Acquired Partial Lipodystrophy Barraquer Simons Syndrome

Acquired partial lipodystrophy (APLD) was first described by Mitchell in 1885 [7] as a 'singular case of absence of adipose tissue in the upper part of the body,' and then as segmental atrophy of the subcutaneous fat layer by Barraquer [8] in 1907, and as 'lipodystrofia progressiva' by Simons in 1911 [9]. APLD is phenotypically characterised by a loss of fat in the upper body segment, namely, in the face, trunk, and arms. In the lower body segment, the subcutaneous fat depots are spared or even increased. Bichat's fat pad is also involved, giving the face an extremely lean appearance (Fig. 1). Women are affected three times more frequently than men. A mesangiocapillary glomerulonephri-tis [10] develops in some 30% of affected subjects within 8-15 years after the onset of the disease, leading to renal failure [10], associated with low levels of serum C3 complement fraction [11-13]. A slight increase of a circulating polyclonal IgG, a C3 nephritic factor, causes activation of the alternative complement pathway and increased consumption of the C3 fraction. C3 nephritic factor could induce fat cell lysis, and then a loss of subcutaneous fat [14]. Other autoimmune disorders, such as rheumatoid arthritis, temporal arteritis, dermatomyositis, thyroiditis, coeliac disease, and systemic lupus erythematosus, have been reported to be associated with APLD [15-18]. No information has been offered so far to explain the segmental involvement of subcutaneous fat and the female prevalence, and no effective therapy is currently available. Cosmetic surgical procedures

Fig. 1a, b. A patient with acquired partial lipodystrophy. Lipoatrophy exclusively involves the upper body segment and face (a), while a marked increase in subcutaneous fat depots is evident in the lower body segment (b)

may be suggested when psychological discomfort impairs the patient's quality of life.

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