Syphilis
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Syphilis (historically called lues) is a sexually transmitted disease (STD) that is caused by a spirochaete bacterium, Treponema pallidum. Syphilis has many alternate names, such as: Miss Siff, the Pox, and has been given many national attributions, e.g. the French disease or the English disease.
The route of transmission for 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.
In the United States, about 36,000 cases of syphilis are reported each year, and the actual number is presumed to be higher. About three-fifths of the reported cases occur in men.
Syphilis can be treated with penicillin or other antibiotics. Statistically, treatment with a course of pills 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 procaine and penicillin into each buttock (the procaine is added to make the pain bearable); the dose must be given half in each buttock because the amount given would be painful if given in a single injection. An alternative treatment is to administer several capsules of azithromycin orally (which has a long duration of action) under observation. This latter course, however, may be falling on hard times, as strains of syphilis resistant to azithromycin have developed and may account for 10% of cases in some areas in 2004. 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 ultimately fatal.
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History
There are two schools of thought on the origin of Syphilis- the Colombian and pre-Colombian thesis. There is ongoing debate in anthropological and historical fields about the validity of either theory.
The pre-Colombian 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 Colombian theory.
The Colombian theory holds that syphilis was a new world disease brought back by Columbus. Although this evidence has been derided as "weak and circumstantial" the first well recorded outbreak of what we know as syphilis occurred in Naples in 1494. There is some documentary evidence to link Columbus' crew to the outbreak. Supporters of the Colombian theory find syphilis lesions on pre-contact Native americans. Again, all the anthropological evidence is heatedly discussed on both sides of the Colombian/pre-Columbian debate. (Baker, et al.)
Alfred Crosby has argued that neither side has the full story. 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. The disease swept across Europe from the early epicenter at Naples. The early form was much more virulent than the disease of today, the incubation period was shorter, only a few months, and the symptoms were more severe. In addition, the disease was more frequently fatal than it is today. By 1546, the disease had evolved into the form we know now.
Syphilis had many different names. Great pox was used during the 16th century to distinguish it from smallpox. Great pox produced a similar rash in its early stages to small pox, but other than that it has no relation to the Variola virus. However the name is misleading, as small pox 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 it is noteworthy that the Italians also called it the "Spanish disease", the French called it the la maladie anglaise - the English disease and "Italian" or "Neapolitan disease", the Russians called it the "Polish disease", and the Arabs called it the "Disease of the Christians". The name "syphilis" was first applied by Girolamo Fracastoro in 1530 from the name of a shepherd in a poem by Leonardo da Vinci.
A number of famous historical personages, including Charles VIII, 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. Syphilis has been cited as the cause for various medical symptoms afflicting Adolf Hitler in his later years.
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.
See also: List of notable people identified as probably syphilitic
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. 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.
Secondary syphilis
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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, anorexia, 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.
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 (joint deformity), 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, a disorder of the spinal cord, often results in a characteristic shuffling gait.
Cardiovascular complications include aortic 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).
Congenital syphilis
Congenital syphilis is syphilis present in utero and at birth, and occurs when a child is born to a mother with secondary or tertiary syphilis. According to the CDC, 40% of births to syphilitic mothers are stillborn, 40-70% of the survivors will be infected, and 12% of these will subsequently die. Manifestations of congenital syphilis include abnormal x-rays; Hutchinson's teeth (centrally notched, widely-spaced peg-shaped upper central incisors); mulberry molars (sixth year molars with multiple poorly developed cusps); frontal bossing; saddle nose; poorly developed maxillae; enlarged liver; enlarged spleen; petechiae; other skin rash; anemia; lymph node enlargement; jaundice; pseudoparalysis; and snuffles, the name given to rhinitis in this situation. Rhagades, linear scars at the angles of the mouth and nose result from bacterial infection of skin lesions. Death from congenital syphilis is usually through pulmonary hemorrhage.
Testing and treatment
Originally, there were no effective treatments for syphilis. The commonest 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".
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 the 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.
Subsequent syphilis tests, such as the Rapid Plasma Reagin (RPR) and Venereal Disease Research Laboratory (VDRL) test, and others based on monoclonal antibodies and immunofluorescence, are used in place of the Wassermann and Hinton tests today.
As the disease became better understood, effective treatments began to be found, beginning with the use of the arsenic-containing drug Salvarsan from 1910, and later, Neosalvarsan. One treatment that was tried was the use of malaria; the intense fever produced by a malarial attack raising the body temperature sufficiently to kill off the spirochaetes. Though this did leave the patient with a malaria infection, it was considered to be preferable to the long term effects of syphilis.
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.
In one of the more shameful episodes of the twentieth century, the Tuskegee syphilis study continued to study the lifetime course of syphilis in a group of black Americans, long after effective treatments for syphilis were available.
In the July 17, 1998 issue of the journal Science, a group of biologists reported the sequencing of the genome of T. pallidum.
To this day, the primary treatment for syphilis remains penicillin. Treatment typically consists of benzathine penicillin G or aqueous procaine penicillin G for several days to weeks. Individuals who have allergic reactions to penicillin (i.e., anaphylaxis) can be effectively treated with oral tetracyclines.
Suffering from syphilis
A detailed although somewhat crude description of how one might have suffered from syphilis before modern antibiotics is provided in a limerick of unknown origin [1] (http://www.ucolick.org/~randi/aecom/syphilis.txt). It begins with a man having a chancre; he goes on to develop secondary syphilis, losing his hair; following which he has all the common complications of tertiary syphilis and finally ends up insane due to neurosyphilis. The limerick also mentions his wife catching it from him and passing it on to his children.
Syphilis in art and literature
In 1530, Girolamo Fracastoro, a physician and poet, wrote a poem from which syphilis derived its name.
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, whose name, suggesting light and truth, is meant to indicate that he is to be taken seriously. 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 Charlotte Brontë's novel Jane Eyre, the character Edward Rochester's first wife, Bertha, is characterised as suffering from the advanced stages of syphilitic infection, general paresis of the insane, and there is plenty of 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. 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: [1] (http://www.ucolick.org/~randi/aecom/syphilis.txt)
Related topics
- a related spirochete bacterium, Treponema pallidum, sp. pertenue, also called Treponema pertenue, is the cause of yaws, a tropical infection of the skin, bones and joints.
- an additional treponematous disease is called pinta (Treponema carateum).
- Chancre
- Penicillin
- Sexually transmitted disease (STD)
References
- From mercury to malaria to penicillin: The history of the treatment of syphilis at the Mayo Clinic, 1916–1955
- Baker, Brenda: Armelagos, George. The Origin and Antiquity of Syphilis: Paleopathological Diagnoses and Interpretation. Current Anthropology 29 (1998).
- Alfred Crosby. The Early History of Syphilis: A Reappraisal. American Anthropologist, Vol 71 (1969).
- McNeill, William H. "Plagues and People." Bantam Doubleday Dell Publishing Group, Inc., New York, NY, 1976, ISBN 0-385-12122-9.
- Sacks, Oliver W. "The Man Who Mistook His Wife for a Hat" (ISBN 0684853949) — contains a chapter on an elderly woman treated in the early stages of tertiary syphilis from an infection decades earlier.
External links
- "A New Gold Standard For Syphilis?" Poster Presentation for European Academy of Dermatology and Venereology 2004 Spring Symposium (http://colman.net/eadv/index.html)
- Syphilis and AIDS: Lessons from history (http://www.cbc.ca/ideas/Aids)
- "Syphilis fact sheet" from the Center for Disease Control (http://www.cdc.gov/std/Syphilis/STDFact-Syphilis.htm)
- Kipkeepers, Pox and Gleet Vendors: A Rapid History of Syphilis (http://www.medinfo.ufl.edu/other/histmed/clancy/index.html)
- The treatment of dementia paralytica by malaria inoculation (A Nobel Prize lecture, December 13, 1927) (http://www.nobel.se/medicine/laureates/1927/wagner-jauregg-lecture.html)
- Secrets of the Dead (PBS): The Syphilis Enigma (http://www.pbs.org/wnet/secrets/case_syphilis/index.html)
- The chapter on syphilis from a book on Dermatology and Venerology (http://www.hkmj.org.hk/skin/syphilis.htm) — contains some good pictures
- POX: Genius, Madness, and the Mysteries of Syphilis (http://www.poxhistory.com/)
- Article on syphilis, cites a "one in four" chance of getting tertiary syphilis (http://www.bcpwa.org/issue6/syph.htm) (references outdated now believed incorrect medical data)
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