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Monilethrix: an electron microscopic and electron histochemical study

C L Gummer, R P Dawber, J A Swift

    The British Journal of Dermatology
    |November 1, 1981
    PubMed
    Summary
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    Structural abnormalities in monilethrix hair shafts originate in the follicle's keratinization zone. These defects affect the hair cortex and cuticle, potentially due to follicular asynchrony.

    Area of Science:

    • Dermatology
    • Cell Biology
    • Microscopy

    Background:

    • Monilethrix is a rare genetic hair disorder characterized by fragile, beaded hair shafts.
    • Previous studies have identified structural abnormalities in monilethrix hair, but the precise origin remains unclear.

    Purpose of the Study:

    • To investigate the ultrastructural basis of hair shaft abnormalities in monilethrix.
    • To identify the specific follicular region responsible for the observed defects.

    Main Methods:

    • Electron microscopy and electron histochemistry were employed.
    • Affected hair follicles and hair shafts were examined at high resolution.

    Main Results:

    • Structural abnormalities were localized to the 'zone of keratinization' within the hair follicle.

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  • Defects included abnormal cortical cell protein orientation, cuticle cell membrane folding, and variable endocuticular material.
  • The inner root sheath adjacent to hair shaft defects also showed abnormalities, suggesting follicular asynchrony.

    • Conclusions:

    • The 'zone of keratinization' is the primary site of structural abnormality in monilethrix hair shafts.
    • Follicular asynchrony may play a role in the pathogenesis of monilethrix.