ESRA Academy. cuevas cairo i. Sep 8, 2016; 138530; 0395 Topic: Chronic Pain Management
Dr. ilmari cuevas cairo
Dr. ilmari cuevas cairo

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Background and Aims:

Primary Erythromelalgia (PE) is a rare autosomal dominant neuropathy (from 0.36 to 1.1 per 100,000) associated to a catastrophic evolution1. We present a critical case of PE successfully treated with the epidural analgesia.


Eighteen ages, woman, admitted in our hospital with triad burning pain (NRS=7), redness, and warmth of  feet since 14 days. Therapy and tests are summarized in Figures.  

At day 11 patient reported a NRS=10 with an overall worsening, and the APS team was involved. PE was diagnosed and the treatment was implemented with Morphine and Lidocaine patches without significant benefits. After an escalation of opioids administration and a worsened liver function, an epidural analgesia was thought. At day 51, after a patient written consent the epidural catheter was inserted at L4-L5 interspace and Ropivacaine 0.2% 10 mL was injected. After 20' patient referred a NRS=3. A continuous infusion of Ropivacaine 0.1% (baseline 5mL/h-1, bolus 3mL, lock out 20 minutes) was started. The days after,  pain and symptoms were regressed, and after further 4 days patient reported NRS=0 and started to walk. Morphine was gradually decreased and discontinued at day 59. The epidural catheter was removed at day 64 and patient discharged asymptomatic. Six months follow-up was negative.


The pathophysiological pathway of PE is unknown. It is caused by a mutation in SCN9A the encoding gene of sodium channel2.


Epidural block have been proved to have an immunomodulatory activity3 that could have contributed to the resolution of inflammation and symptoms, resulting highly recommended  in treatment of severe PE.

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