Necrotising migratory erythema leading to the diagnosis of a metastatic glucagonoma without diabetes
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Date
2020-08
Authors
Makan, Rahm
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Publisher
University of the Free State
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 IO-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 bums, eczema, herpes etc. A satisfactory response to somatostatin as medical therapy in a casestudy patient with metastatic disease is reported.
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Keywords
Dissertation (M.Med (Internal Medicine) -- University of Free State, 2020, Glucagon, Glucagonoma, Neuroendocrine, Octeotide, SPECT, Somatostatin, Pulmonary embolus, Venous thromboembolism, Diabetes, Necrotising migratory erythema - Case studies, Diffuse skin rash, Chronic diarrhoea