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[利多卡因与美西律联合治疗家族性红斑性肢痛症]

[Treatment of familial erythermalgia with the association of lidocaine and mexiletine].

作者信息

Legroux-Crespel E, Sassolas B, Guillet G, Kupfer I, Dupre D, Misery L

机构信息

Service de Dermatologie, CHU, 5, avenue Foch, 29200 Brest.

出版信息

Ann Dermatol Venereol. 2003 Apr;130(4):429-33.

Abstract

INTRODUCTION

Erythermalgia is a rare acrosyndrome characterized by reddening of the skin, local increase heat and pain. The disease is frequently resistant to treatment. Recently, Kuhnert et al. presented very favorable results using a combination of lidocaine and mexiletine. We used this treatment in 4 patients suffering from familial erythermalgia.

OBSERVATIONS

In a family exhibiting severe familial erythermalgia involving 5 members over 3 generations, we treated 4 patients aged 41, 39, 19 and 15 years. In these patients, the erythermalgia known since early childhood, progressed in the form of multiple flares (6 to 7/day) during the day and at night, lasting several hours and often accompanied by headaches. The impact of the disease on their quality of life was major. Only cold-water baths provided temporary relief, obliging them to live with their "feet in cold water". After they had been informed of the modalities of treatment and in the absence of any contraindication, notably cardiologic, 200 mg (100 mg in the youngest patient) of lidocaine were infused in 4 hours in a single intravenous injection on the first day. Mixelitine was introduced on the second day at the dose of 600 mg in 3 oral intakes (200 mg in the youngest patient). The painful paroxistic symptomatology rapidly improved and the flares had disappeared on the 3dr day, thus permitting the progressive reduction in analgesics and major improvement in quality of life. This beneficial effect persisted with oral mexiletine alone, 2 years after the infusion of lidocaine in the first patient treated (and one year after in the other patients).

COMMENTS

Primary familial erythermalgia is highly resistant to treatment. The combined action of lidocain and mexiletine, usually well tolerated (class IB antiarrythmic), blocks the sodium channels. The mechanism of action of their analgesic effect is peripheral or central or even mixed. This benefit warrants confirmation in other forms of erythermalgia.

摘要

引言

红斑性肢痛症是一种罕见的肢端综合征,其特征为皮肤发红、局部发热和疼痛。该疾病通常对治疗有抵抗性。最近,库纳特等人使用利多卡因和美西律联合治疗取得了非常良好的效果。我们对4例患有家族性红斑性肢痛症的患者采用了这种治疗方法。

观察结果

在一个三代中有5名成员患有严重家族性红斑性肢痛症的家族中,我们治疗了4名年龄分别为41岁、39岁、19岁和15岁的患者。这些患者自幼年起就患有红斑性肢痛症,白天和晚上以多次发作(每天6至7次)的形式进展,持续数小时,常伴有头痛。该疾病对他们生活质量的影响很大。只有冷水浴能提供暂时缓解,迫使他们“脚泡在冷水里”生活。在告知他们治疗方式且无任何禁忌证(尤其是心脏病方面)后,第一天将200毫克(最年轻患者为100毫克)利多卡因在4小时内单次静脉注射。第二天开始口服美西律,剂量为600毫克,分3次服用(最年轻患者为200毫克)。疼痛性发作症状迅速改善,第三天发作消失,从而使镇痛药逐渐减少,生活质量得到显著改善。在首例接受治疗的患者输注利多卡因2年后(其他患者为1年后),仅口服美西律这种有益效果仍持续存在。

评论

原发性家族性红斑性肢痛症对治疗具有高度抵抗性。利多卡因和美西律的联合作用通常耐受性良好(ⅠB类抗心律失常药),可阻断钠通道。它们镇痛作用的机制是外周性的、中枢性的甚至是混合性的。这种益处有待在其他类型的红斑性肢痛症中得到证实。

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