Article
Ichthyosis Linearis Circumflexa as the Only Clinical Manifestation of Netherton Syndrome.
Acta dermato-venereologica - 1 Jul 2015
Guerra Liliana, Fortugno Paola, Pedicelli Cristina, Mazzanti Cinzia, Proto Vittoria, Zambruno Giovanna, Castiglia Daniele
Abstract excerpt
Ichthyosis linearis circumflexa (ILC) presents as serpiginous and migratory erythematous patches with double-edged scales. ILC is rarely an isolated skin manifestation, but most commonly a part of Netherton syndrome (NS). NS is caused by SPINK5 mutations, which lead to absent or sometimes reduced expression of the serine protease inhibitor LEKTI. NS is characterised by congenital ichthyosiform erytroderma,...
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