Congenital Generalized Lipodystrophy, Coincident with Portal Hypertension: A Case Report and Review of Literature

Abstract

Congenital generalized lipodystrophy (CGL) is a rare and progressive disorder (1 in 12 milion). Common clinical manifestations are lipoatrophy, acromegaloid feature, acanthosis nigricans, Hyperlipidemia, diabetes mellitus or abnormal glucose tolerance test. Rarely fatty liver and cirrhosis is seen. The highest number reported in literature is 8 cases from Brazil and 6 cases from Scandinavia, respectively. The oldest case was a 19-year-old girl. Our case is a 13-year-old male with all above manifestations, also he had liver cirrhosis. Another case of CGL had been reported by first author of this article, 13 years ago in Iranian Medical Journal.