Corticosteroid fear in parents of children with atopic dermatitis.

Background: Topical corticosteroids (TCS) are the mainstay of therapy for paediatric atopic dermatitis (AD). The use of TCS is often met with fear by parents. Assessing this parental TCS fear in clinical practice is still lacking. Aim: The aim was to assess parental fear and beliefs about TCS. Furthermore, we evaluated the quality of life (QoL) of the family and the disease severity of affected children. Methods: We conducted an observational study with a cross-sectional design. Inclusion criteria were children aged 0 to 5 years with a diagnosis of AD and outpatient treatment. The outcome measures were parental fears and beliefs about TCS, assessed with the "Topical Corticosteroid Phobia Score" (TOPICOP), parental QoL evaluated with the "Family Dermatology Life Quality Index", and disease severity, assessed with the "Scoring atopic dermatitis" (SCORAD). Descriptive statistic was used to analyse the data. Results: The current study found that in 40 affected children, 25 (62.5%), suffered from mild AD, 12 (30%) children had moderate AD, and 3 (7.5%) children had severe AD. TCS fear among parents was notable (mean TOPICOP score 18.1, standard deviation (SD) 7.1). The QoL was moderately affected (mean FDLQI score 6.5, SD 2.8). Conclusions: Our study indicates that fear of TCS is prevalent. Furthermore, our data indicate that severity of TCS fear varies markedly between parents, ranging from parents with almost no fear to parents with high levels of fear. For effective education in clinical practice, the individual level of fear must be recognized and taken into account.

Congenital syphilis in Switzerland: a retrospective cohort study, 2010 to 2019.

AIMS OF THE STUDY: We previously reported a re-emergence of syphilis from 2006 to 2009 with detection of congenital syphilis in Switzerland. This study aimed to reassess the incidence of children exposed to maternal syphilis during pregnancy and congenital syphilis in a following 10-year period in the canton of Zurich, the most populous canton in Switzerland with the highest incidences of syphilis. METHODS: Children were identified both by reviewing medical records at the four major neonatal and paediatric hospitals providing acute care in the canton of Zurich and by the serological database of the syphilis reference laboratory. Inclusion criteria for children were (a) date of birth in the period 2010-2019, (b) place of birth in the canton of Zurich, (c) evaluation for syphilis due to positive syphilis pregnancy screening and (d) age <1 year at diagnosis. Results were compared with epidemiological data provided by the Federal Office of Public Health (FOPH). RESULTS: We identified and evaluated 17 children after potential exposure to maternal syphilis. Residual antibodies of a past infection were found in 11 mothers. Six children were identified as having had real exposure to asymptomatic maternal syphilis. From an epidemiological perspective, the distribution of the cases followed a similar pattern as confirmed syphilis cases in women of childbearing age reported to the FOPH. No cases of congenital syphilis were observed. CONCLUSIONS: In contrast to the rise in syphilis infections, this study identified no cases of congenital syphilis in the canton of Zurich, Switzerland, in the period 2010-2019. Syphilis pregnancy screening may have prevented congenital syphilis by diagnosing and allowing adequate treatment of asymptomatic maternal syphilis.

The characteristics and efficacy of educational nurse-led interventions in the management of children with atopic dermatitis - An integrative review.

OBJECTIVE: This integrative review investigates the modalities, characteristics, and efficacy of educational nurse-led interventions for parents of children with atopic dermatitis (AD). METHODS: We conducted an integrative review with the following inclusion criteria: Quantitative, qualitative and mixed-method studies written in English or German and published between 1 January 2000 and 31 December 2021. We searched for entirely nurse-led interventions. RESULTS: Four RCTs, three pre-post studies without control groups, and one post-test study without control group met the inclusion criteria. Nurse-led interventions were found to have medium to large positive effect sizes on disease severity, and none to large positive effects on quality of life. There was significant heterogeneity across study designs with a wide variety in educational interventions and outcome measures used. The quality of most included studies was low. CONCLUSION: Nurse-led educational interventions for parents of children with AD are beneficial in reducing disease severity. However, it remains unclear, which main components of the nurse-led intervention are most effective. PRACTICE IMPLICATIONS: Based on the results of this review, we think that trained and qualified nurses who are part of the entire treatment of children with AD and do as well educational interventions have the greatest potential to improve outcomes.

Video-Based Education with Storytelling Reduces Parents' Fear of Topical Corticosteroid Use in Children with Atopic Dermatitis: A Randomized Controlled Trial (The EduDerm Study Part II).

OBJECTIVE: To investigate the efficacy of educational videos using storytelling to reduce parents' fear of topical corticosteroid (TCS) use in children affected by atopic dermatitis (AD). METHODS: Children aged 0 to 5 years who had AD were included. The primary outcome measures were parental fear of TCSs, as determined by Topical Corticosteroid Phobia score, and quality of life according to the Family Dermatology Life Quality Index. Disease severity, assessed by the Scoring Atopic Dermatitis tool, served as a secondary outcome measure. Assessments were performed at baseline (T1), 1 to 4 weeks later (T2), and at 3-month follow-up (T3). The intervention group was exposed to the videos between baseline and T2. RESULTS: Forty patients were recruited: 21 in the intervention group and 19 in the control group. A statistically significant decrease in parental TCS fear was found in the intervention group at T2 after video education as compared with the control group (P < .0001); this was maintained at T3 (P = .001). The groups did not significantly differ in FDLQI or SCORAD scores at any point. CONCLUSIONS: These findings suggest that video education based on the method of storytelling is effective in reducing TCS fear. Although the education did not impact disease severity or quality of life, effectively reducing TCS fear remains an important aspect for AD management.

National survey in Switzerland calls for improved diagnosis and treatment in children with scabies.

AIM OF THE STUDY: The global prevalence of scabies is estimated to be up to 200 million cases annually, with young children particularly affected. In Europe, most cases are thought to originate in migrant populations. Scabies management is challenging in children. To identify knowledge gaps and research needs, we aimed to descriptively evaluate the management of children with scabies by different Swiss healthcare providers. METHODS: An invitation for an anonymous online survey (36 questions) was sent to members of Swiss societies of dermatologists, general practitioners, paediatricians, paediatric dermatologists, paediatric infectious diseases specialists, and tropical medicine specialists, inviting clinicians to participate from 25th May to 8th August 2020. One reminder invitation was sent. Hospital pharmacies and the distributor of permethrin were contacted to report consumption trends of scabicides in 2018 and 2019. RESULTS: The survey was completed by 248 clinicians: 146 (59%) paediatricians, 47 (19%) dermatologists, 28 (11%) general practitioners, 6 (2%) paediatric dermatologists, 13 (5%) paediatric infectious diseases specialists, and 8 (3%) tropical medicine specialists. Most consulted up to 10 scabies cases within a 16-month period, with similar numbers in migrant and Swiss children. Dermoscopy was used by 24% of non-dermatologists. Non-dermatologists did not consider co-treatment of close contacts in up to 59% of cases. While permethrin was the first-line treatment, treatment failures were frequently reported in children aged <5 years. Up to 67% of paediatric dermatologists regularly used oral ivermectin off-label in children weighing <15 kg. None of the paediatric dermatologists, 15% of the dermatologists, and 9% of the non-dermatologists used only one treatment cycle.Scabicide consumption increased. Treatment studies on ivermectin use in children weighing <15 kg had the highest research priority. CONCLUSION: In Switzerland, scabies is a frequent dermatosis in migrant and Swiss children. While accessible, optimal diagnostics are underutilised, and treatment is suboptimal. Permethrin resistance appears to be an increasing problem. Dermatologists regularly use ivermectin off-label in children weighing <15 kg. Treatment studies on ivermectin use in children weighing <15 kg, user-friendly diagnostic tools, new treatment protocols, and child-friendly dosage forms are needed to improve the diagnosis and treatment of children with scabies.

Understanding efficacy-safety balance of biologics in moderate-to-severe pediatric psoriasis.

Background: Psoriasis is a chronic immune-mediated inflammatory skin disease affecting both adults and children. To better understand the efficacy-safety profile of biologics in children with moderate-to-severe psoriasis, this study aimed to analyze efficacy and safety data of randomized controlled trials (RCTs) performed in pediatric psoriasis and to compare efficacy outcomes in children with those in adults. Methods: RCTs investigating biologics in children with moderate-to-severe psoriasis were identified in a systematic literature review. PASI75/90 treatment responses at weeks 11/12 were analyzed comparing biologics with control arms. Serious adverse events (SAEs) were analyzed at the end of each study. Efficacy data from RCTs in adults with psoriasis were selected for the same biologics. Risk ratios (RR) of selected RCTs were pooled together in a statistical random effects model using the inverse variance method. Results: For children, there were 1 etanercept, 2 secukinumab, 1 ixekizumab and 1 ustekinumab placebo-controlled RCTs and 1 adalimumab RCT using methotrexate as reference arm at weeks 11/12. For adults, out of 263 RCTs, 7 adalimumab and 15 etanercept (TNF inhibitors) and 4 ixekizumab and 12 ustekinumab (IL-17 and IL-12/23 inhibitors) RCTs reported PASI75/90 efficacy responses at weeks 11/12. Regarding efficacy, all biologics showed improved PASI responses over control arms. RRs ranges were 2.02-7.45 in PASI75 and 4.10-14.50 in PASI90. The highest PASI75 responses were seen for ustekinumab 0.375 mg/kg (RR = 7.25, 95% CI 2.83-18.58) and ustekinumab 0.75 mg/kg (RR = 7.45, 95% CI 2.91-19.06) in the CADMUS study. The highest PASI90 response was seen for ixekizumab (RR = 14.50, 95% CI 4.82-43.58) in the IXORA-PEDS study. SAE incidences in pediatric and adult arms with biologics were 0 to 3% except for a pediatric arm with adalimumab 0.40 mg/kg (8%). For adults, pooled RR also showed improved PASI responses over placebo for all biologics, with highest PASI75 response observed for ixekizumab (pooled RR = 16.18, 95% CI 11.83-22.14). Conclusion: Both adults and children with psoriasis show superior efficacy with biologics compared to control arms after 3 months of treatment with SAE incidences in the low percentages. Additional longer-term clinical studies are warranted to fully understand the overall efficacy-safety profile of biologics in children with moderate-to-severe psoriasis.

PIK3CA Mutational Analysis in Patients With Macrodactyly.

BACKGROUND: Somatic mosaicism for PIK3CA mutations causes various types of growth disorders, which have been summarized under the term PROS (PIK3CA related overgrowth spectrum). Targeted therapy with PI3K inhibitors seems to be a promising alternative for severe PROS cases. Therefore, PIK3CA testing may become more relevant in the future. METHODS: We report on 14 PROS patients, who had surgery for macrodactyly in the majority of cases. Clinical data were retrieved from the patient's records. Macroscopic and microscopic findings were retrospectively reviewed. Mutational analysis was performed on formalin-fixed paraffin-embedded (FFPE) material. RESULTS: Patient age ranged from 7 months to 35 years. Five patients showed additional anomalies. One patient had CLOVES syndrome. The majority of the specimens were ray resections characterized by hypertrophic fat tissue. Overall, microscopy was subtle. The abnormal adipose tissue showed lobules exhibiting at least focally fibrous septa. In each case, we could detect a PIK3CA mutation. CONCLUSION: Histology of affected fat tissue in PROS patients is overall nonspecific. Therefore, mutational analysis represents the key to the diagnosis, especially in unclear clinical cases. We demonstrated that FFPE material is suitable for PIK3CA testing, which can be considered as basis for targeted therapy with PI3K inhibitors.

Phänotypisches und genetisches Spektrum von Incontinentia pigmenti - eine große Fallserie.

HINTERGRUND: Incontinentia pigmenti ist eine seltene X-chromosomal dominant vererbte Systemerkrankung, die vor allem die Haut, aber auch andere neuroektodermale Gewebe wie Zähne, Haare, Augen und das zentrale Nervensystem betrifft. PATIENTEN UND METHODIK: Diese multizentrische Fallserienstudie wurde an drei europäischen Hautkliniken durchgeführt und umfasste 30 Patienten mit Incontinentia pigmenti. Zwanzig Patienten wurden klinisch und genetisch untersucht, weitere zehn nur genetisch. ERGEBNISSE: Die Studie umfasste 28 Frauen und zwei Männer mit einem medianen Alter von drei Jahren. Kutane Manifestationen zeigten sich bei allen 20 Patienten mit klinischen Daten. Stadium I wurde in 90 % dieser Patienten beobachtet. Stadium IV wurde bereits im Alter von einem Jahr beobachtet. Zahn- (81 %), Haar- (78 %) und neurologische Anomalien (53 %) waren häufiger als in bisherigen Berichten. Vierzehn Hautbiopsien zeigten typische Merkmale des entsprechenden Stadiums. Genetische Tests wurden bei 24 Patienten durchgeführt, von denen 14 die häufige Exon 4-10-Deletion und sieben andere pathogene Varianten aufwiesen, darunter drei unveröffentlichte Mutationen. In drei weiteren Fällen wurden keine genetischen Veränderungen gefunden. SCHLUSSFOLGERUNGEN: In dieser Studie reichte der Phänotyp von lediglich subtil ausgeprägter Hautbeteiligung bis hin zu schweren Multisystemerkrankungen. Die extrakutane Beteiligung sollte zum Zeitpunkt der Diagnose und in regelmäßigen Abständen evaluiert werden, da sich einige Manifestationen erst mit der Zeit entwickeln. SUMMARY: Background and objectives Incontinentia pigmenti is a rare X-linked dominantly inherited systemic disease affecting primarily the skin but also other neuroectodermal tissues such as teeth, hair, eyes, and the central nervous system. Patients and methods This multicenter case series study was conducted at three European departments of Dermatology including 30 patients with incontinentia pigmenti. Twenty patients were evaluated clinically and genetically, another ten only genetically. Results The study included 28 females and two males with a median age of three years. Cutaneous manifestations were present in all 20 patients with clinical data. Stage I was observed in 90 % of those patients. Stage IV was observed as early as one year of age. Dental (81 %), hair (78 %) and neurological anomalies (53 %) were more frequent than previously reported. Fourteen skin biopsies showed typical features of the corresponding stage. Genetic testing of 24 patients revealed the common exon 4-10 deletion in 14 cases and seven other pathogenic variants, including three unpublished mutations. In another three cases, no genetic alterations were found. Conclusions In this study, the phenotype ranged from only subtle cutaneous involvement to severe multisystemic disorders. Extracutaneous involvement should be evaluated at the time of diagnosis and in regular intervals, as some manifestations may develop over time.

Phenotypic and genetic spectrum of incontinentia pigmenti - a large case series.

BACKGROUND AND OBJECTIVES: Incontinentia pigmenti is a rare X-linked dominantly inherited systemic disease affecting primarily the skin but also other neuroectodermal tissues such as teeth, hair, eyes, and the central nervous system. PATIENTS AND METHODS: This multicenter case series study was conducted at three European departments of Dermatology including 30 patients with incontinentia pigmenti. Twenty patients were evaluated clinically and genetically, another ten only genetically. RESULTS: The study included 28 females and two males with a median age of three years. Cutaneous manifestations were present in all 20 patients with clinical data. Stage I was observed in 90 % of those patients. Stage IV was observed as early as one year of age. Dental (81 %), hair (78 %) and neurological anomalies (53 %) were more frequent than previously reported. Fourteen skin biopsies showed typical features of the corresponding stage. Genetic testing of 24 patients revealed the common exon 4-10 deletion in 14 cases and seven other pathogenic variants, including three unpublished mutations. In another three cases, no genetic alterations were found. CONCLUSIONS: In this study, the phenotype ranged from only subtle cutaneous involvement to severe multisystemic disorders. Extracutaneous involvement should be evaluated at the time of diagnosis and in regular intervals, as some manifestations may develop over time.

Gianotti-Crosti Syndrom-ähnliche Reaktion bei Molluscum contagiosum -Klinische Charakteristika und Therapieansprechen.

HINTERGRUND UND ZIELE: Molluscum contagiosum (MC) ist eine häufige Virusinfektion der Haut. Bei gewissen Patienten mit MC kann eine Hypersensitivitätsreaktion ähnlich des Gianotti-Crosti-Syndroms beobachtet werden. Diese wird Gianotti-Crosti syndrome-like reaction (GCLR, Gianotti-Crosti-Syndrom-ähnliche Reaktion) genannt. Wir berichten über eine Kohorte von Patienten mit GCLR, um deren klinische Präsentation und Verlauf besser zu charakterisieren. PATIENTEN UND METHODIK: Retrospektive Studie mit Einschluss aller Kinder, welche sich zwischen 2015 und 2020 mit einer GCLR in unserem pädiatrischen Hautzentrum vorgestellt haben. RESULTATE: 26 Patienten (14 männlich) mit einem medianen Alter von 6.5 (3-11,3) Jahren wurden eingeschlossen. Die GCLR hat bei allen Patienten die Streckseiten der Extremitäten betroffen. Bei Kindern mit ausgedehntem Ausschlag waren bei 7 (27 %) auch der Stamm und bei 6 (23 %) auch das Gesicht mitbetroffen. Der Befall der Haut über der Achillessehne war ein auffälliges Phänomen bei 4 (15 %) Kindern. Juckreiz war das vorherrschende Symptom bei 20 (77 %) Patienten. Der Ausschlag hat gut auf die Behandlung mit topischen und/oder systemischen Kortikosteroiden angesprochen und ist innerhalb von 4 Wochen abgeklungen. Bei allen Patienten folgte innerhalb von 9 (4-24) Wochen nach der GCLR die Abheilung der MC. SCHLUSSFOLGERUNGEN: GCLR ist ein charakteristischer, akuter, ausgedehnter, juckender papulöser Ausschlag und führt häufig zu Notfallkonsultationen und Verunsicherung der betroffenen Patienten. Die GCLR spricht gut auf eine Behandlung mit Kortikosteroiden an, hat einen gutartigen Verlauf und geht der Abheilung der MC voraus.

Gianotti-Crosti syndrome-like reaction to molluscum contagiosum-Clinical characteristics and response to treatment.

BACKGROUND AND OBJECTIVES: Molluscum contagiosum (MC) is a common viral infection. Hypersensitivity reactions reminiscent of Gianotti-Crosti syndrome, termed Gianotti-Crosti syndrome-like reaction (GCLR), have been reported in a subset of patients. We report a series of patients with GCLR, better delineating its clinical presentation and course. PATIENTS AND METHODS: Retrospective chart review of all children presenting with GCLR at our Pediatric Skin Center between 2015 and 2020. RESULTS: 26 children (14 boys) with a median age of 6.5 (3-11.3) years were included. GCLR involved the extensor surfaces of the extremities in all patients. More widespread eruptions also affected the trunk and face in 7 (27 %) and 6 (23 %) children respectively. Involvement of the skin overlying the Achilles tendons was a new finding in 4 (15 %) children. Itch was the predominant symptom in 20 (77 %) patients. The rash responded to topical and/or systemic corticosteroids and resolved within four weeks. GCLR was followed by clearance of MC in all patients within 9 (4-24) weeks. CONCLUSIONS: GCLR is a characteristic acute, wide-spread, pruritic papular eruption, which often leads to emergency consultations and anxiety in affected patients. GCLR responds well to corticosteroid treatment, has a benign course, and heralds the healing of MC.

Sleep behavior of infants with infantile hemangioma treated with propranolol-a cohort study.

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

Sinus pericranii with facial involvement-A great mimicker.

Sinus pericranii is a rare vascular anomaly characterized by an abnormal communication between the intra- and extracranial venous systems through a calvarial defect(s). We present three cases of congenital sinus pericranii with facial involvement, emphasizing its cutaneous presentation with diagnostic pitfalls and discuss the multidisciplinary management of this vascular anomaly.

Mycoplasma pneumoniae Genotypes and Clinical Outcome in Children.

Factors leading to the wide range of manifestations associated with Mycoplasma pneumoniae infection are unclear. We investigated whether M. pneumoniae genotypes are associated with specific clinical outcomes. We compared M. pneumoniae loads and genotypes of children with mucocutaneous disease to those of children with pneumonia, family members with upper respiratory tract infection (URTI), and carriers from a prospective cohort study (n = 47; 2016 to 2017) and to those of other children with mucocutaneous disease from a case series (n = 7; 2017 to 2020). Genotyping was performed using macrolide resistance determination, P1 subtyping, multilocus variable-number tandem-repeat analysis (MLVA), and multilocus sequence typing (MLST). Comparisons were performed with a pairwise Wilcoxon rank sum test and a Fisher exact test with corrections for multiple testing, as appropriate. M. pneumoniae loads did not statistically differ between patients with mucocutaneous disease and those with pneumonia or carriers. Macrolide resistance was detected in 1 (1.9%) patient with mucocutaneous disease. MLVA types from 2016 to 2017 included 3-5-6-2 (n = 21 [46.7%]), 3-6-6-2 (n = 2 [4.4%]), 4-5-7-2 (n = 14 [31.1%]), and 4-5-7-3 (n = 8 [17.8%]), and they correlated with P1 subtypes and MLST types. MLVA types were not associated with specific outcomes such as mucocutaneous disease, pneumonia, URTI, or carriage. They were almost identical within families but varied over geographic location. MLVA types in patients with mucocutaneous disease differed between 2016 to 2017 (3-5-6-2, n = 5 [62.5%]) and 2017 to 2020 (4-5-7-2, n = 5 [71.4%]) (P = 0.02). Our results suggest that M. pneumoniae genotypes may not determine specific clinical outcomes.