!Epidermodysplasia verruciformis, tree man syndrome or Lewandowsky-Lutz dysplasia

  • AKA tree man syndrome or Lewandowsky-Lutz dysplasia.
  • Etiology
    • Rare genetic disorder
      • Autossómica recessiva (++ em consanguinidade)
      • Mutações EVER1 and EVER2 → encode transmembrane proteins that play an important role in zinc homeostasis
        • O Zinco nuclear que é um cofator importante que facilita a replicação viral
        • Mais zinco nuclear → aumenta a suscetibilidade acrescida ao HPV.
        • Increased the expression of pro-oncogenic viral genes E6 and E7
    • Acquired Epidermolysis verruciformis in HIV and kidney transplant recipients
  • Mechanism
    • Selective immunodeficiency to human papillomavirus (HPV) infections
      • May also occur in the setting of immunosuppression, due to cell mediated immunity
    • Selectivity: only certain HPV types associated with this syndrome:
      • HPV: 3, 5, 8, 9, 12, 14, 15, 17, 19
      • Some have oncogenic potential
  • Clinical presentation
    • Multiple verrucous lesions in infancy or early childhood
    • Lesions can be extensive and prone to infection
    • Early actinic keratoses and cutaneous cell carcinomas