Shehata Nancy A, Shaik Noor A, Irfan Thalib Husna
Department of Dermatology, King Abdullah Medical Complex, Jeddah, SAU.
Department of Genetic Medicine, Faculty of Medicine at King Abdul Aziz University, Jeddah, SAU.
Cureus. 2024 Aug 12;16(8):e66678. doi: 10.7759/cureus.66678. eCollection 2024 Aug.
Epidermolysis bullosa (EB) is a genetic skin disorder characterized by skin fragility and blister formation. This review explores the genetic basis and management of EB in the Saudi population, emphasizing the need for genetic insights to enable precise diagnosis, targeted treatments, and effective counseling. Diagnosis in Saudi Arabia relies on clinical assessments and genetic testing. Prenatal diagnosis may be suggested in families with children affected by EB, but it is not widely used in the Middle East. Current management focuses on symptom relief, while emerging experimental approaches such as gene and stem cell therapies are under extensive research. Challenges in EB research include developing effective targeted therapies and understanding the variability in how genotypes manifest phenotypically. Continuous research is crucial to enhance diagnostic methods, therapeutic approaches, and overall patient care.
大疱性表皮松解症(EB)是一种遗传性皮肤病,其特征为皮肤脆弱和水疱形成。本综述探讨了沙特人群中EB的遗传基础和管理,强调了获得遗传见解以实现精确诊断、靶向治疗和有效咨询的必要性。沙特阿拉伯的诊断依赖于临床评估和基因检测。对于有EB患儿的家庭可能会建议进行产前诊断,但在中东地区并未广泛应用。目前的管理重点是缓解症状,而基因和干细胞疗法等新兴实验方法正在进行广泛研究。EB研究中的挑战包括开发有效的靶向疗法以及理解基因型如何在表型上表现出变异性。持续研究对于改进诊断方法、治疗方法和整体患者护理至关重要。