A Case of Sjogren-Larsson Syndrome.

Shin, Won Kyu; Ohn, Young Hoon; Park, Song Hee; Shin, Han Ho

Case Report

Journal of the Korean Ophthalmological Society 1995;36(9):1605-1609.
Published online September 1, 1995.

A Case of Sjogren-Larsson Syndrome.

Won Kyu Shin, Young Hoon Ohn, Song Hee Park, Han Ho Shin

Department of Ophthalmology, School of Medicine, Soonchunhyang University, Seoul, Korea.

Sjogren - Larsson 증후군 1 예

박성희(Song Hee Park),신환호(Han Ho Shin),신원규(Won Kyu Shin),온영훈(Young Hoon Ohn)

Abstract

The Sjogren-Larsson syndrome is genetically determined syndrome with autosomal recessive inheritence and characterized by the three cardinal signs: congenital ichthyosis, spastic di/tetraplegia, and mental retardation. Ocular signs include ectropion, blepharitis, conjunctivitis, keratitis, and macular glistening spot. The authors have experienced a case of Sjogren-Larsson syndrome that showed classical triad and macular glistening spot in a 16 month old boy.

Key Words: Sjogren-Larsson syndrome