Definition: a dry form of congenital ichthyosiform erythroderma, characterized by ectropion and large, coarse scales over most of the body with thickened palms and soles; may be fatal with complications of sepsis, protein, and electrolyte loss in the first year of life; histology shows hyperkeratosis, a prominent granular layer in the epidermis, slight acanthosis, many mitotic figures, and normal or reduced epidermal cell turnover. Autosomal recessive inheritance, caused by mutation in the gene encoding keratinocyte transglutaminase (TGM1) on chromosome 14q.
Synonym(s): ichthyosis congenita
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