Davis, Dawn Marie R.

Objective Dermoid cyst (DC) is a congenital cyst with the potential to have intracranial extension (ICE). This study aims to evaluate the imaging yield and surgical outcomes of nasal, medial brow, forehead, and scalp DCs. Design Retrospective review of craniofacial DCs treated at our institution between 1992 and 2024. Results A total of 117 patients (57 females) were included. The median age at cyst detection and removal were 4.8 months (IQR 3.6-9.6) and 1.8 years (IQR 0.9-5.3), respectively. In 42 patients, parents have noticed the presence of the cyst immediately after birth. Cyst wall rupture during surgical removal was reported in 15.4%. The median follow-up time was 1.3 months (IQR 0.5-12.2). Three patients experienced recurrence. No postoperative complication was reported. The regions with the highest prevalence of ICE were the forehead, frontotemporal scalp, and nasal region. The lateral frontal/temporal scalp had a 33.3% rate of ICE. Midline forehead/scalp lesions demonstrated a higher risk of ICE compared to their lateral equivalents (54.5% vs 17.5%, P = .03). The sensitivity and specificity of magnetic resonance imaging (MRI) were 100% and 95.7%, while for computed tomography (CT scans) were 72.7% and 96.5%. The Area Under the Curve for MRI was 0.978, and for CT was 0.846. The sensitivity and specificity of ultrasound were 50% and 100%. Conclusions Midline forehead/scalp DCs are more prone to extend intracranially than lateral DCs. MRI had a higher sensitivity and specificity than CT scans in detecting ICE. Routine screening imaging should be considered in midline forehead/scalp, lateral frontal/temporal, and nasal DCs.

AbstractAn 11‐year‐old female was referred from an outside institution after a diagnostic biopsy and subsequent excision of a progressively enlarging reddish‐brown nodule demonstrated features concerning for a balloon cell nevus with severe atypia versus a high‐grade melanocytoma. Upon review of the initial biopsy specimen and molecular data, we favored the diagnosis to be consistent with a high‐grade melanocytoma with balloon cell changes while considering the possibility of balloon cell melanoma due to concerning histopathologic and genetic abnormalities. In this case study, we discuss critical diagnostic considerations in this rare pediatric case and highlight important pathologic and clinical features of melanocytomas and balloon cell melanoma.

AbstractLinear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disorder impacting children and adults. In this single‐center retrospective chart review of pediatric patients with LABD at a large tertiary referral center, we report the unifying and unique clinical features of 10 pediatric patients. Patients typically presented with the “cluster of jewels” sign (n = 6; 60%), mucous membrane involvement (n = 5; 50%) and had a mean disease duration of 38 months; six patients (60%) required inpatient admission for management of their skin disease, including all five patients who had mucous membrane involvement. Our findings suggest that pediatric LABD may be a disease with high morbidity and may be associated with severe complications when mucous membranes are involved.

AbstractBackgroundLinear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disorder that may be drug‐induced or paraneoplastic. We aim to characterize features of LABD and determine differentiating factors among idiopathic, drug‐induced, or malignancy‐associated diseases.MethodsWe conducted a single‐center retrospective chart review of adult patients with linear IgA bullous dermatosis at a large tertiary referral center and a literature review of adult linear IgA bullous dermatosis.ResultsEighty‐one patients were included in the study. Ten patients (12.3%) had comorbid malignancy and nine (11.1%) had inflammatory bowel disease. Median disease duration was significantly shorter in both drug‐induced (1.2 vs. 48.8 months; P < 0.001) and malignancy‐associated (1.7 vs. 48.8 months; P < 0.001) LABD compared with idiopathic LABD. Recurrent episodes occurred significantly more often in idiopathic LABD compared to those with drug‐induced (76.1 vs. 11.5%; P < 0.001) or malignancy‐associated disease (76.1 vs. 33.3%; P = 0.019). Time to diagnosis was significantly shorter in the drug‐induced (0.2 vs. 5.4 months; P < 0.001) and malignancy‐associated groups (0.7 vs. 5.4 months; P = 0.049) compared with idiopathic; similarly, time to improvement was significantly shorter in both drug‐induced (0.4 vs. 3.0 months; P < 0.001) and malignancy‐associated disease (1.1 vs. 3.0 months; P = 0.016). Clinical morphology was indistinguishable between groups. Limitations included retrospective data collection, data from tertiary referral centers, and limited racial and ethnic diversity.ConclusionScreening for underlying malignancy, as well as for a predisposing medication or possibly inflammatory bowel disease, may be advisable in patients with LABD, particularly when it is newly diagnosed.

AbstractBackgroundBier anemic spots, cyanosis with urticaria‐like eruption (BASCULE) syndrome is a recently described entity with episodic urticarial lesions and white anemic halos on a background of erythrocyanosis, commonly affecting the lower extremities. Possible association with autonomic dysfunction remains poorly understood. Existing publications are limited, but the condition is suggested as highly underrecognized.ObjectiveTo further characterize clinical and epidemiologic data for BASCULE syndrome.MethodsWe performed an IRB‐approved retrospective chart review on patients with BASCULE syndrome evaluated at Mayo Clinic from April 2021 to November 2022.ResultsA total of 17 patients were identified (13 female, 4 male). Median age of onset was 12 years (range 9–17). Lower extremities were involved in all patients (17). Most patients were symptomatic with pruritus (8) or burning pain (8); three were asymptomatic. Triggers were standing (11), hot showers or hot environments (7), or no clear trigger (4). Autonomic dysfunction was present in 10 patients. Treatment responses were observed from propranolol (3) and high‐dose cetirizine (1).ConclusionNovel epidemiologic data from 17 pediatric and young adult patients with BASCULE syndrome further supports an association with autonomic dysfunction and suggests a higher prevalence than previously acknowledged.

AbstractBackgroundFollicular mucinosis (FM) is a rare disease characterized by mucin accumulation in the follicular unit. FM's etiology is still widely debated since its first description in 1957. Follicular mucinosis is usually reported to be benign in children, although reports of malignant transformation, most commonly mycosis fungoides, exist. The present project aims to demonstrate that children with a diagnosis of follicular mucinosis have positive long‐term outcomes and do not develop mycosis fungoides.Materials and methodsThis is a retrospective cohort study where patients with a diagnosis of follicular mucinosis ages 22 years and below were identified. Data surrounding the patient's diagnosis of FM, differential diagnosis, treatments, and long‐term outcomes were collected. Patients who were lost to follow‐up were contacted by phone for an update on the status of their skin and overall health.ResultsOut of 14 patients with follow‐up information, none developed subsequent mycosis fungoides or other hematologic malignancies.ConclusionPediatric patients with follicular mucinosis will likely present with limited disease and not experience malignant transformation.