Epidemiology

Currently, Lyme disease is a global illness. Cases have been reported on all continents except Antarctica. Since its original description more than 25 years ago, Lyme disease has become the most commonly reported (95%) vector-borne illness in the United States. This infection has emerged as a major health hazard for human beings and domestic animals. In 2011, it was the sixth most common nationally notifiable disease. It is endemic in more than 15 states in the United States and in Europe and Asia.

In some patients, Lyme disease may be transitory and of little consequence, but in others it may become chronic and severely disabling. Accurate diagnosis is therefore essential, although better laboratory techniques are still needed.

Retrospectively, the first symptom of Lyme disease apparently was recognized as early as 1908 in Sweden. In the decades that followed, the rash produced by the disease erythema chronicum migrans (ECM) was noted elsewhere in Europe, as were other symptoms that seemed to follow ECM’s eruption. Secondary symptoms, such as impairment of the nervous system, were described in France, Germany, and again in Sweden.

In the United States the European rash was almost unknown until 1969, when a case of a physician bitten by a tick while hunting in Wisconsin was reported. Although a few ECM cases were seen in Americans who had traveled to Europe, there were no further native American cases until 1975, when physicians at the U.S. Navy base in Groton, Connecticut, reported seeing four patients with a rash similar to that of ECM. At the same time, an epidemiologist at the Connecticut State Department of Health and a rheumatologist at Yale University were notified of an unusual cluster of cases of arthritis occurring in children in Lyme, Connecticut.

It was not until 1982 that Burgdorfer and Barbour isolated a previously unrecognized spirochete, now called B. burgdorferi, from I. scapularis ticks, and Lyme disease became a recognized vector-borne, infectious disease. Two factors influence the chance that a bitten patient will contract the disease, the likelihood that local ixodid ticks carry the Lyme spirochete and the likelihood of infection after a bite by an infected tick. The probability of infection after an ixodid tick bite in an area of endemic disease is about 3%, but varies in different regions from less than 1% to as high as 5%. It has been suggested that human leukocyte antigen (HLA)–DR4 (HLA-DR4) and, secondarily, HLA-DR2, may increase the risk that Lyme arthritis will become chronic and fail to respond to antibiotics.

Lyme disease does not occur nationwide and is concentrated heavily in the northeast and upper midwest. The highest number of confirmed cases of Lyme disease to date was 29,959 in 2009 (Fig. 19-3). Persons of all ages and both genders are equally susceptible. In 2011, 96% of Lyme disease cases were reported from 13 states (Fig. 19-4):

Lyme disease is considered an emerging infectious disease because of the impact of changing environmental and socioeconomic factors, such as the transformation of farmland into suburban woodlots favorable for deer and deer ticks. Although pets may represent a spirochete reservoir, it is unlikely that humans can be infected directly by them. In areas of endemic Lyme disease, however, both adult and nymphal ticks, carried into the household by dogs and cats, may infect humans.

Signs and Symptoms

The basic features of Lyme disease are similar worldwide, but there are regional variations, primarily between the illness in America and that in Europe and Asia. In at least 60% to 80% of U.S. patients, Lyme disease begins with a slowly expanding skin lesion, EM, which occurs at the site of the tick bite. The skin lesion is frequently accompanied by flulike symptoms.

The Centers for Disease Control and Prevention (CDC) clinical case definition for Lyme disease includes the presence of EM or at least one objective, late manifesting sign of musculoskeletal, neurologic, or cardiovascular disease and a positive serologic test for antibodies to B. burgdorferi. Many misdiagnosed patients actually have chronic fatigue syndrome or fibromyalgia, both of which can cause similar symptoms, such as joint stiffness or pain, fatigue, and sleep disturbance.

Lyme borreliosis is a multisystem illness that primarily involves the skin, nervous system, heart, and joints (Table 19-2). Lyme disease usually begins during the summer months with EM and flulike symptoms and may be accompanied by right upper quadrant tenderness and a mild hepatitis (stage 1). This stage is followed weeks to months later by acute cardiac or neurologic disease in a minority of untreated individuals (stage 2) and then by arthritis and chronic neurologic disease (stage 3) in many untreated patients weeks to years after disease onset. There is considerable overlap of these stages, but Lyme disease is best characterized as an illness that evolves from early to late disease without reference to an arbitrary staging system. However, a patient may have one or all of the stages, and the infection may not become symptomatic until stage 2 or 3. Most affected patients have EM and 25% manifest arthritis; neurologic manifestations and cardiac involvement are uncommon.

Cutaneous Manifestations

Cutaneous manifestations can be demonstrated as early ECM (Fig. 19-5), secondary lesions (disseminated lesions and lymphocytoma), and late lesions (ACA). Except for the late lesions, cutaneous manifestations generally resolve spontaneously over weeks to months. The red papule at the site of the tick bite is most often located on the thigh, groin, or axilla. Facial EM is seen more frequently in children.

Several days to weeks after the onset of EM, almost 50% of untreated patients develop secondary skin lesions. A rare early manifestation of Lyme disease is Borrelia lymphocytoma, a violaceous, tumor-like swelling or nodule at the base of the earlobe or nipple caused by a dense lymphocytic infiltrate of the dermis. This lesion occurs at the site of a tick bite and in conjunction with other symptoms; it may be confused with lymphoma.

ACA is a late skin manifestation of Lyme disease more prevalent in Europe than in the United States. Lesions display bluish red discoloration, doughy swelling, and fibrotic nodules. Eventually, striking atrophy of the skin and subcutaneous tissues follows. Polyneuropathy coexists in 30% to 45% of patients.

Diagnostic Evaluation

The culture of B. burgdorferi

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