09-01-2021-1907 - Lipodystrophy

Lipodystrophy syndromes are a group of genetic or acquired disorders in which the body is unable to produce and maintain healthy fat tissue.^[1][2] The medical condition is characterized by abnormal or degenerative conditions of the body's adipose tissue. ("Lipo" is Greek for "fat", and "dystrophy" is Greek for "abnormal or degenerative condition".) A more specific term, lipoatrophy, is used when describing the loss of fat from one area (usually the face). This condition is also characterized by a lack of circulating leptin which may lead to osteosclerosis. The absence of fat tissue is associated with insulin resistance, hypertriglyceridemia, non-alcoholic fatty liver disease (NAFLD) and metabolic syndrome.^[3][4] 

Types[edit]

Lipodystrophy can be divided into the following types:^[5]:495–7

• Congenital lipodystrophy syndromes
  • Congenital generalized lipodystrophy (Beradinelli-Seip syndrome)
  • Familial partial lipodystrophy
  • Marfanoid–progeroid–lipodystrophy syndrome
  • Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature syndrome^[6]
• Acquired lipodystrophy syndromes
  • Acquired partial lipodystrophy (Barraquer-Simons syndrome)
  • Acquired generalized lipodystrophy
  • Centrifugal abdominal lipodystrophy (Lipodystrophia centrifugalis abdominalis infantilis)
  • Lipoatrophia annularis (Ferreira-Marques lipoatrophia)
  • Localized lipodystrophy
  • HIV-associated lipodystrophy

Epidemiology[edit]

Congenital lipodystrophy (due to inherited genetic defect) is estimated to be extremely rare, possibly affecting only one per million persons.^[7] Acquired lipodystophy is much more common, especially affecting persons with HIV infection.^[7]

Pathogenesis[edit]

Due to an insufficient capacity of subcutaneous adipose tissue to store fat, fat is deposited in non-adipose tissue (lipotoxicity), leading to insulin resistance.^[7] Patients display hypertriglyceridemia, severe fatty liver disease and little or no adipose tissue.^[8] Average patient lifespan is approximately 30 years before death, with liver failure being the usual cause of death.^[8] In contrast to the high levels seen in non-alcoholic fatty liver disease associated with obesity, leptin levels are very low in lipodystropy.^[7]

Antiretroviral drugs[edit]

Main article: HIV-associated lipodystrophy

Lipodystrophy can be a possible side effect of antiretroviral drugs. Other lipodystrophies manifest as lipid redistribution, with excess, or lack of, fat in various regions of the body. These include, but are not limited to, having sunken cheeks and/or "humps" on the back or back of the neck (also referred to as buffalo hump)^[9] which also exhibits due to excess cortisol. Lipoatrophy is most commonly seen in patients treated with thymidine analogue nucleoside reverse transcriptase inhibitors ^[10] like zidovudine (AZT) and stavudine (d4T).^[11]

https://en.wikipedia.org/wiki/Lipodystrophy

v t e Disorders of subcutaneous fat
Panniculitis	Lobular without vasculitis  Cold Cytophagic histiocytic Factitial Gouty Pancreatic Traumatic needle-shaped clefts  Subcutaneous fat necrosis of the newborn Sclerema neonatorum Post-steroid panniculitis Lipodermatosclerosis Weber–Christian disease Lupus erythematosus panniculitis Sclerosing lipogranuloma with vasculitis: Nodular vasculitis/Erythema induratum Septal without vasculitis: Alpha-1 antitrypsin deficiency panniculitis Erythema nodosum  Acute Chronic with vasculitis: Superficial thrombophlebitis
Lipodystrophy	Acquired generalized: Acquired generalized lipodystrophy partial: Acquired partial lipodystrophy Centrifugal abdominal lipodystrophy HIV-associated lipodystrophy Lipoatrophia annularis localized: Localized lipodystrophy Congenital Congenital generalized lipodystrophy Familial partial lipodystrophy Marfanoid–progeroid–lipodystrophy syndrome Poland syndrome

Categories: 

• Conditions of the subcutaneous fat