Definition: characterized by localized lesions, keratosis palmaris and plantaris, and elevated IgE, associated with hyperkeratosis, hypergranulosis, and reticular degeneration in the upper epidermis; autosomal dominant inheritance, caused by mutation in the epidermolytic palmoplantar keratoderma gene (EPPK) on chromosome 17q. Generalized epidermolytic hyperkeratosis is present in bullous congenital ichthyosiform erythroderma.
Synonym(s): porcupine skin
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