Raynaud phenomenon in dermatology.: Part 1:: Pathophysiology and diagnostic approach
Raynaud phenomenon in dermatology.: Part 1:: Pathophysiology and diagnostic approach
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DOI：
10.1007/s00105-006-1192-x
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发表时间：
2006-09-01
期刊：
影响因子：
--
通讯作者：
Riernelkasten, G.
中科院分区：
文献类型：
--
作者：
Sunderkotter, C.;Riernelkasten, G.
雷诺现象（RP）的特征是手指和脚趾的小指动脉/动脉反复痉挛，通常由寒冷和情绪应激引发。临床上，个别手指突然苍白，随后出现反应性充血，严重者还出现紫绀。我们可以区分原发性RP（即没有潜在疾病或药物摄入的RP）和继发性RIP（与潜在疾病或某些药物（如干扰素、顺铂）摄入有因果关系）。原发性RP是常见的（在北方或中欧的患病率约为13-20%），而继发性RP是罕见的，但系统性硬化症（SSc）的主要表现症状。鉴别诊断包括冷引起的苍白，急性栓塞事件，手指阵发性血肿或红斑性肢痛症。在内皮细胞和血管平滑肌中，血管收缩机制超过血管舒张机制。虽然可溶性介质，如内皮素或某些前列腺素已成功地用于治疗，其参与RP的初始病理生理的程度尚不清楚。继发性RP（与SSc相关）还具有血管腔受损的形态学改变。由于RP可导致严重的不适和并发症，因此及时诊断和治疗至关重要。
Raynaud phenomenon (RP) is characterized by recurrent spasms of small digital arterioles/arteries at fingers and toes, usually triggered by cold and emotional stress. Clinically a sudden pallor of individual digits is followed by reactive hyperemia, in severe cases also by cyanosis. One distinguishes between primary RP, i.e. RP without an underlying disease or drug intake, and secondary RIP, which is causally related to an underlying disease or to intake of certain drugs (e.g. interferon, cisplatin). Primary RP is frequent (prevalence of about 13-20% in northern or central Europe), while secondary RP is rare, but the major presenting symptom for systemic sclerosis (SSc). Differential diagnosis includes cold-induced pallor, acute embolic events, paroxysmal hematoma of the finger or erythromelalgia. Vasoconstrictive mechanisms outweigh vasodilatory ones in endothelial cells and vascular smooth muscle. Although soluble mediators such as endothelin or certain Prostaglandins have been exploited successfully for therapy, the extent of their involvement in the initial pathophysiology of RP is unclear. Secondary RP (associated with SSc) additionally features morphological alterations with compromise of the vessel lumen. As RP can result in severe discomfort and complications, timely diagnosis and treatment is essential.