NECROLYTIC MIGRATORY ERYTHAEMA AND GLUCAGONOMA SYNDROME

Abstract

A 52-year old woman presented with vomiting, vague abdominal pain, progressive weight loss (85 kg to 76 kg) over the past 6 months and recurrent skin eruptions on her face, perineum and the extremities (fi gure A,B,C) which were diagnosed and treated as eczema. First evaluation revealed elevated fasting glucose level (139 mg/dl normal value 60-105) and oesophagogastroduodenoscopy showed refl ux oesophagitis grade A. CT scan and MRI of the abdomen showed a nodular hypervascular mass of 6,5 cm in the tail of the pancreas with lymph node involvement and multiple liver metastases (fi gure D). Other laboratory testing revealed markedly elevated glucagon (2970 pg/ml, normal value < 250), chromogranin A (517 μg/l, normal value 40-70). Diagnosis of a glucagonoma syndrome with necrolytic migratory erythema (NME) was made. Octreotide LAR, 20 mg once monthly was started. She underwent resection of the pancreatic tail and splenectomy. Three months later she underwent two sessions of transarterial doxorubicin based chemoembolisation of the liver, with stabilisation of her disease. She is still in follow-up with three monthly evaluations. Glucagonoma syndrome is a rare malignant disorder of the pancreas tumour, which comprises hyperglucagonaemia, diabetes mellitus, anaemia, weight loss, fatigue and NME. The incidence is 1 in 20 million per year. Dermatologic changes in NME may be misdiagnosed as they can suggest other dermatoses. The pathogenesis of NME remains controversial. Improvement in NME is often seen after surgery, somatostatin analogues and after supplementation. Spiral CT is the initial imaging procedure of choice. The optimal treatment for glucagonoma is dependent on the stage of the tumour and