Answer
Epidermolytic ichthyosis (EI), formerly known as epidermolytic hyperkeratosis (EHK) or bullous congenital ichthyosiform erythroderma (bullous CIE), is a form of congenital ichthyosis. It is inherited in an autosomal dominant fashion, with about 50% of cases representing spontaneous mutations. Epidermolytic ichthyosis presents at birth with erythroderma, blisters, and erosions and evolves over time into varying degrees of hyperkeratosis.
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Media Gallery
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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.
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