Natural history of erythromelalgia -: Presentation and outcome in 168 patients

Natural history of erythromelalgia -: Presentation and outcome in 168 patients
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DOI:
10.1001/archderm.136.3.330
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发表时间:
2000-03-01
影响因子:
--
通讯作者:
Rooke, TW
Rooke, TW
中科院分区:
其他
文献类型:
--
作者:
Davis, MDP;O'Fallon, WM;Rooke, TW

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目的:描述红斑性肢痛症患者的人口学特征、临床表现和转归。红斑性肢痛症是一种罕见且鲜为人知的临床综合征,由红、热、痛三重症状组成。设计:回顾病历,并由SUN EY问卷进行随访。背景:大型三级医疗中心。对象:1970-1994年间在明尼苏达州罗切斯特市梅奥诊所就诊的红斑性肢痛症患者。干预:对168例患者的病历进行分析。结果:所有患者均为白人,女性122例(72.6%),男性46例(27.4%)。患者发病时的平均年龄为55.8岁(5-91岁)。有7名患者(4.2%)从童年起就有症状。6名患者(3.6%)有红斑性肢痛症的一级亲属。间歇性症状163例(97.0%),持续性症状5例(3.0%)。症状主要累及足部(148例患者(88.1%))和手部(43例患者(25.6%))。Kaplan-Meier生存曲线显示,与年龄相近、性别相同的人相比,生存时间显著降低(P
Qbjective: To describe the demographics, presentation, and outcome in patients with erythromelalgia-a rare and poorly understood clinical syndrome defined by the triad of red, hot, painful extremities.Design: Retrospective medical record review with follow-up by sun ey questionnaire.Setting: Large tertiary care medical center.Subjects: Patients with erythromelalgia examined at the Mayo Clinic, Rochester, Minn, between 1970 and 1994.Intervention: The medical records of 168 patients were analyzed. Follow-up data, which consisted of answers to 2 survey questionnaires or the most recent information in the medical record from patients still alive and death certificates or reports of death for those deceased patients, were obtained for all but 13 patients.Main Outcome Measures: Survival, morbidity, and quality of life.Results: All patients were white; 122 (72.6%) were female, and 46 (27.4%) were male. At presentation, the patients' mean age was 55.8 years (age range, 5-91 years). Symptoms had been present since childhood in 7 patients (4.2%). Six patients (3.6%) had a first-degree relative with erythromelalgia. Symptoms were intermittent in 163 patients (97.0%) and constant in 5 (3.0%). Symptoms predominantly involved feet (148 patients [88.1%]) and hands (43 patients [25.6%]). Kaplan-Meier survival curves revealed a significant decrease in survival compared with that expected in persons of similar age and of the same sex (P