congenital generalized lipodystrophies (cgls) are very rare autosomal recessive disorders which have four types.of the four cgl types, bscl2 (berardinelli–seip congenital lipodystrophy type 2) is the result of mutations in the bscl2/seipingene.bscl2 which is the most severe lipodystrophic phenotype is characterized by generalized lipodystrophy, overgrowth,acanthosisnigricans, hepatomegaly, insu...

sent case, the pattern of mosaicism is difficult to classify because mosaicism of adipocytes, which are mesodermal not ectodermal tissue, is currently not well understood. It is very likely, however, that this type of segmental lipodystrophy reflects mosaicism. Investigating genes known to cause inherited, systemic lipodystrophies (eg, AGTAT2, seipin, LMNA, PPAR-g, and ZMPSTE24) could aid in pr...