Dupilumab treatment in paediatric atopic dermatitis (2 to 18 years): Spanish multicentre retrospective real-life study.

BACKGROUND: Moderate-to-severe atopic dermatitis (AD) can be difficult to manage in paediatric patients, with few licensed treatments in this age group. Dupilumab is approved for AD in children older than 6 months. OBJECTIVES: To assess the effectiveness and safety of dupilumab in a real-life cohort of paediatric AD patients in Spain. METHODS: A multicentre, retrospective real-life study on the effectiveness and safety of dupilumab in patients aged 2 to 18 years old with moderate-to-severe AD was conducted. Demographic and clinical characteristics were analysed, and effectiveness (EASI, IGA, DLQI, NRS itch), safety, and drug survival measures were assessed. A comparison of our results with other real-world outcomes and with clinical trials was made. RESULTS: Data from 243 patients from 19 centres was collected, with a mean follow-up of 85 weeks. Dupilumab exhibited significant effectiveness, with marked reductions in severity scores from week 4. By week 16, 79.4% of patients reached EASI75 and 40.5% reached EASI90. Mean percentage reduction in EASI was 79.7%. Increasing improvements were observed until week 52, with 85.8% and 49.6% achieving EASI75 and EASI90, respectively. Forty-three patients developed adverse events (AE) (43/243, 17.7%), being the most frequent ocular surface diseases (20/243, 8.2%), injection site reactions (8/243, 3.3%) and facial redness (7/243, 2.9%). Drug survival was high (96.9% and 93.1% after 1 and 2 years of follow-up, respectively), with only 19 (19/243, 7.8%) patients interrupting treatment: 7 (7/243, 2.9%) due to AE, 2 (2/243, 0.82%) due to secondary failure, 5 (5/243, 2.1%) were lost to follow-up and 5 (5/243, 2.1%) entered remission and stopped treatment. CONCLUSION: Real-life use of dupilumab in paediatric AD showcased sustained effectiveness, high drug survival, and acceptable safety profiles. Longer-term studies are crucial for AE surveillance and how to manage disease remission.

Real-world adalimumab survival and discontinuation factors in hidradenitis suppurativa.

BACKGROUND AND OBJECTIVES: Survival analyses can provide valuable insights into effectiveness and safety as perceived by prescribers. Here, we aimed to evaluate adalimumab (ADA) survival and the interruption risk factors in a multicentre cohort of patients with hidradenitis suppurativa (HS). Moreover, we performed a subanalysis considering the periods before and after the onset of the COVID-19 pandemic. METHODS: We conducted a retrospective study including 539 adult patients with HS who received ADA from 1 May 2015 to 31 December 2022. Overall drug survival was analysed using Kaplan-Meier survival curves and compared between the subgroups via stratified log-rank test. Possible predictors for overall drug survival and reasons for discontinuation were assessed using univariate and multivariate Cox regression. RESULTS: Overall, 50.1% were females with a mean age of 43.5 ± 1 years and a mean BMI of 29.5 ± 6.7. At the start of ADA, 95.29% were biologic-naïve and 24.63% had undergone surgical treatment. During follow-up, 9.46% of patients required dose escalation, while 39.92% interrupted ADA. Concomitant therapy was used in 64.89% of cases. A subanalyses comparing pre- and post-pandemic periods revealed a tendency to initiate ADA treatment at a younger age, among patient with higher BMI and at a lower HS stage after COVID-19 pandemic. Interestingly, ADA demonstrated extended survival compared to previous studies, with a median overall drug survival of 56.2 months (95% CI 51.2 to 80.3). The primary causes for discontinuation were inefficacy (51.69%), followed by adverse effects (21.35%). Female sex, longer delay in HS diagnosis, higher baseline IHS4 score and concomitant spondyloarthritis were associated with poorer ADA survival or increased risk of discontinuation. CONCLUSIONS: ADA demonstrated prolonged survival (median 56.2 months). While addition of antibiotics did not have a positive effect on survival rate, basal IHS4 proved useful in predicting ADA survival.

Use of high frequency ultrasonography in dermatology departments in Spain.

BACKGROUND: High Frequency Ultrasonography (HFUS) is a rapid, reliable, and effective diagnostic technique that has become progressively employed within the past years among dermatologists. OBJECTIVE: To describe the use of HFUS among dermatology departments in Spain. METHODS: An observational, cross-sectional descriptive study was conducted in 23 dermatology departments at Spanish public hospitals. Study data was collected during 10 working days. RESULTS: Fifty dermatologists from 11 Spanish regions participated in the study; 659 HFUS procedures were undertaken in 633 patients. HFUS indications were benign tumors (41%), malignant tumors (28%), inflammatory diseases (18%), skin appendage conditions (10%), and cosmetic conditions (1%). HFUS was performed for assessing the clinical differential diagnosis (58% of cases), surgical planning (17%), follow-up (17%), and other reasons (7%). Among the diagnostic group, the scan confirmed clinical diagnosis in 82% of cases and helped to change the clinical diagnosis in 17% cases (10% of total scans). HFUS procedure duration was less than 5 minutes (45%), between 5 and 10 minutes (32%), and more than 10 minutes (21%). Mean patient satisfaction score (range 0 to 5) was 4.8. CONCLUSIONS: The use of HFUS in usual practice is increasing, especially among younger dermatologists. This procedure is effective both for confirming and changing diagnosis, as well as for therapy decision making and follow-up. It is also worth remarking that a very high degree of patient satisfaction was recorded. The descriptive results reported in this study support the use of ultrasonography techniques and further reinforce their use in Dermatology.

Certolizumab pegol effectiveness, survival and safety in patients with psoriasis: A multicenter retrospective analysis in daily clinical practice by the Spanish Psoriasis Group.

BACKGROUND: Certolizumab is an Fc-free PEGylated tumor necrosis factor-alpha (TNFα) inhibitor recently approved for the treatment of moderate-to-severe plaque psoriasis, although there is limited real-world evidence on the effectiveness and safety in patients with plaque psoriasis treated with certolizumab. The objective of this article is to determine the effectiveness, drug survival, and safety, including pregnancy, childbirth, and lactation, of certolizumab in moderate-to-severe plaque psoriasis under real-world conditions. METHODS: This is a retrospective, multicenter, observational study performed in 15 hospitals in Spain. It evaluates the effectiveness and safety of certolizumab in plaque psoriasis in the clinical practice setting. RESULTS: A total of 67 patients (73% female) were evaluated with a mean baseline Psoriasis Area Severity Index (PASI) of 8.9. At Week 12, the mean PASI was 2.3 (n = 67), 1.3 (n = 57) at Week 24 and 1.3 at Week 52 (n = 34). Absolute PASI < 3 was achieved in 69, 86, and 92% of patients at Weeks 12, 24, and 52, respectively, as observed. For its part, using the under-response imputation analysis, PASI < 3 at Weeks 12, 24, and 52 were achieved by 69, 73, and 49% of the patients, respectively. A total of 35 patients (52%) had concomitant psoriatic arthritis, and, in 24 of them, Disease Activity in Psoriatic Arthritis Score (DAPSA) was recorded at baseline, with a mean value of 17.9 which decreased to 8.2 at Week 12 (n = 22) and to 3.6 at Week 24 (n = 18). Certolizumab treatment was discontinued in 14 out of 67 patients (21%), due to lack/loss of cutaneous or articular effectiveness (n = 11) or patient decision (n = 2) or adverse event in only one patient who developed active tuberculosis. A lower baseline PASI [hazard ratio (HR): 1.12 (1.02-1.23); P = 0.023] and a more significant reduction in PASI at Week 12 [HR: 1.16 (1.07-1.27); P < 0.001] and Week 52 [HR: 1.47 (1.11-1.96); P = 0.007] was shown to be significantly related with better survival for the entire follow-up period. Fourteen patients were treated during pregnancy and/or lactation without reporting adverse events in either the patient or the newborn. CONCLUSIONS: Certolizumab consistently showed high effectiveness and drug survival rates in this real-life cohort. The safety demonstrated in clinical trials during pregnancy and lactation seems to be confirmed in clinical practice.

Treatment of patients with plaque psoriasis with secukinumab in a real-life setting: a 52-week, multicenter, retrospective study in Spain.

Background: The efficacy and safety of secukinumab in patients with plaque psoriasis (PsO) have been demonstrated in randomized clinical trials (RCTs). However, data regarding its efficacy and safety in real-life settings are scarce. Objectives: To evaluate the efficacy and safety of secukinumab in clinical practice in patients with PsO attending 10 dermatology centers in Spain. Methods: Data from 136 patients consecutively treated with secukinumab for at least 52 weeks were collected in a retrospective observational study. Results: After 52 weeks of treatment, 69% and 46% of patients achieved a PASI-75, PASI-90, respectively. PASI-score ≤5 was achieved in 83% of patients, PASI-score ≤3 in 73% and PASI-score ≤1 in 47%. Response rates were found significantly lower in patients with obesity and non-naïve to biologics (p < .05). The most common adverse event (AE) was candidiasis (5/136). Thirty-six patients (26.5%) discontinued treatment by week 52 due to lack or loss of response (n = 29), AEs (n = 2) or other causes (n = 5). Conclusion: These findings complement the efficacy and safety profiles of secukinumab in PsO outlined in RCTs. The effectiveness in clinical practice may be lower in patients with a BMI ≥30 and those previously treated with other biologic agents.

Eruptive juvenile xanthogranuloma associated with relapsing acute lymphoblastic leukemia.

Juvenile xanthogranuloma is a benign, self-healing disorder with characteristic lesions mainly involving the skin. Although most patients with juvenile xanthogranuloma have only cutaneous symptoms, recent articles have documented extracutaneous manifestations: systemic involvement of many organs has been reported and there is a known association between juvenile xanthogranuloma and childhood leukemia, most commonly juvenile chronic myelogenous leukemia. This case provides further corroboration, that in rare instances, juvenile xanthogranuloma may be associated with hematologic malignancies.

Benign lymphangiomatous papules of the skin associated with ovarian fibroma.

Benign lymphangiomatous papules of the skin are considered reactive lymphatic proliferations either caused by disruption of the lymphatic flow or tissue damage produced by operation or radiation therapy. We report a 72-year-old woman with umbilical papules and vesicle-like lesions that led to the diagnosis of a large ovarian fibroma. Histologic study revealed dilated lymphatic spaces manifesting an anastomosing and branched pattern in the papillary and reticular dermis dissecting collagen bundles. The vessels were lined by plump endothelial cells with foci of intravascular papillary endothelial cell hyperplasia. After the ovarian fibroma was removed by laparotomy, umbilical lesions almost disappeared, leaving small flesh-colored papules. A periumbilical dermatosis may herald certain intra-abdominal diseases including those of neoplastic derivation. A heightened awareness of this association may lead to an early diagnosis with a potential for improved patient outcome. Benign lymphangiomatous papules have not been previously described in association with an untreated tumor, without previous operation or radiotherapy. This case advocates for disruption of the lymphatic drainage as the probable pathogenetic mechanism.

[Neonatal listeriosis]. / Listeriosis neonatal.

We present the case of a male with a neonatal Listeria monocytogenes infection. Its evolution was favorable with intravenous ampicillin and gentamicin. Listeriosis is an infrequent cause of neonatal pustulosis. The infection is acquired from the mother after bacteremia with few symptoms (early-onset forms) or while passing through an infected birth canal (late-onset forms). A cytological study and the quick stain technique make fast diagnosis of potentially serious neonatal pustuloses possible.

Aportación a las erupciones acneiformes por inhibidores del receptor del factor de crecimiento epidérmico / Contribution to acneiform eruptions by epidermal growth factor receptor inhibitors

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Listeriosis neonatal / Neonatal listeriosis

Presentamos el caso de un varón con infección neonatal por Listeria monocytogenes. La evolución fue favorable con ampicilina y gentamicina intravenosas. La listeriosis es una causa infrecuente de pustulosis neonatal. El contagio se produce desde la madre, tras una bacteriemia paucisintomática (formas tempranas) o durante el paso por el canal del parto contaminado (formas tardías). El estudio citológico y las tinciones urgentes permiten el diagnóstico rápido de pustulosis neonatales potencialmente graves

We present the case of a male with a neonatal Listeria monocytogenes infection. Its evolution was favorable with intravenous ampicillin and gentamicin. Listeriosis is an infrequent cause of neonatal pustulosis. The infection is acquired from the mother after bacteremia with few symptoms (early-onset forms) or while passing through an infected birth canal (late-onset forms). A cytological study and the quick stain technique make fast diagnosis of potentially serious neonatal pustuloses possible

Nena de 2 anys amb tumoració en extremitat superior detra i febre / Two year-old girl with a right upper extremity mass and febrile illness

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Nen de 5 anys amb lesions pustuloses en plecs i àrees periorificials d’aparició aguda / No disponible

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Síndrome esclerodermiforme por paclitaxel / Sclerodermiform syndrome from paclitaxel

Los síndromes esclerodermiformes se han relacionado con una larga lista de medicamentos, a la que se han añadido en los últimos años los taxanos. Se presenta el caso de una mujer con una neoplasia de mama que recibió tratamiento cíclico con paclitaxel. Tras 17 sesiones presentó edema y eritrodisestesia en ambos pies que, a pesar del tratamiento sintomático y de reducir la dosis de paclitaxel, progresaron hacia un endurecimiento cutáneo en estas localizaciones. Diez meses después de suspender el taxano las lesiones cutáneas se mantenían estables y no existían otras manifestaciones de esclerosis sistémica. Los taxanos pueden producir un síndrome esclerodermiforme que suele afectar a las extremidades inferiores y está precedido por edema y/o eritrodisestesia; no se acompaña de otras manifestaciones de esclerosis sistémica y tiende a estabilizarse o a involucionar al suspender el tratamiento. (AU)