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Related Experiment Videos

Ichthyosis with laminated membrane structures.

K M Niemi1, L Kanerva

  • 1Department of Dermatology, Helsinki University Central Hospital, Finland.

The American Journal of Dermatopathology
|April 1, 1989
PubMed
Summary

This study describes a new ichthyosis subtype, characterized by laminated membrane structures in the epidermis. This rare skin disorder, ichthyosis with laminated membrane structures, presents unique electron microscopic findings.

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Area of Science:

  • Dermatology
  • Histopathology
  • Genetics

Background:

  • Ichthyosis encompasses a group of genetic skin disorders characterized by abnormal keratinization.
  • Lamellar ichthyosis is a severe subtype with widespread scaling.
  • This study investigates a novel ichthyosis variant with distinct clinical and ultrastructural features.

Observation:

  • A 75-year-old woman presented with ichthyosis since childhood, progressing to hyperkeratosis on the neck and axillae.
  • Affected skin exhibited dryness, scaling, thickening, and folding, with other areas appearing shiny and red.
  • Physical development was normal despite the chronic skin condition.

Findings:

  • Light microscopy revealed acanthosis, papillomatosis, hyperkeratosis, and vacuolated granular cells.

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  • Electron microscopy identified diagnostic concentric or parallel lamellar membrane structures and amorphous material in upper epidermal cells.
  • Abnormal keratinosomes and lamellar material in cornified cells suggest a defect in keratinosome formation impacting desquamation.

    • Implications:

    • The findings define a new subtype of lamellar ichthyosis, termed ichthyosis with laminated membrane structures.
    • Electron microscopy is crucial for diagnosing this specific ichthyosis variant.
    • Further research is needed to elucidate the genetic basis and inheritance pattern of this condition.