Autoinflammatory keratinization diseases

Among the genetic causes/predisposing factors for inflammatory keratinization disorders, several factors are associated with autoinflammatory mechanisms. Here we review these inflammatory keratinization disorders with autoinflammatory pathogenic mechanisms and advocate the novel and unique concept of autoinflammatory keratinization diseases (AIKDs). We propose the following definition of AIKD. First, the primary and main inflammation sites are the epidermis and the upper dermis. Second, inflammation in the epidermis and upper dermis leads to hyperkeratosis, which is the main and characteristic phenotype of AIKDs.

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