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ZHANG Lu-xia, HU Zheng-zheng, DENG Wei-ping. Department of Dermatology, Guangdong General Hospital��Guangdong Academy of Medical Sciences��, Guangzhou 510080, China Corresponding author: DENG Wei-ping, Email: oldeng148@yahoo.com.cn ��Abstract�� X-linked ichthyosis ��XLI�� is a relatively common X-linked recessive cutaneous keratinization disorder. It is clinically characterized by skin dryness, large adherent brown scales, and may be complicated by extracutaneous manifestations including corneal opacities, cryptorchidism, and abnormalities associated with some syndromes. Most recent studies have shown that XLI is caused by the absence or mutation of steroid sulfatase��STS�� gene encoding steroid sulfates. Concurrent abnormalities of STS and filaggrin ��FLG�� genes are usually associated with a more severe phenotype. Since histopathological findings are nonspecific, the diagnosis of XLI is usually based on clinical features, and mainly confirmed by biochemical detection and genetic analysis. Recently, in-depth studies have been performed on the pathogenesis of XLI, and some progress has been made in pathogenesis-targeted therapy for XLI. ��Key words�� X-linked ichthyosis; Steryl-sulfatase; Hyperkeratosis

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