Faculty Bibliography

A 4-month-old, full-term girl was referred for evaluation of a supraumbilical raphe and an anomalous aortic arch. Further work up for PHACE(S) syndrome was pursued, despite the absence of a cutaneous infantile hemangioma (IH), which identified hypoplastic and dysplastic cerebral vasculature. We present this case to raise awareness of possible PHACE(S) syndrome even in children without cutaneous IHs and the importance of recognizing supraumbilical raphe as a sentinel finding.

The Patient-Oriented Eczema Measure (POEM) is a validated patient-reported outcome measure for atopic dermatitis (AD), though its use in routine pediatric dermatology practice remains underexplored. In this cross-sectional study of 297 pediatric patients with AD at a tertiary pediatric dermatology clinic, POEM scores were collected and compared with physician-rated investigator global assessment (IGA) and IGA × body surface area, as well as treatment selection. Older patients (> 12 years) had significantly higher mean POEM scores (17.5 vs. 10.4; p < 0.001) with no significant differences observed by race, ethnicity, or socioeconomic status; total POEM scores correlated strongly with IGA (r = 0.68) and moderately with IGA×BSA (r = 0.54), and higher severity scores were associated with more potent topical corticosteroids and systemic treatments (p < 0.001). POEM detected clinically relevant changes, aligned with physician-rated measures, and correlated with treatment intensity, underscoring its utility in both clinical practice and future predictive modeling applications.

BACKGROUND/OBJECTIVE/OBJECTIVE:Juvenile dermatomyositis (JDM) is a systemic autoimmune vasculopathy, which may be complicated by calcinosis of the skin and subcutaneous tissues. Calcinosis is often associated with pain, ulceration, infection, impaired mobility, and reduced quality of life, yet no gold standard exists for its detection and longitudinal monitoring. Current evaluation relies on clinical examination (history plus physical examination) with or without targeted conventional radiography, which may underestimate disease burden and expose children to cumulative doses of ionizing radiation. The EOS 2D/3D imaging system provides rapid, whole-body imaging with substantially reduced radiation exposure. Thus, we sought to explore its utility in assessing calcinosis in JDM. METHODS:In this retrospective case series, we investigated NYU pediatric patients with JDM who underwent EOS imaging for evaluation of calcinosis. EOS images and conventional radiographs were independently reviewed by 2 radiologists blinded to clinical data, with a focus on the anatomic distribution of calcinosis. RESULTS:Seven patients (5 female, 2 male, ages 10 to 17 years) met the inclusion criteria, of whom 6 underwent both EOS and x-ray imaging. EOS imaging accurately identified calcinosis of the trunk and lower extremities in all cases and detected calcinosis not previously appreciated on clinical examination or dedicated radiographs in every patient. In 2 patients, EOS imaging failed to detect all upper-extremity calcinosis, likely due to the use of standard orthopedic positioning. CONCLUSIONS:EOS imaging appears to be a valid alternative to conventional radiography for evaluating calcinosis of the trunk and lower extremities in JDM, while offering the advantages of lower radiation exposure, rapid acquisition, and broader anatomic coverage. Development of JDM-specific positioning protocols may improve the detection of upper-extremity disease.

Primary erythromelalgia (PEM) is a rare neuropathic pain disorder characterized by debilitating burning pain of the extremities relieved by cold exposure. We report a pediatric patient who developed severe, full-thickness lower extremity ulcerations infected with Alternaria alternata and complicated by osteomyelitis following consistent direct skin cooling with window air conditioning. This case demonstrates serious infectious complications that can arise from cooling measures commonly employed for PEM symptom relief.

Striae distensae, or stretch marks, are common dermal scars resulting from skin overstretching. This case series documents the occurrence of severe striae in three pediatric patients with nephrotic syndrome. This report details the clinical manifestations, including widespread and edematous striae, and explores potential etiological factors such as rapid skin stretching from edema and high-dose corticosteroid use underlying this association.

Chemotherapy-induced alopecia and radiation-induced alopecia, the thinning or loss of hair due to cytotoxic chemotherapy and radiation therapy, respectively, are distressing adverse effects of cancer treatment. Chemotherapy, targeted therapies, and radiation therapy used in pediatric oncology often lead to alopecia by damaging hair follicles, with varying degrees of severity depending on the specific treatment type, mechanism of action, and damage-response pathway involved. Pediatric chemotherapy-induced alopecia, radiation-induced alopecia, and permanent alopecia, defined as hair regrowth that remains incomplete 6 months or more after treatment, have significant negative impacts on mental health, self-esteem, and social interactions, highlighting the need for further research into supportive care strategies. There are currently no standard interventions for chemotherapy-induced alopecia or radiation-induced alopecia in children, with most recommendations limited to gentle hair care and camouflaging techniques during treatment. Scalp cooling has demonstrated safety and efficacy in reducing chemotherapy-induced alopecia in adults and is currently under investigation in children and adolescents. Topical and low-dose oral minoxidil have been studied in children for other hair loss disorders and may improve hair regrowth after chemotherapy or radiation. Increased awareness and continued research into management strategies for pediatric chemotherapy-induced alopecia and radiation-induced alopecia are necessary to help mitigate its significant negative impact on quality of life.