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Ichthyosis-Trichothiodystrophy Syndrome

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Neurocutaneous Disorders

Abstract

The term ichthyosis designates a disorder of persistently dry and thickened skin that resembles fish scales or snakeskin. Scaling can be localized or generalized and can be associated with a variety of cutaneous manifestations. In total, 36 different types of ichthyoses are currently included in the international classification, supplanting previous eponyms such as bullous congenital ichthyosiform erythroderma and epidermolytic hyperkeratosis. The variety of ichthyoses carry disparate inherited and acquired forms such as ichthyosis vulgaris (>95% of the cases), lamellar ichthyosis (ALOXE3 and ALOX12B genes) and epidermolytic hyperkeratosis. Management demands a multidisciplinary team based on the severity of clinical symptoms.

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Panteliadis, C.P. (2022). Ichthyosis-Trichothiodystrophy Syndrome. In: Panteliadis, C.P., Benjamin, R., Hagel, C. (eds) Neurocutaneous Disorders. Springer, Cham. https://doi.org/10.1007/978-3-030-87893-1_37

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  • DOI: https://doi.org/10.1007/978-3-030-87893-1_37

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