title: [Erythromelalgia: Diagnosis and therapeutic approach].
abstract: Erythromelalgia is a rare intermittent vascular acrosyndrome characterized by the combination of recurrent burning pain, warmth and redness of the extremities. It is considered in its primary form as an autosomal dominant neuropathy related to mutations of SCN9A, the encoding gene of a voltage-gated sodium channel subtype Nav1.7. Secondary erythromelalgia is associated with myeloproliferative disorders, drugs (bromocriptine, calcium channel blockers), or clinical conditions such as rheumatic diseases or viral infection. Primary familial erythromelalgia include genetics and sporadic forms associated with small fibers neuropathy. Aspirin is a useful treatment of erythromelagia associated with myeloproliferative disorders. Treatment of primary erythromelalgia is difficult, individualized, with sodium channel blockers such as lidocaine, carbamazepine and mexiletine.
authors.affiliation: Département de médecine interne, CHU de Rouen, 1, rue de Germont, 76031 Rouen, France.
authors.forename: S
authors.initials: S
authors.lastname: Miranda
authors.affiliation: Département de médecine interne, CHU de Rouen, 1, rue de Germont, 76031 Rouen, France.
authors.forename: M
authors.initials: M
authors.lastname: Le Besnerais
authors.affiliation: Département de médecine interne, CHU de Rouen, 1, rue de Germont, 76031 Rouen, France.
authors.forename: V
authors.initials: V
authors.lastname: Langlois
authors.affiliation: Département de médecine interne, CHU de Rouen, 1, rue de Germont, 76031 Rouen, France.
authors.forename: Y
authors.initials: Y
authors.lastname: Benhamou
authors.affiliation: Département de médecine interne, CHU de Rouen, 1, rue de Germont, 76031 Rouen, France. Electronic address: herve.levesque@chu-rouen.fr.
authors.forename: H
authors.initials: H
authors.lastname: Lévesque
mesh_terms: D002121:Calcium Channel Blockers / Q000627:therapeutic use; D019937:Diagnostic Techniques and Procedures; D004916:Erythromelalgia / Q000145:classification / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy*; D006801:Humans; D026941:Sodium Channel Blockers / Q000627:therapeutic use
chemical_list: D002121:Calcium Channel Blockers; D026941:Sodium Channel Blockers
keywords: Canal sodique voltage dépendant; Mutations SNC9a; Primary erythromelalgia; SCN9a mutations; Thrombocytose; Thrombocytosis; Voltage-gated sodium channel; Érythromélalgie primaire