胰高血糖素瘤综合征1例

Glucagonoma syndrome :a case report

报道1例46岁男性病人,全身皮肤红斑、水疱3年,加重1年,具有典型的坏死松解性游走性红斑,伴舌炎、糖尿病,体重下降,血胰高血糖素增高。皮肤组织病理变化为表皮浅层坏死,免疫组化发现表皮浅层抗人胰高血糖素抗体着色阳性。腹部CT及锝标记生长抑素类似物(Octreotide)扫描显示患者胰尾及肝脏肿瘤。长效生长抑素治疗后,血胰高血糖素水平在短期内明显下降,皮疹逐渐好转。

A46-year-old male presented with erythemas and blisters for three years that exacerbated for one year was re-ported.He had clinically characteristic necrolytic migratory erythema(NME),glossitis,diabetes mellitus,weight loss and hy-perglucagonemia.Skin biopsies showed necrosis of the superficial epidermis and immunohistochemical studies revealed glucagon deposition in the same areas.Computerized topography scanning and Octreotide radiography revealed multiple tu-mors in his pancreas tail and liver.After treatment with somatostatin analog,Lanreotide,the level of blood glucagon de-creased and the skin rash re covered gradually.