Long‐term treatment experience in a subject with Dunnigan‐type familial partial lipodystrophy: efficacy of rosiglitazone

Dunnigan‐type familial partial lipodystrophy (FPLD) is caused by mutations in LMNA , the gene that encodes nuclear lamins A and C. FPLD is characterized by peripheral fat loss, excess central adiposity, insulin resistance, and hyperlipidaemia, which are difficult to treat. We present our 2 years’ experience of treatment with rosiglitazone in a subject with FPLD. Insulin requirement decreased significantly from 240 IU/day to 76 IU/day (range 20–240 IU/day) and serum triglyceride concentration was lowered from 13.7 ± 14.4 mmol/l to 4.5 ± 4.3 mmol/l and remained stable. Mean HbA 1c prior to rosiglitazone therapy was 9.4 ± 1.32% and decreased to 7.4 ± 0.6% during therapy with rosiglitazone. This case demonstrates the benefits of PPARγ‐agonists on glycaemic control and dyslipidaemia in a patient with FPLD. This in turn implies that PPARγ may play a pathophysiological role in FPLD.