Bullous congenital ichthyosiform erythroderma (BCIE, OMIM 113800), also known as epidermolytic hyperkeratosis (EH) and now classified inside the class of Keratinopatic Ichthyoses [3], is an autosomal dominant disorder of the skin associated to mutations in KRT1 and KRT10 genes. The gene discussed is KRT10; the disease is epidermolytic ichthyosis.