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Published By S. Karger Ag

2296-536x, 2296-5424

2022 ◽  
pp. 1-6
Author(s):  
Werner Kempf ◽  
Christina Mitteldorf

Kutane T-Zell-Lymphome (CTCL) machen den Großteil aller primären kutanen Lymphome (CL) aus. 80 % aller Fälle von CTCL wiederum entfallen auf Mycosis fungoides (MF) und CD30-positive lymphoproliferative Erkrankungen der Haut. Das klinische Bild der verschiedenen Formen von CTCL zeigt Überschneidungen. Darum ist die klinisch-pathologische Korrelation von großer Bedeutung für die finale Diagnosestellung. Die MF zeigt einen charakteristischen Verlauf mit makuläre Läsionen (Patches), infiltrierte Plaques und, bei einem Teil der Patienten (10–20 %), Tumoren. Die Behandlung erfolgt stadienorientiert mit auf die Haut gerichteten Therapien wie UV-Lichttherapien und Kortikosteroiden in frühen Stadien der Erkrankung (Patch- und begrenztes Plaque-Stadium) und systemischen Therapien (Retinoide bzw. Rexinoide, Interferon, Monochemotherapie, zielgerichtete Therapie) und/oder Strahlentherapie (lokal oder Ganzkörperbestrahlung mit Elektronen) in fortgeschrittenen Stadien. Neuartige Ansätze umfassen zielgerichtete Therapien wie Mogamulizumab (Antikörper gegen CCR4) oder Brentuximab Vedotin (Antikörper gegen CD30) sowie Histon-Deacetylase-Inhibitoren. Angesichts des Impacts von zielgerichteten Therapien sind Biomarker wie CD30 nicht nur für die Diagnosestellung und korrekte Klassifikation eines Lymphoms im Einzelfall von großer Bedeutung, sondern als potenzielle Wirkstoff-Zielmoleküle auch für die Therapie. In der kürzlich überarbeiteten WHO-Klassifikation von 2017 und der aktualisierten WHO-EORTC-Klassifikation der CL von 2018 ist erstmals das CD8-positive akrale T-Zell-Lymphom als eigene, noch provisorische Entität aufgeführt. Diese Form zeigt charakteristische klinische, histologische und phänotypische Merkmale und eine hervorragende Prognose. Zu den seltenen, aber aggressiven CTCL zählen das primär kutane aggressive epidermotrope CD8-positive T-Zell-Lymphom und das kutane Gamma/Delta-T-Zell-Lymphom, die durch rasches Auftreten nekrotischer oder ulzerierender Plaques und Tumoren gekennzeichnet sind. Da bei diesen Formen die Prognose ungünstig ist, umfasst die Behandlung Polychemotherapien und hämatopoetische Stammzelltransplantationen.


2022 ◽  
pp. 1-3
Author(s):  
Monira Abdullah Alnasser ◽  
Nour Marwan AlKhawajah ◽  
Nada Al-Qadri ◽  
Asem Mustafa Shadid ◽  
Fahad M. Alsaif 
Keyword(s):  

Unter dem Begriff der kutanen T-Zell-Lymphome (CTCL) wird eine Gruppe lymphoproliferativer Erkrankungen zusammengefasst, die durch Lokalisation neoplastischer T-Lymphozyten in der Haut gekennzeichnet sind. Die Mycosis fungoides (MF) ist die häufigste Form von CTCL; sie macht ∼60 % aller primären kutanen Lymphome aus. Neben der klassischen Form der MF sind zahlreiche klinische und histopathologische Varianten beschrieben worden. Die malignen Lymphozyten bei MF sind meist positiv für CD3, CD4 und CD45RO und negativ für CD8. Ein ungewöhnlicheres immunhistochemisches Profil eines CD4-negativen und CD8-positiven reifen T-Zell-Phänotyps wird bei einer Minderheit der Patienten beschrieben; in bis zu 20 % der Fälle von MF im Frühstadium liegt ein CD8-positiver Phänotyp vor. Über einen für CD4 und CD8 doppelt-negativen MF-Phänotyp gibt es nur einige wenige Berichte in der Literatur. Wir stellen hier den Fall eines 60-jährigen Mannes mit MF mit CD4/CD8-doppelt-negativem Phänotyp vor.


2022 ◽  
pp. 1-2
Author(s):  
Markus Stücker

<b>Importance:</b> One-year outcomes from the Early Venous Reflux Ablation (EVRA) randomized trial showed accelerated venous leg ulcer healing and greater ulcer-free time for participants who are treated with early endovenous ablation of lower extremity superficial reflux. <b>Objective:</b> To evaluate the clinical and cost-effectiveness of early endovenous ablation of superficial venous reflux in patients with venous leg ulceration. <b>Design, Setting, and Participants:</b> Between October 24, 2013, and September 27, 2016, the EVRA randomized clinical trial enrolled 450 participants (450 legs) with venous leg ulceration of less than 6 months’ duration and superficial venous reflux. Initially, 6555 patients were assessed for eligibility, and 6105 were excluded for reasons including ulcer duration greater than 6 months, healed ulcer by the time of randomization, deep venous occlusive disease, and insufficient superficial venous reflux to warrant ablation therapy, among others. A total of 426 of 450 participants (94.7%) from the vascular surgery departments of 20 hospitals in the United Kingdom were included in the analysis for ulcer recurrence. Surgeons, participants, and follow-up assessors were not blinded to the treatment group. Data were analyzed from August 11 to November 4, 2019. <b>Interventions:</b> Patients were randomly assigned to receive compression therapy with early endovenous ablation within 2 weeks of randomization (early intervention, n  =  224) or compression with deferred endovenous treatment of superficial venous reflux (deferred intervention, n  =  226). Endovenous modality and strategy were left to the preference of the treating clinical team. <b>Main Outcomes and Measures:</b> The primary outcome for the extended phase was time to first ulcer recurrence. Secondary outcomes included ulcer recurrence rate and cost-effectiveness. <b>Results:</b> The early-intervention group consisted of 224 participants (mean [SD] age, 67.0 [15.5] years; 127 men [56.7%]; 206 White participants [92%]). The deferred-intervention group consisted of 226 participants (mean [SD] age, 68.9 [14.0] years; 120 men [53.1%]; 208 White participants [92%]). Of the 426 participants whose leg ulcer had healed, 121 (28.4%) experienced at least 1 recurrence during follow-up. There was no clear difference in time to first ulcer recurrence between the 2 groups (hazard ratio, 0.82; 95% CI, 0.57–1.17; P  =  .28). Ulcers recurred at a lower rate of 0.11 per person-year in the early-intervention group compared with 0.16 per person-year in the deferred-intervention group (incidence rate ratio, 0.658; 95% CI, 0.480–0.898; P  =  .003). Time to ulcer healing was shorter in the early-intervention group for primary ulcers (hazard ratio, 1.36; 95% CI, 1.12–1.64; P  =  .002). At 3 years, early intervention was 91.6% likely to be cost-effective at a willingness to pay of £20 000 ($26 283) per quality-adjusted life year and 90.8% likely at a threshold of £35 000 ($45 995) per quality-adjusted life year. <b>Conclusions and Relevance:</b> Early endovenous ablation of superficial venous reflux was highly likely to be cost-effective over a 3-year horizon compared with deferred intervention. Early intervention accelerated the healing of venous leg ulcers and reduced the overall incidence of ulcer recurrence. <b>Trial Registration:</b> ClinicalTrials.gov identifier: ISRCTN02335796.


2022 ◽  
pp. 1-2
Author(s):  
Percy Lehmann

Actinic keratoses are a chronic condition in ultraviolet-damaged skin, with a risk of progressing to invasive skin cancer. The aim of this study was to investigate the preventive potential of field-directed repetitive daylight photodynamic therapy for actinic keratoses. A randomized trial was performed, including 58 patients with ≥5 actinic keratoses on photodamaged facial skin, who received either 5 full-face sessions of daylight photodynamic therapy within a period of 2 years or lesion-directed cryosurgery. Primary outcome was the mean cumulative number of new actinic keratoses developed between visits 2 and 6 (visit 6 being a follow-up). This outcome was lower after daylight photo-dynamic therapy (7.7) compared with cryosurgery (10.2), but the difference did not reach significance (–2.5, 95% confidence interval –6.2 to 1.2; p = 0.18). Several signs of photoageing (fine lines, pigmentation, roughness, erythema, sebaceous gland hyperplasia) were significantly reduced after daylight photodynamic therapy, but not after cryosurgery. Significantly less pain and fewer side-effects were reported during daylight photodynamic therapy than during cryosurgery. This study found that repetitive daylight photodynamic therapy had photo-rejuvenating effects. However, the prevention of actinic keratoses by this therapy could not be proven in a statistically reliable manner.


2021 ◽  
pp. 1-3
Author(s):  
Michael Sticherling

<b>Introduction:</b> Secukinumab, a fully human monoclonal antibody that directly inhibits interleukin-17A, has demonstrated robust efficacy in the treatment of moderate to severe psoriasis (PsO), psoriatic arthritis (PsA) and ankylosing spondylitis (AS), with a rapid onset of action, sustained long-term clinical responses and a consistently favourable safety profile across phase 3 trials. Here, we report the clinical data at enrolment from SERENA, designed to investigate the real-world use of secukinumab across all three indications. <b>Methods:</b> SERENA is an ongoing, longitudinal, observational study conducted at 438 sites across Europe in patients with moderate to severe plaque PsO, active PsA or active AS. Patients should have received at least 16 weeks of secukinumab treatment before enrolment in the study. <b>Results:</b> Overall 2800 patients were included in the safety set; patients with PsA (N = 541) were older than patients with PsO (N = 1799) and patients with AS (N =  460); patients with PsO had a higher mean body weight than patients with PsA and patients with AS; and patients with PsO and patients with AS were predominantly male. Time since diagnosis was longer in patients with PsO compared with patients with PsA and patients with AS, and about 40% of patients were either current or former smokers. The proportion of obese patients (body mass index ≥ 30 kg/m<sup>2</sup>) was similar across indications. Patients were treated with secukinumab for a mean duration of 1 year prior to enrolment (range 0.89–1.04). The percentages of patients with prior biologics exposure were 31.5% PsO, 59.7% PsA and 55% AS. The percentages of patients prescribed secukinumab monotherapy were 75% (n =  1349) in PsO, 48.2% (n = 261) in PsA and 48.9% (n = 225) in AS groups. <b>Conclusion:</b> Baseline demographics of the study population are consistent with existing literature. This large observational study across all secukinumab indications will provide valuable information on the long-term effectiveness and safety of secukinumab in the real-world setting.


2021 ◽  
pp. 1-5
Author(s):  
Kaspar Itin ◽  
Peter Häusermann ◽  
Peter F. Whitington ◽  
Nicole Fosse

Das juvenile Xanthogranulom (JXG) ist die häufigste Form der Non-Langerhans-Zell-Histiozytose. Es handelt sich um einen seltenen, angeborenen oder im späteren Alter auftretenden gutartigen Tumor. Die klassische Form des JXG ist durch rötlich-gelbe benigne Papeln oder Knötchen mit Prädilektion am Kopf, Hals und Rumpf gekennzeichnet, doch können auch den Extremitäten oder extrakutanen Stellen Läsionen auftreten. In den meisten Fällen findet sich nur eine solitäre Läsion, allerdings können auch multiple Papeln oder Knötchen vorliegen. Sonderformen sind unter anderem das gemischte, riesige, subkutane, eruptive, gruppierte und plaqueartige JXG, und das JXG wurde überdies mit systemischen Erkrankungen in Verbindung gebracht. Die Diagnose wird im Wesentlichen auf Grundlage des klinischen Erscheinungsbildes gestellt und in der Regel durch die histologische Untersuchung bestätigt. In der vorliegenden Arbeit berichten wir über einen sehr seltenen Fall eines symmetrischen juvenilen Riesenxanthogranuloms vom Plaque-Typ im Gesicht (symmetrical giant facial plaque-type juvenile xanthogranuloma, SGFP-JXG); außerdem nehmen wir einen Vergleich mit der klassischen Form des JXG sowie JXG-Varianten vor und diskutieren die Differentialdiagnosen. Vorgestellt wurde uns ein 4-jähriges Mädchen kaukasischer Abstammung mit plaqueartigen Läsionen auf beiden Wangen, die aus gelblichen konfluierenden Papeln bestanden. Die histologische Untersuchung zeigte eine histiozytäre Läsion mit Bildung von Touton-Riesenzellen, und die immunhistochemischen Ergebnisse bestätigten die Diagnose SGFP-JXG. Im Vergleich zum klassischen JXG tritt das SGFP-JXG in manchen Fällen später auf, und die spontane Abheilung kann länger dauern. Begleiterkrankungen und eine systemische Beteiligung wurden nicht festgestellt. Die histopathologische Untersuchung ist erforderlich, um diese Form des JXG von anderen Histiozytosen abzugrenzen. Unseres Wissens wurden bisher nur vier Fälle von SGFP-JXG in der Literatur beschrieben.


2021 ◽  
pp. 1-8
Author(s):  
Angelo Valerio Marzano ◽  
Nicoletta Cassano ◽  
Chiara Moltrasio ◽  
Lucio Verdoni ◽  
Giovanni Genovese ◽  
...  

<b>Hintergrund:</b> Die Symptome der Coronavirus-Infektion 2019 (COronaVIrus Disease 2019, COVID-19) fallen bei Kindern weniger schwer aus als bei Erwachsenen. Allerdings liegen zunehmend Berichte über schwere pädiatrische COVID-19-Fälle vor, darunter Patienten mit Kawasaki-Syndrom (KS) oder einem multisystemischen Entzündungssyndrom (multisystem inflammatory syndrome in children, MIS-C), das KS-ähnliche Merkmale aufweisen kann. <b>Zusammenfassung:</b> MIS-C ist ein neuartiges schweres pädiatrisches Syndrom, das mit COVID-19 in Zusammenhang steht, und bei dem Überschneidungen mit KS, KS-Schocksyndrom und toxischem Schocksyndrom bestehen können. Möglicherweise handelt es sich bei MIS-C um eine entzündliche Erkrankung, die sich vom KS unterscheidet und Folge einer überschießenden Immunreaktion ist. Neben einer gastrointestinalen und kardiovaskulären Beteiligung wurde bei MIS-C auch eine hohe Prävalenz von mukokutanen Manifestationen beobachtet. Die häufigsten mukokutanen Befunde waren Konjunktivitis und Exanthem, das oft als makulös und/oder papulös oder polymorph beschrieben wurde. In der vorliegenden Arbeit geben wir einen kurzen Überblick über MIS-C mit besonderem Augenmerk auf mukokutanen Befunden und dem Zusammenhang mit KS.


2021 ◽  
pp. 1-2
Author(s):  
Henning Hamm

Sleep problems are frequently reported in infants treated with propranolol for infantile hemangiomas, possibly serving as a marker for a negative impact on central nervous system function. In this cohort study, we objectively investigate the sleep behavior of infants with infantile hemangiomas on propranolol compared to a healthy, untreated control group. Sleep of propranolol-treated infants and controls was investigated using ankle actigraphy and a 24-h diary for 7–10 days at ages 3 and 6 months. The main outcome measures were the Number of Nighttime Awakenings and Sleep Efficiency. The main secondary outcome measures included 24-hour Total Sleep, daytime sleep behavior, and parent-rated infant sleep quality and behavioral development based on the Brief Infant Sleep Questionnaire (BISQ) and the age-appropriate Ages-and-Stages Questionnaire (ASQ), respectively. Fifty-four term-born infants were included in each cohort. No group difference in any investigated parameter was seen at age 3 months. At age 6 months, the propranolol group exhibited a decrease in Sleep Efficiency and a trend towards an increased Number of Nighttime Awakenings compared to the control group. Treated infants at 6 months also had shorter daytime waking periods. 24-hour Total Sleep was unaffected by propranolol. No negative impact of propranolol on subjective sleep quality and behavioral development was noted. <b>Conclusion:</b> Propranolol exerts a measurable yet mild impact on objectively assessed infants’ sleep measures. Behavioral developmental scores were unaffected. Our results support propranolol as first-line therapy for complicated infantile hemangiomas. <b>What is Known:</b> •Sleep disorders are frequently reported in infants with infantile hemangiomas treated with propranolol and often lead to treatment discontinuation. • Investigations of the sleep pattern in this patient group using objective measures are lacking. <b>What is New:</b> • The sleep pattern of propranolol-treated infants is assessed using actigraphy and a 24-h sleep diary and compared to healthy, untreated controls. • Propranolol leads to a decreased sleep efficiency at night and an increased demand of daytime sleep, yet effects are mild overall.


2021 ◽  
pp. 1-2
Author(s):  
Sandra Philipp

<b>Background:</b> Tralokinumab, a fully human monoclonal antibody, specifically neutralizes interleukin-13, a key cytokine driving peripheral inflammation in atopic dermatitis (AD). In phase II studies, tralokinumab combined with topical corticosteroids provided early and sustained improvements in AD signs and symptoms. <b>Objectives:</b> To evaluate the efficacy and safety of tralokinumab monotherapy in adults with moderate-to-severe AD who had an inadequate response to topical treatments. <b>Methods:</b> In two 52-week, randomized, double-blind, placebo-controlled, phase III trials, ECZTRA 1 and ECZTRA 2, adults with moderate-to-severe AD were randomized (3 : 1) to subcutaneous tralokinumab 300 mg every 2 weeks (Q2W) or placebo. Primary endpoints were Investigator’s Global Assessment (IGA) score of 0 or 1 at week 16 and ≥ 75% improvement in Eczema Area and Severity Index (EASI 75) at week 16. Patients achieving an IGA score of 0 or 1 and/or EASI 75 with tralokinumab at week 16 were rerandomized to tralokinumab Q2W or every 4 weeks or placebo, for 36 weeks. The trials were registered with ClinicalTrials.gov: NCT03131648 and NCT03160885. <b>Results:</b> At week 16, more patients who received tralokinumab vs. placebo achieved an IGA score of 0 or 1: 15·8% vs. 7·1% in ECZTRA 1 [difference 8·6%, 95% confidence interval (CI) 4·1–13·1; P = 0·002] and 22·2% vs. 10·9% in ECZTRA 2 (11·1%, 95% CI 5·8–16·4; P &#x3c; 0·001) and EASI 75: 25·0% vs. 12·7% (12·1%, 95% CI 6·5–17·7; P &#x3c; 0·001) and 33·2% vs. 11·4% (21·6%, 95% CI 15·8–27·3; P &#x3c; 0·001). Early improvements in pruritus, sleep interference, Dermatology Life Quality Index, SCORing Atopic Dermatitis and Patient-Oriented Eczema Measure were observed from the first postbaseline measurements. The majority of week 16 tralokinumab responders maintained response at week 52 with continued tralokinumab treatment without any rescue medication (including topical corticosteroids). Adverse events were reported in 76·4% and 61·5% of patients receiving tralokinumab in ECZTRA 1 and ECZTRA 2, respectively, and in 77·0% and 66·0% of patients receiving placebo in ECZTRA 1 and ECZTRA 2, respectively, in the 16-week initial period. <b>Conclusions:</b> Tralokinumab monotherapy was superior to placebo at 16 weeks of treatment and was well tolerated up to 52 weeks of treatment.


2021 ◽  
pp. 1-2
Author(s):  
Dominik Fugmann ◽  
André Karger

<b>Objective:</b> Our study analysed fear of cancer recurrence (FoR) in long-term cancer survivors in relation to medical variables, depression, anxiety, and quality of life. <b>Methods:</b> We present data obtained from 1,002 cancer survivors (53% male, mean age = 68 years, 26% prostate cancer, 22% breast cancer) across all cancer types 5 (N = 660) and 10 (N = 342) years after diagnosis, who were recruited via a large Clinical Cancer Registry in Germany in a cross-sectional study. FoR, depression, and anxiety were measured using validated self-report questionnaires (12-item short version of the Fear of Progression Questionnaire [FoP-Q-SF], Patient Health Questionnaire-9 [PHQ-9[, and General Anxiety Disorder-7 [GAD-7]). Hierarchical regression models were carried out with FoR as dependent variable and time since diagnosis as control variable. <b>Results:</b> We found high FoR-values in 17% of the cancer survivors (FoP-Q-SF total score&#x3e;33). FoR was higher in the 5-year cohort (P = .028, d = 0.153). Cancer survivors were most worried about the future of the family; they report being nervous prior to doctor’s appointment and being afraid of relying on strangers help. Higher FoR was related to female gender (Beta = .149, P&#x3c;.001), younger age (Beta = -.103, P&#x3c;.001), low social (Beta = -.129, P&#x3c;.001) and emotional functioning (Beta = -.269, P&#x3c;.001), received hormone therapy (P = .025, d = 0.056), and high anxiety levels (Beta = .227, P&#x3c;.001). <b>Conclusions:</b> Even though FoR declines slightly over time, it is still a common mental health problem for long-term survivors even 10 years after cancer diagnosis. Since FoR is associated with reduced emotional and social quality of life, patients who are at greater risk of experiencing FoR must be identified and supported. Particularly at risk are younger women who received hormone therapy.


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