Successful Novel Treatment of Primary Erythromelalgia with Popliteal Sciatic Nerve Block and Peripheral Nerve...

Primary erythromelalgia (PE) is a rare, hereditary disorder characterized by chronic burning pain in the extremities and commonly linked to mutations in the SCN9A gene. The management of erythromelalgia-associated pain remains challenging, since there are currently no standardized guidelines for the treatment thereof due to the rare and refractory nature of the disorder.

A 17-year-old boy with SCN9A-related PE presented with intractable bilateral lower extremity pain complicated by ulcerations and osteomyelitis. After not responding to multiple medications and interventions, he experienced significant relief upon receiving a popliteal sciatic nerve block effected with lidocaine and triamcinolone. Afterward, peripheral nerve stimulation (PNS) was implanted, leading to lasting pain relief, improved functionality, and wound healing for the patient.

PNS offers a promising treatment for refractory PE, warranting further investigation to establish the role of this technique in managing this challenging condition.