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Pseudoxanthoma elasticum. Biochemical and ultrastructural studies

I Pasquali-Ronchetti, D Volpin, M Baccarani-Contri

    Dermatologica
    |January 1, 1981
    PubMed
    Summary

    Pseudoxanthoma elasticum involves mineral deposits in dermal elastin fibers, leading to altered structure and composition. Collagen and ground substance changes also contribute to this connective tissue disorder.

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    Area of Science:

    • Dermatology
    • Biochemistry
    • Histology

    Background:

    • Pseudoxanthoma elasticum is a rare genetic disorder affecting elastic tissues.
    • Abnormalities in dermal connective tissue are characteristic of the condition.

    Purpose of the Study:

    • To investigate the ultrastructural and biochemical changes in the dermis of pseudoxanthoma elasticum patients.
    • To characterize the alterations in elastin, collagen, and ground substance.

    Main Methods:

    • Ultrastructural analysis using electron microscopy.
    • Biochemical assays for amino acid composition, uronic acids, hexosamines, and neutral sugars.

    Main Results:

    • Mineral deposits observed on elastin fibers with altered staining affinity and ultrastructure.

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  • Increased elastin with abnormal amino acid composition in severely affected patients.
  • Lateral fusion of collagen fibers and increased ground substance observed.

    • Conclusions:

    • Dermal alterations in pseudoxanthoma elasticum involve significant changes in elastin, collagen, and ground substance.
    • Mineralization of elastin is a key feature, alongside compositional and structural abnormalities.
    • These findings contribute to understanding the pathophysiology of pseudoxanthoma elasticum.