Lipoatrophic Diabetes

Abstract

LAWRENCE¹suggested the term "lipoatrophic diabetes" to designate the syndrome which he earlier²had 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 Ziegler³in 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, McQuarrie⁴described a 4-year-old boy with similar features; in 1946, Lawrence and Aberd²described one additional case; and at least 14 more cases have been reported.^5-15In all but two of