lipodystrofier
Lipodystrofier, or lipodystrophies, are a group of rare disorders characterized by abnormal distribution or loss of adipose tissue. They may be inherited (genetic) or acquired. Classification is often by the pattern and extent of fat loss or gain: generalized lipodystrophy (near-total loss of body fat), and partial lipodystrophy (loss in specific areas such as limbs, face, or trunk, sometimes with fat accumulation in the neck or abdominal region). Genetic forms include congenital generalized lipodystrophy and familial partial lipodystrophies; acquired forms include HIV-associated lipodystrophy and lipodystrophy linked to autoimmune disease, inflammation, or certain drugs.
Pathophysiology typically involves depletion or dysfunction of subcutaneous fat, leading to ectopic fat deposition in liver
Diagnosis combines clinical assessment with family history and, when available, genetic testing. Laboratory evaluation focuses on
Management is largely supportive and aimed at metabolic control. There is no cure for most forms. In