Congenital ichthyosiform erythroderma Congenital bullous ichthyosiform erythroderma SNOMEDCT_US:254167000 Epidermolytic hyperkeratosis Bullous congenital ichthyosiform erythroderma UMLS:C0079153 HP:0007475 An ichthyosiform abnormality of the skin that presents at birth or shortly thereafter with generalized erythema, blistering, erosions, and peeling. In the subsequent months, erythema and blistering improves but patients go on to develop hyperkeratotic scaling that is especially prominent along the joint flexures, neck, hands and feet. Epidermolytic hyperkeratosis can refer to the disease caused by mutations in the genes keratin 1 or keratin 10, but is here used to refer to the characteristic skin manifestations as a phenotypic feature. SNOMEDCT_US:239071005