(CIE, Non-bullous CIE)
Many experts subdivide lamellar ichthyosis into two categories: Classic lamellar and congenital ichthyosiform erythroderma (CIE). As such, CIE is considered one of the main five types of ichthyosis (the others being lamellar ichthyosis, ichthyosis vulgaris, X-linked ichthyosis, and epidermolytic ichthyosis). Like lamellar ichthyosis, CIE is one of the rare forms, occurring in approximately 1 in 300,000 births. However, several differences separate CIE from lamellar. In CIE, there is an overproduction of skin cells in the epidermis. These cells reach the stratum corneum (the outermost layer of skin) in as few as four days, compared to the normal fourteen. The cells are made faster than they are shed and therefore build up in the stratum corneum and underlying layers. The severity and type of scaling varies with CIE. With CIE the scales are usually fine and white on the face, scalp, and torso, but scales on the legs can be large and plate-like (more like the scales of lamellar ichthyosis). Additionally, the skin is often quite reddish beneath the scales. People with CIE also may have thickened nails, or thickened skin on the palms of the hands and soles of the feet. People with CIE have an increased susceptibility to skin infection, and heat intolerance is common.
CIE is present at birth. Many babies with CIE were born as “collodion babies,” so called because a clear membrane (the collodion) covers 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 that is taut, dark and split. After the membrane is shed, dry red skin is revealed. Often the eyelids and lips are already forced open by the tightness of the skin, and there may be contractures around the fingers.
Many genes are now known to cause CIE, and mutations causing CIE are transmitted via autosomal recessive inheritance. Individuals must inherit two recessive genes in order to show the disease, one from each parent, but the parents (“carrier”) show no signs of CIE. (For more information on the genetics of CIE refer to the Foundation’s publication, Ichthyosis: The Genetics of Inheritance.)
CIE is treated topically with skin barrier repair formulas containing ceramides or cholesterol, moisturizers with petrolatum or lanolin, and mild keratolytics (products containing alpha-hydroxy acids). (For more information on which products contain these ingredients, ask for FIRST’s Skin Care Products List.)
Additionally, CIE can be treated systemically with oral synthetic retinoids (for example, Accutane® or Soriatane®). Retinoids are only used in severe cases of ichthyosis due to their know bone toxicity and other complications.
CIE is present at birth. Many babies with CIE were born as “collodion babies,” so called because a clear membrane (the collodion) covers 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 that is taut, dark and split. After the membrane is shed, dry red skin is revealed. Often the eyelids and lips are already forced open by the tightness of the skin, and there may be contractures around the fingers.
Many genes are now known to cause CIE, and mutations causing CIE are transmitted via autosomal recessive inheritance. Individuals must inherit two recessive genes in order to show the disease, one from each parent, but the parents (“carrier”) show no signs of CIE. (For more information on the genetics of CIE refer to the Foundation’s publication, Ichthyosis: The Genetics of Inheritance.)
CIE is treated topically with skin barrier repair formulas containing ceramides or cholesterol, moisturizers with petrolatum or lanolin, and mild keratolytics (products containing alpha-hydroxy acids). (For more information on which products contain these ingredients, ask for FIRST’s Skin Care Products List.)
Additionally, CIE can be treated systemically with oral synthetic retinoids (for example, Accutane® or Soriatane®). Retinoids are only used in severe cases of ichthyosis due to their know bone toxicity and other complications.
download CIE Fact Sheet
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