Epidermodysplasia Verruciformis: A Case Series

Area of Science:

• Dermatology
• Genetics
• Oncology

Background:

• Epidermodysplasia verruciformis (EV) is an uncommon autosomal recessive genodermatosis.
• EV confers extreme susceptibility to specific human papillomavirus (HPV) types.
• This predisposition significantly elevates the risk of non-melanoma skin cancers, particularly in sun-exposed regions.

Observation:

• This case series presents three patients with Epidermodysplasia verruciformis.
• Presentations varied, including a long-standing case with vulvar and inguinal squamous cell carcinoma (SCC), a rare association with palmar pits, and a classical EV phenotype.
• Histopathology confirmed EV in all cases.

Findings:

• The study highlights the diverse clinical manifestations of Epidermodysplasia verruciformis.
• It underscores the critical link between EV, HPV infection, and the development of skin malignancies like SCC.
• The association with palmar pits is noted as a rare finding.

Implications:

• Early diagnosis and management of EV are crucial due to cancer risks.
• Stringent photoprotection and regular dermatological surveillance are essential for EV patients.
• Treatment with oral retinoids may be beneficial, alongside sun avoidance strategies.