What is Acrodermatitis Chronica Atrophicans (Lyme Disease)?
Acrodermatitis chronica atrophicans (ACA) is a late stage and chronic condition associated with Lyme disease (LB). Commonly, ACA affects the far ends of limbs causing chronic skin thinning and does not go away like other skin signs of Lyme disease such as erythema migrans (EM) and borrelial lymphocytoma (BL). If not treated, ACA can worsen from reddish-blue coloration and inflammation to chronic skin thinning and fibrosis, and late stage becomes more difficult to treat.
The condition was first documented in 1883 in Germany, and cases were found in North America by 1895. The diagnosis of ACA is determined by the symptoms, serologic testing (tests of the blood), and histopathologic confirmation (testing of tissue). Identifying ACA can be difficult due to variable delay in the appearance of the disease after the initial infection, and also because there might be no symptoms causing the delay in seeking treatment.
Lyme borreliosis is the most common disease transmitted by ticks in the northeastern United States. The bacteria called Borrelia burgdorferi, transmitted through an Ixodes tick bite, causes the disease which is a multi-organ infection. The disease is characterized by 3 skin signs: a “bull’s eye rash” (stage 1), which if left untreated, can be followed by early spread of infection, borrelial lymphocytoma (stage 2) with neurological and cardiac abnormalities, and in the late stage, arthritis in North America or ACA (stage 3) in Europe.
ACA is the most common late and chronic sign of Lyme disease. About 20% of ACA patients have a history of a healed EM, usually on a limb where the ACA appeared 6 months to 8 years later.
What Causes Acrodermatitis Chronica Atrophicans (Lyme Disease)?
Borrelia is a type of bacteria that belongs to the Spirochaetacea family. Around 20 different types of Borrelia exist, but only five of them pose a significant health risk to people. These five are B. afzelii, B. garinii, B. bavariensis, B. burgdorferi sensu stricto, and B. spielmanii.
A skin condition known as ACA is mainly caused by an infection with B. afzelii. However, it can also be caused by two other types of Borrelia called European B. burgdorferi sensu stricto or B. garinii.
Risk Factors and Frequency for Acrodermatitis Chronica Atrophicans (Lyme Disease)
Acrodermatitis chronica atrophicans, or ACA for short, is a condition that mostly affects women aged 40 to 70. It is not very common in children. The disease is more often seen in Europe because it’s linked to a specific untreated infection called Borrelia afzelii. While this infection does occur in the United States, ACA is quite rare there. Amongst all European patients with Lyme disease, about 10% also have ACA.
Signs and Symptoms of Acrodermatitis Chronica Atrophicans (Lyme Disease)
Acrodermatitis chronica atrophicans (ACA), is a condition that often progresses without the person realizing they were bitten by a tick. Diagnosis can be quite difficult due to the varying symptoms a person may experience. ACA usually follows a two-stage progression. At first, you may see bluish-red patches on your skin, which appears doughy and swollen. As the condition worsens, your skin may become thin and shiny, a state often described as “cigarette paper skin.” This mainly occurs on the outer surfaces of your limbs. Some people may form fibrous bumps over the joints, particularly on the ulnar or tibial regions.
About 15% of people with ACA may experience a hardened band of skin due to increased collagen, which can limit joint movement. About half of the patients may experience symptoms of peripheral neuropathy such as numbness, tingling, or a sensation of discomfort triggered by a normally harmless stimulus, known as allodynia. In very rare cases, a person’s limb can become enlarged due to tenosynovitis and dactylitis, conditions affecting the tendons and digits respectively.
Testing for Acrodermatitis Chronica Atrophicans (Lyme Disease)
ACA, or Acrodermatitis Chronica Atrophicans, is generally diagnosed based on symptoms spotted by medical professionals and supported by specific blood tests. These tests look at the level of certain antibodies, named Borrelia IgG, in your blood.
For most people with chronic Lyme disease, these blood tests will come back positive for Borrelia IgG antibodies. It’s important to note that another type of antibodies, called Borrelia IgM, can sometimes give a false positive result in later stages of the disease, and therefore, aren’t as useful in diagnosing ACA.
If your blood test for Borrelia IgG comes back negative, that generally means you don’t have ACA. It’s worth noting that in some cases, when it’s not clear whether someone has ACA or not, a skin biopsy may be taken for further examination.
Lastly, the presence of B. burgdorferi, the bacteria responsible for causing Lyme disease, can be confirmed by testing a sample of your blood or cultured cells for its DNA. This can provide further confidence in the diagnosis.
Treatment Options for Acrodermatitis Chronica Atrophicans (Lyme Disease)
The standard method of treatment usually involves giving one of the following antibiotics:
1. Amoxicillin taken orally. The patient typically takes 500 to 1000 mg three times a day for 14 to 28 days.
2. Doxycycline taken orally. The person usually takes 100 mg twice a day or 200 mg once a day for 14 to 28 days.
3. Ceftriaxone given through an IV (Intravenous) line. The patient usually receives 2000 mg every 24 hours for 14 to 28 days.
4. Cefotaxime also given through an IV line. The patient typically gets 2000 mg every 8 hours for 14 to 28 days.
5. Penicillin G given through an IV line. The person typically receives 3 to 4 Million Units (MU) every 4 hours for 14 to 28 days.
What else can Acrodermatitis Chronica Atrophicans (Lyme Disease) be?
Diagnosing ACA, or acroangiodermatitis, can be difficult because it has different appearances and stages. Sometimes, due to their location on the lower legs, ACA skin abnormalities might be mistaken as symptoms of various other conditions. These can include:
- Problems related to blood flow such as chronic venous or arterial insufficiency
- An injury caused by excessive cold temperatures
- Patterns of discoloration on the skin known as livedo reticularis
- A type of skin tightening condition called localized scleroderma
- Erysipelas, which is a bacterial skin infection
- A condition causing swollen lymphatic tissues, known as lymphedema
- Effects of chronic use of potent topical corticosteroids or simply aging
For nodules or lumps seen in ACA, they could potentially be confused with conditions such as:
- Rheumatoid disease
- Gout, a type of arthritis
- An inflammatory condition resulting in lumps under the skin, known as erythema nodosum
Therefore, it is crucial to rule out these possibilities while diagnosing ACA.
What to expect with Acrodermatitis Chronica Atrophicans (Lyme Disease)
Treatment outcomes are generally positive if the early inflammation stage is suitably addressed. However, predicting the outcome for the chronic wear-and-tear phase is challenging, as damage is only partially reversible. Research has found that using a specific type of antibiotic (third-generation cephalosporin) for 28 days can result in the skin lesion either partially or completely disappearing.
However, if the skin lesion remains untreated, it doesn’t usually heal on its own. Over time, the skin can become fibrous and worn out. Adding to this, antibiotics might not be able to reverse neurological symptoms like multiple nerve pain or abnormal sensations.
Possible Complications When Diagnosed with Acrodermatitis Chronica Atrophicans (Lyme Disease)
Acrodermatitis chronica atrophicans, often referred to as ACA, is a skin condition that can result in other serious problems. There are risks associated with this condition, such as bacterial infections developing on top of existing ACA skin lesions. Moreover, ACA is linked with an increased risk of certain types of cancers, which include but are not limited to:
- B-cell lymphoma
- Basal cell carcinoma
- Squamous cell carcinoma
Preventing Acrodermatitis Chronica Atrophicans (Lyme Disease)
Diagnosing Acrodermatitis Chronica Atrophicans (ACA), a skin condition, often takes time as people may not realize they’re unwell or seek medical help straight away. This happens partly due to the disease slowly developing over a few months or even years after a tick bite.
The skin changes in ACA most commonly appear on arms and legs, but they can also show up in different areas like the abdomen or the face. Doctors use blood tests and examination of skin tissue to confirm the diagnosis. It’s crucial to start treatment as soon as possible to stop irreversible damage to the skin.
Lyme disease, from which ACA evolves, is best prevented by avoiding tick bites. If you’re walking in forests or other woodland, remember to protect yourself. Use bug sprays, wear long sleeves, tuck your trousers into your socks or long boots, and put on light-colored clothes so ticks are easier to spot.
After spending time outdoors, it’s important to change your outfit and check your body for any ticks. If you find one, use a pair of fine tweezers to grab the tick as close to your skin as possible and pull it out gently. Dispose of the tick carefully and wash the area where it bit you with soap and water. If you notice a rash or get a fever, see a doctor right away to prevent any complications.
