Introduction:
Lymes disease is one of the most common tick-borne illnesses caused by Borrelia species of bacteria. It is spread by black-legged or deer ticks (Ixodes tick species). Therefore, one is more likely to get Lyme disease if they live or spends time where ticks carrying Lyme disease thrive, like in grassy and heavily wooded areas. The condition is characterized by a rash that appears anywhere on the body, joint pain, fever, chills, fatigue, neck stiffness, neurological problems like Bell’s palsy, numbness, meningitis, etc. The third or late stage of untreated Lymes disease is called acrodermatitis chronica atrophicans.
What Is Acrodermatitis Chronica Atrophicans?
Acrodermatitis chronica atrophicans (ACA) is a late or chronic manifestation of Lymes disease. Chronic cutaneous atrophy is a feature of acrodermatitis chronica atrophicans, primarily affecting the distal parts of the extremities. Acrodermatitis chronica atrophicans does not disappear on its own, unlike other skin manifestations of Lyme disease, such as erythema migrans (EM) and borrelial lymphocytoma (BL). If left untreated, acrodermatitis chronica atrophicans may progress from bluish-red discoloration and inflammation to chronic atrophy and fibrosis, with the late stages being more resistant to treatment.
What Causes Acrodermatitis Chronica Atrophicans?
The Borrelia species of bacteria are the causative agents of acrodermatitis chronica atrophicans. Out of the 20 different species of Borellia, only five are pathogenic to humans: B. afzelii, B. garinii, B. bavariensis, B. burgdorferi sensu stricto, and B. spielmanii. Acrodermatitis chronica atrophicans is caused by an active skin infection primarily by Borrelia afzelii. Borrelia garinii and Borrelia burgdorferi sensu stricto species can also cause the disease.
Who Can Get Acrodermatitis Chronica Atrophicans?
Middle-aged and older individuals, particularly women between 40 to 70 years, are most commonly afflicted by acrodermatitis chronica atrophicans. But anyone can get a borrelial infection, although the cases reported among children are relatively rare. According to the estimates, it develops in 1 to 10 percent of European Lyme disease bacteria-infected individuals.
What Are the Symptoms of Acrodermatitis Chronica Atrophicans?
A unilateral violet discoloration of the extensor sections of the upper or lower limbs, particularly the dorsum of the hand, elbow, ankle, or knee, is the characteristic presentation of acrodermatitis chronica atrophicans. However, it can be bilateral and manifest itself anywhere on the body. The acrodermatitis chronica atrophicans develop in two stages:
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Inflammatory Stage: This stage is characterized by an ill-defined reddish discoloration and swelling of the affected area. Additionally, the skin may become tender or painful.
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Atrophic Stage: The atrophic stage is characterized by thin skin, the disappearance of elastic fibers, loss of adnexal structures (hair and sweat glands), and dilatation of blood vessels. The skin becomes very thin and is called paper-like; it tears and ulcerates easily after minor trauma.
Other less common symptoms of acrodermatitis chronica atrophicans include:
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Papules.
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Pain in the extremities.
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Plaques.
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Skin-colored nodules.
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Musculoskeletal pain.
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Decreased joint movements due to localized increase of collagen.
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Progressive allodynia (sensitive to touch that usually does not provoke pain).
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Paresthesia.
A history of tick bites is common among acrodermatitis chronica atrophicans infected patients. Some might remember having an erythema migrans-like rash months or years prior, which frequently affected the same leg. Around the site of a tick bite contaminated with Borrelia, erythema migrans, a ring-shaped rash, develops.
The foot, lower leg or hand, forearm, and olecranon area are common sites of acrodermatitis chronica atrophicans; proximal locations on the upper arm and shoulder or the thigh and buttock may be seen but are relatively rare. Sometimes the erythema is mild, the swelling is dominant, or the signs are subtle and go unnoticed by the patient or the physician. Lymphadenopathy may be observed.
How Is Acrodermatitis Chronica Atrophicans Diagnosed?
Acrodermatitis chronica migrans is most likely underdiagnosed. A detailed medical history is required to check whether there has been tick exposure (for example, in grass or woodlands in endemic regions) or any prior manifestation of Lyme disease. When acrodermatitis chronica atrophicans is suspected, a thorough general skin examination should be performed.
Laboratory tests are done to confirm acrodermatitis chronica atrophicans which include:
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Borrelia serology reveals a high IgG level in the enzyme-linked immunosorbent assay (ELISA) and on western blot. IgG antibodies can last long-term, even after successful treatment of Lyme disease or acrodermatitis chronica atrophicans.
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On skin biopsy, typical histological features of acrodermatitis chronica atrophicans include a dense perivascular lymphocytic infiltrate with plasma cells and mast cells and dermal edema; in the late stage, epidermal atrophy, loss of adnexal structures, superficial sclerosis, and fibroplasia are seen. Spirochaetes can be identified using special stains.
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A polymerase chain reaction can confirm borrelial infection in the skin (PCR).
How Is Acrodermatitis Chronica Atrophicans Treated?
Antibiotics are effective in treating acrodermatitis chronica atrophicans. The antibiotic used and the duration of treatment are determined by the other organs involved and the severity of the symptoms. Antibiotics used to treat acrodermatitis chronica atrophicans include:
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Doxycycline.
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Amoxicillin.
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Penicillin G.
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Cephalosporins: Ceftriaxone, Cefotaxime, and Cefuroxime Axetil.
How Can Acrodermatitis Chronica Atrophicans Be Prevented?
Since there are no vaccines against Borrelia, the disease can only be prevented by avoiding tick bites or promptly removing the tick.
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Avoid Lyme disease-endemic areas.
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Wear white or light-colored clothes (so the tick can be seen more clearly) with long sleeves, long trousers or pants tucked into socks, or long boots when walking in high grass or woodland.
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Use insect repellents or insecticides.
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Change your clothes and thoroughly inspect your entire body after returning from a walk in an endemic area.
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Check your body for ticks again the next day.
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Remove the tick as soon as possible to reduce the risk of Lyme disease transmission. The area must be disinfected. Tweezers should be used to carefully and steadily pull the tick out of the skin. The site must be disinfected again, and hands must be washed thoroughly.
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For several weeks, the tick bite site must be observed. A doctor must be consulted if the rash is larger than five centimeters or if one has flu-like symptoms.
Conclusion:
Acrodermatitis chronica atrophicans is a third stage or late manifestation of Lymes disease caused by an ongoing active Borrelia infection. Persistent active infection with Borrelia afzelii causes this unusual progressive fibrosing skin process. The infection can be eradicated in the later atrophic stage, but skin changes persist. Avoid tick bites to prevent illness. It is most effectively treated in the early inflammatory stage when the skin changes are reversible.
