Hitoshi S, Okazawa H, Shimizu J, Suzuki K, Sakuta M
Department of Neurology, Japanese Red Cross Medical Center.
Rinsho Shinkeigaku. 1991 Jul;31(7):730-3.
We experienced a 47-year-old Japanese female with polyneuropathy, edema, hypertrichosis, hyperpigmentation, and white nail, which were diagnostic as having Crow-Fukase syndrome. Laboratory and radiological evaluation showed neither plasma cell dyscrasia nor monoclonal gammopathy. Increased factor VIII activity and thrombocytosis, which suggested thrombotic tendency, were observed at the exacerbation of clinical symptoms. In her third exacerbation, she presented marked cyanosis in her right foot, and angiography confirmed narrowing of arteries at the ankle. Increased serum interleukin-6 was also observed, and the production of interleukin-6 by endothelial cells of cutaneous angioma was shown. Possible role of interleukin-6 in Crow-Fukase syndrome was discussed.
我们遇到一位47岁的日本女性，患有多发性神经病、水肿、多毛症、色素沉着和白甲，诊断为Crow-Fukase综合征。实验室和影像学评估未显示浆细胞异常增生或单克隆丙种球蛋白病。在临床症状加重时观察到因子VIII活性增加和血小板增多，提示有血栓形成倾向。在她第三次病情加重时，右脚出现明显发绀，血管造影证实脚踝处动脉狭窄。还观察到血清白细胞介素-6升高，且显示皮肤血管瘤的内皮细胞可产生白细胞介素-6。讨论了白细胞介素-6在Crow-Fukase综合征中的可能作用。
