A Case of Netherton's Syndrome / 대한피부과학회지

ABSTRACT

Nethertons syndrome is characterized by a triad of ichthyosiform dermatosis, multiple hair-shaft defects(including trichorrhexis invaginata), and an atopic diathesis. Intermittent amminoaciduria, mental retardation, or recurrent infection have been observed in some cases. We have seen an 8-year-old girl presenting with polycyclic, erythematous patches bordered by distintive double-edged scales as a skin manifestation and ball-and-socket deformity as a hair defect.