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Lamellar ichthyosis

Definition

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

Causes, incidence, and risk factors

Lamellar ichthyosis is passed down through families (inherited). 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 underneath is red as scaly skin remains underneath. It resembles the surface of a fish.

Signs and tests

Treatment

Moisturizers containing medicines such as urea, ammonium lactate, or other alpha-hydroxy acids may help shed dead skin layers. Also, retinoid medications such as tazarotene may be applied to the skin.

Support Groups

Expectations (prognosis)

Complications

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

Eye problems may occur later in life because the eyes cannot close completely.

Calling your health care provider

Prevention

References

Morelli JG. Disorders of keratinization. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. . 18th ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 657.

Richard G, Rigpfeil F.  Ichthyoses, Erythrokeratodermas and Related Disorders. In: Bolognia JL, Jorizzo JL, Schaffer JV, et al, eds.Dermatology. 3rd ed. Philadelphia, Pa: Mosby Elsevier; 2012:chap 57.

 

Encyclopedia content is provided as information only and not intended to replace the advice and instruction from your personal physician.