A patient with an unusual form of lipodystrophy is described. The sharp demarcation of the fatty and fat free areas at the L-2 L-3 dermatome junction was the striking feature of this case. This observation led to speculation that the pathogenesis of lipodystrophy might be related to a neurologic or neurohumoral abnormality. Plasma catecholamines and urinary catecholamine excretion rates were normal. Sweat analysis revealed greater sweat production in the fat-free areas. Studies of carbohydrate and lipid metabolism revealed similarities to the related disease, lipoatrophic diabetes. A careful review of the literature supported the concepts that in lipodystrophy the line of demarcation between fatty and fat-free areas always conforms to derma-tome junctions and that lipodystrophy should be considered an incomplete variant of lipoatrophic diabetes.

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