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Ultrastructure of inborn errors of keratinization

VII. Porokeratosis mibelli and disseminated superficial actinic porokeratosis

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Summary

The cornoid lamella and the underlying epidermis were studied by electron microscopy on specimens biopsied from 2 patients with porokeratosis Mibelli and 1 patient with actinic porokeratosis.

Findings on the two types of porokeratoses are essentially the same. The cornoid lamella was composed chiefly of extremely irregular dark cells and a few numbers of dyskeratotic cells. Both cells retained a nuclear remnant and many other degraded organelles. The epidermal cells just beneath the cornoid lamella simultaneously demonstrated productive and degenerative signs. Some of these epidermal cells underwent dyskeratosis and appeared as corps ronds-like bodies in the granular layer. Two contradictory phenomena should be attributed to the pathogenesis of cornoid lamella.

In the cornoid lamella above the sweat pore microvilli-structures were found.

Zusammenfassung

Die Cornoid-Lamelle und die daruntergelegene Epidermis von den Hautexcisaten zweier Fälle mit Porokeratosis Mibelli und eines Falles mit actinischer Porokeratosis wurden elektronenmikroskopisch untersucht.

Die Befunde bei beiden Typen der Porokeratosis sind im Wesentlichen gleich. Die Cornoid-Lamelle besteht hauptsächlich aus dunklen Zellen mit extrem unregelmäßigem Umriß und aus einigen dyskeratotischen Zellen. In diesen Zellen bleiben der Kernrest und zahlreiche degradierte Organellen erhalten. Die Epidermiszellen unmittelbar unter der Cornoid-Lamelle zeigen gleichzeitig produktive und degenerative Veränderungen. Einige Epidermiszellen verhornen dyskeratotisch und erscheinen als corps ronds-ähnliche Körper in der Granularschicht. Die Pathogenese der Cornoid-Lamelle ist zwei widersprechenden Phänomenen in den daruntergelegenen Epidermiszellen zuzuschreiben.

In der Cornoid-Lamelle über den Schweißdrüsen-Ausführungsgängen lassen sich Mikrovilli finden.

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Authors and Affiliations

  1. Univ.-Hautklinik Heidelberg, Voßstraße 2, D-6900, Heidelberg, Republic of Germany

    A. Sato, I. Anton-Lamprecht & U. W. Schnyder

  2. Abteilung Dermatologie I, Institut für Ultrastrukturforschung der Haut, Voßstraße 2, D-6900, Heidelberg, Republic of Germany

    A. Sato, I. Anton-Lamprecht & U. W. Schnyder

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  1. A. Sato
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  2. I. Anton-Lamprecht
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  3. U. W. Schnyder
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Scholarship holder of the Alexander von Humboldt foundation.

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Sato, A., Anton-Lamprecht, I. & Schnyder, U.W. Ultrastructure of inborn errors of keratinization. Arch. Derm. Res. 255, 271–284 (1976). https://doi.org/10.1007/BF00561498

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  • DOI: https://doi.org/10.1007/BF00561498

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