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Ichthyosis congenitaQ80.8
Synonym(s)
congenital ichthyosis; Hyperkeratosis ichthyosiformis congenita
DefinitionThis section has been translated automatically.
Only historically relevant generic term for a group of rare, congenital, usually severe ichthyoses with predominantly autosomal recessive inheritance and varying degrees of severity.
Originally, it was subdivided by Riecke into severity grades I to III according to the clinic, as ichthyosis congenita gravis, mitis and tarda. In the new classifications, these syndromes are listed in different categories and cannot be further classified retrospectively, because a molecular genetic classification of the syndromes, which is the basis of every classification today, was not possible at that time (see below Ichthyoses - Overview).