Many of the ichthyoses are associated with inherited disorders of lipid metabolism. These
disorders have provided unique models to dissect physiologic processes in normal
epidermis and the pathophysiology of more common scaling conditions. In most of these
disorders, a permeability barrier abnormality" drives" pathophysiology through stimulation of
epidermal hyperplasia. Among primary abnormalities of nonpolar lipid metabolism,
triglyceride accumulation in neutral lipid storage disease as a result of a lipase mutation …