Surgical treatment of chronic ulcerative wounds of lower limbs in children with primary erythromelalgia: A case report and literature review

To report a case of primary erythromelalgia with chronic lower limb ulcers treated surgically and to enhance understanding of the disease through a review of relevant literature. A 12-year-old male patient was diagnosed with primary erythromelalgia two years prior. He presented with progressive erythema, worsening edema of both lower limbs, and multiple scattered skin ulcers on his calves and feet. Following admission, he underwent two stages of surgical treatment: debridement with artificial skin coverage in the first stage and autologous skin grafting in the second. Effective pain management was achieved through collaboration with the departments of anesthesiology and pain management. After 27 days of treatment, the patient experienced significant pain relief in both lower limbs, with a 100% survival rate of the transplanted skin grafts. He was discharged upon complete wound healing. Follow-up assessments revealed full resolution of lower limb swelling, substantial pain reduction, and good cosmetic outcomes in the wound repair areas. Primary erythromelalgia is a rare neurovascular disorder primarily associated with autosomal dominant mutations in the SCN9A gene encoding voltage-gated sodium channels, posing significant treatment challenges with no established effective therapies. In this case, multidisciplinary collaboration facilitated effective wound repair and marked pain reduction through surgical intervention and optimal pain management.