What is an Angiokeratoma?

An angiokeratoma is a small raised cutaneous lesion of the skin, usually 0.04-0.12 inches (1-3 mm) in diameter, which results from damage or permanent dilation of capillaries. It usually is colored red or purple but might become black if a clot forms and lodges in the blood vessels. The cause is unknown except in the case of Fabry’s disease, a rare genetic disorder. These lesions are most common in women and appear more in those more than 40 years old. They can be removed in most cases and usually are not malignant.

Repeated friction over the site might cause the lesion to bleed and crust. It might look at first glance like melanoma. This similarity is what often brings the patient to a doctor’s office to get the lesion checked. The lesions encompass several types, mostly sporadic angiokeratoma; angiokeratoma of Fordyce; angiokeratoma circumscriptum; and angiokeratoma corporis diffusum, also called Fabry’s disease.

Sporadic angiokeratomas make up the majority of lesions seen in clinical practice. They generally form on the lower extremities in random places. They don’t need any attention unless bleeding or irritation is bothersome but might be treated for cosmetic reasons. If there is cause for concern, the lesion can be removed via excision or punch biopsy and sent to a lab for testing.

Angiokeratoma of Fordyce shows up typically on the outer genitals and the lower abdomen in both men and women. Men are more apt to be affected, especially those older than 40. Multiple lesions often appear and are larger in older patients. Hyperkeratosis — thickened, scaly skin patches — are worse over the larger lesions.

Angiokeratoma circumscriptum occurs mostly as a birthmark, although it might show up in childhood or later. It is found three times more often in females than in males. Commonly called a strawberry mark or stork bite, the red or purplish color deepens on an infant when he or she cries or is agitated. The lesions are benign and mostly appear on the trunk and legs and more rarely on the neck.

Angiokeratoma corporis diffusum, the least common of these types, is symptomatic of Fabry’s disease. Fabry’s is an inherited genetic disorder caused by a deficiency of an alpha-galactosidase enzyme, ceramide trihexosidase. Fever, pain in the hands and feet and the skin lesions are primary symptoms. The disorder causes organ damage primarily in the renal and cardiac systems and is more severe in males than in females. Enzyme replacement therapy can maintain normal function and prevent disease progression.

Not all dermal growths are a sign of disease or problematic in and of themselves. Treatments such as excision, electrocautery or cryotherapy are commonly used to remove troublesome lesions. This eliminates irritation and cosmetic concerns. Prompt medical attention should be given to any changes in existing lesions or the sudden appearance of new ones.