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Chapter: Clinical Dermatology: Diagnosis of skin disorders

Other ichthyosiform disorders

Sometimes ichthyotic skin changes are a minor part of a multisystem disease, but such associations are very rare.

Other ichthyosiform disorders

Sometimes ichthyotic skin changes are a minor part of a multisystem disease, but such associations are very rare. Refsum’s syndrome, an autosomal recessive trait, is caused by deficiency of a single enzyme con-cerned in the breakdown of phytanic acid, which then accumulates in the tissues. The other features (retinal degeneration, peripheral neuropathy and ataxia) over-shadow the minor dryness of the skin.

Rud’s syndrome is an ichthyosiform erythrodermain association with mental retardation and epilepsy. In Netherton’s syndrome, brittle hairs, with a so-called‘bamboo deformity’, are present as well as a curious gyrate and erythematous hyperkeratotic eruption (ichthyosis linearis circumflexa). Other conditions are identified by confusing acronyms: IBIDS (also known as trichothiodystrophy) stands for Ichthyosis, Brittle hair, Impaired intelligence, Decreased fertility and Short stature; the KID syndrome consists of Keratitis, Ichthyosis and Deafness.


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