Lyme borreliosis (disseminated and late infection)

Last Updated: 2023-07-07

Author(s): Anzengruber F., Navarini A.

ICD11: 1C1G.1Z

Disseminated and late infection of Lyme disease.

  • Mostly found in Europe and America.
  • Up to 30-40% of all ticks are infected with Borrelia. 
  • In Switzerland, approx. 10000 people contract Lyme disease per year.
  • In Germany, an incidence of 100-150 infections/100000/year is assumed.
  • Seasonally, infections occur from May-July.

  • Pathogen
    • Borrelia burgdorferi sensu lato (Europe).
    • Borrelia burgdorferi sensu stricto (North America).

3 stages

  • Stage I (localised early infection of the skin).
  • Stage II (disseminated early infection).
  • Stage III (late infection with organ manifestation).
  • Stage I:
    • Erythema chronicum migrans.
    • Ggl. pruritus.
    • Borrelia lymphocytoma.
    • Stage II:
      • Partially asymptomatic.
      • In some cases, flu-like symptoms begin only a few days after the onset of erythema chronicum migrans.
      • Locoregional lymph node adenopathy.
      • If applicable, reduced general condition, febrile temperatures, cephalgias.
      • Multiple erythema migrantia.
      • Unspecific exanthema.
      • Borrelia lymphocytoma.
      • Meningopolyneuritis Garin-Bujadoux-Bannwarth: cephalgias, possibly meningitis or encephalitis, unilateral radiculitis (painful paralyses) and cranial nerve palsies.
      • Arthralgias, myalgias, myo-/pericarditis.
      • Iritis, chrioretinitis.
      • Carditis.
    • Stage III
      • Acrodermatitis chronica atrophicans.
      • Acrodermatitis acuta.
      • Malignant B-cell lymphoma.
      • Peripheral neuropathy.
      • Subacute encephalopathy.
      • Chronic encephalomyelitis.
      • Recurrent chronic arthritis (arthritides are typical in patients from the USA).
      • Fibromyalgias.
      • Chronic pain syndrome.
      • Neurological symptoms are observed mainly in Europe.
      • Acrodermatitis chronica atrophicans (leading symptom). 
      • Nodular Panniculitis (rare).
      • Early summer meningoencephalitis in the context of a double infection (very rare).
      • Progressive encephalomyelitis.
      • CAVE: Stages can be skipped.
      • Post-infectious Lyme disease syndrome (post Lyme disease):
      • Persistent feeling of illness with myalgias and arthralgias after symptoms have subsided and adequate antibiotic therapy.

         

  • Anamnesis: Tick bite remembered? Erythema migrans remembered?
  • In about 60% of patients, circulating immune complexes can also be detected.
  • Borrelia serology (antibodies are not detectable from the beginning).
  • IgM: Detectable after 2 weeks in 50% of patients, after >4 weeks in 80%.
  • IgG: Detectable after 2-3 months.
  • Clinical>pathogen detection (serum, CSF, synovial fluid, tissue)
  • Blood culture.
  • If necessary, CSF puncture (CSF serum index).
  • If necessary. Detection of Borrelia in joint fluid.
  • If necessary, biopsy with pathogen detection.
  • Stage II: long-term ECG, neurological presentation.

Plasma cell-rich, perivascular infiltrates are typical.

  1. Åsbrink E , Hovmark A. Classification, geographic variations, and epidemiology of Lyme borreliosis. Clinics in Dermatology 1993;11:353-7. 
  2.  Aslanzadeh J, Helm KF, Espy MJ, Muller SA , Smith TF. Detection of HSV-specific DNA in biopsy tissue of patients with erythema multiforme by polymerase chain reaction. Br J Dermatol 1992;126:19-23. 
  3.  Baranton G, Postic D, Saint Girons I, Boerlin P, Piffaretti JC, Assous M et al. Delineation of Borrelia burgdorferi Sensu Stricto, Borrelia garinii sp. nov., and Group VS461 Associated with Lyme Borreliosis. International Journal of Systematic Bacteriology 1992;42:378-83. 
  4.  Berglund J, Eitrem R, Ornstein K, Lindberg A, Ringnér Å, Elmrud H et al. An Epidemiologic Study of Lyme Disease in Southern Sweden. New England Journal of Medicine 1995;333:1319-24. 
  5.  Bezold G, Lange M, Schiener R, Palmedo G, Sander CA, Kerscher M et al. Hidden scabies: diagnosis by polymerase chain reaction. British Journal of Dermatology 2001;144:614-8. 
  6.  Bhate C , Schwartz RA. Lyme disease. Journal of the American Academy of Dermatology 2011;64:619-36. 
  7.  Bratton RL, Whiteside JW, Hovan MJ, Engle RL , Edwards FD. Diagnosis and Treatment of Lyme Disease. Mayo Clinic Proceedings 2008;83:566-71. 
  8.  Bunikis J , Barbour AG. Laboratory testing for suspected lyme disease. Medical Clinics of North America 2002;86:311-40. 
  9.  Burgdorfer W, Barbour A, Hayes S, Benach J, Grunwaldt E , Davis J. Lyme disease-a tick-borne spirochetosis? Science 1982;216:1317-9. 
  10.  Darragh TM, Egbert BM, Berger TG , Benedict Yen TS. Identification of herpes simplex virus DNA in lesions of erythema multiforme by the polymerase chain reaction. Journal of the American Academy of Dermatology 1991;24:23-6. 
  11.  Dattwyler RJ, Luft BJ, Kunkel MJ, Finkel MF, Wormser GP, Rush TJ et al. Ceftriaxone Compared with Doxycycline for the Treatment of Acute Disseminated Lyme Disease. New England Journal of Medicine 1997;337:289-95. 
  12.  Dumler JS. Molecular Diagnosis of Lyme Disease: Review and Meta-analysis. Molecular Diagnosis 2001;6:1-11. 
  13.  Gross DM. Identification of LFA-1 as a Candidate Autoantigen in Treatment-Resistant Lyme Arthritis. Science 1998;281:703-6. 
  14.  Hagari Y, Mihara M , Hagari S. Nodular localized cutaneous amyloidosis: detection of monoclonality of infiltrating plasma cells by polymerase chain reaction. Br J Dermatol 1996;135:630-3. 
  15.  Hengge UR, Tannapfel A, Tyring SK, Erbel R, Arendt G , Ruzicka T. Lyme borreliosis. The Lancet Infectious Diseases 2003;3:489-500. 
  16.  Hercogova J. Borrelia burgdorferi: a protagonist in Lyme disease, a bystander in morphoea? Journal of the European Academy of Dermatology and Venereology 2002;16:98-9. 
  17.  Kang D, Ran Y, Li C, Dai Y , Lama J. Impetigo-Like Tinea Faciei Around the Nostrils Caused by Arthroderma vanbreuseghemii Identified Using Polymerase Chain Reaction-Based Sequencing of Crusts. Pediatric Dermatology 2012;30:e136-e7. 
  18.  Klempner MS, Hu LT, Evans J, Schmid CH, Johnson GM, Trevino RP et al. Two Controlled Trials of Antibiotic Treatment in Patients with Persistent Symptoms and a History of Lyme Disease. New England Journal of Medicine 2001;345:85-92. 
  19.  Kramer N, Rickert RR, Brodkin RH , Rosenstein ED. Septal panniculitis as a manifestation of lyme disease. The American Journal of Medicine 1986;81:149-52. 
  20.  Lassalle S, Selva E, Hofman V, Butori C, Vénissac N, Mouroux J et al. Sarcoid-like lesions associated with the immune restoration inflammatory syndrome in AIDS: absence of polymerase chain reaction detection of Mycobacterium tuberculosis in granulomas isolated by laser capture microdissection. Virchows Archiv 2006;449:689-96. 
  21.  Masters EJ, Grigery CN , Masters RW. STARI, or Masters Disease: Lone Star Tick–Vectored Lyme-like Illness. Infectious Disease Clinics of North America 2008;22:361-76. 
  22.  Moylett EH , Demmler GJ. Polymerase Chain Reaction for the Detection of Human Parvovirus B19 Infections in Children. Pediatr Res 1999;45:168A-A. 
  23.  Nachbar F, Classen V, Nachbar T, Meurer M, Schirren CG , Degitz K. Orificial tuberculosis: detection by polymerase chain reaction. Br J Dermatol 1996;135:106-9. 
  24.  Nadelman RB, Nowakowski J, Fish D, Falco RC, Freeman K, McKenna D et al. Prophylaxis with Single-Dose Doxycycline for the Prevention of Lyme Disease after an Ixodes scapularis Tick Bite. New England Journal of Medicine 2001;345:79-84. 
  25.  Nihtyanova SI, Parker JC, Black CM, Bunn CC , Denton CP. A longitudinal study of anti-RNA polymerase III antibody levels in systemic sclerosis. Rheumatology 2009;48:1218-21. 
  26.  Powledge TM. The polymerase chain reaction. AJP: Advances in Physiology Education 2004;28:44-50. 
  27.  Ramamoorthi N, Narasimhan S, Pal U, Bao F, Yang XF, Fish D et al. The Lyme disease agent exploits a tick protein to infect the mammalian host. Nature 2005;436:573-7. 
  28.  Silberer M, Koszik F, Stingl G , Aberer E. Downregulation of class II molecules on epidermal Langerhans cells in Lyme borreliosis. British Journal of Dermatology 2000;143:786-94. 
  29.  Silverstein TD, Johnson RE, Jain R, Prakash L, Prakash S , Aggarwal AK. Structural basis for the suppression of skin cancers by DNA polymerase η. Nature 2010;465:1039-43. 
  30.  Steere AC. Lyme Disease. New England Journal of Medicine 2001;345:115-25. 
  31.  Steere AC. Lyme borreliosis in 2005, 30 years after initial observations in Lyme Connecticut. Wiener klinische Wochenschrift 2006;118:625-33. 
  32.  Wood GS, Tung RM, Heaffner AC, Crooks CF, Liao S, Orozco R et al. Detection of Clonal T-Cell Receptor γ Gene Rearrangements in Early Mycosis Fungoides/Sezary Syndrome by Polymerase Chain Reaction and Denaturing Gradient Gel Electrophoresis (PCR/DGGE). Journal of Investigative Dermatology 1994;103:34-41. 
  33.  Woodgate R. A plethora of lesion-replicating DNA polymerases. Genes & Development 1999;13:2191-5. 
  34.  Wormser GP. Duration of Antibiotic Therapy for Early Lyme Disease. Annals of Internal Medicine 2003;138:697. 
  35.  Wormser GP. Early Lyme Disease. New England Journal of Medicine 2006;354:2794-801. 
  36.  Wormser Gary P, Liveris D, Nowakowski J, Nadelman Robert B, Cavaliere LF, McKenna D et al. Association of Specific Subtypes of Borrelia burgdorferi with Hematogenous Dissemination in Early Lyme Disease. The Journal of Infectious Diseases 1999;180:720-5. 
  37.  Wormser Gary P, Nadelman Robert B, Dattwyler Raymond J, Dennis David T, Shapiro Eugene D, Steere Allen C et al. Practice Guidelines for the Treatment of Lyme Disease. Clinical Infectious Diseases 2000;31:i-i. 
  38.  Lebwohl, Mark. Treatment of Skin Disease: Comprehensive Therapeutic Strategies. Elsevier, 2014. Print. 
  39. Bundesamt für Gesundheit - Borreliose / Lyme-Krankheit. (2016). Bag.admin.ch. Retrieved 24 May 2016, from http://www.bag.admin.ch/themen/medizin/00682/00684/01017/