Effectiveness of gastric bypass surgery in a patient with familial partial lipodystrophy.

F amilial partial lipodystrophy (FPL) is associated with loss of subcutaneous fat in the extremities but preservation, or increase, of fat in the face, neck, and trunk. Patients with FPL manifest marked insulin resistance and develop diabetes and hypertriglyceridemia that are often very difficult to manage with pharmacologic agents, the current standard of therapy for FPL. Roux-en-Y gastric bypass (RYGB), a surgical procedure that leads to marked weight loss in morbidly obese subjects, improves insulin sensitivity (1,2) and reverses (3) or prevents diabetes (4). This is the first report of RYGB in a patient with FPL that resulted in unanticipated dramatic improvement in the metabolic control of her lipodystrophy.