Glucagonomas are
neuroendocrine tumors (NETs) that arise from the alpha cells of the pancreatic islets. They are typically slow-growing
tumors associated with abnormal
glucagon secretion, resulting in one or more non-specific clinical features, such as
necrolytic migratory erythema (NME), diabetes,
diarrhea,
deep vein thrombosis,
weight loss, and depression. Here, we report the case of a 44-year-old male with a history of
diabetes mellitus, presenting with a pruritic and painful disseminated cutaneous eruption of erythematous plaques, with scales and peripheral pustules, misdiagnosed as disseminated pustular
psoriasis and treated for 2 years with oral
retinoid and
glucocorticoids. During this period, the patient complained of
weight loss of 32 kg and
diarrhea and developed
deep vein thrombosis. These symptoms, together with an inadequate response to
therapy of the skin lesions, led to the reassessment of the initial diagnosis. Laboratory tests confirmed elevated plasma
glucagon levels (>1000 pg/mL) and computed tomography (CT) scans revealed a 35/44 mm
tumor in the pancreatic tail. Due to considerable disease complications and the
COVID-19 pandemic, the surgical removal of the
tumor was delayed for nearly 2 years. During this time,
somatostatin analogue
therapy efficiently controlled the
glucagonoma syndrome and likely prevented
tumor progression. As in other functional pancreatic NETs, the early clinical recognition of hormonal hypersecretion syndrome and the multidisciplinary approach are the keys for best patient management.