Topical trametinib for epidermal and sebaceous nevi in a child with Schimmelpenning-Feuerstein-Mims syndrome.

We present the case of a 20-month-old girl with Schimmelpenning-Feuerstein-Mims (SFM) syndrome with extensive head, neck, and torso skin involvement successfully managed with topical trametinib. Trametinib interferes downstream of KRAS and HRAS in the MAPK signaling pathway, of which KRAS was implicated in our child's pathogenic variant. Although other dermatologic conditions have shown benefit from oral trametinib, its topical use has not been well reported. Our patient showed benefit from the use of twice-daily topical trametinib, applied to the epidermal and sebaceous nevi over a 16-month period, leading to decreased pruritus and thinning of the plaques.

Surgical deroofing in pediatric patients with hidradenitis suppurativa.

Hidradenitis suppurativa (HS) is a chronic, inflammatory follicular disorder that most commonly involves the intertriginous areas. It is characterized by recurrent nodules that may progress into deeper abscesses and sinus tracts. Treatment is challenging and often involves a combination of lifestyle modifications, medical therapies, and procedures to control symptoms. Deroofing is a tissue-saving surgical technique that has been well studied in adults and in our clinical experience is an important adjunct therapy in pediatric patients with moderate-to-severe HS. We describe the step-by-step process of deroofing, including surgical clinical pearls specific to the pediatric population.

Validation of remote atopic dermatitis severity assessment with the Eczema Area and Severity Ondex in children using caregiver-provided photos and videos.

BACKGROUND/OBJECTIVES: We sought to quantify the reliability and validity of remote atopic dermatitis (AD) severity assessment using the Eczema Area and Severity Index (EASI) applied to caregiver-provided photos (p-EASI) and videos (v-EASI). METHODS: Children (0-17 years) with a physician diagnosis of AD were recruited. Caregivers took photos and a video of their child's skin. A clinician scored in-person EASI on the same day, then p-EASI and v-EASI for each participant 10 days or more between ratings. Two additional clinicians scored p-EASI and v-EASI. Lin's concordance correlation coefficient (CCC) was employed to assess criterion validity using in-person EASI as the gold standard. Intraclass correlation coefficients (ICCs) were calculated to assess interrater reliability of p-EASI and v-EASI. RESULTS: Fifty racially and ethnically diverse children (age [mean ± SD]: 4.3 ± 4.4 years; 42% female) with a range of AD severity (EASI: 6.3 ± 6.4) and Fitzpatrick skin types (1-2: 9%; 3-4: 60%; 5-6: 31%) were enrolled and received in-person EASI assessment. Fifty had p-EASI and 49 had v-EASI by the same in-person rater, and by two additional raters. The CCC and ICC for p-EASI were 0.89, 95% CI [0.83, 0.95] and 0.81, 95% CI [0.71, 0.89], respectively. The CCC and ICC for v-EASI were 0.75, 95% CI [0.63, 0.88] and 0.69, 95% CI [0.51, 0.81], respectively. CONCLUSIONS: In this diverse population with a range of skin tones, p-EASI showed good criterion validity and good interrater reliability. v-EASI showed moderate to good criterion validity and moderate interrater reliability. Both may be reliable and valid options for remote AD severity assessment.

Determination of Ethnic Variation in Infant Nasolabial Anthropometry Using 3D Photographs: Implications for Bilateral Cleft Lip Nasal Correction.

OBJECTIVE: We aimed to assess significant ethnic variabilities in infants' nasolabial anthropometry to motivate variations in surgical correction of a synchronous bilateral cleft lip/nasal anomaly, specifically whether a long columella is a European feature, therefore accepting a short columella and/or delayed columellar lengthening suitable for reconstruction in ethnic patients. METHODS: Thirty-three infants without craniofacial pathology (10 African American [AA], 7 Hispanic [H], and 16 of European descent [C]), ages 3 to 8 months, presenting to the Johns Hopkins All Children's general pediatric clinic were recruited. Four separate 3D photographs (2 submental and frontal views each) were taken using the Vectra H1 handheld camera (Canfield Imaging). Eighteen linear facial distances were measured using Mirror 3D analysis (Canfield Imaging Systems). Difference between ethnicities was measured using analysis of variance with the Bonferroni/Dunn post hoc comparisons. Pearson correlation was employed for interrater reliability. All statistical analyses were carried out using SPSS version 21.0 (IBM Corp), with statistical significance set at P < .05. RESULTS: Nasal projection (sn-prn) and columella length (sn-c) did not differ significantly between groups (P = .9). Significant differences were seen between ethnic groups in nasal width (sbal-sbal [C-AA; P = .02]; ac-ac [C-AA; P = .00; H-AA; P = .04]; al-al [C-AA; P = .00; H-AA; P = .001]) and labial length (sn-ls [C-AA; P = .041]; sn-sto [C-AA; P = .005]; Cphs-Cphi L [C-AA; P = .013]; Cphs-Cphi R [C-AA; P = .015]). Interrater reliability was good to excellent and significantly correlated for all measures. CONCLUSIONS: African American infants exhibited wider noses and longer lips. No difference was noted in nasal projection or columella length, indicating that these structures should be corrected during the primary cleft lip and nasal repair for all patients and should not be deferred to secondary correction.

Superimposed linear atopic dermatitis treated with dupilumab.

Common polygenic skin disorders, such as atopic dermatitis, may rarely present in a segmental or linear distribution due to cutaneous mosaicism. Only seven cases of superimposed linear atopic dermatitis have been reported to date. Here, we present a child with severe superimposed linear atopic dermatitis and highlight the first successful use of dupilumab in its treatment.

Caring for Pediatric Hidradenitis Suppurativa Patients in the Emergency Department.

ABSTRACT: Hidradenitis suppurativa (HS) is a chronic and recurrent inflammatory skin condition resulting in the formation of nodules, sinus tracts, and abscesses in intertriginous areas. We provide recommendations for the management of children presenting to the emergency department with acute HS flares, based on a review of literature and insights from our own clinical experience. The purpose of the recommendations is to educate clinicians on specific considerations that should be made when caring for children with HS.

Alveolar soft-part sarcoma: Case demonstrating principles for uncommon vascular lesions.

BACKGROUND: Alveolar soft-part sarcoma (ASPS) is an uncommon malignancy that may present in a manner similar to benign vascular tumors. METHODS: A 6-year-old boy with autism spectrum disorder was referred to the Johns Hopkins All Children's Hospital vascular anomalies clinic for the evaluation of a tongue mass. RESULTS: Prior to the presentation, at 5 years of age, neck computed tomography (CT) was performed. This showed a well circumscribed, enhancing mass at the anterior aspect of the tongue. The radiologic impression was that this lesion was most likely a hemangioma. Two years later, the patient was evaluated in the vascular anomalies clinic. At that examination a 2-3 cm swelling was noted on the dorsal aspect of the tongue. The mass was fleshy and firm with discrete borders. Handheld Doppler examination indicated a high-flow lesion. The patient underwent an excisional biopsy. The lesion was identified as an alveolar soft-part sarcoma based on pathologic characteristics. CONCLUSION: Familiarity with common vascular tumors and malformations allows providers to diagnose the majority of these lesions on a combination of clinical history and physical examination. Atypical and combined lesions do benefit from imaging to help characterize and aid in the differential diagnosis. Biopsy enables definitive diagnosis but is necessary in the minority of cases. When in doubt, referral to a specialized, multidisciplinary vascular anomalies clinic will ensure that these patients receive management for this challenging collection of conditions.

Improving Diagnostic Accuracy in Skin of Color Using an Educational Module.

Dermatologic disparities disproportionately affect patients with skin of color (SOC). This study evaluated the effectiveness of a focused educational module for improving diagnostic accuracy and confidence in the treatment of patients with SOC among interprofessional health care providers. An SOC educational module involving a pretest, 15-minute lecture, immediate posttest, and 3-month posttest was created. One hundred participants completed the pretest and immediate posttest; 36 of them also completed the 3-month post-test. Our results suggest that a focused educational module may provide long-term improvements in diagnostic accuracy and confidence for conditions presenting in patients with SOC.

A case of multiple, bilateral Becker's Nevi of the trunk and lower extremities in a young girl: A case report.

A Becker's nevus typically appears as a single unilateral, well-demarcated, hyperpigmented patch over the upper trunk during adolescence. It uncommonly presents as multiple and bilateral patches and rarely involves a lower extremity. We describe the unusual case of a child with multiple, bilateral Becker's nevi of the trunk and lower extremities present since birth.

Caregiver Preferences and Barriers Toward Accessing Pediatric Dermatology Care for Childhood Atopic Dermatitis.

INTRODUCTION: Atopic dermatitis (AD) is a common chronic childhood illness. It is often treated by primary care providers (PCPs) though it may require referral to a dermatology specialist. METHOD: We administered an exploratory survey to 50 caregivers of children aged 0-17 years with AD to assess their preferences and barriers toward accessing dermatology specialty care for their child's AD. RESULTS: Caregivers felt PCPs and specialists equally listened to their child's AD concerns. However, many felt there was a difference in the care provided and control of the AD and preferred to see a specialist for ongoing management. DISCUSSION: Caregivers may benefit from their children being referred to dermatology specialists earlier and more often for their AD. Further work must be done to characterize preferences and barriers toward AD care across race and ethnicity.

Corticosteroids for Acute Orbital Cellulitis.

OBJECTIVES: Standard treatment of children hospitalized for acute orbital cellulitis includes systemic antibiotics. Recent data from single-center studies suggest the addition of systemic corticosteroids may hasten clinical improvement and reduce hospital length of stay (LOS). We investigate the potential relationship between corticosteroid exposure and duration of hospitalization for pediatric orbital cellulitis. METHODS: Using Pediatric Health Information System registry data from 51 children's facilities, we performed a retrospective cohort study of children hospitalized for orbital cellulitis <18 years of age from 2007 to 2018. The primary study outcome was hospital LOS. Secondary outcomes included frequency of surgical interventions, PICU admission, and 30-day related-cause readmission. RESULTS: Of the 5645 children included for study, 1347 (24%) were prescribed corticosteroids within 2 days of admission. Corticosteroid prescription was not associated with LOS in analyses adjusted for age; presence of meningitis, abscess, or vision issues; and operative episode and PICU admission within 2 days (e ß = 1.01, 95% confidence interval [CI]: 0.97-1.06). Corticosteroid exposure was associated with operative episodes after 2 days of hospitalization (odds ratio = 2.05, 95% CI: 1.29-3.27) and 30-day readmission (odds ratio = 2.40, 95% CI: 1.52-3.78) among patients with a primary diagnosis of orbital cellulitis. CONCLUSIONS: In this database query, we were not able to detect a reduction in LOS associated with corticosteroid exposure during hospitalization for orbital cellulitis. Corticosteroid prescription was associated with PICU admission and operative episodes after 2 days of hospitalization. Before the adoption of routine corticosteroid use, prospective, randomized control trials are needed.