以手掌痛性红斑为首发症状的真性红细胞增多症1例  

Polycythemia Vera with Painful Erythema on the Palm as the First Symptom:A Case Report

作　　者：杨雁南 窦清惠[2] 杨静 王俊慧[1] YANG Yannan;DOU Qinghui;YANG Jing;WANG Junhui(Department of Dermatology,Guang′anmen Hospital,China Academy of Chinese Medical Sciences,Beijing 100053,China;Department of Dermatology,the Hospital of Xinjiang Production and Construction Corps,Urumqi 830002,China)

机构地区：[1]中国中医科学院广安门医院皮肤科,北京100053 [2]新疆生产建设兵团医院皮肤科,新疆乌鲁木齐830002

出　　处：《皮肤科学通报》2024年第4期462-465,共4页Dermatology Bulletin

基　　金：中国中医科学院科技创新工程项目(CI2021A02314)。

摘　　要：患者男,66岁,右手掌红斑水肿疼痛1个月。皮肤科情况:右手掌弥漫潮红,大、小鱼际可见暗紫红色水肿斑块,皮温略高,触痛明显。皮损组织病理示:角化过度,表皮增生,真皮浅层毛细血管增生及扩张,血管周围见少量淋巴细胞及散在的中性粒细胞浸润。实验室检查:白细胞计数19.82×10^(9)/L,血红蛋白测定196 g/L,血小板计数494×10^(9)/L;骨髓活检考虑骨髓增殖性肿瘤(MPN),真性红细胞增多症(PV)可能性大;基因检测提示JAK2基因上检测到错义突变。诊断:真性红细胞增多症。予中药口服2次/d,外用0.05%卤米松乳膏1g,1次/d,3周后皮损逐渐消退。A 66-year-old male presented with painful erythematous edema in the right palm for 1 month.Dermatological manifestation:diffuse flushing in the right palm,dark purplish-red edematous plaques were seen in the large and small fissures,skin temperature was slightly high,and tenderness was obvious.Histopathology of the lesions showed hyperkeratosis,epidermal hyperplasia,capillary hyperplasia and dilatation in the superficial dermis,and a small number of lymphocytes and scattered neutrophil infiltration around the blood vessels.Laboratory tests:white blood cell count 19.82×10^(9)/L,hemoglobin 196 g/L,platelet count 494×10^(9)/L;Bone marrow biopsy considered myeloprolitera-tive neoplasms(MPN),high possibility of polycythemia vera(PV);Genetic testing suggested that missense mutations were detected in the JAK2 gene.Diagnosis:polycythemia vera.The patient was given traditional Chinese medicine orally 2 times/d,and topical 0.05%Halometasone cream 1g,1 time/d.The lesions gradually subsided after 3 weeks.

关 键 词：真性红细胞增多症 疼痛 红斑 

分 类 号：R739.5[医药卫生—肿瘤]