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Erythema Chronicum Migrans
Migrans Erythema

Erythema chronicum migrans is a cutaneous manifestation of Lyme disease, a spirochetal infection caused by Borrelia burgdorferi after a tick bite. It is most commonly described as a slowly expanding circular erythematous "target" lesion, although various atypical presentations are reported. For this reason, a detailed inspection of the skin and a complete patient history are most important parts of workup when it comes to identifying this skin lesion.

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Presentation

Lyme disease is a tick-borne infection caused by Borrelia burgdorferi, a spirochetal bacterial pathogen [1] [2]. In approximately 80% of cases, cutaneous manifestations of this infection appear, predominantly in the form of erythema chronicum migrans (or erythema migrans), a pathognomonic finding of Lyme disease. It is described as an erythematous papule or macule at the site of the tick bite [3] [4] [5] [6]. The incubation period is variable (3-30 days), but in the majority of cases, erythema chronicum migrans is seen after 1-2 weeks and can occur on virtually any site of the body [1] [2] [5]. However, the waist, the extremities, the groins, the back, and the head and neck in the pediatric population are most frequent sites [4]. The progression of the erythematous macule, which may be asymptomatic, painful, or pruritic, eventually leads to a "target" or "bull's eye" lesion (as a central clearing around the center of the erythematous lesion is observed), with a diameter of > 5 cm, although a diameter as large as 60 cm has been reported [7] [8]. Numerous reports have confirmed that up to 30% of lesions exhibit significant variations [3] [5] [6] [7]. Firstly, multiple lesions can develop, the reason being the dissemination of the infection [3]. Secondly, an increasing number of cases present only with profound erythema and the absence of central clearing, thus the typical "target" lesion is not a mandatory finding in Lyme disease [5] [6]. Moreover, necrosis of the lesion, central hemorrhage, and formation of bullae are less common, but still possible variants of erythema chronicum migrans [1] [8]. It must be noted that numerous symptoms of Lyme disease can accompany erythema chronicum migrans, such as neurological deficits, fever, neck stiffness (suggesting meningitis), cardiac conduction abnormalities and arthritis (a sign of disseminated disease) [1] [2] [5].

Workup

The importance of recognizing erythema chronicum migrans lies in the fact that up to 60% of patients have negative serology tests for B. burgdorferi in the first several weeks of the infection [5]. For this reason, a meticulous physical examination supported by data obtained during patient history is the pivotal step in confirming Lyme disease and observing its hallmark lesion [6]. Physicians must inquire whether patients reside in or have recently visited at-risk areas, and note the signs and symptoms that possibly suggest an infectious etiology, while the course and progression of the skin lesion should also be discussed with the patient (if the patient had noticed the lesion etc.). The entire body should be examined in order to exclude multiple erythema chronicum migrans, and a close inspection, as well as palpation of the lesion, is vital for raising clinical suspicion of Lyme disease as a potential diagnosis [1] [2] [4] [7] [8]. Physicians must be aware of the incubation period of erythema chronicum migrans, its variable presentation, and the broad differential diagnosis [4]. In all patients in whom this lesion is suspected, microbiological investigation to confirm Lyme disease is mandatory. As serologic testing may be initially negative, blood cultures, biopsy samples of the lesion and polymerase chain reaction (PCR) testing can be implemented [1] [2] [5] [6]. But because of their limited use and sensitivity/specificity in the absence of erythema chronicum migrans, clinical assessment remains the most important part of Lyme disease workup.

Treatment

The primary treatment for ECM and early Lyme disease is antibiotics. Doxycycline is commonly prescribed for adults and children over eight years old. Alternatives include amoxicillin or cefuroxime for those who cannot take doxycycline. The typical course lasts 10 to 21 days, depending on the specific antibiotic used. Early treatment is crucial to prevent the progression of Lyme disease to more severe stages, which can affect the joints, heart, and nervous system.

Prognosis

With prompt and appropriate antibiotic treatment, the prognosis for individuals with ECM is excellent. Most patients experience a complete resolution of the rash and associated symptoms. However, some may experience lingering symptoms, such as fatigue or joint pain, known as post-treatment Lyme disease syndrome (PTLDS). These symptoms usually improve over time, although they can persist for months or even years in some cases.

Etiology

ECM is caused by an infection with the bacterium Borrelia burgdorferi, transmitted to humans through the bite of infected black-legged ticks, also known as deer ticks. These ticks are typically found in wooded and grassy areas. The risk of infection increases with prolonged tick attachment, usually requiring 36 to 48 hours for transmission of the bacteria.

Epidemiology

Lyme disease, and consequently ECM, is most prevalent in the northeastern, north-central, and Pacific coastal regions of the United States, as well as parts of Europe and Asia. The incidence of Lyme disease has been increasing, likely due to factors such as changes in land use, climate change, and increased awareness and reporting. People who spend time in tick-infested areas, such as hikers, campers, and outdoor workers, are at higher risk.

Pathophysiology

Once Borrelia burgdorferi enters the human body through a tick bite, it begins to multiply and spread through the skin, leading to the development of ECM. The bacteria can evade the immune system and disseminate to other parts of the body, potentially affecting the joints, heart, and nervous system. The immune response to the bacteria contributes to the symptoms and inflammation associated with Lyme disease.

Prevention

Preventing ECM involves reducing the risk of tick bites. This can be achieved by avoiding tick-infested areas, using insect repellent containing DEET, wearing long sleeves and pants, and performing thorough tick checks after outdoor activities. Prompt removal of ticks can also reduce the risk of infection, as transmission of Borrelia burgdorferi typically requires prolonged attachment.

Summary

Erythema Chronicum Migrans is a hallmark sign of Lyme disease, characterized by a red, expanding rash often resembling a bull's-eye. Early recognition and treatment with antibiotics are crucial to prevent more severe complications. Understanding the risk factors, symptoms, and preventive measures can help manage and reduce the incidence of this condition.

Patient Information

If you notice a red, expanding rash after a tick bite, it may be Erythema Chronicum Migrans, a sign of Lyme disease. This rash can appear days to weeks after the bite and may be accompanied by flu-like symptoms. Early treatment with antibiotics is effective, so it's important to seek medical attention if you suspect ECM. To prevent tick bites, use insect repellent, wear protective clothing, and check for ticks after spending time outdoors.

References

  1. Murray PR, Rosenthal KS, Pfaller MA. Medical Microbiology. Seventh edition. Philadelphia: Elsevier/Saunders; 2013.
  2. Mandell GL, Bennett JE, Dolin R. Mandel, Douglas and Bennett's Principles and Practice of Infectious Diseases. 8th ed. Philadelphia, Pennsylvania: Churchill Livingstone; 2015.
  3. Eriksson P, Schröder MT, Niiranen K, Nevanlinna A, Panelius J, Ranki A. The many faces of solitary and multiple erythema migrans. Acta Derm Venereol. 2013;93(6):693-700.
  4. Juckett G. Arthropod bites. Am Fam Physician. 2013;88(12):841-847.
  5. Shapiro ED. Lyme Disease. N Engl J Med. 2014;370(18):1724-1731.
  6. Aucott J, Morrison C, Munoz B, Rowe PC, Schwarzwalder A, West SK. Diagnostic challenges of early Lyme disease: Lessons from a community case series. BMC Infect Dis. 2009;9:79.
  7. Schutzer SE, Berger BW, Krueger JG, Eshoo MW, Ecker DJ, Aucott JN. Atypical Erythema Migrans in Patients with PCR-Positive Lyme Disease. Emerg Infect Dis. 2013;19(5):815-817.
  8. Wetter DA, Ruff CA. Erythema migrans in Lyme disease. CMAJ. 2011;183(11):1281.
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