ELOVL4 and erythrokeratodermia variabilis: In the last decade, a number of different heterozygous mutations in different exons of the ELOVL4 gene (Fig. 1) have been reported to cause age-related autosomal dominant spinocerebellar ataxia-34 (SCA34) with or without the skin condition erythrokeratodermia variabilis (35, 36, 37, 38, 39, 43).