A Case of pseudoglucagonoma Syndrome Treated with Medium-chain Triglyceride

Seo, Kyung Hyung; Park, Jung Hum; Jang, Ho Sun; Kwon, Kyung Sool; Chung, Tae Ahn

Abstract

Necrolytic migratory erythema is a rare, distinctive, necrotizing cutaneous eruption usually associated with glucagonoma, an alpha-cell tumor of the pancreas. Other clinical features include ane- mia, diabetes mellitus and weight loss. However, it also occurrs without the pancreatic glucagonoma. So this rare condition is called pseudoglucagonoma syndrome, and has been described in association with chronic hepatic diseases, malabsorption with villous atrophy, chronic pancreatitis, celiac sprue, hypoproteinemia and odontogenic infection. We report a case of pseudoglucagonoma syndrome induced by malnutrition without glucagonoma. A 28-year-old Korean female was referred to our department complaining of multiple erythematous, desquamative, crusted and erosive patches on the extremities, and diffuse scalp alopecia for 5 years. She also had perianal erosion, angular cheilitis without glossitis, amenorrhea, axillary and pubic hair loss, and weight loss. Six years previously she had had Whipples operation for a pancreatic solid and papillary neoplasm. The laboratory data showed hypoalbuminemia, essential fatty acid deficiency, diabetes mellitus and slightly high levels of zinc and glucagon. A skin biopsy showed irregular acanthosis, epidermal edema and pallor, dyskeratosis, areas of superficial epidermal necrosis and vascular dilatation of the papillary dermis. The patient improved dramatically after intravenous hyperalimentation and oral intake of mediurn chain triglyceride. For 2.5 years she has taken medium-chain triglycerides without any other treatment, and no side effects on recurrence has been observed.