The Korean Journal of Pain

The Korean Pain Society (대한통증학회)
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Sympathetic Block for Treating Primary Erythromelalgia

  • Bang, Yoo-Jin (Department of Anesthesiology and Pain Medicine, Kyungpook National University of Hospital) ;
  • Yeo, Jin-Seok (Department of Anesthesiology and Pain Medicine, Kyungpook National University of Hospital) ;
  • Kim, Si-Oh (Department of Anesthesiology and Pain Medicine, Kyungpook National University of Hospital) ;
  • Park, Young-Hoon (Department of Anesthesiology and Pain Medicine, Kyungpook National University of Hospital)
  • Received : 2009.07.30
  • Accepted : 2009.09.07
  • Published : 2010.03.01
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Abstract

Primary erythromelalgia is a rare condition that’s characterized by erythema, an increased skin temperature and burning pain in the extremities. The pain is often very severe, and treating erythromelalgia is frustrating and difficult. We report here on the case of a 12-year old girl with primary erythromelalgia in both lower extremities. The pain was refractory to medical treatment, but a bilateral sympathetic block with lidocaine and triamcinolone resulted in relief from the pain. Our experience with this disease demonstrates that sympathetic blocks are effective in improving the symptoms and they may be attempted on erythromelalgia patients who do not respond to other treatments, including medication and epidural blocks.

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References

  1. Rauck RL, Naveira F, Speight KL, Smith BP. Refractory idiopathic erythromelalgia. Anesth Analg 1996; 82: 1097- 101.
  2. Galimberti D, Pontón A, Rubio L, Torre A, Angles V, Soriano E, et al. A case of primary erythromelalgia. J Eur Acad Dermatol Venereol 2009; 23: 1338-9. https://doi.org/10.1111/j.1468-3083.2009.03212.x
  3. Cohen JS. Erythromelalgia: new theories and new therapies. J Am Acad Dermatol 2000; 43: 841-7. https://doi.org/10.1067/mjd.2000.109301
  4. Park SK, Han KR, Lee YS, Hyun HS, Kim C. Epidural block of bupivacaine and mexiletine for erythromelalgia. Korean J Anesthesiol 2001; 41: 792-5. https://doi.org/10.4097/kjae.2001.41.6.792
  5. Stricker LJ, Green CR. Resolution of refractory symptoms of secondary erythermalgia with intermittent epidural bupivacaine. Reg Anesth Pain Med 2001; 26: 488-90. https://doi.org/10.1097/00115550-200109000-00018
  6. D'Angelo R, Cohen IT, Brandom BW. Continuous epidural infusion of bupivacaine and fentanyl for erythromelalgia in an adolescent. Anesth Analg 1992; 74: 142-4.
  7. Waxman SG, Dib-Hajj SD. Erythromelalgia: a hereditary pain syndrome enters the molecular era. Ann Neurol 2005; 57: 785-8. https://doi.org/10.1002/ana.20511
  8. Dib-Hajj SD, Rush AM, Cummins TR, Hisama FM, Novella S, Tyrrell L, et al. Gain-of-function mutation in Nav1.7 in familial erythromelalgia induces bursting of sensory neurons. Brain 2005; 128: 1847-54. https://doi.org/10.1093/brain/awh514
  9. Natkunarajah J, Atherton D, Elmslie F, Mansour S, Mortimer P. Treatment with carbamazepine and gabapentin of a patient with primary erythermalgia (erythromelalgia) identified to have a mutation in the SCN9A gene, encoding a voltage-gated sodium channel. Clin Exp Dermatol 2009; 34: E640-2. https://doi.org/10.1111/j.1365-2230.2009.03355.x
  10. Nathan A, Rose JB, Guite JW, Hehir D, Milovcich K. Primary erythromelalgia in a child responding to intravenous lidocaine and oral mexiletine treatment. Pediatrics 2005; 115: E504-7. https://doi.org/10.1542/peds.2004-1395
  11. Davis MD, O'Fallon WM, Rogers RS 3rd, Rooke TW. Natural history of erythromelalgia: presentation and outcome in 168 patients. Arch Dermatol 2000; 136: 330-6. https://doi.org/10.1001/archderm.136.3.330
  12. Rudikoff D, Jaffe IA. Erythromelalgia: response to serotonin reuptake inhibitors. J Am Acad Dermatol 1997; 37: 281-3. https://doi.org/10.1016/S0190-9622(97)80144-2
  13. Harrison CM, Goddard JM, Rittey CD. The use of regional anaesthetic blockade in a child with recurrent erythromelalgia. Arch Dis Child 2003; 88: 65-6. https://doi.org/10.1136/adc.88.1.65
  14. Weinstein SM, Herring SA. Lumbar epidural steroid injections. Spine J 2003; 3(3 Suppl): 37-44. https://doi.org/10.1016/S1529-9430(02)00560-0
  15. Seishima M, Kanoh H, Izumi T, Niwa M, Matsuzaki Y, Takasu A, et al. A refractory case of secondary erythermalgia successfully treated with lumbar sympathetic ganglion block. Br J Dermatol 2000; 143: 868-72. https://doi.org/10.1046/j.1365-2133.2000.03795.x
  16. Shiga T, Sakamoto A, Koizumi K, Ogawa R. Endoscopic thoracic sympathectomy for primary erythromelalgia in the upper extremities. Anesth Analg 1999; 88: 865-6. https://doi.org/10.1213/00000539-199904000-00034
  17. Belch JL. Temperature-associated vascular disorder-Raynaud's phonomenon and erythromelagia. In: A textbook of vascular medicine. Edited by Lowe GD, Tooke JE. London, Oxford University Press. 1996, pp 339-52.
  18. Littleford RC, Khan F, Belch JJ. Skin perfusion in patients with erythromelalgia. Eur J Clin Invest 1999; 29: 588-93. https://doi.org/10.1046/j.1365-2362.1999.00510.x
  19. Orstavik K, M$\phi$rk C, Kvernebo K, J$\phi$rum E. Pain in primary erythromelalgia-a neuropathic component? Pain 2004; 110: 531-8. https://doi.org/10.1016/j.pain.2004.03.030

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