The renal lesion associated with total lipodystrophy.

This report concerns a 41-year-old man with total lipodystrophy associated with a specific renal disorder. He had massive proteinuria, mild azotemia and a normal level of serum complement in addition to the generalized loss of subcutaneous fatty tissue. Results of 1 50-gm glucose tolerance test indicated a high fasting insulin level with an exaggerated response to glucose. Hypoglycemic responses to exogenous insulin were reduced. Renal biopsy revealed changes consistent with glomerular lipidosis. This case is unique in that the renal lesion was not the Kimmelstiel-Wilson pathologic change atributable to abnormal glucose metabolism.