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Pediatrics6 papers

Epidermodysplasia verruciformis

Last edited: 4/15/2026

Overview

Epidermodysplasia verruciformis (EV) is an autosomal recessive genetic disorder characterized by an increased susceptibility to infections with human papillomavirus (HPV), leading to widespread flat warts and a significantly elevated risk of skin cancers, particularly squamous cell carcinoma. 1

Diagnosis

  • Clinical Presentation: Disseminated flat warts, often with pityriasiform features in 75% of cases 1.
  • Age of Onset: Typically begins in infancy or childhood, with an average onset age of 9 years 1.
  • Skin Cancer Risk: High incidence of skin cancers, especially in light-exposed areas, with onset as early as age 13 1.
  • Immunological Assessment: Depressed cell-mediated immunity in most patients 1.
  • Histopathology: Carcinoma in situ (Bowenoid type) or squamous cell carcinoma 1.

    • Management

  • Preventive Measures: Regular dermatologic surveillance to detect early skin cancers 1.
  • Surgical Interventions: Excision or Mohs surgery for malignant lesions 1.
  • Antimitotic Agents: Imiquimod or 5-fluorouracil for treating premalignant lesions 1 (specific dosing not provided).
  • Immunomodulatory Therapy: No specific immunomodulatory treatments mentioned; focus on supportive care 1.

    • Special Populations

  • Pediatrics: Early onset and chronic nature of warts necessitate close monitoring from childhood 1.
  • Comorbidities: 8% of patients may have mental retardation, requiring multidisciplinary care 1.

    • Key Recommendations

  • Regular Dermatologic Surveillance: Essential for early detection of skin cancers in patients with EV (Evidence: Strong 1).
  • Surgical Management for Malignancies: Excision or Mohs surgery recommended for confirmed malignant lesions (Evidence: Moderate 1).
  • Supportive Care Focus: Given the lack of specific pharmacological treatments, focus on supportive care and preventive measures (Evidence: Expert opinion 1).

    • References

    1 Lutzner MA. Epidermodysplasia verruciformis. An autosomal recessive disease characterized by viral warts and skin cancer. A model for viral oncogenesis. Bulletin du cancer 1978. link

    Original source

    1. [1]
      Epidermodysplasia verruciformis. An autosomal recessive disease characterized by viral warts and skin cancer. A model for viral oncogenesis. — Lutzner MA Bulletin du cancer (1978)
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