Track: Case Reports

Topic: Inflammatory Diseases

Abstract:

A 49 year old male with history of alcohol use disorder and Roux-en-Y gastric bypass was admitted to the hospital for fatigue, weakness, and tachycardia. Dermatology was consulted for recent history of a widespread, scaly rash associated with pruritus. Upon examination, erythematous, scaly patches were scattered across the patient’s trunk and extremities, some with arcuate, circinate, and polycyclic morphology. Significant desquamation was noted on the plantar feet, and mucosae were spared. Punch biopsy of an abdominal lesion demonstrated broad and diffuse parakeratosis overlying superficial epidermal pallor; findings consistent with necrolytic migratory erythema. Abdominal imaging failed to demonstrate pancreatic mass or inflammation; additionally, hepatitis A/B/C and HIV testing were negative. Further workup revealed low pre-albumin, zinc, copper, vitamin A/D/E, and biotin, suggesting marked nutritional deficiency. After aggressive vitamin and mineral repletion, patient was discharged home, and cutaneous findings were significantly improved at outpatient dermatology follow up. Weeks later, the patient was subsequently readmitted to the hospital with pancytopenia and abdominal ascites. He then unfortunately passed away due to complications associated with hypoalbuminemia from worsening nutritional deficiency. In summary, necrolytic migratory erythema describes a rare and characteristic papulosquamous eruption that is classically associated with a glucagon-secreting alpha-cell tumor of the pancreas. However, even less frequently, a “pseudoglucagonoma syndrome” in the absence of a pancreatic tumor may result from nutrient malabsorption, leading to identical clinical and histopathological findings. Our patient’s case emphasizes the importance of recognizing this unique entity due to the serious outcomes that can result from nutritional deficiency.

Margaret Newsome

Affiliation

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(157) Pseudoglucagonoma Syndrome Presenting with Necrolytic Migratory Erythema