Complex management of a patient with refractory primary erythromelalgia lacking a SCN9A mutation
AffiliationUniv Arizona, Coll Med, Dept Internal Med, Banner Univ Med Ctr
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PublisherDOVE MEDICAL PRESS LTD
CitationComplex management of a patient with refractory primary erythromelalgia lacking a SCN9A mutation 2017, Volume 10:973 Journal of Pain Research
JournalJournal of Pain Research
Rights© 2017 Low et al. This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms. php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License (http://creativecommons.org/licenses/by-nc/3.0/).
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AbstractA 41-year-old woman presented with burning and erythema in her extremities triggered by warmth and activity, which was relieved by applying ice. Extensive workup was consistent with adult-onset primary erythromelalgia (EM). Several pharmacological treatments were tried including local anesthetics, capsaicin, ziconotide, and dantrolene, all providing 24-48 hours of relief followed by symptom flare. Interventional therapies, including peripheral and sympathetic ganglion blocks, also failed. Thus far, clonidine and ketamine have been the only effective agents for our patient. Genetic testing was negative for an EM-associated mutation in the SCN9A gene, encoding the Na(V)1.7 sodium channel, suggesting a mutation in an alternate gene.
NoteOpen Access Journal
VersionFinal published version
SponsorsFoundation for Anesthesia Education and Research (FAER)