Angiokeratoma

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What Is Angiokeratoma?

In this article we will look at the angiokeratoma. We will take a brief look at what angiokeratoma is and the different types of angiokeratomas that exist.

What is angiokeratoma?

Angiokeratomas are also known as AKs and they are a type of benign tumor / nevus (pl. nevi). Usually, they occur in childhood and disappear spontaneously by adulthood. These “spots” are caused by an overproduction of blood vessels in the skin. They are usually pink or purple in color.

This skin condition is present at birth (congenital) in some cases. Sometimes it is also caused by injury later in life or it can even be caused by an allergic reaction to something. Despite their name, angiokeratomas are not actually tumors. They are just areas of increased blood vessel formation in the top layer of the skin.

People usually have between 2 and 20 of these spots. They can occur anywhere on the body but are most common on the legs or arms. They can be solitary or multiple and can vary in size from very small to up to several inches across.

Angiokeratomas do not usually cause any symptoms and they are of no threat to health. They can be unsightly and some people may want them removed by a doctor for cosmetic reasons.

In some cases, if the AKs are large, they can cause problems with movement or become damaged by trauma (e.g. a bad fall). These very large angiokeratomas are called angiosarcomas.

These are not benign tumors and can be very dangerous because they can form blood clots that can travel in the blood and cause life-threatening problems elsewhere (e.g. the brain). For this reason, any angiokeratomas larger than 1cm should be investigated to rule out angiosarcoma.

Families tend to have similar appearances and sometimes the same kinds of nevi. Some people have a genetic mutation that causes angiokeratomas to appear spontaneously. In other cases, angiokeratomas run in families.

There is some suspicion that angiokeratomas may be linked to thyroid disease and other autoimmune diseases.

What are the different types of angiokeratoma?

There are 2 main types of angiokeratomas: solitary and recurrent. Sometimes these types can overlap though.

Solitary angiokeratomas are AKs that only ever appear in one place on the skin. They are usually larger than recurrent angiokeratomas and have varied shapes and colors. They are caused by genetic changes.

Recurrent angiokeratomas are clusters of several angiokeratomas that keep appearing in the same place(s) on the skin no matter how many times they are removed or how much the skin is healing in between. They are caused by genetic and environmental factors.

I also wanted to briefly mention angiokeratoma corporis diffusum (AKD). This is a rare condition in which AKs cover a large part of the body. It is a very severe case of recurrent angiokeratomas.

This skin condition can be very harmful to people with the condition. Children may have trouble walking due to the size and number of spots on the legs and feet. They may also have problems with skin infections due to difficulty of healing properly. This condition is a lot more than just an inconvenience – it can actually be life-threatening.

For this reason, people with AKD often have to have many operations on their legs to remove the AKs.

Who gets angiokeratomas?

Anyone can get angiokeratomas. They occur more frequently in young children and adolescents than in adults though. They affect both males and females more or less equally.

There are certain things that make a person more likely to get angiokeratomas though.

Certain skin disorders such as dermatofibromas and molluscum contagiosum are both associated with angiokeratomas. This tells us that there is probably a genetic cause.

Other things that may trigger angiokeratomas include:

* Exposure to the sun (especially tanning beds)

* Previous cases of chicken pox

* Skin damage caused by heat and injury, e.g. burns and scalds

Where do angiokeratomas appear on the body?

Angiokeratomas can show up anywhere on the body, but they are most likely to appear on the trunk, neck and limbs. They can be itchy or painful but not always.

Angiokeratoma Treatment

Unfortunately there is no known cure for angiokeratomas. The best treatment is simply to remove the affected area of skin by cutting it out. Then the wound must be allowed to heal properly. It can take a long time for AKs to disappear completely and even then there may be a pale white scar.

If the AK has appeared on an area of the body that can’t easily be cut out, such as the eyelids or inside the ear, a type of laser treatment may be used to destroy the affected skin cells.

A very rare type of angiokeratoma known as angiokeratoma corporis continous occurs all over the body from birth. This condition is associated with other developmental problems too.

AKs are very easy to treat and usually heal quickly with no after effects. They can be a little unsightly but they don’t cause any real problems.

You should keep an eye on any angiokeratomas that you have and get them checked out by a doctor if they change in size, shape or color.

Sources & references used in this article:

Angiokeratoma: a clinicopathological study by R Imperial, EB Helwig – Archives of Dermatology, 1967 – jamanetwork.com

Angiokeratoma corporis diffusum by RB PITTELKOW, RR KIERLAND… – AMA archives of …, 1955 – jamanetwork.com

The genetics of angiokeratoma corporis diffusum (Fabry’s disease) and its linkage relations with the Xg locus by JM Opitz, FC Stiles, D Wise, RR Race… – American journal of …, 1965 – ncbi.nlm.nih.gov

Angiokeratoma of the scrotum (Fordyce type) by R Imperial, EB Helwig – The Journal of urology, 1967 – auajournals.org

The renal lesion in angiokeratoma corporis diffusum by JR Colley, DL Miller, MSR Hutt, HJ Wallace… – British medical …, 1958 – ncbi.nlm.nih.gov

Angiokeratoma circumscriptum by PJ Lynch, M Kosanovich – Archives of Dermatology, 1967 – jamanetwork.com