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Emergency department visits and healthcare expenditures for pediatric atopic dermatitis have been increasing over the last two decades. There is a paucity of replicable quality improvement initiatives addressed at educating primary care and emergency medicine clinicians on this condition. The goal of this initiative was to improve clinician knowledge and comfort in the diagnosis and management of pediatric atopic dermatitis and superinfection. Clinicians were recruited via email from academic and community settings in Travis County, Texas, in 2020. They were sent a pre-intervention survey, a series of three quizzes, and a post-intervention survey. After each quiz, participants received performance feedback and various forms of multimodal education. Differences between the first and final quiz scores and clinician confidence levels were analyzed for statistical significance. Fifty-six clinicians completed the intervention. The average overall and treatment-specific scores increased significantly by 10% and 37%, respectively. Further, confidence levels improved significantly in the majority of clinicians. Clinician qualitative feedback revealed high satisfaction. Results from this educational quality improvement project have demonstrated that this is an effective and replicable resource for educating clinicians who manage pediatric atopic dermatitis in the emergency department and outpatient setting. [ABSTRACT FROM AUTHOR]

Background: Ichthyoses are a heterogeneous group of skin disorders characterized by scaling and erythema. Recognizing the variability of scale and erythema by region and ichthyosis subtype, we developed the Ichthyosis Scoring System (ISS) to quantify severity. We previously found ISS to have high inter‐ and intrarater reliability in evaluating photographic images. To confirm ISS clinical utility, we examined its performance at the 2022 Foundation for Ichthyosis and Related Skin Types conference. Methods: Sixty‐five participants were evaluated by 3 of 9 medical professionals trained to score ichthyosis scale and erythema using ISS. Intrarater and interrater intraclass correlation coefficients (ICC) were analyzed using one‐way and two‐way random effects models, respectively. Results: Intrarater reliability was excellent (ICC = 0.931, 95% CI, 0.921–0.940) for scale and good (ICC = 0.876, 95% CI, 0.853–0.899) for erythema scoring. Compared to photo validation with excellent intrarater reliability ratings for both scale (ICC = 0.956, 95% CI, 0.925–0.974) and erythema (ICC = 0.913, 95% CI, 0.855–0.949), ISS demonstrated equivalent reliability for live use. Overall interrater reliability for 10 body sites showed excellent (ICC >0.9) and good (ICC >0.75) agreement and consistency for both scale and erythema. Palms were an exception, demonstrating moderate (ICC >0.5) interrater agreement and consistency for erythema evaluation. Conclusions: ISS is a reliable measure of global and regional ichthyosis severity during in‐person evaluations. Ease‐of‐use, accessibility, and content validity in both live and photographic evaluation endorse ISS as a standard for ichthyosis severity analysis. [ABSTRACT FROM AUTHOR]

IgA vasculitis (IgAV), formerly known as Henoch–Scholein purpura, is a small vessel vasculitis, most commonly seen in pediatric patients, that can affect numerous internal organs including the kidneys, lungs, gastrointestinal tract, and the central nervous system (CNS). CNS manifestations of this condition include hypertensive encephalopathy, thrombosis, optic neuropathy, seizures, CNS vasculitis, and a more recently described phenomenon known as posterior reversible encephalopathy syndrome (PRES). Symptoms of PRES include hypertension, altered mental status, and seizures caused by vasogenic disruption of the blood–brain barrier, and the condition is diagnosed by characteristic edema‐related gray–white matter changes in the parieto‐occipital lobes on magnetic resonance imaging. Herein, we present a rare case of PRES as a presenting sign of IgAV to increase awareness about this unusual association. [ABSTRACT FROM AUTHOR]

We present the case of a 20‐month‐old girl with Schimmelpenning‐Feuerstein‐Mims (SFM) syndrome with extensive head, neck, and torso skin involvement successfully managed with topical trametinib. Trametinib interferes downstream of KRAS and HRAS in the MAPK signaling pathway, of which KRAS was implicated in our child's pathogenic variant. Although other dermatologic conditions have shown benefit from oral trametinib, its topical use has not been well reported. Our patient showed benefit from the use of twice‐daily topical trametinib, applied to the epidermal and sebaceous nevi over a 16‐month period, leading to decreased pruritus and thinning of the plaques. [ABSTRACT FROM AUTHOR]

Pain and anxiety related to medical procedures have long been recognized as a significant healthcare concern. If a patient's procedural pain and anxiety are not addressed, long‐term physical and psychological sequelae including increased perceived pain, anxiety, disruptive behavior, trauma reactions, or refusal of future procedures can occur. The objective of our study was to assess the utility of a virtual reality (VR) headset or noise‐canceling headphones (HP) compared to treatment as usual (TAU) in reducing pain and anxiety during pediatric dermatology procedures. Results indicated a significant difference between pre‐ versus post‐procedure anxiety in the VR and HP groups but not the TAU group suggesting non‐pharmacologic technology‐based interventions such as VR and headphones may reduce patients' anxiety during pediatric dermatology procedures. [ABSTRACT FROM AUTHOR]

Background/Objectives: Gastrostomy tubes (G‐tubes) are a feasible intervention for nutritional support in patients with epidermolysis bullosa (EB) who suffer from gastrointestinal involvement. However, some parents may be apprehensive about this surgical intervention. The purpose of this study is to assess caregiver satisfaction and experiences after G‐tube insertion in children with EB. Methods: Nine institutions sent an anonymous 89‐item online questionnaire to caregivers of patients with EB and active G‐tubes during an 8‐week study period. The questionnaire consisted of eight sections and was formatted with multiple choice, Likert scales, dichotomous scales (yes/no), and free text. Results: Twenty caregiver surveys were completed. All caregivers were parents (100%). The average age of G‐tube placement was 2.7 years (SD: 2.1, range 0–8) and duration of the G‐tube utilization was an average of 12.0 years (SD: 7.8, range 1–25). EB subtypes were dystrophic (n = 15/20, 75%), junctional (n = 3/20, 15%), and simplex (n = 2/10, 10%). Almost all caregivers (n = 18/20, 90%) reported high levels of satisfaction with G‐tube management (scores > 20 on the modified SAGA‐8 scale). More than half of caregivers (n = 12/20, 60%) reported that, retrospectively, they would have desired earlier adoption of the G‐tube. Caregivers rated mealtimes as more enjoyable and easier both for themselves and the individual with EB post‐G‐tube placement. Conclusion: The survey demonstrates a high rate of satisfaction for G‐tube use among caregivers of patients with EB across varying ages and household settings. The free‐text responses provide unique insight into caregiver's individual experiences regarding G‐tubes to be considered for future studies. [ABSTRACT FROM AUTHOR]

The most common bacteria isolated from wound cultures in patients recorded in the Epidermolysis Bullosa Clinical Characterization and Outcomes Database (EBCCOD) are Staphylococcus aureus and Pseudomonas aeruginosa. Given the prevalence of P. aeruginosa in this patient population and prior research implicating P. aeruginosa's potential role in carcinogenesis, we sought to further analyze patients with recorded wound cultures positive for Pseudomonas aeruginosa in the EBCCOD. We provide a descriptive analysis of this subset of patients and highlight potential avenues for future longitudinal studies that may have significant implications in our wound care management for patients with epidermolysis bullosa. [ABSTRACT FROM AUTHOR]

Background/Objectives: We sought to quantify the reliability and validity of remote atopic dermatitis (AD) severity assessment using the Eczema Area and Severity Index (EASI) applied to caregiver‐provided photos (p‐EASI) and videos (v‐EASI). Methods: Children (0–17 years) with a physician diagnosis of AD were recruited. Caregivers took photos and a video of their child's skin. A clinician scored in‐person EASI on the same day, then p‐EASI and v‐EASI for each participant 10 days or more between ratings. Two additional clinicians scored p‐EASI and v‐EASI. Lin's concordance correlation coefficient (CCC) was employed to assess criterion validity using in‐person EASI as the gold standard. Intraclass correlation coefficients (ICCs) were calculated to assess interrater reliability of p‐EASI and v‐EASI. Results: Fifty racially and ethnically diverse children (age [mean ± SD]: 4.3 ± 4.4 years; 42% female) with a range of AD severity (EASI: 6.3 ± 6.4) and Fitzpatrick skin types (1–2: 9%; 3–4: 60%; 5–6: 31%) were enrolled and received in‐person EASI assessment. Fifty had p‐EASI and 49 had v‐EASI by the same in‐person rater, and by two additional raters. The CCC and ICC for p‐EASI were 0.89, 95% CI [0.83, 0.95] and 0.81, 95% CI [0.71, 0.89], respectively. The CCC and ICC for v‐EASI were 0.75, 95% CI [0.63, 0.88] and 0.69, 95% CI [0.51, 0.81], respectively. Conclusions: In this diverse population with a range of skin tones, p‐EASI showed good criterion validity and good interrater reliability. v‐EASI showed moderate to good criterion validity and moderate interrater reliability. Both may be reliable and valid options for remote AD severity assessment. [ABSTRACT FROM AUTHOR]

Background: Accurate diagnosis of epidermolysis bullosa (EB) has significant implications for prognosis, management, and genetic counseling.Objective: To describe diagnostic testing patterns and assess diagnostic concordance of transmission electron microscopy (TEM), immunofluorescence mapping (IFM), and genetic analysis for EB.Methods: A retrospective cohort included patients enrolled in the Epidermolysis Bullosa Clinical Characterization and Outcomes Database from January 1, 2004, to July 8, 2019. Tests concluding the same EB type (EB simplex, junctional EB, dominant dystrophic EB, and recessive dystrophic EB) were considered concordant; those concluding different EB types were considered discordant; and those with nonspecific/nondefinitive results were equivocal.Results: A total of 970 diagnostic tests were conducted from 1984 to 2018 in 771 patients. Genetic analyses were performed chronologically later than IFM or TEM (P < .001). The likelihood of undergoing genetic analysis was greater for junctional EB and recessive dystrophic EB, and the same for dominant dystrophic EB as compared with EB simplex. TEM results in 163 patients were equivocal (55%), concordant (42%), and discordant (3%). IFM results in 185 patients were equivocal (54%), concordant (42%), and discordant (4%).Limitations: Retrospective design.Conclusions: Diagnostic testing has shifted in favor of genetic analysis. TEM and IFM frequently offer equivocal findings when compared to the specificity afforded by genetic analysis. [ABSTRACT FROM AUTHOR]

Although dermatologists are well‐trained in the medical management of complex skin disease, psychosocial care often exceeds a dermatologist's skillset. We aim to elucidate major factors to consider in the comprehensive management of pediatric epidermolysis bullosa (EB) and provide care recommendations. There are many types of trauma a child with EB may experience, from social to psychological to medical. We include information on trauma‐informed care and advice for the dermatologist and multidisciplinary team regarding patient‐centered and family‐centered approaches to recognizing and reducing anxiety and trauma in EB patients. [ABSTRACT FROM AUTHOR]

Stevens-Johnson syndrome (SJS) an toxic epidermal necrolysis are the most severe cutaneous reactions that occur in children. Off-label use of human intravenous immunoglobulin (IVIG) has been reported in a number of autoimmune and cell-mediated blistering disorders of the skin, including severe cutaneous drug reactions. We review 28 previous reports in which IVIG was used in pediatric patients with SJS and toxic epidermal necrolysis and discuss our experience in 7 children with SJS, in whom no new blisters developed within 24 to 48 hours after IVIG administration and rapid recovery ensued. IVIG seems to be a useful and safe therapy for children with severe cutaneous drug reactions. Well-controlled, prospective, mulficenter clinical trials are needed to determine optimal dosing guidelines and to compare the efficacy and safety of IVIG with other potentially effective modalities. Pediatrics 2003;112:1430-1436; Stevens-Johnson syndrome, toxic epidermal necrolysis, intravenous immunoglobulin, IVIG., Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are the most severe cutaneous reactions that occur in pediatrics. Drugs are the predominant inciting agent in both entities. The combined incidence [...]

Invasive fungal dermatitis may be a new complication caused primarily by Candida albicans among very small premature babies. Some newborns under 1,000 g develop whitish crusty patches on the surface of their bodies several days after birth. This new type of disease may enter the infant body via the underdeveloped skin barrier and cause extensive fungal infection throughout. A group of 16 premature infants with an average birth weight of 635 g generated skin lesions after 6 to 14 days. Eleven infant infections were due to Candida albicans, a fungus often found in the vagina. It is thought that vaginal delivery increases the risk of contracting this fungus. More infants with fungal dermatitis were delivered vaginally, received steroids after birth, and had higher blood sugar levels than 48 control infants. Seven of the infants with fungal dermatitis did not survive., Objective. In 1991, we noted the emergence amongst our extremely low birth weight neonates of a new clinical entity, invasive fungal dermatitis, characterized by erosive, crusting lesions and a high rate of subsequent systemic fungal infection. We sought to define this condition and examine potential risk factors. Methods. Sixteen neonates with invasive fungal dermatitis were seen during a 2-year period in three Baylor College of Medicine affiliated intensive care nurseries. Seven were confirmed cases, with skin biopsy evidence of invasion beyond the stratum corneum. Nine had a consistent critical course and a positive potassium hydroxide examination of skin scrapings or isolation of fungi from skin or systemic cultures. Three controls were matched to each case by hospital, date of admission, and, birth weight. Data was collected by retrospective chart; review. Results. Invasive fungal dermatitis occurred in 5.9% of at-risk infants. Case patients had a mean birth weight of 635 g and developed skin lesions at a mean age of 9 days (range, 6 to 14). Candida albicans was the most commonly implicated pathogen, but other Candida species, Aspeigillus, Trichosporon beigelii, and Curvularia were also seen. Disseminated infection occurred in 69%, all due to Candida sp. Case patients were significantly more premature than controls (mean gestation, 24.4 vs 25.9 weeks) and were more likely to be delivered vaginally (81% vs 50%). Postnatal steroids were administered to cases (81%) more often than controls (46%). Case patients had more prolonged hyperglycemia (as assessed by insulin administration) than controls (mean 4.3 vs 2.0 days). Conclusions. Invasive fungal dermatitis is a disease of the smallest, most inimature neonates and. is associated with vaginal birth, steroid administration, and hyperglycemia. We speculate that the skin serves as a portal of entry for colonizing fungal species and may thus lead to disseminated infection. Methods to improve skin barrier function may be useful in preventing this disorder. Pediatrics 1995; 95-682-687; invasive fungal dermatitis, lesions, neonates, skin, Candida, Aspergillus, Tymsporon, Curvularia., ABBREVIATION. KOH, potassium hydroxide. As the survival of infants with birth weights [less than or equal to]1500 g has improved, a new spectrum of infectious agents associated with these susceptible [...]

Chickenpox is one of the more common childhood diseases. It is caused by the varicella-zoster virus. About 3.5 million cases of chickenpox are thought to occur in the US each year; about 4,500 patients require hospitalization. Complications are not common, but chickenpox may lead to secondary bacterial infection of the skin, inflammation of the lungs, cerebellar ataxia (an abnormality of gait resulting from effects on the cerebellum), and encephalitis. The death rate for otherwise healthy children with chickenpox is about 1 in 50,000. The antiviral drug acyclovir has been used successfully to treat varicella infections in normal adults and immunocompromized patients. A study has now been conducted to evaluate the effectiveness of acyclovir in treating normal children with chickenpox. A total of 815 children with chickenpox were randomly assigned to receive treatment with acyclovir or placebo within the first 24 hours of the appearance of the symptomatic "pocks". The children treated with acyclovir had fewer lesions than the children treated with placebo, with an average of 294 versus 347 lesions, respectively. Furthermore, over 95 percent of the children treated with acyclovir stopped developing new skin lesions by the fourth day of infection. In contrast, one-fifth of the placebo-treated children continued to develop new lesions after day five. Four children in the acyclovir group and six in the placebo group developed bacterial infections as a complication of chicken pox. One case of cerebellar ataxia was noted in the placebo group. The incidence of complications was too small, however, to draw any conclusions. Although the results clearly show that acyclovir reduces the severity and the duration of chickenpox, it remains uncertain if the rate of complications is also decreased by acyclovir treatment. (Consumer Summary produced by Reliance Medical Information, Inc.)