[红斑性肢痛症]

Département de médecine interne, CHU Rouen-Boisguillaume.

Rev Prat. 1998 Oct 1;48(15):1669-72.

Erythermalgia is an unfrequent disorder characterised by recurrent, red, warm and painful extremities. Classification of erythermalgia is clinical: primary erythermalgia usually starting during childhood; erythromelalgia secondary to myeloproliferative disorders (especially thrombocytosis or polycythaemia vera); erythermalgia seconndary to drugs or vasculitis. Intravascular platelet activation and aggregation with plugging of the arterioles has been suggested as the mechanism of secondary erythromelalgia; aspirin is an effective treatment. Conversely, as yet unelucidated pathophysiologic events underlie primary erythermalgia, which usually fails to respond to aspirin.

红斑性肢痛症是一种罕见的病症，其特征为四肢反复出现发红、发热和疼痛。红斑性肢痛症的分类基于临床：原发性红斑性肢痛症通常始于儿童期；继发于骨髓增殖性疾病（尤其是血小板增多症或真性红细胞增多症）的红斑性肢痛症；继发于药物或血管炎的红斑性肢痛症。血管内血小板活化和聚集以及小动脉堵塞被认为是继发性红斑性肢痛症的发病机制；阿司匹林是一种有效的治疗方法。相反，原发性红斑性肢痛症的发病机制尚未阐明，通常对阿司匹林治疗无效。