Erythromelalgia: Pathophysiology and Clinical Treatment Options, a Narrative Review

Erythromelalgia (EM) is an uncommon, multifaceted neurovascular pain disorder. The distal extremities are typically affected, with symptoms of overwhelming warmth, burning pain, and erythema. There is a wide range of presentations with varying severities and courses, including occasional flares, intermittent episodes, and chronic debilitating discomfort. In this narrative review, we aim to raise awareness of EM and synthesize the current state of knowledge through exploring pathophysiology, clinical presentation, differential diagnoses, and treatment options for EM.

Patients adapt by avoiding triggers, such as excessive heat exposure and exercise, and utilizing various cooling and relaxation methods to seek relief. Physicians individualize treatment based on the unique patient presentation to focus on symptom management: lifestyle changes, pharmacologic methods, pain-relieving medication (aspirin), sodium channel blockers (lidocaine, bupivacaine), and procedural strategies such as botulinum toxin injection or dorsal root ganglion stimulation. EM is classified as either primary, secondary, or idiopathic. Customized approaches for pain management are necessary for the differing presentations of EM. However, diagnosing EM is challenging because there are many overlapping features with other conditions, for example, cellulitis and complex regional pain syndrome. Differential diagnoses and the process of elimination are crucial to the diagnosis of EM. Future investigations should more clearly outline the diagnostic criteria and develop effective targeted treatments for EM. While large-scale clinical trials may be unattainable related to the rarity of EM, the field requires smaller studies and further research to fill these knowledge gaps.