Lipodystrophies

Excerpt

Lipodystrophies are the category of conditions that share the common finding of a reduction in subcutaneous fat. There are multiple subtypes of lipodystrophy, which may be either congenital or acquired and vary in the distribution of fat loss. Although all are relatively rare in incidence, acquired variants have become more common as an adverse effect of certain medications and iatrogenic mechanisms. Regardless of subtype, decreases in overall adipose burden may lead to metabolic complications, with subsequent increases in morbidity and mortality in lipodystrophy patients.

Congenital Generalized Lipodystrophy

Congenital generalized lipodystrophy, sometimes referred to as Berardinelli-Seip syndrome, is an uncommon lipodystrophy variant with significant and sometimes near-total fat loss.

Familial Partial Lipodystrophy

Familial partial lipodystrophy is most often an autosomal dominant condition with fat loss primarily involving the extremities, more commonly lower than upper.

Acquired Generalized Lipodystrophy

Acquired generalized lipodystrophy is an extremely rare condition of widespread subcutaneous fat loss.

Acquired Partial Lipodystrophy

Acquired partial lipodystrophy, or Barraquer-Simons syndrome, is characterized by gradual loss of fat from the upper body and truncal region during childhood. Over the past few decades, however, highly active anti-retroviral therapy induced lipodystrophy has become the most common form of acquired partial lipodystrophy.