John A M, Schwartz R A
Dermatology and Pathology, Rutgers-New Jersey Medical School, Newark, NJ, USA.
Rutgers University School of Public Affairs and Administration, Newark, NJ, USA.
J Eur Acad Dermatol Venereol. 2016 Dec;30(12):2016-2022. doi: 10.1111/jdv.13752. Epub 2016 Jul 16.
Glucagonoma syndrome is defined by the presence of an alpha-cell secreting tumour of the pancreas, elevated levels of glucagon, and a characteristic rash called necrolytic migratory erythema (NME). NME is usually a specific and often initial finding of glucagonoma syndrome, but it may occur in other settings unassociated with an alpha-cell pancreatic tumour (pseudoglucagonoma syndrome). Glucagonoma syndrome must be distinguished from pseudoglucagonoma syndrome. Prompt recognition of NME and subsequent workup for a glucagonoma can allow for an earlier diagnosis and enhance the chances of a favourable outcome. In particular, metastases occur late, so early recognition of glucagonoma syndrome before liver metastases can be life-saving. Surgical resection is the definitive treatment for glucagonoma syndrome, although chemotherapeutic agents, somatostatin analogues and radionuclide therapy are also employed. Herein, we offer an approach to workup after identifying NME and an update on its current treatment modalities.
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称为坏死性游走性红斑（NME）的特征性皮疹。NME通常是胰高血糖素瘤综合征的特异性且常为首发表现，但也可能出现在与胰腺α细胞瘤无关的其他情况下（假性胰高血糖素瘤综合征）。必须将胰高血糖素瘤综合征与假性胰高血糖素瘤综合征区分开来。及时识别NME并随后对胰高血糖素瘤进行检查可实现早期诊断并增加获得良好预后的机会。特别是，转移发生较晚，因此在肝转移之前早期识别胰高血糖素瘤综合征可能挽救生命。手术切除是胰高血糖素瘤综合征的确定性治疗方法，不过也会使用化疗药物、生长抑素类似物和放射性核素疗法。在此，我们提供一种在识别NME后进行检查的方法以及其当前治疗方式的最新情况。
