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Lamellar ichthyosis

Definition

Lamellar ichthyosis is a rare skin condition. It appears at birth and continues throughout life.

Causes

Lamellar ichthyosis is passed down through families. Both parents must have at least one abnormal gene to pass the condition to their children.

Symptoms

A baby with this condition is born with a shiny, waxy layer of skin called a collodian membrane. The membrane sheds within the first 2 weeks of life. The skin underneath the membrane is red and scaly. It resembles the surface of a fish.

Treatment

Moisturizers containing medicines such as urea or other substances may help shed dead skin layers. Also, medicines that contain retinoids may be applied to the skin.

Possible Complications

Babies are at risk for infection when they shed the collodian membrane.

Eye problems may occur later in life 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. 19th ed. Philadelphia, PA: Elsevier Saunders; 2011:chap 650.

Richard G, Rigpfeil F. Ichthyoses, erythrokeratodermas and related disorders. In: Bolognia JL, Jorizzo JL, Schaffer JV, et al, eds. Dermatology. 3rd ed. Philadelphia, PA: Elsevier Mosby; 2012:chap 57.


Review Date: 11/12/2014
Reviewed By: Richard J. Moskowitz, MD, dermatologist in private practice, Mineola, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
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