Accurate diagnosis of epidermolytic ichthyosis (EI) is important in order to properly inform and counsel parents. Genetic counseling and prenatal diagnosis also can be offered. [18]
Newborns with epidermolytic ichthyosis who have denuded skin are at increased risk for infection, secondary sepsis, and electrolyte imbalance. These newborns should be transferred to the neonatal ICU to monitored and treated as needed. They should be handled gently to avoid further trauma to the skin.
Wound care for blistering and moisturization/emollients are important in the newborn period. In older children, topical emollients and topical keratolytics are generally the mainstays of treatment. The accumulation of scale predisposes to overgrowth of bacteria, in particular with Staphylococcus aureus, which is often associated with odor. Patients may benefit from the use of mild antibacterial soaps or dilute bleach baths. Some patients may also benefit from therapy with oral or topical retinoids. [19, 20]
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Pathology of epidermolytic ichthyosis (hematoxylin and eosin stain).
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Pathology of epidermolytic ichthyosis (hematoxylin and eosin stain).
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The scale in epidermolytic ichthyosis is classically described as "corrugated". Patients often experience erosions as a result of skin fragility.
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The scale in epidermolytic ichthyosis is classically described as "corrugated". Patients often experience erosions as a result of skin fragility.
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Palms and soles may have varying degrees of hyperkeratosis.
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Hyperkeratosis involving the abdomen.
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Hyperkeratosis involving the knee.
