2022 5th Comprehensive Medical Sciences(CMS)

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演者：AKIYAMA, Masashi, M.D., Ph.D. Professor and Chairman, Department of Dermatology, Nagoya University G

演題：　Ichthyosis and autoinflammatory keratinization disease, two sides of the same coin?

座長：　藤田医科大学大学院　医学研究科　
皮膚科学　教授　杉浦　一充　先生

Abstract：
Interplay of skin barrier defects and inflammatory responses is the major pathogenetic mechanisms of both ichthyosis and autoinflammatory keratinization disease (AiKD).
Corneocyte lipid envelope, mainly a single layer of acylceramides, is a key structure for the stratum corneum barrier, and defects in ceramide synthesis/metabolism/transport are genetic causes of many types of ichthyosis. Inflammatory keratinization diseases of the skin with genetic autoinflammatory pathomechanisms are AiKDs, and generalized pustular psoriasis is a typical disease of AiKD. Interestingly, important immune profiles, activation of innate immunity and IL-17 signatures, are shared between ichthyosis and AiKD. Considering the immune profiles of the diseases, ichthyosis and AiKD may be two sides of the same coin.

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