What is Fordyce angiokeratoma and how is it treated?

Fordyce Angiokeratoma: Definition and Treatment

Fordyce angiokeratoma (angiokeratoma of Fordyce) consists of benign vascular lesions presenting as dark red to blue-black papules with a scaly surface, most commonly located on the scrotum in males and labia majora in females, and when symptomatic or cosmetically concerning, pulsed dye laser (PDL) or long-pulsed Nd:YAG laser are the preferred treatment options. 1, 2, 3

Clinical Presentation and Pathophysiology

Fordyce angiokeratomas are benign vascular tumors characterized by:

• Small, slightly raised, purplish-red to blue-black, non-blanching papules that range from pinhead size to several millimeters 4
• Progressive increase in number and size with age, typically becoming more prominent over time 4
• Common locations: scrotum, penis, labia majora, and pubic area 1, 3, 5
• Pathophysiology: caused by weakening of capillary walls and vascular ectasia (dilation) within the epidermis and dermis 4
• Histology: marked dilation of papillary dermal vessels forming large, blood-filled cavernous channels 5

Clinical Significance

• Usually asymptomatic but may cause patient anxiety and social embarrassment 1, 3
• Bleeding risk: lesions may bleed spontaneously or after mechanical trauma, including sexual intercourse 1, 3
• Large genital lesions are particularly susceptible to thrombosis and bleeding 4
• Incidence increases with age, affecting approximately half of older males 6

Important Differential Diagnosis

Must differentiate from malignant melanoma, angiosarcoma, and other pigmented lesions, particularly when lesions are dark or irregular 3

Treatment Approach

When to Treat

Treatment is indicated for:

• Symptomatic lesions (bleeding, itching, pain) 6
• Cosmetic concerns causing patient distress 1, 3, 6
• Recurrent bleeding after trauma 1, 3

Asymptomatic lesions do not require treatment as they are benign 1

First-Line Treatment: Laser Therapy

Pulsed Dye Laser (PDL)

PDL at 585-595 nm is the preferred first-line treatment for Fordyce angiokeratoma, offering excellent efficacy with minimal side effects:

• Parameters: 5.5-8.0 J/cm² fluence, variable pulse width, with Dynamic Cooling Device (DCD) 1, 3
• Treatment sessions: 1-7 sessions (mean 3.38 sessions), with intervals of 1-3 months 3
• Efficacy:
  • 58% of patients achieve excellent response (75-100% clearance) 1
  • 42% achieve good response (50-75% clearance) 1
  • Successful removal in all treated patients in one study 3
• Side effects: transient purpura and pain in all patients, bleeding during treatment in 42% of cases, but no permanent complications, scarring, or dyspigmentation 1, 3
• Recurrence: observed in 17% of patients after 0.5-1 year 3

Long-Pulsed Nd:YAG Laser

Long-pulsed Nd:YAG laser (1064 nm) is an excellent alternative, particularly for darker skin types:

• Parameters: 90-130 J/cm² energy, 10-20 ms pulse duration, 5-7 mm spot size 2
• Clinical endpoint: lesions should shrink and turn pale immediately after irradiation 2
• Treatment interval: minimum 8 weeks between sessions 2
• Efficacy: 82% cured, 18% improved, with mean 2.2 treatment sessions 2
• Advantages: simple, rapid, no bleeding during treatment, no scar formation 2
• Patient satisfaction: 100% in reported series 2

Alternative Treatment Options

When laser therapy is unavailable or contraindicated:

Cryotherapy

• Widely used method with good efficacy 6
• Advantages: readily available, lower cost 6
• Disadvantages: may cause more discomfort during healing, potential for scarring 6

Electrocauterization

• Effective for symptomatic lesions 6
• Considerations: may cause more pain and longer recovery compared to laser 6
• Risk of bleeding and wound infection during procedure 6

Radiofrequency Ablation

• Effective for removal of multiple lesions 5
• Used successfully for extensive vulvar involvement 5

Surgical Excision

• Reserved for large, pedunculated lesions or when histological confirmation is needed 4
• More invasive with higher risk of scarring 6

Treatment Algorithm

1. Confirm diagnosis clinically (dark red to blue-black papules on genital skin with scaly surface) 1, 3, 5
2. If diagnostic uncertainty exists (concern for melanoma or angiosarcoma), obtain biopsy before treatment 3, 5
3. For isolated or few lesions:
   • First choice: 595-nm PDL with DCD (3-4 sessions expected) 3
   • Alternative: Long-pulsed Nd:YAG laser (2-3 sessions expected) 2
4. For multiple extensive lesions:
   • Long-pulsed Nd:YAG laser may be more efficient due to no bleeding during treatment 2
   • Consider radiofrequency ablation 5
5. If laser unavailable: cryotherapy or electrocauterization 6
6. Follow-up at 6-12 months to monitor for recurrence 3

Critical Pitfalls to Avoid

• Do not confuse with Fabry disease angiokeratomas, which are systemic manifestations of α-galactosidase A deficiency, appear earlier (age 5-13), and require enzyme replacement therapy rather than local treatment 4
• Fabry disease angiokeratomas typically involve hips, back, thighs, buttocks, and oral mucosa, and are associated with acroparesthesias, cornea verticillata, and renal/cardiac disease 4
• Always rule out malignancy when lesions are atypical, rapidly growing, or irregularly pigmented 3
• Avoid aggressive ablative treatments that may cause genital scarring and decreased patient satisfaction 6
• Counsel patients about recurrence risk (up to 17%) and need for potential retreatment 3
• Warn about transient purpura lasting several days after PDL treatment to manage expectations 1, 3