Endocrine Abstracts

Introduction: Generalized lipodystrophies are rare disorders characterized by almost total loss of adipose tissue, and they are often accompanied by metabolic complications such as severe insulin resistance, diabetes mellitus, hypertriglyceridemia, and fatty liver.

Case report: We diagnosed a 17-year-old woman whose subcutaneous adipose tissue began to decrease in her late elementary school days with the acquired and generalized types of idiopathic lipoatrophic diabetes. She had no family history of lipoatrophy. She had extreme insulin resistance, fatty liver, hyperlipidemia, and an increased basal metabolic rate in addition to a decreased subcutaneous fat mass, partial white hair, acanthosis nigricans, hyperkeratinized skin of the fingers and toes, and arachnodactyly. On first examination, height was 164 cm, weight was 43 kg, fasting plasma glucose level was 100 mg/dl, glycated hemoglobin (HbA1c) level was 6.7%, and fasting immunoreactive insulin level was 80 IU/ml. Her serum insulin level gradually decreased, her glycemic control worsened, and her HbA1c level increased above 12% even with 1000 U of insulin per day. To overcome this severe insulin resistance, we added pioglitazone hydrochloride to the insulin therapy. Her glycemic control successfully improved along with an increase in the serum leptin (before and after pioglitazone medication: 4.5 and 9.6 ng/dl, respectively) and adiponectin levels (1.24 and 3.10 mg/ml, respectively). After starting pioglitazone, subcutaneous axillary lipomas on both sides of her body (right 9.7 × 7.6 cm, left 7.1 × 6.4 cm) were found, and fatty liver was improved.

Conclusions: Pioglitazone (thiazolidinedione) is a ligand for peroxisome proliferator-activated receptor-γ a nuclear receptor expressed mainly in adipocytes that promotes their development. In the present case, pioglitazone caused proliferation of the rest of the adipocytes and subcutaneous axillary lipoma growth. The current case also demonstrated that the metabolic complications of lipoatrophic diabetes can be partially resolved with pioglitazone treatment.