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Published on: August 6, 2015
The Successful Treatment of a Patient with Ehlers-Danlos Syndrome (EDS) After an Extensive Burn Injury: A Case Report
Karolina Ziółkowska1, Anna Słaboń1,2, Justyna Glik1,3
1Stanislaw Sakiel Burn Treatment Center in Siemianowice Śląskie, Jana Pawła II Street 2, 41-100 Siemianowice Śląskie, Poland.
Abstract:
Introduction: Ehlers-Danlos Syndromes (EDSs) are a heterogeneous group of monogenic connective tissue disorders (e.g., joint hypermobility and dislocation, skin hyperelasticity and fragility, chronic pain, delayed wound healing process,, etc.). The primary objective of this study was to present a specialized therapeutic wound management process for a burn-injured female patient diagnosed with EDS. Case Presentation: A 34-year-old female patient presented with extensive thermal burns (biofireplace explosion). The patient had a family history of diagnosed EDS. Additionally, the patient was in a poor mental condition and, since 2020, had been undergoing pharmacotherapy with antidepressant and anti-anxiety medication. This might be the first such clinical observation in the world, but a correlation has been observed between psychiatric medication use and EDS wound healing impairment. During the hospitalization process, the patient underwent a series of surgeries aimed at the fastest and most effective closure of wounds. The patient, after 182 days of hospitalization in our facility, was discharged home. Materials and Methods: During the patient's hospital stay, the patient underwent multiple procedures involving debridement of necrotic tissues. Additionally, allogeneic acellular dermal matrix (ADM) grafting was performed on the wounds, and a procedure was conducted in which skin was grafted using the MEEK technique. The in vitro cultured skin cells, as the advanced therapy medicinal products (ATMPs), were used. During the patient's stay in the hospital, images were taken using low-energy laser speckle contrast analysis (LASCA) to asses microperfusion or lack thereof. The measurements were taken at intervals of several days. Conclusions: The treatment of burn wounds in patients with EDS requires a long hospitalization period. It also may require a multi-stage approach utilizing innovative preparations (e.g., ADMs and ATMPs). The assessment of wound healing progress can be performed using advanced equipment, such as laser speckle contrast analysis (LASCA).
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