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.

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.

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.

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.

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.