Bullous congenital ichthyosiform erythroderma (BCIE), also known as epidermolytic hyperkeratosis (EHK) [1] and epidermal differentiation disorder (EDD) [2], is a rare autosomal dominant disorder caused by mutations in the keratin 1 (KRT1) or keratin 10 (KRT10) genes, encoding structural proteins essential for epidermal integrity [3]. The gene discussed is KRT1; the disease is epidermolytic ichthyosis.