Cambridge Encyclopedia :: Cambridge Encyclopedia Vol. 73

syphilis

A chronic sexually transmitted disease caused by Treponema pallidum. Initially, a primary lesion (a chancre) develops on the genitals or anus. This is followed several weeks later by features of a generalized infection, with fever and a rash. The condition may then remain dormant for years. Nodules (gumma) then form in the skin and mucous membranes, and these may break down to form large ulcers. Thereafter the cardiovascular and nervous systems become affected, resulting in damage to the heart valves, swelling of the aorta (aneurysm), and a combination of inco-ordination, loss of sensation, and dementia known as generalized paralysis of the insane.

Syphilis
Classifications and external resources
Depression-era U.S. poster advocating early syphilis treatment
ICD-10 A50.-A53. 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;

Syphilis can be treated with penicillin or other antibiotics. 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.

If not treated, syphilis can cause serious effects such as damage to the nervous system, heart, or brain.

History

There are two thoughts on the origin of syphilis: the Columbian and pre-Columbian theories.

The pre-Columbian theory holds that syphilis symptoms are described by Hippocrates in Classical Greece in its venereal/tertiary form. 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 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. Supporters of the Columbian theory find syphilis lesions on pre-contact Native Americans. Syphilis is a form of Yaws, 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.

Syphilis had many different names. 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".

A number of famous historical personages, including Charles VIII, Hernando Cortez of Spain, and Ivan the Terrible, have been alleged to have had syphilis. Al Capone contracted syphilis as a young man. 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;

See also: List of people speculated to have been syphilitic

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 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;

Stages of syphilis

Different manifestations occur at each stage of the disease:-

Primary syphilis

Primary syphilis is manifested after an incubation period of 10-90 days (the average is 21 days) with a primary sore.

Secondary syphilis

Secondary syphilis is characterized by a skin rash that appears 1-6 months (commonly 6 to 8 weeks) after the primary infection. A patient with syphilis is most contagious when he or she has secondary syphilis.

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. 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.

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.

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.

Neurosyphilis

Neurosyphilis refers to a site of infection involving the neurologic system. The precise extent and significance of neurologic involvement in HIV-infected patients with syphilis, reflected by either laboratory or clinical criteria, remain incompletely characterized.

Asymptomatic Neurosyphilis Meningovascular Syphilis Tabes Dorsalis General Paresis

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; 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.

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.

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.

University of Phoenix

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.

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.

Treatment

History

There were originally no effective treatments for syphilis.

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. 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.

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. 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).

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; 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.

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) 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.

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.

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. 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.

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. this is a reference to the brittleness of bones engendered by the use of mercury which was then widely used to treat syphilis. (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.

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:

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

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 character of Darla in Buffy the Vampire Slayer and Angel is a colonial-era prostitute dying of syphilis when she is sired by the Master.

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