A 48-year-old man presented to the dermatology clinic complaining of an itchy rash that had started 2 months ago. According to the patient, the rash started in the genital area and then progressed to the torso, hands and legs. He was recently diagnosed with diabetes mellitus and since receiving the diagnosis had lost 13 kg weight.
Examination revealed erythematous plaques and patches that were at various stages of healing. Histopathological findings showed ballooning of the upper spinous keratinocytes with a thick crust of necrotic debris. The findings were consistent with necrolytic migratory erythema. In addition, an underlying glucagonoma was suspected.
Necrolytic migratory erythema or dermatitis on the feet, belly and face often filled with pus or crusty is a common presentation of glucagonoma. Other signs include increased blood sugar, excessive thirst and hunger, frequent waking up in the night to urinate, diarrhoea, deep vein thrombosis and unintentional weight loss.
Glucagonoma
Glucagonoma is a rare tumour that involves the alpha cells of the pancreas. The alpha cells of the pancreas produce a hormone glucagon that works with insulin to control the amount of sugar in the blood. The tumour cells produce a large amount of glucagon, creating life-threatening, painful and severe symptoms. 5 to 10% of neuroendocrine tumours that develop in the pancreas are glucagonomas.
The patient’s levels of glucagon were elevated. In addition to this, CT scan of the abdomen showed an exophytic hypervascular mass in the pancreatic tail with focal calcification. Pathological examination confirmed the diagnosis. A distal pancreatectomy was performed and the rash began to abate soon after surgery.
The rash resolved completely 3 months later. There were no signs of rash recurrence or glucagonoma at 5 years of follow-up.
References
Banerjee, A., & Shah, S. R. (2020). Necrolytic Migratory Erythema Associated with a Glucagonoma. New England Journal of Medicine, 383(6), e39.