免疫グロブリン大量静注療法が有効であった特発性肢端紅痛症の1例

A Case of Primary Erythromelalgia Successfully Treated with High-dose Intravenous Immunoglobulin Therapy

肢端紅痛症は四肢末端の疼痛，発赤，熱感を主徴とする稀な疾患である。近年，自己免疫異常に伴うC線維の障害を発端とした小径線維ニューロパチーがその病態において注目されているが，治療法は未だ確立されていない。筆者らは，免疫グロブリン大量静注療法が著効した特発性肢端紅痛症の1例を経験した。その治療効果は，一部の特発性肢端紅痛症に免疫グロブリン大量静注療法が有用であるとともに，その病態に自己免疫異常が関わっている可能性を示唆した。

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. (Received August 30, 2013; Accepted September 24, 2013; Published February 1, 2014)