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Ichthyosis - hepatosplenomegaly - cerebellar degeneration

Synonym(s) Dykes-Markes-Harper syndrome
Prevalence <1 / 1 000 000
Inheritance Autosomal recessive
or X-linked recessive
Age of onset Infancy
  • Q87.8
  • C1275088
MeSH -
MedDRA -


This syndrome is characterised by ichthyosis, hepatosplenomegaly and late-onset cerebellar ataxia. It has been described in two brothers. Transmission is either autosomal recessive or X-linked.

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Practical genetics
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