Abstract

Congenital and acquired ichthyosis and kidney diseases are common diseases in clinic.The mutation or deletion of genes encoding filaggrin,keratin,steroid sulfatase(STS) and transglutaminase-1(TGM-1) will result in ichthyosis.STS gene deletion leads to accumulation of cholesterol sulfate in the glomerular visceral epithelial cells,induces TGM-1 dysfunction,thus causing dysfunction of slit diaphragm of the glomerular visceral epithelial cells and proteinuria,and finally results in the occurrence and development of kidney diseases.The concomitant kidney diseases of ichthyosis may be manifested as proteinuria,steroid-resistant nephrotic syndrome,chronic pyelonephritis,renal failure,or even renal carcinoma.

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