Lamellar Ichthyosis

Lamellar Ichthyosis (or classical Lamellar Ichthyosis) is one of the more commonly seen types of ichthyosis. It is one of the most severe forms, and it occurs in approximately 1 in 300,000 births. Recessive genes cause lamellar ichthyosis, similar to blue eyes. Only when a person receives two recessive genes for lamellar ichthyosis will he or she actually have the disorder.

In lamellar ichthyosis, the skin cells are produced at a normal rate, but they do not separate normally at the surface of the stratum corneum (the outermost layer of the skin) and are not shed as quickly as they should be. The result is a build-up of scales. The entire body is covered with broad, dark, plate-like scales separated by deep cracks. People with lamellar ichthyosis may experience a condition called ectropion (a turning out of the eyelids to expose the red inner lid). People with lamellar ichthyosis may also have thickened nails and hair loss due to the thickness of the scales on their scalp. They may also have reddened skin (erythroderma), thickened skin on the palms of the hands and soles of the feet, and decreased sweating with heat intolerance.

Lamellar ichthyosis is present at birth. Many babies born with lamellar ichthyosis are also “collodion babies” because a clear membrane (the collodion) may cover their bodies. The collodion is then shed within a few days to a few weeks. Sometimes described as having a shellacked appearance, these newborns have skin which is taut, dark and split. Often the eyelids and lips are forced open by the tightness of the skin, and there may be contractures around the fingers. Problems with temperature regulation, water loss, secondary infections, and systemic infection can occur in the newborn with lamellar ichthyosis.

Lamellar ichthyosis is typically treated topically with moisturizers and keratolytics. Creams with high concentrations of alpha-hydroxy acids are commonly used. Lamellar ichthyosis may be treated systemically with oral synthetic reinoids (Accutane or Soriatane). Retinoids are used only in severe cases due to their know bone toxicity and other complications.