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.

Descriptor ID	D017488
MeSH Number(s)	C16.131.831.512.400.375 C16.320.850.400.375 C16.614.492.400.375 C17.800.428.333.250.375 C17.800.804.512.400.375 C17.800.827.400.375
Concept/Terms	Hyperkeratosis, Epidermolytic Hyperkeratosis, Epidermolytic 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

Below are MeSH descriptors whose meaning is more general than "Hyperkeratosis, Epidermolytic".

Below are MeSH descriptors whose meaning is related to "Hyperkeratosis, Epidermolytic".

Below are MeSH descriptors whose meaning is more specific than "Hyperkeratosis, Epidermolytic".