Case definitions for the clinical forms of Lyme disease

source: Stanek G, Clin Microbiol Infect, 2011 a If the lesion diameter is <5 cm, a recent history of a tick bite, an incubation period (after the bite) of at least 2 days, and a rash with an increasing diameter at the bite site are mandatory criteria for establishing the diagnosis. b Initial and follow-up samples must be tested in parallel to avoid inter-assay variability. c In early cases, the intrathecal synthesis of specific antibodies may still be absent at the time of the initial sample collection.

  

Clinical case definition 

Biological diagnosis: essential 

Biological/clinical evidence: supportive of the diagnosis 

Erythema migrans (EM) 

Ring-shaped erythema (≥ 5 cm in diameter)a, with or without a clear center, spreading outward, with peripheral elevation 

None 

Detection of Borrelia burgdorferi sensu lato (Bb sl) by culture and/or PCR on skin biopsy 

Borrelia lymphocytoma 

Painless erythematous or bluish-purple nodule, usually on the earlobe or helix, nipple, or scrotum; more common in children (particularly on the ear). 

Seroconversion or positive serology.
Histological confirmation for doubtful cases 

Histological confirmation.
Detection of Bb sl by culture and/or PCR on skin biopsy. Recent or concurrent
EM 

Chronic atrophic acrodermatitis (ACA) 

Erythematous or bluish-purple lesions with a long course, usually on the extensor surfaces of the extremities. Edematous, "doughy" infiltrates. Lesions may eventually become atrophic. Possible skin induration and fibromatous nodules near large joints. 

Specific IgG serology with elevated levels 

Histological confirmation.
Detection of Bb sl by culture and/or PCR on skin biopsy 

Lyme neuroborreliosis 

In adults, primarily meningoradiculitis, meningitis; rarely encephalitis, myelitis; very rarely cerebral vasculitis.
In children, primarily meningitis and facial paralysis 

Pleocytosis with lymphocytosis in CSF and/or intrathecal synthesis of specific anti-Bb sl c antibodies 

Detection of Bb sl by culture and/or PCR on CSF.
Intrathecal synthesis of total IgM, and/or IgG, and/or
IgA. Specific anti-Bb antibodies in serum Recent or concurrent
EM 

Lyme arthritis 

Arthritis affecting one or a few large joints; possibility of recurrence
Other causes must be ruled out. 

Specific IgG serology 

Analysis of synovial fluid or tissue:
Detection of Bb sl by PCR and/or culture in synovial fluid and/or synovial tissue 

Cardiac manifestations (rare) 

Atrioventricular conduction disorders (AV block I–III), arrhythmias, occasionally myocarditis or pancarditis. Other
causes must be ruled out. 

Serology with specific antibodies 

Detection of Bb sl by culture and/or PCR on myocardial biopsy. Recent or concurrent
EM and/or neurological manifestations 

Ophthalmological manifestations (rare) 

Conjunctivitis, uveitis, papilledema, episcleritis, keratitis 

Serology with specific antibodies 

Recent or concurrent manifestations of Lyme borreliosis. Detection of Bb sl by culture and/or PCR in the vitreous