RESUMO
Glucagonoma is a rare neuroendocrine tumor of α cells of the pancreas. The tumor excessively secretes glucagon and causes glucagonoma syndrome.70%-90% of patients with glucagonoma will develop necrolytic migratory erythema (NME). We reported a patient of glucagonoma syndrome who was presented to the dermatology outpatient clinic with a 2-year-history of recurrent erythema and scaling on the skin migrating throughout the body. A skin biopsy was performed and resulting features matched with NME, whilst imaging examinations suggested a soft tissue density tumor present in the tail of the pancreas with somatostatin receptor expression and laboratory tests found an elevated levels of serum glucagon. After the diagnosis was confirmed, the patient was treated with surgical resection of the glucagonoma and the skin eruptions resolved rapidly in 4 days. Meanwhile, we reviewed relevant literature published in recent years and summarized its clinical characteristics in order to improve its understanding by clinicians, including clinical manifestations, laboratory and imaging examinations, diagnosis and treatments.
RESUMO
Necrolytic migratory erythema (NME) is a typical cutaneous manifestation of glucagonoma syndrome. The entire syndrome consists of NME, glucose intolerance, weight loss, anemia, glossitis, diarrhea, and increased glucagon levels. We herein report a patient with glucagonoma syndrome who was diagnosed as having NME. A 48-year-old male presented with a 2-month history of painful erythematous, desquamative, erosive papules and plaques on both lower extremities. Histological examination revealed an intraepidermal cleft, the presence of vacuolated, pale epidermal cells, and necrosis in the upper epidermis. His glucagon level was 2650 pg/ml, with the upper limit of a normal range being 250 pg/ml. The patient was treated with octreotide, and showed an improvement of the skin eruption with normalization of the glucagon level within 4 weeks.