V'lckova-Laskoska Marija, Balabanova-Stefanova Margareta, Arsovska-Bezhoska Irina, Caca-Biljanovska Nina, Laskoski Dimitri
Professor Marija V'lckova-Laskoska, MD, PhD, Department of Dermatology , University Hospitals and Clinics, University of Sts. Cyril and Methodius Vodnjanska 17, 1000 Skopje, Macedonia;
Acta Dermatovenerol Croat. 2018 Dec;26(4):329-332.
Necrolytic migratory erythema is considered an obligatory cutaneous paraneoplastic sign associated with glucagonoma. Glucagonoma syndrome is defined by the presence of an alpha-cell secreting tumor of the pancreas, elevated levels of glucagon, and a characteristic rash called necrolytic migratory erythema. Although necrolytic migratory erythema is a specific finding in glucagonoma syndrome, it may occur in other settings, unassociated with an alpha-cell pancreatic tumor (pseudoglucagonoma syndrome). The rarity of glucagonoma imposes a challenge, with most patients being diagnosed after a long period of treatment for their skin rash. The main prognostic sign of glucagonoma are the subsequent metastases that come late in the course of the disease. Herein, we present a 55-year-old female patient with a 5-year history of unrecognized cutaneous and systemic manifestations of glucagonoma syndrome. Based on the investigations, the diagnosis of glucagonoma syndrome without metastases was established. After surgical removal of pancreatic carcinoma/glucagonoma, complete healing and a long disease-free period was achieved. Appropriate awareness of the characteristics of necrolytic migratory erythema in physicians/dermatologists often leads to an early diagnosis of glucagonoma syndrome and enhances the chances of a favorable outcome.
坏死性游走性红斑被认为是与胰高血糖素瘤相关的一种必然出现的皮肤副肿瘤体征。胰高血糖素瘤综合征的定义为存在胰腺α细胞分泌肿瘤、胰高血糖素水平升高以及一种名为坏死性游走性红斑的特征性皮疹。尽管坏死性游走性红斑是胰高血糖素瘤综合征的特异性表现，但它也可能在其他情况下出现，与胰腺α细胞瘤无关（假性胰高血糖素瘤综合征）。胰高血糖素瘤极为罕见，这带来了挑战，大多数患者在因皮疹接受长期治疗后才被诊断出来。胰高血糖素瘤的主要预后指标是疾病后期出现的转移。在此，我们报告一名55岁女性患者，有5年未被识别的胰高血糖素瘤综合征皮肤和全身表现病史。根据检查结果，确诊为无转移的胰高血糖素瘤综合征。手术切除胰腺癌/胰高血糖素瘤后，实现了完全治愈且疾病缓解期较长。医生/皮肤科医生对坏死性游走性红斑特征的适当认识通常会导致胰高血糖素瘤综合征的早期诊断，并增加获得良好预后的机会。
