THU536 Pseudoglucagonoma Syndrome: A Rare Complication After Partial Pancreatectomy

Disclosure: E.P. Monsour: None. S. Badour: None. K.M. Tuna: None. J. Leey: None. M. Lamas: None. Introduction/Background: Necrolytic migratory erythema (NME) is a specific cutaneous eruption associated with glucagonoma, an alpha-cell secreting pancreatic neuroendocrine tumor. Glucagonoma syndrome is a paraneoplastic syndrome characterized by NME, diabetes, stomatitis, anemia, weight loss, and gastrointestinal disturbances. Rarely NME can occur in the absence of an alpha-cell tumor of the pancreas, termed pseudoglucagonoma syndrome, seen in other clinical entities such as hepatic disease and intestinal malabsorption. Here, we describe a rare case of pseudoglucagonoma syndrome as a long-term complication following partial pancreatectomy. Clinical Case: A 45-year-old male with a history of benign pancreatic head cyst s/p partial pancreatectomy, pancreatic exocrine insufficiency, and pancreatogenic diabetes reported a 2-week history of a rash on his bilateral upper and lower extremities that was accompanied with blistering and skin peeling. Associated symptoms included tongue pain, diarrhea, and worsening paresthesias. Medications included subcutaneous insulin aspart, pantoprazole, and pancrelipase. Physical examination revealed a patchy rash with hyperpigmented plaques and shellac-like scales, a smooth red tongue, and symmetric hyperreflexia. Preliminary punch biopsy was concerning for NME; thus, the patient was evaluated for possible glucagonoma. Laboratory evaluation revealed normocytic anemia with hemoglobin of 9.8 mg/dL, plasma glucose of 104 mg/dL, and glucagon level of 103 ng/L (RR: <208 ng/L). MRCP was negative for any pancreatic lesions. The patient was further evaluated for nutritional deficiencies and had strinkingly low vitamin A, D, E, K, B6, serum copper, and zinc levels. Plasma amino acids revealed low phenylalanine and tryptophan levels. He was started on a multivitamin (with minerals) and oral nutritional supplements with resolution of rash and subsequent improvement in symptoms. Final pathology of punch biopsy revealed parakeratosis with dermal edema, most compatible with nutritional deficiency. Discussion and conclusion: The pathogenesis and etiology of NME in glucagonoma and pseudoglucagonoma syndromes are not entirely understood. NME is thought to be multifactorial to multiple (essential) nutrient and vitamin deficiencies, as seen in our patient and other nutrient deficiency dermatoses such as zinc deficiency, necrolytic acral erythema, and pellagra. After further investigation, the patient reported poor adherence to pancrelipase supplementation, likely contributing to his clinical presentation and symptoms. Whereas in glucagonoma, excess glucagon levels cause a state of catabolism and exert its effect on the liver to increase gluconeogenesis via amino acid catabolism leading to deficiency. It is essential for clinicians to be aware of this rare and underrecognized phenomenon. Presentation: Thursday, June 15, 2023