Gedde-Dahl et al. (1984) described a family in which 2 sisters and a brother with presumably nonconsanguineous parents had ichthyosis of intermediate severity closely resembling the nonscaly hyperkeratosis of the Sjogren-Larsson syndrome (SLS; 270200). Di- or quadriplegia and ... Gedde-Dahl et al. (1984) described a family in which 2 sisters and a brother with presumably nonconsanguineous parents had ichthyosis of intermediate severity closely resembling the nonscaly hyperkeratosis of the Sjogren-Larsson syndrome (SLS; 270200). Di- or quadriplegia and mental retardation, obligatory signs of SLS (Jagell et al., 1981), were, however, lacking. This may represent a genetic compound of the SLS gene and another recessive ichthyosis gene. The patients were born in the county of Nordland, Norway, and their parents' ancestors came from 2 neighboring areas in northern Norway adjacent to the area of Sweden (Vasterbotten) where the frequency of SLS heterozygotes is about 2%. SLS has not been found in Norway. The paternal ancestors of the family lived in an area where Swedish Lapps from Vasterbotten went with their reindeer in summertime in former centuries and where some settled.