Heidrich H
Vasa. 2010 Feb;39(1):33-41. doi: 10.1024/0301-1526/a000003.
Raynauds syndrome, acrocyanosis and erythromelalgia are functional vascular diseases that differ with respect to prevalence, clinical picture, therapy, prognosis, and impairment of quality of life. Raynauds syndrome occurs in 5 to 20 % of the population in Europe, is observed four times more often in women than in men and appears first at the age of 40 (3 to 80), on the average. Raynauds attacks are characterized by a paroxysmal white-blue-red or just white and blue discoloration of the fingers and toes; the attacks are induced by cold or stress, usually, cease after no more than some minutes (average 23 min.), but can also persist for hours. A distinction must be made between primary (aetiology unknown), secondary (aetiology known) and suspected secondary Raynauds syndromes (causal underlying disease suspected). There are several different therapy options, but not all of them have been substantiated by evidence. Acrocyanosis is rarer than Raynauds syndrome, and contrary to the latter, is characterized by nonparoxysmal, in most cases persistent, painless bluish-red symmetrical discolorations of the hands, feet and knees. It is more frequent in women than in men and becomes manifest before the 25th year of age, on the average (15th to 70th year of age). A distinction is made between primary acrocyanosis without detectable underlying disease and secondary acrocyanosis with a specific underlying disease. No effective therapy for primary acrocyanosis is known, but secondary forms can sometimes be treated. Patients with primary and secondary erythromelalgia, a very rare condition, sustain paroxysmal burning pain with marked reddening of the legs, feet and less often the hands. The attacks are triggered by warmth. Women are affected more often than men. The age of first manifestation is 40 to 55 years, but the first attacks may just as well occur during childhood. There are different therapeutic approaches with occasional success, but no general recommendations.
雷诺综合征、手足发绀症和红斑性肢痛症是功能性血管疾病，在患病率、临床表现、治疗、预后及生活质量受损方面存在差异。在欧洲，5%至20%的人口患有雷诺综合征，女性患者的发病率是男性的四倍，平均首次发病年龄为40岁（3至80岁）。雷诺发作的特征是手指和脚趾阵发性出现白-蓝-红或仅白和蓝的变色；发作通常由寒冷或压力诱发，一般不超过几分钟（平均23分钟）后停止，但也可能持续数小时。必须区分原发性（病因不明）、继发性（病因已知）和疑似继发性雷诺综合征（怀疑有潜在病因疾病）。有几种不同的治疗选择，但并非所有都有充分的证据支持。手足发绀症比雷诺综合征少见，与后者不同的是，其特征是手部、足部和膝盖出现非阵发性、多数情况下持续存在的、无痛的蓝红色对称性变色。女性比男性更常见，平均在25岁之前（15至70岁）发病。可区分无可检测潜在疾病的原发性手足发绀症和有特定潜在疾病的继发性手足发绀症。目前尚无治疗原发性手足发绀症的有效方法，但继发性类型有时可以治疗。原发性和继发性红斑性肢痛症都非常罕见，患者会出现阵发性灼痛，腿部、足部明显发红，手部较少见。发作由温暖诱发。女性比男性更易患病。首次发病年龄为40至55岁，但首次发作也可能在儿童期出现。有不同的治疗方法，偶尔会取得成功，但没有通用的建议。
