Faculty Bibliography

BACKGROUND:Pediatric skin-limited discoid lupus erythematosus (DLE-only) is rare, with limited data on risk factors for progression to systemic lupus erythematosus (SLE). OBJECTIVE:To assess incidence, risk factors, and phenotype of pediatric DLE-only progression to SLE. METHODS:In this 17-site retrospective cohort of pediatric DLE, the primary outcome was time to SLE diagnosis (ACR classification criterion ≥4). Kaplan-Meier estimates for 1-, 2-, and 5-year progression to SLE were generated. Cox proportional hazards modeling identified baseline predictors of progression to SLE. RESULTS:The 1-year progression rate from DLE-only to SLE was 14.4% (95% CI: 9.6-18.9%). Progression to SLE was most strongly associated with baseline ANA positivity (HR 3.71) and older age (HR 1.11/yr). Antiphospholipid antibodies and cytopenias also predicted progression to SLE in multivariable analysis. The SLE phenotype was relatively mild, with most patients developing mucocutaneous and laboratory criteria (22/236, 9%) and few developing other end-organ disease (7/236, 3%). LIMITATIONS/CONCLUSIONS:Retrospective design, missing data. CONCLUSION/CONCLUSIONS:ANA-positive DLE-only patients warrant close monitoring for progression SLE, especially within the first year. Severe end-organ disease in DLE patients who progress to SLE is uncommon. Future studies should test whether early recognition and intervention in DLE-only slows progression to SLE.

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.