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Safe, reliable treatment for pediatric erythromelalgia still needed

The disease course for pediatric erythromelalgia is variable, and a safe and reliable treatment course has yet to be defined, researchers reported in a recent issue of the Journal of the American Academy of Dermatology.

Robert H. Cook-Norris, MD, and colleagues conducted a retrospective review of patients (n=32) aged 5 to 18 years who were diagnosed with erythromelalgia and assessed at the Mayo Clinic in Rochester, between 1970 and 2007. Of the patients, seven had a first-degree relative with erythromelalgia, with four being from the same family.

According to the researchers, noninvasive vascular studies comparing temperature, laser Doppler flow and transcutaneous oximetry in the toes demonstrated vascular abnormalities for 13 of 14 patients. Additionally, neurophysiologic studies with autonomic reflex screening revealed signs of small-fiber neuropathy involving the skin in 10 of 17 patients; however, no evidence of large-fiber neuropathy was found in 20 patients among whom electromyographic and nerve conduction studies were performed. Topical lidocaine was shown to be the most commonly prescribed treatment, used in 44% of patients.

“Fifteen patients were monitored for an average of 9.1 years (median: 5 years; range: 0.4-23.7 years). At last follow-up, five patients had stable disease, four showed improvement, two had resolution, one reported worsening of symptoms, and three had died (one suicide),” the researchers wrote.