Lyme disease is a tick-transmitted inflammatory disorder characterized by an
early focal skin lesion, and subsequently a growing red area on the skin (erythema
chronicum migrans or ECM). The disorder may be followed weeks later by
neurological, heart or joint abnormalities. Symptomatology The first symptom of
Lyme disease is a skin lesion. Known as erythema chronicum migrans, or ECM, this
usually begins as a red discoloration (macule) or as an elevated round spot
(papule). The skin lesion usually appears on an extremity or on the trunk,
especially the thigh, buttock or the under arm. This spot expands, often with
central clearing, to a diameter as large as 50 cm (c. 12 in.). Approximately 25%
of patients with Lyme disease report having been bitten at that site by a tiny
tick 3 to 32 days before onset of ECM. The lesion may be warm to touch. Soon
after onset nearly half the patients develop multiple smaller lesions without
hardened centers. ECM generally lasts for a few weeks. Other types of lesions
may subsequently appear during resolution. Former skin lesions may reappear
faintly, sometimes before recurrent attacks of arthritis. Lesions of the mucous
membranes do not occur in Lyme disease. The most common symptoms accompanying
ECM, or preceding it by a few days, may include malaise, fatigue, chills, fever,
headache and stiff neck. Less commonly, backache, muscle aches (myalgias),
nausea, vomiting, sore throat, swollen lymph glands, and an enlarged spleen may
also be present. Most symptoms are characteristically intermittent and changing,
but malaise and fatigue may linger for weeks. Arthritis is present in about half
of the patients with ECM, occurring within weeks to months following onset and
lasting as long as 2 years. Early in the illness, migratory inflammation of many
joints (polyarthritis) without joint swelling may occur. Later, longer attacks
of swelling and pain in several large joints, especially the knees, typically
recur for several years. The knees commonly are much more swollen than painful;
they are often hot, but rarely red. Baker's cysts (a cyst in the knee) may form
and rupture. Those symptoms accompanying ECM, especially malaise, fatigue and
low-grade fever, may also precede or accompany recurrent attacks of arthritis.
About 10% of patients develop chronic knee involvement (i.e. unremittent for 6
months or longer). Neurological abnormalities may develop in about 15% of
patients with Lyme disease within weeks to months following onset of ECM, often
before arthritis occurs. These abnormalities commonly last for months, and
usually resolve completely. They include: 1. lymphocytic meningitis or
meningoencephalitis 2. jerky involuntary movements (chorea) 3. failure of muscle
coordination due to dysfunction of the cerebellum (cerebellar ataxia) 4. cranial
neuritis including Bell's palsy (a form of facial paralysis) 5. motor and
sensory radiculo-neuritis (symmetric weakness, pain, strange sensations in the
extremities, usually occurring first in the legs) 6. injury to single nerves
causing diminished nerve response (mononeuritis multiplex) 7. inflammation of
the spinal cord (myelitis). Abnormalities in the heart muscle (myocardium) occur
in approximately 8% of patients with Lyme disease within weeks of ECM. They may
include fluctuating degrees of atrioventricular block and, less commonly,
inflammation of the heart sack and heart muscle (myopericarditis) with reduced
blood volume ejected from the left ventricle and an enlarged heart (cardiomegaly).
When Lyme Disease is contracted during pregnancy, the fetus may or may not be
adversely affected, or may contract congenital Lyme Disease. In a study of
nineteen pregnant women with Lyme Disease, fourteen had normal pregnancies and
normal babies. If Lyme Disease is contracted during pregnancy, possible fetal
abnormalities and premature birth can occur. Etiology Lyme disease is caused by
a spirochete bacterium (Borrelia Burgdorferi) transmitted by a small tick called
Ixodes dammini. The spirochete is probably injected into the victim's skin or
bloodstream at the time of the insect bite. After an incubation period of 3 to
32 days, the organism migrates outward in the skin, is spread through the
lymphatic system or is disseminated by the blood to different body organs or
other skin sites. Lyme Disease was first described in 1909 in European medical
journals. The first outbreak in the United States occurred in the early 1970's
in Old lyme, Connecticut. An unusually high incidence of juvenile arthritis in
the area led scientists to investigate and identify the disorder. In 1981, Dr.
Willy Burgdorfer identified the bacterial spirochete organism (Borrelia
Burgdorferi) which causes this disorder. Affected Population Lyme Disease occurs
in wooded areas with populations of mice and deer which carry ticks, and can be
contracted during any season of the year. Related Disorders Rheumatoid Arthritis
is a disorder similar in appearance to Lyme disease. However, the pain in
rheumatoid arthritis is usually more pronounced. Morning stiffness and symmetric
joint swelling more commonly occur in rheumatoid arthritis, and knotty lumps
under the skin may be present over bony prominences. Bony decalcification which
can be prominent in Rheumatoid Arthritis is detected on X-rays. Brachial
Neuritis, also known as Parsonnage-Turner Syndrome, is a common inflammation of
a group of nerves that supply the arm, forearm, and hand (brachial plexus). It
is characterized by severe neck pain in the area above the collarbone (supraclavicular)
that may radiate down the arm and into the hand. There also may be weakness and
numbness (hyperesthesia) of the fingers and hands. Although many cases have no
apparent cause, this syndrome may occur following an immunization (tetanus or
diptheria), surgery, or infection with Lyme Disease. Therapies: Standard For
adults with Lyme disease the antibiotic tetracycline is the drug of choice.
Penicillin V and erythromycin have also been used. In children penicillin V is
recommended rather than tetracycline. Penicillin V is now recommended for
neurological abnormalities. It is not yet clear whether antibiotic treatment is
helpful later in the illness when arthritis is the most predominant symptom.
Treatment should be started as soon as the rash appears, even before the Enzyme
Linked Immunoabsorbent Assay (ELISA) test is completed. Results of this test may
be inaccurate if patients have had antibiotics soon after contracting Lyme
Disease, or in those who have weakened immune systems. If lyme Disease is
contracted during pregnancy, careful monitoring by physicians is highly
recommended to avoid possible fetal abnormalities and/or complications. For
tense knee joints due to increased fluid flowing in the joint spaces
(effusions), the use of crutches is often helpful. Aspiration of fluid and
injection of a corticosteroid may be beneficial. If the patient with Lyme
disease has marked functional limitation, excision of the membrane lining the
joint (synovectomy) may be performed for chronic (6 months or more despite
therapy) knee effusions, but spontaneous remission can occur after more than a
year of continuous knee involvement. When Lyme Disease is contracted during
pregnancy, treatment with penicillin should begin immediately to avoid the
possibility of fetal abnormalities. In 1989 a new Lyme Disease antibody test,
manufactured by Cambridge Biosciences Corp., was approved by the FDA. This test
is being used by local laboratories throughout the nation, making tests more
available to the general population. However, it is 97% specific for antibodies
to Lyme disease when compared to Western blot tests, but it cannot identify the
live bacteria in patients who have not yet developed the antibodies. Therapies:
Investigational Researchers are trying to develop a test that will identify the
Lyme disease bacteria in patients who have not yet developed the antibodies.
This would enable doctors to diagnose Lyme disease very early in the course of
the illness. This disease entry is based upon medical information available
through July 1989. Since NORD's resources are limited, it is not possible to
keep every entry in the Rare Disease Database completely current and accurate.
Please check with the agencies listed in the Resources section for the most
current information about this disorder.