Mutations in AGPAT2 (OMIM#603100) on chromosomes 9q34 [1] or BSCL2 (OMIM#606158) on chromosome 11q12.3 [10] account for 95 % of BSCL cases [11]. This evidence concerns the gene AGPAT2 and Berardinelli-Seip congenital lipodystrophy.