Pain Med Case Rep. 2026 Apr;10(2):115-119.
ABSTRACT
BACKGROUND: Erythromelalgia (EM) is a very rare condition characterized by severe paroxysmal pain in the upper or lower extremities and erythema, resulting in significant morbidity and incapacity. Typically, multiple specialty evaluations rule out competing autoimmune, inflammatory, and neuropathic etiologies. Both primary and secondary causes have been described, including peripheral nerve injury, carpal tunnel syndrome, and its surgical interventions. The condition is linked in primary EM to mutations in the sodium voltage-gated channel alpha subunit 9 gene, which encodes the Nav1.7 voltage-gated sodium channel. Numerous underlying conditions can cause secondary EM, believed to be associated with small fiber neuropathies, including peripheral neuropathy or nerve damage from carpal tunnel syndrome, diabetes, autoimmune diseases, sciatica, and frostbite. The compression and decompression of the median nerve are recognized precipitants of small fiber neuropathy, a proposed mechanism in the pathogenesis of secondary EM. Burning pain, erythema, and heat sensitivity symptom flares are commonly triggered by exertion or heat, relieved with cooling, and are classic manifestations of EM. Peer-reviewed studies and systematic reviews acknowledge that nerve injury can initiate or exacerbate secondary EM.
In summary, EM is marked by recurring redness, intense burning sensations, and elevated limb warmth. The symptoms can severely impact the quality of life of those affected, resulting in considerable disability and suicidal tendencies. Treatment efficacy varies among individuals. A personalized approach incorporating genetic testing, multidisciplinary care, including interventional pain procedures, and long-term monitoring, is essential to optimize patient outcomes.
CASE REPORT: In the following case report, a patient was successfully treated for the condition with dorsal root ganglion (DRG) stimulation. EM can be challenging to treat because no 2 cases are the same. The response to different treatment options varies among individuals, and using interventional pain procedures, such as DRG stimulation, can be a potential strategy for patients suffering from this rare condition.
CONCLUSIONS: DRG stimulation can be an effective and safe treatment for pain related to EM.
PMID:42066262