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  • Title: Lethal congenital erythroderma: a newly recognised genetic disorder.
    Author: Shield JP, Judge MR, Reardon W, Baraitser M, Nohria M, Malone M, Harper JI.
    Journal: Clin Genet; 1992 May; 41(5):273-7. PubMed ID: 1535034.
    Abstract:
    We report 4 patients and their extended families comprising 17 cases, all of whom had congenital exfoliative erythroderma resistant to treatment, associated with failure to thrive and hypoalbuminaemia. All died in the first year of life. This condition appears to be inherited in an autosomal recessive manner and the underlying defect remains unknown.
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