Introduction
Lipodystrophies 101
Suat Hoon Tan, Hong Liang Tey and Joel Hua Liang Lim
Subtypes: Lipodystrophy: lipoatrophy, lipohypertrophy
Key features
Lipodystrophies are inherited or acquired disorders characterized by a paucity or complete absence of fat that may be generalized, partial, or localized; lipohypertrophy may accompany the lipoatrophy
Though the terms lipodystrophy and lipoatrophy are often used interchangeably, lipoatrophy should be used specifically for selective loss of fat, with lipodystrophy implying a redistribution of fat, in part due to a hypertrophic compensation of the non-atrophic fat
Lipodystrophy syndromes represent a heterogeneous group of disorders that are characterized by lipoatrophy ± fat accumulation in characteristic body distribution patterns
Fat is a metabolically active organ, thus fat loss can be associated with metabolic derangements, which parallel the extent and duration of lipoatrophy
Lipodystrophy is often accompanied by the metabolic syndrome, which includes insulin resistance, diabetes mellitus, hyperinsulinemia, dyslipidemia, cardiovascular disease, and hepatic steatosis
In addition to the metabolic syndrome, systemic manifestations of generalized and partial lipodystrophy syndromes include hormonal abnormalities, organ dysfunction, anabolic features, glomerulo nephritis, and autoimmune disorders
A distinct syndrome of peripheral lipoatrophy, central obesity, breast hypertrophy, dorsocervical fat pad enlargement, hyperlipidemia and insulin resistance occurs in patients with HIV infection undergoing treatment with antiretroviral therapy (ART)
Isolated or localized lipoatrophy can occur at the site of medication injection, trauma or pressure, in association with autoimmune connective tissue disease, or following certain panniculitic inflammatory or neoplastic processes
Microscopically, there may be a complete absence of subcutaneous fat or a decrease in adipocyte size and number. An inflammatory panniculitis may be seen in early disease