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Lamellar ichthyosis
Definition
Lamellar ichthyosis is a rare skin condition that appears at birth and continues throughout a person's life.
Causes
Lamellar ichthyosis is passed down through families (inherited). Both parents must have at least one abnormal gene to pass it to their children.
Symptoms
A baby is born with a collodian membrane -- a shiny, waxy layer of skin that sheds within the first 2 weeks of life. Red, scaly skin remains underneath. It resembles the surface of a fish.
Treatment
Moisturizers containing urea, ammonium lactate, or other alpha-hydroxy acids may help. Also, retinoid medications such as tazarotene may be used on the skin (topically).
Gene therapy to correct the genetic defect may be possible in the future.
Possible Complications
When the collodian membrane is shed, babies are at risk for infection.
Later in life, eye problems may occur because the eyes cannot close completely.
References
Morelli JG. Disorders of keratinization. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 657.
Reviewed By: Linda J. Vorvick, MD, Medical Director, MEDEX Northwest Division of Physician Assistant Studies, University of Washington School of Medicine. Also reviewed by Kevin Berman, MD, PhD, Atlanta Center for Dermatologic Disease, Atlanta, GA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
