Faculty Bibliography

Across the world, there are varied cultural practices applied in the newborn period that pediatric dermatologists need to be familiar with. This report details a 9-day-old girl who presented with black, spike-like hairs across the back after her mother had been rubbing breast milk on her back in a circular motion for the first 7 days of life. On dermatoscopic exam, these lesions were found to be tight bundles of lanugo hairs, consistent with a diagnosis of knotted lanugo. Improved understanding of cultural practices and newborn skin care routines is critical for diagnosis, treatment, and counseling.

PURPOSE OF REVIEW/OBJECTIVE:This review provides updates in the evaluation and management of key dermatologic diseases encountered in the hospitalized child. RECENT FINDINGS/RESULTS:Our understanding of dermatologic disorders in children continues to evolve. Staphylococcal scalded skin syndrome (SSSS) is a potentially severe blistering disorder typically seen in children under the age of 4 with an incidence that is increasing in the United States. Recent research has highlighted that the majority of cases are due to methicillin-sensitive Staphylococcus aureus (MSSA) and most patients can be adequately managed with beta-lactams. Toxic epidermal necrolysis (TEN) is one of the most feared dermatologic disorders. Currently, there is a lack of consensus on the most efficacious first-line systemic therapy. Etanercept is increasingly being used based on studies showing a shorter time to re-epithelization and decreased mortality. Lastly, the COVID-19 pandemic introduced the novel inflammatory condition multisystem inflammatory syndrome in children (MIS-C) in which approximately three out of four children present with a mucocutaneous eruption. Early recognition of the dermatologic features of MIS-C is important in potentially establishing a diagnosis and differentiating it from the many other causes of childhood fever and rash. SUMMARY/CONCLUSIONS:There are no clear universal treatment guidelines for these rare diagnoses, and therefore, clinicians must remain informed of the latest findings in diagnosis and therapeutics.

IMPORTANCE/UNASSIGNED:Morphea is an insidious inflammatory disorder of the skin and deeper tissues. Determining disease activity is challenging yet important to medical decision-making and patient outcomes. OBJECTIVE/UNASSIGNED:To develop and validate a scoring tool, the Morphea Activity Measure (MAM), to evaluate morphea disease activity of any type or severity that is easy to use in clinical and research settings. DESIGN, SETTING, AND PARTICIPANTS/UNASSIGNED:This pilot diagnostic study was conducted from September 9, 2019, to March 6, 2020, in 2 phases: development and validation. During the development phase, 14 morphea experts (dermatologists and pediatric dermatologists) used a Delphi consensus method to determine items that would be included in the MAM. The validation phase included 8 investigators who evaluated the tool in collaboration with 14 patients with pediatric morphea (recruited from a referral center [Medical College of Wisconsin]) during a 1-day in-person meeting on March 6, 2020. MAIN OUTCOMES AND MEASURES/UNASSIGNED:During the development phase, online survey items were evaluated by experts in morphea using a Likert scale (score range, 0-10, with 0 indicating not important and 10 indicating very important); agreement was defined as a median score of 7.0 or higher, disagreement as a median score of 3.9 or lower, and no consensus as a median score of 4.0 to 6.9. During the validation phase, reliability (interrater and intrarater agreement using intraclass correlation coefficients), validity (using the content validity index and κ statistics as well as correlations with the modified Localized Scleroderma Severity Index and the Physician Global Assessment of Activity using Spearman ρ coefficients), and viability (using qualitative interviews of investigators who used the MAM tool) were evaluated. Descriptive statistics were used for quantitative variables. Data on race and ethnicity categories were collected but not analyzed because skin color was more relevant for the purposes of this study. RESULTS/UNASSIGNED:Among 14 survey respondents during the development phase, 9 (64.3%) were pediatric dermatologists and 5 (35.7%) were dermatologists. After 2 rounds, a final tool was developed comprising 10 items that experts agreed were indicative of morphea activity (new lesion in the past 3 months, enlarging lesion in the past 3 months, linear lesion developing progressive atrophy in the past 3 months, erythema, violaceous rim or color, warmth to the touch, induration, white-yellow or waxy appearance, shiny white wrinkling, and body surface area). The validation phase was conducted with 14 patients (median age, 14.5 years [range, 8.0-18.0 years]; 8 [57.1%] female), 2 dermatologists, and 6 pediatric dermatologists. Interrater and intrarater agreement for MAM total scores was good, with intraclass correlation coefficients of 0.844 (95% CI, 0.681-0.942) for interrater agreement and 0.856 (95% CI, 0.791-0.901) for intrarater agreement. Correlations between the MAM and the modified Localized Scleroderma Severity Index (Spearman ρ = 0.747; P < .001) and the MAM and the Physician Global Assessment of Activity (Spearman ρ = 0.729; P < .001) were moderately strong. In qualitative interviews, evaluators agreed that the tool was easy to use, measured morphea disease activity at a single time point, and should be responsive to changes in morphea disease activity over multiple time points. CONCLUSIONS AND RELEVANCE/UNASSIGNED:In this study, the MAM was found to be a reliable, valid, and viable tool to measure pediatric morphea activity. Further testing to assess validity in adults and responsiveness to change is needed.

• Discoid lupus may be associated with SLE. In this study, most children with discoid lupus did not have systemic disease. Concurrent SLE was highest in female adolescents (>10 years of age) with generalized discoid lupus, who had clinically aggressive disease. • Discoid lupus in adolescence should prompt thorough evaluation for SLE.

Morbilliform eruptions inspire a broad and varied differential spanning across inflammatory and infectious categories. The goal of this article is to help the clinician develop an approach toward the pediatric patient with a morbilliform eruption in the emergency room or hospital setting. The authors review several high-yield clinical scenarios with a focus on recently emerging and reemerging childhood diagnoses.

Variants in RAS are known drivers of certain pediatric blood and solid cancers, including brain tumors. Though most RAS-driven cancers are thought to occur sporadically, genetic syndromes caused by germline RAS variants portend a slightly higher risk of rhabdomyosarcoma (RMS) development. Three new cases and a review of the literature demonstrate that in rare cases, certain somatic RAS variants are associated with an increased risk of RMS and that RMS development may be heralded by the presence of concomitant RAS-driven birthmarks. Further prospective studies are needed to establish incidence and recommend appropriate monitoring guidelines for patients at risk.