MON-587 A Case of Pseudoglucagonoma Syndrome Post Bariatric Surgery

Introduction Necrotizing migratory erythema (NME) is hallmark clinical finding of Glucagonoma, an islet cell tumor of the pancreas. NME can sometimes be seen in the absence of Glucagonoma, a condition referred to as Pseudo-glucagonoma syndrome(PGS). We report a case of NME associated with severe nutritional deficiency. Case 48 y/o female presented to dermatology clinic with diffuse itchy rash of 6 months duration. The rash started on the arms and spread to involve lower abdomen, legs and perioral area. It was unresponsive to both topical and high dose PO steroids. Biopsy of the rash showed confluent parakeratosis and mild spongiosis secondary to nutritional deficiencies vs NME. Her medical history was significant for Nissen fundoplication 20 years ago with a revision to Roux-en Y gastric by-pass, 6 years ago. She did not have a history of Diabetes or Inflammatory Bowel Disease. She was not on any vitamin supplementation, as she did not follow up for post bariatric care. On admission to the hospital for sepsis from secondary infection of the rash, diffuse erythematous, eczematous papules and plaques were noted on bilateral forearms, thighs, calves, buttocks and ankles. She also had perioral erythema and fissuring. Laboratory evaluation showed low prealbumin and multiple nutritional deficiencies including copper, zinc, vitamin B6, Vitamin D, ferritin. Her HbA1c was 5.8 % and fasting glucagon levels were normal. Her liver function was initially normal but later developed transaminitis from septic shock. She was started on enteral feeds with nutritional supplementation. This resulted in significant improvement of her rash with correction of underlying nutritional deficiencies. Discussion NME in the absence of Glucagonoma is extremely rare and is seen in hepatic cirrhosis, malabsorption disorders, inflammatory bowel disease and nutritional deficiencies including zinc deficiency, Pellagra, Kwashiorkor. Though the exact mechanism for NME in these conditions is unclear. It is postulated that unabsorbed nutrients in the gut lumen are potent stimulators of enteroglucagon which in turn mediates the development of NME. The treatment of NME associated with PGS is to correct the underlying cause. Our patient had history of gastric bypass surgery and did not get routine post bariatric care. She presented with multiple nutritional deficiencies which likely caused NME. It is important to recognize that post bariatric surgery, patients are at risk for both macro and micronutrient deficiencies and hence need frequent nutrition assessment, supplementation and monitoring. References Tierney EP, Badger J. Etiology and pathogenesis of necrolytic migratory erythema: review of the literature. MedGenMed 2004