Optimizing pediatric periprocedural pain management part I-Evolving ethics and topical anesthetics.

Pediatric procedure-related pain management is often incompletely understood, inadequately addressed, and critical in influencing a child's lifelong relationship with the larger health care community. We highlight the evolution of ethics and expectations around optimizing periprocedural pain management as a fundamental human right. We investigate the state-of-the-art of topical anesthetics, reviewing their mechanisms of action and providing comparisons of their relative safety and efficacy data to help guide clinical selection. In total, this two-part review offers a combination of conventional approaches and innovative techniques that should be used multimodally-in series and in parallel-to help optimize pain management and provide alternatives to sedation medication and general anesthesia.

Optimizing pediatric periprocedural pain management part II-Adjunct therapies to support the use of infiltrative anesthetics.

Pediatric procedure-related pain management is often incompletely understood, inadequately addressed, and critical in influencing a child's lifelong relationship with the larger healthcare community. We present a comprehensive review of infiltrative anesthetics, including a comparison of their mechanisms of action and relative safety and efficacy data to help guide clinical selection. We also describe the multimodal utilization of adjunct therapies-in series and in parallel-to support the optimization of pediatric periprocedural pain management, enhance the patient experience, and provide alternatives to sedation medication and general anesthesia.

Transition of care in adolescents with epidermolysis bullosa: The provider perspective.

The characteristics of epidermolysis bullosa (EB) demand higher than average provider support for transition from pediatric to adult care. We administered an online Qualtrics survey to members of the Epidermolysis Bullosa Clinical Research Consortium (EBCRC), a group of providers who care for patients with EB, in order to examine their practices and perspectives on transition of care (TOC) and identify barriers to successful implementation. Sixteen of eighteen medical centers completed the survey. Eighty-eight percent of center representatives expressed concerns about their patients transitioning/transferring from the pediatric to adult-centered care. Thirty-eight percent of providers reported having a formal TOC program in place. Our findings support the desire for formal TOC programs, the need for a team-based approach and, in particular, identification of adult providers to participate in the transition to improve this often challenging time.

Improving pediatric atopic dermatitis care in the emergency department and primary care setting: a collaborative quality improvement pilot project.

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.

Underrepresented Groups and Perceived Educational Barriers for Residency and Fellowship Success.

BACKGROUND: The study aimed to compare barriers perceived by medical students and resident physicians identifying as of underrepresented groups in medicine (UIM) and/or as sexual and gender minorities (SGM) to individuals not identifying with these groups, especially for trainees with an interest in dermatology. METHODS: Cross-sectional survey of medical students and resident physicians based in the United States from February 2021 to July 2021, with subgroup analysis of trainees with interest in dermatology. FINDINGS: Among trainees interested in dermatology, the most notable barriers for the UIM group were 1) lack of home program in specialty/fellowship of interest (4.71±1.73); 2) lack of connections/networking opportunities (4.14±1.29); 3) lack of opportunity to obtain AOA membership (4.00±1.96); 4) obtaining mentorship (4.00±1.47); and lack of diversity in specialty/fellowship of interest (3.93±1.14). CONCLUSIONS AND RELEVANCE: Increasing focused mentorship programs and fostering environments that embrace diversity are key to reducing perceived barriers for minority candidates. J Drugs Dermatol. 2023;22(12):1210-1215. doi:10.36849/JDD.7528R1.

Cutaneous collagenous vasculopathy in a pediatric patient.

We report the case of a 13-year-old female who presented with punctate, erythematous macules coalescing into patches on the upper extremities and left thigh. A skin biopsy demonstrated dilated capillary-sized blood vessels in the papillary dermis consistent with a diagnosis of cutaneous collagenous vasculopathy (CCV). To our knowledge, this is the youngest patient to present with CCV and will represent the third pediatric case in the literature.

Ichthyosis, petechiae, and arthrogryposis in a neonate.

Gaucher disease is a rare lysosomal storage disorder caused by a deficiency in glucocerebrosidase. This enzyme deficiency leads to the accumulation of toxic metabolites in various organs. Multiple subtypes of this disease have been described; however, the perinatal-lethal form is extremely rare and challenging to diagnose. We present a case of a newborn girl with ichthyosis, petechiae, and arthrogryposis, later found to be homozygous for a pathogenic variant of the glucocerebrosidase gene. This case highlights the potential role of dermatologists in the recognition of this rare disease.

Virtual reality and noise canceling headphone distraction during pediatric dermatologic procedures.

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.

Assessing pain catastrophizing and functional disability in pediatric epidermolysis bullosa patients.

BACKGROUND/OBJECTIVES: The primary objective was to assess pain catastrophizing and functional disability in pediatric patients with epidermolysis bullosa (EB) and their parents/guardians. Secondary objectives included examining relationships between pain catastrophizing, functional disability, and correlations with other factors (e.g., age, disease severity, and percent of body surface area (BSA) involved). METHODS: Patients with EB ages 8-16 and their parents/guardians who were English or Spanish speaking completed a one-time online survey. Parent measures included: demographics questionnaire, Pain Catastrophizing Scale-Parent (PCS), and Parent Functional Disability Inventory (FDI). Child measures included: PCS child and child FDI. Higher scores on both scales indicate higher levels of catastrophizing and functional disability. RESULTS: Of 31 children, the mean age was 11.47 years and the majority (70.97%) had dystrophic EB. Mean scores were: 35.84 = PCS parent; 34.58 = PCS child; 30.87 = parent FDI; 29.77 = child FDI. Total scores for PCS parent, parent FDI, and child FDI increased significantly with disease severity and percentage of involved BSA (p < .01 for all). Total scores for PCS child increased significantly with percent of EB skin involvement (p = .04) but not disease severity. Older children reported more functional disability than their parents and younger children (p = .02). CONCLUSIONS: Our results demonstrate significant positive correlations between negative thoughts related to pain and the experience of functional difficulties in patients with EB and their caregivers. Psychological, psychiatric, and/or behavioral interventions to help managing chronic pain may be effective for patients with EB.

Surgical deroofing in pediatric patients with hidradenitis suppurativa.

Hidradenitis suppurativa (HS) is a chronic, inflammatory follicular disorder that most commonly involves the intertriginous areas. It is characterized by recurrent nodules that may progress into deeper abscesses and sinus tracts. Treatment is challenging and often involves a combination of lifestyle modifications, medical therapies, and procedures to control symptoms. Deroofing is a tissue-saving surgical technique that has been well studied in adults and in our clinical experience is an important adjunct therapy in pediatric patients with moderate-to-severe HS. We describe the step-by-step process of deroofing, including surgical clinical pearls specific to the pediatric population.

Pediatric BehÒ«et disease: Update in diagnosis and management.

BehÒ«et disease is a multisystem inflammatory disease and variable vessel vasculitis involving primarily the oral and genital mucosa, skin, and eyes. Diagnosis is challenging due to the lack of a specific diagnostic test and overlap with other autoinflammatory diseases. Treatment of pediatric BehÒ«et disease aims to reduce inflammation and prevent future flares. The goal of this review is to provide guidance on the diagnostic workup and multidisciplinary approach of pediatric BehÒ«et disease and review evidence-based treatment strategies for patients with refractory mucocutaneous manifestations.

A case of prolidase deficiency in a male patient.

Prolidase deficiency is an extremely rare, autosomal recessive disorder resulting in defective collagen formation. We report a case of prolidase deficiency in a male child, highlighting the dermatologic features. Early diagnosis is important as these patients encounter significant multisystem comorbidities requiring multispecialty care.

Reducing anxiety and preventing trauma in pediatric epidermolysis bullosa.

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.

Validation of remote atopic dermatitis severity assessment with the Eczema Area and Severity Ondex in children using caregiver-provided photos and videos.

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.

Superimposed linear atopic dermatitis treated with dupilumab.

Common polygenic skin disorders, such as atopic dermatitis, may rarely present in a segmental or linear distribution due to cutaneous mosaicism. Only seven cases of superimposed linear atopic dermatitis have been reported to date. Here, we present a child with severe superimposed linear atopic dermatitis and highlight the first successful use of dupilumab in its treatment.

Caring for Pediatric Hidradenitis Suppurativa Patients in the Emergency Department.

ABSTRACT: Hidradenitis suppurativa (HS) is a chronic and recurrent inflammatory skin condition resulting in the formation of nodules, sinus tracts, and abscesses in intertriginous areas. We provide recommendations for the management of children presenting to the emergency department with acute HS flares, based on a review of literature and insights from our own clinical experience. The purpose of the recommendations is to educate clinicians on specific considerations that should be made when caring for children with HS.

Off-label use of dupilumab for pediatric patients with atopic dermatitis: A multicenter retrospective review.

BACKGROUND: Atopic dermatitis (AD) is a common, chronic type 2 inflammatory skin disease, typically starting in infancy, with increased risk for subsequent extracutaneous atopic morbidities. Dupilumab is the first biologic agent targeting type 2 inflammation approved by the U.S. Food and Drug Administration (USFDA); it was licensed in 2017 for adults with moderate to severe AD and 2 years later for adolescents. Systemic treatment for pediatric AD remains a significant unmet medical need. OBJECTIVE: To analyze off-label use of dupilumab in children with AD. METHODS: Multicenter retrospective review that evaluated children who were prescribed dupilumab for moderate to severe AD. RESULTS: One hundred eleven of 124 patients (89.5%) gained access to dupilumab after a mean of 9 weeks. The dosing range was 4 to 15.5 mg/kg for the loading dose and 2.0 to 15.3 mg/kg every other week for maintenance. The range was widest for 6- to 11-year-olds and was related to use of either full or half of adult dosing. Associated morbidities, treatment response, and adverse events were comparable to those in previous adolescent and adult trials. LIMITATIONS: The retrospective design of the study limited uniform data collection. CONCLUSION: Access to dupilumab was achievable for the majority of children after a mean 9-week delay because of insurance payment denial. This review supports dupilumab response and tolerability in children. Optimal dosing for patients younger than 12 years has not been defined. Availability of the drug in 2 different concentrations is an important safety issue.

Pediatric trichotillomania: Review of management.

Trichotillomania can present in childhood, with many families seeking initial evaluation by a dermatologist for hair loss. Prompt and accurate diagnosis by dermatologists is crucial, as children can suffer from academic or social impairments as well as mental health sequelae. Children are especially vulnerable to lasting psychological distress from appearance-related bullying. This article reviews the psychosocial impacts of pediatric trichotillomania and the current interventions studied in this population. Included are studies evaluating behavioral therapies as well as pharmacologic options. This review highlights the importance of early and appropriate identification, intervention, and the need for more treatment studies in the pediatric population.

Use of psychologic strategies to reduce pain and anxiety related to dermatology procedures.

The fear of painful procedures is at an increased incidence within the pediatric population. This can be an extremely challenging obstacle in dermatology where injections, blood draws, and cryotherapy are often required during the care of patients. Psychologic techniques based on cognitive behavioral therapy (CBT) can be implemented leading up to and while performing these procedures to help reduce patient anxiety.

Management of a neonate with diffuse cutaneous mastocytosis: Case report and literature review.

Mastocytosis is an accumulation of clonal mast cells within tissues, commonly caused by mutations in the KIT proto-oncogene. This report describes the management of a neonate with diffuse cutaneous mastocytosis (DCM) caused by a rare activating KIT mutation, specifically internal tandem duplication of the Ala502Tyr503 pair on exon 9, and reviews current data regarding work-up of DCM in pediatric patients.