D Sai Kavya, S Sukesh Gautam, Wahab Afthab Jameela
Department of Dermatology, Saveetha Medical College and Hospitals, Saveetha Institute of Medical and Technical Sciences (SIMATS) Saveetha University, Chennai, IND.
Cureus. 2024 Apr 11;16(4):e58076. doi: 10.7759/cureus.58076. eCollection 2024 Apr.
Pseudoglucagonoma syndrome is defined as the presence of necrolytic migratory erythema in the absence of a glucagon-secreting tumor. Necrolytic migratory erythema is the hallmark of glucagonoma syndrome but can also occur due to pancreatitis, pancreatic insufficiency, gastrointestinal dysfunction, inflammatory bowel disease, celiac disease, malabsorption disorders, nutritional deficiencies, hepatocellular dysfunction, and hypoalbuminemia. Pseudoglucagonoma syndrome is extremely rare, and the diagnosis is often delayed, resulting in delayed treatment. We report a rare case of pseudoglucagonoma syndrome in a malnourished male patient following Frey's surgery. The patient presented with a skin rash which gradually progressed over 20 days with diffuse hair loss. On cutaneous examination, multiple irregular erythematous and eroded plaques surrounded by a hyperpigmented scaly border were present over the dorsal aspect of the lower limbs, upper limbs, gluteal region, and genitals. Routine investigations showed normocytic normochromic anemia, neutropenia, lymphocytosis, dyslipidemia, and hypoalbuminemia. Rapid resolution of the skin lesions was observed with improved nutrition.
假胰高血糖素瘤综合征的定义为，在无分泌胰高血糖素肿瘤的情况下出现坏死松解性游走性红斑。坏死松解性游走性红斑是胰高血糖素瘤综合征的标志，但也可因胰腺炎、胰腺功能不全、胃肠功能障碍、炎症性肠病、乳糜泻、吸收障碍、营养缺乏、肝细胞功能障碍和低白蛋白血症而发生。假胰高血糖素瘤综合征极为罕见，诊断往往延迟，导致治疗延误。我们报告一例在Frey手术后发生于一名营养不良男性患者的罕见假胰高血糖素瘤综合征病例。该患者出现皮疹，在20天内逐渐进展并伴有弥漫性脱发。皮肤检查发现，下肢、上肢、臀部和生殖器背侧有多个不规则的红斑和糜烂斑块，周围有色素沉着的鳞屑边界。常规检查显示正细胞正色素性贫血、中性粒细胞减少、淋巴细胞增多、血脂异常和低白蛋白血症。随着营养状况改善，皮肤病变迅速消退。
