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Published on: October 12, 2012
Bloodstream Infection in Children With Epidermolysis Bullosa
Laura García-Espinosa1, Teresa Del Rosal2,3,4,5, Lucía Quintana6
1From the Pediatrics Department, La Paz University Hospital, Madrid, Spain.
Insights
Bloodstream infections (BSI) are a significant concern in children with Epidermolysis Bullosa (EB), often caused by drug-resistant Pseudomonas aeruginosa and Staphylococcus aureus. Early skin cultures can guide sepsis treatment in these vulnerable patients.
Area of Science:
- Pediatric Infectious Diseases
- Dermatology
- Microbiology
Background:
- Epidermolysis Bullosa (EB) is a group of rare genetic skin disorders characterized by fragile skin that blisters and tears easily.
- Skin infections are a common complication in EB, increasing the risk of bacteremia and bloodstream infections (BSI).
- Limited data exists on the characteristics and outcomes of BSI in pediatric EB patients.
Purpose of the Study:
- To describe the epidemiology, causative microorganisms, antimicrobial resistance patterns, and outcomes of bloodstream infections (BSI) in children with Epidermolysis Bullosa (EB).
- To investigate the role of skin cultures in guiding the management of BSI in EB patients.
- To assess the association between BSI and mortality in children with severe EB.
Main Methods:
- Retrospective study of BSI episodes in pediatric patients (0-18 years) with EB treated at a national reference unit in Spain from 2015 to 2020.
- Data collection included patient demographics, EB type, BSI causative agents, antimicrobial susceptibility, and clinical outcomes.
- Correlation between skin culture results and blood culture findings was analyzed.
Main Results:
- 37 BSI episodes were identified in 15 pediatric EB patients, predominantly in those with recessive dystrophic EB (RDEB).
- Pseudomonas aeruginosa and Staphylococcus aureus were the most common pathogens, exhibiting high rates of resistance to common antibiotics, including ceftazidime, meropenem, quinolones, methicillin, and clindamycin.
- Skin cultures, performed in 68% of BSI episodes, identified the same pathogens as blood cultures in 52% of cases, with consistent antimicrobial resistance patterns in 9 isolates.
- Mortality was 10% in the study cohort, with BSI being the cause of death in one case. A history of BSI was associated with increased mortality in severe RDEB.
Conclusions:
- Bloodstream infections represent a significant source of morbidity in children with severe Epidermolysis Bullosa.
- High antimicrobial resistance rates among common pathogens like Pseudomonas aeruginosa and Staphylococcus aureus necessitate careful treatment selection.
- Utilizing skin cultures can aid in optimizing antimicrobial therapy for sepsis management in EB patients.
Background:
Epidermolysis bullosa (EB) is frequently complicated by skin infection, which can lead to bacteremia. However, bloodstream infections (BSI) in patients with EB have not been well described.
Methods:
Retrospective study of BSI in children 0-18 years with EB from a national reference unit in Spain, in 2015-2020.
Results:
Among 126 children with EB, we identified 37 BSI episodes in 15 patients (14 recessive dystrophic EB, 1 junctional EB). The most frequent microorganisms were Pseudomonas aeruginosa (n = 12) and Staphylococcus aureus (n = 11). Five P. aeruginosa isolates were ceftazidime-resistant (42%), 4 of which were also resistant to meropenem and quinolones (33%). As for S. aureus , 4 (36%) were methicillin-resistant and 3 (27%) clindamycin-resistant. In 25 (68%) BSI episodes skin cultures had been performed in the previous 2 months. The most frequent isolates were also P. aeruginosa (n = 15) and S. aureus (n = 11). In 13 cases (52%), smear and blood cultures grew the same microorganism, with the same antimicrobial resistance pattern in 9 isolates. Twelve patients (10%) died during follow-up (9 RDEB and 3 JEB). BSI was the cause of death in 1 case. In patients with severe RDEB, a history of BSI was associated with higher mortality (OR 6.1, 95% CI: 1.33-27.83, P = 0.0197).
Conclusions:
BSI is an important cause of morbidity in children with severe forms of EB. The most frequent microorganisms are P. aeruginosa and S. aureus , with high rates of antimicrobial resistance. Skin cultures can help guide treatment decisions in patients with EB and sepsis.
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