This review is based on longitudinal studies on our seven patients with
congenital generalized lipodystrophy, our patient with acquired
generalized lipodystrophy, and published papers on these subjects. An inability to store energy in adipose tissue is of pathogenetic importance. In congenital
lipodystrophy,
insulin resistance is present from birth, resulting in hyperinsulinaemia, dyslipidaemia. and
insulin-resistant diabetes with an anabolic syndrome worsened by a voracious appetite. Clinically, we observed increased height velocity in pre-school age children, and organomegaly with
hypertrophic cardiomyopathy, which seems to be lethal in early adulthood: three of our patients died at the ages of 24, 32 and 37 years. The oldest alive, 39 years, suffers from
stenocardia. Regarding treatment, it is most important to reduce energy consumption. The congenital form is recessively inherited. The aetiology may be related to
insulin receptor or postreceptor mechanisms. Acquired
generalized lipodystrophy seems to be an autoimmune disorder with secondary destruction of the adipose organ: the anabolic syndrome with
insulin-resistant diabetes is secondary. Our patient died when 24 years old from
pneumonia.