Overview
- Definition
A multisystem disease caused by Borrelia burgdorferi sensu lato, a group of spirochetes transmitted by Ixodes ticks
Characterized by skin, musculoskeletal, neurologic, ocular, and cardiac manifestations
Symptoms
Varies widely depending on stages of diseaseConjunctivitis: redness
Episcleritis: redness, irritation
Keratitis: photophobia, blurry vision
Intraocular inflammation: photophobia, redness, blurry vision, floaters
Optic nerve involvement: visual field and color deficits
Orbital inflammation: pain, swelling, diplopia
Cranial nerve palsy (CN 4, 5, 6, and 7): diplopia, facial weakness
- Laterality
Typically bilateral
Course
Applies to both systemic and ocular manifestationsEarly localized stage: self-limited over 3–4 weeks
Early disseminated stage: days to weeks after tick bite
- Late disseminated stage: months to years after tick bite
Note: Lyme disease can remain silent for months to years between tick bite and disseminated stages; some patients may never have disseminated disease.
- Age of onset
All age groups affected
- Gender/race
Slight male predominance
More common during warmer months
- Reported regularly in North America, Europe, and Asia
USA: Northeast, Mid-Atlantic, and upper Midwest
Europe: More common in Eastern Europe (highest prevalence in Austria and Slovenia)
- Systemic association
- Early localized stage
- Erythema migrans (also called erythema chronicum migrans) (70–80%)
Average of 7–10 days after tick bite
At least 5 cm diameter, and may expand by 1 cm/day (up to 20–30 cm)
Fever, malaise, fatigue, myalgia, and arthralgia
- Early disseminated stage
- Skin
Erythema chronicum migrans
Borrelial lymphocytoma (rare in the USA, more common in Europe)
- Central and peripheral nervous systems (30–40%)
- Cranial neuropathy
Facial nerve is most commonly involved (1/3 of cases are bilateral, which help differentiate from idiopathic Bell’s palsy); usually resolves with or without antibiotics, but chance of Lyme arthritis is much higher in untreated patients
Motor and sensory radiculopathy
Encephalitis/myelitis
- Cardiac (<5%)
AV (atrioventricular) block of different degrees
Others: myocarditis, pericarditis
- Late disseminated stage
- Joints (80%)
Chronic or recurrent mono- or oligoarthritis, with each episode lasting days to months
Can affect smaller joints, but general predilection for large joints, especially the knee
Often asymmetric
May be the only Lyme manifestation in children
- Neurologic disease
Peripheral nervous system: cranial neuropathies, radiculoneuritis
Central nervous system: meningitis, encephalomyelitis, benign intracranial hypertension, encephalopathy
- Acrodermatitis chronica atrophicans
Bluish-red lesions found on extremities of older females; eventually wrinkled and atrophic
Seen in European cases
Exam: Ocular
- Early localized stage
Follicular conjunctivitis
Episcleritis
- Early disseminated stage
Uveitis affecting any segment, but intermediate uveitis with significant vitritis is most common
Retinitis and retinal vasculitis
Exudative RD (retinal detachment)
Optic neuritis/papillitis
Neuroretinitis
Cranial neuropathy (may affect multiple cranial nerves)
Papilledema due to meningitis and increased intracranial pressure
Pupillary abnormalities (Horner’s syndrome, tonic pupil, mydriasis)
Orbital inflammation
- Late disseminated stage
Episcleritis
Keratitis (bilateral patchy and nebular subepithelial and stromal infiltration)
Chronic uveitis
Exam: Systemic
- Skin
Erythema chronicum migrans: reddish round rash, which enlarges with central clearing (bull’s eye or target lesion), without itching or pain, but may have some warmth. Diameter of at least 5 cm; expands by 1 cm/day up to 20–30 cm
Borrelial lymphocytoma (aka lymphadenosis benigna cutis): bluish-red lesions with predilection for earlobes in children and nipples in adults; rare in the USA, more common in Europe
Acrodermatitis chronica atrophicans: more often found on extremities of older females, bluish-red lesions that eventually become wrinkled and atrophic; rare in the USA, more common in Europe
Cardiac: bradycardia
- Joints
Asymmetric knee or other joint swelling and erythema
- CNS
Facial palsy: 1/3 of Lyme-related facial palsy is bilateral: a crucial distinction from idiopathic Bell’s palsy, which tends to be unilateral
Multifocal asymmetric weakness
Decreased vibratory sensation of the lower distal extremities
Imaging
Due to the varying ocular presentations, there is no specific ocular imaging that would be particularly helpful in differentiating Lyme from other uveitic entities
Laboratory and Radiographic Testing
- ELISA screening for serum Lyme antibodies, with confirmatory Western blot:
Both may be negative in the initial 2–4 weeks after infection as it takes time for antibodies to develop
Differential Diagnosis
Coinfection with Babesia and Anaplasma should be ruled out in patients with ongoing nonspecific symptoms despite appropriate treatment for Lyme disease, or in the presence of anemia, leukopenia, and or thrombocytopenia
- DDx of erythema migrans:
Insect bite hypersensitivity: usually more rapid onset (within hours), shorter duration, and smaller size
STARI (Southern tick-associated rash illness following the bite of the Lone Star tick-Amblyomma)
Contact dermatitis
Bacterial cellulitis
Granuloma annulare
Hyperkeratotic disorder
Juvenile idiopathic arthritis.
Rheumatoid arthritis (symmetric joint involvement; more likely to involve smaller joints than Lyme)
Systemic lupus erythematosus.
Treatment
- Based on IDSA (Infectious Disease Society of America) guidelines, doxycycline prophylaxis is recommended only if
Attached tick is identified as an adult or nymphal Ixodes scapularis (deer) tick.
Tick is estimated to have been attached for ≥36 hours (based upon how engorged the tick appears or the amount of time since outdoor exposure).
Antibiotic can be given within 72 hours of tick removal.
Local rate of tick infection with B. burgdorferi is ≥20% (known to occur in parts of New England, parts of the mid-Atlantic states, and parts of Minnesota and Wisconsin).
Patient can safely take doxycycline (e.g., not pregnant or breastfeeding; not child under 8 years of age).
If the person meets ALL of the above criteria, the recommended dose of doxycycline is a single dose of 200 mg for adults and 4 mg/kg, up to a maximum dose of 200 mg, in children ≥8 years.
- Early disease/erythema migrans
Doxycycline 100 mg BID for 10–21 days
Alternatives: amoxicillin 500 mg TID or cefuroxime 500 mg BID for 14–21 days
- Disseminated disease
Doxycycline 100 mg BID for 14–28 days
Alternatives: amoxicillin 500 mg TID or cefuroxime 500 mg BID for 14–28 days
- Neurological involvement (including ocular disease involving the posterior segment): may need IV (intravenous) therapy; in Europe, oral antibiotics appear to be as effective as IV therapy for meningitis. In the USA, IV therapy is used more commonly
Ceftriaxone 2 g once daily
Cefotaxime 2 g Q8H
Penicillin G 18–24 MU/day divided Q4H
Topical corticosteroids for nummular keratitis and anterior uveitis, and oral corticosteroids for posterior segment inflammation, once proper antibiotic has been started
Referral/Comanagement
Infectious Disease
Rheumatology
Dermatology
Neurology