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Systemic hyalinosis or juvenile hyaline fibromatosis

Ultrastructural and biochemical study of cultured skin fibroblasts

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Summary

Biochemical examinations of a hyalinized skin tumor of systemic hyalinosis (juvenile hyaline fibromatosis) revealed an increase in chondroitin sulfate synthesis by skin fibroblasts with an enlarged, rough surfaced endoplasmic reticulum as well as an increase in chondroitin sulfate content. These results indicate that systemic hyalinosis is a connective tissue disorder characterized by abnormal GAG synthesis of fibroblasts.

Zusammenfassung

Durch biochemische Untersuchungen von hyalinisierten Hauttumoren der systematisierten Hyalinose (juvenile hyaline Fibromatose) zeigte sich eine gesteigerte Chondroitinsulfatsynthese von dermalen Fibroblasten, die sich in einem erweiterten rauhen endoplasmatischen Reticulum darstellt, sowie eine Steigerung des Chondroitinsulfat-Gehaltes im befallenen Hautbereich. Diese Ergebnisse weisen auf, daß es sich bei der systematisierten Hyalinose um eine Bindegewebserkrankung handelt, die durch eine abnorme Glykosaminoglykan-Synthese der Fibroblasten charakterisiert ist.

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Authors and Affiliations

  1. Department of Dermatology, University of Gunma, School of Medicine, 371, Maebashi, Japan

    Shinjiro Iwata, Ryuya Horiuchi, Hidefumi Maeda & Hidekazu Ishikawa

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  1. Shinjiro Iwata
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  2. Ryuya Horiuchi
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  3. Hidefumi Maeda
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  4. Hidekazu Ishikawa
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Supported by Grant-in-Aid for Co-operative Research from the Ministry of Education, Science and Culture, Japan

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Iwata, S., Horiuchi, R., Maeda, H. et al. Systemic hyalinosis or juvenile hyaline fibromatosis. Arch Dermatol Res 267, 115–121 (1980). https://doi.org/10.1007/BF00569097

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  • DOI: https://doi.org/10.1007/BF00569097

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