Congenital generalized lipodystrophy

Congenital generalized lipodystrophy (also known as Berardinelli–Seip syndrome) is a rare autosomal dominant skin condition, characterized by an extreme paucity of fat in the subcutaneous tissues.

Presentation
Berardinelli-Seip congenital lipodystrophy is a rare autosomal recessive condition associating insulin resistance, absence of subcutaneous fat and muscular hypertrophy. With characteristic features like hepatomegaly, accelerated growth, muscle hypertrophy, lack of adipose tissue, hirsutism and hypertriglyceridemia. Common cardiovascular alterations related to this syndrome are cardiac hypertrophy and arterial hypertension. and it is associated with metabolic syndrome.