Renal Disease in Partial Lipodystrophy

Five patients, two male and three female, with partial lipodystrophy and renal disease are presented. The lipodystrophy followed a childhood infective illness in at least two patients. Its extent was variable but facial involvement was constant. Other features found apart from renal disease were hirsuties, pigmentation, hepatosplenomegaly, and diabetes mellitus. Hypertension was a constant feature. There was a strong suggestion that renal disease in the three female patients had either started in pregnancy or had been aggravated by it. Renal function varied from normal to chronic renal failure, and one patient required renal transplantation. Proteinuria was a constant finding and the nephrotic syndrome occurred in three patients. Renal histology was obtained in all the patients and showed a glomerulonephritis of variable morphology. Electron microscopy was performed on two of the samples and confirmed the presence of glomerulonephritis and showed an unevenly thickened capillary basement membrane containing electron-dense deposits. It is suggested that the nephropathy in partial lipodystrophy is secondary to some as yet unspecified immunological lesion.