What is Atrophie Blanche?
Atrophie blanche (AB) is a type of scarring pattern that develops due to a medical condition known as livedoid vasculopathy. This is a chronic disease that causes recurring, painful ulcers on the lower legs, ankles, or top of the foot, mainly in young to middle-aged women. Atrophie blanche is the name given to the distinctive white, thin, star-shaped scar that forms when the ulcers heal, typically with additional tiny blood vessels around the edge.
Livedoid vasculopathy, another name for atrophie blanche, involves a primary ulcerated lesion and is not well understood. Scientists think it might occur when the small blood vessels in the top layer of skin become blocked, resulting in skin ulcers. This condition has several other less common names, such as segmental hyalinizing vasculitis, livedo reticularis with summer ulceration, and painful purpuric ulcers with reticular patterning on the lower limbs.
The physical symptoms of livedoid vasculopathy can vary depending on the stage of the condition, but it tends to commonly appear in middle-aged women. They show up as painful, reddish-purple lower leg lesions that further develop into small, round ulcerations. These ulcers are chronic, take a long time to heal, and leave behind unique star-shaped, white scars. While more common in middle-aged women, this condition can also affect men and has been reported in children.
Other conditions, such as cutaneous small-vessel vasculitis and antiphospholipid antibody syndrome, can also cause scars resembling those seen in atrophie blanche. However, these conditions can be distinguished as they do not have a history of forming small, round ulcers before scarring.
What Causes Atrophie Blanche?
The exact reason behind livedoid vasculopathy, a skin condition, is still not fully known, despite many theories. It is thought to be caused by blockages in the blood vessels near the surface of the skin, which can then lead to skin ulcers.
Risk Factors and Frequency for Atrophie Blanche
Atrophie blanche is a rare disease, affecting between 1% to 5% of the population. It generally occurs in middle-aged women, with an average diagnosis age of 45. However, there are some rare reports of children also having the disease.
Signs and Symptoms of Atrophie Blanche
People with this medical condition usually experience certain symptoms, depending on the phase of the disease. Initially, they often suffer from painful skin spots on both sides of their body, which eventually turn into small, circular, open sore wounds. These spots can be accompanied by a net-like pattern of blood vessels just under the skin. The sores heal into porcelain-white, star-shaped scars, which may have tiny, widened, red blood vessels around them. Patients usually experience recurring ulcers, which tend to worsen during hot weather and pregnancy.
There are other possible causes of these symptoms that one may need to rule out. It’s crucial to check whether the patient has other underlying illnesses such as peripheral arterial disease, venous stasis (a condition where blood flow in the veins is slow), or even cancer. A person who has white scarring similar to atrophie blanche (a skin condition resulting in porcelain-white scars), but doesn’t have a medical history or signs of open, circular wounds, could be facing a different issue.
Testing for Atrophie Blanche
Atrophie blanche, also known as livedoid vasculopathy, can often mirror the symptoms of several other diseases. To accurately diagnose livedoid vasculopathy, it’s important to rule out any other possible conditions. Here are some tests doctors usually need to run to confirm it:
- The Doppler studies and ankle-brachial index test can help rule out issues with leg vein flow or artery blockages.
- A skin biopsy could also be performed to check for inflammation in your veins and small blood vessels; this helps in ruling out skin vasculitis.
- To test for a condition known as the antiphospholipid antibody syndrome, which can cause blood clots, your doctor might conduct screening coagulation studies.
- Your doctor could request you to have a hemoglobin A1C test and a full blood count, which could provide valuable information about your general health.
- Both the basic metabolic panel, a group of (usually) 8 tests, and Antinuclear antibody (ANA) tests can help rule out potential autoimmune conditions.
- If your doctor suspects that you might have diabetes, autoimmune syndromes or cancer, they might also check for rheumatoid factor and C-reactive protein (CRP), which are substances in your blood that can indicate inflammation.
Note that this isn’t an exhaustive list, and your doctor may suggest more tests based on your symptoms and medical history.
Treatment Options for Atrophie Blanche
Treating ‘atrophie blanche’, a skin condition, when there’s no underlying disease, usually begins with drugs that prevent blood clots (antiplatelet), dissolve clots (fibrinolytic), or thin the blood (anticoagulation). Medications like aspirin, dipyridamole, pentoxifylline, and heparin are commonly used. Sulfasalazine, danazol, or stanozolol may also help, either by themselves or in combination with other treatments.
Other successful treatment methods include hyperbaric oxygen, which exposes the body to pure oxygen in a pressurized room, and PUVA, a process that combines drug treatment with UV radiation. For stubborn conditions that don’t respond to initial treatments, doctors might also prescribe rivaroxaban, oral steroids, prostanoids, and IVIG (a therapy that uses a mix of antibodies).
It’s crucial for patients to be educated about steps they can take to manage the condition. For example, they may be advised to quit smoking, avoid things that irritate their skin or shoes that don’t fit properly, and take care of wounds on their lower extremities. This can be done by elevating legs, applying compression bandages, and using wound coverings that seal out air (occlusive dressings).
What else can Atrophie Blanche be?
There are several other medical conditions that can appear similar to atrophie blanche in their final stages. These conditions include skin inflammation due to small blood-vessel disease, ulcers related to the medication hydroxyurea, sickle cell disease, and a disorder where the body’s immune system mistakenly attacks normal proteins in the blood, known as antiphospholipid antibody syndrome.
These other illnesses can usually be differentiated from atrophie blanche as the patient’s medical history will not involve previous instances of specific types of skin ulcers, described as ‘punched-out’.
What to expect with Atrophie Blanche
The outlook for a condition known as atrophie blanche can differ from person to person. However, it’s generally seen as a long-term disease that tends to come back again and again.
Possible Complications When Diagnosed with Atrophie Blanche
Atrophie blanche, a skin condition, may lead to several possible complications. These include:
- Infection
- Scarring
- Pain
- Ulceration (formation of sores)
- Blood loss
- Stricture formation (narrowing of bodily passages)
- Poor quality of life
Preventing Atrophie Blanche
Educating patients is crucial. They should be advised to stop using tobacco products and to avoid irritating substances and shoes that don’t fit well. They should also be taught the basics of how to take care of wounds on their lower extremities, such as legs. These basics include elevating the leg, applying compression therapy, and dressing wounds in a manner that seals them off from the environment.
