Archiv für dermatologische Forschung

, Volume 248, Issue 2, pp 149–172 | Cite as

Zur Ultrastruktur hereditärer Verhornungsstörungen

III. Autosomal-dominante Ichthyosis vulgaris
  • I. Anton-Lamprecht
Article

Zusammenfassung

Die autosomal-dominante Ichthyosis vulgaris unterscheidet sich durch einen Defekt der Keratohyalin-Synthese von der x-chromosomal recessiven Ichthyosis Wells-Kerr. In dem stets einreihigen Str. granulosum werden nur geringe Mengen eines atypischen, krümlig oder schwammartig erscheinenden Materials gebildet, an dessen Synthese Polyribosomen beteiligt zu sein scheinen. Der nucleäre Anteil des Keratohyalins wird demgegenüber in den spätesten Stadien der granulosum-Zellen in normaler Menge und Feinstruktur gebildet. Trotz des Keratohyalin-Defekts ist in den Zellen des orthohyperkeratotischen Horns ein normales Keratin-Muster nachweisbar. Eine Verminderung und feinstrukturelle Abweichungen der Keratinosomen konnten nicht bestätigt werden.

Ultrastructure of inborn errors of keratinization

III. Autosomal Dominant Ichthyosis vulgaris

Summary

A defective keratohyalin synthesis, resulting in minute granules of crumbly or spongy appearance in only one layer of granular cells, is an intrinsic feature of autosomal dominant ichthyosis vulgaris significantly differentiating this common inherited disease from x-linked recessive ichthyosis Wells-Kerr. Polyribosomes obviously participate in the synthesis of this abnormal keratohyalin. The nuclear type of keratohyalin, however, is formed in normal manner in the latest stages of granular cells. In spite of the defective heratohyalin, the hyperkeratotic horny layer reveals a normal keratin pattern. In contrast to Takaki's observations no reduction or abnormal ultrastructure has been found in keratinosomes. Also, tonofibrils, desmosomes, and the marginal envelope of the horny cells are ultrastructurally normal.

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Copyright information

© Springer-Verlag 1973

Authors and Affiliations

  • I. Anton-Lamprecht
    • 1
  1. 1.Universitäts-Hautklinik HeidelbergBRD

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