Comparative Efficacy and Tolerability of Treatments for Erythromelalgia: A Systematic Review

<i>Background and Objectives</i>: 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....

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Main Authors: Abdullah S. Algarni, Reem M. Alharthi, Shaden O. Alqurashi, Ruba M. Alghanmi, Rimaz R. Aldawsari, Maysaa A. Alghamdi, Ramy Samargandi
Format: Article
Language:English
Published: MDPI AG 2025-05-01
Series:Medicina
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Online Access:https://www.mdpi.com/1648-9144/61/5/920
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author Abdullah S. Algarni
Reem M. Alharthi
Shaden O. Alqurashi
Ruba M. Alghanmi
Rimaz R. Aldawsari
Maysaa A. Alghamdi
Ramy Samargandi
author_facet Abdullah S. Algarni
Reem M. Alharthi
Shaden O. Alqurashi
Ruba M. Alghanmi
Rimaz R. Aldawsari
Maysaa A. Alghamdi
Ramy Samargandi
author_sort Abdullah S. Algarni
collection DOAJ
description <i>Background and Objectives</i>: 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. <i>Materials and Methods</i>: 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”). <i>Results</i>: 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 (<i>n</i> = 8), misoprostol (n = 21), topical amitriptyline-ketamine (<i>n</i> = 36), lidocaine (<i>n</i> = 27), chemical lumbar sympathectomy (CLS, <i>n</i> = 13), and various pharmacological agents (<i>n</i> = 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. <i>Conclusions</i>: 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.
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spelling doaj-art-6084ea1d6fef44aab2f26ed31db048f22025-08-20T03:48:02ZengMDPI AGMedicina1010-660X1648-91442025-05-0161592010.3390/medicina61050920Comparative Efficacy and Tolerability of Treatments for Erythromelalgia: A Systematic ReviewAbdullah S. Algarni0Reem M. Alharthi1Shaden O. Alqurashi2Ruba M. Alghanmi3Rimaz R. Aldawsari4Maysaa A. Alghamdi5Ramy Samargandi6Department of Medicine, College of Medicine, University of Jeddah, Jeddah 23218, Saudi ArabiaFaculty of Medicine, Al-Baha University, Al-Aqiq 65522, Saudi ArabiaCollage of Medicine, Taif University, Taif 21944, Saudi ArabiaCollege of Medicine, University of Jeddah, Jeddah 23218, Saudi ArabiaCollege of Medicine, University of Jeddah, Jeddah 23218, Saudi ArabiaCollege of Medicine, University of Jeddah, Jeddah 23218, Saudi ArabiaDepartment of Surgery, College of Medicine, University of Jeddah, Jeddah 23218, Saudi Arabia<i>Background and Objectives</i>: 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. <i>Materials and Methods</i>: 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”). <i>Results</i>: 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 (<i>n</i> = 8), misoprostol (n = 21), topical amitriptyline-ketamine (<i>n</i> = 36), lidocaine (<i>n</i> = 27), chemical lumbar sympathectomy (CLS, <i>n</i> = 13), and various pharmacological agents (<i>n</i> = 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. <i>Conclusions</i>: 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.https://www.mdpi.com/1648-9144/61/5/920erythromelalgiatreatment efficacymanagementtolerabilitypain managementsystematic review
spellingShingle Abdullah S. Algarni
Reem M. Alharthi
Shaden O. Alqurashi
Ruba M. Alghanmi
Rimaz R. Aldawsari
Maysaa A. Alghamdi
Ramy Samargandi
Comparative Efficacy and Tolerability of Treatments for Erythromelalgia: A Systematic Review
Medicina
erythromelalgia
treatment efficacy
management
tolerability
pain management
systematic review
title Comparative Efficacy and Tolerability of Treatments for Erythromelalgia: A Systematic Review
title_full Comparative Efficacy and Tolerability of Treatments for Erythromelalgia: A Systematic Review
title_fullStr Comparative Efficacy and Tolerability of Treatments for Erythromelalgia: A Systematic Review
title_full_unstemmed Comparative Efficacy and Tolerability of Treatments for Erythromelalgia: A Systematic Review
title_short Comparative Efficacy and Tolerability of Treatments for Erythromelalgia: A Systematic Review
title_sort comparative efficacy and tolerability of treatments for erythromelalgia a systematic review
topic erythromelalgia
treatment efficacy
management
tolerability
pain management
systematic review
url https://www.mdpi.com/1648-9144/61/5/920
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