Epidermodysplasia verruciformis is a rare genetic dermatological condition characterized by a compromised immune ability to defend against and eliminate certain types of human papillomavirus (HPV). This leads to persistent HPV infections and a significantly increased lifetime risk of developing cutaneous dysplasia and malignancy. Individuals with this condition develop HPV-derived cutaneous lesions at a significantly higher rate than the general population. This genodermatosis primarily manifests as verrucous cutaneous lesions, including multiple persistent verrucae, pityriasis versicolor-like lesions, and other verrucous or “warty” lesions. This can also lead to the development of Bowen disease and squamous cell carcinoma. The lesions associated with epidermodysplasia verruciformis tend to exhibit a characteristic histopathologic appearance. No definitive treatment exists for epidermodysplasia verruciformis. However, several potential options are recommended, including excision, acitretin, imiquimod, interferons with retinoids, and topical calcipotriol. The efficacy of cimetidine is debated. As the lesions commonly occur in sun-exposed areas, counseling on sun protection and adherence to preventive measures are essential for managing the condition. This activity reviews the epidemiology, genetics, clinical presentation, and management of epidermodysplasia verruciformis, providing insights into current and emerging treatment options, including antiviral therapies, immunomodulators, and surgical interventions. This activity also highlights the role of an interprofessional healthcare team in improving patient care through regular monitoring and early intervention to manage complications and reduce cancer risk.
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