Management of Erythromelalgia: a narrative review and expert opinion

Erythromelalgia (EM) is a rare clinical entity predominantly affecting distal extremities characterized by episodes of painful burning erythema, often precipitated by heat and exercise. Despite its significant impact on quality of life, no guidelines exist for treatment of this complex disorder, and management remains challenging due to limited evidence.

This article provides an overview of the available literature on EM, with special emphasis on management strategies including lifestyle modification, nonpharmacologic, pharmacologic, procedural, and rehabilitative interventions. PubMed was searched using the advanced search function with the terms 'erythromelalgia AND therapies AND management.' No date restrictions were applied, and the search included articles available from database inception through 16 April 2026.

Management of EM is challenging as there is no cure. It requires an individualized step wise approach due to its heterogenous presentation and variable treatment responses. The primary goal of treatment is to control symptoms and maintain quality of life. In this article, we outline our treatment approach for management including an initial evaluation for underlying causes or associations and treating them if present. First-line therapies include lifestyle modifications, trigger avoidance, and trial of various topical therapies. If not fully responding to first line therapy, we consider initiation of systemic therapies and pain rehabilitative programs.