Pediatric Emergency Department Visits with Dystrophic Epidermolysis Bullosa More Frequent in Summer; Hospitalization Rates Exceed 50%

Pediatric patients with dystrophic epidermolysis bullosa (DEB) are more likely to visit hospital emergency departments in the summer months versus young patients without DEB, according to a study published in Pediatric Dermatology.¹

Furthermore, researchers also reported that more than half of patients presenting to emergency departments were ultimately hospitalized.

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Background and Methods

Individuals with DEB face significantly more frequent visits to emergency departments than members of the general population. According to a study published in the Orphanet Journal of Rare Diseases, 22.8% of patients with DEB visited a emergency department during a 12-month follow-up period, with 23.8% requiring an in-patient admission.²

Given this heightened risk of emergency department visits and hospitalization, authors of the present study, Feinstein et al, sought to examine this prevalence in a pediatric population. They noted that no prior research has explored the characteristics and risk factors of patients with DEB in emergency rooms within the US.

Researchers analyzed the US Nationwide Emergency Department Sample data collected between 2015 and 2019, with the exception of 2015, where only fourth-quarter data was included due to coding issues. Patients assessed in the study were under the age of 18 with a diagnosis of DEB. According to researchers, the sample represented nearly a quarter (23%) of US hospital emergency departments.

Findings

Out of a total of 27,223,220 pediatric emergency visits during the study period, 53 visits were associated with DEB, with 7 visits having DEB as the primary diagnosis and 46 visits having DEB as a secondary diagnosis.

DEB-related visits were significantly more frequent in the summer (35.7%) compared to the fall (13.1%), contrasting with non-DEB visits, which were more common in the fall (30.2%) and less frequent in the summer (21.4%).

The cost of an emergency department visit for patients with DEB also averaged significantly higher than non-DEB patients ($5,028.74 versus $2,143.37).

In addition, a significant proportion of patients with DEB (56.2%) were admitted to the hospital, compared to only 3.4% of patients without DEB.

Among visits for patients with a secondary DEB diagnosis, the most frequent primary diagnoses included infectious and gastrointestinal issues. Other notable primary diagnoses included fever (8.8%), constipation (6.6%), and bone marrow transplant aftercare (4.9%).

Patients with DEB also had significantly higher rates of several comorbid conditions compared to their non-DEB counterparts, including gastroesophageal reflux disease (25.2% vs. 0.9%), esophageal obstruction (23.5% vs. 0.006%), iron deficiency anemia (22.2% vs. 0.1%), and constipation (16.9% vs. 7.8%); they were also more prone to complications such as high blood pressure, cellulitis, sepsis, and vitamin D deficiency.

Conclusions

"Through better understanding the characteristics and medical emergencies of patients with DEB in the [emergency department] setting, healthcare providers can improve management and mitigate the high admission rates," according to Molnar et al.

Limitations of the study included its small sample size and the potential of misdiagnosis via database miscoding.

Moving forward, researchers suggested further exploration of additional emergency department databases and the impact of recent or novel therapies in the emergency department.

References

1. Molnar BA, Yang LJ, Paller AS, Ren Z. Increased healthcare burden and comorbidity risks of pediatric patients with dystrophic epidermolysis bullosa: analysis of Nationwide Emergency Department Sample 2015–2019. Pediatr Dermatol. July 25, 2024. https://doi.org/10.1111/pde.15688
2. Feinstein JA, Bruckner AL, Chastek B, Anderson A, Roman J. Clinical characteristics, healthcare use, and annual costs among patients with dystrophic epidermolysis bullosa. Orphanet J Rare Dis. September 29, 2022. doi: 10.1186/s13023-022-02509-0.