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Lamellar Ichthyosis


Disorder due to an accelerated rate of epidermal turnover with proliferative hyperkeratosis and abnormal epidermis formation via transglutaminase gene mutations. Autosomal dominant but also recessive forms have been described. At birth affected newborn may display the clinical phenotype of a collodion baby. In adulthood patients present with erythroderma and generalized dark platelike scaling, especially in flexural areas, associated with ectropion, a decrease in sweating and heat intolerance.




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