Epidermolytic Ichthyosis

**Semantic type:** Disease or Syndrome

**Definition:** An autosomal dominant inherited skin disorder caused by mutations in the KRT1 and KRT10 genes. It is manifested at birth and is characterized by generalized erythema, skin blisters and skin fragility.

**Synonyms:** - BCIE - Bullous Congenital Ichthyosiform Erythroderma - Epidermolytic Hyperkeratosis