Syphilis
From Wikipedia, the free encyclopedia
Syphilis is a sexually transmitted infection (STI) caused by a spirochaete bacterium, Treponema pallidum. Syphilis has many alternate names, including "syph", "Cupid's Disease", "the Pox" (or "great pox", to distinguish it from smallpox), "lues" (hence luiphobia, or fear of syphilis), and the "French disease."
The route of transmission of syphilis is almost invariably by sexual contact; however, there are examples of direct contact infections (see yaws) and of congenital syphilis (transmission from mother to child in utero).
The signs and symptoms of syphilis are myriad; before the advent of serological testing, diagnosis was more difficult and the disease was dubbed the "Great Imitator" because it was so often confused with other diseases.
Syphilis can be treated with penicillin or other antibiotics. Statistically, oral treatment is dramatically less effective than other treatments because patients tend not to complete the course. The oldest, and still most effective, method is to inject benzathine penicillin into each buttock (procaine is added to make the pain bearable); the dose must be given half in each buttock because the amount given would be too painful if given in a single injection.
Another treatment is to administer several tablets of azithromycin (which has a long duration of action) orally under observation. This latter course may soon be obsolete, as strains of syphilis resistant to azithromycin have developed, and accounted for 56% of San Francisco infections in 2004.[1] Other treatments are less effective as the patient is required to take pills several times a day.
If not treated, syphilis can cause serious effects such as damage to the nervous system, heart, or brain. Untreated syphilis can be fatal.
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| Depression-era U.S. poster advocating early syphilis treatment | |
| ICD-10 | A50.-A53. |
| ICD-9 | 090-097 |
Contents |
[edit] History
There are two thoughts on the origin of syphilis: the Columbian and pre-Columbian theories. There are ongoing debates in anthropological and historical fields about the validity of either theory.
The pre-Columbian theory holds that syphilis symptoms are described by Hippocrates in Classical Greece in its venereal/tertiary form. Some passages in the Bible could refer to syphilis, especially Exodus 20:5 where the sins of the father are visited unto the third and fourth generation. There are other suspected syphilis findings for pre-contact Europe, including at a 13-14th century Augustinian friary in the northeastern English port of Kingston upon Hull. The anthropological evidence is contested by those who follow the Columbian theory.
The Columbian Exchange theory holds that syphilis was a New World disease brought back by Columbus. The first well-recorded outbreak of what we know as syphilis occurred in Naples in 1494. There is some documentary evidence to link Columbus's crew to the outbreak. Supporters of the Columbian theory find syphilis lesions on pre-contact Native Americans. Again, all the anthropological evidence is heatedly discussed on both sides of the Columbian/pre-Columbian debate. (Baker, et al.)
Alfred Crosby has argued that neither side has the full story. Syphilis is a form of Yaws, similar to tuberculosis which has existed in the Old World since time immemorial. Crosby argues that syphilis is a specific form of Yaws that had evolved in the New World and was brought back to the old, "the differing ecological conditions produced different types of treponematosis and, in time, closely related but different diseases". (ref:225 Crosby)
The epidemiology of the first syphilis epidemic indicates that the disease was either new or a mutated form of an earlier disease. The disease swept across Europe from its early epicenter at Naples. As Jared Diamond describes it, "when syphilis was first definitely recorded in Europe in 1495, its pustules often covered the body from the head to the knees, caused flesh to fall people's faces, and led to death within a few months." In addition, the disease was more frequently fatal than it is today. Diamond concludes that "by 1546, the disease had evolved into the disease with the symptoms so well known to us today."[1]
Syphilis had many different names. Great pox was used during the 16th century to distinguish it from smallpox. In its early stages, great pox produced a rash similar to smallpox (variola) -- hence the use of the word pox; but has no relation to the smallpox virus -- hence use of the adjective great. However, the name is misleading, as smallpox was a far more deadly disease. Because of the outbreak in the French army, it was first called morbus gallicus, or the French disease. In that time the Italians also called it the "Spanish disease", the French called it the la maladie anglaise ("the English disease") and "Italian disease" or "Neapolitan disease", the Russians called it the "Polish disease", and the Arabs called it the "Disease of the Christians". The term "lues" was also used.
A number of famous historical personages, including Charles VIII, Hernando Cortez of Spain, and Ivan the Terrible, have been alleged to have had syphilis. Guy de Maupassant and Friedrich Nietzsche are both thought to have been driven insane and ultimately killed by the disease. Al Capone contracted syphilis as a young man. By the time he was incarcerated at Alcatraz, it reached its third stage, neurosyphilis, making him confused and disoriented. The painter Paul Gauguin is also said to have suffered from syphilis.
The insanity caused by late-stage syphilis was once one of the more common forms of dementia; this was known as the general paresis of the insane.
[edit] Etymology
"Syphilis" is (somewhat inaccurate) Greek for "lover of swine" (correctly συ-φιλος) or "one who makes love" (συμ-φιλος). It was the name of a shepherd or swineherd who was the protagonist in an epic poem written in Latin by the Italian physician and poet Girolamo Fracastoro, entitled Syphilis sive morbus gallicus (Latin for "Syphilis or The French Disease") (1530); in the author's intention "lover" in the name may not have been intended to have a sexual meaning. In the poem, Syphilis was the first who contracted the disease, which was sent as a plague by the god Apollo, as punishment for the defiance that Syphilis and his followers showed him. Later, people who did not know the story guessed wrongly from the title that "Syphilis" was another name for the disease; compare "mayhem" and "premises" and "algorithm" for words whose main meanings have changed because of people misunderstanding their context.
[edit] Stages of syphilis
Different manifestations occur at each stage of the disease:-
[edit] Primary syphilis
Primary syphilis is manifested after an incubation period of 10-90 days (the average is 21 days) with a primary sore. During the initial incubation period, individuals are asymptomatic. The sore, called a chancre, is a firm, painless skin ulceration localized at the point of initial exposure to the bacterium, often on the penis, vagina or rectum. Local lymph node swelling can occur. The primary lesion may persist for 4 to 6 weeks and then heal spontaneously.
[edit] Secondary syphilis
Secondary syphilis is characterized by a skin rash that appears 1-6 months (commonly 6 to 8 weeks) after the primary infection. This is a symmetrical reddish-pink non-itchy rash on the trunk and extremities, which unlike most other kinds of rash involves the palms of the hands and the soles of the feet; in moist areas of the body the rash becomes flat broad whitish lesions called condylomata lata. Mucous patches may also appear on the genitals or in the mouth. A patient with syphilis is most contagious when he or she has secondary syphilis.
Other symptoms common at this stage include fever, sore throat, malaise, weight loss, headache, meningismus, and enlarged lymph nodes. Rare manifestations include an acute meningitis that occurs in about 2% of patients, hepatitis, renal disease, hypertrophic gastritis, patchy proctitis, ulcerative colitis, rectosigmoid mass, arthritis, periostitis, optic neuritis, iritis, and uveitis.
[edit] Tertiary syphilis
Tertiary syphilis occurs from as early as one year after the initial infection but can take up to ten years to manifest - though cases have been reported where this stage has occurred fifty years after initial infection. This stage is characterised by gummas, soft, tumor-like growths, readily seen in the skin and mucous membranes, but which can occur almost anywhere in the body, often in the skeleton. Other characteristics of untreated syphilis include Charcot's joints (a degeneration of joint surfaces resulting from loss of proprioception), and Clutton's joints (bilateral knee effusions). The more severe manifestations include neurosyphilis and cardiovascular syphilis.
Neurological complications at this stage include generalized paresis of the insane which results in personality changes, changes in emotional affect, hyperactive reflexes, and Argyll-Robertson pupils, a diagnostic sign in which the small and irregular pupils constrict in response to focusing the eyes, but not to light; Tabes dorsalis, also known as locomotor ataxia, a disorder of the spinal cord, often results in a characteristic shuffling gait.
Cardiovascular complications include syphilitic aortitis, aortic aneurysm, aneurysm of sinus of Valsalva, and aortic regurgitation, and are a frequent cause of death. Syphilitic aortitis can cause de Musset's sign (a bobbing of the head that de Musset first noted in Parisian prostitutes).
[edit] Latent syphilis
Latent syphilis is defined as having serologic proof of infection without signs or symptoms of disease. Latent syphilis is further described as either early or late. Early latent syphilis is defined as having syphilis for one year or less from time of initial infection without signs or symptoms of disease. Late latent syphilis, then, is infection for greater than one year but having no clinical evidence of disease. The distinction is important for two reasons, therapy and risk for transmission.
In practice, the time of initial infection is often not known and in this case should then be presumed to be late for the purpose of therapy. Early latent syphilis may be treated with a single IM injection of a long-acting penicillin. Late latent syphilis, however, requires three such injections, each a week apart. For infectiousness, however, late latent syphilis is not considered contagious while early latent is. Thus, if the duration of infection is not known, one should presume the patient is early and contagious.
[edit] Neurosyphilis
Neurosyphilis refers to a site of infection involving the neurologic system. As such, neurosyphilis may occur at any stage of syphilis. Neurosyphilis in patients with HIV infection is well described. Reports of neurosyphilis in HIV-infected persons are similar to cases reported before the HIV epidemic. The precise extent and significance of neurologic involvement in HIV-infected patients with syphilis, reflected by either laboratory or clinical criteria, remain incompletely characterized. Furthermore, the alteration of host immunosuppression by ART in recent years has further complicated such characterization.
There are four clinical types.
Approximately 35% to 40% of persons with secondary syphilis have asymptomatic CNS involvement, as demonstrated by any of these on CSF examination:-
- An abnormal cell count, protein level, or glucose level;
- Demonstrated reactivity to Venereal Disease Research Laboratory (VDRL) antibody test.
Acute syphilitic meningitis usually occurs within the first 2 years of infection; 10% of cases are diagnosed at the time of the secondary rash. Patients present with headache, meningeal irritation, and cranial nerve abnormalities, typically involving cranial nerves at the base of the brain. Meningovascular syphilis occurs a few months to 10 years (average, 7 years) after the primary infection. Unlike the sudden onset of thrombotic or embolic stroke syndromes, meningovascular syphilis is associated with prodromal symptoms lasting weeks to months before focal deficits of a vascular syndrome are identifiable. Prodromal symptoms include unilateral numbness, paresthesias, extremity weakness, headache, vertigo, insomnia, and psychiatric abnormalities such as personality changes. The focal deficits initially are intermittent or progress slowly over a few days.
[edit] Testing
It was only in the 20th century that effective tests and treatments for syphilis were developed.
In 1906, the first effective test for syphilis, the Wassermann test, was developed. Although it had some false positive results, it was a major advance in the prevention of syphilis. By allowing testing before the acute symptoms of the disease had developed, this test allowed the prevention of transmission of syphilis to others, even though it did not provide a cure for those infected. In the 1930s the Hinton test, developed by William Augustus Hinton, and based on flocculation, was shown to have fewer false positive reactions than the Wasserman test. Both of these early tests have been superseded.
Present-day syphilis tests, such as the Rapid Plasma Reagin (RPR) and Venereal Disease Research Laboratory (VDRL) tests, while useful, are still not completely specific, as many other conditions can cause a positive result. Tests based on monoclonal antibodies and immunofluorescence, including Treponema pallidum haemagglutination assay (TPHA) and Fluorescent Treponemal Antibody Absorption (FTA-ABS), are more specific, but are still unable to rule out related treponomal infections such as yaws and pinta. However, a simple microscopy of chancre fluid using dark ground illumination provide a quick and effective test.
Neurosyphilis is diagnosed by finding lymphocytes in the CSF in the presence of positive syphilis serology. There is anecdotal evidence that the incidence of neurosyphilis is higher in HIV patients, and some have recommended that all HIV-positive patients with a diagnosis of syphilis should also have a lumbar puncture to look for asymptomatic neurosyphilis.[3]
In one of the best-documented episodes of unethical human medical experimentation in the twentieth century, the Tuskegee syphilis study continued to study the lifetime course of syphilis in a group of African Americans long after effective treatments for syphilis were available.
In the July 17, 1998 issue of the journal Science, a group of biologists reported complete sequencing of the genome of T. pallidum.
[edit] Treatment
[edit] History
There were originally no effective treatments for syphilis. The most common in use were guaiacum and mercury: the use of mercury gave rise to the saying "A night in the arms of Venus leads to a lifetime on Mercury".[citation needed] It was administered multiple ways including by mouth and by rubbing it on the skin. One of the more fascinating methods was fumigation, in which the patient was placed in a closed box with his head sticking out. Mercury was placed in the box and a fire was started under the box which caused the mercury to vaporize. It was a gruelling process for the patient and the least effective for delivering mercury to the body.
As the disease became better understood, more effective treatments were found, beginning with the use of the arsenic-containing drug Salvarsan from 1910, and later Neosalvarsan.
Unfortunately, these drugs were not 100% effective, especially in late disease. It had been observed that some who develop high fevers could be cured of syphilis. Thus, for a brief time malaria was used as treatment because it produces prolonged and high fevers. This was considered an acceptable risk because the malaria could later be treated with quinine which was available at that time. Malaria as a treatment for syphilis was usually reserved for late disease, especially neurosyphilis, and then followed by either Salvarsan or Neosalvarsan as adjuvunct therapy.
These treatments were finally rendered obsolete by the discovery of penicillin, and its widespread manufacture after World War II allowed syphilis to be effectively cured for the first time.
[edit] Current treatment
The first choice treatment for primary, secondary, and early latent infection remains penicillin, in the form of Benzathine penicillin G, 2.4 MU IM in a single dose. Individuals who have severe allergic reactions to penicillin (e.g., anaphylaxis) may be effectively treated with oral tetracyclines (100 mg orally twice a day for 14 days). Ceftriaxone may be considered as an alternative therapy, although the optimal dose is not yet defined and close clinical and serologic follow-up is essential. If ceftriaxone is used for the treatment of early syphilis, some experts recommend 1 g daily, given intramuscularly or intravenously, for 8 to 10 days. (47,159,160)
Late latent and infections of unknown duration If CSF examination yields no evidence of neurosyphilis, then a total of 7.2 million units of benzathine penicillin G is recommended (administered as 3 doses of 2.4 million units by intramuscular injection weekly for 3 successive weeks). If allergic, then tetracyclines may be used for this stage also, but for 28 days instead of the normal 14.
For patients diagnosed with neurosyphilis (including ocular or auditory syphilis with or without positive LP results), aqueous crystalline penicillin G is the treatment of choice (administered as 18-24 million units intravenously per day; ie, 3-4 million units every 4 hours or continuous infusion for 10-14 days). If intravenous administration is impossible, then aqueous procaine penicillin G is an alternative (administered as 2.4 million units intramuscularly daily plus probenecid 500 mg by mouth 4 times daily for 14 days). Procaine injections are painful, however, and patient compliance may be difficult to ensure. To approximate the 21-day course of therapy for late latent disease and to address concerns about slowly dividing treponemes, most experts now recommend 3 weekly doses of benzathine penicillin G (total 7.2 million units intramuscularly) after the completion of a 14-day course of aqueous crystalline or aqueous procaine penicillin G for neurosyphilis. No oral antibiotic alternatives are recommended for the treatment of neurosyphilis, but in special circumstances the only alternative that has been studied is ceftriaxone 2 g intramuscularly for 14 days.
Alternative regimens (eg, tetracyclines) are not well studied in HIV infection and a careful follow-up is recommended. Tetra-cyclines are contraindicated in pregnancy. Skin testing or desensitization to facilitate therapy with penicillin is recommended in pregnant patients and for treatment of latent syphilis and neurosyphilis in other patients with HIV infection. Follow-up includes clinical evaluation at 1 to 2 weeks followed by clinical and serologic evaluation at 3, 6, 9, 12, and 24 months after treatment.
Oral Azithromycin given as a single dose of 2 g has been used successfully to treat syphilis in a pilot study of 328 patients in Tanzania (Riedner 2005), but resistance to azithromycin (eg, as high as 56% in San Francisco in 2004)[2] has made it an unacceptable alternative.
HIV-infected patients with early syphilis may have a higher risk of neurologic complications and a higher rate of treatment failure with currently recommended regimens. The magnitude of these risks, however, although not precisely defined, is probably small.
Before administering any treatment, clinicians should warn all patients about the possibility of a Jarisch-Herxheimer reaction, which occurs most often in secondary syphilis and with penicillin therapy, and may be more common in HIV-infected patients.[3] This reaction is characterized by fever, fatigue, and transient worsening of any mucocutaneous symptoms, and usually subsides within 24 hours. These symptoms can be alleviated with acetaminophen and should not be mistaken for drug allergy. In addition, clinicians should inform HIV-infected patients that currently recommended regimens may be less effective for them than for patients without HIV infection and that close serologic follow-up is therefore essential.
[edit] Prevention
Avoidance of sexual contact is the only completely reliable method of prophylaxis but is an impractical public health measure for obvious reasons.
Test all patients with syphilis for HIV. Presumptively treat individuals sexually exposed to a person with primary, secondary, or early latent syphilis within 90 days preceding the diagnosis even if seronegative. If the exposure was more than 90 days before the diagnosis, treat presumptively if serologic testing is not immediately available or if follow-up is uncertain. Patients with syphilis of unknown duration and nontreponemal serologic titers ≥1:32 may be considered as having early syphilis for purposes of partner notification and presumptive treatment of sex partners. Evaluate long-term sex partners of patients with late syphilis clinically and serologically and treat appropriately. Educate patients as previously described.
[edit] Syphilis in art and literature
There are references to syphilis in William Shakespeare's play Measure for Measure, particularly in a number of early passages spoken by the character Lucio. For example, Lucio says "[...] thy bones are hollow"; this is a reference to the brittleness of bones engendered by the use of mercury which was then widely used to treat syphilis.
In Shakespeare's play Othello, the clown at the beginning of Act III makes jest of Cassio, who is leading a musician troupe for Othello, by asking him if he had just arrived from Naples and playing with his nose. (Alluding to the reputation of Naples of being a likely place to contract syphilis, which eats away at the bridge of the nose)
Jonathan Swift's poetry mentions syphilis as a condition of prostitution which reaches the highest ranks of society. See, for example, "A Beautiful Young Nymph Going To Bed" and "The Progress of Beauty".
William Hogarth's A Harlot's Progress, a series of six paintings (1731, now lost) and engravings (1732) shows the story of Moll Hackabout, a country girl who moves to London, becomes a prostitute, and eventually dies of a sexually transmitted infection, widely interpreted as being syphilis.
Some critics have argued that the character of Edward Rochester's first wife, Bertha, in Charlotte Brontë's novel Jane Eyre, suffers from the advanced stages of syphilitic infection, general paresis of the insane, and point to corroborative evidence within the text to substantiate this view.
Henrik Ibsen's controversial (at the time) play Ghosts has a young man who is suffering from a mysterious unnamed disease. Though it is never named, the events of the play make it plain that this is syphilis, an inheritance from his dissolute father. Dr Rank in Ibsen's play A Doll's House also has inherited syphilis.
The novel Candide by Voltaire describes Candide's mentor and teacher, Pangloss, as having contracted syphilis from a maidservant he slept with; the syphilis has ravaged and deformed his body. Pangloss explains to Candide that syphilis is 'necessary in the best of worlds' because the line of infection - which he explains - leads back to Christopher Columbus. If Columbus had not sailed to America and brought back syphilis, Pangloss states, the Europeans would not have been able to enjoy 'New World wonders' such as chocolate. (One of the purposes of the novel was to satirize Leibniz's philosophy as Pangloss's disingenuous rose-tinted viewpoint.) Pangloss eventually loses an eye and an ear to the syphilis before he is cured.
The artist Kees van Dongen produced a series of illustrations for the anarchist publication L'Assiette au Beurre showing the descent of a young prostitute from poverty to her death from syphilis as a criticism of the social order at the end of the 19th century.
Also, in Charles Dickens' novel Tale of Two Cities, references are made that allude to the main character, Sydney Carton, having syphilis.
Mention must be made of the anonymous American medical students' description of syphilis in a series of early 20th-century American limericks, using medical terminology to ghastly comic effect. It was first published in Journal of the American Medical Association January 1942: [4]
Thomas Disch in his novel Camp Concentration describe a fictional strain of syphilis that enhances intelligence but is lethal.
In Thomas Mann's novel Doktor Faustus, the Faust character, Adrian Leverkühn, acquires his genius for musical composition from the neurological effects of syphilis.
In Dick Francis' novel, Bonecrack the character Enso Rivera is suffering from megalomania caused by syphilis.
The Russian Author Leo Tolstoy suffered from syphilis during his youth, which was cured using arsenic treatment [4]
Neal Stephenson's trilogy The Baroque Cycle has multiple characters and historical figures who have syphilis, most notably James II of England and Jack Shaftoe; the latter is cured of the disease by running a high fever.
The character of Darla in Buffy the Vampire Slayer and Angel is a colonial-era prostitute dying of syphilis when she is made into a vampire by the Master.
The character of George in Grey's Anatomy got syphilis after sleeping with Nurse Olivia, who got it from sleeping with Alex first.
[edit] See also
- Other treponematous diseases:-
- Rapid Plasma Reagin
[edit] References
- ^ Jared Diamond (1997), Guns, Germs and Steel (New York: W.W. Norton), p. 210.
- ^ Richard B. Jamess, MD, PhD (2002). "Syphilis- Sexually Transmitted Infections, 2006.". Sexually transmitted diseases treatment guidelines.
- ^ Walter T, Lebouche B, Miailhes P, et al. (2006). "Symptomatic relapse of neurologic syphilis after benzathine penicillin G therapy for primary or secondary syphilis in HIV-infected patients". Clin Infect Dis 43 (6): 787–90. PMID 16912958.
- ^ A. N. Wilson, "Tolstoy: A Biography," http://www.amazon.com/Tolstoy-Biography-N-Wilson/dp/0393321223/sr=1-1/qid=1157902690/ref=sr_1_1/103-5801230-9691031?ie=UTF8&s=books.
[edit] External links
- UCSF HIV InSite Knowledge Base Chapter: Syphilis and HIV
- Sexually Transmitted Diseases/Infections Resource Center from the Association of Reproductive Health Professionals
- "A New Gold Standard For Syphilis?" Poster Presentation for European Academy of Dermatology and Venereology 2004 Spring Symposium
- Kipkeepers, Pox and Gleet Vendors: A Rapid History of Syphilis
- POX: Genius, Madness, and the Mysteries of Syphilis
- Secrets of the Dead (PBS): The Syphilis Enigma
- Syphilis and AIDS: Lessons from history
- "Syphilis fact sheet" from the Center for Disease Control
- The chapter on syphilis from a book on Dermatology and Venerology — contains some good pictures
- The treatment of dementia paralytica by malaria inoculation (A Nobel Prize lecture, December 13, 1927)
- Homosexual men boost increase in syphilis rate
- National Institute of Allergy and Infectious Diseases Factsheet
