Pediatric Dermatology最新文献

{"title":"Ustekinumab as an Effective Therapeutic Option in Papillon-Lefèvre Syndrome: Long-Term Follow-Up.","authors":"Ozlem Apti Sengkioun, Deniz Yücelten, Tülin Ergun","doi":"10.1111/pde.70351","DOIUrl":"https://doi.org/10.1111/pde.70351","url":null,"abstract":"<p><p>Papillon-Lefèvre syndrome (PLS) is a rare autosomal recessive genodermatosis characterized by palmoplantar hyperkeratosis and severe periodontitis, leading to premature loss of both primary and permanent teeth, and treatment responses are often unsatisfactory. We report a 20-year-old female patient with PLS who presented with extensive palmoplantar hyperkeratosis and widespread psoriasiform plaques refractory to multiple conventional therapies. Treatment with ustekinumab resulted in marked clinical improvement by the sixth month after three subcutaneous injections. The patient has remained in sustained clinical remission during 6 years of continuous therapy, with only mild and intermittent recurrent plaques on the knees, suggesting that biologic therapy may represent a sustainable option in selected cases of PLS.</p>","PeriodicalId":19819,"journal":{"name":"Pediatric Dermatology","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148875387","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinicopathologic Characteristics, Treatment Outcomes of Pediatric Mycosis Fungoides in Turkey: A National Multicenter Retrospective Cohort.","authors":"Dilek Bayramgürler, Dilek Seçkin, Ayşe Deniz Yücelten, Esin Diremsizoğlu, Esra Adışen, Bengü Nisa Akay, Salih Levent Çınar, Pelin Kartal, Aslı Aksu, Burhan Engin, Arzu Ferhatosmanoğlu, Mehmet Melikoğlu, Zeynep Karaca Ural, Tuğba Kevser Uzunçakmak, Serkan Yazıcı, Esma İnan Yüksel, Ayşegül Yalçınkaya İyidal, Özlem Özbağçıvan, Emre Güven, Gizem Nur Öztürk Dönmez, Muhammed Salih Karagöz, Hatice Erdi Şanlı","doi":"10.1111/pde.70349","DOIUrl":"https://doi.org/10.1111/pde.70349","url":null,"abstract":"<p><strong>Background and objectives: </strong>This study aimed to characterize the clinical spectrum, diagnostic delay, treatment patterns, and outcomes of pediatric mycosis fungoides (MF) and to identify predictors of early response.</p><p><strong>Methods: </strong>This national multicenter retrospective study included patients diagnosed with histopathologically confirmed MF before 18 years of age. Demographic, clinical, histopathologic, immunophenotypic, staging, treatment, and follow-up data were collected. Early response at 3-6 months (partial/complete response) was analyzed using logistic regression.</p><p><strong>Results: </strong>A total of 133 patients were included; 60.9% (81/133) were male. Median age at diagnosis was 13 years (IQR, 9-15) and median diagnostic delay was 18 months (IQR, 8-48). Classic morphology was recorded in 78.2% (104/133), and hypopigmented MF in 36.1% (48/133); overlapping morphologies were documented in 42.1% (56/133). Stage IA disease was present in 59.4% (79/133). Phototherapy was used in 60.9% (81/133). Early response occurred in 91.0% (121/133). BSA < 10% was independently associated with higher odds of early response (aOR 8.42, 95% CI 1.71-41.54; p = 0.009), whereas older age at diagnosis predicted lower odds (aOR 0.81 per year, 95% CI 0.67-0.98; p = 0.029). Relapse occurred in 28.5% (37/130) within median follow-up of 22 months (median time to relapse of 8 months); 67.6% (25/37) relapsed within 12 months.</p><p><strong>Conclusion: </strong>In this cohort, pediatric MF presented at an early stage. Despite favorable early responses, relapse occurred in nearly one third, often within the first year, supporting long-term surveillance. Higher BSA involvement and older age at diagnosis were associated with reduced early response.</p>","PeriodicalId":19819,"journal":{"name":"Pediatric Dermatology","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148875341","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pediatric Non-Neural Granular Cell Tumor With Unexpected Molecular Identity: A Case That Challenges Classification.","authors":"Grace S Saglimbeni, Eduardo Zambrano, Sierra Wolter, Nawal Merjaneh, Kristian Schafernak, Brian Keehn, Peaches Ulrich, Maria Epino, Danielle Vargas de Stefano","doi":"10.1111/pde.70324","DOIUrl":"https://doi.org/10.1111/pde.70324","url":null,"abstract":"<p><p>A 7-year-old boy presented with a 3-year history of a slow-growing superficial cutaneous nodule adjacent to the right acromion, with subsequent development of a second subcutaneous mass near the right scapula. Biopsy of the primary lesion revealed a rare non-neural granular cell tumor (NNGCT) lacking S100 expression, distinguishing it from classic granular cell tumors (GCT), and molecular profiling identified a DCTN1::ALK fusion alongside an ATP6AP2 frameshift mutation, the latter reported predominantly in GCT. To our knowledge, this is the first documented NNGCT harboring both alterations, suggesting potential biologic overlap between NNGCT and GCT while expanding the current framework of granular cell tumor classification. This case also highlights the reported potential for recurrence and multifocal disease or regional spread in NNGCT, supporting complete surgical excision with long-term follow-up.</p>","PeriodicalId":19819,"journal":{"name":"Pediatric Dermatology","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148875366","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"GLP-1 Receptor Agonists and Pediatric Dermatology: An Emerging Clinical Intersection.","authors":"Seema Kumar","doi":"10.1111/pde.70353","DOIUrl":"https://doi.org/10.1111/pde.70353","url":null,"abstract":"","PeriodicalId":19819,"journal":{"name":"Pediatric Dermatology","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148875347","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Vibration, Pressure, and Cryoanalgesia to Minimize Acral Injection Pain.","authors":"Shauna Rice, Kelly Flanagan, Dori Goldberg","doi":"10.1111/pde.70338","DOIUrl":"https://doi.org/10.1111/pde.70338","url":null,"abstract":"<p><p>Acral injections for conditions such as palmoplantar hyperhidrosis are associated with significant procedural pain, frequently limiting their use in pediatric populations. While nerve blocks, cryoanalgesia, and topical anesthetics can mitigate discomfort during palmar injections, each has practical limitations. This report describes a multimodal technique combining cryoanalgesia, pressure, and vibration using a handheld massager that substantially reduces injection-related pain and patient distress.</p>","PeriodicalId":19819,"journal":{"name":"Pediatric Dermatology","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148866248","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Autosomal Dominant Severe Epidermolysis Bullosa Simplex Treated With Dupilumab.","authors":"Travis Jackson, Taylor S Davis, Christopher Castelow, Jonathan A Dyer","doi":"10.1111/pde.70336","DOIUrl":"https://doi.org/10.1111/pde.70336","url":null,"abstract":"<p><p>Epidermolysis bullosa simplex (EBS) is an inherited mechanobullous disorder with limited systemic treatment options. Emerging evidence suggests that inflammatory pathways and pruritus may significantly contribute to disease severity, creating the possibility for therapeutic targets. This report describes a premature infant with EBS, severe (EBS-sev) and failure to thrive, who was treated with dupilumab at 5 months and 12 days of age. Treatment resulted in sustained improvement in skin integrity and clinical stability, suggesting that dupilumab may be considered as a potential therapeutic option for future cases of EBS.</p>","PeriodicalId":19819,"journal":{"name":"Pediatric Dermatology","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148850938","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Illuminating Connections: Exploring the Dynamic Relationship Between Phototherapy and the Skin.","authors":"Meshi Paz, Peter Lio","doi":"10.1111/pde.70330","DOIUrl":"https://doi.org/10.1111/pde.70330","url":null,"abstract":"<p><p>The neonatal period represents a critical window for skin barrier maturation, microbial colonization, and immune development. As such, early-life exposures may exert lasting effects on dermatologic and systemic health. A common early-life exposure is blue light phototherapy (BLP), a life-saving treatment for neonatal hyperbilirubinemia. Although BLP is traditionally regarded as a localized, superficial intervention, emerging evidence suggests that it is a biologically active exposure that directly interacts with developing skin, thereby influencing barrier integrity, the developing microbiome, and immune signaling. As an active immune organ, neonatal skin has been proposed to engage in dynamic crosstalk with the nervous, gut, and endocrine systems. Therefore, BLP may act systemically by modulating these interconnections. These effects raise concerns about the potential long-term impact of BLP during this critical developmental period. The development of immune-mediated conditions, such as atopic dermatitis (AD), is of particular interest as they are hypothesized to engage in these cross-system networks. This review examines the multifactorial relationship between BLP and neonatal skin development, highlighting BLP as an active exposure that may interact with developing skin, microbiome, and immune system, while emphasizing the need for longitudinal studies to clarify long-term clinical implications.</p>","PeriodicalId":19819,"journal":{"name":"Pediatric Dermatology","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148850993","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Congenital Skin Sloughing in the Setting of Hydrops Fetalis Secondary to Congenital Diffuse Lymphangiectasia Mimicking Epidermolysis Bullosa.","authors":"Sophia Rafferty, Kate L Wilson, Jennifer B Mancuso, Nicole Larsen, Grace A Osborne, Jacqueline Meadow","doi":"10.1111/pde.70332","DOIUrl":"https://doi.org/10.1111/pde.70332","url":null,"abstract":"<p><p>We present a case where the combination of severe hydrops fetalis, lymphangiectasia, and skin desquamation created a clinical picture that masqueraded as epidermolysis bullosa (EB). A neonate presented at birth with severe hydrops fetalis and extensive skin sloughing. Skin biopsy findings were consistent with EB; however, the clinical course and genetic testing were inconsistent with this diagnosis, and the postmortem examination revealed the underlying cause to be severe congenital diffuse lymphangiectasia. It is important for clinicians to consider edema bulla and tissue hypoxia as a cause of skin blistering and sloughing in the setting of hydrops fetalis.</p>","PeriodicalId":19819,"journal":{"name":"Pediatric Dermatology","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148850987","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Are Pediatric GLP-1 Receptor Agonist Cutaneous Adverse Events Being Overlooked? Emerging Age-Specific Patterns and Their Implications for Dermatologists.","authors":"Margaret F Cote, Vikash S Oza, Seth J Orlow","doi":"10.1111/pde.70340","DOIUrl":"https://doi.org/10.1111/pde.70340","url":null,"abstract":"<p><p>Glucagon-like peptide-1 receptor agonists (GLP-1 RAs) use in children is rapidly expanding, though in contrast to adults, morphologic data on dermatologic adverse events (AEs) in pediatric patients remain sparse, underscoring the need for further focused investigation. We review and synthesize the existing literature on dermatologic AEs of GLP-1 RAs in children in comparison to adults, to summarize current evidence, identify knowledge gaps, and highlight areas for future research. Available reports suggest that rash, urticaria, and alopecia may occur with greater frequency in children on injectable GLP-1 RAs compared with adults, while facial lipodystrophy has been more often described in adults. However, pediatric dermatologic AE data are not as robust as adult data, reducing the ability for direct comparisons. Based on this initial investigation, dermatologic AEs of GLP-1 RAs are seemingly under-described in children and may have important differences compared with adult dermatologic AEs.</p>","PeriodicalId":19819,"journal":{"name":"Pediatric Dermatology","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148840578","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Macular Arteritis: A Report of Two Pediatric Cases.","authors":"Aina Estany-Bécares, Trinidad Repiso Montero, Berta Ferrer Fábrega, Sergi Pujadas Vilà, Vicente García-Patos Briones","doi":"10.1111/pde.70326","DOIUrl":"https://doi.org/10.1111/pde.70326","url":null,"abstract":"<p><p>We report two pediatric cases of macular arteritis presenting as asymptomatic retiform pigmented macules on the legs. Biopsy showed deep dermal-subcutaneous lymphocytic arteritis with fibrin deposition and luminal narrowing, with a negative infectious and autoimmune workup. Both patients remained well with stable lesions at 1 and 7 years of follow-up. These cases add to the limited pediatric literature and support macular arteritis as an infrequent benign cutaneous arteritis, helping avoid unnecessary investigations and aggressive treatment.</p>","PeriodicalId":19819,"journal":{"name":"Pediatric Dermatology","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148840743","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}