Other terms
Epidermolytic Hyperkeratoses,Epidermolytic Hyperkeratosis,Hyperkeratoses, Epidermolytic,Congenital Bullous Ichthyosiform Erythroderma,Congenital Ichthyosiform Erythroderma, Bullous,Ichthyosiform Erythroderma, Bullous Congenital,Bullous Ichthyosiform Erythroderma Congenital,Bullous Congenital Ichthyosiform Erythroderma,Erythroderma Ichthyosiforme, Bullous,Bullous Erythroderma Ichthyosiforme,Bullous Erythroderma Ichthyosiformes,Erythroderma Ichthyosiformes, Bullous,Ichthyosiforme, Bullous Erythroderma,Ichthyosiformes, Bullous Erythroderma
Description
Hyperkeratosis, Epidermolytic: A form of congenital ichthyosis inherited as an autosomal dominant trait and characterized by ERYTHRODERMA and severe hyperkeratosis. It is manifested at birth by blisters followed by the appearance of thickened, horny, verruciform scales over the entire body, but accentuated in flexural areas. Mutations in the genes that encode KERATIN-1 and KERATIN-10 have been associated with this disorder.
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