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According to the consensus classification of congenital ichthyoses published in 2010,8 congenital ichthyoses are categorized into non-syndromic (when all symptoms are caused by the epidermal barrier defect and there are only cutaneous signs) and syndromic ichthyoses (when the causal gene has extracutaneous functions that determine manifestations in other organs).9 Following this criterion, we have included diseases described later or that have been better characterized in recent years, updating them both clinically and molecularly