Netherton's syndrome: an electronmicroscopic study.
An ultrastructural study of 2 patients with Netherton's syndrome (bamboo hair, scaling dermatosis, and an atopic diathesis) showed features of psoriasis and a dermatitis. Although the biopsies from the patients showed histological and ultrastructural similarities, the clinical presentations were different. One patient had ichthyosis linearis circumflexa, while the other had a generalized ichthyosiform eruption. The ultrastructural findings in the 2 patients, while not specific, may help distinguish the eruption of Netherton's syndrome from other scaling dermatoses.
D000328:Adult; D003876:Dermatitis, Atopic / Q000473:pathology; D004476:Ectodermal Dysplasia / Q000473:pathology; D005110:Extracellular Space; D006197:Hair; D006801:Humans; D007057:Ichthyosis / Q000473:pathology*; D007223:Infant; D008297:Male; D011565:Psoriasis / Q000473:pathology; D012536:Scalp Dermatoses / Q000473:pathology*; D012867:Skin / Q000648:ultrastructure*; D013577:Syndrome