Pathogenesis of permeability barrier abnormalities in the ichthyoses: inherited disorders of lipid metabolism.

@article{Elias2008PathogenesisOP,
  title={Pathogenesis of permeability barrier abnormalities in the ichthyoses: inherited disorders of lipid metabolism.},
  author={Peter M Elias and Mary Lou Williams and Walter M. Holleran and Yan Jane Jiang and Matthias Schmuth},
  journal={Journal of lipid research},
  year={2008},
  volume={49 4},
  pages={697-714}
}
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… CONTINUE READING
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