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[Kindler syndrome. Case report with ultrastructure study].

J L Verret, M Avenel, M Larrègue

    Annales De Dermatologie Et De Venereologie
    |January 1, 1984
    PubMed
    Summary
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    Kindler syndrome is a rare genetic disorder characterized by skin atrophy, blisters, and keratosis. This case report details a patient

    Area of Science:

    • Dermatology
    • Genetics
    • Pathology

    Background:

    • Kindler syndrome is a rare genodermatosis.
    • It presents with progressive poikiloderma, cutaneous atrophy, and blistering.

    Observation:

    • A 20-year-old male presented with progressive poikiloderma, cutaneous atrophy, plantar keratosis, and infantile-onset blistering.
    • Ultrastructural analysis revealed cleavage between the lamina densa and basal cell membrane in affected skin lesions.

    Findings:

    • The patient's presentation and ultrastructural findings align with Kindler syndrome.
    • Review of 60 literature cases identified 13 similar to the original Kindler description, suggesting a distinct entity.

    Implications:

    • This case reinforces Kindler syndrome as a unique genodermatosis.

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  • Understanding the ultrastructural pathology aids in diagnosis and classification.
  • Further research into the genetic basis and inheritance patterns is warranted.