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Systemic hyalinosis (juvenile hyaline fibromatosis)

Ultrastructure of the hyaline with particular reference to the cross-banded structure

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Summary

Systematic hyalinosis (juvenile hyaline fibromatosis) is characterized by hyalinized skin lesions. Electron microscopic examination of a hyalinized skin tumor from a 19-year-old man with this syndrome revealed that the hyaline was composed of ruthenium red-positive ultrastructures (granules, filaments, and a kind of cross-banded structure), indicating the presence of glycosaminoglycan or glycoprotein, and a small number of thin collagen fibrits. Using a new ruthenium red staining method combined with an enzymatic digestion procedure, it was demonstrated that the cross-banded structure and granules consisted of chondroitin sulfate-proteoglycan and/or glycoprotein, and that the cross-banded structure is probably a noncollageneous aggregate of the granules attached to the parallel-arranged filaments of hyaluronic acid-like nature.

Zusammenfassung

Systematisierte Hyalinose (Juvenile Hyaline Fibromatose) ist durch die hyalinisierten Hautefflorescenzen gekennzeichnet. Die elektronenmikroskopische Untersuchung eines hyalinisierten Hauttumors von einem 19jährigen Mann mit diesem Syndrom ergab, daß die Hyalinsubstanz sich aus den Rutheniumrot-positiven Ultrastrukturen des Glykosaminoglykans oder des Glykoproteins (d. h. Körnchen, Fäden und einer Struktur mit Querstreifen) und einigen dünnen Kollagenfibrillen zusammensetzte. Mit unserer neuen Rutheniumrotfärbung in der Kombination mit der Enzymverdauungsmethode wurde demonstriert, daß die Struktur mit Querstreifen und die Körnchen aus Chondroitinsulfat-Proteoglykan und/oder Glykoprotein besteht, und daß es sich bei der Struktur mit Querstreifen höchstwahrscheinlich um ein nichtkollagenes Aggregationsprodukt der Körnchen handelt, die eng an die parallel angeordneten, Hyaluronsäure-ähnlichen Fäden angelegt sind.

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Authors and Affiliations

  1. Department of Dermatology (Director: Prof. Hidekazu Ishikawa), University of Gunma, School of Medicine, 371, Maebashi, Japan

    Hidekazu Ishikawa, Hidefumi Maeda, Hisao Takamatsu & Yosio Saito

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  1. Hidekazu Ishikawa
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  2. Hidefumi Maeda
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  3. Hisao Takamatsu
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  4. Yosio Saito
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Supported by Research grants from the Ministry of Education, Japan

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Ishikawa, H., Maeda, H., Takamatsu, H. et al. Systemic hyalinosis (juvenile hyaline fibromatosis). Arch Dermatol Res 265, 195–206 (1979). https://doi.org/10.1007/BF00407885

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  • DOI: https://doi.org/10.1007/BF00407885

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