Kuroda Takeshi, Sugimoto Azusa, Ishigaki Seiichirou, Murakami Hidetomo, Kawamura Mitsuru
Department of Neurology, Showa University School of Medicine.
Brain Nerve. 2014 Feb;66(2):185-9.
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.
红斑性肢痛症是一种罕见病症，其特征为四肢持续或阵发性灼痛、红斑以及皮肤温度升高。最近，由于自身免疫系统受累导致的C纤维功能损害被认为是红斑性肢痛症的主要病因。然而，尚未确立成功的治疗方法。我们报告一例39岁患有原发性红斑性肢痛症的女性病例，伴有高脑脊液蛋白浓度和轴索性神经病。她接受了各种抗癫痫和抗炎药物治疗，但症状未改善。她最终接受了大剂量静脉注射免疫球蛋白治疗，症状显著改善，脑脊液蛋白浓度恢复正常。这一结果证明了大剂量静脉注射免疫球蛋白治疗原发性红斑性肢痛症的有效性以及自身免疫系统受累的可能性。
