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Congenital Ichthosiform Erythroderma (CIE)

 

CIE (Nonbullous Congenital Ichthyosiform Erythroderma) is considered one of the more commonly seen types of ichthyosis. Like lamellar ichthyosis,

CIE is rare, occurring in 1 in 300,000 births. Recessive genes cause it. 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. New skin cells are made faster than old cells are shed and build up in the stratum corneum and underlying layers. The severity and scaling of CIE varies. The scales on the face, scalp and torso are usually fine and white, but the scales on the legs can be large and plate-like (like the scales of lamellar ichthyosis). The skin is often quite reddish beneath the scales.

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.

CIE is treated topically with moisturizers and keratolytics. Creams with high concentrations of alphahydroxy acids are commonly used. CIE can be treated systemically with oral synthetic retinoids (Accutane, Soriatane). Retinoids are only used in severe cases due to their known bone toxicity and other complications.

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