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Syphilis (Encyclopedia of Nursing & Allied Health)

Definition

Syphilis is an infectious systemic disease that may be either congenital or acquired through sexual contact or by exposure to contaminated needles.

Description

Syphilis has both acute and chronic forms that produce a wide variety of symptoms affecting most of the body's organ systems. Acquired syphilis has four stages, including primary, secondary, latent, and tertiary, and can be spread by sexual contact during the first three of these four stages.

Syphilis has been a major public health problem since the sixteenth century. The disease was treated with mercury or other unsuccessful remedies until World War I, when effective treatments based on arsenic or bismuth were introduced. Antibiotics were introduced after World War II. At that time, the number of syphilis cases in the general population decreased, partly due to public health measures. But since 1980, the number of cases of syphilis in the United States has risen steadily. This increase includes men and women, all races, all parts of the nation, and all age groups, including adults over age 60. The number of women of childbearing age with syphilis is the highest that has been recorded since the 1940s. About 25,000 cases of infectious syphilis in adults are reported annually in the United States. It is estimated, however, that 400,000 people in the United States need treatment for syphilis every year, and that the annual worldwide total of persons infected with syphilis is 50 million.

The increased incidence of syphilis in recent years is associated with drug abuse as well as changes in sexual behavior. The connections between drug abuse and syphilis include needle sharing and exchanging sex for drugs. In addition, people using drugs are more likely to engage in risky sexual practices. With respect to changing patterns of conduct, a sharp increase in the number of people having sex with multiple partners makes it more difficult for public health professionals to trace the contacts of infected individuals. High-risk groups for syphilis include:

• sexually active teenagers
• people infected with another sexually transmitted disease (STD), including AIDS
• sexually abused children
• women of childbearing age
• prostitutes of either gender and their customers
• prisoners
• people who abuse drugs or alcohol

The chances of contracting syphilis from an infected person in the early stages of the disease during unprotected sex are 30-50%.

Causes and symptoms

Syphilis is caused by Treponema pallidum, a spirochete, which is a thin spiral-or coil-shaped bacterium that enters the body through the mucous membranes or breaks in the skin. In 90% of cases, the spirochete is transmitted by sexual contact.

Primary syphilis

Primary syphilis refers to the initial stage of the organism's entry into the body. The first signs of infection are not always noticed. After an incubation period ranging between 10 and 90 days, an individual develops a chancre, which is a small blister-like sore about 0.5 inches (13 mm) in size. Most chancres are on the genitals, but they may also develop in or on the mouth or on the breasts. Rectal chancres are common among male homosexuals. Chancres in women are sometimes overlooked if they develop in the vagina or on the cervix. The chancres are not painful and disappear in three to six weeks, with or without treatment. They resemble the ulcers of lymphogranuloma venereum, herpes simplex virus, or skin tumors.

About 70% of people with primary syphilis also develop swollen lymph nodes near the chancre. The nodes may have a firm or rubbery feel, but they are not usually painful.

Secondary syphilis

Syphilis enters its secondary stage between six to eight weeks and six months after the initial infection begins. Chancres may still be present but are usually healing. Secondary syphilis is a systemic infection marked by the eruption of skin rashes and ulcers in the mucous membranes. The skin rash may mimic a number of other skin disorders such as drug reactions, rubella (German measles), ringworm, mononucleosis, and pityriasis rosea. Characteristics of the rash that point to syphilis include:

• a coppery color
• absence of pain or itching
• occurrence on the palms of hands and soles of feet

The skin eruption may resolve in a few weeks or last as long as a year. A person may also develop condylomata lata, which are watery pink or gray areas of flattened skin in the moist areas of the body. The skin rashes, mouth and genital ulcers, and condylomata lata are all highly infectious.

About 50% of people with secondary syphilis develop swollen lymph nodes in the armpits, groin, and neck areas; about 10% develop inflammations of the eyes, kidney, liver, spleen, bones, joints, or the meninges (membranes covering the brain and spinal cord). They may also have a flu-like general illness with a low fever, chills, loss of appetite, headaches, runny nose, sore throat, and aching joints.

Latent syphilis

Latent syphilis is a phase of the disease characterized by relative absence of external symptoms. The latent phase is sometimes divided into early latency (less than two years after infection) and late latency. During early latency, people are at risk for spontaneous relapses marked by recurrence of the ulcers and skin rashes of secondary syphilis. In late latency, these recurrences are much less likely. Late latency may either resolve spontaneously or continue for the rest of the person's life.

Tertiary syphilis

Untreated syphilis progresses to a third, or tertiary, stage in about 350% of people. Individuals with tertiary syphilis cannot infect others with the disease. It is thought that the symptoms of this stage are a delayed hypersensitivity reaction to spirochetes. Some people develop so-called benign late syphilis, which begins between three and 10 years after initial infection and is characterized by the development of gummas. Gummas are rubbery tumor-like growths that are most likely to involve the skin or long bones but may also develop in the eyes, mucous membranes, throat, liver, or stomach lining. Gummas are increasingly uncommon since the introduction of antibiotics for treating syphilis. Benign late syphilis is usually rapid in onset and responds well to treatment.

CARDIOVASCULAR SYPHILIS. Cardiovascular syphilis occurs in 105% of people who have progressed to tertiary syphilis. It develops between 10 and 25 years after initial infection and often occurs together with neurosyphilis. Cardiovascular syphilis usually begins as an inflammation of the arteries leading from the heart and causes heart attacks, scarring of the aortic valves, congestive heart failure, or the formation of an aortic aneurysm.

NEUROSYPHILIS. About 8% of persons with untreated syphilis will develop problems in the central nervous system that include both physical and psychiatric symptoms. Neurosyphilis can appear at any time, from five to 35 years after the onset of primary syphilis. It affects men more frequently than women and Caucasians more frequently than African Americans.

Neurosyphilis is classified into four types:

• Asymptomatic: In this form, the person's spinal fluid gives abnormal test results, but there are no symptoms affecting the central nervous system.
• Meningovascular: This type is marked by changes in the blood vessels of the brain or inflammation of the meninges. A person develops headaches, irritability, and visual problems. If the spinal cord is involved, an individual may experience weakness of the shoulder and upper arm muscles.
• Tabes dorsalis: This type causes a progressive degeneration of the spinal cord and nerve roots. People lose their sense of perception of their body position and orientation in space (proprioception), resulting in difficulties with walking and the loss of muscle reflexes. They may also have shooting pains in the legs and periodic episodes of pain in the abdomen, throat, bladder, or rectum. Tabes dorsalis is sometimes called locomotor ataxia.
• General paresis: This type refers to the effects of neurosyphilis on the cortex of the brain. A person experiences slow but progressive losses of memory, ability to concentrate, and interest in self-care. Personality changes may include irresponsible behavior, depression, delusions of grandeur, or complete psychosis. General paresis is sometimes called dementia paralytica, and is most common among people over age 40.

Special populations

CONGENITAL SYPHILIS. Congenital syphilis has increased at a rate of 40000% over the past decade, on the basis of criteria introduced by the Centers for Disease Control (CDC) in 1990. In 1994, more than 2,200 cases of congenital syphilis were reported in the United States. The prognosis for early congenital syphilis is poor: about 54% of infected fetuses die before or shortly after birth. Those which survive may look normal at birth but show signs of infection between three and eight weeks later.

Infants with early congenital syphilis have systemic symptoms that resemble those of adults with secondary syphilis. There is a 400% chance that a child's central nervous system will be infected. These infants may have symptoms ranging from jaundice, enlargement of the spleen and liver, and anemia to skin rashes, condylomatalata, inflammation of the lungs, a persistent runny nose, and swollen lymph nodes.

CHILDREN. Children who develop symptoms after the age of two years are said to have late congenital syphilis. The characteristic symptoms include facial deformities (saddle nose), Hutchinson's teeth (abnormal upper incisors), saber shins, dislocated joints, deafness, mental retardation, paralysis, and seizure disorders.

PREGNANT WOMEN. Syphilis can be transmitted from a mother to her fetus through the placenta at any time during pregnancy, or through the child's contact with syphilitic ulcers during the birth process. The chances of infection are related to the stage of the mother's disease. Almost all infants of mothers with untreated primary or secondary syphilis will be infected, whereas the infection rate drops to 40% if the mother is in the early latent stage, and 64% if she has late latent syphilis.

Pregnancy does not affect the progression of syphilis in the mother. However, pregnant women should not be treated with tetracyclines as this drug will discolor the teeth of her infant.

PEOPLE WITH HIV. Syphilis has been closely associated with HIV infection since the late 1980s. Syphilis sometimes mimics the symptoms of AIDS. Conversely, AIDS appears to increase the severity of syphilis in people suffering from both diseases, and to speed up the development or appearance of neurosyphilis. People with HIV are also more likely to develop lues maligna, a skin disease that sometimes occurs in secondary syphilis. In addition, people with HIV have a higher rate of treatment failure with penicillin than those without HIV.

Diagnosis

Personal history and physical diagnosis

Because of the long-term risks of untreated syphilis, certain groups of people are now routinely screened for the disease, including:

• pregnant women
• sexual contacts or partners of people diagnosed with syphilis
• children born to mothers with syphilis
• individuals with HIV infection
• persons applying for marriage licenses

When a physician takes a person's history, there will be questions about recent sexual contacts to determine whether the person falls into a high-risk group. Symptoms such as skin rashes or swollen lymph nodes will be noted with respect to the dates of the person's sexual contacts. Definite diagnosis, however, depends on the results of laboratory blood tests.

Blood tests

There are several types of blood tests for syphilis presently used in the United States. Some are used in follow-up monitoring of infected people as well as diagnosis.

NON-TREPONEMAL ANTIGEN TESTS. Non-treponemal antigen tests are used with initial screening. They measure the presence of reagin, which is an antibody formed in reaction to syphilis. In the venereal disease research laboratory (VDRL) test, a sample of a person's blood is mixed with cardiolipin and cholesterol. If the mixture forms clumps or masses of matter, the test is considered reactive, or positive.

The rapid plasma reagin (RPR) test, which is available as a kit, works on the same principle as the VDRL. A person's serum is mixed with cardiolipin on a plasticcoated card that can be examined with the naked eye.

Non-treponemal antigen tests require a physician's interpretation and sometimes further testing. They can yield both false-negative and false-positive results. False-positive results can be caused by other infectious diseases, including mononucleosis, malaria, leprosy, rheumatoid arthritis, and lupus. People with HIV have a particularly high rate (4%, compared to 0.8% of people who are HIV-negative) of false-positive results on reagin tests. False-negatives can occur when individuals are tested too soon after exposure to syphilis; it takes about 141 days after infection for the blood to become reactive.

TREPONEMAL ANTIBODY TESTS. Treponemal antibody tests are used to rule out false-positive results on reagin tests. They measure the presence of antibodies that are specific for T. pallidum. The most commonly used tests are the microhemagglutination-T. pallidum (MHATP) and the fluorescent treponemal antibody absorption (FTA-ABS) tests. In the FTA-ABS test, a person's blood serum is mixed with a preparation that prevents interference from antibodies to other treponemal infections. In a positive reaction, syphilitic antibodies in the blood coat the spirochetes on the slide. In the MHA-TP test, red blood cells from sheep are coated with T. pallidum antigen. The cells will clump if the person's blood contains antibodies for syphilis. Treponemal antibody tests are more expensive and more difficult to perform than non-treponemal tests. They are therefore used to confirm the diagnosis of syphilis rather than to screen large groups of people. These tests are, however, very specific and very sensitive; false-positive results are relatively unusual.

Other laboratory tests

MICROSCOPE STUDIES. The diagnosis of syphilis can also be confirmed by identifying spirochetes in samples of tissue or lymphatic fluid.

SPINAL FLUID TESTS. Testing of cerebrospinal fluid (CSF) is an important part of monitoring programs as well as being a diagnostic test. The VDRL and FTA-ABS tests can be performed on CSF as well as on blood. An abnormally high white cell count and elevated protein levels in the CSF, together with positive VDRL results, suggest a possible diagnosis of neurosyphilis. CSF testing is not used for routine screening. It is most frequently used for infants with congenital syphilis, people who are HIV-positive, and individuals of any age who are not responding to penicillin treatment.

Treatment

Medications

Syphilis is treated with antibiotics given either intramuscularly (benzathine penicillin G or ceftriaxone) or orally (doxycycline, minocycline, tetracycline, or azithromycin). Neurosyphilis is treated with a combination of aqueous crystalline penicillin G, benzathine penicillin G, or doxycycline. It is important to keep the levels of penicillin in the person's tissues at sufficiently high levels over a period of days or weeks because the spirochetes have a relatively long reproduction time. Penicillin is more effective in treating the early stages of syphilis than the later stages.

Physicians do not usually prescribe separate medications for the skin rashes or ulcers of secondary syphilis. A person is advised to keep the rashes clean and dry, and to avoid exposing others to fluid or discharges from condylomata lata.

Pregnant women should be treated as early in pregnancy as possible. Infected fetuses can be cured if the mother is treated during the second and third trimesters of pregnancy. Infants with proven or suspected congenital syphilis are treated with either aqueous crystalline penicillin G or aqueous procaine penicillin G. Children who acquire syphilis after birth are treated with benzathine penicillin G.

Jarisch-Herxheimer reaction

The Jarisch-Herxheimer reaction, first described in 1895, is a reaction to penicillin treatment that may occur during the late primary, secondary, or early latent stages. A person develops chills, fever, headache, and muscle pains within two to six hours after the penicillin is injected. The chancre or rash temporarily gets worse. The Jarisch-Herxheimer reaction, which lasts about a day, is thought to be an allergic reaction to toxins released when the penicillin kills massive numbers of spirochetes.

Alternative treatment

Antibiotics are essential for the treatment of syphilis. Recovery from the disease can be assisted by dietary changes, changes in sexual practices, sleep, exercise, and stress reduction.

HOMEOPATHY. Homeopathic practitioners are forbidden by law in the United States to claim that homeopathic treatment can cure syphilis. The remedies most frequently recommended by alternative practitioners who treat people with syphilis are Medorrhinum, Syphilinum, Mercurius vivus, and Aurum.

Prognosis

The prognosis is good for the early stages of syphilis if a person is treated promptly and given sufficiently large doses of antibiotics. There are no definite criteria for cure for individuals with primary and secondary syphilis, although people who are symptom-free and have had negative blood tests for two years after treatment are usually considered to be free of syphilis. Treated people should follow up with blood tests at one, three, six, and 12 months after treatment, or until the results are negative. CSF should be examined after one year. People with recurrences during the latency period should be tested for re-infection. The prognosis for people with untreated syphilis is spontaneous remission for about 30%, lifelong latency for another 30%, and potentially fatal tertiary forms of the disease in 40%.

Health care team roles

Trained lay people often take medical and personal histories. Phlebotomists draw blood for testing. A pathologist often interprets the results of specialized tests. A physician may also administer and check test results and provide treatment. Psychiatrists or other counselors may treat psychiatric symptoms.

Prevention

Immunity

People with syphilis do not acquire lasting immunity against the disease. As of 2001, no effective vaccine for syphilis has been developed. Prevention depends on a combination of personal and public health measures.

Lifestyle choices

The only reliable methods for preventing transmission of syphilis are sexual abstinence or monogamous relationships between uninfected partners. Latex condoms offer some protection but protect only the covered parts of the body.

Public health measures

CONTACT TRACING. The law requires reporting of syphilis cases to public health agencies. Sexual contacts of people diagnosed with syphilis are traced and tested for the disease. This includes all contacts for the past three months in cases of primary syphilis, and for the past year in cases of secondary disease. Neither the affected people nor their contacts should have sex with anyone until they have been tested and treated.

All people who test positive for syphilis should be tested for HIV infection at the time of initial diagnosis.

PRENATAL TESTING OF PREGNANT WOMEN. Pregnant women should be tested for syphilis at the time of their first visit for prenatal care, and again shortly before delivery. Proper treatment of secondary syphilis in the mother reduces the risk of congenital syphilis in the infant from 90% to less than 2%.

EDUCATION AND INFORMATION. People diagnosed with syphilis should be given information about the disease and counseling regarding sexual behavior and the importance of completing antibiotic treatment. It is also important to inform the general public about the transmission and early symptoms of syphilis, and provide adequate health facilities for testing and treatment.

KEY TERMS

Chancrehe initial skin ulcer of primary syphilis, consisting of an open sore with a firm or hard base.

Condylomata lataighly infectious patches of watery pink or gray skin that appear in the moist areas of the body during secondary syphilis.

General paresis form of neurosyphilis in which a person's personality, as well as the control of movement, is affected.

Gumma symptom that is sometimes seen in tertiary syphilis, characterized by a rubbery swelling or tumor that heals slowly and leaves a scar.

Jarisch-Herxheimer reaction temporary reaction to penicillin treatment for syphilis that includes fever, chills, and worsening of the skin rash or chancre.

Lues maligna skin disorder of secondary syphilis in which areas of ulcerated and dying tissue are formed.

Spirochete type of bacterium with a long, slender, coiled shape.

Tabes dorsalis progressive deterioration of the spinal cord and spinal nerves associated with tertiary syphilis.

Resources

BOOKS

Larsen, Sandra A., Victoria Pope, and Robert E. Johnson. Syphilis: A Manual of Tests and Supplement. Washington, DC: American Public Health Association, 1999.

Lukehart, Shiela A., and King Holmes. "Syphilis." In Harrison's Principles of Internal Medicine, 14th ed., edited by Anthony S. Fauci et al. New York: McGraw-Hill, 1998, 1023-33.

Reverby, Susan. Tuskegee's Truths: Rethinking the Tuskegee Syphilis Study. Chapel Hill, NC: University of North Carolina Press, 2000.

PERIODICALS

Finelli, L., W. C. Levine, J. Valentine, and M. E. St. Louis. "Syphilis Outbreak Assessment." Sexually Transmitted Disease 28 no. 3 (2001): 131-35.

Gayle, H. D., and G. W. Counts. "Syphilis Elimination: A Unique Time in History." Journal of the American Medical Women's Association 56 no. 1 (2001): 2-3.

Patel, A., D. Moodley, and J. Moodley. "An Evaluation of On-Site Testing for Syphilis." Tropical Doctor 31 no. 2 (2001): 79-82.

Polsky, I., and S. C. Samuels. "Neurosyphilis. Screening Does Sometimes Reveal an Infectious Cause of Dementia." Geriatrics 56 no. 3 (2001): 60-2.

Warner, L., et al. "Missed Opportunities for Congenital Syphilis Prevention in an Urban Southeastern Hospital." Sexually Transmitted Disease 28 no. 2 (2001): 92-8.