Necrotising migratory erythema leading to the diagnosis of a metastatic glucagonoma without diabetes

Abstract

A case of necrotising migratory erythema (NME), which is one of the distinctive paraneoplastic skin manifestations associated with the glucagonoma syndrome, is described and discussed. In 80% of all patients with glucagonoma, NME is the first clinical sign. The glucagonoma syndrome is a constellation of clinical features: NME, weight loss, anaemia, diabetes, diarrhoea, thromboembolism and neuropsychiatric symptoms. The global incidence of glucagonoma is one in 20 million people per year. The male to female ratio is 0.8:1 with the mean age of diagnosis being 52.2 years. The median time in relation to the initial onset of symptoms and the correct diagnosis is 3.5 years. The 10-year survival rate in patients with metastatic disease is 51.6% and without metastatic disease 64.3%. SPECT scan has a sensitivity range of 67–100% for detecting neuroendocrine tumours. Differential diagnoses of other skin conditions that mimic NME are: bullous pemphigoid, vasculitis, acrodermatitis enteropathica, chronic mucocutaneous candidiasis, seborrhoeic dermatitis, contact dermatitis, pellagra, inflammatory bowel disease, liver cirrhosis, coeliac disease, chemical burns, eczema, herpes etc. A satisfactory response to somatostatin as medical therapy in a case-study patient with metastatic disease is reported.