Sjogren-Larsson Syndrome is a rare autosomal disorder which occurs with 100% penetrance and is classically characterized by ichthyosis, spasticity and mental handicap. This has rarely been described in people of Indian origin, case reports mainly being restricted to Caucasians. We have described’a 6-year-old girl with classical features of this syndrome, the symptoms having started in infancy. The spasticity is mainly diplegic in nature. Skin changes of ichthyosis are generalized and more in the flexural areas. Mental retardation is severe. Management is supportive.
Key wordsIchthyosis Spasticity Mental deficiency
- 1.Sjogren T, Larsson T. Oligophrenia in combination with congenital ichthyosis and spastic disorders. A clinical and generic study.Acta Psychiatr Neurol Scand 1957; 32 (suppl): 1–113.Google Scholar
- 5.Pardo-Castello V, Faz H, Ichthyosis — Little’s disease.Arch Derm Syph (chic) 1932; 26: 915–916.Google Scholar
- 6.Pisani D, Cacchione A, Dermatosi FE. Frenastenia edermatosi.Riv Sper Frehiat 1934; 58: 722–738.Google Scholar
- 14.Fiverson DP, Lucky AW, Lannocon S. Sjogren Larsson syndrome associated with Dandy Walker malformation: report of a case.Pediatr Dermotol 1989; 6 : 312–315.Google Scholar
- 16.Jagell S, Liden S. Treatment of the ichthyosis of the Sjogren-Larsson syndrome with etretinate (Tigason).Acta Dermatovener 1983; 63: 89–91.Google Scholar