Abstract
LAWRENCE 1 suggested the term lipoatrophic to designate the syndrome which he earlier 2 had characterized as having the following features: (1) lack of adipose tissue, (2) accentuated muscularity, (3) hepatomegaly with cirrhosis, (4) diabetes which is resistant to insulin and without ketosis, and (5) elevated metabolic rate without hyperthyroidism. The first recorded example of this syndrome was a patient reported by Ziegler 3 in 1928 as a case of lipodystrophy in a 27-year-old woman in whom the subcutaneous tissue had disappeared early in the second decade of life and who had muscles of masculine appearance, hepatomegaly, a high metabolic rate, and diabetes that appeared early in the third decade of life. In 1944, McQuarrie 4 described a 4-year-old boy with similar features; in 1946, Lawrence and Aberd 2 described one additional case; and at least 14 more cases have been reported. 5-15 In all but two of
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