Erythromelalgia:
cyclosporine, gabapentine and buflomedil, therapeutic alternatives for a disease with few options
Keywords:
erythromelalgia, cyclosporine, treatment, buflomedil, gabapentinAbstract
OPTIONSAbstractErythromelalgia is a rare disease characterized by redness, increased skin temperature and inferior limb pain, which is generally relieved by submersion of them in freezing water. Its pathophysiology and treatment are mostly unknown and its therapeutic options are few. We report here the case of a 19 year-old male, whose symptoms of erythromelalgia started at 11 years old. Due to the intensity of the symptoms, the patient had been bedridden for two years with permanent limb immersion in cold water. The patient has a family history of erythromelalgia (father and grandfather), being this a case of primary hereditary erythromelalgia. At the time of our evaluation, he had been in that situation for two years, and severely malnourished (SGA - C), presenting ulcers with mycotic infection due to permanent immersion. Treatment with cyclosporine was started, taken from another clinical case report.1 The patient showed noticeable improvement with this treatment, but it couldn’t be continued due to repeated serious respiratory infections. For this reason, we switched to a treatment with buflomedil and gabapentin, with which the patient had a good symptoms control and was able to live a nearly normal life for 9 years now. As a current problem, the patient presents bilateral avascular bone necrosis. We consider that cyclosporine (in patients that have no other immunosuppressive factors), as well as buflomedil and gabapentine are therapeutic options for a rare pathology with little evidence and few treatment options.Keywords: erythromelalgia, cyclosporine, treatment, buflomedil, gabapentin.
Downloads
References
Shigetoshi S, Satoshi I, Kunihiko Y. Tretment oferythromelalgia primary with cyclosporine. N Engl JMed 349: 816-817, 2003.
Mitchell SW. On a rare vasomotor neurosis of theextremities and on the maladies with wich it may beconfounded. Am J Med Sci 76:2-36, 1878.
Smith LA, Allen EV. Erythermalgia (erythromelalgia) ofthe extremities. A syndrome characterized by redness,heat and pain. Am Heart J 16:175-88, 1938.
Pipili C; Cholongitas E. Erythromelalgia in a diabeticpatient managed with gabapentin. Diabetes research andclinical practice 79: E15-e16, 2008.
Mork C, Salerud EG, Asker CL, Kvernebo K.The prostaglandin E1 analog misoprostol reducessymptoms and microvascular arteriovenous shunting inerythromelalgia – a double – blind, crossover, placebo –compared study. J Invest Dermatol. 122: 587-593, 2004.
Kalgaard OM, MorK C, Dvernebo K. Prostacyclin reduces symptoms and sympathetic dysfunction in eritromelalgia in a double-blind randomized pilto study. Acta Derm Venereol. 83 (6):442-4, 2003.
Mork C, Asker CL, Salerud EG, Dvernebo K.Microvascular arteriovenous shunting is a probablepathogenetic mechanism in erythromelalgia. J InvestDermatol 114: 643-646, 2000.
Marumo T, Nakaki T, Hishikawa K, Suzuki H y col.Cyclosporin A inhibits nitric oxide synthase inductionin vascular smooth muscle cells. Hypertension 25:764-8, 1995.
Abassi ZA, Pieruzzi F, Nakhoul F, Keiser HR. Effects ofcyclosporine A on the synthesis, excretion and metabolism of endotelin in the rat. Hypertension 27:1140-8, 1996.
Leal, M; Matos. Resumos de disertasoes: Necroseavascular óssea na terapia imunossupressora emtransplantados renais Rev baiana saúde publica 34:89 90,2010
Published
How to Cite
Issue
Section
License
Licencia Atribución-CompartirIgual 4.0 Internacional (CC BY-SA 4.0)
https://creativecommons.org/licenses/by-sa/4.0/deed.es
