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Zur Ultrastruktur und Histochemie der granulösen Degeneration bei bullöser Erythrodermie congénitale ichthyosiforme

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Summary

Electron microscopic and histochemical studies of a non0bullous protion of skin from a case of Erythrodermie congénitale ichthyosiforme bulleuse have revealed the following findings:

  1. 1.

    Tonofilaments are excessively produced; this is accompanied by increase in numbers of ribosomes and mitochondria in the cytoplasm of epidermal cells.

  2. 2.

    Tonofibrils are clumped and form thick perinuclear shells. They show a cross striation with a periodicity of 170 Å which is not present in normal tonofibrils; this suggests malformation of tonofibrils.

  3. 3.

    Keratohyalin is present in excessive quantity and forms large masses (“granular degeneration”).

  4. 4.

    The formation of horn complex is qualitatively impaired.

The relation of these morphologic findings to those in other diseases with intraepidermal blisters is discussed.

Zusammenfassung

In einer nichtbullösen Hautstelle bei Erythrodermie congénitale ichthyosiforme bulleuse ließen sich elektronenmikroskopisch und histochemisch die folgenden Befunde erheben:

  1. 1.

    Überproduktion von Tonofilamenten mit Vermehrung von Ribosomen und Mitochondrien.

  2. 2.

    Die Tonofibrillen sind verklumpt, bilden perinucleäre Schalen und zeigen eine von der Norm abweichende Querstreifung, was als Tonofibrillenfehlbildung aufgefaßt wird.

  3. 3.

    Keratohyalin ist vermehrt und bildet große Schollen („granulöse Degeneration”).

  4. 4.

    Die Horkomplexbildung ist qualitativ gestört.

Die morphologischen Beziehungen zu andern intraepidermalen Blasenkrankheiten werden besprochen.

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Authors and Affiliations

  1. Anatomisches Institut der Universität Zürich, Zürich, Schweiz

    E. R. Weibel & U. W. Schnyder

  2. Dermatologische Universitäts-Klinik Zürich, Zürich, Schweiz

    E. R. Weibel & U. W. Schnyder

  3. Universitäts-Hautklinik Heidelberg, Heidelberg, Deutschland

    E. R. Weibel & U. W. Schnyder

  4. Anatomisches Institut der Universität, Bühlstr. 26, CH-3000, Bern

    E. R. Weibel

Authors
  1. E. R. Weibel
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  2. U. W. Schnyder
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Weibel, E.R., Schnyder, U.W. Zur Ultrastruktur und Histochemie der granulösen Degeneration bei bullöser Erythrodermie congénitale ichthyosiforme. Arch. klin. exp. Derm. 225, 286–298 (1966). https://doi.org/10.1007/BF00517258

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