Wernicke Encephalopathy Associated with Malabsorption in Degos Disease.

A 40-year-old female with systemic Degos disease presented with 4 days of confusion, somnolence, and ataxia. She was dependent on total parenteral nutrition (TPN) following ileocecectomy for Degos-related gastrointestinal complications and had recently transitioned from intravenous (IV) to oral thiamine supplementation. Although central nervous system involvement of Degos was initially suspected, magnetic resonance imaging findings, low thiamine levels, and rapid improvement with IV thiamine confirmed Wernicke's encephalopathy. This case highlights the importance of recognizing nutritional deficiencies in TPN-dependent patients with neurologic symptoms, even in the context of a rare underlying disease.