syphilis

Syphilis

 

Definition

Syphilis is an infectious systemic disease that may be either congenital or acquired through sexual contact or 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. The range of symptoms makes it easy to confuse syphilis with less serious diseases and ignore its early signs. Acquired syphilis has four stages (primary, secondary, latent, and tertiary) and can be spread by sexual contact during the first three of these four stages.

Syphilis, which is also called lues (from a Latin word meaning plague), has been a major public health problem since the sixteenth century. The disease was treated with mercury or other ineffective remedies until World War I, when effective treatments based on arsenic or bismuth were introduced. These were succeeded by antibiotics after World War II. At that time, the number of cases in the general population decreased, partly because of aggressive public health measures. This temporary decrease, combined with the greater amount of attention given to AIDS in recent years, leads some people to think that syphilis is no longer a serious problem. In actual fact, the number of cases of syphilis in the United States has risen since 1980. This increase affects both sexes, all races, all parts of the nation, and all age groups, including adults over 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 is 50 million persons.

In 1999, the Centers for Disease Control and Prevention (CDC) joined several other federal agencies in announcing the "National Plan to Eliminate Syphilis in the United States." Eliminating the disease was defined as the absence of transmission of the disease; that is, no transmission after 90 days following the report of an imported index case. The national goals for eliminating syphilis include bringing the annual number of reported cases in the United States below 1000, and increasing the number of syphilis-free counties to 90% by 2005. In November 2002, the CDC released figures for 2000–2001, which indicate that the number of reported cases of primary and secondary syphilis rose slightly. This rise, however, occurred only among men who have sex with other men. The CDC also stated that the number of new cases of syphilis has actually declined among women as well as among non-Hispanic blacks.

The increased incidence of syphilis since the 1970s 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. As of 2002, the risk of contracting syphilis is particularly high among those who abuse crack cocaine.

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 doctors to trace the contacts of infected persons. Women are not necessarily protected by having sex only with other women; in the past few years, several cases have been reported of female-to-female transmission of syphilis through oral-genital contact. In addition, the incidence of syphilis among men who have sex with other men continues to rise. Several studies in Latin America as well as in the United States reported in late 2002 that unprotected sexual intercourse is on the increase among gay and bisexual men.

Changing patterns of sexual behavior have led to a striking increase in the number of cases of syphilis in eastern Europe since the collapse of the Soviet Union; Slovenia reported an 18-fold increase in reported cases of syphilis just between 1993 and 1994. Over half of the new cases were linked to a source of infection in another European country.

In general, high-risk groups for syphilis in the United States and Canada include:

• sexually active teenagers
• people infected with another sexually transmitted disease (STD), including AIDS
• sexually abused children
• women of childbearing age
• prostitutes of either sex and their customers
• prisoners
• persons 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 between 30-50%.

Causes and symptoms

Syphilis is caused by a spirochete, Treponema pallidum. A spirochete is a thin spiralor 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. Transmission by blood transfusion is possible but rare; not only because blood products are screened for the disease, but also because the spirochetes die within 24 hours in stored blood. Other methods of transmission are highly unlikely because T. pallidum is easily killed by heat and drying.

Primary syphilis

Primary syphilis is the 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, the patient develops a chancre, which is a small blister-like sore about 0.5 in (13 mm) in size. Most chancres are on the genitals, but may also develop in or on the mouth or on the breasts. Rectal chancres are common in 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 even without treatment. They resemble the ulcers of lymphogranuloma venereum, herpes simplex virus, or skin tumors.

About 70% of patients with primary syphilis also develop swollen lymph nodes near the chancre. The nodes may have a firm or rubbery feel when the doctor touches them but are not usually painful.

Secondary syphilis

Syphilis enters its secondary stage between six to eight weeks and six months after the 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, ringworm, mononucleosis, and pityriasis rosea. Characteristics 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. The patient may also develop condylomata lata, which are weepy pinkish or grey 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 patients 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 flulike 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 term latent does not mean that the disease is not progressing or that the patient cannot infect others. For example, pregnant women can transmit syphilis to their unborn children during the latency period.

The latent phase is sometimes divided into early latency (less than two years after infection) and late latency. During early latency, patients 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 patient's life.

Tertiary syphilis

Untreated syphilis progresses to a third or tertiary stage in about 35-40% of patients. Patients with tertiary syphilis cannot infect others with the disease. It is thought that the symptoms of this stage are a delayed hypersensitivity reaction to the spirochetes. Some patients develop so-called benign late syphilis, which begins between three and 10 years after 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 10-15% of patients who have progressed to tertiary syphilis. It develops between 10 and 25 years after 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 patients with untreated syphilis will develop symptoms in the central nervous system that include both physical and psychiatric symptoms. Neurosyphilis can appear at any time, from 5-35 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 of neurosyphilis, the patient's spinal fluid gives abnormal test results but there are no symptoms affecting the central nervous system.
• Meningovascular. This type of neurosyphilis is marked by changes in the blood vessels of the brain or inflammation of the meninges (the tissue layers covering the brain and spinal cord). The patient develops headaches, irritability, and visual problems. If the spinal cord is involved, the patient may experience weakness of the shoulder and upper arm muscles.
• Tabes dorsalis. Tabes dorsalis is a progressive degeneration of the spinal cord and nerve roots. Patients lose their sense of perception of one's body position and orientation in space (proprioception), resulting in difficulties walking and 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. General paresis refers to the effects of neurosyphilis on the cortex of the brain. The patient has a slow but progressive loss 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 in patients over 40.

Special populations

CONGENITAL SYPHILIS. Congenital syphilis has increased at a rate of 400-500% over the past decade, on the basis of criteria introduced by the Centers for Disease Control (CDC) in 1990. In 1994, over 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 who 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 40-60% chance that the 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, condylomata lata, inflammation of the lungs, "snuffles" (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 the mother to the 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 6-14% 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.

HIV PATIENTS. 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 patients suffering from both diseases, and to speed up the development or appearance of neurosyphilis. Patients with HIV are also more likely to develop lues maligna, a skin disease that sometimes occurs in secondary syphilis. Lues maligna is characterized by areas of ulcerated and dying tissue. In addition, HIV patients have a higher rate of treatment failure with penicillin than patients without HIV.

Diagnosis

Patient history and physical diagnosis

The diagnosis of syphilis is often delayed because of the variety of early symptoms, the varying length of the incubation period, and the possibility of not noticing the initial chancre. Patients do not always connect their symptoms with recent sexual contact. They may go to a dermatologist when they develop the skin rash of secondary syphilis rather than to their primary care doctor. Women may be diagnosed in the course of a gynecological checkup. Because of the long-term risks of untreated syphilis, certain groups of people are now routinely screened for the disease:

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

When the doctor takes the patient's history, he or she will ask about recent sexual contacts in order to determine whether the patient falls into a high-risk group. Other symptoms, such as skin rashes or swollen lymph nodes, will be noted with respect to the dates of the patient'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 patients as well as diagnosis.

NONTREPONEMAL ANTIGEN TESTS. Nontreponemal antigen tests are used as screeners. 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 the patient'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 serum sample can be diluted several times to determine the concentration of reagin in the patient's blood.

The rapid plasma reagin (RPR) test works on the same principle as the VDRL. It is available as a kit. The patient's serum is mixed with cardiolipin on a plastic-coated card that can be examined with the naked eye.

Nontreponemal antigen tests require a doctor'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. HIV patients have a particularly high rate (4%, compared to 0.8% of HIV-negative patients) of false-positive results on reagin tests. False-negatives can occur when patients are tested too soon after exposure to syphilis; it takes about 14-21 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 (MHA-TP) and the fluorescent treponemal antibody absorption (FTA-ABS) tests. In the FTA-ABS, the patient's blood serum is mixed with a preparation that prevents interference from antibodies to other treponemal infections. The test serum is added to a slide containing T. pallidum. In a positive reaction, syphilitic antibodies in the blood coat the spirochetes on the slide. The slide is then stained with fluorescein, which causes the coated spirochetes to fluoresce when the slide is viewed under ultraviolet (UV) light. In the MHA-TP test, red blood cells from sheep are coated with T. pallidum antigen. The cells will clump if the patient's blood contains antibodies for syphilis.

Treponemal antibody tests are more expensive and more difficult to perform than nontreponemal 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.

INVESTIGATIONAL BLOOD TESTS. Currently, ELISA, Western blot, and PCR testing are being studied as additional diagnostic tests, particularly for congenital syphilis and neurosyphilis.

Other laboratory tests

MICROSCOPE STUDIES. The diagnosis of syphilis can also be confirmed by identifying spirochetes in samples of tissue or lymphatic fluid. Fresh samples can be made into slides and studied under darkfield illumination. A newer method involves preparing slides from dried fluid smears and staining them with fluorescein for viewing under UV light. This method is replacing darkfield examination because the slides can be mailed to professional laboratories.

SPINAL FLUID TESTS. Testing of cerebrospinal fluid (CSF) is an important part of patient monitoring as well as 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 used most frequently for infants with congenital syphilis, HIV-positive patients, and patients 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 patient'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.

In the fall of 2000, the CDC convened a group of medical advisors to discuss backup medications for treating syphilis. Although none of the newer drugs will displace penicillin as the primary drug, the doctors recommended azithromycin and ceftriaxone as medications that should have a larger role in the treatment of syphilis than they presently do.

Doctors do not usually prescribe separate medications for the skin rashes or ulcers of secondary syphilis. The patient is advised to keep them 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. The patient develops chills, fever, headache, and muscle pains within two to six hours after the penicillin is injected. The chancre or rash gets temporarily 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, sleep, exercise, and stress reduction.

Homeopathy

Homeopathic practitioners are forbidden by law in the United States to claim that homeopathic treatment can cure syphilis. Given the high rate of syphilis in HIV-positive patients, however, some alternative practitioners who are treating AIDS patients with homeopathic remedies maintain that they are beneficial for syphilis as well. The remedies suggested most frequently are Medorrhinum, Syphilinum, Mercurius vivus, and Aurum. The historical link between homeopathy and syphilis is Hahnemann's theory of miasms. He thought that the syphilitic miasm was the second oldest cause of constitutional weakness in humans.

Prognosis

The prognosis is good for the early stages of syphilis if the patient is treated promptly and given sufficiently large doses of antibiotics. Treatment failures can occur and patients can be reinfected. There are no definite criteria for cure for patients with primary and secondary syphilis, although patients who are symptom-free and have had negative blood tests for two years after treatment are usually considered cured. Patients should be followed 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. Patients with recurrences during the latency period should be tested for reinfection.

The prognosis for patients with untreated syphilis is spontaneous remission for about 30%; lifelong latency for another 30%; and potentially fatal tertiary forms of the disease in 40%.

Prevention

Immunity

Patients with syphilis do not acquire lasting immunity against the disease. Currently, 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. 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 patients 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 patients nor their contacts should have sex with anyone until they have been tested and treated.

Because of the rising incidence of syphilis abroad, a growing number of public health physicians are recommending routine screening of immigrants, refugees, and international adoptees for syphilis as of late 2002.

All patients who test positive for syphilis should be tested for HIV infection at the time of 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%.

As of late 2005, many obstetricians and gynecologists are recommending routine screening of nonpregnant as well as pregnant women for syphilis. At present, only about half of obstetricians and gynecologists in the United States screen nonpregnant women for chlamydia and gonorrhea, while fewer than a third screen them for syphilis.

EDUCATION AND INFORMATION. Patients 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

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

Condylomata lata — Highly infectious patches of weepy pink or gray skin that appear in the moist areas of the body during secondary syphilis.

Darkfield — A technique of microscope examination in which light is directed at an oblique angle through the slide so that organisms look bright against a dark background.

General paresis — A form of neurosyphilis in which the patient's personality, as well as his or her control of movement, is affected. The patient may develop convulsions or partial paralysis.

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

Index case — The first case of a contagious disease in a group or population that serves to call attention to the presence of the disease.

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

Lues maligna — A skin disorder of secondary syphilis in which areas of ulcerated and dying tissue are formed. It occurs most frequently in HIV-positive patients.

Spirochete — A type of bacterium with a long, slender, coiled shape. Syphilis is caused by a spirochete.

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

Resources

Books

Beers, Mark H., MD, and Robert Berkow, MD., editors. "Syphilis." In The Merck Manual of Diagnosis and Therapy. Whitehouse Station, NJ: Merck Research Laboratories, 2004.

Periodicals

Augenbraun, M. H. "Treatment of Syphilis 2001: Nonpregnant Adults." Clinical Infectious Diseases 35, Supplement 2 (October 15, 2002): S187-S190.

Campos-Outcalt, D., and S. Hurwitz. "Female-to-Female Transmission of Syphilis: A Case Report." Sexually Transmitted Diseases 29 (February 2002): 119-120.

Centers for Disease Control. "Primary and Secondary Syphilis—United States, 2000–2001." Morbidity and Mortality Weekly Report 51 (November 1, 2002): 971-973.

Dennis, L. K., and D. V. Dawson. "Meta-Analysis of Measures of Sexual Activity and Prostate Cancer." Epidemiology 13 (January 2002): 72-79.

Gibbs, R. S. "The Origins of Stillbirth: Infectious Diseases." Seminars in Perinatology 26 (February 2002): 75-78.

Grgic-Vitek, M., I Klavs, M. Potocnik, and M. Rogl-Butina. "Syphilis Epidemic in Slovenia Influenced by Syphilis Epidemic in the Russian Federation and Other Newly Independent States." International Journal of STD and AIDS 13, Supplement 2 (December 2002): 2-4.

Hagedorn, H. J., A. Kraminer-Hagedorn, K. de Bosschere, et al. "Evaluation of INNO-LIA Syphilis Assay as a Confirmatory Test for Syphilis." Journal of Clinical Microbiology 40 (March 2002): 973-978.

Hogben, M., J. S. Lawrence, D. Kasprzyk, et al. "Sexually Transmitted Disease Screening by United States Obstetricians and Gynecologists." Obstetrics and Gynecology 100 (October 2002): 801-807.

Kolivras, A., J. de Maubeuge, M. Song, et al. "A Case of Early Congenital Syphilis." Dermatology 204 (2002): 338-340.

Pao, D., B. T. Goh, and J. S. Bingham. "Management Issues in Syphilis." Drugs 62 (2002): 1447-1461.

Ross, M. W., L. Y. Hwang, C. Zack, et al. "Sexual Risk Behaviours and STIs in Drug Abuse Treatment Populations Whose Drug of Choice is Crack Cocaine." International Journal of STD and AIDS 13 (November 2002): 769-774.

Stauffer, W. M., D. Kamat, and P. F. Walker. "Screening of International Immigrants, Refugees, and Adoptees." Primary Care 29 (December 2002): 879-905.

Sutmoller, F., T. L. Penna, C. T. de Souza, et al. "Human Immunodeficiency Virus Incidence and Risk Behavior in the 'Projeto Rio': Results of the First 5 Years of the Rio de Janeiro Open Cohort of Homosexual and Bisexual Men, 1994–98." International Journal of Infectious Diseases 6 (December 2002): 259-265.

Whittington, W. L., T. Collis, C. Dithmer-Schreck, et al. "Sexually Transmitted Diseases and Human Immunodeficiency Virus-Discordant Partnerships Among Men Who Have Sex With Men." Clinical Infectious Diseases 35 (October 15, 2002): 1010-1017.

Organizations

Centers for Disease Control and Prevention. 1600 Clifton Rd., NE, Atlanta, GA 30333. (800) 311-3435, (404) 639-3311. http://www.cdc.gov.

syphilis

 [sif´ĭ-lis]

a contagious, usually sexually transmitted disease that leads to many structural and cutaneous lesions and is caused by a spirochete, Treponema pallidum. A congenital type can be acquired by a fetus in utero from the mother.

Diagnosis. Testing for syphilis is usually by a serologic test for syphilis, of which there are two types: (1) the nontreponemal antigen tests detect antibodies to substance (reagin) derived from host tissues; they originated with the wassermann test and are now represented by the vdrl and rapid plasma reagin tests; (2) the treponemal antigen tests detect specific antitreponemal antibodies; they originated with the Treponema pallidum immobilization test and are now represented by tests such as the microhemagglutination assay–T. pallidum (MHA-TP), and assays using enzyme-linked immunosorbent assay (ELISA) methods. The term “serologic tests for syphilis” is occasionally used with reference only to nontreponemal antigen tests.



A positive test for syphilis should be repeated. A false positive result can be due to other diseases such as malaria, leprosy, and advanced pulmonary tuberculosis, and therefore should not be ignored. A false negative serological test can occur when the infection is too recent to have triggered the production of antibodies. A negative result can also occur if the disease is late symptomatic syphilis or if the patient's immune system is not functioning normally. If treatment of syphilis had been started before the test, the patient's blood could be temporarily nonreactive. Since alcohol interferes with and decreases the intensity of a reaction, it should be considered as a possible cause of a negative result. Once treatment has been started, patients with early syphilis should have repeated testing every three months for one full year.

Primary Syphilis. Within a few hours after the spirochetes penetrate the skin or a mucous membrane, they enter the bloodstream, and usually in about a week they spread throughout the body. The first sign is a painless sore, called a chancre, that appears 9 days to 3 months (usually about 3 weeks) after infection. Usually firm or hard, the chancre may resemble a blister, pimple, or ulcerated open sore. In men, it appears usually on or near the head of the penis. In women, the chancre is commonly found on the labia, but it may be concealed inside the vagina, where it may not be felt or seen. Chancres sometimes develop elsewhere, such as on the lips of the mouth, a breast, or a finger. They also may appear in the anal region. The nearby lymph nodes become hard and swollen.



Even if no treatment is given, the chancre will disappear in 10 to 40 days, often leading to the false conclusion that the disease is cured. Occasionally a chancre fails to develop or is too small to be noticed. Primary syphilis can be cured with antibiotics in adequate doses. (See Atlas 2, Part P.)

Secondary Syphilis. Two to six months after the primary sore disappears, the secondary stage of syphilis begins; it may last up to 2 years. A rash is usually one of the first symptoms. It may cover any part of the body and often spreads over the entire skin surface, including the palms and soles. It does not itch and may resemble the rash of measles as well as of many other diseases. It can be identified positively as a symptom of syphilis only by a blood test. During secondary syphilis, thin white sores may appear on the mucosa of the mouth and throat and around the genitalia and rectum. Headache, fever, and a general feeling of illness are common. Hair may fall out in patches, bones and joints may be painful, and anemia may develop. Sometimes the eyes are affected. Syphilis is highly contagious in this stage and of great danger to others. If mouth sores are present, the disease may be spread by kissing.



Like primary syphilis, the secondary stage disappears by itself, generally within 3 to 12 weeks, but may return later if the organisms are still present. As in the primary stage, the disease can be cured in the secondary stage by the use of penicillin or other antibiotics. Together, the primary and secondary stages are known as early syphilis.

Tertiary Syphilis. The third, or tertiary, stage of the disease is also known as late syphilis. Its symptoms may develop soon after the secondary symptoms have vanished or they may lie hidden for 15 or more years. A person may be unaware that the disease is present. Even a blood test may be negative.



Late syphilis is less contagious to others but is extremely dangerous to the person who has it. It may be fatal, particularly if the central nervous system or heart is affected. The spirochete can invade any cell of the body and can damage any organ or structure of the body, including the internal organs, bones, joints, and skin. The characteristic lesion of tertiary syphilis is a soft gummy tumor called a gumma.

If late syphilis attacks the heart, aorta, or aortic valve, death may result from rupture of the weakened aorta or from heart failure. When it attacks the central nervous system, general paresis, a severe disease of the brain, may result; if not treated promptly, it will cause insanity and death. Another serious disorder of the nervous system caused by late syphilis is tabes dorsalis, in which there is pain and loss of position sense. Blindness may result if the infection involves the eyes. Other possible effects are deep ulcers on the legs or elsewhere, chronic inflammation of the bones, which is especially painful at night, and perforation of the soft palate.

Cure of late or tertiary syphilis takes longer and is more difficult than that of primary or secondary syphilis. Sometimes the disease cannot be completely cured. As with early syphilis, however, it may be successfully treated with penicillin and other antibiotics.

Congenital Syphilis. Congenital syphilis is transmitted from a diseased mother to her unborn child through the placenta; this often results in spontaneous abortion or stillbirth. Infants with congenital syphilis who are born alive may have a nasal discharge called snuffles, caused by inflammation of the nose, and may be generally weak and sickly. Syphilitic rashes, especially in the genital area, may occur when the baby is 3 to 8 weeks old. Many are born with deformities or later develop any of a wide variety of impairments and disabilities.



To prevent congenital syphilis all pregnant women should have a blood test for syphilis during the early months of pregnancy. Treatment before the fifth month will always prevent infection of the unborn child. A syphilitic mother who is not treated early has only one chance in six of having a healthy child. If a child is born with syphilis, immediate treatment may be effective if the disease has not progressed too far.

Prevention. The skin lesions associated with primary and secondary syphilis can be highly contagious. The Centers for Disease Control and Prevention recommends standard precautions when caring for a patient with syphilis.

endemic syphilis nonvenereal syphilis.

late benign syphilis a form of tertiary syphilis that responds very rapidly to treatment and is therefore relatively benign, in which the typical lesion is the gumma.

nonvenereal syphilis a chronic treponemal infection mainly seen in children, occurring in many areas of the world, caused by an organism indistinguishable from Treponema pallidum, and transmitted directly by nonsexual contact and indirectly by common use of table and drinking utensils. The first lesions are usually oral mucous patches; subsequent lesions are concentrated in the axillae, inguinal region, and rectum. Then, after a latent period, there develop destructive lesions of the skin and bones. Researchers have hypothesized that venereal syphilis mutated from one of the nonvenereal forms.

syph·i·lis

(sif'i-lis), Do not confuse this word with erysipelas.

An acute and chronic infectious disease caused by the bacterium Treponema pallidum and transmitted by direct contact, usually through sexual intercourse. After an incubation period of 12-30 days, the first symptom is a chancre, followed by slight fever and other constitutional symptoms (primary syphilis), followed by a skin eruption of various appearances with mucous patches and generalized lymphadenopathy (secondary syphilis), and subsequently by the formation of gummas, cellular infiltration, and functional abnormalities usually resulting from cardiovascular and central nervous system lesions (tertiary syphilis).

Synonym(s): lues venerea, malum venereum

[Mod. L. syphilis (syphilid-), (?) fr. a poem, Syphilis sive Morbus Gallicus, by Fracastorius, Syphilus being a shepherd and principal character]

syphilis

(sĭf′ə-lĭs)

n.

An infectious disease caused by a spirochete (Treponema pallidum),usually transmitted sexually or in utero, marked initially by local formation of chancres and progressing if untreated to bacteremia and widespread organ damage, such as skin ulcerations and tabes dorsalis.

syphilis

STD A multisystem STD caused by a spirochete, Treponema pallidum, when it penetrates broken or abraded mucotaneous tissue through sexual contact; ≥ 100,000 new cases/yr, US; it is the third most commonly reported infectious disease–gonorrhea is the first; it is common in urban areas, especially in the US South and affects young adults Clinical Primary stage or chancre stage causes a nasty looking, but painless rounded ulcer, which may heal spontaneously. See Benign late syphilis, Congenital syphilis, Latent syphilis, Neurosyphilis, Primary syphilis, Secondary syphilis, Tertiary syphilis.

syph·i·lis

(sif'i-lis)

An acute and chronic infectious disease caused by Treponema pallidum and transmitted by direct contact, usually through sexual intercourse. After an incubation period of 12-30 days, the first symptom is a chancre (a painless, indurated ulcer), followed by slight fever and other constitutional symptoms (primary syphilis), followed by a skin eruption of various appearances with mucous patches and generalized lymphadenopathy (secondary syphilis), and subsequently by the formation of gummata, cellular infiltration, and functional abnormalities usually resulting from cardiovascular and central nervous system lesions (tertiary syphilis).

syphilis

(sif'i-lis) [ Syphilis, a shepherd having the disease in a Latin poem]

SYPHILIS: Courtesy of Dr. Henry Foong

SYPHILIS: Courtesy of Dr. Art. Huntley

A multistage infection caused by the spirochete Treponema pallidum. The disease is typically transmitted sexually, although a small number of congenital infections occur during pregnancy. In the U.S. the incidence of syphilis fluctuates from year to year and decade to decade. In 2009 13, 997 cases were reported in the US, a rate of 4.6 per 100,000. syphilitic (sif?i-lit'ik), adjective Synonym: luesillustration;

Syphilis is typically passed from person to person by direct contact with skin or mucous membranes. Spirochetes readily penetrate skin and disseminate from the initial site of inoculation to regional lymph nodes, the bloodstream, and multiple other sites including the central nervous system (CNS). After an incubation period of 10 days to 2 months, a papule appears on the skin that develops into a painless ulcer (chancre) characteristic of the primary stage of infection. Chancres and other syphilitic skin lesions are highly infectious. The genitals are the most common site of primary infection and formation of chancres although chancres may appear on other points of contact, e.g., the lips, mouth, anus, or rectum.

Chancres usually disappear within 3 to 6 weeks even without treatment. Within a few days to several months, the secondary stage appears: a widespread body rash, often with systemic symptoms, e.g., fever, headache, generalized lymph node swelling, nausea, vomiting, weight loss, and malaise. Highly infectious, moist, broad, pink or grayish-white papules may appear in the perineum (condyloma latum), along with shallow ulcers in the mouth (mucous patches). Hair loss, usually temporary, may also occur, and the nails may become brittle and pitted. If the disease is not eradicated with antibiotics, it establishes latent infection that may cause multiple destructive changes in many organ systems years later.

In the latent (formerly called tertiary stage), tissue destruction occurs in the aorta, the CNS, bone, and skin. The consequences include aortic aneurysm, meningitis, sensory and gait disturbances, dementia, and optic atrophy.

Serological Tests for Syphilis

The common laboratory tests for syphilis lack optimal sensitivity or specificity. Screening is usually performed with the nontreponemal rapid plasma reagin test (RPR) or the Venereal Disease Research Laboratory test (VDRL); either test may yield inaccurate results. Both tests become reactive about 1 to 2 weeks after initial infection. If either test result is positive, a confirmatory test is done: (1) by identifying the responsible bacterium, T. pallidum on dark-field examination of material from a genital lesion; (2) with the microhemagglutination assay for antibody to T. pallidum (MHA-TP); or (3) with the fluorescent treponemal antibody absorption test (FHA-ABS). Two-stage testing increases the likelihood of obtaining an accurate diagnosis.

CAUTION!

Those diagnosed with syphilis may have other STDs, esp. HIV infection. Public health experts recommend testing everyone with either of these diseases for the other one and for other STDs (gonorrhea, Chlamydia, or trichomoniasis).

Treatment

Intravenous or long-acting intramuscular preparations of penicillin are typically given to patients with syphilis. The duration of treatment varies depending on the stage of the disease and on whether there are comorbid illnesses, e.g., HIV infection, or complications, e.g., evidence of neurosyphilis. Doxycycline or tetracycline may be substituted in nonpregnant patients who are allergic to penicillin although, because of potential bacterial resistance, patients allergic to penicillin should be considered candidates for desensitization. Pregnant patients are not given tetracycline or doxycycline because they discolor primary teeth in the infant.

Patient care

The patient is taught about the illness and the importance of locating all sexual contacts, treatment, and the need for follow-up care. The patient should avoid sexual contact with anyone until the full course of therapy has been completed, including previous partners who have not received adequate evaluation and treatment, if indicated, for syphilis. Contact precautions are instituted from the time the disease is suspected until 24 hr after initiation of proper antibiotic therapy and whenever draining lesions are present. Standard precautions apply. The patient is informed about safe sex practices and consistent condom use to prevent infection with syphilis and other STDs. Pregnant patients are screened for syphilis to prevent prenatal transmission. Rape victims are tested at the time of the attack and again 1 to 2 weeks later. All cases of syphilis must be reported to local public health authorities by both health care providers and laboratories.

cardiovascular syphilis

Tertiary syphilis involving the heart and great blood vessels, esp. the aorta. Saccular aneurysms of the aorta and aortic insufficiency frequently result.

congenital syphilis

Syphilis transmitted from the mother to the fetus in utero. Transplacental fetal infection may occur if a pregnant woman is not treated by the 18th week of gestation or contracts the disease later in pregnancy. In the U.S. in 2000, 529 cases of congenital syphilis were reported.

Synonym: prenatal syphilis

endemic syphilis

Chronic, nonvenereal syphilis infection of childhood. It is characterized in its early stages by mucocutaneous or membrane lesions. Later, gummas of bone and skin occur. Penicillin is the treatment of choice.

extragenital syphilis

Syphilis in which the primary chancre is located elsewhere than on genital organs.

latent syphilis

The phase of syphilis during which symptoms are absent and the disease can be diagnosed only by serological tests.

meningovascular syphilis

A form of neurosyphilis in which the meninges and vascular structures of the brain and spinal cord are involved. It may be localized or general.

Synonym: meningovascular neurosyphilis

prenatal syphilis

Congenital syphilis.

serological tests for syphilis

See: serological tests for syphilis

tertiary syphilis

The third and most advanced stage of syphilis.

visceral syphilis

Syphilis in which visceral organs are involved.

syphilis

A sexually or congenitally transmitted disease caused by the spirochaete Treponema pallidum . In the adult form the first sign is the chancre-a single, small, painless, hard-edged ulcerated crater with a wet base, teeming with spirochaetes, that appears on the genitalia about 3 weeks after exposure. The local lymph nodes enlarge. The chancre heals and the spirochaetes disperse throughout the body to cause a secondary stage, featuring conspicuous skin rashes, and a tertiary stage, many years later, in which the nervous system and the larger arteries may be affected, with serious consequences. Tertiary syphilis may involve TABES DORSALIS, GENERAL PARALYSIS OF THE INSANE and ANEURYSM of the AORTA. The prevalence of syphilis was declining until the 1980s but rose again in the late 1990s and in some risk groups has increased more than ten-fold. Treatment is by a single depot dose of benzathine penicillin G which maintains bactericidal levels for weeks. Resistance to the macrolide antibiotic azithromycin has been detected. This is associated with an A to G point mutation in the 23S ribosomal RNA genes of T. pallidum . See also CONGENITAL SYPHILIS.

syphilis

a human disease of the sexual organs, caused by the spirochaete bacterium Treponema pallidum, that is normally spread by sexual contact. The organism penetrates mucous membranes, giving rise within a few days to primary ulceration of the membranes. This may give way to a secondary stage of low-grade fever and enlargement of the lymph nodes. A final tertiary stage can take place in which serious lesions occur in many organs and blindness is common. The bacterium can be transmitted from an infected mother to her foetus, resulting in congenital syphilis which may be fatal.

anisocoria 

Condition in which the pupils of the eyes are not of equal size. Typically one pupil is abnormal and cannot either dilate or constrict. It may be physiological (e.g. in antimetropia) or it may be part of a syndrome, the most common being those of Adie's and Horner's. Physiological anisocoria remains constant irrespective of the level of illumination. Anisocoria can occur as a result of injury (e.g. to the iris sphincter muscle), inflammation (e.g. iridocyclitis), diseases of the iris, paralysis of the third nerve, angle-closure glaucoma, systemic diseases (e.g. diabetes, syphilis) or accidental drug instillation into the eye (if the drug or substance has anticholinergic properties the condition is then referred to as anticholinergic mydriasis or 'atropine' mydriasis). The search for the cause of anisocoria is facilitated by testing the pupil light reflexes and responses to locally instilled drugs (Fig. A14). See efferent pupillary defect; pupil light reflex; pupillometer.

Fig. A14 Anisocoria

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conjunctivitis 

Inflammation of the conjunctiva. It may be acute, subacute or chronic. It may be due to an allergy, an infection (e.g. Staphylococcus, Streptococcus, Haemophilus, etc.), a virus inflammation, an irritant (dust, wind, chemical fumes, ultraviolet radiation or contact lenses), or as a complication of gonorrhoea, syphilis, influenzae, hay fever, measles, etc. Conjunctivitis is characterized by various signs and symptoms, which may include conjunctival injection, oedema, small follicles or papillae, secretions (purulent, mucopurulent, membranous, pseudomembranous or catarrhal), pain, itching, grittiness and blepharospasm. The most common type of conjunctivitis is that due to a bacterium and in many cases is self-limiting and subsides without treatment. Treatment of that type includes irrigation of the lid and the use of topical antibiotics. See conjunctival concretions; herpes zoster ophthalmicus; conjunctival injection; mycophthalmia; ophthalmia neonatorum; Stevens-Johnson syndrome; trachoma.
actinic conjunctivitis See actinic keratoconjunctivitis.
acute conjunctivitis Conjunctivitis characterized by an onset of hyperaemia (most intense near the fornices), purulent or mucopurulent discharge and symptoms of irritation, grittiness and sticking together of the eyelids on waking. In severe cases there will be chemosis, eyelid oedema, subconjunctival haemorrhages and photophobia. The bacterial type is caused by Staphylococcus epidermidis, Staph. aureus, Haemophilus influenzae (H. aegyptius, Koch-Weeks bacillus), Streptococcus pneumoniae (pneumococcus). A rare form of acute conjunctivitis is caused by the Neisseria species (gonococcus, meningococcus, e.g. gonococcal conjunctivitis), which produce a more severe form of the disease referred to as hyperacute bacterial conjunctivitis or acute purulent conjunctivitis. These require immediate treatment with systemic and topical antibiotics. Acute conjunctivitis is also caused by viruses (viral conjunctivitis), such as herpes simplex or adenoviruses. All forms of acute conjunctivitis occasionally spread to the cornea. Bacterial conjunctivitis often resolves without treatment within two weeks. Management consists of topical antibiotic therapy (e.g. chloramphenicol, erythromycin) and cold compresses to relieve symptoms. Acute allergic conjunctivitis most typically resolves spontaneously, otherwise treatment includes sodium cromoglicate. Acute viral conjunctivitis caused by herpes simplex is treated with antiviral agents (e.g. acyclovir), although viral conjunctivitis caused by other viruses does not respond well to any drug therapy. Supportive treatment such as cold compresses relieves symptoms.
acute haemorrhagic conjunctivitis A highly contagious viral infection of the anterior segment resulting in haemorrhage of the bulbar conjunctiva. The infection is caused by a picornavirus, often associated with pre-auricular adenopathy and a follicular conjunctivitis. The infection is self-limited and lasts 7-10 days. No specific treatment is presently available.
adult inclusion conjunctivitis An acute conjunctivitis caused by the serotypes D to K of Chlamydia trachomatis and typically occurring in sexually active adults in whom the genitourinary tract is infected. Signs in the eye usually appear one week following sexual exposure. It may also occur after using contaminated eye cosmetics or soon after having been in a public swimming pool, or in newborn infants (called neonatal inclusion conjunctivitis or neonatal chlamydial conjunctivitis), which is transmitted from the mother during delivery and appears some 5 to 14 days after birth. The conjunctivitis is mucopurulent with follicles in the fornices, which often spread to the limbal region. The condition is commonly associated with punctate epithelial keratitis, preauricular lymphadenopathy, marginal infiltrates and, in long-standing infection, micropannus in the superior corneal region may also appear. Differentiation from viral follicular conjunctivitis is made through culture, serological and cytological studies. Treatment consists of using both systemic and topical tetracyclines, although in pregnant or lactating women erythromycin is preferable. Syn. trachoma-inclusion conjunctivitis (TRIC). See conjunctival follicle; punctate epithelial keratitis; lymphadenopathy; ophthalmia neonatorum; trachoma.
allergic conjunctivitis Conjunctivitis which is due to a type 1 hypersensitivity reaction to allergens. Common allergens are pollens associated with hay fever, grass (seasonal allergic conjunctivitis) and air pollutants, house dust mites, smoke (perennial allergic conjunctivitis). It is characterized by hyperaemia, itching, burning, swelling, tearing, discharge and small papillae. Conjunctival scrapings contain a large number of eosinophils and serum IgE is elevated. The condition is commonly associated with rhinitis (allergic rhinoconjunctivitis) in which there is also sneezing and nasal discharge. Treatment commonly includes decongestants, oral antihistamines, mast cell stabilizers (e.g. lodoxamine, sodium cromoglicate) and if severe, topical corticosteroid eyedrops. See vernal conjunctivitis; decongestants; hypersensitivity.
angular conjunctivitis Subacute bilateral inflammation of the conjunctiva due to the diplobacillus of Morax-Axenfeld. It involves the conjunctiva in the region of the canthi.
bacterial conjunctivitis See acute conjunctivitis.
catarrhal conjunctivitis Type of conjunctivitis associated with the common cold or catarrhal irritation. It can appear in the acute or chronic form.
contagious conjunctivitis Acute conjunctivitis caused by Koch-Weeks bacillus, adenovirus types 3, 7 or 8 and 19, or a pneumococcus infection. It may be transmitted by respiratory or ocular infections, contaminated towels or equipment (e.g. tonometer heads). It is characterized by acute onset, redness, tearing, discomfort and photophobia. The condition is often self-limiting but keratitis is a common complication. Syn. epidemic conjunctivitis; epidemic keratoconjunctivitis; pink eye (colloquial).
eczematous conjunctivitis See phlyctenular conjunctivitis.
egyptian conjunctivitis See trachoma.
epidemic conjunctivitis See contagious conjunctivitis.
flash conjunctivitis Conjunctivitis due to exposure to an electric arc, as from a welder's torch.
follicular conjunctivitis Conjunctivitis characterized by follicles (usually in one eye only) caused by adenoviruses or chemical or toxic irritation and frequently associated with lymph-adenopathy. See adult inclusion conjunctivitis; conjunctival follicle; lymphadenopathy.
fungal conjunctivitis See mycophthalmia.
giant papillary conjunctivitis (GPC) Conjunctivitis, characterized by the appearance of 'cobblestones' (large papillae of 0.5 mm or more) on the tarsal conjunctiva of the upper eyelid (and sometimes the lower eyelid). Symptoms include itching, discomfort, mucous discharge and poor vision due to the presence of mucus. The condition may be induced by contact lens wear, ocular prosthesis, or exposed sutures following surgery. This conjunctivitis closely resembles vernal conjunctivitis and is also believed to be an allergic condition. In its early stages as a contact lens-induced condition, it is often referred to as contact lens papillary conjunctivitis or contact lens associated papillary conjunctivitis (CLPC, CLAPC). In these cases the regular use of surfactant and protein removal tablets as well as frequent lens replacement reduce the incidence of this condition, which is less prevalent with the wear of rigid gas permeable than soft contact lenses. Management may also include mast cell stabilizers (e.g. sodium cromoglicate) or antihistamine (e.g. levocabastine) and cessation of lens wear. See vernal conjunctivitis; contact lens deposits; enzyme; surfactant.
gonococcal conjunctivitis See acute conjunctivitis.
granular conjunctivitis See trachoma.
lacrimal conjunctivitis Chronic conjunctivitis caused by an infection of the lacrimal passages. See lacrimal apparatus.
ligneous conjunctivitis A rare, chronic conjunctivitis characterized by the formation of a firm, whitish membrane or pseudomembrane on the tarsal conjunctiva, usually of the upper eyelid. It is typically bilateral, begins in childhood although it may present in patients up to age 85, is more common in females than in males and may persist for months or years. Its cause is unknown but the predisposing factors include bacterial and viral infections, trauma, hypersensitivity reactions and increased vascular permeability, and it is often associated with inflammations of other mucous membranes. The most effective treatment is surgical excision followed by topical cyclosporine drops, but the condition has a tendency to recur. See pseudomembranous conjunctivitis.
membranous conjunctivitis See pseudomembranous conjunctivitis.
neonatal conjunctivitis See ophthalmia neonatorum.
phlyctenular conjunctivitis See phlyctenular keratoconjunctivitis.
pseudomembranous conjunctivitis A non-specific inflammatory reaction characterized by the formation on the conjunctiva of a coagulated fibrinous plaque consisting of inflammatory cells and an exudate containing mucus and proteins. This plaque forms either a membrane or a pseudomembrane. The latter is loosely adherent to the conjunctival epithelium and can be peeled off without bleeding or damage to the underlying epithelium. A true membrane, on the other hand, usually occurs with intense inflammation (membranous conjunctivitis). In this case the conjunctival epithelium becomes necrotic and adheres firmly to the overlying membrane which when peeled leaves a raw, bleeding surface. The cause of either condition may be an infection, of which the common sources are herpes simplex virus, adenovirus, beta-haemolytic Streptococcus, Neisseria gonorrhoeae or as a result of the Stevens-Johnson syndrome, ligneous conjunctivitis, ocular cicatricial pemphigoid, atopic keratoconjunctivitis, chemical burns (especially alkali burns), radiation injury or post-surgical complications.
sun lamp conjunctivitis See actinic keratoconjunctivitis.
swimming pool conjunctivitis See adult inclusion conjunctivitis.
vernal conjunctivitis Chronic, bilateral conjunctivitis which recurs in the spring and summer and is more often seen in boys than girls. Its origin is probably due to an allergy. It is characterized by hard flattened papillae of a bluish-white colour separated by furrows and having the appearance of 'cobblestones' located in the upper palpebral portion of the conjunctiva with mucus deposition between the papillae. A second type of vernal conjunctivitis exists which affects the limbal region of the bulbar conjunctiva, characterized by the formation of small, gelatinous white dots called Trantas' dots or Horner-Trantas' dots. The chief symptom of the disease is intense itching. Treatment consists mainly of cold compresses and limited (because of side effects) use of topical corticosteroids (e.g. dexamethasone, prednisolone). Sodium cromoglicate or lodoxamide have also been found to be very successful in treating this condition and with fewer side effects than corticosteroids. Syn. vernal keratoconjunctivitis (VKC) (although this is not strictly speaking a synonym since the condition often involves the cornea; spring catarrh; vernal catarrh (Fig. C14). See antihistamine; atopic keratoconjunctivitis; mast cell stabilizers.
viral conjunctivitis Conjunctivitis caused by a virus. A variety of viruses can produce the disease. See acute conjunctivitis.

Fig. C14 'Cobblestones' papillae in severe vernal conjunctivitis

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dacryoadenitis

Inflammation of the lacrimal gland. The acute type is characterized by localized pain, swelling and redness over the upper temporal area of the eye. The chronic type is painless and develops slowly. A frequent cause is an associated systemic infection such as mumps, infectious mononucleosis, influenza, or it can be due to a local condition such as trachoma, herpes zoster or staphylococcal infection. The chronic type may be due to any of the granulomatous diseases (tuberculosis, syphilis, sarcoidosis). Treatment consists mainly of warm compresses and antibiotics. See Mikulicz's syndrome.

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luxation of the lens

Pathological and complete dislocation of the lens relative to the pupil. If the luxation is incomplete it is called subluxation of the lens (or dislocation or ectopia lentis). Subluxation is one of the causes of monocular diplopia. If the luxation is complete the eye becomes markedly hyperopic and is unable to accommodate. Luxation occurs in contusion of the globe, in many ocular (e.g. buphthalmos) and other diseases (e.g. syphilis) or it can be inherited (e.g. the bilateral, symmetrical, superior subluxation commonly found in Marfan's syndrome or homocystinuria). It is sometimes associated with ectopic pupils and keratoconus. Unless there are complications (e.g. secondary glaucoma) or monocular diplopia the lens is left in place and management is optical. (Fig. L21) See corectopia; iridodonesis; pupillary block.

Fig. L21 Dislocation of the lens (usually in the vitreous humour)

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madarosis

Loss of, either or both, the eyebrows and the eyelashes. It may occur in blepharitis, in the presence of hair follicle mites, trauma from rubbing, in alopecia, in leprosy, or as a complication of acquired syphilis.

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neuritis, optic

Inflammation of the optic nerve, which can occur anywhere along its course from the ganglion cells in the retina to the synapse of these cell fibres in the lateral geniculate body. If the inflammation is restricted to the optic nerve head the condition is called papillitis (or intraocular optic neuritis) and if it is located in the orbital portion of the nerve it is called retrobulbar optic neuritis (or orbital optic neuritis).In papillitis the optic nerve head is hyperaemic with blurred margins and slightly oedematous. Haemorrhages and exudates may also appear. In retrobulbar optic neuritis, there are usually no visible signs in the fundus of the eye until the disease has advanced and optic atrophy may appear. However, both types are accompanied by a loss of visual acuity along with a central scotoma and impairment of colour vision. The loss of vision may occur abruptly over a few hours and recovery may be equally rapid but in some patients the loss may be slow. In retrobulbar optic neuritis, there is also pain on movement of the eyes and sometimes tenderness on palpation. The disease is usually unilateral although the second eye may become involved later. It is usually transient and full or partial recovery takes place within weeks. The primary cause of optic neuritis is multiple sclerosis but it may also be associated with severe inflammation of the retina or choroid, vitamin B deficiency, diabetes mellitus, thyroid disease, lactation, toxicity or syphilis. See Devic's disease; papilloedema; Marcus Gunn pupil; Kollner's rule; photostress test.

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tabes dorsalis 

A degenerative disease of the posterior columns of the spinal cord, the posterior spinal roots and the peripheral nerves accompanied by a number of ocular signs and symptoms such as atrophy of the optic nerve, visual field defects, ptosis, Argyll Robertson pupil and paralysis of one or more of the extraocular muscles. The disease is a result of neurosyphilis.

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uveitis 

Inflammation of the uvea. All three tissues of the uvea tend to be involved to some extent in the same inflammatory process because of their common blood supply. However, the most severe reaction may affect one tissue more than the others as in iritis, cyclitis or choroiditis or sometimes two tissues, e.g. iridocyclitis. The symptoms also vary depending upon which part of the tract is affected. Acute anterior uveitis is accompanied by pain, photophobia and lacrimation and some loss of vision because of exudation of cells (aqueous flare), protein-rich fluid and fibrin into either the anterior chamber or vitreous body, as well as ciliary injection, adhesion between the iris and lens (posterior synechia), miosis and keratic precipitates. The condition is often associated with ankylosing spondylitis, rheumatoid arthritis, sarcoidosis, syphilis or tuberculosis (usually with granulomatous uveitis). It is the most common form of uveitis. Many cases are HLA-B27 positive. Treatment includes corticosteroids and mydriatics to reduce the risk of posterior synechia and to relieve a spasm of the ciliary muscle. See juvenile idiopathic arthritis; Reiter's disease; Busacca's nodules; Koeppe's nodules; sympathetic ophthalmia; Behçet's syndrome; phthisis bulbi; synchisis scintillans; Vogt-Koyanagi-Harada syndrome; Table I6.
fungal uveitis Uveitis caused by a fungus such as Candida albicans, Cryptococcus neoformans and Histoplasma capsulatum. It is often accompanied by other disorders (e.g. choroiditis, retinitis). It may have spread from other bodily tissues (e.g. skin, mouth, gastrointestinal tract) in patients who are intravenous drug addicts, patients with indwelling venous catheters or patients who are immunosuppressed.
intermediate uveitis A chronic inflammation of the ciliary body (cyclitis) or its pars plana zone (pars planitis) or of the peripheral retina and vitreous (peripheral uveitis). The cause is unknown in most cases but others are associated with systemic conditions such as multiple sclerosis, sarcoidosis or HIV infection. It affects mainly young adults and is bilateral in about 80% of cases. Symptoms are floaters and, sometimes, blurred vision, and there may be anterior chamber cells and flare. Ophthalmoscopic examination may show vitreous condensation and gelatinous exudates ('cotton balls' or 'snowballs'). Snowbanking, i.e. a whitish plaque or exudates involving the pars plana, often the inferior part of it, appears mainly in pars planitis. Intermediate uveitis may be associated with retinal vasculitis (i.e. inflammation of a retinal blood vessel). In a few cases the condition is self-limiting within a few months. However, in most cases the condition lasts several years may lead to complications such as cystoid macular oedema, posterior subcapsular cataract, retinal detachment or cyclitic membrane formation. Treatment includes corticosteroids and in resistant cases immunosuppressive agents.
posterior uveitis A uveitis involving the posterior segment of the eye. Symptoms include floaters and visual loss if the choroiditis involves the macular area. Ophthalmoscopically there is an accumulation of debris in the vitreous and choroidal lesions appear as yellow-white areas of infiltrates surrounded by normal fundus. Retinitis is also present in most cases, as well as retinal vasculitis. Posterior uveitis may be associated with AIDS, Behçet's disease, Lyme disease, histoplasmosis, sarcoidosis, toxoplasmosis, syphilis, tuberculosis, Vogt-Koyanagi-Harada syndrome, sympathetic ophthalmia, etc.
viral uveitis Uveitis caused by a virus. Common viruses are: herpes simplex, which is usually associated with keratitis and may cause anterior uveitis; herpes zoster which may also be associated with keratitis; human T-cell lymphotrophic virus; measles; cytomegalovirus; rubella; human immunodeficiency virus (HIV). See herpes simplex blepharoconjunctivitis; herpes zoster ophthalmicus.

syph·i·lis

(sif'i-lis)

An acute and chronic infectious disease caused by the bacterium Treponema pallidum and transmitted by direct contact, usually sexual.