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Netherton's syndrome

An Electronmicroscopic Study

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Summary

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.

Zusammenfassung

Eine elektronenmikroskopische Untersuchung der Epidermis von 2 Patienten mit Netherton's Syndrom (Bambushaar, schuppende Dermatose und eine atopische Diathese) zeigten Züge von Psoriasis und Dermatitis. Obwohl die Biopsien der Patientenhistologische und ultrastrukturelle Ähnlichkeiten aufwiesen, waren die klinischen Erscheinungsbilder unterschiedlich. Ein Patient litt an Ichthyosis linearis circumflexa während der andere eine generalisierte ichthyosiforme Eruption aufwies. Die ultrastrukturellen Befunde bei den beiden Patienten waren zwar nicht spezifisch, scheinen aber doch dazu beitragen zu können, die Hauteruptionen von Netherton's Syndrom von anderen schuppenden Dermatosen abzugrenzen.

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Thorne, E.G., Zelickson, A.S., Mottaz, J.H. et al. Netherton's syndrome. Arch. Derm. Res. 253, 177–183 (1975). https://doi.org/10.1007/BF00582069

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  • DOI: https://doi.org/10.1007/BF00582069

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