LB1536 A case of familial focal dermal hypoplasia: A report of 3 cases in consecutive generations

Abstract

Patients with epidermolysis bullosa (EB) require care of wounds that are often colonized with bacteria. A subset of EB patients are at risk for squamous cell carcinoma (SCC) and certain bacterial-host interactions have been implicated in this risk. The EB Clinical Characterization and Outcomes Database serves as a repository of information from EB patients at multiple centers in the US and Canada. Using this resource, we conducted a retrospective analysis of 739 wound culture results reported from 159 patients between 2001 and 2017 in the Research Electronic Data Capture Research Electronic Data Capture registry. No growth was reported in 98 (13%) cultures while 641 (87%) cultures grew at least one microbial species. Staphylococcus aureus (SA) grew in 132 (83%) patients, Pseudomonas aeruginosa (PA) in 56 (35%) patients, group A Streptococcus in 32 (20%) patients, Corynebacterium in 30 (19%) patients and Proteus in 14 (9%) patients. Of 117 patients with recorded SA methicillin susceptibility testing, 68% were MSSA and 47% MRSA. 18 (16%) patients grew both MSSA and MRSA. Of 15 patients with reported mupirocin susceptibility, 11 (64%) were mupirocin susceptible (mupS) and 6 (35%) were mupirocin resistant (mupR). Two patients grew both mupS and mupR isolates. SCC was reported in 22 patients, 10 of whom reported cultures that grew SA, PA and Proteus in 90%, 50% and 20% of cases, respectively. Cultures from SCC negative patients grew SA, PA and Proteus in 83%, 34% and 8% of cases, respectively. This data demonstrates the prevalence of positive cultures including resistant bacteria on EB wounds. Additional studies are needed to determine if wound microbiome interventions inhibit the development of SCC and improve outcomes.