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Revista chilena de pediatría

Print version ISSN 0370-4106

Abstract

VEGA ALMENDRA, Nadia  and  ARANIBAR DURAN, Ligia. Hereditary ichthyosis: A diagnostic and therapeutic challenge. Rev. chil. pediatr. [online]. 2016, vol.87, n.3, pp.213-223. ISSN 0370-4106.  http://dx.doi.org/10.1016/j.rchipe.2015.07.025.

Hereditary ichthyoses are a group of genetic disorders of cornification, which are characterised by hyperkeratosis and scaling. The new classification identifies 36 types of ichthyosis, which are subdivided according to their frequency, pattern of inheritance and extracutaneous involvement. The diagnosis is mainly based on clinical features, since genetic studies are not available in our setting. Treatment is symptomatic and management should be performed by a multidisciplinary team. In this article, the diagnostic and therapeutic aspects of different types of ichthyosis are reviewed, taking into account the nomenclature and modifications presented in the new classification.

Keywords : Ichthyoses; Disorders of cornification; Collodion baby.

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