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Ichthyosis is a genetic disease in which the skin is mainly affected, it has different types depending on the inheritance pattern. Within the dominants we find the ichthyosis vulgaris and on the recessive ones we have the lamellar ichthyosis, harlequin Ichthyosis and Ichthyosiform erythroderma.Lamellar Ichthyosis is a congenital genodermatitis that according to international statistical data it reports 1:200 000-300 000 newborns. Within the databases consulted in Honduras no prevalence and incidence of the disease was found.Materials and methods: It was analyzed the genealogical tree of 6 patients plus the valuation of clinical manifestations of each individual to determine the specific inheritance pattern to identify the type of Ichthyosis.Results: The analysis demonstrated an autosomal inheritance pattern and consanguinity in the patient’s parents.Conclusions: The realization of a pedigree it’s essential in conjunction with clinical examination for the diagnosis of ichthyosis in absence of histopathologic and genetical tests.

Reporte de casos de ictiosis laminar en honduras; desafío diagnóstico.