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Marfan syndrome: demonstration of abnormal elastic fibers in skin

T Tsuji

    Journal of Cutaneous Pathology
    |April 1, 1986
    PubMed

    Insights

    Electron microscopy revealed abnormal elastic fibers in Marfan syndrome skin, characterized by fine, cobweb-like structures suggesting incomplete elastic unit fusion.

    Area of Science:

    • Dermatology
    • Connective Tissue Disorders
    • Electron Microscopy

    Background:

    • Marfan syndrome is a genetic disorder affecting connective tissue.
    • Skin elasticity is often compromised in Marfan syndrome patients.

    Purpose of the Study:

    • To investigate the ultrastructural changes in the skin of a patient with Marfan syndrome.
    • To characterize the morphology of abnormal elastic fibers in affected skin.

    Main Methods:

    • Scanning Electron Microscopy (SEM) was used to examine the overall structure of elastic fibers.
    • Transmission Electron Microscopy (TEM) was employed to analyze the substructure of the abnormal fibers.

    Main Results:

    • SEM revealed abnormal portions of elastic fibers with a cobweb-like appearance, composed of fine fibers (5-22 nm).
    • TEM showed these fine fibers as substructures within the amorphous material, surrounded by microfibrils.

    Conclusions:

    • The observed fine fibers likely represent abnormal elastic fibers.
    • Incomplete fusion of elastic units is suggested as the cause of these structural abnormalities in Marfan syndrome skin.

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