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Familial severe twenty-nail dystrophy

A M Arias, C W Yung, S Rendler

    Journal of the American Academy of Dermatology
    |September 1, 1982
    PubMed
    Summary
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    Severe nail dystrophy, a disorder affecting nails, can be inherited. This study identifies a new autosomal dominant trait, "familial severe twenty-nail dystrophy," in a large family across five generations.

    Area of Science:

    • Dermatology
    • Genetics
    • Ophthalmology

    Background:

    • Severe nail dystrophy is an acquired nail disorder with variable presentations including thinning, thickening, pitting, and opalescence.
    • While often reported in children with gradual resolution, adult cases and persistent symptoms are documented.
    • Nail changes can range from mild to severe, with some nails potentially unaffected.

    Observation:

    • A five-generation pedigree was studied, revealing 21 out of 37 members affected by dystrophic nail changes.
    • The familial pattern of the disorder was observed to be consistent across multiple generations.
    • The inheritance pattern strongly suggested an autosomal dominant trait.

    Findings:

    • The study identified a novel autosomal dominant inherited pattern of severe nail dystrophy.

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  • This represents the first reported instance of a familial inheritance pattern for this specific nail disorder.
  • The proposed term for this familial condition is "familial severe twenty-nail dystrophy".

    • Implications:

    • This discovery provides crucial insights into the genetic basis of severe nail dystrophy.
    • Understanding the genetic transmission can aid in diagnosis and genetic counseling for affected families.
    • Further research into the specific genes involved may uncover new therapeutic targets.