Lyme Disease: Classification

Classification of Lyme disease into three stages

Note: The disease manifests itself differently from individual to individual, i.e., it can occur with any of the various early or late manifestations!

Stage Designation Time (p. i.) Description
I Early Lyme disease 1-5 weeks
  • Erythema migrans (wandering redness) or erythema chronicum migrans (89-95.4%).
  • Lymphadenosis cutis benigna Bäfverstedt (Borrelia lymphocytoma) (2%).
II Intermediate Lyme disease Weeks to months Disseminated infection (spread through the organism; multiorgan disease):

  • Fever, muscle and joint pain, diffuse headache, possibly adenopathy, multifocal erythema;
  • Carditis (inflammation of the heart) with cardiac arrhythmias and AV blockages.
  • Early neuroborreliosis (acute neuroborreliosis) (Lyme neuroborreliosis): painful meningoradiculitis (meningitis with inflammation of adjacent spinal nerve roots) (synonym: Bannwarth syndrome) (3%)Onset: approximately 3-6 weeks after primary infection with:
    • Cranial nerve palsies (cranial nerves): facial nerve palsy with unilateral drooping of the corner of the mouth (bilateral facial nerve palsy is associated with Lyme disease in approximately 96 percent of cases) and abducens nerve
    • Radicular (“originating from the nerve roots”) pain, especially at night; often multilocular (“many places”) and migratory
    • Inflammatory cerebrospinal fluid syndrome
  • Temporary blindness in children due to pressure on the optic nerve (optic nerve).
  • Lyme arthritis (joint inflammation; mid to late manifestation) – in the early phase, transient and migratory arthralgia (joint pain); later, the actual Lyme arthritis (as mono- or oligoarthritis/joint inflammation in less than 5 joints); usually large joints such as the knee joint are affected Manifestation: late phase of disease (several weeks to months/possibly up to two years after pathogen transmission).
  • Lymphadenosis cutis benigna Bäfverstedt (Borrelia lymphocytoma).
III Late Lyme disease Months to years Persistent (continuing) infection:

  • Lyme arthrtis (as monoarthritis or oligoarthritis) (5%).
  • Late neuroborreliosis (chronic neuroborreliosis):
  • Acrodermatitis chronica atrophicans Herxheimer (ACA) – inflammatory skin disease of the ends of the body; triad skin atrophy (thinning of the skin; cigarette paper thin), homogeneous reddish (to livid) coloration of the skin and increased vascular markings (1%).
    • Predilection sites (body regions where the disease occurs preferentially): dorsum of hands and feet, elbows and knees.