Abstract

Genetic types of extreme insulin resistance include type A insulin resistance syndrome, leprechaunism, congenital generalized lipodystrophy, and Rabson-Mendelhall syndrome (1). Acanthosis nigricans and ovarian masculinization are frequently associated with these syndromes. Genetic defects in insulin action at the receptor or postreceptor levels are responsible for insulin resistance. Since thiazolidinediones enhance insulin sensitivity in patients with insulin resistance, we administered pioglitazone to an insulin-resistant patient with a mutation of the insulin receptor. A 15-year-old Japanese female was referred to the hospital for an evaluation of hirsutism. Acanthosis nigricans was present in the axillary area and on the neck. She had been amenorrheic since menarche at the age of 12 years. She had …

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