Lamellar ichthyosis in Infant/Neonate
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Synopsis
Lamellar ichthyosis is an inherited disorder, most often due to an error in the keratinocyte transglutaminase gene I, resulting in a defect in cell envelope formation and cornification. The inheritance is almost always autosomal recessive. The hallmark of the disease is a collodion membrane at birth, followed by thick plate-like scales on most of the skin surface. Collodion babies tend to be premature, having all the associated risks of prematurity.
Codes
ICD10CM:
Q80.2 – Lamellar ichthyosis
SNOMEDCT:
205550003 – Lamellar ichthyosis
Q80.2 – Lamellar ichthyosis
SNOMEDCT:
205550003 – Lamellar ichthyosis
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Last Updated:01/18/2022
Lamellar ichthyosis in Infant/Neonate