Abstract：
Patients with sickle cell disease (SCD) often experience painful vaso-occlusive crises and chronic haemolytic anaemia, as well as various acute and chronic complications, such as leg ulcers. Leg ulcers are characterized by their unpredictability, debilitating pain and prolonged healing process. The pathophysiology of SCD leg ulcers is not well defined. Known risk factors include male gender, poor social conditions, malnutrition and a lack of compression therapy when oedema occurs. Leg ulcers typically start with spontaneous pain, followed by induration, hyperpigmentation, blister formation and destruction of the epidermis. SCD is characterized by chronic haemolysis, increased oxidative stress and decreased nitric oxide bioavailability, which promote ischaemia and inflammation and consequently impair vascular function in the skin. This cutaneous vasculopathy, coupled with venostasis around the ankle, creates an ideal environment for local vaso-occlusive crises, which can result in the development of leg ulcers that resemble arterial ulcers. Following the development of the ulcer, healing is hindered as a result of factors commonly observed in venous ulceration, including venous insufficiency, oedema and impaired angiogenesis. All of these factors are modulated by genetic factors. However, our current understanding of these genetic factors remains limited and does not yet enable us to accurately predict ulceration susceptibility.
摘要：
镰状细胞病（SCD）患者经常经历痛苦的血管闭塞危象和慢性溶血性贫血，以及各种急性和慢性并发症，如腿部溃疡。腿部溃疡的特点是不可预测性，使人衰弱的疼痛和延长的愈合过程。SCD腿部溃疡的病理生理学尚不明确。已知的危险因素包括男性，恶劣的社会条件,营养不良和水肿时缺乏压迫治疗。腿部溃疡通常以自发性疼痛开始，其次是硬结，色素沉着过度,表皮的水泡形成和破坏。SCD的特点是慢性溶血，氧化应激增加，一氧化氮生物利用度降低，促进缺血和炎症，从而损害皮肤的血管功能。皮肤血管病变,再加上脚踝周围的静脉阻塞,为局部血管闭塞危机创造了理想的环境，这可能导致类似动脉溃疡的腿部溃疡的发展。随着溃疡的发展，由于静脉性溃疡中常见的因素，愈合受到阻碍，包括静脉功能不全,水肿和血管生成受损。所有这些因素都受到遗传因素的调节。然而,我们目前对这些遗传因素的了解仍然有限,尚不能准确预测溃疡易感性.
