully Treated With Baricitinib","authors":"Risa Yamada,&nbsp;Hisamu Tanaka,&nbsp;Kentaro Yamamura,&nbsp;Tomoko Yoshida,&nbsp;Teruki Dainichi","doi":"10.1111/1346-8138.70106","DOIUrl":"10.1111/1346-8138.70106","url":null,"abstract":"","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"53 2","pages":"e152-e154"},"PeriodicalIF":2.7,"publicationDate":"2025-12-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145769844","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}
引用次数: 0
Amicrobial Pustulosis of the Folds Successfully Treated With Apremilast: Report of Two Cases and Review of the Literature 阿普米司特成功治疗皱襞细菌性脓疱病2例报告并文献复习。
IF 2.7 3区 医学
Journal of Dermatology Pub Date : 2025-12-15 DOI: 10.1111/1346-8138.70113
Luca Bettolini, Carlo Alberto Maronese, Stefano Bighetti, Giovanni Genovese, Luisa Sarno, Stefano Buffon, Francesca Laura Boggio, Federica Derlino, Angelo Valerio Marzano
{"title":"Amicrobial Pustulosis of the Folds Successfully Treated With Apremilast: Report of Two Cases and Review of the Literature","authors":"Luca Bettolini,&nbsp;Carlo Alberto Maronese,&nbsp;Stefano Bighetti,&nbsp;Giovanni Genovese,&nbsp;Luisa Sarno,&nbsp;Stefano Buffon,&nbsp;Francesca Laura Boggio,&nbsp;Federica Derlino,&nbsp;Angelo Valerio Marzano","doi":"10.1111/1346-8138.70113","DOIUrl":"10.1111/1346-8138.70113","url":null,"abstract":"<div>\u0000 \u0000 <p>Amicrobial pustulosis of the folds (APF) is a rare, chronic-relapsing neutrophilic dermatosis characterized by sterile pustules affecting major and minor skin folds. It predominantly affects women and is frequently associated with autoimmune diseases such as systemic lupus erythematosus, inflammatory bowel disease, and autoimmune thyroiditis. Due to its rarity, standardized treatment guidelines are lacking, and management remains challenging. Systemic corticosteroids, dapsone, colchicine, methotrexate, and biologics have been employed with variable outcomes, but long-term control is often difficult to achieve. Apremilast, an oral phosphodiesterase-4 inhibitor with anti-inflammatory properties, has demonstrated efficacy in various neutrophilic dermatoses. We report two women with treatment-refractory APF who achieved clinical remission within 2 months of initiating apremilast, with sustained disease control at 6 months and successful corticosteroid tapering. A literature review of 78 APF cases confirmed a strong female predominance (93.6%) and frequent association with autoimmune conditions (91%). Systemic corticosteroids were the most frequently employed treatment but often failed to provide sustained disease control without the addition of other systemic agents. These cases represent the first reports of apremilast use in APF and suggest its potential as a safe and effective steroid-sparing option in patients with refractory disease. Further studies are needed to validate its role in this setting.</p>\u0000 </div>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"53 2","pages":"339-350"},"PeriodicalIF":2.7,"publicationDate":"2025-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145759111","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}
引用次数: 0
Association Between Prurigo Nodularis and Malignancy: A Real-World Multicenter Retrospective Cohort Study 结节性痒疹与恶性肿瘤的关系:一项真实世界多中心回顾性队列研究。
IF 2.7 3区 医学
Journal of Dermatology Pub Date : 2025-12-12 DOI: 10.1111/1346-8138.70112
Yu-Hsiang Lin, Yu-Ching Wang, Tai-Li Chen, Ling-Chuan Jaun, Cheng-Jui Tsai, Chih-Chiang Chen, Cian-Hao Ye, Sheng-Hsiang Ma
{"title":"Association Between Prurigo Nodularis and Malignancy: A Real-World Multicenter Retrospective Cohort Study","authors":"Yu-Hsiang Lin,&nbsp;Yu-Ching Wang,&nbsp;Tai-Li Chen,&nbsp;Ling-Chuan Jaun,&nbsp;Cheng-Jui Tsai,&nbsp;Chih-Chiang Chen,&nbsp;Cian-Hao Ye,&nbsp;Sheng-Hsiang Ma","doi":"10.1111/1346-8138.70112","DOIUrl":"10.1111/1346-8138.70112","url":null,"abstract":"<div>\u0000 \u0000 <p>Prurigo nodularis (PN) is a chronic inflammatory skin disorder associated with various systemic disorders. However, its potential link to increased malignancy risk remains unclear. We conducted a retrospective cohort study using the US Collaborative Network in the TriNetX database, encompassing data from January 1, 2016 to January 1, 2022. Adults diagnosed with PN (<i>n</i> = 10 941) were matched 1:1 with controls without PN (<i>n</i> = 10 941) based on demographics, comorbidities, and medication use. The primary outcome was the hazard ratio (HR) for malignancy occurring between 3 months and 5 years after the index date. The HRs and 95% confidence intervals (CIs) were calculated using Cox proportional hazards models. Patients with PN exhibited a significantly increased risk of developing malignancies compared with the controls (HR 2.10; 95% CI 1.81–2.43). Notably, higher risks were observed for cutaneous squamous cell carcinoma (HR 4.24; 95% CI 2.69–6.69), basal cell carcinoma (HR 2.49; 95% CI 1.68–3.69), hematopoietic cancers (HR 1.97; 95% CI 1.26–3.06), gastrointestinal cancers (HR 1.87; 95% CI 1.24–2.81), respiratory system cancers (HR 1.86; 95% CI 1.23–2.82), and female genital cancers (HR 2.77; 95% CI 1.29–5.95). In conclusion, PN is associated with a significantly elevated risk of malignancy, particularly cutaneous cancers. These preliminary findings underscore the necessity for heightened clinical vigilance. Further prospective studies are needed to confirm this association, elucidate the underlying mechanisms, and evaluate the potential benefits of routine cancer screening in this high-risk population.</p>\u0000 </div>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"53 2","pages":"281-287"},"PeriodicalIF":2.7,"publicationDate":"2025-12-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145746523","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}
引用次数: 0
Generalized Linear Porokeratosis Treated With Ixekizumab Ixekizumab治疗全身性线状角化症。
IF 2.7 3区 医学
Journal of Dermatology Pub Date : 2025-12-12 DOI: 10.1111/1346-8138.70103
Yihe Zheng, Yunhao Zhu, Jianjun Qiao
{"title":"Generalized Linear Porokeratosis Treated With Ixekizumab","authors":"Yihe Zheng,&nbsp;Yunhao Zhu,&nbsp;Jianjun Qiao","doi":"10.1111/1346-8138.70103","DOIUrl":"10.1111/1346-8138.70103","url":null,"abstract":"<div>\u0000 \u0000 <p>We reported a 15-year-old boy with extensive linear porokeratosis confirmed by clinical, histopathologic, and immunohistochemical assessment. Ixekizumab treatment lead to a near-complete clearance of lesions by 3 months, while nail dystrophy showed minimal response. The presence of IL-17A-positive infiltrates supported the immunologic basis for therapeutic efficacy. These findings suggest that ixekizumab is a promising targeted therapy for refractory linear porokeratosis.</p>\u0000 </div>","PeriodicalId":54848,"journal":{"name":"Journal of Dermatology","volume":"53 2","pages":"e146-e148"},"PeriodicalIF":2.7,"publicationDate":"2025-12-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145746580","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}
引用次数: 0
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