non-bullous congenital ichthyosiform erythroderma

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non-bullous congenital ichthyosiform erythroderma

A non-bullous ichthyosis (OMIM:242100)—a skin disorder characterised by abnormal cornification of the epidermis—that is milder than lamellar ichthyosis and has a greater variability in intensity of erythema, size and type of scales. Here, the scales are usually white, fine and powdery, and accompanied by palmoplantar keratoderma, often with painful fissures, digital contractures and loss of pulp volume.

Molecular pathology
Defects of TGM1, which encodes a transglutaminase, are a cause of nonbullous congenital ichthyosiform erythroderma.
Segen's Medical Dictionary. © 2012 Farlex, Inc. All rights reserved.
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