Sjogren-Larsson Syndrome

Abstract

We present a case report of two brothers suffering from Sjogren-Larsson syndrome, who were bornto consanguineous parents. Sjogren-Larsson syndrome is one of the congenital icthyoses with an autosomal recessiveinheritance . It is characterized by the combination of 1 congenital ichthyosis with spastic diplegia, moderate mentalretardation and retinopathy.2,3,16. Defects in essential fatty acid metabolism have been attributed to as the cause4,8,13,15.