BACKGROUND: 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.

CASE REPORT: 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.

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

KEYWORDS: Primary erythromelalgia, case report, peripheral nerve stimulation, sciatic nerve block