Necrolytic Migratory Erythema—A Case Report of Pancreatic Occupying Lesion

Glucagonoma is a rare neuroendocrine tumor of α cells of the pancreas. The tumor excessively secretes glucagon and causes glucagonoma syndrome.70%-90% of patients with glucagonoma will develop necrolytic migratory erythema (NME). We reported a patient of glucagonoma syndrome who was presented to the...

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Main Authors: HU Zhonghui, YANG Lu, LIU Yuehua, JIN Hongzhong, XU Qiang, LIU Wenjing, LUO Yaping, YANG Hongbo, WANG Tao
Format: Article
Language:zho
Published: Editorial Office of Journal of Rare Diseases 2023-07-01
Series:罕见病研究
Subjects:
Online Access:https://jrd.chard.org.cn/article/doi/10.12376/j.issn.2097-0501.2023.03.014
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author HU Zhonghui
YANG Lu
LIU Yuehua
JIN Hongzhong
XU Qiang
LIU Wenjing
LUO Yaping
YANG Hongbo
WANG Tao
author_facet HU Zhonghui
YANG Lu
LIU Yuehua
JIN Hongzhong
XU Qiang
LIU Wenjing
LUO Yaping
YANG Hongbo
WANG Tao
author_sort HU Zhonghui
collection DOAJ
description Glucagonoma is a rare neuroendocrine tumor of α cells of the pancreas. The tumor excessively secretes glucagon and causes glucagonoma syndrome.70%-90% of patients with glucagonoma will develop necrolytic migratory erythema (NME). We reported a patient of glucagonoma syndrome who was presented to the dermatology outpatient clinic with a 2-year-history of recurrent erythema and scaling on the skin migrating throughout the body. A skin biopsy was performed and resulting features matched with NME, whilst imaging examinations suggested a soft tissue density tumor present in the tail of the pancreas with somatostatin receptor expression and laboratory tests found an elevated levels of serum glucagon. After the diagnosis was confirmed, the patient was treated with surgical resection of the glucagonoma and the skin eruptions resolved rapidly in 4 days. Meanwhile, we reviewed relevant literature published in recent years and summarized its clinical characteristics in order to improve its understanding by clinicians, including clinical manifestations, laboratory and imaging examinations, diagnosis and treatments.
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spelling doaj.art-ebd0b8fe17d84f6b9d912dcd2e439a052024-01-02T05:49:33ZzhoEditorial Office of Journal of Rare Diseases罕见病研究2097-05012023-07-012342743110.12376/j.issn.2097-0501.2023.03.014Necrolytic Migratory Erythema—A Case Report of Pancreatic Occupying LesionHU Zhonghui0YANG Lu1LIU Yuehua2JIN Hongzhong3XU Qiang4LIU Wenjing5LUO Yaping6YANG Hongbo7WANG Tao8Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, ChinaDepartment of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, ChinaDepartment of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, ChinaDepartment of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, ChinaDepartment of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, ChinaDepartment of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, ChinaDepartment of Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, ChinaDepartment of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, ChinaDepartment of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, ChinaGlucagonoma is a rare neuroendocrine tumor of α cells of the pancreas. The tumor excessively secretes glucagon and causes glucagonoma syndrome.70%-90% of patients with glucagonoma will develop necrolytic migratory erythema (NME). We reported a patient of glucagonoma syndrome who was presented to the dermatology outpatient clinic with a 2-year-history of recurrent erythema and scaling on the skin migrating throughout the body. A skin biopsy was performed and resulting features matched with NME, whilst imaging examinations suggested a soft tissue density tumor present in the tail of the pancreas with somatostatin receptor expression and laboratory tests found an elevated levels of serum glucagon. After the diagnosis was confirmed, the patient was treated with surgical resection of the glucagonoma and the skin eruptions resolved rapidly in 4 days. Meanwhile, we reviewed relevant literature published in recent years and summarized its clinical characteristics in order to improve its understanding by clinicians, including clinical manifestations, laboratory and imaging examinations, diagnosis and treatments.https://jrd.chard.org.cn/article/doi/10.12376/j.issn.2097-0501.2023.03.014necrolytic migratory erythemaglucagonomaglucagonoma syndromepancreatic occupying lesion
spellingShingle HU Zhonghui
YANG Lu
LIU Yuehua
JIN Hongzhong
XU Qiang
LIU Wenjing
LUO Yaping
YANG Hongbo
WANG Tao
Necrolytic Migratory Erythema—A Case Report of Pancreatic Occupying Lesion
罕见病研究
necrolytic migratory erythema
glucagonoma
glucagonoma syndrome
pancreatic occupying lesion
title Necrolytic Migratory Erythema—A Case Report of Pancreatic Occupying Lesion
title_full Necrolytic Migratory Erythema—A Case Report of Pancreatic Occupying Lesion
title_fullStr Necrolytic Migratory Erythema—A Case Report of Pancreatic Occupying Lesion
title_full_unstemmed Necrolytic Migratory Erythema—A Case Report of Pancreatic Occupying Lesion
title_short Necrolytic Migratory Erythema—A Case Report of Pancreatic Occupying Lesion
title_sort necrolytic migratory erythema a case report of pancreatic occupying lesion
topic necrolytic migratory erythema
glucagonoma
glucagonoma syndrome
pancreatic occupying lesion
url https://jrd.chard.org.cn/article/doi/10.12376/j.issn.2097-0501.2023.03.014
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