Medical Term:

congenital total lipodystrophy


Definition: characterized by almost complete lack of subcutaneous fat, accelerated rate of growth and skeletal development during the first 3–4 years of life, muscular hypertrophy, cardiac enlargement, hepatosplenomegaly, acanthosis nigricans, hypertrichosis, renal enlargement, hypertriglyceridemia, and hypermetabolism; autosomal recessive inheritance.

Synonym(s): Berardinelli syndrome, Seip syndrome