In the animal model of bullous ichthyosiform erythroderma (also known as epidermolytic hyperkeratosis), both homozygous and heterozygous mice lacking keratin-10 compared to wild type mice presented significantly increased levels of ceramide 2 (CER[NS]) with a concomitant reduction of several other classes of ceramides to the total amount of SC lipids, and also reduced levels of sphingomyelin and glucosylceramide [17]. Here, CBLN1 is linked to epidermolytic ichthyosis.