Acquired generalized lipodystrophies (AGL) typically manifest in childhood, are associated with a generalized loss of subcutaneous fat  and often lack any distinguishable family history. Congenital generalized lipodystrophy (CGL) or Berardinelli-Seip syndrome typically begins in infancy and is associated with near complete loss of body fat in addition to metabolic abnormalitiesdisorders such as insulin resistance, hepatic steatosis, and cardiomyopathy. A category of familial partial lipodystrophy is also described which is characterized by subcutaneous fat loss in limbs, hips and buttocks and a regional distribution of excess fat that may give a cushingoid like appearance. Deficiency of leptin, a peptide hormone principally made in adipose cells and enterocytes in the small intestine, has been implicated as having a role in the pathogenesis of many of the associated metabolic abnormalities. Metabolic complications seem to be related to a reduced concentration of adipocytokines resulting from the total body fat reduction. Leptin is an adipocytokine which seems to play an important role in BSCL and whose low levels are strongly correlated with changes in lipid and glucose metabolism. Lipid alterations are characterized by hypertriglyceridemia with deposition of triglycerides in the liver and lymphoreticular tissues. Due to the limited capacity for the storage of glucose in the form of fat, glucose is then stored asin the form of glycogen in hepatic, skeletal and cardiac muscle. The disturbance in glucose homeostasis leads to the appearance of insulin resistance and development of diabetes often beginning in childhood.