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BACKGROUND/OBJECTIVES The Pediatric Dermatology Research Alliance (PeDRA) connects pediatric dermatologists, trainees, basic scientists, allied health professionals, and patient advocates to improve the lives of children with skin disease through research. As a training pipeline for future pediatric dermatologists and steward of research in the field, PeDRA has a responsibility to examine its history and take actionable steps to diversify its membership, grant recipients, study leads, research priorities, and leadership. METHODS In 2020, PeDRA formed an Equity, Diversity, and Inclusion Task Force to address this need. In an effort to assess PeDRA's past and plan for PeDRA's future, a review of PeDRA's membership, leadership, grant awardees, and research topics was conducted. RESULTS/CONCLUSIONS Results demonstrated gaps in PeDRA's current operational efforts to diversify the pediatric dermatology workforce and identified areas for improvement. Recommendations are proposed as a call to action for the community.

Melanoma is rare in pediatric patients and even more so in those with darker Fitzpatrick skin types. Although risk factors for conventional melanoma are similar in both adult and pediatric cases, the presentation of melanoma in pediatric patients is often distinct from adults. Here, we describe a case of amelanotic ulcerated nodular melanoma with regional lymph node metastases treated with nivolumab in a patient with Fitzpatrick skin type VI.

Barriers to healthcare access are healthcare inequities that have been widely studied across different medical specialties. No studies have previously evaluated the state of barriers to healthcare access research in pediatric dermatology. A systematic review was conducted to examine the types of barriers identified within pediatric dermatology literature. Relevant information was extracted and categorized into the themes of systemic, sociocultural, or individual barriers. The systemic barriers we found include finances, wait times, and geography. The sociocultural barriers included culture beliefs and communication. Patient beliefs and health knowledge were found as individual barriers. The small number and limited scope of studies we identified suggest that barriers to healthcare access in pediatric dermatology remain an understudied topic. Additional research is needed to further characterize these barriers to dermatologic care, as well as the impact of any interventions designed to overcome them.

Purpose To describe a series of children with extensive PNF or treatment refractory PLGG treated on a compassionate basis with trametinib. Methods We report on six patients with NF‐1 treated with trametinib on a compassionate basis at British Columbia Children's Hospital since 2017. Data were collected retrospectively from the patient record. RAPNO and volumetric criteria were used to evaluate the response of intracranial and extracranial lesions, respectively. Results Subjects were 21 months to 14 years old at the time of initiation of trametinib therapy and 3/6 subjects are male. Duration of therapy was 4–28 months at the time of this report. All patients had partial response or were stable on analysis. Two patients with life‐threatening PNF had a partial radiographic response in tandem with significant clinical improvement and developmental catch up. One subject discontinued therapy after 6 months due to paronychia and inadequate response. The most common adverse effect (AE) was grade 1–2 paronychia or dermatitis in 5/6 patients. There were no grade 3 or 4 AEs. At the time of this report, five patients remain on therapy. Conclusion Trametinib is an effective therapy for advanced PNF and refractory PLGG in patients with NF‐1 and is well tolerated in children. Further data and clinical trials are required to assess tolerance, efficacy and durability of response, and length of treatment required in such patients., Here, we report on response to Trametinib, a MEK inhibitor in children with NF‐1. Our experience presented in this report demonstrates that Trametinib is an effective therapy for advanced, life‐threatening PNF, and treatment‐refractory PLGG in patients with NF‐1 and is well tolerated in children.

There are 70.8 million persons displaced worldwide due to war, persecution, and violence. Eighty percent of displaced persons reside in low- and middle-income countries with limited healthcare resources. Cutaneous diseases are commonly reported among displaced persons owing to numerous interrelated factors such as inadequate housing, overcrowding, food insecurity, environmental exposures, violence including torture, and breakdown of healthcare infrastructure. Diagnosis and management of these conditions, as well as an understanding of the context in which they present, is crucial to providing dermatologic care for displaced populations worldwide. Herein, we define displaced populations and, within this context, review the epidemiology of skin diseases, discuss pertinent skin conditions, examine challenges to care provision, and present approaches for improving dermatologic care. Inflammatory and communicable infectious disorders are the most common skin diseases seen in displaced populations. Other relevant conditions include skin manifestations of heat injuries, cold injuries, immersion foot syndromes, macronutrient and micronutrient deficiencies, torture, and sexual and gender-based violence. Provision of dermatologic care to displaced populations is hampered by limited diagnostic and therapeutic resources and specialist expertise. Medical screening for cutaneous disorders, context-relevant dermatology training, and telemedicine are potential tools to improve diagnosis and management of skin diseases in displaced populations.

X-linked hypohidrotic ectodermal dysplasia (XLHED) is the most common form of ectodermal dysplasia. Clinical and genetic heterogeneity between different ectodermal dysplasia types and evidence of incomplete penetrance and variable expressivity increase the potential for misdiagnosis. We describe a family with X-linked hypohidrotic ectodermal dysplasia (XLHED) presenting with variable expressivity of symptoms between affected siblings. In addition to the classical signs of hypohidrosis, hypotrichosis and hypodontia, the index patient—a 5 year old boy, also presented with a severe atopy phenotype that was not observed in the other two affected brothers. Exome sequencing in the index and the mother identified a pathogenic nonsense variant in EDA (NM_001399.4: c.766 C>T; p. Gln256Ter). This study highlights how exome sequencing was crucial in establishing a precise molecular diagnosis of XLHED by enabling us to rule out other differential diagnoses including NEMO deficiency syndrome, that was initially presented as a clinical diagnosis to the family.

B-cell lymphoma/leukemia 11B (BCL11B) is a C2H2 zinc finger transcription factor that is critically important for regulating the development and function of a variety of systems including the central nervous system, the skin, and the immune system. Germline heterozygous variants are associated with a spectrum of clinical disorders, including severe combined immunodeficiency as well as neurological, craniofacial, and dermal defects. Of these individuals, ~50% present with severe allergic disease. Here, we report the detailed clinical and laboratory workup of one of the most severe BCL11B-dependent atopic cases to date. Leveraging a zebrafish model, we were able to confirm a strong T-cell defect in the patient. Based on these data, we classify germline BCL11B-dependent atopic disease as a novel primary atopic disorder.

Social pediatric initiatives aim to improve health outcomes for vulnerable children by working in the community to empower families, to enhance protective factors that mitigate adverse childhood experiences (ACEs), and to deliver place-based health care. In 2012, pediatric dermatology was added as a component of the Responsive, Interdisciplinary Intersectoral Child and Community Health Education and Research (RICHER) social pediatric program in Vancouver, BC. We share our experience with inclusion of pediatric dermatology in a well-established social pediatric program as well as lessons we have learned in the first 8 years of our partnership. Partnership, bridging trust, knowledge sharing, empowerment, consistency, and flexibility were found to be central elements in the success of this endeavor.

Background/objectives Efficacy and safety data of scabies treatments in infants are limited. Although topical permethrin is used in the treatment of scabies in adults, it is not approved for use in infants younger than 2 months of age in many parts of the world. This study aimed to describe treatment practices in the management of scabies in infants younger than 2 months. Methods An online survey was developed and distributed to physicians worldwide through the Society of Pediatric Dermatology and the Pediatric Dermatology Research Alliance. Data collected included demographics, medication availability, experience using medications, deterrents to medication use, medication administration preferences, perceived and experienced medication side effects, and preferred treatment agent in this population. Results In total, 57 physicians from seven countries responded. The majority of respondents were board-certified in pediatric dermatology (48/57, 84.2%) and resided in the United States (44/57, 77.2%). Respondents had experience using permethrin (47/57, 82.5%) and precipitated sulfur (35/57, 61.4%) most frequently. Most (38/57, 66.7%) preferred permethrin as their treatment of choice. Among those who did not use permethrin, potential side effects (8/10, 80%) were most frequently reported as a deterrent from its use. However, only 4.3% (22/47) of those who used permethrin reported side effects, including itching, erythema, and xerosis. Conclusions Permethrin is frequently used in the treatment of infants younger than 2 months with scabies. Furthermore, our results demonstrate that permethrin is the preferred treatment agent among sampled dermatologists for infants younger than 2 months. Few side effects were reported, and none were serious.

Dermatologic disease is among the most frequently reported health problems in homeless and refugee populations. Overcrowding, lack of access to bathing and laundry facilities, and increased exposure to the elements contribute to increased rates of disease in this population. Because it is the most visible part of the body, the skin also is a key part of human interaction. Changes in appearance of the skin, whether from purposeful alteration or due to a disease process, impact self-concept as well as perception by others. Symptoms of skin problems may include itching, pain, embarrassment, and social isolation. Because of these features, skin problems may be of particularly high concern for youth and may lead young people to seek medical attention, thus opening the door for discussions about other health and social concerns. This chapter first reviews the cause and management of some of the more common dermatology conditions with some practical tips for management. In the second portion of this chapter, cutaneous conditions that are of particular concern among homeless and refugee youth are reviewed.

Atopic dermatitis (AD) is a chronic inflammatory skin condition, also referred to as atopic eczema, that is identified by itching and recurrent eczematous lesions. It often starts in infancy where it affects up to 20% of children but is also highly prevalent in adults. AD inflicts a significant psychosocial burden on patients and their families and increases the risk of other immune-mediated inflammatory conditions, such as asthma and allergic rhinitis, food allergy, and mental health disorders. It is a lifelong condition associated with epidermal barrier dysfunction and altered immune function. Through the use of emollients and anti-inflammatory agents, current prevention and treatment therapies attempt to restore epidermal barrier function. Acute flares are treated with topical corticosteroids. Topical calcineurin inhibitors (TCIs) and topical corticosteroids (TCSs) are used for proactive treatment to prevent remission. There remains a need and opportunity to improve AD care through future research directed toward an improved understanding of the heterogeneity of the disease and its subtypes, the role of autoimmunity in its pathogenesis, the mechanisms behind disease-associated itch and response to specific allergens, and the comparative effectiveness and safety of therapies.

OBJECTIVE Refractory symptomatic plexiform neurofibromas (PNF) and inoperable refractory low grade gliomas (LGG) pose a clinical challenge that may be life threatening. Phase 1 and 2 clinical trials of MEK inhibition with selumetinib in inoperable PNF and LGG have demonstrated promising results in pediatrics, however access has been limited to enrollment on clinical trial. Phase 1 clinical trial for trametinib a MEK 1 and 2 inhibitor has been completed, publication is pending. Thus we have treated a series of children on a compassionate basis with extensive PN or LGG refractory disease with trametinib, as this is available in Canada. METHODS We have treated children with trametinib on a compassionate basis in our province since 2017. Review of the clinical data regarding this therapy has been IRB approved. RESULTS Two young patients were treated for indication of life threatening extensive PNF and have had tumor shrinkage and improvement of clinical status. Treatment has been complicated by paronychiae, eczema exacerbation, chondrodermatitis nodularis helicis, RSV and influenza B infection and CTCAE grade 2 pneumonia. In spite of the side effects these two patients remain on treatment due to clear benefit from therapy including: improved respiratory compromise, hearing and dysphagia. We will present the data of additional patients treated with trametinib. CONCLUSION Trametinib is an effective therapy for life threatening PNF by changing the natural history of tumor growth in young children. Further data is required in terms of tolerance, efficacy and durability of response in such patients in the setting of clinical trials.

Background Medication nonadherence is a significant problem in psoriasis. Although previous research has aimed to quantify the prevalence of nonadherence in topical medication users, patient motivations for intentional nonadherence are not fully understood. Objective Our purpose was to determine the relative importance of factors contributing to nonadherence in topical corticosteroid users with psoriasis. Methods A survey was distributed to psoriasis patients visiting an outpatient clinic. Results Among the 53 persons surveyed, medication nonadherence was 40%. Frustration with medication efficacy, inconvenience, and fear of side effects were the most important reasons patients deviated from usage instructions. Subjects who did not receive instructions on topical treatment duration used corticosteroids longer (>8 weeks) than subjects who were instructed (P Limitations A relatively small sample size prevented trends in demographic predictors of adherence from reaching statistical significance. The patient self-report format has the potential to introduce recall bias. Conclusions Factors contributing to intentional nonadherence, including dissatisfaction with efficacy, inconvenience, and fear of side effects, are the most important patient-identified barriers to appropriate use of topical steroids in psoriasis. Clear patient instructions on the intended steroid treatment duration may reduce adverse effects from overuse.

Background. Green tea extracts have gained popularity as ingredients in topical skin care preparations to treat aging skin. Green tea polyphenolic compounds have significant antioxidant and anti-inflammatory activities, and studies suggest that these extracts help mediate ultraviolet radiation damage. Objective. To evaluate the effects of a combination regimen of topical and oral green tea supplementation on the clinical and histologic characteristics of photoaging. Methods. Forty women with moderate photoaging were randomized to either a combination regimen of 10% green tea cream and 300 mg twice-daily green tea oral supplementation or a placebo regimen for 8 weeks. Results. No significant differences in clinical grading were found between the green tea–treated and placebo groups, other than higher subjective scores of irritation in the green tea–treated group. Histologic grading of skin biopsies did show significant improvement in the elastic tissue content of treated specimens (p

Objective Recently a number of new therapies have been introduced to treat psoriasis, but concerns have been expressed about their high cost. The purpose of this study was to determine whether most psoriasis treatments lie within the accepted range of cost-utility. Methodology 32 patients with moderate to severe psoriasis were administered the Euro-Qol 5 Dimension (EQ-5D) survey to calculate their health state utility. Economic modeling was performed with a range of therapeutic costs applying the calculated utility score. Paired t-tests were used to calculate significance. Results At the conclusion of 2 weeks of therapy, the mean psoriasis area and severity index (PASI) improved 35% to 7.2 (p

Background. Anecdotal evidence from program directors and Mohs surgeons suggests that Mohs fellowships are becoming increasingly popular and competitive among dermatology trainees. Objective. To assess the characteristics and investigate the motivating factors of those pursuing Mohs fellowships. Methods. Anonymous surveys were distributed to recent dermatology residency graduates taking a board exam review course in years 1999–2002. Results. In 2002, 2001, and 1999, the percentages of recently trained dermatologists pursuing Mohs fellowships were 9.4%, 8.5%, and 8.8%, respectively. There were no significant differences between Mohs fellows and the rest of the recently graduated dermatologists in terms of debt levels, marital status, parenting status, and spousal employment status. The Mohs fellows were slightly more likely to be male than their non-Mohs counterparts. The factor considered the most important by both groups when choosing a job was location. Conclusions. Further research is needed to discover potential factors that may be playing a role in the increased popularity of Mohs surgery. The number of Mohs surgeons is increasing and is likely to expand over time. It remains to be seen what effect the growth will have on the supply of Mohs surgery and whether it will outpace the increased demand for services.

Background Emberger syndrome is caused by mutation in GATA2 and predisposes to myelodysplastic syndrome (MDS)/acute myelogenous leukaemia (AML), lymphedema, warts, subtle dysmorphic features, and, rarely, congenital anomalies. Objectives To describe the heterogeneity associated with GATA2 mutation and highlight features that should prompt testing. Design/methods Case report of 2 families with mutation-confirmed Emberger syndrome. Results Family 1: The proband presented with warts and mouth ulcers. WHIM syndrome was considered when neutropenia and B-cell deficiency developed, despite negative CXCR4 testing. Years later, the patient developed AML with monosomy 7 and underwent chemotherapy and stem cell transplantation. Idiopathic leg lymphedema occurred. Family history was significant for warts. Re-examination identified subtle dysmorphisms. GATA2 sequencing detected a missense mutation. Family 2: Sibling 1 had a history of warts, mouth ulcers, ectopic anus, mild sensorineural hearing loss, and new-onset neutropenia. Bone marrow testing diagnosed MDS with monosomy 7. Sibling 2 had hypocellular, mildly dysplastic bone marrow. History included warts and leg lymphedema. Sibling 3 had mild bone marrow hypocellularity. Family history was significant for a parent with childhood warts and mouth ulcers. Siblings 1–3 had subtle dysmorphisms. GATA2 sequencing identified a nonsense mutation. Sibling 1 underwent stem cell transplantation. Siblings 2–3 are closely monitored. Conclusions The literature is biassed towards individuals who present with overt haematological malignancies; however, Emberger syndrome can be recognised earlier. Suspicion should be high in individuals with persistent hematologic/immunologic abnormalities, warts refractory to treatment, and/or lymphedema particularly in the setting of subtle, but typical, dysmorphisms. Intervention before development of AML decreases morbidity/mortality.

Hand dermatitis is a common occupational disease. Altered skin pH plays an important role in the development of skin irritation. A glove that maintains tight control over skin pH may reduce hand dermatitis in glove users. The purpose of the study was to characterize the effect of glove wearing on skin pH, investigate the impact of study glove on skin pH compared with standard gloves and determine whether wearing study gloves reduced irritation. 20 healthy volunteers enrolled in a 4-week double-blind comparison of study and control gloves and served as their own controls. Gloves were worn 8 hr per day for 5 days per week. Skin pH and transepidermal water loss were measured during and 2 days after the glove-wearing period. The subject and an observer assessed the skin for irritation. The study glove maintained lower skin pH than the control glove (P < 0.05) and trended towards having less irritation. Observers noted increases in dryness and scale in both hands after 4 weeks but significantly less dryness in the study hand at week 4 (P = 0.006). Glove wearing increased skin pH and dryness. The pH 5.5 glove maintained lower skin pH levels than the control glove and may reduce irritation in long-term glove wearers.

In most developing countries there are few, if any, skin specialists and primary care providers often receive little or no training in skin care. Skin problems are often overlooked when global health issues are discussed, yet those affected range from 28% to 87% of the population, depending on the locale. An estimated 24% of primary care visits in developing countries are related to skin concerns. When health care is focused on life-threatening diseases, skin concerns may not be addressed, which can lead to infection, discomfort, and even disfigurement and community ostracism.With somany lacking access to care, andwith a shortage ofmedical providers skilled in skin disease, a sustainable benefit must come from training local health care providers, from physicians to community health workers, in their communities. To initiate this effort, the International Foundation for Dermatology (http://www.ifd.org) was formed as an arm of the International League of Dermatological Societies (representing[25,000 members from[60 nations) as a nonprofit organization whose principal mission is to improve dermatologic care in underserved areas of the developing world. One of its activities is support of the Regional Dermatology Training Center in Moshi, Tanzania. Another is publication of the Community Dermatology Journal (http://www.ifd.org/news_commderm.html). Ten years ago, Dr Raymond Cornelison Jr, as American Academy of Dermatology (AAD) president, raised concern aboutmore than 3billionpeople in developing countries who lacked access to basic dermatologic care. Subsequently the AADdeveloped a relationship with Health Volunteers Overseas (HVO), a nonprofit organization that had been sending health care professionals to developing countries

To evaluate the protective effects of a jellyfish sting inhibitor formulated in sunscreen lotion vs conventional sunscreen against Chrysaora fuscescens and Chiropsalmus quadrumanus jellyfish.Twenty-four healthy subjects at 2 research sites were randomly assigned to receive the jellyfish sting inhibitor (Nidaria Technology Ltd, Jordan Valley, Israel) to one forearm and conventional sunscreen to the other arm in a blinded fashion. Subjects were stung with jellyfish tentacles on each forearm for up to 60 seconds. Erythema and pain were assessed at 15-minute intervals over a 2-hour period.In the C. fuscescens group, all 12 arms pretreated with conventional sunscreen demonstrated erythema, and all subjects noted subjective discomfort. In contrast, no arm pretreated with the jellyfish sting inhibitor had objective skin changes (P.01). Two subjects noted minimal discomfort in the arm treated with the sting inhibitor (P.01). In the C. quadrumanus group, discomfort was reported in 3 of the 12 inhibitor-treated arms compared with 10 of the 12 placebo-treated arms (P.05). Erythema was noted on 1 arm treated with the inhibitor and 9 arms treated with the placebo (P.01).The jellyfish sting inhibitor prevented sting symptoms of C. fuscescens jellyfish in 10 of 12 subjects and diminished the pain of the jellyfish sting in the remaining 2 subjects. The jellyfish sting inhibitor also inhibited the more severe sting of the C. quadrumanus jellyfish in the majority of subjects. The jellyfish sting inhibitor does not eliminate the sting from C. fuscescens or C. quadrumanus jellyfish but significantly reduces the frequency and severity of stings.