Insulin resistance, acanthosis nigricans, and hypertriglyceridemia

Abstract

A 23-year-old white female was seen in consultation in the Dermatology Clinic at the National Institutes of Health, in Bethesda, Maryland for plaques involving the neck, axillae, and inguinal areas. At 4 months of age, she was noted to have hepatomegaly and hypertriglyceridemia with values as high as 6000 mg/dL. The patient described a ravenous appetite from a young age. Type 2 diabetes mellitus was diagnosed at age 12 and, despite attempts at dietary modification, her blood glucose levels remained poorly controlled. Treatment with metformin was initiated at age 14, followed shortly thereafter by insulin because of continued poor control. Menarche occurred at age 15, and the patient described a history of oligomenorrhea. A liver biopsy at age 18 prompted by elevated transaminases yielded a diagnosis of nonalcoholic steatohepatitis (NASH) with early cirrhosis. The patient's medications at the time of presentation included metformin 850 mg three times a day and U-500 insulin totaling 800 units daily.