A Case of Netherton's Syndrome / 대한피부과학회지

ABSTRACT

Netherton's syndrome is a rare autosomal recessive disorder characterized by the concurrence of ichthyosis, structural hair shaft abnormality, and atopy. The pathognomonic finding is that of trichorrhexis invaginata identified by light and scanning electron microscopic examination of hair shaft. We herein report a case of Netherton's syndrome in a 51-year-old woman who had polycyclic, serpiginous and erythematous patches with a characteristic, migratory, double-edged scale at the margins as a skin manifestation and trichorrhexis invaginata as a hair defect.