Article

Treatment of familial erythermalgia with the association of lidocaine and mexiletine

Service de Dermatologie, CHU, 5, avenue Foch, 29200 Brest.
Annales de Dermatologie et de Vénéréologie (Impact Factor: 0.67). 05/2003; 130(4):429-33.
Source: PubMed

ABSTRACT 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.
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).
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.

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    • "In comparison, gain-of-function mutations in the related SCN9A gene, which encodes NaV1.7, can result in primary erythromelalgia (PEM), either a familial or a sporadic chronic neuropathic pain syndrome (Dib-Hajj et al., 2005). Several publications have reported an analgesic effect of mexiletine in patients suffering from this monogenic pain disorder (Kuhnert et al., 1999; Legroux-Crespel et al., 2003; Dib-Hajj et al., 2005; Nathan et al., 2005; Choi et al., 2009). The apparent absence of pro-arrhythmic and other major adverse effects at analgesic doses of mexiletine points to a mode of action that preferentially affects pathological channels. "
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