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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]

Dupilumab, a first-in-class therapy targeting the two key cytokines involved in the persistent underlying inflammatory pathway in atopic dermatitis (AD), is approved for treatment of moderate-to-severe AD in Europe, USA, Japan and several other countries. To assess dupilumab effects on SCORing Atopic Dermatitis (SCORAD) and component scores (objective and subjective SCORAD) over time in adults with moderate-to-severe AD. This post hoc analysis included 2,444 patients in four placebo-controlled, double-blind, randomized, phase 3 trials. SOLO 1 and 2 (NCT02277743; NCT02277769) evaluated 16 weeks of dupilumab monotherapy against placebo. CAFÉ (NCT02755649) and CHRONOS (NCT02260986) evaluated dupilumab with concomitant topical corticosteroids (TCS) against TCS alone for 16 and 52 weeks, respectively. 2,444 patients randomized to treatment in SOLO 1 and 2 (N = 1,379), CAFÉ (N = 325) and CHRONOS (N = 740) were analyzed. Dupilumab treatment significantly improved overall SCORAD and individual components as early as Week 1 or 2, with significant and clinically meaningful differences vs. control through end of treatment (p <.0001). These results occurred irrespective of dupilumab regimen, 300 mg subcutaneously weekly or every 2 weeks. In four large phase 3 trials in adults with moderate-to-severe AD, dupilumab treatment with or without concomitant TCS resulted in rapid and sustained improvements in all SCORAD outcomes vs. placebo or TCS alone. [ABSTRACT FROM AUTHOR]

Background: Atopic dermatitis (AD) is a chronic, relapsing, inflammatory skin disease. Therapeutic patient education (TPE) has been demonstrated to be effective in AD in reducing disease severity and improving coping and quality of life. Objectives: To describe the sociodemographic and clinical characteristics of children and adolescents with AD who had attended TPE sessions, as well as the characteristics of their parents, and compare them with those who did not attend TPE. Methods: Parents of children with AD aged 6–17 years old were recruited from a representative sample of the French population contacted by e‐mail. Sociodemographic data and clinical information were collected in patients and parents. Clinical severity was assessed by parents using a proxy version of the Patient‐Oriented Eczema Measure (POEM). Attendance to TPE sessions was assessed by the following question 'did your child or one or both parents attended TPE for AD?'. Also, the number of sessions was recorded. Determinants of TPE attendance were evaluated by univariable and multivariable analyses. Results: Data were collected on 1063 parents and children with AD. A total of 131 (12.3%) children and/or parents attended TPE sessions. Most of them attended 2–5 TPE sessions. In that group, there were 85 boys (64.9%), and severity evaluated by POEM was mild in 29.8%, moderate in 52.7% and severe in 17.6% of patients. In the multivariable model, attending TPE sessions was significantly associated with sex of the child (boy vs. girl), consultation with a dermatologist or a paediatrician, high clinical severity and presence of AD in parents. Conclusions: Despite recommendations, the use of TPE in children with AD is still low in France. There is a need for implementing such programmes in the management of the disease, in particular when the disease is severe. [ABSTRACT FROM AUTHOR]

Background: Our suggested 'modern' concepts of 'neutrophilic dermatoses' (ND) and 'neutrophilic disease' were based on observations in adult patients and have not been studied in paediatric patients. Only a minority of ND occurs in children, and little is known about age‐specific characteristics. Objectives: To describe age‐specific characteristics of ND in children and to study whether our suggested 'modern' classification of ND may be applied to children. Methods: We conducted a retrospective multicentre study in a French cohort of 27 paediatric patients diagnosed with pyoderma gangrenosum (PG) or Sweet's syndrome (SS). Results: Demographics and distribution of typical/atypical forms were similar in patients diagnosed with PG and SS. Atypical ND were more frequent in infants (90%), when compared to young children (60%) and adolescents (33%). Neutrophilic disease was observed in 17/27 patients and was most frequent in infants. Neutrophilic disease of the upper respiratory tract, as well as cardiac neutrophilic disease, was only observed in infants, whereas other locations were similarly found in infants, young children and adolescents. In infants and young children, ND were associated with a large spectrum of general diseases, whereas in adolescents associations were limited to inflammatory bowel disease and Behçet's disease. Conclusions: Our study describes the concept of ND in paediatric patients and shows that they have some characteristics different from ND occurring in adults. ND occurring in infants can be associated with a large spectrum of general diseases. Occurrence of neutrophilic disease is frequent in children. Thus, ND occurring in young paediatric patients should incite clinicians to schedule complementary explorations in order to search for involvement of other organs and to rule out monogenetic autoinflammatory syndromes. [ABSTRACT FROM AUTHOR]

Abstract: Background: There are gaps in our knowledge of the prevalence of adult atopic dermatitis (AD). Objective: To estimate the prevalence of AD in adults and by disease severity. Methods: This international, cross‐sectional, web‐based survey was performed in the United States, Canada, France, Germany, Italy, Spain, United Kingdom, and Japan. Adult members of online respondent panels were sent a questionnaire for AD identification and severity assessment; demographic quotas ensured population representativeness for each country. A diagnosis of AD required subjects to be positive on the modified UK Working Party/ISAAC criteria and self‐report of ever having an AD diagnosis by a physician. The proportion of subjects with AD who reported being treated for their condition was determined and also used to estimate prevalence. Severity scales were Patient‐Oriented SCORAD, Patient‐Orientated Eczema Measure, and Patient Global Assessment. Results: Among participants by region, the point prevalence of adult AD in the overall/treated populations was 4.9%/3.9% in the US, 3.5%/2.6% in Canada, 4.4%/3.5% in the EU, and 2.1%/1.5% in Japan. The prevalence was generally lower for males vs females, and decreased with age. Regional variability was observed within countries. Severity varied by scale and region; however, regardless of the scale or region, proportion of subjects reporting severe disease was lower than mild or moderate disease. Conclusions: Prevalence of adult AD ranged from 2.1% to 4.9% across countries. Severe AD represented a small proportion of the overall AD population regardless of measure or region. [ABSTRACT FROM AUTHOR]

Abstract: This guideline was developed as a joint interdisciplinary European project, including physicians from all relevant disciplines as well as patients. It is a consensus‐based guideline, taking available evidence from other guidelines, systematic reviews and published studies into account. This second part of the guideline covers antimicrobial therapy, systemic treatment, allergen‐specific immunotherapy, complementary medicine, psychosomatic counselling and educational interventions, whereas the first part covers methods, patient perspective, general measures and avoidance strategies, basic emollient treatment and bathing, dietary intervention, topical anti‐inflammatory therapy, phototherapy and antipruritic therapy. Management of AE must consider the individual clinical variability of the disease. Systemic immunosuppressive treatment with cyclosporine, methotrexate, azathioprine and mycophenolic acid is established option for severe refractory cases, and widely available. Biologicals targeting the T helper 2 pathway such as dupilumab may be a safe and effective, disease‐modifying alternative when available. Oral drugs such as JAK inhibitors and histamine 4 receptor antagonists are in development. Microbial colonization and superinfection may cause disease exacerbation and can require additional antimicrobial treatment. Allergen‐specific immunotherapy with aeroallergens may be considered in selected cases. Psychosomatic counselling is recommended especially in stress‐induced exacerbations. Therapeutic patient education (‘Eczema school’) is recommended for children and adult patients. General measures, basic emollient treatment, bathing, dietary intervention, topical anti‐inflammatory therapy, phototherapy and antipruritic therapy have been addressed in the first part of the guideline. [ABSTRACT FROM AUTHOR]

Abstract: This guideline was developed as a joint interdisciplinary European project, including physicians from all relevant disciplines as well as patients. It is a consensus‐based guideline, taking available evidence from other guidelines, systematic reviews and published studies into account. This first part of the guideline covers methods, patient perspective, general measures and avoidance strategies, basic emollient treatment and bathing, dietary intervention, topical anti‐inflammatory therapy, phototherapy and antipruritic therapy, whereas the second part covers antimicrobial therapy, systemic treatment, allergen‐specific immunotherapy, complementary medicine, psychosomatic counselling and educational interventions. Management of AE must consider the individual clinical variability of the disease; highly standardized treatment rules are not recommended. Basic therapy is focused on treatment of disturbed barrier function by hydrating and lubricating topical treatment, besides further avoidance of specific and unspecific provocation factors. Topical anti‐inflammatory treatment based on glucocorticosteroids and calcineurin inhibitors is used for flare management and for proactive therapy for long‐term control. Topical corticosteroids remain the mainstay of therapy, whereas tacrolimus and pimecrolimus are preferred in sensitive skin areas and for long‐term use. Topical phosphodiesterase inhibitors may be a treatment alternative when available. Adjuvant therapy includes UV irradiation, preferably with UVB 311 nm or UVA1. Pruritus is targeted with the majority of the recommended therapies, but some patients may need additional antipruritic therapy. Antimicrobial therapy, systemic anti‐inflammatory treatment, immunotherapy, complementary medicine and educational intervention will be addressed in part II of the guideline. [ABSTRACT FROM AUTHOR]

Résumé: Les gammapathies monoclonales peuvent s’associer à diverses manifestations cliniques. Les mécanismes à l’œuvre sont variés et parfois mal compris. Parmi les manifestations cliniques associées aux gammapathies monoclonales, la peau est un organe fréquemment atteint. L’atteinte cutanée peut être inaugurale voire isolée. Elle peut aussi être discrète, au second plan d’un tableau systémique dont elle peut représenter un élément diagnostique majeur. Cette mise au point a pour objectif de passer en revue ces différentes manifestations cutanées. [ABSTRACT FROM AUTHOR]

Background It is not yet known whether the risk of developing atopic dermatitis ( AD) is influenced by preterm birth. Moreover, AD risk has not been assessed in a large sample of extremely preterm infants (< 29 weeks' gestation). Objectives To determine whether the risk of AD is influenced by preterm birth. Methods We investigated the relationship between gestational age ( GA) and AD using data from two independent population-based cohorts, including a total of 2329 preterm infants, of whom 479 were born extremely preterm. Results There was a lower percentage of children with AD in the extremely preterm group compared with those born at a greater GA (Epipage cohort, 2-year outcome: 13·3% for 24-28 weeks, 17·6% for 29-32 weeks, 21·8% for 33-34 weeks, P = 0·02; LIFT cohort, 5-year outcome: 11% for 24-28 weeks, 21·5% for 29-32 weeks, 19·6% for 33-34 weeks, P = 0·11). After adjusting for confounding variables, a lower GA (< 29 weeks) was significantly associated with decreased risk of AD in the Epipage cohort [adjusted odds ratio ( aOR) 0·57, 95% confidence interval (CI) 0·37-0·87; P = 0·009] and the LIFT cohort ( aOR 0·41, 95% CI 0·18-0·90; P = 0·03). Conclusions Very low GA (< 29 weeks) was associated with a lower risk of AD compared with higher GA (29-34 weeks) and full-term birth. [ABSTRACT FROM AUTHOR]

Abstract: Many cutaneous adverse events have been identified with recently developed targeted treatments. Some of them are common and specific, like paradoxical psoriasiform eruptions with anti-TNFα, papulopustular eruptions and paronychias with EGFR inhibitors and peculiar hand-foot skin reactions with multitargeted kinase inhibitors sorefenib and sunitinib. Patients treated with these recently available biologics need a careful monitoring. [Copyright &y& Elsevier]

Background: Patient-oriented medicine is an emerging concept, encouraged by the World Health Organization, to greater involvement of the patient in the management of chronic diseases. The Patient-Oriented SCORing Atopic Dermatitis (PO-SCORAD) index is a self-assessment score allowing the patient to comprehensively evaluate the actual course of atopic dermatitis (AD), using subjective and objective criteria derived mainly from the SCORAD, a validated AD severity clinical assessment tool. Objectives: To validate the PO-SCORAD index in a large European population of patients exhibiting all forms of AD severity by assessing its correlation with the SCORAD index. Patients/methods: Four hundred and seventy-one patients (185 adults, 286 children) consulting for AD in hospitals from 9 European countries were recruited. The investigators and the patients used the SCORAD and PO-SCORAD scales, respectively, to assess AD severity at inclusion (D0) and 28 ± 7 days later (D28). Results: Patient-Oriented SCORing Atopic Dermatitis and SCORAD scores were significantly correlated at D0 [r = 0.67 (95% CI: 0.62; 0.72), P < 0.0001]. Consistency was confirmed at D28, with a stronger linear correlation between both scales [r = 0.79 (95% CI: 0.75; 0.83), P < 0.0001]. Absolute changes from baseline in SCORAD and PO-SCORAD scores were also significantly correlated [r= 0.71 (95% CI: 0.64; 0.76), P < 0.0001]. Although no specific intervention was investigated, AD improved over the study, with a decrease of PO-SCORAD and SCORAD scores from D0 to D28 by )19.19% and )24.39%, respectively. The consistency of the correlations was similar in the adult and children groups. Conclusions: This study validated the use of PO-SCORAD to self-assess AD severity and demonstrated its good correlation with SCORAD. [ABSTRACT FROM AUTHOR]

Lamellar ichthyosis is a rare congenital disorder that can be encountered by plastic surgeon in a daily practice. Its clinical diagnosis makes it an significant pathology to identify and to know how to treat. We report the case of a patient suffering from lamellar ichthyosis complicated by erosive pseudo pustulosis of the scalp. Our treatment protocol with two intra-lesional delayed-corticoids (Kenacort ®) injections three months apart showed significant clinical improvement of the lesions. Congenital lamellar ichthyosis regroups various clinical presentations. Most of the therapeutic strategies described in the literature involve local and systemic treatments, weighing on patients and leading to modest results. Surgical treatment or hyaluronic injections have also been reported but they raise problematics regarding morbidity and efficiency. Our therapeutic strategy by two Kenacort ® injections three months apart is simple, reproductible and has shown efficiency in the treatment of our patient suffering from congenital lamellar ichthyosis complicated with erosive pseudo pustulosis of the scalp. [ABSTRACT FROM AUTHOR]

The article offers information on a study which deals with atopic eczema (AE) which is the most common chronic inflammatory skin disease. It mentions information on association between AE severity and the likelihood to use emollients and topical steroids. It states that topical steroids and emollients remain the mainstay of treatment but there are many barriers to medication adherence, including topical steroid phobia and the daily burden of complex topical treatments.

A letter to the editor is presented in response to an article related to the schnitzler syndrome.

In chronic skin diseases such as atopic dermatitis (AD), therapeutic failure due to poor patient adherence to treatment is commonly reported. Therapeutic patient education (TPE) is an approach to improve self‐management and adherence. Several studies demonstrated that TPE programmes have positive effects on disease management resulting in decreased disease severity and improved quality of life in AD patients. Various healthcare professionals (dermatologists, nurses, psychologists, dieticians) have been involved. TPE performed by trained dermatology nurses are highly efficient and improve various health‐related outcomes. The aim of this position paper is to analyse the aims, modalities and efficacy of TPE in AD, to identify specific roles of dermatology nurses, to assess qualification requirements, and to propose practical recommendations. Potential activities of nurses in ongoing and future TPE programmes for AD patients will be discussed. [ABSTRACT FROM AUTHOR]

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]

Dr. Chernyshov, Dr. Stalder, Dr. Svensson, Dr. Kunz reports no conflict of interest. At least 3 weeks are recommended between the two COVID-19 vaccine doses, which increases the risk of AD flares and loss of AD control if the systemic AD medication is paused or reduced in dose for longer periods. European Task Force on Atopic Dermatitis: position on vaccination of adult patients with atopic dermatitis against COVID-19 (SARS-CoV-2) being treated with systemic medication and biologics. [Extracted from the article]

BACKGROUND Prurigo nodularis is a chronic, debilitating, and severely pruritic neuroimmuno-logic skin disease. Nemolizumab, an interleukin-31 receptor alpha antagonist, down-regulates key pathways in the pathogenesis of prurigo nodularis. METHODS In this phase 3, double-blind, multicenter, randomized trial, we assigned adults with moderate-to-severe prurigo nodularis to receive an initial 60-mg dose of nemolizumab followed by subcutaneous injections of 30 mg or 60 mg (depending on baseline weight) every 4 weeks for 16 weeks or matching placebo. The primary end points were an itch response (a reduction of ≥4 points on the Peak Pruritus Numerical Rating Scale [PP-NRS; scores range from 0 to 10, with higher scores indicating more severe itch]) and an Investigator's Global Assessment (IGA) response (a score of 0 [clear] or 1 [almost clear] on the IGA [scores range from 0 to 4] and a reduction from baseline to week 16 of ≥2 points). There were five key secondary end points. RESULTS A total of 274 patients underwent randomization; 183 were assigned to the nemolizumab group, and 91 to the placebo group. Treatment efficacy was shown with respect to both primary end points at week 16; a greater percentage of patients in the nemolizumab group than in the placebo group had an itch response (56.3% vs. 20.9%; strata-adjusted difference, 37.4 percentage points; 95% confidence interval [CI], 26.3 to 48.5), and a greater percentage in the nemolizumab group had an IGA response (37.7% vs. 11.0%; strata-adjusted difference, 28.5 percentage points; 95% CI, 18.8 to 38.2) (P<0.001 for both comparisons). Benefits were observed for the five key secondary end points: itch response at week 4 (41.0% vs. 7.7%), PP-NRS score of less than 2 at week 4 (19.7% vs. 2.2%) and week 16 (35.0% vs. 7.7%), and an improvement of 4 or more points on the sleep disturbance numerical rating scale (range, 0 [no sleep loss] to 10 [unable to sleep at all]) at week 4 (37.2% vs. 9.9%) and week 16 (51.9% vs. 20.9%) (P<0.001 for all comparisons). The most common individual adverse events were headache (6.6% vs. 4.4%) and atopic dermatitis (5.5% vs. 0%). CONCLUSIONS Nemolizumab monotherapy significantly reduced the signs and symptoms of prurigo nodularis. [ABSTRACT FROM AUTHOR]

The authors reflect on the need to validate eczema diagnosis to improve the reliability of epidemiological studies. They reference the article "Assessment of atopic dermatitis using self-report and caregiver report: a multicentre validation study" by JI Silverberg and colleagues which appeared within the issue.

The article discusses research on the use of magnetic resonance imaging (MRI) in children suffering from congenital melanocytic naevi (CMN). Topics covered include the impacts of mutations in neuroblastoma ras gene, extracutaneous symptoms of CMN, and characteristics of neurocutaneous melanosis (NCM). The importance of early central nervous system MRI in children with CMN is also mentioned.

Atopic dermatitis (AD) is a common inflammatory skin disease that affects both children and adults, including a large number of adults of reproductive age. Several guidelines for the treatment of AD exist, yet specific recommendations for the treatment of pregnant or lactating women and for adults planning to have a child are often lacking. This position paper from the European Task force on Atopic Dermatitis (ETFAD) is based on up‐to‐date scientific literature on treating pregnant and lactating women as wells as adults with AD planning to have a child. It is based on the expert opinions of members of the ETFAD and on existing safety data on the proposed treatments, many of which are derived from patients with other inflammatory diseases or from transplantation medicine. For treating future parents, as well as pregnant and lactating women with AD, the use of topical treatments including moisturizers, topical corticosteroids, tacrolimus, antiseptics such as chlorhexidine, octenidine, potassium permanganate and sodium hypochlorite (bleach) is deemed to be safe. Ultraviolet (UV) therapy may also be used. Systemic treatment should be prescribed only after careful consideration. According to the opinion of the ETFAD, treatment should be restricted to systemic corticosteroids and cyclosporine A, and, in selected cases, azathioprine. [ABSTRACT FROM AUTHOR]

Background: Atopic dermatitis (AD) is a heterogeneous inflammatory skin disease with different clinical phenotypes based on factors such as age, race, comorbidities, and clinical signs and symptoms. The effect of these factors on therapeutic responses in AD has only been scarcely studied and not for upadacitinib. Currently, there is no biomarker predicting response to upadacitinib. Objectives: Evaluate the efficacy of the oral Janus kinase inhibitor upadacitinib across patient subgroups (baseline demographics, disease characteristics and prior treatment) in patients with moderate‐to‐severe AD. Methods: Data from phase 3 studies (Measure Up 1, Measure Up 2 and AD Up) were utilized for this post hoc analysis. Adults and adolescents with moderate‐to‐severe AD were randomized to receive once daily oral upadacitinib 15 mg, upadacitinib 30 mg or placebo; patients enrolled in the AD Up study received concomitant topical corticosteroids. Data from the Measure Up 1 and Measure Up 2 studies were integrated. Results: A total of 2584 patients were randomized. A consistently greater proportion of patients achieved at least 75% improvement in the Eczema Area and Severity Index, a 0 or 1 on the validated Investigator Global Assessment for Atopic Dermatitis, and improvement in itch (including an achievement of a reduction of ≥4; and score of 0/1 in Worst Pruritus Numerical Rating Scale) with upadacitinib compared with placebo at Week 16, regardless of age, sex, race, body mass index, AD severity, body surface area involvement, history of atopic comorbidities or asthma, or previous exposure to systemic therapy or cyclosporin. Conclusions: Upadacitinib had consistently high skin clearance rates and itch efficacy across subgroups of patients with moderate‐to‐severe AD through Week 16. These results support upadacitinib as a suitable treatment option in a variety of patients. TRIAL REGISTRATION: ClinicalTrials.gov Identifiers: NCT03569293 (Measure Up 1), NCT03607422 (Measure Up 2) and NCT03568318 (AD Up). [ABSTRACT FROM AUTHOR]

The article offers information regarding a retrospective analysis of 55 cases of measles resurgence. It mentions that the incidence of higher percentages of measles patients and their hospitalizations are higher in France since 2008. It also mentions that the incidence of notified measles cases was 0.96 per 100,000 population in 2008 (604 cases), and rose to 2.5 per 100,000 in 2009 (1.544 cases) and approximately 2000 cases were reported from January to May 2010.

The article comments on a study by C. H. Lin and colleagues which investigated the link between atopic dermatitis (AD) and type 1 diabetes (T1DM). Topics discussed include the higher incidence of AD in patients with T1DM, the use of Cox proportional hazards regression model to analyze AD risk, and the possibility of the existence of different endophenotypes within the AD and T1DM spectrum.

Le zinc est un cofacteur de nombreux enzymes. Le déficit acquis en zinc se manifeste chez les nouveau-nés par une dermatose périorificielle aigue (pseudoacrodermatite enteropathique) et peut être du à une carence d’apport, des pertes excessives. Nous rapportons 5 cas chez des prématurés de faible poids de naissance en nutrition parentérale (NPE) exclusive au cours d’entérocolites ulcéronécrosantes pour 3 cas, malgré un apport en zinc adapté dans quatre cas. Il s’agissait de 5 nouveau-nés prématurés (24 à 31 SA) de poids de naissance faible à très faible (650 gr à 1270 gr) atteints d’entérocolites ulcéronécrosantes et/ou nourris par NPE prolongée exclusive. Deux nouveau-nés étaient porteurs d’un retard de croissance intra utérin (-3 DS). 4/5 recevaient une supplémentation en zinc selon les recommandations (780 μg/kg/jr). Les 5 nouveau-nés présentaient, de 7 à 20 semaines de vie, une dermatose périorificelle inflammatoire aiguë. Ces signes étaient plus précoces chez le nouveau-né non substitué (7 semaines) par rapport aux 4 nouveau-nés substitués (13, 18, 19 et 20 semaines). L’examen neurologique était normal. Les lésions guérissaient totalement et rapidement sous supplémentation en zinc (1500 μg/kg/jr) en 4 à 16 jours. Les dosages plasmatiques de zinc réalisés dans 4 cas sur 5 étaient inférieurs aux taux normaux pour l’age (108 μg/l à 490 μg/l, N > 500 μg/l). Aucune rechute n’était observée à l’arrêt du traitement. Les cas rapportés présentaient une dermatose identique chez des prématurés sous NPE observés sur 5 ans. Le diagnostic repose sur : l’aspect clinique identique, le contexte (prématurité (faible stock en zinc), NPE exclusive avec substitution inadéquate en zinc dans1 cas, enterocolite ulcéronécrosante ou entéropathie), l’absence d’autre étiologie possible (amino-acidopathie), la guérison sous supplémentation sans rechute à l’arrêt, une zincémie basse dans 4 cas sur 5. L’absence de substitution en zinc dans 1 cas explique la précocité des symptomes. L’apparition d’un déficit symptomatique chez les 4 cas recevant une substitution adéquate illustre qu’il existe plusieurs facteurs de risques de carence (NPE, malabsorption, prématurité, perte digestives excessives) et doit rendre le clinicien vigilant afin de dépister et traiter précocement ces enfants. [Copyright &y& Elsevier]

Le purpura fulminans (PF) de l’enfant est une maladie grave associant un purpura cutané nécrotique et une coagulation intravasculaire disséminée (CIVD). La cause la plus fréquente est l’infection à méningocoque. Nous rapportons un cas secondaire à un déficit acquis de la protéine S, sans cause infectieuse identifiée. Un garçon de 17 mois était vu pour deux lésions purpuriques des cuisses. L’état général était normal. Il n’avait aucun antécédent hormis une varicelle 9 mois auparavant. Quelques heures après, les lésions cutanées s’étendaient. Le bilan révélait une CIVD. Il n’y avait aucun argument pour une infection bactérienne (procalcitonine et hémocultures normales, PCR méningocoque dans le LCR négative). La prise en charge associait une administration de plasma frais congelé, une antibiothérapie probabiliste à large spectre, un support hémodynamique, et une héparinothérapie. Une amélioration était observée rapidement. Une exérèse des tissus nécrosés suivie d’une greffe autologue étaient réalisés. On retrouvait un déficit complet transitoire en protéine S avec détection d’un anticorps antiprotéine S circulant. La recherche d’une étiologie infectieuse était négative. Il n’y avait aucun argument pour une maladie autoimmune sous jacente. Nous rapportons le cas d’un nourrisson atteint d’un PF secondaire à un déficit acquis de la protéine S. Les sepsis bactériens (méningocoque, pneumocoque) sont les causes les plus fréquentes. Le PF post infectieux est un tableau différent très rare qui débute 7 à 10 jours après une varicelle. Les patients présentent un purpura nécrotique extensif et une CIVD. Il n’y a pas de sepsis et les prélèvements bactériologiques sont négatifs. Cela est du à la présence transitoire dans le serum d’anticorps anti protéine S entrainant un déficit acquis en protéine S. Les déficits génétiques en proteine C et S et les déficits acquis sont responsables de tableaux thromboemboliques pédiatriques variés et de CIVD dont le purpura fulminans. Notre observation est originale car nous n’avons pas retrouvé de cause infectieuse malgré une recherche exhaustive des causes connues dans la littérature. Ceci suggère que des étiologies non identifiées sont potentiellement en cause. Le déficit en protéine S acquis est une cause rare de purpura fulminans de l’enfant. [Copyright &y& Elsevier]

COVID-19 lockdown induced acral dermatosis in children Recently, several case series of cutaneous acral manifestations in patients with suspected or confirmed coronavirus disease 2019 (COVID-19) have been reported.1-3 A majority of these acral manifestations have a pseudo-chilblain pattern with erythema and oedema and sometimes vesicles. These asymptomatic lesions were located over the bony prominences of the feet and legs, suggesting a frictional dermatosis. [Extracted from the article]

A letter to the editor is presented in response to the article about the efficiency of ciclosporin in treating Kimura's disease, which appeared within the issue.

A letter to the editor is presented in response to the article about neurofibromatosis 1 (NF1).

Background Schnitzler syndrome is characterized by an urticarial rash, a monoclonal gammopathy, and clinical, histological, and biological signs of neutrophil-mediated inflammation. The aim of this study was to assess the applicability and validity of the existing diagnostic criteria in real-life patients. Methods This multicentric study was conducted between 2009 and 2014 in 14 hospitals in which patients with Schnitzler syndrome or controls with related disorders were followed up. We compared the sensitivities and specificities and calculated the positive and negative predictive values of the Lipsker and of the Strasbourg criteria for the patients with Schnitzler syndrome and for the controls. We included 42 patients with Schnitzler syndrome, 12 with adult-onset Still's disease, 7 with cryopyrin-associated periodic disease, 9 with Waldenström disease, and 10 with chronic spontaneous urticaria. Results All patients with Schnitzler syndrome met the Lipsker criteria. According to the Strasbourg criteria, 34 patients had definite Schnitzler syndrome, five had probable Schnitzler syndrome, and three did not meet the criteria. One control met the Lipsker criteria and had probable Schnitzler syndrome according to the Strasbourg criteria. Sensitivity and specificity of the Lipsker criteria were 100% and 97%, respectively. For the Strasbourg criteria, sensitivity for definite and probable diagnosis was 81% and 93%, respectively, with a corresponding specificity of 100% and 97%. Conclusion Diagnostic criteria currently in use to diagnose Schnitzler syndrome are reliable. More investigations must be done to attest their efficiency in patients with recent-onset manifestations. [ABSTRACT FROM AUTHOR]

Background: Dupilumab is the first biotherapy available for the treatment of moderate‐to‐severe childhood atopic dermatitis (AD). Objective: The aim of this study was to evaluate the effectiveness and safety of dupilumab in daily practice. Methods: Patients aged 6–11, who had received a first dose of dupilumab, were included in this multicentre retrospective cohort study. The primary endpoint was change in SCORAD after 3 months of treatment. Secondary endpoints were change in IGA score at 3 months, proportion of patients with SCORAD50 and SCORAD75, description of adverse events and proportion of children in our cohort who would be excluded from pivotal phase 3 clinical trial. Results: Eighty patients were included. After 3 months of treatment, there was a significant decrease in SCORAD (mean: 21.8 ± 13.8 vs 53.9 ± 18.5; P < 0.0001) and IGA (1.3 ± 0.8 vs 3.5 ± 0.7; P < 0.0001). Conjunctivitis was observed in 11.3% (n = 9/80); three patients experienced dupilumab facial redness (DFR); 17.5% (n = 14/80) reported injection site reactions; 6.3% (n = 5/80) discontinued treatment. 61.2% (n = 49/80) children were ineligible in the phase 3 trial. Limitations: There is no control group. Because it was a real life study based on information from patient medical records in a French multicentre cohort, we cannot rule out the presence of reporting bias generated by the use of patient reported characteristics and missing information. Conclusion: These real‐life data confirm the efficacy and safety of dupilumab in children with moderate to severe AD extended to dyshidrosis and atopic prurigo, but it also revealed a lower frequency of DFR and conjunctivitis. However, administration in injectable form may be a barrier in this age group. [ABSTRACT FROM AUTHOR]

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 paediatric 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. [ABSTRACT FROM AUTHOR]

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 paediatric, adolescent, pregnant and breastfeeding patients. [ABSTRACT FROM AUTHOR]

Background: Neurofibromatosis 1 (NF1) is one of the most common inherited disorders characterized by mutations in the tumour suppressor gene NF1. Its clinical manifestations are highly variable and unpredictable. A specific NF1 mutation does not predict the severity or complications of the disease. Objective: The objective of this study was to build an empirical classification scheme without any a priori hypotheses to identify the underlying NF1 subtypes that best explain the observed heterogeneity. Methods: We performed latent class analysis (LCA) of 1351 consecutive NF1 patients aged >17 years seen between 2002 and 2014. Data and phenotypic features were collected prospectively on a standardized form. Results: The median age was 36.8 (17–81) years. A three‐class model showed the best fit: 706 (52%) belonged to the LC1 'Cutaneous neurofibromas' class having preferentially cutaneous neurofibromas (99%), plexiform neurofibromas (63%) and blue‐red macules (29%); 593 (44%) belonged to the LC2 'Subcutaneous neurofibromas' class characterized by the presence of at least 10 subcutaneous neurofibromas (21%) and a familial form (77%) and 52 (4%) belonged to the LC3 'Dysmorphic phenotype' class characterized by dysmorphic features (78%) and learning difficulties (87%). Patients in LC1 had a higher likelihood of developing scoliosis (RR = 1.7, 95% confidence interval (CI) [1.2–2.4]). Patients in LC2 were more likely to be men (RR = 1.4, 95% CI [1.1–1.7]). Patients in LC3 were at higher risk of having an optic pathway glioma (RR = 4.8, 95% CI [1.9–11.8]) and epilepsy (RR = 4.5, 95% CI [1.8–11.6]). Conclusion: Our findings invite the performance of a larger cohort study to test whether the various latent classes reflect different underlying genetic modifiers of these phenotypic traits. [ABSTRACT FROM AUTHOR]

Résumé: La dermatite atopique est une maladie fréquente, dont les soins locaux doivent être continuellement adaptés à l’état cutané de l’enfant. Au-delà de la consultation, le suivi de la prescription se heurte à l’évolution capricieuse de la maladie. Parents et enfants doivent disposer d’informations qui les aident à réaliser le traitement selon l’évolution de l’eczéma. Le Plan d’action personnalisé écrit pour l’atopique (PA2P) a été adapté du modèle utilisé dans l’asthme en intégrant les conseils relatifs à la dermatite atopique de l’enfant. L’objectif de cette étude était d’évaluer auprès de pédiatres la pertinence et l’utilité de l’outil PA2P dans la prise en charge du patient et de sa famille. Après présentation du PA2P, 479 pédiatres ont répondu à un questionnaire adressé par courrier électronique pour recueillir leur jugement. L’outil a été perçu favorablement par la quasi-totalité des pédiatres interrogés : 99 % le déclaraient pertinent en le qualifiant de « clair » et « logique ». Le PA2P paraît, selon les pédiatres interrogés, adapté au besoin du patient atopique en améliorant l’implication de la famille dans les soins locaux ; il permet une personnalisation de la prise en charge et simplifie la proposition de soin du médecin. Finalement 72 % des médecins interrogés se sont portés volontaires pour participer à une étude ultérieure de validation auprès des patients. [ABSTRACT FROM AUTHOR]

Abstract: Schnitzler syndrome (SS) is a rare clinical entity, which belongs to the spectrum of monoclonal gammapathy-associated systemic disorders. Its pathophysiology remains elusive, even if it is tempting to consider it as a late onset and probably acquired auto-inflammatory syndrome. SS mainly occurs in the fifth and sixth decade, and present with an urticariform rash with periodic fever and/or osteoarticular pain. Systemic inflammation and monoclonal gammapathy (overwhelmingly IgM kappa) are constant features. SS is a chronic disease, which can severely impair quality of life of the affected individuals. Many drugs have been used and proved disappointing. In the last few years, accumulating reports provided evidence for the dramatic efficacy of anakinra, which has revolutionized the management of most severe cases. The main long-term threat to these patients is to develop a lymphoproliferative disorder (mainly Waldenström''s macroglobulinemia). The mechanisms underlying the different facets of the disease remain to be elucidated. [Copyright &y& Elsevier]

The patient-oriented eczema measure: development and initial validation of a new tool for measuring atopic eczema severity from the patients' perspective. Atopic dermatitis (AD) is a chronic disease with a strong impact on patients and their families.1 Clinical severity is usually evaluated by the clinician; however, it may be perceived in a different way by the patient and the caregiver. Kappa < 0 represents disagreement, 0-0.20 very low agreement, 0.21-0.40 low agreement, 0.41-0.60 moderate agreement, 0.61-0.80 strong agreement and 0.80-1.00 almost total agreement. [Extracted from the article]

Abstract: Introduction: The face is frequently involved in systemic sclerosis. The main stomatologic manifestations include limited mouth opening, xerostomia, skin atrophy, trigeminal neuralgia. The objective of this study was to describe oral and facial manifestations observed in scleroderma patients from our cohort. Methods: Between March and October 2006, a stomatologic consultation was included in the follow-up of scleroderma patients seen during consultation or daily hospital in internal medicine or dermatology units. Demographic, clinical and biological data were collected. Stomatologic examination comprised measure of the mouth opening, sugar''s and Schirmer''s tests, orthopantomogram analysis, and evaluation of the repercussion of symptoms on quality of life using a visual analogical scale (VAS between 0 and 10). Results: This study included 30 patients (women 87%, mean age 58.6±13.6 years). Mean duration of systemic sclerosis (n =20 limited cutaneous form, n =10 diffuse form) was eight years. Stomatologic manifestations were: skin atrophy (n =28), peribuccal rhagades (n =25), telangiectasia (n =21), decreased mouth opening (n =20), xerostomia (n =20), xerophtalmia (n =16), periodontal ligament space widening (n =10), bone resorptions (n =2), trigeminal neuralgia (n =1). Xerostomia was considered more discomforting (mean VAS=3.8) than decreased mouth opening (mean VAS=2.6). Xerostomia was the second more discomforting sign of scleroderma and was significantly associated to the limited cutaneous form (p =0.045) and to anticentromeres antibodies expression (p =0.002). Decreased mouth opening was correlated to oesophageal involvement (p =0.025). Conclusion: Oral and facial manifestations are frequently observed in scleroderma patients. These manifestations lead to major functional discomfort, mainly due to decreased mouth opening that seems to be frequently associated to oesophageal involvement. Xerostomia is also frequent and is commonly observed in anticentromere antibodies positive cutaneous limited forms of systemic sclerosis. Evolution of radiographic abnormalities like periodontal ligament space widening (33% of cases), or osteolytic lesions (7%) is poorly known. [Copyright &y& Elsevier]

Twenty experts, members of a French medical network devoted to neurofibromatosis 1 have elaborated recommendations for the management of the disease. Bibliography was obtained through a Medline of articles from 1966 to 1999 for the terms neurofibromatosis, NF1, neurofibroma and from textbooks. A consensual document was written taking into account extracted data. An annual careful clinical examination is recommended except in cases with complications. Screening investigations are not recommended due to the rarity of complications, generally symptomatic and easily detected during the clinical follow-up. The only controversial exception might be magnetic resonance imaging for early detection of optic pathway gliomas in young children. A co-ordinated follow-up in specialised multidisciplinary centres, providing patients with a rational management, is recommended. [ABSTRACT FROM AUTHOR]

Dermatological manifestations of hereditary fibrosing poikiloderma with tendon contractures, myopathy and pulmonary fibrosis (POIKTMP): a case series of 28 patients. Recently, the autosomal dominant hereditary fibrosing POIKiloderma with tendon contractures, myopathy and pulmonary fibrosis (POIKTMP; MIM 615704) has been described.1,2 POIKTMP is due to missense variations in the I FAM111B i gene. [Extracted from the article]

Dr. Seneschal has been an investigator, speaker, or consultant for Novartis, Abbvie, Sanofi, LeoPharma and Eli Lilly. Dr. De Raeve is a consultant, member of scientific advisory boards and/ or received personal fees and non-financial support from LEO Pharma, Pierre Fabre, Sanofi-Genzyme and Bioderma. Dr. Vestergaard has been investigator, speaker, or consultant for Novartis, Abbvie, Sanofi, LeoPharma and Eli Lilly. [Extracted from the article]

Background: Very few studies have evaluated the quality of life (QoL) of children suffering from low‐flow vascular malformations. This is the first study investigating the influencing factors. Objectives: To identify the factors influencing QoL in children with low‐flow vascular malformations. Methods: We conducted a qualitative study employing focus group interviews (Clinical Trials Number: NCT03440827). The study was a prospective, interventional, non‐comparative, multicentre study performed in four expert centres for vascular anomalies. Qualitative data about personal experiences, feelings, difficulties, needs and various factors influencing behaviours were collected. Theme‐based content analysis (manual and specialist textural software guided) were used to analyse the verbatim transcripts of all focus group sessions. Manual qualitative discourse analysis was performed to identify the different themes and categories. Informatics' analyses were subsequently performed for each individual category. Results: Ten focus groups (26 individuals including 10 children aged 11 to 15 years) were conducted until saturation. Influencing factors were related to 4 categories: medical care, self‐image, social impact on daily activities and challenging social relationships. These factors were responsible for intrafamily upheavals and may lead to future identity‐building problems. Conclusions: This study provides an essential framework from which physicians can develop strategies to improve patient care and quality of life. These data may also be useful to develop specific age‐sensitive QoL questionnaires. [ABSTRACT FROM AUTHOR]