Altered lamellar body secretion and stratum corneum membrane structure in Netherton syndrome: differentiation from other infantile erythrodermas and pathogenic …
M Fartasch, ML Williams, PM Elias - Archives of dermatology, 1999 - jamanetwork.com
Background The infant with Netherton syndrome (NS) typically displays a generalized
erythroderma covered by fine, translucent scales, which can be difficult to distinguish
clinically from erythrodermic psoriasis, nonbullous congenital ichthyosiform erythroderma, or
other infantile erythrodermas. Some infants with NS develop progressive hypernatremic
dehydration, failure to thrive, and enteropathy. Such complications can be fatal. Diagnosis is
typically delayed until the appearance of a pathognomonic hair shaft anomaly, trichorrhexis …
erythroderma covered by fine, translucent scales, which can be difficult to distinguish
clinically from erythrodermic psoriasis, nonbullous congenital ichthyosiform erythroderma, or
other infantile erythrodermas. Some infants with NS develop progressive hypernatremic
dehydration, failure to thrive, and enteropathy. Such complications can be fatal. Diagnosis is
typically delayed until the appearance of a pathognomonic hair shaft anomaly, trichorrhexis …