Journal Der Deutschen Dermatologischen Gesellschaft最新文献

{"title":"Kongresskalender 2025","authors":"","doi":"10.1111/ddg.15729","DOIUrl":"https://doi.org/10.1111/ddg.15729","url":null,"abstract":"","PeriodicalId":14758,"journal":{"name":"Journal Der Deutschen Dermatologischen Gesellschaft","volume":"23 3","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-03-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143564640","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":"Immuncheckpoint-Inhibitoren bei Kindern mit Xeroderma pigmentosum und fortgeschrittenem kutanen Plattenepithelkarzinom: Fallvorstellung und kurzer Überblick","authors":"Thilo Gambichler, Julia Hyun, Frank Oellig, Jürgen C. Becker, Alexander Kreuter","doi":"10.1111/ddg.15648_g","DOIUrl":"https://doi.org/10.1111/ddg.15648_g","url":null,"abstract":"<p>Xeroderma pigmentosum (XP) ist eine seltene, autosomal-rezessiv vererbte Geno-Photodermatose, die durch Defekte in den DNA-Reparaturmechanismen gekennzeichnet ist und zu einer extremen Empfindlichkeit gegenüber ultravioletter (UV-)Strahlung führt. XP-Patienten haben ein hohes Risiko für schwere Sonnenbrände, Hauttrockenheit, fortschreitende Pigmentstörungen, vorzeitige Lichtalterung und ein dramatisch erhöhtes Auftreten von bösartigen Hauttumoren in UV-exponierten Bereichen wie Gesicht, Hals und Kopf.<span><sup>1-3</sup></span> Das Risiko, an Hautkrebs zu erkranken, ist bei XP-Patienten im Vergleich zur Allgemeinbevölkerung bis zu 10 000-mal höher für nichtmelanozytäre Hauttumoren (<i>non-melanoma skin cancers</i>; NMSC) wie Basalzellkarzinome und kutane Plattenepithelkarzinome (<i>cutaneous squamous cell carcinomas</i>; cSCC); die Inzidenz von Melanomen ist Berichten zufolge 2000-mal höher. Andere Hauttumoren wie Keratoakanthome, Talgdrüsenkarzinome, Fibrosarkome und Angiosarkome treten ebenfalls häufiger auf. Patienten mit XP, die an Hautkrebs erkranken, neigen zu aggressiveren Malignomen mit einem höheren Risiko der Metastasierung und des krebsbedingten Todes. Das mittlere Sterbealter bei XP-Patienten liegt bei 32 Jahren, wobei 60% der Todesfälle vor dem 20. Lebensjahr auftreten.<span><sup>4-6</sup></span> Studien zeigen, dass XP-bedingter Hautkrebs eine 3,6-fach höhere Mutationslast als sporadischer Hautkrebs aufweist. Besonders deutlich wird dies bei XP-Gruppen mit Defiziten in der globalen Genom-Nukleotid-Exzisionsreparatur (NER, zum Beispiel XP-C und XP-E) und solchen, denen die funktionelle Translesionssynthese-Polymerase η fehlt (XP-V). Die mittlere Tumormutationslast (TMB) für einzelne Basensubstitutionen in bestimmten XP-Gruppen kann bis zu 350 Mutationen pro Megabase erreichen. Der vorherrschende Mutationstyp, der in XP-Tumoren beobachtet wird, ist der C>T-Übergang, insbesondere an Dipyrimidin-Stellen, die häufig Ziel von UV-Schäden sind. Jede XP-Gruppe weist unterschiedliche Mutationssignaturen auf, die auf die unterschiedlichen DNA-Reparaturfähigkeiten und Expositionsverläufe zurückzuführen sind.</p><p>Daher sollten cSCC von XP-Patienten sehr empfänglich für Therapien mit Immuncheckpoint-Inhibitoren (ICI) sein. Während Cemiplimab und andere ICI bei immunkompetenten Patienten mit fortgeschrittenem cSCC hervorragende Ergebnisse in Bezug auf das progressionsfreie Überleben und das Gesamtüberleben gezeigt haben,<span><sup>7-23</sup></span> ist die Wirksamkeit von ICI bei XP-Patienten, insbesondere bei pädiatrischen Fällen, weniger gut beschrieben. Wir berichten über ein vollständiges Ansprechen auf Cemiplimab-Therapie bei einem Jungen mit fortgeschrittenem cSCC und zugrundeliegender XP und geben einen kurzen Überblick zum Thema.</p><p>Im November 2023 behandelten wir einen 7-jährigen Jungen aus Afghanistan mit XP, Typ C. Er hatte zwei Geschwister, die ebenfalls an XP litten und beide im <i>Friedensdorf</i>, einer gemeinnützigen Ei","PeriodicalId":14758,"journal":{"name":"Journal Der Deutschen Dermatologischen Gesellschaft","volume":"23 3","pages":"303-310"},"PeriodicalIF":5.5,"publicationDate":"2025-03-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ddg.15648_g","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143565018","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cutaneous plasmacytoma as presentation of multiple myeloma.","authors":"Raquel María Domínguez López, Alejandra Veliz Domínguez, Jorge Naharro-Rodríguez","doi":"10.1111/ddg.15664","DOIUrl":"https://doi.org/10.1111/ddg.15664","url":null,"abstract":"","PeriodicalId":14758,"journal":{"name":"Journal Der Deutschen Dermatologischen Gesellschaft","volume":" ","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143542225","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":"High detection rate for perivascular deposits of immunoglobulins in immune complex vasculitis from biopsies of early macular lesions","authors":"Luisa Herda,&nbsp;Christiane Michl,&nbsp;Cord Sunderkötter","doi":"10.1111/ddg.15636","DOIUrl":"10.1111/ddg.15636","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>In immune complex vasculitis the detection of perivascular immunoglobulins by direct immunofluorescence (DIF) not only helps to confirm the diagnosis, but also to define the type of vasculitis (e.g., IgA-, IgG/IgM-, rheumatoid or cryoglobulinemic vasculitis). The value of DIF, though, has been questioned due to the heterogeneous yield of positive reactions in various studies. One major reason for a negative DIF is a biopsy of older lesions. To ensure selection of fresh lesions, we consistently apply morphological criteria: partially blanchable macules with only a minor petechial and papular component and in proximity to palpable or retiform purpura. This study aimed to evaluate retrospectively the detection rate attainable by this procedure.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Patients and Methods</h3>\u0000 \u0000 <p>In our department, we identified 56 patients from 2017–2024 with histologically and clinically confirmed immune complex vasculitis from whom a corresponding biopsy had been obtained.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>92.9% of these patients showed perivascular deposition of at least one immunoglobulin (mostly IgA (85,7%), with or without IgG or IgM, 7,1% showed no IgA, but IgG or IgM). Biopsies positive only for C3 were considered negative. Of the IgA-positive patients 15% had a systemic, 83% a skin-limited IgA-vasculitis and 2% recurrent macular vasculitis.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>When using defined morphological or clinical criteria for selecting appropriate biopsy sites, DIF demonstrates high sensitivity in identifying the nature of perivascular immunoglobulins in immune complex vasculitis and may serve as a valid criterion in diagnostic algorithms.</p>\u0000 </section>\u0000 </div>","PeriodicalId":14758,"journal":{"name":"Journal Der Deutschen Dermatologischen Gesellschaft","volume":"23 4","pages":"479-485"},"PeriodicalIF":5.5,"publicationDate":"2025-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/ddg.15636","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143536946","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Regression of cutaneous melanoma metastases concomitant with the onset of vitiligo.","authors":"Nadia Ghariani Fetoui, Nour El Imen Ouni, Sana Mokni, Haifa Mkhinini, Najet Ghariani, Mohamed Denguezli","doi":"10.1111/ddg.15666","DOIUrl":"https://doi.org/10.1111/ddg.15666","url":null,"abstract":"","PeriodicalId":14758,"journal":{"name":"Journal Der Deutschen Dermatologischen Gesellschaft","volume":" ","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143542229","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":"Epidemiology of generalized pustular psoriasis in Germany: Analyzing factors influencing prevalence estimates from claims data.","authors":"Michael Schultze, Nils Kossack, Christian Kromer, Thomas M Zimmermann, Nikolaus Kolb","doi":"10.1111/ddg.15633","DOIUrl":"https://doi.org/10.1111/ddg.15633","url":null,"abstract":"<p><strong>Background and objectives: </strong>Generalized pustular psoriasis (GPP) is a rare, chronic, potentially life-threatening skin disease. We aimed to establish criteria to accurately approximate GPP prevalence in Germany.</p><p><strong>Methods: </strong>A retrospective analysis of the WIG2 health claims database (1/1/2016-31/12/2020) was conducted. Patients aged ≥ 12 years continuously enrolled in their statutory health insurance with one inpatient or confirmed outpatient diagnosis code for GPP (International Classification of Diseases, 10^th Revision [ICD-10] L40.1) were included. Scenarios with increasingly strict criteria were used to identify the GPP population.</p><p><strong>Results: </strong>From 2016-2020, 5,236 potential GPP cases were identified based on a recorded GPP diagnosis. The scenario of ≥ 1 GPP diagnosis yielded the highest prevalence (336-390 patients/million) followed by > 1 GPP diagnosis in ≥ 2 quarters (189-288 patients/million); scenarios resulting in the lowest prevalence were diagnosis in ≥ 2 quarters AND two independent diagnoses (17-28/million) and diagnosis in ≥ 2 quarters AND two independent diagnoses or diagnosis by a specialist AND potential flare (58-61 patients/million).</p><p><strong>Conclusions: </strong>This study suggests that diagnosis in ≥ 2 quarters by a specialist or two independent physicians may be the most clinically robust and reliable criteria for estimating GPP prevalence; therefore, 50-100 patients/million may represent a reasonable prevalence estimate range for Germany.</p>","PeriodicalId":14758,"journal":{"name":"Journal Der Deutschen Dermatologischen Gesellschaft","volume":" ","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143536943","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":"Rising challenges in dermatosurgery: Cutler-Beard flap for total upper eyelid defect after squamous cell carcinoma.","authors":"Raphael Wilhelm, Klajdi Begaj, Jakob Veeser, Mahmoud Abdel-Aal, Janina Dietzel, Lilian Kaufmann, Michael Schultheis, Henner Stege, Stephan Grabbe, Hadrian Nassabi","doi":"10.1111/ddg.15673","DOIUrl":"https://doi.org/10.1111/ddg.15673","url":null,"abstract":"","PeriodicalId":14758,"journal":{"name":"Journal Der Deutschen Dermatologischen Gesellschaft","volume":" ","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143542232","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":"Modified nasolabial reading man flap for the reconstruction of nasal sidewall defects.","authors":"Rafael Salido-Vallejo, Javier Antoñanzas, Inés Oteiza-Rius, Elena Querol-Cisneros, Agustín España","doi":"10.1111/ddg.15685","DOIUrl":"https://doi.org/10.1111/ddg.15685","url":null,"abstract":"","PeriodicalId":14758,"journal":{"name":"Journal Der Deutschen Dermatologischen Gesellschaft","volume":" ","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-03-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143536949","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":"Vasculopathy with neurological symptoms.","authors":"Morna F Schmidt, Jan van der Laden, Matthias Begemann, Amir S Yazdi","doi":"10.1111/ddg.15668","DOIUrl":"https://doi.org/10.1111/ddg.15668","url":null,"abstract":"","PeriodicalId":14758,"journal":{"name":"Journal Der Deutschen Dermatologischen Gesellschaft","volume":" ","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-03-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143536951","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":"Clinical characteristics and treatment outcomes of linear IgA bullous dermatosis.","authors":"Kristie Mar, Fiona Landells, Bushra Khalid, Aswen Sriranganathan, Ou Jia Wang, Samantha Y Starkey, Ivan V Litvinov, Ilya Mukovozov","doi":"10.1111/ddg.15644","DOIUrl":"https://doi.org/10.1111/ddg.15644","url":null,"abstract":"<p><p>Linear immunoglobulin A (IgA) bullous dermatosis (LABD), also referred to as chronic bullous disease of childhood (CBDC), is characterized by the linear deposition of IgA antibodies within the dermal-epidermal junction. While dapsone is typically recommended, alternative modalities may be considered based on accessibility, severity, and prior response to therapy. This review aims to provide an updated overview of the clinical characteristics and treatment outcomes for LABD/CBDC. A systematic review was conducted using MEDLINE and Embase in adherence to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines. Results from 650 articles, encompassing 1,627 cases, revealed 52% of cases were male. Vesicles (49%) and bullae (47%) were the predominant morphologies occurring across multiple body locations simultaneously, such as the legs (52%), abdomen (49%), and back (49%). Vancomycin was the most reported causative medication; however, most cases were not drug-induced. Dapsone was the primary therapy for LABD/CBDC while biologics were reported with higher complete response rates. Other immunomodulators, such as IVIG and mycophenolate mofetil, were reported to have comparative responses in CBDC cases, but lower response rates in LABD cases. Colchicine and amoxicillin-clavulanate had lower response rates overall. The variety of treatment options underscores management challenges due to the variable clinical presentations and underlying causes.</p>","PeriodicalId":14758,"journal":{"name":"Journal Der Deutschen Dermatologischen Gesellschaft","volume":" ","pages":""},"PeriodicalIF":5.5,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143523277","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}