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.

Prevalence and Prognostic Role of Lymphedema in Patients with Deep Venous Thrombosis and Thrombophlebitis.

Background: Deep venous thrombosis (DVT) poses a substantial disease burden. Lymphedema may present with similar symptoms making the diagnosis process more difficult. Data on the epidemiology of lymphedema are lacking. Methods: The German nationwide inpatient sample served to analyze all patients hospitalized owing to DVT and/or thrombophlebitis (referred to as DVT) of the legs in Germany between 2005 and 2020. We stratified these patients for additional lymphedema and analyzed the impact of lymphedema on adverse in-hospital events. Results: Overall, 1,136,574 hospitalizations related to DVT were recorded in Germany during 2005-2020 (53.3% women; 51.3% aged ≥70 years). Lymphedema was coded in 9974 (0.9%) patient-cases (82.0% not elsewhere classified, 17.7% secondary lymphedema). Annual numbers of hospitalizations with lymphedema among DVT patients increased from 450 (2005) to 613 (2016) (ß 0.57; 95% confidence interval [CI]: 0.48-0.66], p < 0.001) and decreased thereafter. Despite similar age, DVT patients with lymphedema had higher prevalence of cardiovascular diseases, chronic organ failure, and all types of investigated cancer. Prevalence of pulmonary embolism (PE) with shock/CPR (4.1% vs. 1.5%), acute renal failure (6.7% vs. 2.5%), and stroke (5.2% vs. 4.2%) was higher in DVT patients with lymphedema than without. Lymphedema was independently associated with PE with shock/CPR (OR: 2.1; 95% CI: 1.9-2.3) as well as death (OR: 1.3; 95% CI: 1.2-1.4). Conclusions: Comorbidity conditions like cancer, obesity, and cardiovascular risk factors, and also infectious complications, were more prevalent in DVT patients with lymphedema than in those without. Lymphedema was independently associated with severe in-hospital complications, particularly when its genesis was related to severe comorbidities.

Zinc Oxide Patches Are a Highly Effective Treatment for Chronic Prurigo: A Randomized Split-Body Study.

BACKGROUND: Chronic prurigo (CPG) presents with pruriginous lesions and reduced quality of life (QoL). Established treatment options are often unsatisfying. Little is known about the efficacy of topical occlusive treatments. Patients often report rapid relief of symptoms when using topical occlusive zinc oxide patches (ZOP). We, therefore, aimed to assess the efficacy of ZOP. METHODS: In this randomized controlled split-body crossover study, 22 participants were analyzed, receiving three treatments sequentially: ZOP, topical betamethasone 17-valerate (topical glucocorticosteroids [TGCs]), and both ZOP and TGC combined (ZOP + TGC). Each intervention was applied to either the right or left side of the body for seven consecutive days. Outcomes were a count of active excoriated pruriginous lesions (APLs), itch, recurrence of APL, QoL, and treatment comfort. They were assessed through photographs and questionnaires: modified Prurigo Activity and Severity Score, modified Itchy Quality of Life Questionnaire, and Therapy Comfort Score. RESULTS: We observed a significant reduction of 46% in APL count for ZOP (95% CI from 30% to 58%, p value: <0.0001). Similar reduction was seen for ZOP + TGC, and a lower reduction was seen for TGC alone (48% [95% CI from 33% to 60%, p value: <0.0001] vs. 26% [95% CI from 4% to 43%, p value: 0.02]). APL counts on the non-treated side remained stable. Significant reduction in itch was observed after all treatments, with the largest improvement for ZOP + TGC, followed by TGC and, lastly, ZOP alone (-2.3 units [95% CI from -3.5 to -1.1, p value: 0.00015] vs. -1.5 units [95% CI from -2.8 to -0.3, p value: 0.01 vs. -1.4 units [95% CI from -2.6 to -0.2, p value: 0.02]). QoL increased significantly after ZOP + TGC as well as after TGC (-8.3 units [95% CI from -13.6 to -3.1, p value: 0.0018] vs. -5.7 units [95% CI from -10.9 to -0.5, p value: 0.03]). A good subjective response concerning treatment comfort was observed. CONCLUSION: ZOP are effective in reducing APL after 1 week of treatment. Adding TGC to ZOP did not add considerable benefit in reducing APL. All three treatments reduced itch and improved QoL, with the largest improvement shown by ZOP combined with TGC. Patients tolerated ZOP well and reported no adverse events. We therefore suggest ZOP combined with TGC as an effective, fast-acting, low-cost treatment for reducing APL and itch in patients with CPG.

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.

A very mild phenotype in six individuals of a three-generation family with the novel HRAS variant c.176C > G p.(Ala59Gly): Emergence of a new HRAS-related RASopathy distinct from Costello syndrome.

Costello syndrome is a clinically recognizable, severe neurodevelopmental disorder caused by heterozygous activating variants in HRAS. The vast majority of affected patients share recurring variants affecting HRAS codons 12 and 13 and a relatively uniform phenotype. Here, we report the unique and attenuated phenotype of six individuals of an extended family affected by the HRAS variant c.176C>T p.(Ala59Gly), which, to our knowledge, has never been reported as a germline variant in patients so far. HRAS Alanine 59 has been previously functionally investigated as an oncogenic hotspot and the p.Ala59Gly substitution was shown to impair intrinsic GTP hydrolysis. All six individuals we report share a phenotype of ectodermal anomalies and mild features suggestive of a RASopathy, reminiscent of patients with Noonan syndrome-like disorder with loose anagen hair. All six are of normal intelligence, none have a history of failure to thrive or malignancy, and they have no known cardiac or neurologic pathologies. Our report adds to the previous reports of patients with rare variants affecting amino acids located in the SWITCH II/G3 region of HRAS and suggests a consistent, attenuated phenotype distinct from classical Costello syndrome. We propose the definition of a new distinct HRAS-related RASopathy for patients carrying HRAS variants affecting codons 58, 59, 60.

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.

Infliximab for the treatment of recalcitrant bullous Sweet syndrome in a 10-year-old girl.

We report the case of a 10-year-old girl with bullous Sweet syndrome, recalcitrant to high-dose systemic corticosteroids. Subsequent treatment with infliximab resulted in a rapid improvement in cutaneous lesions and systemic symptoms. Cutis laxa was noted in the healed skin. We propose early second-line treatment with infliximab in children with steroid-refractory Sweet syndrome.

Secukinumab Therapy for Netherton Syndrome.

Importance: Netherton syndrome (NS) is a rare, severe genetic disorder of cornification with high morbidity. Treatment for NS has been notoriously difficult. Recent studies showed an upregulated helper T cell (TH) 17/interleukin 23 (IL-23) pathway in NS, suggesting the possibility of treatment strategies that target IL-17. Objective: To evaluate the clinical response of NS to treatment with the IL-17 antagonist secukinumab. Design, Setting, and Participants: This case series study reports the experience of compassionate use therapy with secukinumab in 4 patients with severe NS, including 2 children, from December 1, 2018, to December 1, 2019, with 3 patients still undergoing treatment at the time of final analysis. Data were analyzed from December 1, 2018, to December 1, 2019. Main Outcomes and Measures: Expression of IL-17 in the skin was evaluated by immunohistochemical analysis, and serum cytokine concentrations were measured using a commercially available assay. Treatment response was assessed using the Ichthyosis Area and Severity Index (IASI) total score, including measures of erythema and scaling, the Dermatology Life Quality Index (DLQI), and the 5-D itch scale. Results: In all 4 patients (age range, 9-27 years; 3 male and 1 female), immunostaining with an IL-17A antibody showed an increased number of positive cells in lesional skin. Cytokine assessment in serum samples revealed increased levels of CCL20. Treatment duration with secukinumab was 3 to 12 months at the time of this report. After 3 months of therapy, IASI scores were reduced by 44% to 88%, DLQI scores were reduced by 40% to 76%, and 5-D itch scale scores were reduced by 27% to 62%. This outcome was sustained at the 6-month follow-up. Two patients with an erythrodermic phenotype showed marked improvement of all parameters. A refractory palmoplantar eczematous eruption occurred in 2 patients, and a candidal nail infection developed in 2 patients. No severe adverse events were reported. Conclusions and Relevance: This initial case series reporting the use of anti-IL-17 therapy in NS demonstrated marked cutaneous improvement, particularly in 2 pediatric patients with erythrodermic phenotypes. Further studies are needed to evaluate the long-term benefit of this potential treatment modality.

Congenital hemangioma exhibiting postnatal growth.

Congenital hemangiomas are vascular tumors that are fully formed at birth, typically without postnatal growth. Noninvoluting congenital hemangiomas (NICH) have a distinctive clinical, radiologic, and histopathological profile and lack of expansion or involution over time. Herein, we describe two cases of NICH with atypical postnatal growth.

Self-healing juvenile cutaneous mucinosis: Clinical and histopathologic findings of 9 patients: The relevance of long-term follow-up.

BACKGROUND: Self-healing juvenile cutaneous mucinosis (SHJCM) is a rare disorder, and its pathogenesis and long-term prognosis are unknown. OBJECTIVE: To elucidate the clinical and histopathologic characteristics, pathogenesis, and outcome in patients with SHJCM. METHODS: Retrospective study of 9 patients with SHCJM. To complement initial findings, data collection forms were sent to the referring physicians. RESULTS: All patients had an acute onset of firm nodules. Of the 9 patients, 6 presented initially with waxy papules on the dorsum of the hands; 5 suffered from periorbital edema, and 6 had a febrile prodrome. Histopathologic assessment of the papules revealed dermal mucin deposition, whereas the nodules showed proliferative fasciitis-like features or nonspecific chronic lobular panniculitis. Laboratory studies elicited evidence of active viral infection in 2 patients (human herpes virus 6 and rotavirus). Seven cases had spontaneous resolution within 6 months, and 2 patients with incomplete resolution showed subsequent transition to fibroblastic rheumatism and an autoinflammatory rheumatologic disease, respectively. LIMITATIONS: This was a retrospective study with incomplete data from referring physicians. CONCLUSIONS: Although spontaneous complete regression is expected, patients with SHJCM need long-term follow-up because of the possible development of dematorheumatolgic conditions. The pathogenetic role of microbial agents deserves further investigation.

Tinea genitalis: a new entity of sexually transmitted infection? Case series and review of the literature.

OBJECTIVE: Investigation on recent cases of tinea genitalis after travelling to South East Asia. METHODS: Patients with tinea in the genital region, which emerged after sex in South East Asia, underwent further assessment including microscopy, cultures and DNA analyses. RESULTS: The case series includes seven patients. In six patients, Trichophyton interdigitale (former Trichophyton mentagrophytes) was detected. Three patients suffered from a severe inflammatory reaction of the soft tissue and two of them needed hospitalisation due to severe pain. In four patients, cicatrising healing was noticed. Five patients were declared incapacitated for work. CONCLUSIONS: Sexual activity should be considered as a potentially important and previously underappreciated means of transmission of T. interdigitale. To avoid irreversible scarring alopecia, prompt initiation of antifungal treatment is essential and adequate isolation and identification of the pathogen is mandatory.