What is Angiokeratoma Circumscriptum?
Angiokeratomas are a group of disorders related to harmless blood vessel abnormalities in the upper layer of the skin, known as the papillary dermis. These abnormalities are associated with excessive skin growth, thickening, or bulging, also known as hyperkeratosis, papillomatosis, and acanthosis. There are five different ways angiokeratomas can show up, including isolated or multiple angiokeratomas, angiokeratoma of Mibelli, angiokeratoma of Fordyce, widespread angiokeratoma, and angiokeratoma circumscriptum.
Among these, angiokeratoma circumscriptum is the rarest form. Usually, it first shows up at birth as reddish skin spots. Over time these spots can turn into dark red to bluish nodules or patches with rough, warty, or excessively thickened skin features. Although these usually appear on one side of the lower body parts, they can also be found on the trunk, neck, scrotum, and tongue.
What Causes Angiokeratoma Circumscriptum?
The exact cause of angiokeratoma circumscriptum, a condition where capillaries in your skin enlarge, is not known. Some believe that certain factors might trigger its development. These factors could include changes in blood flow, long-term physical harm, lack of oxygen in tissue, inborn factors, pregnancy, or chilblains, a condition affecting skin after exposure to cold.
Epidermal changes (changes to the skin’s outer layer) are a reaction to these triggers. These changes get more pronounced as a person ages and they are happening after blood vessel enlargement. This condition is harmless and is not usually associated with diseases affecting the entire body. However, in rare cases, it has been connected with Klippel-Trenauney syndrome and Cobbs syndrome, which are disorders affecting the veins and skin.
Risk Factors and Frequency for Angiokeratoma Circumscriptum
Angiokeratoma circumscriptum is mainly a condition that people are born with, although some people can develop it later in life. It affects people of all racial backgrounds. While angiokeratomas in general are slightly more common in males, it has been observed that Angiokeratoma circumscriptum is three times more likely to occur in females.
Signs and Symptoms of Angiokeratoma Circumscriptum
Angiokeratoma circumscriptum is a skin condition that usually affects one lower body part, often the buttocks or thigh. It usually shows up at birth as multiple red spots. Over time, these spots can evolve into dark red or purple, rough bumps that might join together to form patches. These patches could be as small as a few centimeters or as big as covering a quarter of the body’s skin. Patients might experience occasional bleeding and discomfort with minor trauma.
During a physical examination, the affected areas typically feel tender, well-defined, firm, and do not pulse or compress when touched. Generally, angiokeratoma circumscriptum does not fade or disappear naturally over time.
Sometimes, angiokeratoma circumscriptum is associated with Klippel-Trenaunay syndrome (KTS), a condition typically characterized by abnormal blood vessels, a port-wine stain birthmark, and overgrown bones and soft tissues. In rare cases, instead of the port wine stain, experts have observed angiokeratoma circumscriptum.
Angiokeratoma circumscriptum can also be correlated with several other conditions such as Cobb Syndrome, nevus flammus, cavernous hemangioma, and traumatic arteriovenous fistula.
Testing for Angiokeratoma Circumscriptum
Angiokeratoma circumscriptum is a condition often identified based on its signs and symptoms and later confirmed by tissue examination. This condition is characterized by irregularly dilated blood vessels in the uppermost layer of the skin, along with an abnormal increase in skin cells which causes the skin to thicken. It’s common to find blood clots in these blood vessels, which can be identified by finding large amounts of red blood cells upon tissue examination.
During a physical check-up, a patch of angiokeratoma circumscriptum might look like melanoma, a type of skin cancer, especially when blood vessels start to form clots, which cause the skin patches to darken.
It’s important to note that the diagnosis of angiokeratoma circumscriptum can be made only when the deeper layers of the skin and the layer beneath the skin, known as the subcutaneous tissue, are uninvolved. If these deeper layers show involvement, then it’s likely that the actual condition is verrucous hemangioma, which is a rare form of non-cancerous skin tumor.
An ultrasound of angiokeratoma circumscriptum would likely show a linear-shaped, low-density soft-tissue mass that only has limited vascular supply and doesn’t involve the bone beneath it.
Most of the time, further investigation isn’t needed. But, if lots of lesions are present in the lower back or buttock area that lies over the spine of a newborn or an infant, an MRI scan of the spine would be required to rule out conditions like spinal dysraphism or Cobb syndrome, both of which involve the improper formation of the spinal cord during embryonic development.
Treatment Options for Angiokeratoma Circumscriptum
If you have a skin condition known as angiokeratoma circumscriptum, there might come a time when it needs to be treated. You might choose to seek treatment for cosmetic reasons or due to recurring bleeding.
For smaller areas affected by this condition, doctors can use different methods including heat treatment (diathermy), scraping (curettage) and burning (electrocautery), freezing (cryosurgery), or cutting it out (surgical excision). But for larger areas, these methods may cause too much damage to the appearance of your skin. In such cases, laser treatment is often the best option.
Several different types of lasers can be used to treat angiokeratoma circumscriptum, including argon lasers, carbon dioxide lasers, Cooper vapor lasers, neodymium-doped yttrium-aluminum-garnet (Nd:YAG) lasers, pulsed-dye lasers, and intense pulsed light source systems. The treatment usually starts with a laser (like carbon dioxide or erbium lasers) that removes the thickened outer layer of skin. After that, a laser that targets blood (like the potassium titanyl phosphate (KTP) or pulsed dye laser) is used. Benefits of the carbon dioxide laser treatment include precise control, quick procedure, blood control, and limited pain and swelling after the procedure.
Not all laser treatments are the same though. Some lasers like the pulsed-dye laser, Cooper vapor laser, pulsed argon laser, and Nd:YAG laser are non-ablative. This means they don’t remove any skin and usually require several treatment sessions to achieve full clearance of the affected skin. These lasers sometimes struggle to penetrate the thickened outer skin layer that angikokeratoma circumscriptum can cause.
On the other hand, ablative lasers like the carbon dioxide laser and continuous-wave argon lasers do remove the outer skin layer and can reach the affected area in one pass. However, these types of lasers are more likely to cause texture and color changes in the skin, though this risk can be minimized with careful technique, informing the patients correctly, and providing quality care after the procedure.
What else can Angiokeratoma Circumscriptum be?
Doctors need to rule out the following conditions when trying to diagnose a skin condition:
- Verruous hemangioma (a type of skin growth)
- Melanocytic nevus (commonly known as a mole)
- Melanoma (a type of skin cancer)
- Lymphangioma circumscriptans (a skin lesion)
- Capillary aneurysm (a bulging blood vessel)
- Pigmented basal cell carcinoma (a type of skin cancer)
- Spitz nevus (another type of mole)
What to expect with Angiokeratoma Circumscriptum
Angiokeratoma circumscriptum, a type of skin lesion, can be permanently removed with surgical or laser treatment. It is rare for these lesions to come back after treatment.
Possible Complications When Diagnosed with Angiokeratoma Circumscriptum
Treatment methods for a condition called angiokeratoma circumscriptum using laser, cryotherapy, electrocautery, or diathermy carry some risks. These include changes in skin texture or post-inflammatory color changes. Additionally, similar to all surgeries, there can be complications. There may be disfigurement to the appendages or body depending upon where the large angiokeratoma circumscriptum plaques are removed.
Common Risks:
- Changes in skin texture
- Post-inflammatory color changes
- Typical surgical complications
- Disfigurement of the appendages or main body section
Preventing Angiokeratoma Circumscriptum
Patients should know that this condition is not life-threatening and be aware of the different treatment options available to them. It’s important to note, however, that some treatments may cause physical changes or alterations to appearance.
