Other names: n-CIE; non-bullous congenital ichthyosiform erythroderma
OMIM: 242300
Inheritance: autosomal recessive
Incidence: 1:200,000
Key findings:
Associated findings: apparently none in those with a transglutaminase mutation
Age at first appearance: birth, usually as collodion baby
Long-term course: lifelong; little fluctuation in severity of scale or redness; increased susceptibility to bacterial and fungal infections of skin; heat intolerance may be a problem for some
Diagnostic tests: analysis of cellular DNA
Abnormal gene: transglutaminase 1 in 50-75% of individuals; unknown in others
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