It is caused by mutation of gene encoding seipin (BSCL2).

Congenital disorders, usually autosomal recessive, characterized by severe generalized lack of ADIPOSE TISSUE, extreme INSULIN RESISTANCE, and HYPERTRIGLYCERIDEMIA.

It is caused by mutations of gene encoding 1-acylglycerol-3-phosphate O-acyltransferase-2 (AGPAT2).

Synonyms: Congenital Lipodystrophy, Berardinelli-Seip, Generalized Lipodystrophies, Brunzell Syndrome, AGPAT2-Related, Syndrome, Seip, Total Lipodystrophy, Berardinelli Seip Congenital Lipodystrophy Type 1, Berardinelli Seip Congenital Lipodystrophy Type 2, Lipodystrophies, Total, Diabete, Congenital Lipoatrophic, Berardinelli-Seip Congenital Lipodystrophy Type 2, Berardinelli Seip Syndrome, Berardinelli-Seip Congenital Lipodystrophy Type 1, Generalized Lipodystrophy, Congenital, Lipodystrophy, Berardinelli-Seip Congenital, Syndrome, AGPAT2-Related Brunzell, Brunzell Syndrome (with Bone Cysts), Lipodystrophy, Total, Total Lipodystrophy and Acromegaloid Gigantism, Brunzell Syndrome, BSCL2-Related, Seip Syndrome, Berardinelli-Seip Syndrome, Lipodystrophy, Generalized, Berardinelli-Seip Congenital Lipodystrophy, Type 2, Total Lipodystrophies, Berardinelli-Seip Congenital Lipodystrophy, Type 1, Berardinelli-Seip Congenital Lipodystrophy, Generalized Lipodystrophies, Congenital, Syndrome, Berardinelli, Lipodystrophy, Total, And Acromegaloid Gigantism, Lipoatrophic Diabete, Congenital, Syndrome, Brunzell (with Bone Cysts), Congenital Generalized Lipodystrophy Type 2, Congenital Generalized Lipodystrophy Type 1, Lipoatrophic Diabetes, Congenital, Lipodystrophy, Congenital Generalized, Type 1, Lipodystrophy, Berardinelli-Seip Congenital, Type 1, Berardinelli Seip Congenital Lipodystrophy, Lipodystrophy, Berardinelli-Seip Congenital, Type 2, Syndrome, BSCL2-Related Brunzell, Lipodystrophy, Congenital Generalized, BSCL2-Related Brunzell Syndrome, Lipodystrophy, Congenital Generalized, Type 2, Congenital Generalized Lipodystrophy, Generalized Lipodystrophy, Syndrome, Brunzell, Berardinelli Syndrome, Diabetes, Congenital Lipoatrophic, Brunzell Syndrome, Brunzell Syndrome, AGPAT2 Related, Lipodystrophies, Generalized, Brunzell Syndrome, BSCL2 Related, Congenital Lipoatrophic Diabetes, Lipodystrophies, Congenital Generalized, Syndrome, Berardinelli-Seip, Congenital Lipoatrophic Diabete, Berardinelli Seip Congenital Lipodystrophy, Type 1, Congenital Generalized Lipodystrophies, AGPAT2-Related Brunzell Syndrome, Berardinelli Seip Congenital Lipodystrophy, Type 2