Hyperkeratosis, Epidermolytic

Synonyms

Bullous Congenital Ichthyosiform Erythroderma

Bullous Erythroderma Ichthyosiforme

Bullous Erythroderma Ichthyosiformes

Bullous Erythroderma Ichthyosiformis Congenita of Brocq

Bullous Ichthyosiform Erythroderma

Bullous Ichthyosiform Erythroderma Congenital

Bullous Ichthyosiform Erythrodermas

Congenital Bullous Ichthyosiform Erythroderma

Congenital Ichthyosiform Erythroderma, Bullous

Epidermolytic Hyperkeratoses

Epidermolytic Hyperkeratosis

Epidermolytic Ichthyosis

Erythroderma Ichthyosiforme, Bullous

Erythroderma Ichthyosiformes, Bullous

Erythroderma, Bullous Ichthyosiform

Erythrodermas, Bullous Ichthyosiform

Hyperkeratoses, Epidermolytic

Ichthyosiform Erythroderma, Bullous

Ichthyosiform Erythroderma, Bullous Congenital

Ichthyosiform Erythrodermas, Bullous

Ichthyosiforme, Bullous Erythroderma

Ichthyosiformes, Bullous Erythroderma

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.