Overview
What are the signs and symptoms of Lyme disease?
Which physical findings are characteristic of early disseminated Lyme disease?
What is the CDC recommended serologic testing for Lyme disease?
What is the role of Western blot testing in the diagnosis of Lyme disease?
Which tests may be performed in the evaluation of Lyme disease?
What is the focus for the treatment of Lyme disease?
What are the treatment options for Lyme disease?
What are the treatment options for Lyme arthritis?
What are the treatment options for Lyme carditis?
What is the clinical presentations of Lyme disease?
How is Lyme disease diagnosed and treated?
What is the historical background of Lyme disease?
Which areas in the US are commonly affected by Lyme disease?
What is the infectious cycle of Borrelia burgdorferi?
What is the mode of transmission of Lyme disease?
What is the pathogenesis of Lyme disease?
What is the clinical progression of Lyme disease?
What are the signs and symptoms of Stage 1 (early localized) Lyme disease?
What is Stage 2 (early disseminated) Lyme disease?
What is Stage 3 (chronic) Lyme disease?
Which species of Borrelia burgdorferi sensu lato that causes Lyme disease?
How does the clinical presentation of Lyme disease differ by causative agent?
Where is of Lyme disease endemic?
What is the incidence of Lyme disease in the US?
Which US states have the highest rates of Lyme disease?
What is the incidence of Lyme disease when does peak incidence occur?
What is the global prevalence of Lyme disease?
What are racial predilections of Lyme disease?
How does the incidence of Lyme disease vary by sex?
How does the incidence of Lyme disease vary by age?
What is the prognosis of Lyme disease?
What is the prognosis of post-treatment Lyme disease syndrome (PTLDS)?
What is included in patient education about Lyme disease?
How should inspection and tick removal be performed for the prevention of Lyme disease?
What is the role of repellents in the prevention of Lyme disease?
What is the role of prophylactic antibiotics in the prevention of Lyme disease?
What is the role of pets in the prevention of Lyme disease?
Presentation
Which clinical history findings are characteristic of Lyme disease?
What is the frequency of reinfection and recurrence of Lyme disease?
How does the clinical presentation of Lyme disease vary based on species of Borrelia?
What are the stages of Lyme disease progression?
How is erythema migrans (EM) characterized in Lyme disease?
What are the non-cutaneous signs and symptoms of Stage 1 (early) Lyme disease?
What is the prevalence of co-infection in Lyme disease?
What is the clinical presentation of Stage 2 (early disseminated) Lyme disease?
How does arthritis present in Lyme disease?
What are neurologic manifestations of Lyme disease?
What are cutaneous manifestations in Stage 2 (local disseminated) Lyme disease?
What are the signs and symptoms of Stage 3 (chronic) Lyme disease?
Which physical findings are characteristic of Lyme disease?
What are the physical characteristics of erythema migrans (EM) in patients with Lyme disease?
What are atypical presentations of erythema migrans (EM) in patients with Lyme disease?
Where are erythema migrans (EM) located in Lyme disease?
How are hypersensitivity reactions differentiated from the erythema migrans (EM) of Lyme disease?
What is borrelial lymphocytoma in Lyme disease?
Which dermatologic manifestations of Lyme disease have been reported in European patients?
Which musculoskeletal findings suggest Lyme disease?
Which physical findings indicate arthritis in patients with Lyme disease?
Which neurologic findings suggest Lyme disease?
Which neuropsychiatric findings are characteristic of late-stage Lyme disease?
Which cardiac findings suggest Lyme disease?
Which ophthalmic findings suggest Lyme disease?
Which ocular findings are characteristic of Lyme disease?
DDX
How is Lyme disease diagnosed?
Which conditions should be included in the differential diagnosis of Lyme disease?
How are co-infections diagnosed in patients with Lyme disease?
What are the differential diagnoses for Lyme Disease?
Workup
What is the initial approach to evaluation of suspected Lyme disease?
What is the CDC recommended testing procedure for the diagnosis of Lyme disease?
What is the role of lab testing in patients with clinical findings of Lyme disease?
When is joint aspiration indicated in the evaluation of Lyme disease?
What is the role of cerebrospinal fluid (CSF) analysis in the evaluation of Lyme disease?
What is the Rule of 7's and how is it used to determine risk for Lyme disease?
What is the role of electrocardiograms (ECGs) in the workup of Lyme disease?
When are imaging studies indicated in the workup of Lyme disease?
What is the role of serologic testing in the evaluation of Lyme disease?
What are tiers of serologic testing in the evaluation of Lyme disease?
Which serologic findings confirm a diagnosis of Lyme disease?
What is the accuracy of serologic testing for the diagnosis of Lyme disease?
When is serologic testing indicated in the workup of Lyme disease?
What is the role of C6 peptide testing in the diagnosis of Lyme disease?
What is the role of polymerase chain reaction (PCR) testing in the diagnosis of Lyme disease?
What are role of blood studies in the workup of Lyme disease?
What is the role of synovial fluid analysis in the workup of Lyme disease?
What is the role of lumbar puncture in the workup of Lyme disease?
What are the diagnostic criteria for neuroborreliosis in patients with Lyme disease?
What is the role of imaging studies in the workup of Lyme disease?
What is the role of culture in the workup of Lyme disease?
What is the role of biopsy in the workup of Lyme disease?
Which histologic findings of erythema migrans are characteristic of Lyme disease?
Which histologic findings of borrelial lymphocytoma are characteristic of Lyme disease?
Which histologic findings of acrodermatitis chronica atrophicans are characteristic of Lyme disease?
Treatment
What guides the selection of treatment for Lyme disease?
How is cardiac involvement managed in Lyme disease?
How is persistent arthritis managed in Lyme disease?
How is neuroborreliosis managed in Lyme disease?
How is borrelial lymphocytoma managed in Lyme disease?
When is antibiotic prophylaxis indicated for the prevention of Lyme disease?
What are the treatment guidelines for Lyme disease?
What is the controversy surrounding the IDSA treatment guidelines for Lyme disease?
What are the treatment options for early localized Lyme disease?
How is early Lyme disease managed in pregnant women?
What are the regimens for treatment of Lyme arthritis in adults?
What are the regimens for Lyme arthritis in pediatric patients?
What are the treatment options for Lyme arthritis following an initial antibiotic regimen?
What are the treatment options for Lyme carditis?
What are treatment options for neurologic manifestations of Lyme disease?
What are treatments options for ocular manifestations of Lyme disease?
How is acrodermatitis chronica atrophicans managed in patients with Lyme disease?
What are the treatments options for post-treatment Lyme disease syndrome (PTLDS)?
When should co-transmitted infective organisms be considered in patients with Lyme disease?
How is Lyme disease prevented?
How is tick removal performed to reduce the likelihood of contracting Lyme disease?
When is antibiotic prophylaxis indicated in the prevention of Lyme disease?
What are contraindications for doxycycline prophylaxis against Lyme disease?
What is the role of vaccination in the prevention of Lyme disease?
Which medical personnel provide consultations to patients with Lyme disease?
What is included in the long-term monitoring of patients with Lyme disease?
Guidelines
Which organizations have released guidelines on the diagnosis and management of Lyme disease?
What are the IDSA/AAN/ACR guidelines for the diagnosis of Lyme disease?
What are the IDSA/AAN/ACR guidelines for diagnosis of Lyme neuroborreliosis?
What are the IDSA/AAN/ACR guidelines for treatment of Lyme neuroborreliosis?
What are the IDSA/AAN/ACR guidelines for treatment of Lyme carditis?
What are the IDSA/AAN/ACR guidelines for treatment of Lyme arthritis?
Medications
What is the role of antibiotic therapy in the treatment of Lyme disease?
Which medications in the drug class Aminoquinolines are used in the treatment of Lyme Disease?
Which medications in the drug class Macrolides are used in the treatment of Lyme Disease?
Which medications in the drug class Penicillins are used in the treatment of Lyme Disease?
Which medications in the drug class Tetracyclines are used in the treatment of Lyme Disease?
-
Lyme disease. The bacterium Borrelia burgdorferi (darkfield microscopy technique, 400X; courtesy of the US Centers for Disease Control and Prevention).
-
Lyme disease. Magnified ticks at various stages of development.
-
Ticks are the most common vectors for vector-borne diseases in the United States. In North America, tick bites can cause Lyme disease, human granulocytic and monocytic ehrlichiosis, babesiosis, relapsing fever, Rocky Mountain spotted fever, Colorado tick fever, tularemia, Q fever, and tick paralysis. Europe has a similar list of illnesses caused by ticks, but additional concerns include boutonneuse fever and tick-borne encephalitis. Lyme disease is one of the most prominent tick-borne diseases, and its main vector is the tick genus Ixodes, primarily Ixodes scapularis. Image courtesy of the US Centers of Disease Control and Prevention.
-
Lyme disease. Approximate US distribution of Ixodes scapularis. Image courtesy of the US Centers for Disease Control and Prevention.
-
Lyme disease. In general, Ixodes scapularis must be attached for at least 24 hours to transmit the spirochete to the host mammal. Prophylactic antibiotics are more likely to be helpful if feeding is longer. This photo shows 2 I scapularis nymphs. The one on the right is unfed; the other has been feeding for 48 hours. Note its larger size and the fact that the midgut diverticula (delicate brown linear areas on the body) are blurred. Photo by Darlyne Murawski; reproduced with permission.
-
Lyme disease. Normal and engorged Ixodes ticks.
-
Amblyomma americanum is the tick vector for monocytic ehrlichiosis and tularemia. An adult and a nymphal form are shown (common match shown for size comparison). Image by Darlyne Murawski; reproduced with permission.
-
Approximate US distribution of Amblyomma americanum. Image courtesy of the US Centers for Disease Control and Prevention.
-
The soft-bodied tick of the genus Ornithodoros transmits various Borrelia species that cause relapsing fever. Photo courtesy of Julie Rawlings, MPH, Texas Department of Health. Relapsing fever is characterized by recurrent acute episodes of fever (usually >39°C). It is a vector-borne illness spread by lice and ticks. The spirochete species Borrelia is responsible.
-
The Ixodes scapularis tick is considerably smaller than the Dermacentor tick. The former is the vector for Lyme disease, granulocytic ehrlichiosis, and babesiosis. The latter is the vector for Rocky Mountain spotted fever. This photo displays an adult I scapularis tick (on the right) next to an adult Dermacentor variabilis; both are next to a common match displayed for scale. Photo by Darlyne Murawski; reproduced with permission.
-
Approximate US distribution of Dermacentor andersoni. Image courtesy of the US Centers for Disease Control and Prevention.
-
Rhipicephalus ticks are vectors for babesiosis and rickettsial infections, among others. Image courtesy of Dirk M. Elston, MD. In typical practice, testing ticks for tick-borne infectious organisms is not generally recommended. However, healthcare practitioners should become familiar with the clinical manifestations of tick-borne diseases (eg, Lyme disease, especially those practicing in endemic areas) and maintain a high index of suspicion during warmer months. Ticks can be placed in a sealed container with alcohol if they need to be transported and identified.
-
To remove a tick, use fine-tipped forceps and wear gloves. Grasp the tick as close to the skin surface as possible, including the mouth parts, and pull upward with steady, even traction. Do not twist or jerk the tick because this may cause the mouth parts to break off and remain in the skin; however, note that the mouth parts themselves are not infectious. When removing, wear gloves to avoid possible infection.
-
Lyme disease. This patient's erythema migrans rash demonstrates several key features of the rash, including size, location, and presence of a central punctum, which can be seen right at the lateral margin of the inferior gluteal fold. Note that the color is uniform; this pattern probably is more common than the classic pattern of central clearing. On history, this patient was found to live in an endemic area for ticks and to pull ticks off her dog daily.
-
Erythema migrans, the characteristic rash of early Lyme disease.
-
Lyme disease. The thorax and torso are typical locations for erythema migrans. The lesion is slightly darker in the center, a common variation. In addition, this patient worked outdoors in a highly endemic area. Physical examination also revealed a right axillary lymph node.
-
Lyme disease. Photo of the left side of the neck of a patient who had pulled a tick from this region 7 days previously. Note the raised vesicular center, which is a variant of erythema migrans. The patient had a Jarisch-Herxheimer reaction approximately 18 hours after the first dose of doxycycline.
-
Lyme disease. Classic target lesion with concentric rings of erythema, which often show central clearing. Although this morphology was emphasized in earlier North American literature, it only represents approximately 40% of erythema migrans lesions in the United States. This pattern is more common in Europe. Courtesy of Lyme Disease Foundation, Hartford, Conn.
-
Typical appearance of erythema migrans, the bull's-eye rash of Lyme disease.
-
Lyme disease. Bulls-eye rash.
-
Lyme disease. Photo of erythema migrans on the right thigh of a toddler. The size and location are typical of erythema migrans, as is the history of the patient vacationing on Fire Island, NY, in the month of August. No tick bite had been noted at this location. Approximately 25% of patients with Lyme disease are children, which is the same percentage of patients who do not recall a tick bite. Courtesy of Dr John Hanrahan.
-
Lyme disease. Multiple lesions of erythema migrans occur in approximately 20% of patients. A carpenter from Nantucket who worked predominantly outside had been treated with clotrimazole/betamethasone for 1 week for a presumed tineal infection, but the initial lesion grew, and new ones developed. He then presented to the emergency department with the rashes seen in this photo. The patient had no fever and only mild systemic symptoms. He was treated with a 3-week course of oral antibiotics.
-
Lyme disease. The rash on the ankle seen in this photo is consistent with both cellulitis (deep red hue, acral location, mild tenderness) and erythema migrans (presentation in July, in an area highly endemic for Lyme disease). In this situation, treatment with a drug that covers both diseases (eg, cefuroxime or amoxicillin-clavulanate) is an effective strategy.
-
Lyme disease. Borrelial lymphocytoma of the earlobe, which shows a bluish red discoloration. The location is typical in children, as opposed to the nipple in adults. This manifestation of Lyme disease is uncommon and occurs only in Europe. Courtesy of Lyme Disease Foundation, Hartford, Conn.
-
A rarely reported noninfectious complication for tick bites is alopecia. It can begin within a week of tick removal and typically occurs in a 3- to 4-cm circle around a tick bite on the scalp. A moth-eaten alopecia of the scalp caused by bites of Dermacentor variabilis (the American dog tick) has also been described. No particular species appears more likely to cause alopecia. Hair regrowth typically occurs within 1-3 months, although permanent alopecia has been observed.
-
Acrodermatitis chronica atrophicans is found almost exclusively in European patients and comprises an early inflammatory phase and a later atrophic phase. As the term suggests, the lesion occurs acrally and ultimately results in skin described as being like cigarette paper. Courtesy of Lyme Disease Foundation, Hartford, Conn.
-
Blood smear showing likely babesiosis. Babesiosis can be difficult to distinguish from malaria on a blood smear.
-
Life cycle of the Ixodes dammini tick. Courtesy of Elsevier.
-
Lyme disease in the United States is concentrated heavily in the northeast and upper Midwest; it does not occur nationwide. Dots on the map indicate the infected person's county of residence, not the place where they were infected. Courtesy of the US Centers for Disease Control and Prevention (CDC).
Tables
- Table 1. Clinical presentation and therapy for the stages of Lyme disease
- Table 2. Adult and pediatric treatment options, dosages, and routes of administration
- Table 3. Comparison of Infectious Diseases Society of America (IDSA) and International Lyme and Associated Diseases Society (ILADS) recommendations for Lyme disease treatment
Disease Stage |
Clinical Manifestations |
Treatment |
Duration |
Early localized |
Erythema migrans |
Oral |
Doxycycline, 10 days Amoxicillin or cefuroxime axetil, 14 days Azithromycin, 7 days |
Early disseminated |
Multiple erythema migrans |
Oral |
14 days |
Isolated cranial nerve palsy |
Oral |
14-21 days |
|
Meningoradiculoneuritis |
Oral |
14-28 days |
|
Meningitis |
Intravenous or oral |
14-21 days |
|
Carditis |
|
|
|
-Ambulatory |
Oral |
14-21 days |
|
-Hospitalized |
Intravenous followed by oral |
14-21 days |
|
Borrelial lymphocytoma |
Oral |
14 days |
|
Late |
Arthritis |
Oral |
28 days |
Recurrent arthritis after oral therapy |
Oral or intravenous |
28 days or 14-28 days |
|
Encephalitis |
Intravenous |
14-28 days |
|
Acrodermatitis chronica atrophicans |
Oral |
21-28 days |
|
Treatment |
Adult Dose |
Pediatric Dose |
Oral Therapy |
Doxycycline (patients > 8 y) |
100 mg twice a day |
4 mg/kg (up to 100 mg) twice a day |
Amoxicillin |
500 mg three times a day |
50 mg/kg/day (up to 500 mg) in three divided doses |
|
Cefuroxime axetil |
500 mg twice a day |
30 mg/kg/day (up to 500 mg) in two divided doses |
|
Phenoxymethylpenicillin |
500 mg four times a day, or 1 gm three times a day |
50-100 mg/kg/day in three divided doses; maximum 1 g/dose |
|
Azithromycin (for patients unable to take doxycycline or beta-lactams) |
500 mg once a day
|
50-100 mg/kg/day in three divided doses; maximum 1 g/dose |
|
Intravenous therapy |
Ceftriaxone |
2 g once a day |
10 mg/kg/day (maximum, 500 mg/day) |
Cefotaxime |
2 g every 8 h |
150-200 mg/kg (up to 2 g) every 8 h |
|
Penicillin G |
18-24 million U/d divided every 4 h |
200,000-400,000 mg/kg (up to 2 g) every 8 h |
Treatment Focus |
IDSA |
ILADS |
Treatment of a tick bite without symptoms of Lyme disease |
Doxycycline, 200 mg as a single dose |
Doxycycline, 100 mg bid for 20 days |
Erythema migrans |
Doxycycline, amoxicillin, or cefuroxime for 14-21 days |
Doxycycline, amoxicillin, or cefuroxime for 28-42 days or azithromycin for at least 21 days |
“Persisting symptoms of Lyme disease” |
No antibiotic therapy |
Multiple agents (individually or in combination) are mentioned without specific doses or duration recommended |
