Progressive Lipodystrophy: A Clinical Study of 50 Patients

Progressive lipodystrophy is rare, but the clinical features of this entity have aroused the interest of clinicians for more than half a century. The term "progressive lipodystrophy" was coined by Simons 1 in 1911 and, according to his description, was characterized by a painless, progressive loss or wasting of subcutaneous fatty tissue, beginning in the face and later involving neck, shoulders, arms, and trunk. This sequence of progression, later confirmed by other authors, 2,3 is usually symmetric in distribution. The onset of the lipodystrophic process is usually during childhood and occasionally follows an acute febrile illness. 4,5 In the female after puberty, abundant adipose tissue often develops about the hips and thighs that is grotesquely incongruous when contrasted with the emaciated, haggard face and upper part of the body. 1,4,6 The clinical features of this process had been described by Shaw, 7 Barraquer, 8 and Pic and Gardere 9 prior