What Is Angiokeratoma?
Angiokeratoma is a condition in which the skin develops small, dark spots. They can appear on the body in any location. These lesions occur when tiny blood vessels called capillaries dilate or expand close to the surface of the skin.
Angiokeratomas may feel rough to the touch. They often occur in clusters on the skin around the following areas on the body:
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Labia majora.
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Vulva.
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Penis.
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Scrotum.
They might be mistaken for something like a rash, skin cancer, or certain conditions such as genital warts or herpes. However, the majority of the time, angiokeratomas are not harmful and do not require treatment.
Angiokeratomas can sometimes be a sign of some other underlying condition, like the rare genetic disorder Fabry disease (FD). Therefore, to avoid complications, an individual with angiokeratoma may need to see a doctor for treatment.
What Are the Different Types of Angiokeratoma?
There are five known types of angiokeratoma which are described as follows:
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Solitary Angiokeratoma- As the name suggests, this type of angiokeratoma often appears alone. They are most commonly found on the arms and legs and are not harmful.
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Angiokeratoma of Fordyce- This type of angiokeratoma appears on the skin of the vulva or scrotum. On the scrotum, they are most commonly found as large clusters. This can also develop on the vulva of pregnant women. However, they are not harmful but are prone to bleeding if scratched.
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Angiokeratoma of Mibelli- Dilated blood vessels closest to the epidermis, or the top layer of the skin, cause this type of angiokeratoma. They do not cause harm. However, hyperkeratosis is the process by which they tend to thicken and harden over time.
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Angiokeratoma Circumscriptum- This much rarer form of angiokeratoma appears in clusters on the legs or torso. A person can be born with this type. Over time, it tends to change appearance, becoming darker or changing to various shapes.
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Angiokeratoma Corporis Diffusum- This type of angiokeratoma is a symptom of Fabry disease (FD). It can be associated with other lysosomal disorders, affecting how cells function. Other noticeable symptoms, such as burning in the hands and feet or difficulty seeing, are associated with these uncommon conditions. The lower body is the most common location for these angiokeratomas, anywhere from the bottom portion of the torso to the upper thighs.
What Are the Symptoms of Angiokeratomas?
The exact shape, size, and color of angiokeratomas can vary from one person to another. In addition, if an associated condition, such as Fabry disease (FD), is present, affected individuals may also exhibit additional symptoms.
Angiokeratomas typically exhibit the following symptoms:
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Appear as bumps ranging in size from one millimeter (mm) to five millimeters (mm) or in wart-like, irregular patterns.
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It may have the shape of a dome.
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Appear to be hard or thick on the surface.
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Appear on their own or in small clusters of a few to nearly a hundred.
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They are dark in color, such as black, blue, red, or purple.
Recent angiokeratomas typically have a reddish appearance. However, most of the time, spots that have been on the skin for a while are darker.
Along with redness over a large portion of the scrotum, angiokeratomas on the scrotum may also appear. Scratches may also cause angiokeratomas on the scrotum or vulva to bleed more easily than those on other parts of your body.
If angiokeratoma-affected individuals also have an associated condition like FD that may be causing angiokeratomas to appear, a few other symptoms that they might experience include:
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Pain in the hands and feet (acroparesthesias).
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A ringing sound in the ears (tinnitus).
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Cloudiness in the vision (corneal opacity).
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Unable to sweat properly (hypohidrosis).
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Pain in the stomach and bowels.
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The urge to defecate after a meal.
What Causes Angiokeratoma?
The dilation of blood vessels close to the skin's surface leads to angiokeratomas. Solitary angiokeratomas are most likely the result of previous trauma to the area in question.
Angiokeratomas can be caused by Fabry disease (FD), which is passed down through families. According to the genetics department of the United States National Library of Medicine, FD affects between one in 40,000 and 60,000 men.
Angiokeratomas are associated with FD and other lysosomal conditions, but the underlying cause is not always clear. Some of the possible causes may include the following:
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High blood pressure in veins near the skin, also known as hypertension.
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A condition that affects the local blood vessels, such as hemorrhoids, inguinal hernia, or varicocele (enlargement of the veins in the scrotum).
How Is Angiokeratoma Diagnosed?
Angiokeratomas are generally harmless. It does not always need a doctor’s consultation for a diagnosis.
But if the affected individual notices other symptoms, like frequent bleeding or symptoms of FD, they must immediately see a doctor for a diagnosis and treatment. They may also want to see the doctor if they suspect a spot that looks like an angiokeratoma, as it might be cancerous.
An angiokeratoma will be diagnosed with a tissue sample taken by the doctor. A biopsy is a term for this. An angiokeratoma may be excised, or cut out, from the skin during this procedure so that it can be examined. In addition, the doctor may remove the angiokeratoma from its base beneath the skin with a scalpel during this procedure.
The doctor may also suggest a GLA gene test to rule out FD. This gene can be mutated to cause FD.
How Are Angiokeratomas Treated?
If angiokeratoma-affected individuals do not have pain or discomfort, they usually do not need to be treated. However, they may need to be removed if they frequently bleed or for cosmetic reasons. In this case, several treatment options are available:
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Electrodesiccation and Curettage (ED&C)- The doctor uses electric cautery and tools to scrape the angiokeratomas off and remove tissue after numbing the area around them with local anesthesia.
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Laser Removal- Angiokeratomas are caused by dilated blood vessels which lasers, such as a pulsed dye laser, can destroy.
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Cryotherapy- The angiokeratomas and the tissues that surround them are removed after the doctor freezes them.
Treatment for FD may involve medications such as:
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Agalsidase Beta (Fabrazyme)- Fabrazyme injections may be required on a regular basis to help the body break down extra cell fat that has built up because there is not enough of an enzyme because of GLA gene mutations.
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Neurontin (Gabapentin) or Carbamazepine (Tegretol)- These medications can be used to treat hand and foot pain.
Conclusion
Skin lesions known as angiokeratomas appear as hard bumps. The condition occurs when the skin's surface thickens, and tiny blood vessels called capillaries grow larger or break open close to the surface. As a result, red, blue, purple, or black bumps form under the skin. Angiokeratomas are a type of benign tumor that is not cancerous.
