Progressive lipodystrophy is a relatively rare disease characterized by complete absence of subcutaneous fat in certain areas of the body. The skin above it appears to be perfectly normal. Many of the articles concerning this condition have been in the foreign literature; however, the first report in 1885 was by an American, Weir Mitchell,1 whose description is excellent, and the photographs are remarkably good. While progressive lipodystrophy has been reported intermittently for 75 years, a variant, or different condition, known as lipoatrophic diabetes began to appear in the literature about 15 years ago. Although approximately 200 cases of progressive lipodystrophy have been reported, except for brief descriptions in Andrews' Diseases of the Skin,2 Pillsbury, Shelley, and Kligman's textbook of Dermatology,3 and the case of Paul Gross,4 no other references to it were found in the American dermatologic literature. Since this is not intended to be a