Applications of the Diazacholesterol Animal Model of Ichthyosis

Abstract

Although scaling skin diseases traditionally have been considered disorders of protein metabolism, i.e., keratinization, there is little support for this hypothesis. Instead, the expanding list of inherited and acquired disorders that are associated with primary lipid abnormalities (Table 1, Fig. 1), implies that it is stratum corneum lipids which regulate stratum corneum cohesion and desquamation (reviewed in [4, 16]). Whereas relevant examples of inherited scaling dermatoses that are associated with abnormal lipid metabolism include recessive x-linked ichthyosis (RCLI), Refsum’s disease; multiple sulfatase deficiency, and neutral lipid storage disease, examples of acquired disorders include ichthyosis occurring following administration of hypocholesterolemic agents (reviewed in [16]). Essential fatty acid deficiency (EFAD), both in rodents and in man, represents an additional scaling disorder where stratum corneum barrier function and scaling have been attributed to separate abnormalities in lipid metabolism [14].