History: This boy and his younger sister had a combination of icthyosis, eczema and alopecia since birth.  Netherton's syndrome is a rare, autosomal recessive disorder characterized by ichthyosis, hair shaft defects, and atopy.   The affected infants are usually erythrodermic with dry skin but the characteristic polycyclic and serpiginous plaques with double-edged scale(ichthyosis linearis circumflexa ) and trichorrhexis invaginata (bamboo hair) usually develop later.