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.
See Also