Ichthyosis-hepatosplenomegaly-cerebellar degeneration syndrome is characterized by ichthyosis, hepatosplenomegaly and late-onset cerebellar ataxia. It has been described in two brothers. Transmission is either autosomal recessive or X-linked. The disease is Monarch Disease Ontology id MONDO_0009445 (ichthyosis-hepatosplenomegaly-cerebellar degeneration syndrome). Also known as: Dykes-Markes-Harper syndrome, Dykes-Marks-Harper syndrome.