Ziółkowska Karolina, Słaboń Anna, Glik Justyna, Maj Mariusz, Olszak Magdalena, Mikuś-Zagórska Karolina, Strzelec Przemysław, Czerny Katarzyna, Maciejowski Ryszard, Gierek Marcin, Łabuś Wojciech
Stanislaw Sakiel Burn Treatment Center in Siemianowice Śląskie, Jana Pawła II Street 2, 41-100 Siemianowice Śląskie, Poland.
Doctoral School of the Medical University of Silesia in Katowice, Faculty of Health Sciences in Bytom, Medical Uniwersity of Silesia in Katowice, 41-902 Bytom, Poland.
Medicina (Kaunas). 2025 Mar 21;61(4):554. doi: 10.3390/medicina61040554.
: 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. : 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. : 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. : 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).
埃勒斯-当洛综合征（EDSs）是一组异质性单基因结缔组织疾病（如关节活动过度和脱位、皮肤弹性过度和脆弱、慢性疼痛、伤口愈合延迟等）。本研究的主要目的是为一名被诊断患有EDS的烧伤女性患者介绍一种专门的治疗性伤口管理流程。
一名34岁女性患者因生物壁炉爆炸导致大面积热烧伤。该患者有EDS家族病史。此外，患者精神状态不佳，自2020年以来一直在接受抗抑郁和抗焦虑药物的药物治疗。这可能是世界上首例此类临床观察，但已观察到精神科药物使用与EDS伤口愈合受损之间存在关联。在住院过程中，患者接受了一系列旨在最快、最有效闭合伤口的手术。在我们的机构住院182天后，患者出院回家。
在患者住院期间，患者接受了多次坏死组织清创手术。此外，对伤口进行了异体脱细胞真皮基质（ADM）移植，并采用MEEK技术进行了皮肤移植。使用了体外培养的皮肤细胞作为先进治疗药品（ATMPs）。在患者住院期间，使用低能量激光散斑对比分析（LASCA）拍摄图像以评估微灌注情况或是否存在微灌注不足。测量每隔几天进行一次。
EDS患者烧伤创面的治疗需要较长的住院时间。还可能需要采用创新制剂（如ADM和ATMP）的多阶段方法。可以使用先进设备，如激光散斑对比分析（LASCA）来评估伤口愈合进展。
