CASE REPORT Glucagonoma syndrome: a case …...lesions vanished. When comparing our results to review other cases in the literature, clinical presentation of our patient was typical.
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OncoTargets and Therapy 2014:7 1449–1453
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http://dx.doi.org/10.2147/OTT.S66285
Glucagonoma syndrome: a case report with focus on skin disorders
Sheng FangShuang LiTao CaiDepartment of Dermatology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, People’s Republic of China
Correspondence: Tao Cai Department of Dermatology, The First Affiliated Hospital of Chongqing Medical University, 1 Youyi Road, Chongqing, 400016, People’s Republic of China Email [email protected]
Abstract: Glucagonoma syndrome is a rare paraneoplastic phenomenon. Necrolytic migratory
erythema is often one of the first presenting symptoms. We report a case of a 55-year-old man
with a 2-year history of recalcitrant eruption. Abdominal computer tomography was performed,
which revealed a tumor in the tail of the pancreas. After pancreatectomy, his cutaneous lesions
vanished in a few days. Skin symptoms are important, as they are often essential for early
diagnosis of glucagonoma syndrome and may prevent metastatic disease; hence, in this report,
we focus on skin disorders in glucagonoma syndrome.
Keyword: necrolytic migratory erythema
IntroductionGlucagonoma syndrome is a rare paraneoplastic phenomenon defined as necrolytic
eczema, and pemphigus. Usually, the correct diagnosis is
delayed. Since prompt diagnosis and treatment may preclude
metastasis of the tumor and lead to a better prognosis, it is
important to keep the diagnosis of glucagonoma syndrome
in mind.
As glucagonomas are malignant, treatment should be
undertaken as soon as possible after diagnosis. An effective
treatment strategy should decrease the serum glucagon levels.
Surgical removal of the tumor is the generally accepted treat-
ment because of the weak response to chemotherapy. Before
operation, efforts should be made to control glucose levels,
improve skin rash, give nutritional support, and prevent vein
thrombosis. If tumor metastasis has occurred, long-acting Figure 3 Computed tomography scan of the abdomen of the patient showing a large tumor in the tail of the pancreas.
Figure 2 Skin biopsy in necrolytic migratory erythema showing a large zone of necrolysis in the upper epidermis (arrow).Notes: Hematoxylin and eosin staining; magnification ×40.
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Glucagonoma syndrome
DisclosureThe authors report no conflicts of interest in this work.
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