LIPOATROPHIC DIABETES MELLITUS (GENERALIZED LIPODYSTROPHY): ROENTGEN FINDINGS IN TWO BROTHERS WITH CONGENITAL DISEASE

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Abstract
The syndrome of lipoatrophic diabetes is reviewed. The unique roentgenographic findings in 2 brothers with the congenital form of this disease are presented. Absent body fat, combined with well developed muscles, produced a characteristic appearance of the soft tissues. Other striking features were a generalized increase in skeletal density, localized areas of bone sclerosis, unusually elongated epiphyses, abnormalities of skull, and organomegaly. Advanced skeletal maturation was demonstrated in the younger brother. The possible pathophysiologic significance of some of these findings is discussed.