Eritema necrolítico migratorio: clave diagnóstica en el síndrome del glucagonoma

Moreno-Suárez, F.; Pulpillo-Ruiz, Águeda; Alberdi, María Fontillón

doi:10.1016/j.medcli.2016.07.001

Cited by 2 publications

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“…Total parenteral nutrition with amino acid and caloric supplementation may be used to counteract the catabolic effects of high glucagon 19. Surgical resection of the pancreatic nodule is indicated whenever possible 25 - 29. After tumor resection, the symptoms of GS commonly decrease 22.…”

Section: Discussionmentioning

confidence: 99%

Diarrhea: a missed D in the 4D glucagonoma syndrome

et al. 2019

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Glucagonoma is a rare and slow-growing pancreatic tumor that usually manifests as glucagonoma syndrome. It is mainly characterized by a typical Dermatosis named necrolytic migratory erythema (NME), Diabetes and glucagon oversecretion. Deep vein thrombosis and Depression complete this set. We report the case of an advanced glucagonoma with liver spread, where all these 4D symptoms occurred but a chronic secretory Diarrhea was the most relevant feature. A 65-year-old man was referred to our center to investigate multiple hepatic nodules evidenced by abdominal tomography. He had a recent diagnosis of diabetes and complained of significant weight loss (25 kg), crusted skin lesions and episodes of a large amount of liquid diarrhea during the past 6 months. On admission, there were erythematous plaques and crusted erosions on his face, back and limbs, plus angular cheilitis and atrophic glossitis. The typical skin manifestation promptly led dermatologists to suspect glucagonoma as the source of our patient’s symptoms. A contrast-enhanced abdominal computed tomography showed a hypervascularized pancreatic lesion and multiple hepatic nodules also hypervascularized in the arterial phase. Despite initial improvement of diarrhea after subcutaneous octreotide, the patient’s impaired nutritional status limited other therapeutic approaches and he died of respiratory failure due to sepsis. His high levels of serum glucagon were not yet available so we performed an autopsy, confirming the diagnosis of metastatic glucagonoma with NME on histology. Chronic diarrhea is not a common feature in glucagonoma syndrome; however, its severity can lead to serious nutritional impairment and set a poor outcome.

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Section: Discussionmentioning

confidence: 99%

Diarrhea: a missed D in the 4D glucagonoma syndrome

et al. 2019

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Glucagonoma syndrome with severe erythematous rash

Wang

Wang²,

Zhao³

2019

Medicine

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Rationale:Glucagonoma is a rare neuroendocrine tumor of the pancreas. Glucagonoma syndrome is often misdiagnosed as other skin lesions by clinicians due to a typical clinical sign of necrolytic migratory erythema (NME) with severe erythematous rash.Patient concerns:A 48-year-old female patient was admitted to our department because she presented with unclear recurrent severe erythematous rash. The patient was diagnosed as skin disease.Diagnoses:Histopathologic examination revealed a pancreatic glucagonoma. Immnohistochemical staining of tumor tissue was positive for glucagon.Interventions:The distal pancreatectomy plus splenectomy was performed in 2017.Outcomes:The skin lesions disappeared after surgery. She was followed up and showed no recurrence until now.Lessons:Clinicians should consider the diagnosis of glucagonoma according to the typical initial symptoms. Early diagnosis is very important to provide a better prognosis. A multidisciplinary approach is effective in patients with unresectable metastatic tumors.

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Eritema necrolítico migratorio: clave diagnóstica en el síndrome del glucagonoma

Cited by 2 publications

References 0 publications

Diarrhea: a missed D in the 4D glucagonoma syndrome

Diarrhea: a missed D in the 4D glucagonoma syndrome

Glucagonoma syndrome with severe erythematous rash

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