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May 1970


Arch Dermatol. 1970;101(5):620. doi:10.1001/archderm.1970.04000050124025

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To the Editor.—  I should like to present a classification (Table) of the ichthyosiform dermatoses based on genetic studies and the clinical course of the disease. Refsum's disease (autosomal recessive) has been excluded since it has been found to be a metabolic disorder and the ichthyosis is not a major component. In both lamellar ichthyosis and Conradi's syndrome, the ichthyosiform erythroderma clears after birth. Lamellar ichthyosis is a separate type, but has been confused in the recent literature. The nonbullous CIE disorder does not clear during the patient's lifetime, but may improve with age. Ichthyosis may be noted in conjunction with mongolism (Down's syndrome), and several patients were reported as having Rud's syndrome in cases where either this or the sexlinked ichthyosis in conjunction with mental deficiency and hypogonadism were noted. This syndrome should also be excluded.References will be sent on request.

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