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Background: Phototherapy is used to treat atopic dermatitis (AD). Evidence for its efficacy, impact on quality of life, cost‐effectiveness and short‐ and long‐term safety with real‐life usage is weak. Objectives: We established a taskforce to examine how phototherapy is currently being used as a treatment for AD across the United Kingdom and Europe to inform our understanding and guide future research into management of patients with AD using UV‐based phototherapies. Methods: An anonymous electronic multiple‐response survey exploring phototherapy prescribing practices and experience of phototherapy modalities was developed by the study authors and sent to members of phototherapy networks from the United Kingdom and Europe. Responses were received between February and July 2021. Results: About 144 respondents from 27 European countries completed the survey. NBUVB was the most widely used [n = 138 (96%)]. Home‐based NBUVB was available in 8/27 countries (25/144 respondents, 17%). Oral psoralen‐UVA (PUVA) was more widely available than bath PUVA (n = 106, 74% vs. n = 60, 42%) and used mainly in adult patients. 49/144 (34%) of respondents had access to UVA1. Phototherapy would be considered instead of systemic treatment in 96% of adults and 82% of children for NBUVB, versus 40% of adults and 3% of children for PUVA. Starting doses, standard dosing increments, length of treatment courses, lifetime limits for treatments and thresholds for performing annual skin assessments varied between responders. Conclusions: NBUVB was the most widely used phototherapy for AD in adult and paediatric patients, while PUVA and UVA1 were less used. Prescribing practices varied considerably, highlighting the lack of consensus practice in many different aspects of phototherapy for the treatment of AD in children and adults. This indicates that further studies are required to determine optimal phototherapeutic regimens for AD and informs our understanding of parameters that should be included in future high‐quality randomized controlled trials (RCT) of phototherapy. [ABSTRACT FROM AUTHOR]

Background: There is limited data about the clinical meaningfulness of the Scoring of Atopic Dermatitis (SCORAD) and Patient‐Oriented SCORAD (PO‐SCORAD), particularly in children with mild‐to‐moderate AD. Regular use of patient‐reported outcomes, may deliver more accurate information about the overall health status of AD patients than routine but sparse physician assessments. Objective: To confirm the correlation between SCORAD, PO‐SCORAD, Patient‐Oriented Eczema Measure (POEM) and Investigator's Global Assessment (IGA). To evaluate the interpretability and clinical usefulness of the SCORAD and PO‐SCORAD scores in children. Methods: Data were drawn from a 12‐week randomized controlled trial in 335 children, aged 2–6 years, with mainly mild‐to‐moderate AD. Investigators captured SCORAD and IGA at each study visit. Parents used PO‐SCORAD twice‐weekly, and POEM once‐weekly. Results: There were strong correlations between PO‐SCORAD and SCORAD (r = 0.874), PO‐SCORAD and POEM (0.734) and PO‐SCORAD and IGA (0.613). The best fit ('k' statistic: 0.68) between SCORAD and IGA classes was noted for the following SCORAD categories: <12 (clear/almost clear); 12–25 (mild); and ≥25 (moderate/severe). PO‐SCORAD area under the curve over 8 weeks was significantly greater than that of SCORAD (p = 0.0002), giving a better estimate of disease severity between visits. Patients with a flare within the next 7 days had significantly higher PO‐SCORAD scores 7 days before the flare (p < 0.0001). Moderate erythema was the most significant flare predictor (p < 0.0001). Conclusion: PO‐SCORAD is robust and reliable and appears to warrant far greater utility in routine clinical practice than other scores. PO‐SCORAD, used twice‐weekly, may improve the management of patients with AD. [ABSTRACT FROM AUTHOR]

Humans with monogenic inborn errors responsible for extreme disease phenotypes can reveal essential physiological pathways. We investigated germline mutations in GNAI2 , which encodes G αi2 , a key component in heterotrimeric G protein signal transduction usually thought to regulate adenylyl cyclase-mediated cyclic adenosine monophosphate (cAMP) production. Patients with activating G αi2 mutations had clinical presentations that included impaired immunity. Mutant G αi2 impaired cell migration and augmented responses to T cell receptor (TCR) stimulation. We found that mutant G αi2 influenced TCR signaling by sequestering the guanosine triphosphatase (GTPase)-activating protein RASA2, thereby promoting RAS activation and increasing downstream extracellular signal-regulated kinase (ERK)/mitogen-activated protein kinase (MAPK) and phosphatidylinositol 3-kinase (PI3K)-AKT S6 signaling to drive cellular growth and proliferation.

Background: Data on dermatological manifestations of Costello syndrome (CS) remain heterogeneous and lack in validated description., Objectives: To describe the dermatological manifestations of CS; compare them with the literature findings; assess those discriminating CS from other RASopathies, including cardiofaciocutaneous syndrome (CFCS) and the main types of Noonan syndrome (NS); and test for dermatological phenotype-genotype correlations., Methods: We performed a 10-year, large, prospective, multicentric, collaborative dermatological and genetic study., Results: Thirty-one patients were enrolled. Hair abnormalities were ubiquitous, including wavy or curly hair and excessive eyebrows, respectively in 68% and 56%. Acral excessive skin (AES), papillomas and keratotic papules (PKP), acanthosis nigricans (AN), palmoplantar hyperkeratosis (PPHK) and 'cobblestone' papillomatous papules of the upper lip (CPPUL), were noted respectively in 84%, 61%, 65%, 55% and 32%. Excessive eyebrows, PKP, AN, CCPUL and AES best differentiated CS from CFCS and NS. Multiple melanocytic naevi (>50) may constitute a new marker of attenuated CS associated with intragenic duplication in HRAS. Oral acitretin may be highly beneficial for therapeutic management of PPHK. No significant dermatological phenotype-genotype correlation was determined between patients with and without HRAS c.34G>A (p.G12S)., Conclusions and Relevance: This validated phenotypic characterization of a large number of patients with CS will allow future researchers to make a positive diagnosis, and to differentiate CS from CFCS and NS., (© 2024 The Authors. Journal of the European Academy of Dermatology and Venereology published by John Wiley & Sons Ltd on behalf of European Academy of Dermatology and Venereology.)

Background: Therapeutic patient education (TPE) is recommended for children with atopic dermatitis (AD), but no consensus has been reached on the optimal tailoring of delivery. While repeated multidisciplinary group education sessions have shown effectiveness, the benefits of one-on-one educational interventions led by nurses for children with AD have not yet been assessed., Objectives: To assess the benefits of additional, well-structured, 1-h nurse-led individual TPE interventions in children with AD and their families compared with standard care alone., Methods: Children with moderate-to-severe AD and their parents were randomized to receive a 1-h nurse-led education session in addition to standard care vs. standard care alone. The primary outcome was the area under the curve (AUC) of the SCORing of Atopic Dermatitis index (SCORAD) from baseline to week 24 (lower AUC values represent better long-term control of the disease)., Results: In our study, 176 patients were randomized across 11 centres, and 153 were included in the full analysis set. The mean (SD) age was 4.47 (4.57) years. By week 24, there were no significant differences in the AUCs of the SCORAD between the two groups (P = 0.3). Secondary outcomes including patient-reported severity and quality of life [AUCs of the patient-oriented SCORAD (PO-SCORAD) and Infants' Dermatitis Quality of Life Index (IDLQI), Children's Dermatitis Quality of Life Index (CDLQI) and Family Dermatitis Quality of Life Index (FDLQI)] were not significantly different between the two groups. The only significant change observed in the intervention group, when compared with the one receiving standard care, was a decrease in topical steroid phobia, as assessed by the topical corticosteroid phobia (TOPICOP) score. Prespecified subgroup analyses showed that disease severity in the intervention group was significantly lower throughout the study, compared with the standard-care group when participants had moderate AD at baseline (n = 47); while participants with severe AD at baseline (n = 106) did not show benefit from the intervention. Participants showed no additional benefit from the intervention regardless of age group., Conclusions: This study did not show any additional effectiveness, in long-term severity control, of a 1-h nurse-led TPE intervention in children with AD treated with standard care, compared with those treated with standard care alone. However, it should be noted that the intervention reduced the fear of using topical steroids and may be beneficial for patients in the subgroup with moderate AD., Competing Interests: Conflicts of interest S.B. declares payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events and support for attending meetings and/or travel from AbbVie, Alexion, Almirall, AstraZeneca, Chiesi, Eli Lilly, Galderma, Janssen, LEO Pharma, Novartis, Pfizer, Sanofi-Genzyme and UCB Pharma. H.A. declares consulting fees from Abbvie, BMS, Lilly, Novartis and Sanofi; payment of honoraria from AbbVie, Lilly, Novartis, Pierre Fabre, Sanofi and UCB; and support for attending meetings from AbbVie and Almirall. N.R.-P. declares support for attending meetings and/or travel from Sanofi-Genzyme., (© The Author(s) 2024. Published by Oxford University Press on behalf of British Association of Dermatologists. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

In patients with moderate to severe atopic dermatitis (AD) showing an inadequate response to dupilumab 300mg/2weeks, few real-life studies reported the response to alternative regimen maintaining dupilumab. To assess and analyze the response to an increased dose of dupilumab or its combination with cyclosporin A (CsA), methotrexate (MTX), or itraconazole (ITRA), all adult AD patients from 7 French University Hospitals were retrospectively included if they achieved an inadequate response to dupilumab 300mg/2weeks and were subsequently treated with an increased dose of dupilumab (300mg every 7 or 10 days), or a combination of dupilumab 300mg/2weeks with CsA, MTX or ITRA. The response after 3 months, along with epidemiological, clinical, and therapeutic baseline characteristics, were collected. Overall, 68.75% of the 48 included patients achieved an improved response, including 45.8% of complete response (CR). No strategy proved significantly better. Patients showing an initial no response never achieved a further CR versus 52.4% of patients with an initial partial response (p = 0.025). Digestive intolerance and tachycardia led to MTX and ITRA discontinuation in 3 patients. Increasing the dose of dupilumab or combining it with CsA, MTX, or ITRA could be alternative and safe options, to be evaluated in further medico-economic studies., Competing Interests: R Strizzolo reports no competing interest. J Seneschal reports the following competing interests: Principal investigator or consultant or speaker for AbbVie, Almirall, Lilly, Sanofi, LeoPharma, Pierre Fabre, Pfizer; support for attending meetings from AbbVie. A Soria reports the following competing interests: Consultant or speaker for Novartis, Sanofi, LEO Pharma, Abbvie, Lilly, Pfizer and Bioprojet D Staumont-Sallé reports the following competing interests: Investigator, consultant and/or speaker for AbbVie, Almirall, Amgen, Astra-Zeneca, Eli Lilly, Galderma, Leo Pharma, Novartis, Pfizer, Sanofi-Regeneron, UCB. S Barbarot reports the following competing interests: principal investigator, speaker and consultant: Astrazeneca, Almirall, Sanofi-Genzyme, Abbvie, Galderma, Alexion, Novartis, Janssen, Leo-Pharma, Pfizer, Eli Lilly, UCB Pharma, Chiesi. M Viguier reports the following competing interests: travel fees, speaker, consultant and/or board member for Lilly, Abbvie, Janssen-Cilag, Galderma, Boehringer Ingelheim, Novartis, UCB, Nordic, Medac, Bristol Myers Squibb. M Jachiet reports the following competing interests: investigator, consultant and/or speaker for AbbVie, Eli Lilly, Galderma, Leo Pharma, Pfizer, Sanofi-Regeneron. A Nosbaum reports the following competing interests: investigator, consultant and/or speaker for AbbVie, Leo Pharma, Pfizer, Sanofi, Lilly, Medac. A Clément reports the following competing interests: principal investigator, speaker and/or consultant for LeoPharma, Sanofi-Regeneron, Abbvie, Lilly, Janssen. M Tauber reports the following competing interests: principal investigator, speaker and/or consultant for LeoPharma, Sanofi-Regeneron, Abbvie, Lilly, Medac, Almirall, Jenssen, Pfizer. S Mallet reports the following competing interests: principal investigator or sub investigator for Lilly, Sanofi, LeoPharma, consulting fees from Lilly, Sanofi, LeoPharma, Pfizer, speaker for Lilly, Sanofi, LeoPharma, Pfizer, support for attending meetings Lilly, Sanofi, LeoPharma, participation on advisory board: Sanofi, LeoPharma, Pfizer. A Du-Thanh reports the following competing interests: principal investigator, speaker and consultant for LeoPharma, Sanofi, Pfizer, Abbvie, Lilly., (© 2024 The Authors.)

SIDeMaST (Società Italiana di Dermatologia Medica, Chirurgica, Estetica e delle Malattie Sessualmente Trasmesse) contributed to the development of the present guideline on the systemic treatment of chronic plaque psoriasis. With the permission of EuroGuiDerm, SIDeMaST adapted the guideline to the Italian healthcare context to supply a reliable and affordable tool to Italian physicians who take care of patients affected by atopic dermatitis. The evidence- and consensus-based guideline on atopic eczema was developed in accordance with the EuroGuiDerm Guideline and Consensus Statement Development Manual. Four consensus conferences were held between December 2020 and July 2021. Twenty-nine experts (including clinicians and patient representatives) from 12 European countries participated. This first part of the guideline includes general information on its scope and purpose, the health questions covered, target users and a methods section. It also provides guidance on which patients should be treated with systemic therapies, as well as recommendations and detailed information on each systemic drug. The systemic treatment options discussed in the guideline comprise conventional immunosuppressive drugs (azathioprine, ciclosporin, glucocorticosteroids, methotrexate and mycophenolate mofetil), biologics (dupilumab, lebrikizumab, nemolizumab, omalizumab and tralokinumab) and janus kinase inhibitors (abrocitinib, baricitinib and upadacitinib). Part two of the guideline will address avoidance of provocation factors, dietary interventions, immunotherapy, complementary medicine, educational interventions, occupational and psychodermatological aspects, patient perspective and considerations for pediatric, adolescent, pregnant and breastfeeding patients.

SIDeMaST (Società Italiana di Dermatologia Medica, Chirurgica, Estetica e delle Malattie Sessualmente Trasmesse) contributed to the development of the present guideline on the systemic treatment of chronic plaque psoriasis. With the permission of EuroGuiDerm, SIDeMaST adapted the guideline to the Italian healthcare context to supply a reliable and affordable tool to Italian physicians who take care of patients affected by atopic dermatitis. The evidence- and consensus-based guideline on atopic eczema was developed in accordance with the EuroGuiDerm Guideline and Consensus Statement Development Manual. Four consensus conferences were held between December 2020 and July 2021. Twenty-nine experts (including clinicians and patient representatives) from 12 European countries participated. This second part of the guideline includes recommendations and detailed information on basic therapy with emollients and moisturizers, topical anti-inflammatory treatment, antimicrobial and antipruritic treatment and UV phototherapy. Furthermore, this part of the guideline covers techniques for avoiding provocation factors, as well as dietary interventions, immunotherapy, complementary medicine and educational interventions for patients with atopic eczema and deals with occupational and psychodermatological aspects of the disease. It also contains guidance on treatment for pediatric and adolescent patients and pregnant or breastfeeding women, as well as considerations for patients who want to have a child. A chapter on the patient perspective is also provided. The first part of the guideline, published separately, contains recommendations and guidance on systemic treatment with conventional immunosuppressive drugs, biologics and janus kinase (JAK) inhibitors, as well as information on the scope and purpose of the guideline, and a section on guideline methodology.

The evidence- and consensus-based guideline on atopic eczema, published in JEADV on 18 August 2022 (part 1) and 3 September 2022 (part 2) was developed in accordance with the EuroGuiDerm Guideline and Consensus Statement Development Manual. Four consensus conferences were held between December 2020 and July 2021. Twenty-nine experts (including clinicians and patient representatives) from 12 European countries participated. To reflect the most recent evidence on novel systemic medications, an update was published in October 2022. According to the purpose of the Italian Society of Dermatology and STD (SIDEMAST), the Italian Association of Hospital Dermatologists (ADOI) and the Italian Society of Allergological and Environmental Dermatology (SIDAPA) to adapt the EuroGuiDerm guideline on the treatment of atopic eczema into the Italian Healthcare setting, the original update has been supplemented by inserting notes, well highlighted by the original text, to emphasize the laws, rules, procedures and suggestions of the Italian Ministry of Health and regional Health authorities.

Background: Few large-scale international studies have broadly characterized the burden of atopic dermatitis (AD) across age groups among children and adolescents., Objectives: To better characterize the AD burden in paediatric patients by disease severity., Methods: This cross-sectional, web-based survey of paediatric patients (6 months to < 18 years old) was conducted in 18 countries representing North America, Latin America, Europe, Middle East/Eurasia and East Asia. Patients with diagnosed AD were identified based on the International Study of Asthma and Allergies in Childhood criteria and self-/parent-report of ever being told by a physician that they or their child had eczema. AD severity was assessed using the Patient-Oriented Eczema Measure and Patient Global Assessment. Outcomes included measures of itch, skin pain, sleep, health-related quality of life (HRQoL), missed school days and atopic comorbidities., Results: The survey included 1489 children aged 6 months to < 6 years; 2898 children aged 6 to < 12 years; and 3078 adolescents aged 12 to < 18 years diagnosed with AD. Although the burden of mild AD was substantial, paediatric patients with moderate or severe AD had more itch, skin pain, sleep problems and impaired HRQoL, and missed more school days relative to those with mild AD; greater burden was observed among those with severe relative to moderate AD. At least one atopic comorbidity was present in 92.5% of all respondents., Conclusions: These results highlight the burden of AD in paediatric patients, especially those with moderate-to-severe disease, and suggest the need for assessments that include the impact of AD on function and daily life., Competing Interests: Conflicts of interest A full list of the authors’ conflicts of interest is available in Appendix S1 (see Supporting Information)., (© The Author(s) 2023. Published by Oxford University Press on behalf of British Association of Dermatologists.)

Introduction: Neurofibromatosis type 1 (NF1) is a genetic pathology that can lead to impaired social functioning that has a negative impact on patients' quality of life. To date, although the hypothesis of impaired social cognition has been proposed as a potential explanation for these difficulties, very few studies have focused on theory of mind in children with NF1. Furthermore, other complex sociocognitive abilities have never been investigated. The aim of the present study was to assess theory of mind, moral reasoning, and social information processing in children with NF1 compared with a control group., Method: We administered the Paediatric Evaluation of Emotions, Relationships and Socialization® to 38 children with NF1 aged between 8 years and 16 years 11 months (mean = 11.4, SD  = 2.3) and 43 control children with comparable sociodemographic characteristics., Results: Patients performed significantly worse than controls on moral reasoning and social information processing tests, but there was no significant difference on theory of mind., Conclusions: These results seem to confirm the presence of social cognition difficulties in NF1 that could explain, at least in part, their social difficulties, although not all dimensions are concerned. The differences between the processes we assessed are discussed in relation to the methodologies used to measure them, and raises questions about the complementarity of traditional tools and more ecological assessments.

Neurofibromatosis type 1 (NF1) is a genetic disease that can lead to impaired social adaptation and functioning, thus affecting quality of life. To date, studies of these children's social cognition abilities have been scant and far from exhaustive. Therefore, the purpose of the present study was to assess the ability of children with NF1, compared with controls, to process facial expressions of emotions - not only including the usual primary emotions (happiness, anger, surprise, fear, sadness and disgust), but secondary emotions, too. To do so, the links between this ability and the characteristics of the disease (mode of transmission, visibility, and severity) were examined. A total of 38 children with NF1 aged 8-16 years 11 months (mean = 11.4, SD  = 2.3) and 43 sociodemographically comparable control children performed the emotion perception and recognition tests of a social cognition battery. Results confirmed that the processing of primary and secondary emotions is impaired in children with NF1, but there were no significant links with either mode of transmission, severity, or visibility. These results encourage further comprehensive assessments of emotions in NF1, and suggest that investigations should be extended to higher level social cognition skills, such as theory of mind and moral judgments.