1999
DOI: 10.1001/archderm.135.7.823
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Altered Lamellar Body Secretion and Stratum Corneum Membrane Structure in Netherton Syndrome

Abstract: 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… Show more

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Cited by 93 publications
(22 citation statements)
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“…Micro-splits in the stratum corneum were frequently observed in the stratum corneum in the normal as well as in NS skin, but granular cell splits were not found in 20 normal control skin samples. Previously reported electron microscopic examinations of NS have found various morphological changes, including premature secretion of LG contents, and abnormal extracellular material in the stratum corneum (Fartasch et al, 1999). Premature detachment or entirely missing stratum corneum has also been documented (De Wolf et al, 1996, Hausser andAnton-Lamprecht, 1996).…”
Section: Figurementioning
confidence: 97%
“…Micro-splits in the stratum corneum were frequently observed in the stratum corneum in the normal as well as in NS skin, but granular cell splits were not found in 20 normal control skin samples. Previously reported electron microscopic examinations of NS have found various morphological changes, including premature secretion of LG contents, and abnormal extracellular material in the stratum corneum (Fartasch et al, 1999). Premature detachment or entirely missing stratum corneum has also been documented (De Wolf et al, 1996, Hausser andAnton-Lamprecht, 1996).…”
Section: Figurementioning
confidence: 97%
“…elektronenmikroskopisch als Zeichen der Differenzierungsstörung die typische Akkumulationen von intrazytoplasmatischen Einschlusskörperchen ("lamellar bodies") als auch interzytoplasmatisch angesammeltes amorphes Material im Stratum corneum [4,12].…”
Section: Diskussionunclassified
“…These complications are due to the loss of the epidermal barrier function [9]. Indeed, skin biopsies of patients demonstrate impaired maturation of lamellar membrane structures and premature secretion of lamellar body contents, leading to disorganization of the epidermal barrier [9]. …”
Section: Introductionmentioning
confidence: 99%