How many is too many? A review of the significant numbers in pediatric skin lesions and their recommended evaluation.

Pediatric dermatologists are frequently consulted to evaluate children for cutaneous signs of systemic disorders. Numerical thresholds of significance have been described in the dermatologic literature for various skin findings where the likelihood of an associated extracutaneous abnormality or known genetic syndrome increases significantly. Knowledge of these numerical thresholds facilitates diagnosis and management, which improves clinical outcomes and avoids severe complications. This review highlights the clinical presentation, complications, evaluation, and numerical significance, when applicable, for the following skin findings: infantile hemangiomas, capillary malformations, café-au-lait macules, hypopigmented macules, juvenile xanthogranulomas, pilomatricomas, and angiofibromas.

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.

An investigation of vascular anomalies centers' transition of care practices.

This study aims to examine transition of care (TOC) practices of multidisciplinary vascular anomalies centers (VACs). Thirty-seven of 71 VAC leaders to whom the survey was sent completed the questionnaire. TOC and transfer practices varied with only 16% of VACs having TOC programs. The most frequently cited barriers to developing a TOC program were lack of resources and difficulty finding expert adult providers.

Characterization of wound microbes in epidermolysis bullosa: A focus on Pseudomonas aeruginosa.

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.

Review of transition of care literature: Epidermolysis bullosa-A paradigm for patients with complex dermatologic conditions.

BACKGROUND: Transition from pediatric to adult care is a critical component of health care for children with long-term needs. The characteristics of epidermolysis bullosa (EB) demand higher than average levels of provider support. There is consensus among health care professionals regarding the importance of transition; however, there is a scarcity of practical information regarding models for patients with EB. OBJECTIVE: To review transition of care programs in varying specialties. Highlight practical considerations to facilitate the development of programs for patients with EB and other complex dermatologic conditions. METHODS: Articles were identified via MEDLINE and EMBASE health literature databases and screened for relevance to transition of care. RESULTS: Various models for transition exist. A well-executed formal transition program, early introduction, interdisciplinary collaboration, and psychosocial support were themes associated with successful outcomes. LIMITATIONS: Transition of care programs that have not been described in the literature are not reflected in this review. CONCLUSIONS: Patients with EB have unique needs that affect transition and span expertise across traditional boundaries, such as dependency on others for daily skin care, failure to thrive, and risk of squamous cell carcinoma. Given the rarity of the disease, patients with EB will benefit from collaborative efforts to develop programs to optimize successful transition.

A retrospective analysis of diagnostic testing in a large North American cohort of patients with epidermolysis bullosa.

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.

Analysis of existing "how to" manuals for the care of inpatients with epidermolysis bullosa.

"How to" skin care manuals assist health care professionals caring for hospitalized patients with epidermolysis bullosa and other disorders. Manuals created by Epidermolysis Bullosa Clinical Research Consortium sites were collected and analyzed. Analysis of manuals revealed variable content. Creating a unified, optimized "how" to manual would benefit providers and patients.

Lumps and bumps: What not to miss.

The evaluation of pediatric patients with subcutaneous nodules remains a diagnostic challenge. Pediatric dermatologists are regularly confronted with patients who have a nonspecific nodule. Though most masses that require evaluation are ultimately benign, the possibility of a more aggressive process, including borderline or malignant neoplasms, underscores the pivotal role of the pediatric dermatologist in recognizing these lesions. The aim of this review is to provide an overview of lumps and bumps that are important to recognize to prevent delay in diagnosis or treatment of a serious underlying condition. Clinical clues that may lead the pediatric dermatologist to have a higher index of suspicion for more aggressive lesions are reviewed. Suggestions for evaluation and workup, as well as tips for the difficult to discern lesion, are proposed.

Acquired epidermodysplasia verruciformis (AEV) in three children after cardiac transplantation: A case series and review of the literature.

Acquired epidermodysplasia verruciformis (AEV) describes epidermodysplasia verruciformis developing in an immunocompromised host. There is limited information in the literature regarding AEV in the pediatric population; of the patients reported, most patients described had HIV, with only two reported cases of children who developed AEV post-transplantation. This case series describes three pediatric patients who developed AEV on immunosuppressant therapy following cardiac transplantation. We review risk factors, treatment options, and prognosis of AEV in the pediatric population.

A newly described cutaneous reaction at sites of insulin pump use in a child with Type 1 diabetes.

Continuous subcutaneous insulin infusion (CSII), or insulin pumps, with or without continuous glucose monitoring (CGM) devices have become the standard of care for patients with type 1 diabetes. While increasingly popular, a wide range of reported skin reactions to CSII and CGM devices was found. We present this case of a pyogenic granuloma-like neutrophilic and granulomatous response to an insulin pump to increase awareness of a previously uncharacterized cutaneous adverse reaction at insulin pump infusion sites.

The use of respiratory pathogen panel nasal polymerase chain reaction testing in predicting cutaneous enteroviral infections in the pediatric population.

BACKGROUND/OBJECTIVE: To characterize the relationship between the presence of enteroviral skin infection, defined as a positive skin polymerase chain reaction (PCR) test, and the nasopharyngeal (NP) respiratory pathogen panel (RPP) PCR test which includes enterovirus/rhinovirus as an analyte. METHODS: A retrospective chart review was performed on 543 subjects, age 18 years or younger, who had enterovirus (EV) skin swabs performed at an academic medical center in New York City between September 2014 and November 2019. Those patients with positive EV skin PCR were considered to have an enteroviral skin infection, and those with a negative EV skin PCR were considered not to have an enteroviral skin infection. Of those 543 children who had EV skin PCR, 170 also had an NP swab RPP performed. These NP swab RPP results were characterized as positive or negative, and if positive, it was noted if the patient was positive or negative for enterovirus/rhinovirus. The positive predictive value (PPV), negative predictive value (NPV), specificity, and sensitivity of a NP swab RPP for enteroviral skin infection were then calculated. RESULTS: An enterovirus/rhinovirus NP swab RPP had a NPV of 95%, PPV of 43%, sensitivity of 90%, and specificity of 62% for cutaneous enterovirus infection. CONCLUSION: The enteroviral skin PCR test is an assay that was validated at this institution. In clinically suspicious cases of EV, a positive NP swab RPP for enterovirus/rhinovirus is a sensitive test. A negative test is highly predictive of not having EV on the skin. Although further data are needed, given that NP swab RPP is readily available, these data may suggest that an NP swab RPP, when appropriately utilized, can support or exclude a clinical diagnosis of cutaneous enterovirus in the pediatric population.

Characterization of wound microbes in epidermolysis bullosa: Results from the epidermolysis bullosa clinical characterization and outcomes database.

BACKGROUND/OBJECTIVES: Patients with epidermolysis bullosa (EB) require care of wounds that are colonized or infected with bacteria. A subset of EB patients are at risk for squamous cell carcinoma, and bacterial-host interactions have been considered in this risk. The EB Clinical Characterization and Outcomes Database serves as a repository of information from EB patients at multiple centers in the United States and Canada. Access to this resource enabled broad-scale analysis of wound cultures. METHODS: A retrospective analysis of 739 wound cultures from 158 patients from 13 centers between 2001 and 2018. RESULTS: Of 152 patients with a positive culture, Staphylococcus aureus (SA) was recovered from 131 patients (86%), Pseudomonas aeruginosa (PA) from 56 (37%), and Streptococcus pyogenes (GAS) from 34 (22%). Sixty-eight percent of patients had cultures positive for methicillin-sensitive SA, and 47%, methicillin-resistant SA (18 patients had cultures that grew both methicillin-susceptible and methicillin-resistant SA at different points in time). Of 15 patients with SA-positive cultures with recorded mupirocin susceptibility testing, 11 had mupirocin-susceptible SA and 6 patients mupirocin-resistant SA (2 patients grew both mupirocin-susceptible and mupirocin-resistant SA). SCC was reported in 23 patients in the entire database, of whom 10 had documented wound cultures positive for SA, PA, and Proteus species in 90%, 50%, and 20% of cases, respectively. CONCLUSIONS: SA and PA were the most commonly isolated bacteria from wounds. Methicillin resistance and mupirocin resistance were reported in 47% and 40% of patients tested, respectively, highlighting the importance of ongoing antimicrobial strategies to limit antibiotic resistance.

Skin cleansing and topical product use in patients with epidermolysis bullosa: Results from a multicenter database.

BACKGROUND/OBJECTIVES: Epidermolysis bullosa (EB) comprises a group of inherited skin blistering diseases. There is currently no cure, and management includes skin protection and prevention of infection. To date, there has been no systematic investigation of home skin care practices among EB patients on a multicenter scale. METHODS: This cross-sectional, observational study included data collected from patients with EB enrolled in the Epidermolysis Bullosa Characterization and Clinical Outcomes Database (EBCCOD) who provided answers to a patient-directed questionnaire between January 1, 2017, and December 31, 2017. RESULTS: Of 202 respondents, 130 (64.4%) had dystrophic EB, 51 (25.2%) had EB simplex, 21 (7.4%) had junctional EB, 3 (1.5%) had Kindler syndrome, and 3 (1.5%) had an unspecified subtype. Seventy-eight patients reported cleansing in plain water only (39%). Of those who used an additive in their cleansing water, 75 (57%) added salt, 71 (54%) added bleach, 36 (27%) added vinegar, and 34 (26%) endorsed the use of an "other" additive (multiple additives possible). Reported concentrations of additives ranged widely from 0.002% sodium hypochlorite and 0.002% acetic acid solutions, which are thought to have negligible effects on microbes, to 0.09% sodium hypochlorite and 0.156% acetic acid, concentrations shown to be cytotoxic. One hundred eighty-eight patients answered questions regarding topical product use (93%). Of those, 131 reported topical antimicrobial use (70%). Mupirocin and bacitracin were the most commonly reported topical antibiotics (59, 58 [31.4%, 30.9%], respectively). CONCLUSIONS: These findings highlight the variety of skin care routines and frequent use of topical antimicrobials among EB patients and have potential implications for antibiotic resistance. The reported range of bleach and vinegar additives to cleansing water, including cytotoxic concentrations, emphasizes the need for clear and optimized skin cleansing recommendations.

Punched-out erosions with scalloped borders: Group A Streptococcal pustulosis.

We present a case of an infant with severe atopic dermatitis infected with Streptococcus pyogenes who presented with pustules, honey-colored crusts, and punched-out erosions with scalloped borders mimicking eczema herpeticum or eczema coxsackium. We draw attention to this case to highlight the similar clinical appearance between bacterial and viral infections in the setting of atopic dermatitis and the importance of a broad workup and coverage while awaiting results.

Erythema nodosum arising during everolimus therapy for tuberous sclerosis complex.

Indications for everolimus and other drugs within the mammalian target of rapamycin inhibitor class have recently expanded to include tuberous sclerosis complex. Everolimus is generally well tolerated, but it is important for physicians to identify and manage associated cutaneous adverse effects. We report the first case of a child developing erythema nodosum while undergoing everolimus therapy.

Texting atopic dermatitis patients to optimize learning and eczema area and severity index scores: A pilot randomized control trial.

BACKGROUND/OBJECTIVES: Atopic dermatitis is a common, chronic, debilitating disease. Poor adherence to treatment is the most important preventable contributor to adverse outcomes. Thus, improving adherence can improve patient outcomes. Text message reminders with embedded condition-specific information have been shown to improve pediatric immunization adherence but have not been assessed in atopic dermatitis. The objective was to assess the effect of daily text messages on Eczema Area Severity Index scores and caregiver knowledge of atopic dermatitis. METHODS: In this pilot randomized controlled trial, caregivers of children with atopic dermatitis enrolled during their initial appointment with a pediatric dermatologist and randomized 1:1 to standard care or daily text messages with patient education material and treatment reminders. Participants completed a multiple-choice atopic dermatitis knowledge quiz at initial and follow-up visits, and Eczema Area Severity Index scores were assessed. RESULTS: Forty-two patients enrolled, and 30 completed the study: 16 standard care group, 14 text message group. There was no significant difference in Eczema Area Severity Index score between the standard care and text message groups at follow-up, with mean decreases in Eczema Area Severity Index score of 53% and 58%, respectively. Mean score on follow-up atopic dermatitis knowledge quiz was significantly higher in the text message group (84% correct) than in the standard care group (75% correct) (P = .04). CONCLUSION: This pilot study did not demonstrate a difference in Eczema Area Severity Index scores with text message reminders. The significantly higher follow-up atopic dermatitis quiz score in the text message group indicates that participants read and retained information from text messages. Limitations include small sample size and short duration of follow-up.

Wound culture isolated antibiograms and caregiver-reported skin care practices in children with epidermolysis bullosa.

BACKGROUND/OBJECTIVES: Many patients with epidermolysis bullosa (EB) require intensive daily wound care and individualized treatment plans. Understanding patient's home skin care routines and emerging antibiotic resistance patterns in EB wounds is necessary to optimize treatment recommendations. The objective was to identify patterns of antimicrobial resistance in EB wounds and characterize patient's home practices of skin care and bathing. METHODS: This was an observational study of 23 children with EB at an outpatient pediatric dermatology practice in New York City from 2012 to 2014. Information on individual bathing and skin care practices and wound cultures was collected as part of routine examinations and an institutional review board-approved antibiogram protocol. RESULTS: Sixty wound cultures were collected from 23 patients. Eleven organisms were isolated, most commonly methicillin-susceptible Staphylococcus aureus, methicillin-resistant S. aureus, Streptococcus species, and Pseudomonas aeruginosa. Six patients (26%) were colonized with methicillin-resistant S. aureus. Over the course of the study, 13 patients (56%) were found to have mupirocin-resistant S. aureus. More than half of participants reported mupirocin or bacitracin use. Fewer than half indicated that they regularly used dilute bleach or dilute vinegar as part of their bathing routine. CONCLUSION: Numerous organisms, including resistant bacteria, are known to colonize the wounds of individuals with EB. Mupirocin resistance was prevalent and more than half of the participants reported its use. Testing for mupirocin resistance may be considered for certain patients. These observations may help guide questions for future longitudinal multicenter studies with the goal of optimizing EB wound care recommendations.

Multifocal vascular lesions.

Multifocal vascular lesions are important to recognize and appropriately diagnose. Generally first noticed on the skin, multifocal vascular lesions may have systemic involvement. Distinguishing among the different types of multifocal vascular lesions is often based on clinical features; however, radiological imaging and/or biopsy are frequently needed to identify distinct features and guide treatment. Knowledge of the systemic associations that can occur with different vascular anomalies may reduce life-threatening complications, such as coagulopathy, bleeding, cardiac compromise, and neurologic sequelae. This review provides a synopsis of the epidemiology, pathogenesis, presentation, workup, and treatment of several well-recognized multifocal vascular tumors and malformations.