Congenital generalized lipodystrophy

[2] It is a type of lipodystrophy disorder where the magnitude of fat loss determines the severity of metabolic complications.

[4] Congenital generalized lipodystrophy (CGL) is a rare autosomal recessive disorder which manifests with insulin resistance, absence of subcutaneous fat and muscular hypertrophy.

[3] The condition appears in early childhood with accelerated linear growth, quick aging of bones, and a large appetite.

As the child grows up, acanthosis nigricans (hyperpigmentation and thickening of skin) will begin to present itself throughout the body – mainly in the neck, trunk, and groin.

The absence of adipose tissue where they normally occur causes the body to store fat in the remaining areas.

[7] Common cardiovascular problems related to this syndrome are cardiac hypertrophy and arterial hypertension (high blood pressure).

Thus, the mechanism is similar to Type 3, in that the caveolae are unable to properly form and carry out their role in lipid regulation in both.

[13] Medical diagnosis of CGL can be made after observing the physical symptoms of the disease: lipoatrophy (loss of fat tissues) affecting the trunk, limbs, and face; hepatomegaly; acromegaly; insulin resistance; and high serum levels of triglycerides.

[15] Metformin reduces appetite and improves symptoms of hepatic steatosis and polycystic ovary syndrome.

[4] Leptin can also be used to reverse insulin resistance and hepatic steatosis, to cause reduced food intake, and decrease blood glucose levels.

[16] CGL patients have to maintain a strict diet for life, as their excess appetite will cause them to overeat.