What is the treatment for Elephantiasis nostras verrucosa?

Treatment of Elephantiasis Nostras Verrucosa

The treatment of Elephantiasis Nostras Verrucosa (ENV) should begin with surgical debridement of verrucous lesions, followed by compression therapy to manage the underlying lymphedema.

Understanding ENV

Elephantiasis nostras verrucosa is a rare clinical condition characterized by:

• Chronic non-filarial lymphedema
• Hyperkeratotic, verrucous, and papillomatous skin lesions
• Progressive dermal fibrosis
• Significant enlargement of affected body parts

Unlike elephantiasis tropica (caused by filarial parasites), ENV results from chronic secondary lymphedema due to non-filarial causes including:

• Recurrent infections (particularly streptococcal)
• Surgery
• Radiation
• Tumor obstruction
• Congestive heart failure
• Obesity

Diagnostic Approach

When evaluating a patient with suspected ENV:

• Examine for characteristic cobblestone-like verrucous changes
• Assess for non-pitting edema in affected areas
• Look for malodorous hyperkeratosis with lichenification
• Consider skin biopsy to confirm diagnosis (will show pseudoepitheliomatous hyperplasia with dilated lymphatic spaces in the dermis)
• Rule out filarial infection through serology and nocturnal blood microscopy if travel history to endemic areas

Treatment Algorithm

First-Line Treatment

1. Surgical debridement of verrucous lesions

   • Shaving the verrucous area with a blade followed by abrading using a motor-powered grinder 1
   • Complete reepithelization typically occurs within 2 weeks
   • Provides rapid and aesthetically acceptable results

2. Management of underlying lymphedema

   • Compression therapy with stockings or bandages
   • Elevation of affected limbs
   • Lymphatic massage/manual lymphatic drainage
   • Pneumatic compression devices for refractory cases 2

3. Infection control

   • Treat any secondary bacterial infections
   • Prophylactic antibiotics may be needed for recurrent infections
   • Debride any eschar overlying ulcers before administering local therapy 3

Additional Therapeutic Options

• Topical therapy:

  • Low to moderate potency topical corticosteroids for inflammation
  • Urea-containing (5-10%) moisturizers to repair skin barrier
  • Avoid frequent washing with hot water and skin irritants

• Systemic therapy:

  • Oral retinoids (e.g., Acitretin 30 mg/day) may be considered but have variable results and require monitoring for side effects such as elevated liver enzymes 4

Special Considerations

• Severe cases: In advanced cases with complications such as chronic osteomyelitis, more aggressive surgical approaches including amputation may be necessary 5

• Monitoring: Regular follow-up is essential to:

  • Assess treatment response
  • Monitor for recurrence (which is common without addressing underlying lymphedema)
  • Detect early signs of complications

Prognosis and Complications

• Without proper management, ENV tends to progress and worsen over time
• Complications include:
  • Secondary infections
  • Chronic ulcerations
  • Decreased mobility and quality of life
  • Psychological distress due to disfigurement

Treatment Pitfalls to Avoid

1. Focusing only on skin manifestations: Treating only the verrucous lesions without addressing the underlying lymphedema will lead to recurrence

2. Delaying treatment: Early intervention is crucial to prevent progression to more severe, difficult-to-treat disease

3. Inadequate infection control: Recurrent infections can worsen lymphedema and accelerate disease progression

4. Neglecting patient education: Patients need to understand the chronic nature of the condition and the importance of long-term compression therapy and skin care

Remember that while surgical debridement provides good cosmetic results, it treats only the cutaneous manifestations. Long-term management of the underlying lymphedema is essential for preventing recurrence and improving quality of life.