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Ichthyosis linearis circumflexa comèl with trichorrhexis invaginata (netherton's syndrom)

An Ultrastructural Study of the Skin Changes

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Summary

An electron microscopical study of skin from 2 patients with ichthyosis linearis circumflexa and trichorrhexis invaginata revealed a severe and localized disturbance of keratinization occuring at the keratotic border of figurate skin lesions. It is characterized by a marked decrease of the desmosome-tonofilament system, lack of membrane coating granules and keratohyalin, and the appearence of roundoval, electron dense bodies in the upper spinous layers. The result is a completely disorganized “stratum corneum”. We consider this unique keratinization disorder to be specific for this disease.

Zusammenfassung

Die elektronenmikroskopische Untersuchung der Haut von zwei Patienten mit Ichthyosis linearis circumflexa und Trichorrhexis invaginata zeigte eine schwere, am Rande figurierter Hautveränderungen vorkommende Verhornungsstörung. Sie ist charakterisiert durch eine deutliche Verminderung der Tonofibrillen und Desmosomen, durch Fehlen von Kerationsomen und Keratohyalin und durch das Auftreten rundlichovaler, elektronendichter Körper in den oberen Malpighi-Schichten. Das Resultat dieser Veränderungen ist eine völlig dysorganisierte Hornschicht. Wir vermuten, daß die beschriebene Verhornungsstörung für diese Krankheit spezifisch ist.

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Authors and Affiliations

  1. Clinique universitaire de dermato-vénéréologie Lausanne, Switzerland

    E. Frenk

  2. Centre de microscopie électronique de l'Université de Lausanne, Switzerland

    B. Mevorah

Authors
  1. E. Frenk
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  2. B. Mevorah
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Frenk, E., Mevorah, B. Ichthyosis linearis circumflexa comèl with trichorrhexis invaginata (netherton's syndrom). Arch. Derm. Forsch. 245, 42–49 (1972). https://doi.org/10.1007/BF00596151

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  • DOI: https://doi.org/10.1007/BF00596151

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