Prominent Insulin Resistance in Congenital Generalized Lipoatrophy

A 35-year-old female was admitted to the division of endocrinology and metabolism for generalized lipoatrophy and hyperglycemia. She was markedly deficient of adipose tissue since birth and developed diabetes and hypertension at the age of 20 years. She began receiving premixed biosynthetic human insulin with a ratio of 30% regular and 70% NPH (neutral protamine Hagedorn) insulin at the age of 27 years. The glycemic control remained poor despite the increase in her insulin dose to 60–80 units/day. Her hemoglobin A1c levels remained high, and they varied between 8 and 11%. Current admission revealed a female with 161 cm height, body weight 53.0 kg, body mass index (BMI) 20.4 kg/m2, and systemic blood pressure of 146/86 mm Hg. She had muscular stature with generalized loss of subdermal fatty tissue. She also had umbilical hernia and acromegalic features with slight enlargement of her hands, feet, and mandible (Fig. 3.1a). She had difficulty in controlling her excessive appetite.