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Related Experiment Videos

Epidermodysplasia verruciformis accompanied by familial large granular lymphocytosis and a decrease in T lymphocytes

H Aoyama1, S Seki, T Abo

  • 1Department of Dermatology, School of Medicine, Tohoku University, Sendai, Japan.

Acta Dermato-Venereologica
|February 1, 1993
PubMed
Summary
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Epidermodysplasia verruciformis may be linked to inherited large granular lymphocyte expansion and reduced T cells. These blood abnormalities were observed in affected individuals and their relatives, suggesting a genetic component.

Area of Science:

  • Immunology
  • Genetics
  • Dermatology

Background:

  • Epidermodysplasia verruciformis (EV) is a rare genetic disorder characterized by persistent human papillomavirus infections and a high risk of skin cancer.
  • The immunological underpinnings of EV are not fully understood, particularly concerning lymphocyte subsets.

Observation:

  • A 40-year-old male patient with EV presented with decreased peripheral blood T cells and an abnormal expansion of large granular lymphocytes (LGLs).
  • Increased natural killer (NK) cell activity and a specific LGL subset (CD 57+ and CD 16+) were noted in the patient.
  • Similar hematological findings, including decreased T cells and increased CD 16+ NK cells, were observed in family members, even those without EV skin lesions.

Findings:

  • The study identified a potential association between epidermodysplasia verruciformis and a hereditary predisposition to abnormal large granular lymphocyte expansion.

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  • A decrease in T cells was a consistent finding across affected and some unaffected family members.

    • Implications:

    • These findings suggest a possible genetic link between EV and specific immune system dysregulations, particularly involving LGLs and T cells.
    • Further research into the genetic and immunological basis of EV could reveal novel diagnostic markers or therapeutic targets.