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Lamellar ichthyosis is a rare congenital skin condition characterized by thick, scaly skin. Babies born with it are referred to as “collodion babies” and are at risk of complications. There is no cure, but symptoms can be relieved with moisturizers and eye drops.
Lamellar ichthyosis, also called ichthyosis lammellaris or nonbullous congenital ichthyosis, is a congenital skin condition characterized by hyperkeratosis, or thickening of the stratum corneum, the outermost layer of skin. It results in a scaly appearance to the skin, particularly on the neck, groin, armpits, inner elbow, and similar joints. Lamellar ichthyosis is very rare, affecting only one in 600,000 people. It is a moderate form of ichthyosis, a group of genetic disorders ranging from the very mild and relatively common ichthyosis vulgaris to life-threatening harlequin-type ichthyosis.
Babies born with lamellar ichthyosis are referred to as “collodion babies” because they are born with a collodion membrane, which looks like an extra layer of skin, and sheds about two weeks after birth. The skin is shiny, waxy, and tight, and the eyes and mouth may pop wide. Because of their abnormal skin, babies on collodion are at risk of hypothermia, dehydration, skin infection, and intoxication from topically applied products. Nursing care in a NICU until the collodion membrane is removed, which often includes the use of topical moisturizers and a humidified incubator, helps address and prevent these risks. The condition is not painful or uncomfortable for the child, although complications may be.
Not all collodion babies have lamellar ichthyosis, and about ten percent experience no further symptoms after the collodion membrane falls off. About 15% have a different genetic skin disorder. Lab examination of your baby’s skin, blood, or hair can help doctors make a diagnosis.
In addition to scaly and thickened skin, lamellar ichthyosis in children and adults can cause overheating due to faulty sweating, dry and irritated eyelids, and temporary hair loss due to flaky scalp. Children with this condition sometimes develop tight bands around their fingers or toes that restrict circulation. There is no cure for lamellar ichthyosis, but it can be relieved with skin moisturizers and eye drops for dry eyes. Additionally, patients should avoid heat and strenuous exercise to prevent overheating.
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