Abstract

Lamellar ichthyosis of the newborn is a distinct genetic entity, probably autosomal recessive in inheritance. Babies born with this condition are covered with a collodion-like membrane, but the skin clears almost completely in the first months of life and they remain free of ichthyosis. Two brothers had lamellar ichthyosis of the newborn, and a similar child subsequently developed cataracts and other abnormalities. None of these three patients had stippled epiphyses. A fourth patient, a child with classical Conradi's disease (stippled epiphyses and cataracts) had erythroderma at birth. This cleared completely in a few months, but other skin and hair abnormalities have persisted.

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