title: [A case of primary erythromelalgia successfully treated with high-dose intravenous immunoglobulin therapy].
abstract: Erythromelalgia is a rare condition characterized by constant or paroxysmal burning pain, erythema, and the elevation of skin temperature in the extremities. Recently, the impairment of C-fiber function due to autoimmune system involvement is considered as the primary cause of erythromelalgia. However, a successful treatment has yet not been established. We report a case of a 39-year-old woman with primary erythromelalgia accompanied by high cerebrospinal fluid protein concentration and axonal neuropathy. She received various antiepileptic and anti-inflammatory drugs, but failed to improve. She finally underwent high-dose intravenous immunoglobulin therapy, which dramatically improved her symptoms and normalized cerebrospinal fluid protein concentration. This result demonstrates the effectiveness of high-dose intravenous immunoglobulin therapy for the treatment of primary erythromelalgia and the possibility of autoimmune system involvement.
authors: Department of Neurology, Showa University School of Medicine.
authors: Takeshi
authors: T
authors: Kuroda
authors: Azusa
authors: A
authors: Sugimoto
authors: Seiichirou
authors: S
authors: Ishigaki
authors: Hidetomo
authors: H
authors: Murakami
authors: Mitsuru
authors: M
authors: Kawamura
mesh_terms: D000328:Adult; D004916:Erythromelalgia / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage / Q000627:therapeutic use*; D010146:Pain / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D016896:Treatment Outcome
chemical_list: D016756:Immunoglobulins, Intravenous