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Proteus syndrome: association with gingival hyperplasia

T M Arendorf1, B Hanslo

  • 1Oral Health Centre, Faculty of Dentistry, University of the Western Cape, Cape Town, South Africa.

Journal of Oral Pathology & Medicine : Official Publication of the International Association of Oral Pathologists and the American Academy of Oral Pathology
|September 1, 1995
PubMed
Summary

This case report details a 9-year-old boy with Proteus syndrome, experiencing gigantism and recurrent gingival hyperplasia. It highlights the oral manifestations of this rare genetic disorder.

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Area of Science:

  • Medical Genetics
  • Oral Pathology
  • Dermatology

Background:

  • Proteus syndrome is a rare congenital disorder characterized by segmental overgrowth.
  • Oral manifestations are not well-documented in existing literature.
  • Gingival hyperplasia is a potential, though rarely reported, feature.

Observation:

  • A 9-year-old Black male presented with significant gigantism of hands and feet.
  • The patient also exhibited recurrent episodes of gingival hyperplasia.
  • Diagnosis was confirmed as Proteus syndrome.

Findings:

  • This case represents the first documented instance of gingival hyperplasia associated with Proteus syndrome.
  • Detailed description of the oral manifestations observed in this patient.

Related Experiment Videos

  • Highlights the phenotypic variability of Proteus syndrome.

    • Implications:

    • Suggests gingival hyperplasia should be considered in the oral examination of Proteus syndrome patients.
    • Contributes to a more comprehensive understanding of Proteus syndrome's clinical spectrum.
    • Emphasizes the need for further research into the oral manifestations of rare genetic disorders.