Faculty Bibliography

BACKGROUND/OBJECTIVE/OBJECTIVE:Papular scars are a recently described clinical phenotype of acne scarring characterized by papules occurring on the nose and chin. We have observed a similar presentation of nasal papules among patients seen in our clinic for acne and sought to further characterize the clinical and histopathological characteristics of this entity. METHODS:In this single-site case series, a retrospective review of electronic medical records of patients with nasal papules in association with acne vulgaris between April 2018 and April 2019 was performed. Clinical and histopathologic findings were recorded. RESULTS:We identified 20 patients who presented with a similar clinical phenotype of predominantly skin-colored, dome-shaped papules concentrated on the nose and chin in association with a history of more classic facial acne vulgaris. Papular lesions were seen predominately in adolescent Hispanic males. Concomitant acne on other areas of the face was identified in 18 patients at presentation while two patients had a history of adolescent acne. Biopsies were performed for five patients. Histopathologic examination demonstrated features of fibrosis and dilated thin-walled blood vessels, typical of angiofibromas. CONCLUSION/CONCLUSIONS:We present a series of adolescent patients with large, flesh-colored to erythematous papules seen predominantly on the nose. These lesions are histologically indistinguishable from angiofibromas and may represent an under-recognized yet disfiguring sequela of acne that may disproportionately affect adolescents with skin of color.

BRAF inhibitor-induced neutrophilic panniculitis is a rare event that is well-characterized in adults undergoing therapy for metastatic melanoma. To date, there are very few reports of this event in children undergoing BRAF inhibitor therapy for low-grade gliomas, all of which were seen with vemurafenib. We report a case of dabrafenib-induced neutrophilic panniculitis in a 9-year-old girl that manifested within several weeks of initiating dual BRAF-MEK inhibitor therapy for glioblastoma multiforme. This case highlights neutrophilic panniculitis as a side effect of dabrafenib in children and serves as a reminder to consider cutaneous side effects of BRAF inhibitors as they are increasingly used to treat children with primary brain tumors.

Photograph editing has been present for centuries, but the past decade has seen an unprecedented rise in the practice, particularly on social media platforms. Common filters are able to soften complexion, remove blemishes, and even reshape facial features. Children and adolescents with skin conditions may find solace in using these applications to mask their disease online, but there is increasing concern about negative overall body image among those who filter their photographs. Pediatric dermatologists should remain aware of the practice of photograph editing among young people and consider asking patients about their social media presence.

Nevus psiloliparus is a rare fatty tissue nevus that is a marker for encephalocraniocutaneous lipomatosis, a neurocutaneous syndrome with ocular and central nervous system anomalies. Clinically, nevus psiloliparus is often described as a congenital alopecia and appears as an irregularly shaped, circumscribed area of alopecia on the scalp. Histopathology demonstrates a near-complete absence of mature hair follicles with preservation of arrector pili muscles and mature adipocytes within the dermis. The pathogenesis of nevus psiloliparus may be related to mosaic mutations in fibroblast growth factor receptor 1. Herein we report the histopathological features of a nevus psiloliparus in an 11-year-old girl diagnosed from transverse sections, which show "shadow" follicular units characterized by columns of loosely arranged collagen and a relative paucity of elastic fibers.

Infectious panniculitis from hematogenous spread is uncommon and usually occurs in immunocompromised patients. Dissemination of gram-positive organisms to the subcutaneous tissue is rare with only several reports of disseminated panniculitis caused by Streptococcal species. We report a case of an immunocompetent 2-year-old boy presenting with diffuse neutrophilic panniculitis arising from methicillin-resistant Staphylococcus aureus septicemia. This case represents a highly atypical manifestation of severe MRSA infection and serves as a reminder to consider MRSA as a cause of disseminated neutrophilic panniculitis, particularly in high-risk populations.

BACKGROUND/OBJECTIVES/OBJECTIVE:The COVID-19 pandemic has raised questions about the approach to management of systemic immunosuppressive therapies for dermatologic indications in children. Given the absence of data to address concerns related to SARS-CoV-2 infection while on these agents in an evidence-based manner, a Pediatric Dermatology COVID-19 Response Task Force (PDCRTF) was assembled to offer time-sensitive guidance for clinicians. METHODS:A survey was distributed to an expert panel of 37 pediatric dermatologists on the PDCRTF to assess expert opinion and current practice related to three primary domains of systemic therapy: initiation, continuation, and laboratory monitoring. RESULTS:Nearly all respondents (97%) reported that the COVID-19 pandemic had impacted their decision to initiate immunosuppressive medications. The majority of pediatric dermatologists (87%) reported that they were pausing or reducing the frequency of laboratory monitoring for certain immunosuppressive medications. In asymptomatic patients, continuing therapy was the most popular choice across all medications queried. The majority agreed that patients on immunosuppressive medications who have a household exposure to COVID-19 or test positive for acute infection should temporarily discontinue systemic and biologic medications, with the exception of systemic steroids, which may require tapering. CONCLUSIONS:The ultimate decision regarding initiation, continuation and laboratory monitoring of immunosuppressive therapy during the pandemic requires careful deliberation, consideration of the little evidence available, and discussion with families. Consideration of an individual's adherence to COVID-19 preventive measures, risk of exposure, and the potential severity if infected must be weighed against the dermatological disease, medication, and risks to the patient of tapering or discontinuing therapies.

Childhood exanthems are commonly encountered by pediatricians in the hospital and the office. In the last several decades, we have seen a shift in the epidemiology of many of these diseases. After being deemed eliminated at the turn of 21st century, measles has experienced a resurgence secondary to falling vaccination rates, raising public health concerns. A new variant of hand, foot, and mouth disease caused by coxsackievirus A6 has been associated with more widespread and atypical disease, which can present diagnostic challenges to clinicians. Parvovirus B19, which is traditionally associated with fifth disease, is also the leading cause of papular purpuric gloves and socks syndrome, a rare condition with which providers may be unfamiliar. Since the introduction of routine vaccination, there has been a shift in the epidemiology and clinical presentation of primary varicella and herpes zoster. Finally, the recently described phenomenon of Mycoplasma pneumoniae-induced rash and mucositis will be discussed. [Pediatr Ann. 2020;49(3):e116-e123.].

Background/Purpose : DLE is a rare, disfiguring disorder in children. Small retrospective studies suggest 20-25% of patients progress to SLE. Progression risk factors are poorly understood, but DLE has been associated with delay in SLE diagnosis and reduced access to care. This multicenter retrospective cohort study aimed to describe baseline characteristics and clinical phenotypes of pediatric DLE patients at diagnosis. Methods : Medical records at eighteen sites were reviewed for pediatric dermatology and rheumatology patients with DLE. For inclusion, patients required clinical and/or histopathologic findings consistent with DLE. Baseline data were collected at the first documented visit including sociodemographic data, ACR/SLICC SLE criteria (i.e. DLE+SLE), date of DLE onset/diagnosis, DLE distribution, family history, comorbidities, and treatment. Outcome variables included ACR (primary outcome) /SLICC SLE criteria. Rates of progression from skin-limited DLE (DLE) to SLE (DLE+SLE) were evaluated. Analysis included descriptive statistics, chi-square and Wilcoxon tests. Results : Out of >1,000 patients reviewed, 441 met inclusion criteria. The cohort was predominantly female (72%) and racially/ethnically diverse (Table 1). A minority presented at baseline with SLE based on ACR and SLICC criteria, respectively (n=165, 37%; n=183, 42%). DLE+SLE patients were older (median 13.7y vs 10.2y) with shorter time from DLE onset to diagnosis (median 2 mo vs 7 mo), compared to DLE patients (p< 0.001). DLE patients presented with low incidence of renal involvement, serositis, seizures or psychosis (p< 0.001, Table 2). DLE+SLE patients had more positive serologies and higher-titer ANAs (p< 0.001, Table 3), although 5% were ANA negative. Among 231 DLE patients with31 follow up visit, median follow-up was 2.7 y (range 0-13.9y) with 747 total subject-years. Progression to SLE occurred in 20% and 25% of patients based on ACR and SLICC criteria, respectively. Conclusion : To date, this is the largest investigation of pediatric DLE. Patients with DLE+SLE were most likely to present in adolescence with abnormal serologies and end-organ disease. Progression of DLE to SLE occurred at rates consistent with previous literature. All patients with DLE require SLE surveillance at diagnosis and regular follow-up, particularly during adolescence. Limitations include the retrospective study design with potential for misclassification, and analysis restricted to the baseline visit. Further analysis of follow up visits will evaluate for baseline risk factors and biomarkers of evolving SLE, as well as timing of progression, identifying DLE patients at highest risk for systemic disease

Well-known causes of zinc deficiency, also referred to as acrodermatitis enteropathica (AE), include defects in intestinal zinc transporters and inadequate intake, but a rare cause of acquired zinc deficiency discussed here is an iatrogenic nutritional deficiency caused by parenteral nutrition administered without trace elements. While zinc-depleted parenteral nutrition causing dermatosis of acquired zinc deficiency was first reported in the 1990s, it is now again relevant due to a national vitamin and trace element shortage. A high index of suspicion may be necessary to diagnose zinc deficiency, particularly because early clinical findings are nonspecific. We present this case of acquired zinc deficiency in a patient admitted to a pediatric intensive care unit for respiratory distress and atypical pneumonia, who subsequently developed a severe bullous eruption due to iatrogenic zinc deficiency but was treated effectively with enteral and parenteral zinc supplementation, allowing for rapid re-epithelialization of previously denuded skin.