Abstract
We diagnosed a 17-year-old woman whose subcutaneous adipose tissue began to decrease in her late elementary school days as a case of acquired and generalized lipoatrophic diabetes. She had extreme insulin resistance, fatty liver, hyperlipidemia, elevation of basal metabolism, and decreased subcutaneous fat mass, white hair, hyperkeratinized skin, and arachnodactylia. Insulin binding capacity to her EBV-transformed lymphocytes was about half that of control lymphocytes. Her serum insulin gradually decreased, and her glycemic control worsened even with 1,000 units of insulin per day. To overcome this severe insulin resistance, we added pioglitazone hydrochloride to insulin therapy. Her glycemic control improved with the increase in serum leptin and adiponectin. After the start of pioglitazone, bilateral subcutaneous axillary lipoimas were found, and her liver enlarged.
