Erythromelalgia: Vasculopathy, neuropathy, or both? A prospective study of vascular and neurophysiologic studies in erythromelalgia
Erythromelalgia: Vasculopathy, neuropathy, or both? A prospective study of vascular and neurophysiologic studies in erythromelalgia
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DOI：
10.1001/archderm.139.10.1337
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发表时间：
2003-10-01
影响因子：
--
通讯作者：
Low, PA
中科院分区：
文献类型：
--
作者：
Davis, MDP;Sandroni, P;Low, PA
目的：评估红斑性肢痛症患者的血管变化和神经系统异常的频率和类型。设计：前瞻性研究，对就诊时无自发症状和有激发症状的患者进行研究。设置：三级转诊中心。患者：1999年至2001年，明尼苏达州罗切斯特马约诊所的67例红斑性肢痛症患者。干预措施：在没有症状的患者中测试神经和血管功能;通过运动或增加环境温度激发症状后测试血管功能。主要结果测量：在可以引起症状的患者中，通过测量局部皮肤温度、激光多普勒血流，和经皮血氧测定法。神经功能评估包括肌电图，神经传导研究，和自主神经反射筛选（使用定量的催汗轴突反射试验，肾上腺素能功能测试，深呼吸的心率反应，和Valsalva比率）。在这57例患者中，46例（81%）有异常的定量催汗轴突反射试验结果，14例（25%）有异常的肾上腺素能功能，15例（26%）有异常的心迷走神经功能。换句话说，在57名进行自主神经反射筛查的患者中，有49名（86%）结果异常。这49例患者中有46例（94%）出现严重的催汗异常（即无汗液产生或汗液产生明显减少）; 14例（29%）肾上腺素能功能异常，15例（31%）心迷走神经异常。67例患者中有24例（36%）进行了肌电图和神经传导研究。在这24例患者中，14例（58%）肌电图结果异常，10例（42%）神经传导研究结果异常。在67名患者中的13名中进行了血管功能研究，有症状和无症状。在出现症状期间，脚趾皮肤的平均温度升高了7.8摄氏度，血流量增加了10.2倍。Parapartically，平均经皮血氧测量值并没有changed.Conclusion：这项前瞻性研究扩展并证实了我们以前的观察，除了其他形式的神经病变，大多数红斑性肢痛症患者有小纤维神经病变。
Objective: To assess the frequency and type of vascular changes and neurologic abnormalities in patients with erythromelalgia.Design: Prospective study of patients with no spontaneous symptoms at the time of their visit and with provoked symptoms.Setting: Tertiary referral center.Patients: Sixty-seven patients presenting with erythromelalgia at Mayo Clinic, Rochester, Minn, from 1999 through 2001.Interventions: Testing nerve and vascular function in patients without symptoms present; testing vascular function after provoking symptoms with exercise or by increasing ambient temperature.Main Outcome Measures: In patients in whom symptoms could be elicited, vascular function with and without symptoms was assessed by measurement of local skin temperature, laser Doppler flow, and transcutaneous oximetry. Neurologic assessment included electromyography, nerve conduction studies, and autonomic reflex screening (using the quantitative sudomotor axon reflex test, adrenergic function testing, heart rate response to deep breathing, and the Valsalva ratio).Results: Autonomic reflex screening was performed on 57 (85%) of the 67 patients. Of these 57 patients, 46 (81%) had abnormal quantitative sudomotor axon reflex test results; 14 (25%) had abnormal adrenergic function; and 15 (26%) had abnormal cardiovagal function. Put in another way, results were abnormal for 49 (86%) of the 57 patients who had autonomic reflex screening. Severe sudomotor abnormalities (ie, absent or markedly reduced sweat production) were present in 46 (94%) of these 49 patients; 14 (29%) had abnormal adrenergic function, and 15 (31%) had a cardiovagal abnormality. Electromyography and nerve conduction studies were performed in 24 (36%) of the 67 patients. Of these 24 patients, 14 (58%) had abnormal electromyographic results and 10 (42%) had abnormal nerve conduction study results. Vascular function studies, with and without symptoms present, were performed in 13 of the 67 patients. During symptoms, the mean temperature of the toe skin increased by 7.8degreesC, and blood flow increased 10.2-fold. Paradoxically, mean transcutaneous oximetry measurements did not change.Conclusion: This prospective study extends and confirms our previous observation that, in addition to other forms of neuropathy, most patients with erythromelalgia have small-fiber neuropathy.