angiokeratomas

Angiokeratomas are benign cutaneous vascular lesions characterized by dilated capillaries in the papillary dermis with overlying epidermal hyperkeratosis, producing small, dark red to blue‑black papules. They may be solitary or clustered and can occur anywhere on the body, most commonly on the lower limbs or in mucocutaneous regions such as the scrotum or vulva.

Clinical variants include angiokeratoma of Fordyce (scrotal and vulvar distribution), angiokeratoma circumscriptum naeviforme (ACN), and angiokeratoma corporis diffusum. The diffuse form is associated with Fabry disease when widespread; the circumscriptum type tends to be present from birth or early childhood as grouped plaques or papules on the extremities. Acquired angiokeratomas can appear in adults and may be related to local factors such as venous insufficiency, trauma, or radiation. Lesions are usually asymptomatic but can bleed or thicken if traumatized.

Diagnosis is primarily clinical, supported by dermoscopy showing a lacunar pattern and, if needed, histopathology. Biopsy reveals dilated, congested capillaries in the superficial dermis with an overlying thickened epidermis. In suspected Fabry disease, systemic evaluation, including enzymatic activity testing and genetic analysis, is indicated.

Treatment is typically for cosmetic reasons or symptom relief. Options include pulsed-dye laser, Nd:YAG laser, electrocautery, cryotherapy, or surgical excision. Recurrence can occur after treatment. Prognosis is excellent, with lesions remaining benign; monitoring for associated conditions is important when widespread angiokeratomas suggest an underlying systemic disorder.