Search

Resumen: Introducción: Existen diversas opciones terapéuticas para los hemangiomas infantiles (HI). El propranolol se utiliza con base en un ensayo pivotal. Nuestro objetivo fue describir las características del HI en la práctica clínica, incluyendo las terapias utilizadas, así como comparar las características de los pacientes tratados con propranolol y las de los pacientes del ensayo, para valorar su validez externa. Métodos: Se incluyó consecutivamente a los pacientes que acudieron a doce hospitales españoles desde junio de 2016 a octubre de 2019 (n = 601). Resultados: La edad media fue de 3,9 (DE:1,9) meses, con una ratio mujer-varón de 2:1. La mayoría de los HI fueron de tipo localizado (82%, 495), superficial (64%, 383) y ubicados en cara (25%, 157) y tronco (31%, 188). El tamaño mediano fue de 17 (RI: 10-30) x 12 (RI: 7-20) mm. Se encontraron complicaciones en 16 (3%) pacientes. Se inició tratamiento en el 52% (311) de los casos. La mayoría de los pacientes recibió timolol (76%, 237), reservándose propranolol para las complicaciones o los HI de alto riesgo. El compromiso estético fue el principal motivo de iniciar la terapia (64%, 199). Las diversas características de los pacientes y de los HI tratados con propranolol fueron similares a las del ensayo clínico pivotal, aunque 1/3 de los HI no alcanzó el diámetro mínimo para cumplir los criterios de inclusión, y no se comunicó información pronóstica importante. Conclusiones: Dado que muchos pacientes reciben tratamiento debido al compromiso estético, existe una necesidad de conocer mejor los resultados estéticos de las terapias e incrementar la evidencia sobre el uso de timolol, que actualmente es la terapia más común. El propranolol está siendo utilizado en una población generalmente similar a la del ensayo; sin embargo, esta afirmación no puede confirmarse de manera definitiva. Abstract: Background: There are several therapeutic options for infantile haemangiomas (IH). Propranolol is used according to a pivotal trial. We aimed to describe the characteristics of IH in clinical practice, including the therapies used, and to compare the characteristics of patients treated with propranolol with those of the trial to assess its external validity. Methods: Consecutive patients attending 12 Spanish hospitals from June 2016 to October 2019 were included (n = 601). Results: The mean age was 3.9 (SD:1.9) months, with a 2:1 female-to-male ratio. Most IHs were localized (82%, 495), superficial (64%, 383) and located in the face (25%, 157) and trunk (31%, 188). Median size was 17 (IR: 10-30) x 12 (IR: 7-20) mm. Complications were found in 16 (3%) patients. Treatment was initiated for 52% (311). Most patients received timolol (76%, 237); propranolol was reserved for complications or high-risk IHs. Aesthetic impairment was the main reason for starting therapy (64%, 199). Several characteristics of the patients and IHs treated with propranolol are similar to those of the pivotal clinical trial, but 1/3 of IHs did not reach the minimum diameter to meet the inclusion criteria, and important prognostic information was not reported. Conclusions: As most patients receive treatment for aesthetic impairment, there is a need to better understand the aesthetic results of therapies and to increase evidence on the use of timolol, which is currently the most common therapy. Propranolol is being used in a population generally similar to that of the trial; however, this statement cannot be definitely confirmed.

Importance: Although clinical practice guidelines exist for the treatment of infantile hemangiomas (IHs), recommendations are heterogeneous, and wide practice variations in IH management have been reported., Objective: To analyze the degree of agreement in treatment choices for IH among pediatric dermatologists in North America and Europe and assess whether there are differences across IH risk categories., Design, Setting, and Participants: This cross-sectional interrater and intrarater agreement study was conducted through a survey based on the Spanish Academy of Dermatology and Venereology IH prospective cohort. The survey used 50 vignettes of IH cases that were randomly selected from the cohort. It was administered twice in 2023, 1 month apart, to allow for interrater and intrarater agreement assessments. Data were analyzed in January 2024. The study involved pediatric dermatologists from North America (via the Pediatric Dermatology Research Alliance) and Europe (via the European Society of Pediatric Dermatologists)., Exposures: Participants were asked to choose 1 of 3 treatment options (propranolol, topical timolol, or observation) for each vignette., Main Outcome and Measure: The primary outcome was the interrater agreement in treatment choices for IH cases, measured using κ statistics (Gwet AC1 coefficient)., Results: The global interobserver agreement among 90 pediatric dermatologists was fair (AC1, 0.38; 95% CI, 0.29-0.46). In North America (45 pediatricians), agreement was moderate (AC1, 0.41; 95% CI, 0.33-0.49), while in Europe (45 pediatricians) it was fair (AC1, 0.37; 95% CI, 0.28-0.46). The degree of agreement varied depending on the risk category of IH, with excellent agreement in high-risk IH and only moderate agreement in intermediate-risk and low-risk IHs. Propranolol was predominantly chosen for high-risk IH, while observation was most frequent for low-risk IH (55.9%). The second survey had 61 respondents, with no significant intrarater differences., Conclusions and Relevance: The results of this survey study suggest that there is an important variability in the treatment of intermediate-risk and low-risk IH. The study findings support the need for more evidence regarding the role of topical timolol in IH treatment, which may help harmonize treatment approaches and improve consistency in IH management globally.

Background: Functional impairment is the main consideration when it comes to choosing therapy for infantile hemangiomas (IH). However, since most hemangiomas are treated for cosmetic reasons, it is important to know the cosmetic outcome assessed by the parents., Objective: To evaluate the aesthetic outcomes of IH, considering the characteristics of the lesions and the treatments used., Patients and Methods: The Spanish Infantile Hemangioma Nationwide Prospective Cohort (2016-2022) recruited all consecutive patients diagnosed with IH in 12 Spanish hospitals. The children included had 2 photos of the IH lesion (at both baseline and at the end of the study). A panel of parents blindly assessed all available photos using a scale from 0 (worst cosmetic outcomes) to 10 (best cosmetic outcomes). The different scores -both before and after treatment-as well as the outcomes percent considered excellent (> 9) were described and compared. We analyzed the effect of receiving different therapies and performed causal model analyses estimating the mean treatment effect of parents' assessments., Results: The median follow-up was 3.1 years. A total of 824 photos were evaluated. Baseline aesthetic impact was higher in the propranolol group vs the topical timolol and observation treatment groups (1.85 vs 3.14 vs 3.66 respectively; p < 0.001). After treatment, the aesthetic impact was similar between both treatment groups (7.59 vs 7.93 vs 7.90; p > 0.2). The causal model could only be applied to the comparison between topical timolol and observation, revealing no differences whatsoever., Conclusion: This is the first prospective cohort to analyze the aesthetic outcome of IH. The final aesthetic results of the 3 therapies were similar, with nearly 40% of patients achieving excellent aesthetic outcomes., (Copyright © 2024 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.)

The use of postoperative radiotherapy (PORT) for the treatment of keloids in the pediatric population is rare, despite being a common pathology at this age. Recurrences after surgery are very common. The absence of a standardized protocol for the management of recurrent keloids further complicates therapeutic decision-making. Here, we present a clinical case involving a recurrent giant retroauricular keloid in a pediatric patient, demonstrating a satisfactory response to a comprehensive approach involving surgery, PORT, and periodic injections of 5-fluorouracil with corticosteroid. We believe that reporting this case adds value as a potential therapeutic option in the absence of established protocols for recurrent keloids in pediatric patients., (© 2024 Wiley Periodicals LLC.)

Aim: To investigate the efficacy and tolerability of a cream (Rilastil Xerolact PB) containing a mixture of prebiotics and postbiotics, and to validate the PRURISCORE itch scale in the management of atopic dermatitis. Methods: The study is based on 396 subjects of both sexes in three age groups (i.e., infants, children, adults) suffering from mild/moderate Atopic Dermatitis, recruited from 8 European countries and followed for 3 months. Results: The product demonstrated good efficacy combined with good/very good tolerability in all age groups. In particular, SCORAD, PRURISCORE and IGA scores decreased significantly over the course of the study. The PRURISCORE was preferred to VAS by the vast majority of patients. Conclusion: Even though the role of prebiotics and postbiotics was not formally demonstrated since these substances were part of a complex formulation, it can be reasonably stated that prebiotics and postbiotics have safety and standardization features that probiotics do not have. In addition they are authorized by regulatory authorities, whereas topical probiotics are not.

Introduction: As research continues, new drugs will no doubt be added to the current pool of treatments for moderate-to-severe atopic dermatitis (AD). This raises the need for studies to determine prescriber preferences for different pharmacological options and the factors that influence their choice of treatment. Here we aim to explore physician preferences in the systemic treatment of moderate-to-severe AD, identify the sociodemographic characteristics that can influence physician preferences, and evaluate their satisfaction with current AD therapies., Methods: A discrete-choice experiment (DCE) survey was administered to physicians treating patients with AD in Spain. Results were analyzed using a conditional logit model to estimate the relative importance of each attribute and the maximum risk accepted to achieve therapeutic benefit., Results: A total of 28 respondents completed the DCE survey (67.9% female, mean age 45.9 years). Participants identified objective clinical efficacy and risk of severe adverse events (AEs) as the most important attributes, followed by improvement in sleep and pruritus and faster onset of action from the start of the treatment. Respondents gave less importance to mode of administration and therapeutic benefit in other atopic conditions. Respondents were willing to accept an increased risk of severe AEs and mild-to-moderate AEs leading to treatment discontinuation due to intolerance in order to obtain improvements in efficacy, sleep, and pruritus, and long-term clinical benefit., Conclusion: Our findings can help prescribers choose the most appropriate systemic AD therapy., (© 2022. The Author(s).)

Generalized pustular psoriasis (GPP) represents the rarest form of psoriasis, which may be potentially fatal. In the last decade, (likely) pathogenic variants in the IL36RN, CARD14 and AP1S3 genes have been associated with monogenic GPP forms. Despite these advances, the genetic basis of most patients with GPP remains unidentified. Treatment of GPP patients is often difficult, with no consensus about the best available options to date. We report herein an infant with severe GPP in whom the disease started at the age of 2 months. Genetic investigations identified a heterozygous pathogenic variant in the IL36RN gene associated with a heterozygous variant of uncertain significance in the CARD14 gene. After previous treatment failures with acitretin, cyclosporin and anakinra, treatment with the interleukin-17 antagonist secukinumab resulted in a dramatic and prompt positive response that persisted at 12-month follow up. According to our experience, we believe secukinumab can be an effective and safe treatment for pediatric patients with GPP even before 1 year of age., (© 2021 Japanese Dermatological Association.)

Objectives: Infantile hemangiomas (IHs) are common; some cases require timely referral and treatment to prevent complications. We developed and validated a reliable instrument for timely and adequate referral of patients with IH to experts by nonexpert primary physicians., Methods: In this multicenter, cross-sectional, observational study, we used a 3-stage process: (1) development of the Infantile Hemangioma Referral Score (IHReS) tool by IH experts who selected a representative set of 42 IH cases comprising images and a short clinical history, (2) definition of the gold standard for the 42 cases by a second independent committee of IH experts, and (3) IHReS validation by nonexpert primary physicians using the 42 gold standard cases., Results: A total of 60 primary physicians from 7 different countries evaluated the 42 gold standard cases (without reference to the IHReS tool); 45 primary physicians evaluated these cases using the IHReS questionnaire, and 44 completed retesting using the instrument. IHReS had a sensitivity of 96.9% (95% confidence interval 96.1%-97.8%) and a specificity of 55.0% (95% confidence interval 51.0%-59.0%). The positive predictive value and negative predictive value were 40.5% and 98.3%, respectively. Validation by experts and primary physicians revealed substantial agreement for interrater reliability and intrarater repeatability., Conclusions: IHReS, a 2-part algorithm with a total of 12 questions, is an easy-to-use tool for primary physicians for the purpose of facilitating correct and timely referral of patients with IH. IHReS may help practitioners in their decision to refer patients to expert centers., Competing Interests: POTENTIAL CONFLICT OF INTEREST: Dr Léauté-Labrèze is a consultant and clinical trial investigator for Pierre Fabre and is a member of the Infantile Hemangioma European Task Force (IHETF) and received consultant fees. Dr Baselga Torres is a consultant and speaker for Pierre Fabre, is a member of the IHETF and received consultant fees, is a consultant for Ferrer and Sanofi, is the founder and consultant for Vanthera, and is a speaker for Almirall. Dr Weibel is member of the IHETF and received consultant fees; she is a consultant and speaker for Sanofi, MEDA, Merz, Permamed, and La Roche Posay (L’Oréal) and the principal investigator in clinical trials with AbbVie, Novartis, Eli Lilly, and Pfizer. Dr Boon is member of the IHETF and received consultant fees and has relationships with the following companies: Deciphera Pharmaceuticals, Inc (Waltham, MA), Venthera, Inc (Palo Alto, CA), and Pfizer, Inc (Ixelles, Belgium) (sirolimus or VASE study). Dr El Hachem is member of the IHETF and received consultant fees. Dr van der Vleuten is a member of the IHETF and received consultant fees. Dr Roessler is a consultant for Pierre Fabre, is a member of the IHETF and received consultant fees, is a consultant for SOBI, and has received research grants from Pfizer and travel grants from Roche. Dr Troilius Rubin is a member of the IHETF and received consultant fees., (Copyright © 2020 by the American Academy of Pediatrics.)

Background: The superficial lymphatic component of vascular malformations poses a significant treatment challenge. It is responsible for the majority of symptoms presented, and to date, there is no consensus regarding treatment., Objective: To evaluate the effectiveness of topical rapamycin in treating superficial lymphatic malformations (LM)., Methods: A case series study was performed of patients with superficial LM, treated with topical rapamycin. The clinical characteristics of patients and the concentration and application mode of the drug were recorded. The changes in the signs and symptoms observed and associated adverse effects were noted and analyzed., Results: The study population consisted of 11 patients of an average age of 10.5 years. All were treated with topical rapamycin: 6 patients with a 1% concentration, 1 with a 0.8% concentration, and 4 with a 0.4% concentration. Changes in the clinical appearance of the lesions were observed in all patients. The associated symptoms, present in 9 of 11 patients, improved in every case. The mean follow-up time was 16.1 months., Limitations: This study is retrospective, with a small sample size and considerable heterogeneity of lesions and treatment approaches., Conclusion: Treatment with topical rapamycin modifies the clinical appearance and alleviates symptoms of superficial LM., (Copyright © 2018 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Introduction: Infantile haemangiomas are benign tumours produced by the proliferation of endothelial cells of blood vessels, with a high incidence in children under the age of one year (4-10%). It is estimated that 12% of them require treatment. This treatment must be administered according to clinical practice guidelines, expert experience, patient characteristics and parent preferences., Methods: The consensus process was performed by using scientific evidence on the diagnosis and treatment of infantile haemangiomas, culled from a systematic review of the literature, together with specialist expert opinions. The recommendations issued were validated by the specialists, who also provided their level of agreement., Results: This document contains recommendations on the classification, associations, complications, diagnosis, treatment, and follow-up of patients with infantile haemangioma. It also includes action algorithms, and addresses multidisciplinary management and referral criteria between the different specialities involved in the clinical management of this type of patient., Conclusions: The recommendations and the diagnostic and therapeutic algorithms of infantile haemangiomas contained in this document are a useful tool for the proper management of these patients., (Copyright © 2015 Asociación Española de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.)

Linear morphea and lichen striatus are distinct conditions that have been linked in only one previous case report. We describe two patients with facial lichen striatus preceding linear morphea at the same site. A possible pathogenic relationship is discussed., (© 2015 Wiley Periodicals, Inc.)

Background: Intralesional injection of Candida antigen appears to be an effective alternative for the treatment of warts., Aim: To determine the efficacy and safety of this treatment., Methods: We retrospectively reviewed records of all children who received intralesional injection of Candida antigen at our center from January 2008 to July 2013., Results: From a total of 220 patients, 156 (70.9%) had a complete response, 37 (16.8%) had a partial response, and 27 (12.2%) had no improvement. An average of 2.73 treatments was needed. Forty-seven of the patients with more than one wart (21.3%) also noted at least partial resolution of untreated warts at distant sites. Twenty-seven of the 47 patients (57.4%) had complete resolution. All treated patients experienced some discomfort at the time of the injection, but no serious side effects were reported., Discussion: We report our results using this approach in a large group of children., Conclusion: Intralesional injection of Candida antigen is an effective and safe therapy for children with multiple and recalcitrant cutaneous warts., (© 2015 Wiley Periodicals, Inc.)

Background: There is a paucity of evidence for the use of systemic agents in children with atopic eczema refractory to conventional therapy, resulting in considerable variation in patient management., Objectives: The European TREatment of severe Atopic eczema in children Taskforce (TREAT) survey was established to collect data on current prescribing practice, to identify factors influencing the use of specific systemic agents, and to inform the design of a clinically relevant intervention study., Methods: Consultant physician members of the paediatric dermatology societies and interest groups of eight European countries were invited to participate in a web-based survey. The multiple-response format questionnaire collated data on clinical practice in general, as well as detailed information on the use of systemic agents in refractory paediatric atopic eczema., Results: In total, 343/765 members (44·8%) responded to the invitational emails; 89·2% were dermatologists and 71% initiate systemic immunosuppression for children with severe atopic eczema. The first-line drugs of choice were ciclosporin (43·0%), oral corticosteroids (30·7%) and azathioprine (21·7%). Ciclosporin was also the most commonly used second-line medication (33·6%), with methotrexate ranked as most popular third choice (26·2%). Around half of the respondents (53·7%) replied that they routinely test and treat reservoirs of cutaneous infection prior to starting systemic treatment. Across the eight countries, penicillins were the first-line antibiotic of choice (78·3%)., Conclusions: In the absence of a clear evidence base, the European TREAT survey confirms the wide variation in prescribing practice of systemic immunosuppression in refractory paediatric atopic eczema. The results will be used to inform the design of a randomized controlled trial relevant to patient management across Europe., (© 2013 British Association of Dermatologists.)