Skip to Content

lamellar ichthyosis

 

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

See Also: collodion baby, harlequin fetus

Disclaimer: This site is designed to offer information for general educational purposes only. The health information furnished on this site and the interactive responses are not intended to be professional advice and are not intended to replace personal consultation with a qualified physician, pharmacist, or other healthcare professional. You must always seek the advice of a professional for questions related to a disease, disease symptoms, and appropriate therapeutic treatments.
© Copyright 2017 Wolters Kluwer. All Rights Reserved. Review Date: Sep 19, 2016.
Hide