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Current pain management strategies for patients with erythromelalgia: a critical review

Authors Tham SW, Giles M

Received 29 April 2018

Accepted for publication 6 June 2018

Published 30 August 2018 Volume 2018:11 Pages 1689—1698


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 2

Editor who approved publication: Dr Katherine Hanlon

See Wan Tham,1,2 Marian Giles1,2

1Seattle Children’s Research Institute, Seattle, WA, USA; 2Department of Anesthesiology and Pain Medicine, University of Washington School of Medicine, Seattle, WA, USA

Abstract: Erythromelalgia (EM) is a rare disorder characterized by erythematous, warm, painful extremities, which is often precipitated by cold conditions. The pathophysiology of EM is incompletely understood. Recent investigations have identified sodium channelopathy as a genetic cause for this pain condition, classified as primary inherited EM. Other subtypes are idiopathic EM and secondary EM. The management of pain in EM is challenging as no single therapy has been found to be effective. There is varying response to pharmacotherapy and significant variability within this clinical population, resulting in a stepwise trial and error approach. Consequently, EM is often associated with poorer health-related quality of life with higher morbidity. There is currently no consensus or guidelines on management of pain in EM. This is a review of the literature on management of pain using pharmacologic, procedural intervention and nonpharmacologic treatment in children and adults with EM.

Keywords: erythromelalgia, pain, voltage gated sodium channel, therapy

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