Algarni Abdullah S, Alharthi Reem M, Alqurashi Shaden O, Alghanmi Ruba M, Aldawsari Rimaz R, Alghamdi Maysaa A, Samargandi Ramy
Department of Medicine, College of Medicine, University of Jeddah, Jeddah 23218, Saudi Arabia.
Faculty of Medicine, Al-Baha University, Al-Aqiq 65522, Saudi Arabia.
Medicina (Kaunas). 2025 May 19;61(5):920. doi: 10.3390/medicina61050920.
: Erythromelalgia (EM) is an uncommon condition marked by recurring redness, intense burning sensations, and elevated limb warmth. This syndrome can be significantly debilitating, and finding effective treatment options often proves to be quite difficult. The symptoms can severely impact the quality of life of those affected, resulting in considerable disability. This systematic review aims to compare available medical treatments for EM by evaluating their efficacy and safety. : Following PRISMA guidelines, the search included the PubMed, Medline, and Web of Science databases, using the keywords ("Erythromelalgia" OR "Mitchell's Disease") AND ("Erythromelalgia Treatment" OR "Erythromelalgia Management"). : From the 103 papers extracted through the database search, six articles were considered suitable for the systematic review. The included studies investigated various interventions used for a total of 120 patients, including iloprost ( = 8), misoprostol (n = 21), topical amitriptyline-ketamine ( = 36), lidocaine ( = 27), chemical lumbar sympathectomy (CLS, = 13), and various pharmacological agents ( = 11). The outcomes showed significant improvements in areas like pain reduction, cooling scores, and temperature regulation. Iloprost and misoprostol exhibited notable benefits in cooling scores, sympathetic dysfunction, and EM severity compared to placebos. About 75% of the patients reported pain relief with topical amitriptyline-ketamine, while lidocaine reduced nociceptive feelings in a dose-dependent manner. : Comparing interventions demonstrated consistent clinical benefit with varied tolerability. However, adverse events ranged from mild gastrointestinal symptoms to severe complications such as disability and depression, requiring careful monitoring. Given EM's diverse symptoms and comorbidities, treatment efficacy varies among individuals. A personalized approach incorporating genetic testing, multidisciplinary care, and long-term monitoring is essential to optimize outcomes. Continued research is vital to advance understanding of EM's pathophysiology and improve patient care.
红斑性肢痛症（EM）是一种罕见病症，其特征为反复出现的皮肤发红、强烈的灼烧感以及肢体温度升高。这种综合征可能会使人严重衰弱，而且往往很难找到有效的治疗方法。这些症状会严重影响患者的生活质量，导致相当程度的残疾。本系统评价旨在通过评估现有医学治疗方法的疗效和安全性来对其进行比较。：遵循PRISMA指南，检索范围包括PubMed、Medline和科学网数据库，使用的关键词为（“红斑性肢痛症”或“米切尔病”）以及（“红斑性肢痛症治疗”或“红斑性肢痛症管理”）。：通过数据库检索提取的103篇论文中，有6篇文章被认为适合进行系统评价。纳入的研究调查了针对总共120名患者使用的各种干预措施，包括伊洛前列素（n = 8）、米索前列醇（n = 21）、局部用阿米替林 - 氯胺酮（n = 36）、利多卡因（n = 27）、化学性腰交感神经切除术（CLS，n = 13）以及各种药物制剂（n = 11）。结果显示在疼痛减轻、降温评分和体温调节等方面有显著改善。与安慰剂相比，伊洛前列素和米索前列醇在降温评分、交感神经功能障碍和EM严重程度方面表现出显著益处。约75%的患者报告局部用阿米替林 - 氯胺酮可缓解疼痛，而利多卡因以剂量依赖方式减轻伤害性感受。：比较不同干预措施显示出一致的临床益处，但耐受性各不相同。然而，不良事件范围从轻微的胃肠道症状到严重并发症，如残疾和抑郁，需要仔细监测。鉴于EM症状多样且存在合并症，治疗效果因人而异。采用个性化方法，包括基因检测、多学科护理和长期监测，对于优化治疗结果至关重要。持续研究对于增进对EM病理生理学的理解和改善患者护理至关重要。
