Lyme Disease: Erythema chronicum migrans

Discussion

• deer tick (Ixodes) borne spirochetal infection leading to widespread symptoms;
  • in U.S. Lyme disease is most prevalent in areas with heavy deer population (North East);
  • life cycle includes larval, nymph, and adult (with the nymphal stage transmitting bacteria);
• infection is due to Borrelia burgdorferi spirochetes;
  • contains several outer surface proteins that allow organism to survive in different host environments;
  • tick takes up to 24 hours to attach and then takes 48 hours or longer for Borrelia burgdorferi to proliferate within tick and
    then spread back into host;
  • in Europe it is caused by B. afzelii, B. garinii, B. burgdorferi, and occasionally by other species of borrelia;
• less than 1% of tick bites result in Lyme disease;

Clinical Features

• acute severe joint pain:
  • ref: Primary Lyme Arthritis of the Pediatric Hip
• erythema chronicum migrans:
  • will occur in 80% of patients with Lyme disease;
  • rash occurs one week to one month after tick bite in 50-70% of patients;
  • look for expanding red papule or macule, most often greater than 5 cm in diameter;
  • most common in the inguina, axilla, behind the knee;
  • partial or complete central clearing may develop which accounts for the bullseye appearance;
  • rash often fades spontaneously after 4 weeks;
  • references:
    • Review: Diagnosis of Lyme Disease Based on Dermatologic Manifestations.
    • Images in Clinical Medicine: Disseminated Lyme Disease
• constitutional symptoms
  • flu like symptoms: fatigue, headache, myalgias, and arthralgias;
• neurologic symptoms
  • neurologic symptoms may occur early or late;
  • look for headache, neck stiffness, facial palsy, encephalitis;
  • may see subacute encephalopathy, axonal polyneuropathy, and leukoencephalitis;
  • may see pure motor neuron disease which may be confused with amyotrophic lateral sclerosis
  • references:
    • Immunologic reactivity against Borrelia burgdorferi in patients with motor neuron disease.
    • Lyme disease and the peripheral nervous system.
    • Peripheral neuropathies after arthropod stings not due to Lyme disease: a report of five cases and review of the literature.
• carditis
• acute or chronic arthritis
  • 10% of patients will have chronic arthritis;
  • with chronic arthritis, consider PCR testing of joint fluid in order to rule out a persistent low grade infection;
  • references:
    • Chronic arthritis of the knee in Lyme disease. Review of the literature and report of two cases treated by synovectomy.
    • Lyme Arthritis Presenting as Acute Septic Arthritis in Children.
  • respiratory and GI complaints are notably absent;

diff dx

• adult arthritis
• childhood arthritis
• sepic knee
• references: Predictive Factors for Differentiating Between Septic Arthritis and Lyme Disease of the Knee in Children

Labs

• culture:
  • B.Burgodorferi can be cultured on Barbour-Stoenner-Kelly medium, early in the disease, usually from erythema
    migrans lesions;
• PCR: may allow organism identification from joint fluid (and if necessary from spinal fluid);
• antibody response (serum lyme titer) helps make the diagnosis;
  • ELISA is sensitive but not specific, where as the Western blot is specific;
  • in the first several weeks, there is an IgM response (which is persistent), followed by an IgG response after one month;
  • references:
    • NEJM lab diagnosis table
    • Prospective study of serologic tests for lyme disease

Prevention

• vaccines have be developed which mimic the B burgdorferi outer-surface lipoprotein A (OspA);
• w/ repeated dosing, efficacy of over 90% has been reported;
•  traditionally we were taught that outside of the Northeast this disease is uncommon, but the disease may actually more common
  than reported;
• references:
  • A vaccine consisting of recombinant Borrelia burgdorferi outer-surface protein A to prevent Lyme disease. Recombinant Outer-Surface Protein A Lyme Disease Vaccine Study Consortium.
  • Vaccination against Lyme disease with recombinant Borrelia burgdorferi outer-surface lipoprotein A with adjuvant. Lyme Disease Vaccine Study Group.
  • Immunization against Lyme disease - an important first step.

Treatment

• oral antibiotic treatment is sufficient for mild symptoms but IV antibiotics (ceftriaxone) is required with neurologic symptoms,
  carditis, or resistant arthritis;
• doxycycline 100 mg PO bid for 2-3 weeks;
  • avoid use in children younger than 8 years;
• tetracycline
• penicillins:
  • penicillin v (1st choice in child & pregnancy)
  • amoxicillin 500 mg PO tid;
• erythromycin
• A. atrophicans:
  • penicillin g (20 million units/day for 10 days;
  • alternatively, consider ceftriaxone 1gm IV q12hr for 14 days;
  • relapses should be retreated;
  • stage ii: neurologic or cardiac, stage iii: arthritis;
  • for the later three rx with ceftriaxone or penicillin g (hd);

length of treatment

• proper length of treatment remains controversial, but it is clear that treatment needs to be continued far beyond the resolution
  of the acute symptoms;
• references:
  • Safety of intravenous antibiotic therapy in patients referred for treatment of neurologic Lyme disease
  • Counterpoint: long-term antibiotic therapy improves persistent symptoms associated with lyme disease
  • Two controlled trials of antibiotic treatment in patients with persistent symptoms and a history of Lyme disease

References

• Medical Progress: Lyme Disease.
• Lyme arthritis in children. An orthopaedic perspective.
• Lyme Disease.
• A critical appraisal of "chronic Lyme disease".
• Borrelia Being Bashful
• Lyme Arthritis in Children Presenting with Joint Effusions
• Commentary on an article by Matthew D. Milewski, MD, et al.: "Lyme Arthritis in Children Presenting with Joint Effusions".
• Effective treatment of Lyme disease-related arthritis may depend on proper diagnosis