What is the treatment for Epidermodysplasia verruciformis?

Treatment of Epidermodysplasia Verruciformis

Wide local excision is the treatment of choice for squamous cell carcinomas (SCCs) that develop in epidermodysplasia verruciformis (EV), while a combination of surgical and non-surgical approaches is recommended for non-malignant EV lesions. 1

Understanding Epidermodysplasia Verruciformis

Epidermodysplasia verruciformis is a rare genodermatosis characterized by:

• Persistent human papillomavirus (HPV) infection
• Polymorphic, disseminated skin lesions
• High risk of malignant transformation (approximately 50% of patients)
• Typically presents with flat, wart-like lesions, scaly hypo- and hyperpigmented macules/patches

Surveillance and Early Detection

Regular monitoring is essential due to high risk of malignant transformation:

• Regular clinical surveillance for suspicious lesions 1
• Biopsy of any suspicious areas for histological evaluation 1
• Patient education about clinical features that might indicate malignancy
• More frequent monitoring (every 3 months) for patients with history of SCC 1

Warning Signs Requiring Biopsy

• Non-healing wounds
• Rapid growth of lesions
• Change in appearance of existing lesions
• Pain or bleeding

Treatment Algorithm for EV

1. For Malignant Lesions (SCC)

Primary Treatment:

• Wide local excision is the gold standard treatment 1
• Ensure complete removal with adequate margins
• Multidisciplinary review for all EV patients presenting with SCC 1

For Advanced Cases:

• When excision is not possible, amputation of digit or limb may be necessary 1
• Regional lymph node dissection if nodal SCC is identified 1
• Staging may be required for proper management 1
• Imaging (MRI or CT) for tumors ≥5cm or in difficult anatomical sites 1

Adjunctive Therapy:

• Radiotherapy may be useful as palliative treatment for inoperable SCCs or metastases 1
• Note: Radiotherapy should be delivered in smaller fractions to minimize risk of severe skin desquamation 1
• EGFR antagonists and tyrosine kinase inhibitors may provide palliation in advanced cases 1

2. For Non-Malignant EV Lesions

Surgical Options:

• Curettage followed by application of 35% trichloroacetic acid has shown good cosmetic results with low recurrence rates 2
• Complete surgical excision for larger or symptomatic lesions 1

Non-Surgical Options:

• Photodynamic therapy (PDT):
  • 5-aminolevulinic acid PDT has shown promising results 3, 4
  • Can be used alone or in combination with other treatments
  • Particularly useful for facial lesions where cosmetic outcome is important

Combination Approaches:

• Combined PDT and oral retinoids have demonstrated excellent clinical outcomes with long-lasting remission 5
• Combined surgery and PDT for refractory cases 3

Important Considerations

Sun Protection

• Strict sun protection is essential as UV exposure increases risk of malignant transformation 6
• High SPF sunscreen use is recommended 1
• Avoidance of outdoor activities during peak sun hours

Follow-up Care

• Regular follow-up examinations (every 3 months for patients with history of SCC) 1
• Lymph node assessment for potential metastatic SCC 1
• Patient education about self-examination and warning signs

Complications and Pitfalls

• Incomplete removal of lesions may lead to recurrence
• Patients who have received radiotherapy previously may experience earlier malignant transformation of EV lesions 6
• No current treatment provides permanent cure, so ongoing management is necessary

End-of-Life Care

For patients with advanced disease:

• Appropriate analgesia should be prescribed 1
• Psychological support for both patient and family/caregivers is vital 1