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ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferencesInformation for PatientsView all Images (71)
Lyme disease in Adult
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Lyme disease in Adult

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Contributors: Andrew Walls MD, Susan Burgin MD
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Synopsis

Lyme disease, with cutaneous manifestations including erythema migrans, acrodermatitis chronica atrophicans (Europe), and Borrelia lymphocytoma, is an immune-mediated inflammatory disease resulting from infection with the spirochete Borrelia burgdorferi sensu lato, composed of 4 distinct genospecies: Borrelia burgdorferi sensu stricto, Borrelia garinii, Borrelia afzelii, and candidatus Borrelia mayonii. Disease usually begins with a slowly expanding skin lesion – erythema migrans (EM) – at the site of the tick bite (early localized disease).

Early disseminated disease occurs within weeks as the spirochete disseminates to the nervous system, heart, joints, and other organs. At this time, patients may develop multiple widespread skin lesions of disseminated EM along with acute neurologic abnormalities, atrioventricular block (AV), or myocarditis. Months later, untreated patients may progress to late stage disease that manifests with arthritis, mild encephalopathy, neuropathies, and, in Europe, acrodermatitis chronica atrophicans.

In the United States, Lyme disease is primarily seen in New England, the Midwest states, and the west coast. It is endemic to most of Europe. The ticks that transmit Lyme disease are of the genus Ixodes. Mice and deer are the major animal reservoirs. Transmission occurs most commonly in the spring and summer months. Increased risk of infection is strongly linked to the amount of time spent in wooded or rural areas.

Lyme disease has been rarely reported in China, Japan, and Russia. Although Lyme disease is not considered endemic to countries in Africa, there have been few cases of infection reported.

Lyme disease is subdivided clinically into 3 phases:
  1. Early localized disease
  2. Early disseminated disease
  3. Late disease
Systemic Symptoms:
Systemic symptoms are seen in approximately half of patients with early localized or early disseminated disease. These symptoms are described as flu-like and include a combination of fatigue, headache, neck stiffness, myalgias / arthralgias, lymphadenopathy, or fever. With B. mayonii infection, which at present seems to be limited to the upper midwestern United States, nausea and vomiting may also occur, and the rash may be more diffuse.

Early Localized (days to weeks following tick bite):
  • EM at the site of the tick bite develops in approximately 60%-90% of patients.
  • In Europe, early lesions sometimes present as Borrelia lymphocytomas.
  • There may or may not be systemic symptoms.
  • False negative serological testing is common.
Early Disseminated (weeks to months following tick bite):
  • Multiple widespread skin lesions represent dissemination of the infection with or without systemic symptoms.
  • Approximately 10% of patients develop neurologic features including meningitis and cranial or peripheral neuropathies. Facial nerve palsy (Bell Palsy) is the most commonly associated cranial nerve neuropathy.
  • Approximately 5% of patients may experience cardiac manifestations, usually AV block or myocarditis.
  • Serological testing is usually positive, but false negatives may still occur.
Late (months to few years):
Untreated cases can also lead to:
  • Chronic arthritis (typically knees)
  • Mild encephalopathy with subtle cognitive deficits
  • Axonal polyneuropathies
  • Acrodermatitis chronicum atrophicans (Europe)
  • Serological testing is virtually always positive.
"Chronic" Lyme Disease or Post-Lyme Disease Syndrome:
A small percentage of patients diagnosed with Lyme disease who received adequate therapeutic treatment have reported ongoing nonspecific symptoms that typically improve within one year. Persistent infections in humans despite treatment have not been reliably demonstrated. Despite extensive study of this entity, numerous expert reviews have concluded that no consistent, reproducible syndrome or evidence of persistent infection resulting in these vague symptoms exists. However, primary reinfection has been demonstrated in individuals in endemic areas.

Related topic: Lyme Keratitis

Codes

ICD10CM:
A69.20 – Lyme disease, unspecified

SNOMEDCT:
23502006 – Lyme disease

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Differential Diagnosis & Pitfalls

Early:
  • Southern tick-associated rash illness (STARI) clinically presents with EM but is transmitted by the lone star tick Amblyomma americanum.
  • Erythema annulare centrifugum has a fine collarette of scaling inside the border of the lesion (trailing scale).
  • Arthropod bite reaction develops rapidly after bite and does not demonstrate a flat, slowly expanding lesion.
  • Arthropod bite granuloma can be firm and typically does not expand.
  • Fixed drug reactions do not expand and are often <5 cm.
  • Tinea corporis – Associated lesions have overlying scale.
  • Contact dermatitis may develop vesicles or bullae within lesions but it is typically more symptomatic and less symmetric.
  • Erysipelas / cellulitis has a more rapid onset with tender, streaking, or raised erythema as it expands from the site of initial infection.
Early Disseminated:
Multiple Lesions
  • Erythema multiforme lesions are more numerous, smaller, duskier, and are symmetrically distributed, favoring the hands, face, and forearms. Oral mucositis or crusting may be present. It is associated with concomitant herpes or mycoplasma infection.
  • Secondary syphilis can have a characteristic "rust" color and overlying scale.
  • Pityriasis rosea presents with a herald patch and thin, raised, scaly plaques in characteristic "fir tree" distribution over the trunk.
  • Urticarial lesions are edematous, pruritic, and typically resolve within 24 hours.
  • Pseudolymphoma (lymphocytoma) may result from a number of stimuli, including arthropod bites, that cause an inflammatory reaction pattern in the skin leading to the formation of nodular lesions clinically and histologically resembling cutaneous lymphoma. Borrelia lymphocytoma is a subset of pseudolymphomas.
  • Nodular scabies is more common in the groin and extremely pruritic.
  • Carcinoma erysipeloides presents as an ill-defined, jagged, light pink plaque typically overlying the breast or upper chest.
  • Granuloma faciale is more raised, edematous, and restricted to the face.
Late (Acrodermatitis Chronica Atrophicans):
  • Circulatory insufficiency with dependent rubor.
  • Eosinophilic fasciitis is rare but favors the distal extremities and produces woody induration of the skin but typically stops near the wrist or metacarpal joints and does not involve the distal hands or fingers.
  • Dermatomyositis has a characteristic heliotrope rash; atrophic dermal papules of dermatomyositis (formerly called Gottron papules) over dorsal distal interphalangeal (DIP), proximal interphalangeal (PIP), metacarpophalangeal (MCP) joints; and a "shawl" sign.
  • Morphea
  • Atrophoderma of Pasini and Pierini

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Last Updated: 10/27/2016
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Lyme disease in Adult
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Lyme disease (Early Localized, B. burgdorferi) : Annular, Malaise, Tick bite, Bulls-eye, Blanching patch, Myalgia, Low grade fever
Clinical image of Lyme disease
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