Primary erythromelalgia in a child responding to intravenous lidocaine and oral mexiletine treatment
Primary erythromelalgia in a child responding to intravenous lidocaine and oral mexiletine treatment
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DOI：
10.1542/peds.2004-1395
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发表时间：
2005-04-01
期刊：
影响因子：
8
通讯作者：
Milovcich, K
中科院分区：
文献类型：
--
作者：
Nathan, A;Rose, JB;Milovcich, K
红斑性肢痛症是一种罕见的、慢性的、使人衰弱的疾病，其特征是四肢末端发红、发热和剧烈的灼痛。脚比手更容易受到影响。疼痛是由温度升高和运动引起的。患者通常通过将患肢浸入冷水中获得缓解。这种疼痛通常难以治疗。对于许多患者来说，多种止痛药在完全缓解疼痛症状方面一直无济于事。先前关于青少年红斑性肢痛症的报道表明，硝普钠输注、硬膜外局部麻醉剂输注或加巴喷丁治疗可延长疼痛缓解时间。我们报告一例11岁的白人男性儿童，患有原发性红斑性肢痛症，其最初症状始于学龄前几年，其童年的特点是脚部发热和红肿，特别是由于体温升高和活动而加剧的疼痛发作。所有传统的疼痛治疗方法都不能缓解我们的患者的症状，他只有将受影响的四肢浸泡在冰水中才能获得一些缓解。他体验到了去看疼痛心理学家的微乎其微的好处，这位心理学家帮助他开发了应对疼痛的技术。在提出报告时，患者的疼痛发作次数已增加到每天15至20次，有证据表明他的脚皮肤有慢性浸泡损伤。在他最近一次住院之前，疼痛已经蔓延到他的双手。患者在入院时焦虑不安，无法参加学校或社会活动。在他目前的住院期间，他确实对硝普钠输液表现出了一些治疗反应，但不幸的是，由于副作用和他的家人希望离开ICU环境，不得不停止输注，这对患者来说是有压力的。他对腰部硬膜外局部麻醉药的输注也有一定的反应，但由于他发现伴随着他的止痛而产生的运动阻滞无法忍受，因此无法继续。然而，静脉注射利多卡因，随后过渡到口服美西律治疗，证明在减少疼痛发作的频率和严重程度方面非常有效。患者通过口服美西律治疗出院，并在疼痛管理诊所接受监测。他回到学校，完成了学业，参加了夏令营，享受着积极的幸福生活。他走路不会引起脚部剧痛，每晚睡9到10个小时。在出院后的6个月里，他只需要浸泡4次脚。他的生活质量有了显著的提高。他没有表现出肝脏毒性的证据，他的美西律水平一直稳定。
Erythromelalgia is a rare, chronic, debilitating condition characterized by redness, warmth, and severe burning pain of the distal extremities. The feet are more commonly affected than the hands. Pain is precipitated by increases in temperature and by exercise. Patients often obtain relief by immersing the affected extremity in cold water. The pain is often refractory to treatment. For many patients, multiple pain medications have been useless in achieving complete relief of pain symptoms. Previous reports of erythromelalgia among adolescents indicated prolonged relief of pain with sodium nitroprusside infusions, epidural infusions of local anesthetics, or gabapentin treatment. We present a case of an 11-year-old, white, male child with primary erythromelalgia, whose initial symptoms started in his preschool years and whose childhood was marked by escalating episodes of pain with warmth and redness of his feet, precipitated especially by increases in temperature and by activity. All conventional pain management techniques had failed to relieve our patient of his symptoms, and he obtained some relief only by soaking his affected extremities in ice water. He had experienced minimal benefit from seeing a pain psychologist, who helped him develop techniques to cope with the pain. At the time of presentation, the patient's episodes of pain had increased to 15 to 20 per day, and there was evidence of chronic immersion injury to the skin of his feet. Before his most recent hospitalization, the pain had spread to involve his hands as well. The patient was overwhelmed with anxiety and could not participate in school or social activities at the time of admission. During his current hospitalization, he did show some therapeutic response to sodium nitroprusside infusion, which unfortunately had to be discontinued because of side effects and because his family desired to leave the ICU environment, which was stressful to the patient. He also had some response to lumbar epidural infusion of local anesthetics, which could not be continued because he found the motor blockade that accompanied his analgesia intolerable. However, intravenous lidocaine infusion, with subsequent transition to oral mexiletine therapy, proved very effective in reducing the frequency and severity of the pain episodes. The patient was discharged from the hospital with oral mexiletine therapy and has been monitored at the pain management clinic. He returned to and completed school, attended summer camp, and enjoys an active happy life. He walks without precipitating pain in his feet and sleeps 9 to 10 hours every night. He has needed to soak his feet on only 4 occasions in the 6 months since his discharge from the hospital. His quality of life has improved significantly. He has shown no evidence of liver toxicity, and his mexiletine levels have been stable.