Glucagonoma syndrome with atypical necrolytic migratory erythema. 2021

Shujuan He, and Weihui Zeng, and Songmei Geng, and Jinjing Jia
Department of Dermatology, The Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, China.

Necrolytic migratory erythema is most commonly associated with glucagonoma syndrome. We report a rare case of glucagonoma syndrome with necrolytic migratory erythema presenting as pruritic papules and follicular pustules in a 57-year-old woman; showing eosinophilic infiltration on histology. However, the final diagnosis was confirmed by demonstrating neuroendocrine tumour on histopathological examination of the liver metastases. Nutrition therapy was administered as a palliative treatment. This case also highlights the atypical clinical features and nonspecific histology of necrolytic migratory erythema which makes the diagnosis difficult.

UI MeSH Term Description Entries
D008113 Liver Neoplasms Tumors or cancer of the LIVER. Cancer of Liver,Hepatic Cancer,Liver Cancer,Cancer of the Liver,Cancer, Hepatocellular,Hepatic Neoplasms,Hepatocellular Cancer,Neoplasms, Hepatic,Neoplasms, Liver,Cancer, Hepatic,Cancer, Liver,Cancers, Hepatic,Cancers, Hepatocellular,Cancers, Liver,Hepatic Cancers,Hepatic Neoplasm,Hepatocellular Cancers,Liver Cancers,Liver Neoplasm,Neoplasm, Hepatic,Neoplasm, Liver
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D010190 Pancreatic Neoplasms Tumors or cancer of the PANCREAS. Depending on the types of ISLET CELLS present in the tumors, various hormones can be secreted: GLUCAGON from PANCREATIC ALPHA CELLS; INSULIN from PANCREATIC BETA CELLS; and SOMATOSTATIN from the SOMATOSTATIN-SECRETING CELLS. Most are malignant except the insulin-producing tumors (INSULINOMA). Cancer of Pancreas,Pancreatic Cancer,Cancer of the Pancreas,Neoplasms, Pancreatic,Pancreas Cancer,Pancreas Neoplasms,Pancreatic Acinar Carcinoma,Pancreatic Carcinoma,Acinar Carcinoma, Pancreatic,Acinar Carcinomas, Pancreatic,Cancer, Pancreas,Cancer, Pancreatic,Cancers, Pancreas,Cancers, Pancreatic,Carcinoma, Pancreatic,Carcinoma, Pancreatic Acinar,Carcinomas, Pancreatic,Carcinomas, Pancreatic Acinar,Neoplasm, Pancreas,Neoplasm, Pancreatic,Neoplasms, Pancreas,Pancreas Cancers,Pancreas Neoplasm,Pancreatic Acinar Carcinomas,Pancreatic Cancers,Pancreatic Carcinomas,Pancreatic Neoplasm
D010257 Paraneoplastic Syndromes In patients with neoplastic diseases a wide variety of clinical pictures which are indirect and usually remote effects produced by tumor cell metabolites or other products. Paraneoplastic Syndrome,Syndrome, Paraneoplastic,Syndromes, Paraneoplastic
D005260 Female Females
D005935 Glucagonoma An almost always malignant GLUCAGON-secreting tumor derived from the PANCREATIC ALPHA CELLS. It is characterized by a distinctive migratory ERYTHEMA; WEIGHT LOSS; STOMATITIS; GLOSSITIS; DIABETES MELLITUS; hypoaminoacidemia; and normochromic normocytic ANEMIA. Adenoma, alpha-Cell,alpha-Cell Tumor,Glucagonoma Syndrome,Adenoma, alpha Cell,Adenomas, alpha-Cell,Glucagonoma Syndromes,Glucagonomas,Syndrome, Glucagonoma,Syndromes, Glucagonoma,Tumor, alpha-Cell,Tumors, alpha-Cell,alpha Cell Tumor,alpha-Cell Adenoma,alpha-Cell Adenomas,alpha-Cell Tumors
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D058568 Necrolytic Migratory Erythema Recurrent cutaneous manifestation of GLUCAGONOMA characterized by necrolytic polycyclic migratory lesions with scaling borders. It is associated with elevated secretion of GLUCAGON by the tumor. Other conditions with elevated serum glucagon levels such as HEPATIC CIRRHOSIS may also result in similar skin lesions, which are referred to as pseudoglucagonoma syndrome. Pseudoglucagonoma Syndrome,Erythema, Necrolytic Migratory,Erythemas, Necrolytic Migratory,Migratory Erythema, Necrolytic,Migratory Erythemas, Necrolytic,Necrolytic Migratory Erythemas,Pseudoglucagonoma Syndromes,Syndrome, Pseudoglucagonoma,Syndromes, Pseudoglucagonoma
D018358 Neuroendocrine Tumors Tumors whose cells possess secretory granules and originate from the neuroectoderm, i.e., the cells of the ectoblast or epiblast that program the neuroendocrine system. Common properties across most neuroendocrine tumors include ectopic hormone production (often via APUD CELLS), the presence of tumor-associated antigens, and isozyme composition. Neuroendocrine Tumor,Tumor, Neuroendocrine,Tumors, Neuroendocrine

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