SIMULTANEOUS BILATERAL FACIAL RECONSTRUCTION OF A BARRAQUER-SIMONS LIPODYSTROPHY WITH FREE TRAM FLAPS

Lipodystrophies are rare conditions in which the most remarkable feature is an atrophy of the subcutaneous fatty tissue. They have been divided into partial and total forms, depending on the distribution of the fat atrophy and several other features. Barraquer and Simons1 described in the beginning of this century a disease characterized by a progressive atrophy of the subcutaneous fat limited to the upper part of the body, including the face. The etiology is unknown. Females are preferentially affected, and familial occurrence is controversial. The onset is usually during childhood, occasionally following a febrile or infective illness.2 Patients lose a variable amount of fat symmetrically up to a certain degree and then the process arrests but with persistence of the acquired deformities.2,3 The face is always involved, rendering the normal facial skeleton more prominent. The trunk and arms may be spared, partially affected, or totally devoid of subcutaneous fat. The legs tend, paradoxically, to be fatter than normal. Histologic examinations show normal skin and muscles with atypical fat atrophy. The most frequent associated feature is a progressive renal disease characterized by a hypocomple- mentemic glomerulonephritis. The relationship between hypocomplementemia and partial lipodystrophy remains to be clarified, and the cause of the curious distribution of the lipodystrophic changes is unexplained. © 1995 American Society of Plastic Surgeons.