坏死松解性游走性红斑——胰腺占位一例  

Necrolytic Migratory Erythema--A Case Report of Pancreatic Occupying Lesion

作　　者：胡中慧 杨璐 刘跃华[1] 晋红中[1] 徐强[2] 刘雯静[2] 罗亚平[3] 阳洪波[4] 王涛[1] HU Zhonghui;YANG Lu;LIU Yuehua;JIN Hongzhong;XU Qiang;LIU Wenjing;LUO Yaping;YANG Hongbo;WANG Tao(Department of Dermatology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;Department of General Surgery,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;Department of Nuclear Medicine,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;Department of Endocrinology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医院皮肤科,北京100730 [2]中国医学科学院北京协和医院基本外科,北京100730 [3]中国医学科学院北京协和医院核医学科,北京100730 [4]中国医学科学院北京协和医院内分泌科,北京100730

出　　处：《罕见病研究》2023年第3期427-431,共5页Journal of Rare Diseases

基　　金：中央高水平医院临床科研业务费(2022-PUMCH-A-164);北京市自然科学基金(Z210017)。

摘　　要：胰高血糖素瘤是一种罕见的来源于胰腺α细胞的神经内分泌肿瘤,该肿瘤因过度分泌胰高血糖素,引起胰高血糖素瘤综合征,70%~90%的患者会伴发坏死松解性游走性红斑(NME)。本文报道1例首诊于皮肤科的胰高血糖素瘤综合征患者,临床表现为周身反复红斑、脱屑伴瘙痒,经皮肤组织病理学检查确诊为NME,影像学检查提示胰尾部软组织密度肿瘤,生长抑素高表达,外周血胰高血糖素水平升高,确诊为胰高血糖素瘤综合征。行胰尾部胰高血糖素瘤切除术,术后第4天皮疹消退。同时本文回顾了近年来国内外相关文献,对胰高血糖素瘤相关NME的临床表现、实验室及影像学特点、诊断及治疗等方面进行总结,以提高临床医师对该病的认识。Glucagonoma is a rare neuroendocrine tumor ofαcells of the pancreas.The tumor excessively secretes glucagon and causes glucagonoma syndrome.70%-90% of patients with glucagonoma will develop necrolytic migratory erythema(NME).We reported a patient of glucagonoma syndrome who was presented to the dermatology outpatient clinic with a 2-year-history of recurrent erythema and scaling on the skin migrating throughout the body.A skin biopsy was performed and resulting features matched with NME,whilst imaging examinations suggested a soft tissue density tumor present in the tail of the pancreas with somatostatin receptor expression and laboratory tests found an elevated levels of serum glucagon.After the diagnosis was confirmed,the patient was treated with surgical resection of the glucagonoma and the skin eruptions resolved rapidly in 4 days.Meanwhile,we reviewed relevant literature published in recent years and summarized its clinical characteristics in order to improve its understanding by clinicians,including clinical manifestations,laboratory and imaging examinations,diagnosis and treatments.

关 键 词：坏死松解性游走性红斑 胰高血糖素瘤 胰高血糖素瘤综合征 胰腺占位 

分 类 号：R735.9[医药卫生—肿瘤] R758.61[医药卫生—临床医学]