Definition: a severe autosomal recessive form of collodion baby in a newborn, usually premature, infant; a form of ichthyosiform erythroderma characterized by encasement of the body in grayish brown, often fissured plaques resembling plates of armor and by grotesque deformity of the face with eclabium and gangrene of terminal phalanges; usually fatal within a few days, although treatment with 13-cis-retinoic acid has been successful in some cases.
Synonym(s): ichthyosis fetalis1
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