Lipodystrophies

Classification (link)

Definition

Lipodystrophies are a group of diseases characterised by a morphological and/or functional impairment of the adipose tissue. From this group are excluded obesity and some fat-loss associated disorders as undernutrition, consumptive diseases (cancer, etc) or some endocrine diseases (Cushing syndrome, hyperthyroidism) .

In general, the term Lipodystrophy is associated with disorders in whom there is a lost of adipose tissue, albeit, in a strict sense, also it should be considered lipodystrophic syndromes those in whom lipohypertrophy appears.

The most prevalent type of Lipodystrophy is that associated to anti-retroviral high activity drugs for AIDS. The rest of lipodystrophic pictures are very unfrequent, thus they are considered Rare Diseases.

Lipodystrophies can be genetics or acquired, and, considering the extension of the lost of fat, can be classified in generalised, partial and local.

In some subtypes, the lost (or lack) of adipose tissue is the predominant phenotypical trait, and that is conditioning the complications. Such is the case of Berardinelli-Seip Syndrome or Congenital Generalised Lipodystrophy, Lawrence Syndrome or Acquired Generalised Lipodystrophy or Dunnigan disease (type 2 Familial Partial Lipodystrophy). In other occasions, the lost of adipose tissue is just other clinical sign of a more complex picture, as is the case of Hutchinson-Gilford Progeria Syndrome or CANDLE syndrome, as two examples.