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.

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.

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.

A rare case of suspected lupus erythematous panniculitis as the presenting skin feature of juvenile dermatomyositis: A case report.

Juvenile dermatomyositis is a rare autoimmune myopathy of childhood, associated with systemic vasculopathy, primarily affecting the capillaries. Panniculitis is seen histologically in about 10% of patients with dermatomyositis; however, its clinical presentation is rare, with only 30 cases presented in the literature to date. The histopathology overlaps with other inflammatory disease states, and is almost identical to the panniculitis seen in lupus erythematous panniculitis. In the cases with both panniculitis and dermatomyositis, skin and muscle inflammation is usually the first clinical manifestation. We present a case of a 16-year-old female with panniculitis as the initial presenting feature of juvenile dermatomyositis in the context of a prior diagnosis of indeterminate colitis.

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.

Recommendations for Vaccination in Children with Atopic Dermatitis Treated with Dupilumab: A Consensus Meeting, 2020.

Dupilumab is the only biologic therapy currently approved in Europe and the United States for severe atopic dermatitis in patients 6 years of age or older. Off-label use is rationalized in younger children with severe atopic dermatitis. Decisions about vaccination for children on dupilumab are complex and depend on both the child's current treatment and the type of vaccination required. To achieve consensus on recommendations for vaccination of pediatric patients with atopic dermatitis treated with or planning to start dupilumab, a review of the literature and a modified-Delphi process was conducted by a working group of 5 panelists with expertise in dermatology, immunology, infectious diseases and vaccination. Here, we provide seven recommendations for vaccination of pediatric patients with atopic dermatitis treated with or planning to start dupilumab. These recommendations serve to guide physicians' decisions about vaccination in children with atopic dermatitis treated with dupilumab. Furthermore, we highlight an unmet need for research to determine how significantly dupilumab affects cellular and humoral immune responses to vaccination with live attenuated and inactivated vaccines.

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.

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.

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.

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.