En form av medfödd, autosomalt dominant iktyos som kännetecknas av erytrodermi och svår hyperkeratos. Den visar sig vid födseln som blåsor som följs av hornartade, vårtliknande fjäll över hela kroppen, mest framträdande i böjningsområden.
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.
Inga svenska synonymer finns.
Epidermolytic Hyperkeratoses — Hyperkeratoses, Epidermolytic — Bullous Congenital Ichthyosiform Erythroderma — Congenital Bullous Ichthyosiform Erythroderma — Ichthyosiform Erythroderma, Bullous Congenital — Epidermolytic Ichthyosis — Bullous Ichthyosiform Erythroderma Congenital — Bullous Erythroderma Ichthyosiformis Congenita of Brocq — Bullous Ichthyosiform Erythroderma — Bullous Ichthyosiform Erythrodermas — Erythroderma, Bullous Ichthyosiform — Erythrodermas, Bullous Ichthyosiform — Ichthyosiform Erythroderma, Bullous — Ichthyosiform Erythrodermas, Bullous — Epidermolytic Hyperkeratosis — Bullous Erythroderma Ichthyosiforme — Erythroderma Ichthyosiforme, Bullous — Bullous Erythroderma Ichthyosiformes — Erythroderma Ichthyosiformes, Bullous — Ichthyosiforme, Bullous Erythroderma — Ichthyosiformes, Bullous Erythroderma — Congenital Ichthyosiform Erythroderma, Bullous