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Erythrodermia ichthyosiformis congenita bullosa Brocq. Über die sogenannte granulöse Degeneration

III. Elektronenmikroskopische Untersuchung der Granularschicht
  • Y. Ishibashi
  • G. Klingmüller
Article

Zusammenfassung

Bei der Erythrodermia ichthyosiformis congenita bullosa Brocq läßt sich elektronenmikroskopisch in der Granularschicht im Vergleich mit der normalen Epidermis folgendes finden:
  1. 1.

    Die Keratohyalinbildung ist manchmal schon in der untersten Stachelzellschicht zu erkennen (vorzeitige Keratohyalinbildung). Sie ist nach oben zumeist vermehrt.

     
  2. 2.

    Eine Keratohyalinanlagerung mit reichlich Ribosomen läßt sich nicht nur an den Tonofilamentsträhnen, sondern auch an den -klumpen in gleicher Weise erkennen. Die Größe, Gestalt und Verteilung ist unregelmäßig und mannigfaltig. Gelegentlich sind rundlich gestaltete Keratohyalinkugeln mit zahlreichen Ribosomen kaum oder ohne Tonofilamente erkennbar.

     
  3. 3.

    Keratinosomen, die sich manchmal schon in der unteren Malpighischen Schicht erkennen lassen, sind in der oberen Schicht besonders in der Zellperipherie mäßig oder gelegentlich auffällig vermehrt.

     
  4. 4.

    In der unteren Malpighischen Schicht scheinen sie sich aus keineren vacuoligen oder knäuelförmigen Elementen zu entwickeln, die zum Teil wahrscheinlich aus dem Golgi-Apparat entstanden sind.

     
  5. 5.

    Die stäbchenförmigen Elemente, die inter- oder intracellulär direkt unter der Hornschicht zu erkennen sind, scheinen morphologisch den lamellösen Strukturen der Keratinosomen zu entsprechen. Das könnte als eine inter- oder intracelluläre Autolyse von Keratinosomen aufgefaßt werden.

     

Abkürzungen zu den Abbildungen

D

Desmosom

Ek

Knäuelförmige Elemente

Ev

Vacuolige Elemente

G

die oberste Malpighische Zelle

GIM

„Granuläre intracytoplasmatische Membran”

Gol

Golgi-Apparat

H

Hornschicht

Is

Intercellulärer Spalt

K

Kern

Kh

Keratohyalin

Khk

Keratohyalinkugel

Ks

Keratinosom

Ksh

Keratinscholle

Kv

Vesiculäre Komponente

M

Mitochondrium

R

Ribosomen

Tfb

Tonofilamentbündel

Tfk

Tonofilamentklumpen

Tfs

Tonofilamentsträhne

Ts

Stäbchenförmige Teilchen

Tsh

Tonofilamentschale

Ü

Übergangszelle

B

Bleicitrat

Kontr

Nachkontrastierung

U

Uranylacetat

Erythrodermia ichthyosiformis congenita bullosa Brocq. On the so-called granular degeneration

III. Electron microscopic investigation of the granular cell layer

Summary

An electron microscopic investigation in cases of congenital ichthyosiform erythroderma discloses the following in the granular cell layer by comparison with the normal epidermis:
  1. 1.

    The formation of keratohyalin sometimes is already seen in the lowest prickle cell layer (precocious formation of keratohyalin) and it becomes usually more and more prominent in the upper layer.

     
  2. 2.

    A deposit of keratohyalin with a large number of ribosomes is seen not only at the site of tufts of the tonofilaments, but also at the clumps. The size, shape and distribution of this material is irregular and varying. Occasionally “balls of keratohyalin” with numerous ribosomes and with few or no tonofilaments, are observed.

     
  3. 3.

    The keratinosomes, which are sometimes already found in the lower Malpighian layer, are increased in number moderately or occasionally striking in the upper Malpighian layer, particularly in the peripheral parts of the cells.

     
  4. 4.

    In the lower Malpighian layer they appear to develope from small vacuolated or clue shaped elements, some of which may probably originate from the Golgiapparat.

     
  5. 5.

    The rod shaped elements, which are observed intercellular or intracellular directly under the stratum corneum, seem to correspond morphologically to the lamellar structures of keratinosomes. This finding may explain an intercellular or intracellular autolysis of keratinosomes.

     

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Copyright information

© Springer-Verlag 1968

Authors and Affiliations

  • Y. Ishibashi
    • 1
  • G. Klingmüller
    • 1
  1. 1.Universitäts-Hautklinik BonnBonnDeutschland

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