Proceedings of the 10th Pediatric Dermatology Research Alliance (PeDRA) Annual Conference.

The 10th Pediatric Dermatology Research Alliance (PeDRA) Annual Conference occurred November 3-5, 2022 in Bethesda, Maryland. This conference was the first in-person PeDRA conference after 2 years of a virtual format due to COVID-19. Fittingly, given the effects of the pandemic, the conference theme was "Reimagining Community." The conference included presentations and panel sessions on finding individual and collective purpose, leveraging community in pursuit of a shared goal, and creating a community of resources in collaboration with NIH. The goal of this meeting was to connect clinicians, basic scientists, patients, patient advocates, and industry partners. The reimagined community of pediatric dermatology research is a synergistic space for all members to better understand, prevent, treat, and cure dermatologic diseases and conditions in children. This two-and-a-half-day conference with over 300 attendees featured educational seminars including a keynote address, didactic lecture and panel sessions, skill-building workshops, 13 topic-specific breakout sessions, and an interactive poster session where 108 active and finished research projects could be discussed.

Stevens-Johnson syndrome in children.

PURPOSE OF REVIEW: The concept of Stevens-Johnson syndrome (SJS) in children is evolving. This manuscript reviews recent advances with the lens of new terminology namely infection-triggered reactive infectious mucocutaneous eruption and drug-induced epidermal necrolysis, with the objective of integrating this novel terminology practically. RECENT FINDINGS: Traditionally considered to exist on a spectrum with toxic epidermal necrolysis, SJS in children is more often caused or triggered by infections instead of medications. Proposed pediatric-specific terminology can be applied to literature to gain further insights into blistering severe cutaneous adverse reactions. SUMMARY: Distinguishing infection-triggered from drug-triggered blistering reactions is useful for 3 main reasons: (1) early clinically recognizable different features such as isolated or predominant mucositis, (2) different initial management depending on trigger, (3) avoiding the label of a drug reaction on cases triggered by infection.

Porokeratotic eccrine ostial and dermal duct nevus associated with an 11 megabase 3p deletion.

Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a rare eccrine hamartoma; the etiology is incompletely understood. A patient presented with congenital, widespread PEODDN. Clinical assessment, histopathologic, cytogenetic, and molecular genetic investigations on affected cells were pursued. Histopathology confirmed PEODDN, and chromosomal microarray on affected tissues identified a mosaic 3p26.3p25.3 deletion in affected tissues. This 11Mb deletion encompasses 47 OMIM genes. We propose that this and other chromosomal deletions may be implicated in some cases of PEODDN, suggesting locus heterogeneity and underscoring the importance of incorporating cytogenetic and molecular investigations into the multidisciplinary care of individuals with suspected mosaic genetic skin disorders.

Psoriasiform dermatitis during dupilumab treatment for moderate-to-severe atopic dermatitis in children.

BACKGROUND/OBJECTIVES: Psoriasiform eruptions after initiation of dupilumab have been previously described in adults. This report details the risk of developing or unmasking psoriasiform eruptions after initiation of dupilumab in children. METHODS: Records of patients ≤18 years of age with atopic dermatitis who developed psoriasiform dermatitis during treatment with dupilumab were reviewed retrospectively. RESULTS: Six children, 4-18 years of age, on dupilumab for severe atopic dermatitis developed new-onset psoriasiform dermatitis at a median duration of 8 months (range, 6-12 months) after dupilumab initiation. Typical locations of psoriasis were involved (face, scalp, trunk, and extensor extremities). The majority showed clearance or near clearance with the use of medium-strength to potent topical corticosteroid ointments and 83% continued use of the dupilumab. A 7th patient had psoriasis, in addition to severe atopic dermatitis, and the psoriasis was unmasked by its failure to respond to dupilumab. CONCLUSION: Although unusual, psoriasiform lesions can appear during effective treatment with dupilumab for atopic dermatitis, potentially reflecting a shift toward cutaneous IL-23/TH 17 pathway activation with dupilumab-induced suppression of type 2 immunity.

Approach to Chilblains During the COVID-19 Pandemic [Formula: see text].

Coronavirus disease (COVID-19) chilblains is a well-reported cutaneous pattern of severe acute respiratory syndrome coronavirus (SARS-CoV-2). Through this narrative review, we provide an evidence-based overview of idiopathic and secondary chilblains, distinguishing features of COVID-19 chilblains, and a systematic clinical approach to history, examination, investigations, and treatment. In the absence of cold or damp exposure, COVID-19 should be considered as a cause of acute chilblains. The timing of onset of COVID-19 chilblains relative to active SARS-CoV-2 viremia remains unclear. Patients with suspected COVID-19 chilblains should thus follow public health guidelines for COVID-19 testing and self-isolation.

Treatment of Mycoplasma pneumoniae-Induced Rash and Mucositis With Cyclosporine [Formula: see text].

BACKGROUND: Mycoplasma pneumoniae-induced rash and mucositis (MIRM) is a relatively newly recognized clinical entity that typically presents with predominant mucositis accompanied by variable cutaneous involvement 7-9 days after the onset of prodromal symptoms. There are no evidence-based guidelines for treatment, and current standards of care may include supportive therapy, antibiotics, corticosteroids, and intravenous immunoglobulin . OBJECTIVE: This case series aims to describe the potential efficacy of cyclosporine A (CsA) in the treatment of MIRM. METHODS: The present case series details our use of CsA early in the course of MIRM in 3 pediatric patients. RESULTS: Rapid clinical resolution was observed following CsA therapy. CONCLUSIONS: We suggest that early initiation of CsA may be an effective therapeutic option for MIRM.

Approach to the Assessment and Management of Pediatric Patients With Atopic Dermatitis: A Consensus Document. Section I: Overview of Pediatric Atopic Dermatitis.

Pediatric atopic dermatitis (AD) is one of the most common dermatoses encountered by health-care providers treating children. Diagnosis of AD is clinical, with no universally accepted biomarkers or assessment tools. Patient-reported outcomes and subjective assessments of quality of life in both the patient and family are important considerations when treating pediatric AD. Here, we provide an overview of pediatric AD epidemiology, its clinical presentation, burden, diagnosis, and assessment, with a focus on implications for patient counseling in order to optimize care.

Approach to the Assessment and Management of Pediatric Patients With Atopic Dermatitis: A Consensus Document. Section IV: Consensus Statements on the Assessment and Management of Pediatric Atopic Dermatitis.

This document is intended to provide practical guidance to physicians treating pediatric atopic dermatitis (AD), especially dermatologists, pediatricians, allergists, and other health-care professionals. The recommendations contained here were formalized based on a consensus of 12 Canadian pediatric dermatologists, dermatologists, pediatricians, and pediatric allergists with extensive experience managing AD in the pediatric population. A modified Delphi process was adopted with iterative voting on a 5-point Likert scale, with a prespecified agreement cutoff of 75%. Topic areas addressed in the 17 consensus statements reflect areas of practical management, including counselling, assessment, comorbidity management, and therapy.

Pediatric Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Halted by Etanercept.

We report a case of an 11-year-old female with Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) overlap, most likely triggered by sulfamethoxazole-trimethoprim, who was treated with the combination of methylprednisolone, cyclosporine, and etanercept. Her condition stabilized and her skin involvement did not progress after the addition of etanercept. To our knowledge, this is the first report of etanercept for pediatric SJS/TEN.

Derivation, Evaluation, and Validation of Illustrations of Key Counselling Points for a Pediatric Eczema Action Plan.

BACKGROUND: Current eczema action plans (EAP) are based on written instructions without illustrations. Incorporating validated illustrations into EAPs can significantly improve comprehension and usability. OBJECTIVE: To produce and validate a set of illustrations for key counselling points of a pediatric EAP. METHODS: Illustrations were developed using key graphic elements and refined by subject experts. Illustrations were evaluated during one-on-one structured interviews with parents/caregivers of children ages 9 and younger, as well as with children ages 10 to 17 years between September 2015 and June 2016. The concepts of transparency, translucency, and short-term recall were assessed for validation. RESULTS: Of 245 participants, 81.3% were parents and/or caregivers of children 0 to 9 years old, and 18.7% were children between 10 and 17 years old. A total of 15 illustrations and 2 storyboards were evaluated; 9 illustrations and 2 storyboards were redesigned to reach the preset validation targets. Overall, 13 illustrations and 2 storyboards were validated. CONCLUSION: A set of illustrations for use in an EAP was prospectively designed and validated, achieving acceptable transparency, translucency, and recall, with input from patients and a multidisciplinary medical team. The incorporation of validated illustrations into eczema action plans benefits patients with limited health literacy. Future studies should evaluate if illustrations improve understanding of eczema management and translate into improved clinical outcomes.

Usability, Satisfaction, and Usefulness of an Illustrated Eczema Action Plan.

BACKGROUND: An eczema action plan (EAP) is an individualized tool to help caregivers and patients self-manage eczema. While novel illustrated EAPs have been developed and validated, there is limited literature examining the value of EAPs from patient and caregiver perspectives. OBJECTIVES: The objective of this study was to test the usability, satisfaction, and usefulness of our validated EAP from the perspective of patients and caregivers. METHODS: Consecutive patients from the pediatric dermatology clinic of a tertiary children's hospital from July 2016 to July 2017 were offered enrolment in a prospective survey study; informed consent was obtained from participants. The illustrated EAP was explained to the participant by a trained research assistant. Participants were sent electronic postvisit surveys using Likert scale questions via REDCap on EAP usability and satisfaction (9 items) as well as on usefulness (3 items). RESULTS: Of 233 consecutive clinic patients, 192 participants (82%) were enrolled, and 112 (58%; 85 caregivers and 22 patients) completed the postvisit surveys. Characteristics were similar between responders and nonresponders. Overall, participants rated the usability (96%), satisfaction (85%), and usefulness (78%) of the EAP positively. Education level, experience with eczema, previous dermatology consultation, and participant type (caregiver vs patient) did not significantly affect the usability or usefulness ratings. However, caregivers' overall EAP ratings were significantly higher ( P = .02) than the patients'. CONCLUSION: The caregivers and participants demonstrate that the EAP is a useful and highly usable tool. Future research should examine the effectiveness of EAP use on objective atopic dermatitis outcomes using a pragmatic clinical trial design.

The Effectiveness of Written Action Plans in Atopic Dermatitis.

Atopic dermatitis (AD) is a chronic dermatosis requiring a stepwise and dynamic approach to management. The use of written action plans has been shown to improve outcomes in other chronic diseases that require a similar incremental approach. A systematic review was performed to evaluate the effect of a written eczema action plan (EAP) in AD management and to identify characteristics of effective action plans in children with eczema. Only two trials were identified as eligible, which highlights the need for more research on EAPs.

Quality of life in pediatric patients before and after cosmetic camouflage of visible skin conditions.

BACKGROUND: Visible vascular and pigmentary conditions have a negative impact on children's and adolescents' quality of life (QoL). We sought to quantitate the effect of visible skin anomalies and their camouflage on QoL. METHODS: In all, 41 patients, 5 years of age and older, were taught to use cosmetic camouflage. QoL was assessed using the Children's Dermatology Life Quality Index (CDLQI) before and 6 months after the intervention. Satisfaction and use were evaluated after 1 and 6 months. RESULTS: Baseline QoL scores revealed a small impact of vascular anomalies (CDLQI score 4.2) and a small to moderate effect of pigmentary anomalies (CDLQI score 6.1). Six months after the intervention, QoL improved in the study population as a whole (CDLQI score 5.1 vs 2.1, P<.001), with significant improvements documented for facial lesions and vascular malformations. Cosmetic camouflage was well tolerated and patients with pigmentary anomalies were more likely to continue using the products. LIMITATIONS: Limitations include small study population, few male patients, cultural influences not addressed, and limited range of conditions. CONCLUSIONS: Children and teenagers with visible vascular and pigmentary anomalies experience an impairment of QoL that is abrogated by introduction to use of cosmetic camouflage.

Stigmatization and Mental Health Impact of Chronic Pediatric Skin Disorders.

Importance: Chronic skin disorders in children frequently are visible and can cause stigmatization. However, the extent of stigmatization from chronic skin disease and association with mental health needs further study. Objective: To examine the extent of stigma, dependence on disease visibility and severity, and association with mental health and quality of life (QOL) in chronic pediatric skin disease. Design, Setting, and Participants: A cross-sectional, single-visit study was conducted at 32 pediatric dermatology centers in the US and Canada from November 14, 2018, to November 17, 2021. Participants included patients aged 8 to 17 years with chronic skin disease and 1 parent. Main Outcomes and Measures: Using the Patient-Reported Outcomes Measurement Instrumentation System (PROMIS) Stigma-Skin, the extent of stigma with child-, caregiver-, and physician-assessed disease visibility (primary outcome) and severity was compared, as well as reduced QOL (assessed by Skindex-Teen), depression, anxiety, and poor peer relationships (PROMIS child and proxy tools) (secondary outcomes). Results: The study included 1671 children (57.9% female; mean [SD] age, 13.7 [2.7] years). A total of 56.4% participants had self-reported high disease visibility and 50.5% had moderate disease severity. Stigma scores significantly differed by level of physician-assessed and child/proxy-assessed disease visibility and severity. Among children with chronic skin disorders, predominantly acne, atopic dermatitis, alopecia areata, and vitiligo, only 27.0% had T scores less than 40 (minimal or no stigma) and 43.8% had at least moderate stigma (T score ≥45) compared with children with a range of chronic diseases. Stigma scores correlated strongly with reduced QOL (Spearman ρ = 0.73), depression (ρ = 0.61), anxiety (ρ = 0.54), and poor peer relationships (ρ = -0.49). Overall, 29.4% of parents were aware of bullying of their child, which was strongly associated with stigma (Cohen d = -0.79, with children who were not bullied experiencing lower levels of stigma). Girls reported more stigma than boys (Cohen d = 0.26). Children with hyperhidrosis and hidradenitis suppurativa were most likely to have increased depression and anxiety. Conclusions and Relevance: The findings of this study suggest that physician assessment of disease severity and visibility is insufficient to evaluate the disease impact in the patient/caregiver. Identifying stigmatization, including bullying, and tracking improvement through medical and psychosocial interventions may be a key role for practitioners.

Management of Drug-Induced Epidermal Necrolysis (DEN) in Pediatric Patients: Moving from Drug-Induced Stevens-Johnson Syndrome, Overlap and Toxic Epidermal Necrolysis to a Single Unifying Diagnosis of DEN.

Pediatric Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but life-threating blistering diseases triggered by medications that affect the skin and mucosae. Drug-induced epidermal necrolysis is a better term for medication-triggered cases because there is a spectrum of disease severity that otherwise is divided into the separate entities of SJS, overlap SJS/TEN, and TEN. This manuscript reviews the management of drug-induced epidermal necrolysis (DEN), including diagnosis, investigations to exclude differential diagnoses, and treatment. Diagnosis of DEN relies on clinical features and a detailed medication history. The primary differential diagnosis is reactive infectious mucocutaneous eruption, which can be clinically distinguished by its disproportionate mucous membrane involvement relative to (sparse or absent) skin lesions. Identification and discontinuation of culprit medications is the mainstay of treatment of DEN. Early initiation of immunomodulatory therapy may prevent progression, reducing maximal disease severity and the risk of sequelae. A checklist approach to detailed management of DEN is proposed.