Clinical Presentation & Protocol
Patient Usually Complains Of
Patient presents with a history of characteristic necrolytic migratory erythema (NME), typically involving the perioral, perineal, and distal extremity regions. Associated symptoms include significant weight loss, new-onset or poorly controlled diabetes mellitus, glossitis, cheilitis, and episodic diarrhea. History of venous thromboembolism (VTE) or neuropsychiatric symptoms should be noted.
Clinical Examination Findings
Physical examination reveals characteristic erythematous, blistering, and crusting plaques in a migratory pattern, often with central clearing. Oral examination shows glossitis and angular cheilitis. Abdominal palpation may reveal a palpable mass or hepatomegaly if metastatic disease is present. Assess nutritional status, skin integrity, and signs of peripheral edema or deep vein thrombosis.
Treatment Protocol
Management includes surgical resection of the primary pancreatic neuroendocrine tumor (pNET) if feasible. Medical management for hyperglucagonemia includes somatostatin analogs (e.g., octreotide, lanreotide) to control symptoms and skin lesions. Nutritional support with zinc supplementation, amino acids, and fatty acids is indicated. Management of secondary diabetes and prophylaxis against VTE are essential components of the treatment plan.