SJOGREN-LARSSON SYNDROME

Abstract

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