Angiokeratomas (Fordyce Angiokeratomas)
The small vascular lesions on the scrotum are called angiokeratomas, specifically angiokeratomas of Fordyce, which are benign cutaneous vascular lesions caused by weakening of the capillary wall and vascular ectasia within the epidermis and dermis. 1
Clinical Characteristics
Appearance and Distribution:
- These lesions initially appear as small, slightly raised, purplish-red, non-blanching angiectases 1
- They typically manifest between 5 and 13 years of age 1
- With age, there is progressive increase in the number and size of these lesions 1
- The hips, back, thighs, buttocks, penis, and scrotum are commonly affected, as well as the oral mucosa and conjunctiva 1
- Most lesions are 1-5 mm in size, though larger lesions can occur 2
- They present as purple, red, and/or blue papules, with 70% displaying more than one color 2
Clinical Significance:
- Angiokeratomas are one of the earliest clinical signs of Fabry disease and may be seen in both genders 1
- Among female carriers, frequencies of occurrence up to 35% have been reported 1
- The large genital lesions are particularly susceptible to thrombosis and bleeding 1
- Nearly half of cases may cause symptoms like itching and bleeding 3
Histopathology
Microscopic Features:
- Dilated, thin-walled vascular structures in the upper portion of the dermis 4
- Overlying acanthosis and/or hyperkeratosis 4, 5
- Ectatic blood vessels in the papillary dermis 4
Important Clinical Pitfall
When encountering scrotal angiokeratomas, you must consider Fabry disease as a potential underlying etiology. Fabry disease is a systemic lysosomal storage disorder where angiokeratomas serve as an early clinical marker, and missing this diagnosis can delay treatment of a progressive multisystem disease affecting the kidneys, heart, and nervous system. 1
Treatment Considerations
Treatment Options:
- Treatment is not always necessary but is primarily considered for cosmetic reasons, bleeding, or patient embarrassment 3
- Laser therapy (in 56% of treated cases) is the most common treatment modality 2
- Specific laser options include Long-Pulse Alexandrite Laser, variable pulse width 532-nm Neodymium, and flash lamp-pumped dye laser 1, 3
- Less common interventions include electrocautery, radiofrequency, and excision 2
- Results of various laser methods have been mixed or discouraging, and treatment does not prevent formation of new lesions 1
- More pedunculated lesions may be treated with a series of liquid nitrogen treatments prior to laser therapy 1
- Many patients (27%) decide to observe their angiokeratomas without treatment 2
Treatment Outcomes: