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Anais Brasileiros de Dermatologia

On-line version ISSN 1806-4841

Abstract

DUQUIA, Rodrigo Pereira; ALMEIDA JR, Hiram Larangeira de; SOUZA, Paulo Ricardo Martins  and  VETTORATO, Gerson. Netherton's Syndrome with a 20-year follow-up. An. Bras. Dermatol. [online]. 2006, vol.81, n.6, pp. 563-566. ISSN 1806-4841.  http://dx.doi.org/10.1590/S0365-05962006000600008.

Netherton's syndrome is a recessive autosomal skin disease, characterized by congenital erythroderma, hair anomalies such as trichorrhexis invaginata, and atopic manifestations. The case of a female patient with a 20-year follow-up is described, with an important improvement of hair alterations after use of oral acitretine.

Keywords : Ichthyosis; Mutation; Retinoids.

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