Articles

Derrick Garwood, Freelance Medical Writer and Editor

The profound impact of syphilis on the human psyche is indicated by the fact that the disease has not just one, but two, patron saints – St. Fiacre and St. George. Perhaps Pope Alexander VI's reputed death from it is not entirely unrelated! The excellent RealAudio slide show identifies a number of 'great syphilitics of history' including Henry VIII, Ivan the Terrible, Baudelaire, Donizetti, Cortes, Friedrich Nietzche and Toulouse Lautrec, and reviews evidence for the theory that Christopher Columbus brought the condition back from the New World in 1493.

Certainly the first unambiguous descriptions of syphilis begin around 1500 AD, by which time it had reached epidemic proportions in Europe. Each nation tended to blame the scourge on a neighbour currently out of favour; it was variously known as the Disease of Naples, French Pox, Turkish Disease, Italian Pox and Spanish Disease. Some researchers argue that there is evidence of pre-Columbian syphilis in several European countries. Others dispute this, and contend that the existence of New World skeletons at least 800 years old bearing signs of syphilis convincingly supports the Columbus theory.

Only during the first half of the 20th century were significant advances made in treatment , with the introduction of malaria therapy, heat therapy and finally penicillin. It says a lot for the dread inspired by this disease that patients at the Mayo Clinic were willing to submit themselves to repeated bouts of malaria (with a mortality rate of 5%) for the chance of a cure. Two major ancillary benefits derived from the evolution of syphilis treatment during this period: the development of routine intravenous techniques and the establishment of the first large co-operative clinical trials.

The causative organism is the spirochaete Treponema pallidum subsp pallidum, a helically-coiled, corkscrew-shaped bacterium, 6 to 15 µm long, which has not yet been cultured in vitro. A highly invasive pathogen, it rapidly disseminates after inoculation. Infection is by sexual contact, requires fewer than 10 organisms, and occurs world-wide. Since the late 1950s the incidence has been rising, particularly in undeveloped countries, although a major increase in the West during the late 1980s has been attributed to the spread of crack cocaine usage among inner city minorities. This site has extensive coverage of the structure and biology of the organism, as well as the epidemiology and pathogenesis of the disease. For additional light microscope pictures of the spirochaete go here, and for genetic data try here.

The Merck Manual has a good description of the usual clinical course. After an incubation period, usually of 3 – 4 weeks, the primary lesion or chancre appears at the site of infection. It begins as a red papule which soon erodes to give a painless ulcer with an indurated base, accompanied by painless enlargement of the regional lymph nodes. Photographs of typical lesions at can be found here and here. The chancre, if left untreated, heals in about 4 – 8 weeks.

The secondary stage can affect many tissues of the body, including the eyes, bones, joints, meninges, kidneys, liver and spleen. Over 80% of patients have mucocutaneous lesions and 50% have generalised lymphadenopathy. Typically, cutaneous rashes appear some 6 -12 weeks after infection and may persist for months, but eventually heal without scarring. The other classical lesions of secondary syphilis are snail track ulcers of the oral mucosa (see photograph) and condylomata lata: hypertrophic, dull pink or grey papules at mucocutaneous junctions and in moist areas of the skin (see photographs).

There then follows a period of latency which may last as long as three decades, although during the first few years secondary symptoms may reappear. Patients with late latent syphilis appear normal and are non-infectious. Approximately one third develop tertiary syphilis, which may be clinically described as benign tertiary syphilis, cardiovascular syphilis, or neurosyphilis.

The typical lesion of benign tertiary syphilis is the gumma: an inflammatory mass that can affect virtually any organ, is frequently localised and evolves to give necrosis and fibrosis. Patients with cardiovascular syphilis may exhibit an aortic aneurysm, narrowing of the coronary ostia or aortic valvular insufficiency, while neurosyphilis produces various clinical syndromes in a small percentage of patients.

The disease may be passed from mother to baby by transplacental passage of T. pallidum during pregnancy, or by contact with an infected lesion during birth. The risk of infection depends upon the stage of the disease in the mother. Signs and symptoms of congenital syphilis are arbitrarily divided into early manifestations occurring during the first two years of life, and late manifestations. The former include bony lesions in the femur and humerus, hepatosplenomegaly, rhinitis, a maculopapular rash and fissures around the lips, nares and anus. Late manifestations result from scarring caused by the early systemic disease: gummas, interstitial keratitis and characteristic notching of the upper anterior teeth (Hutchinson's incisors). If you want to read about congenital syphilis in truly encyclopaedic detail, try Neonatology on the Web.

The standard treatment for primary, secondary and early latent syphilis is a single intramuscular injection of penicillin. Alternative antibiotics such as tetracycline, doxycycline, minocycline, erythromycin or ceftriaxone are administered to those with a hypersensitivity to penicillin. Around 50% of patients experience a worsening of symptoms within hours of starting treatment (the Jarisch-Herxheimer reaction), as a result of the release of endotoxin by dying bacteria. Symptoms usually include mild fever, malaise and headache lasting a few hours.

The treatment of late latent and tertiary syphilis requires a different approach, because there are questions about the efficacy of penicillin in neurosyphilis, which may be present but asymptomatic. Repeated doses are given but failures do occur with the recommended regimes, so long term follow-up is particularly important. After scrutinising this site, which describes in detail the difficulties of eliminating treponemes, it is impossible not to recall the words of a pathology lecturer more than twenty years ago, "There's one way you can be certain you have been cured of syphilis – you can catch it again!"

The details presented here were accurate at the time of publication, but remember that information on the Web has a tendency to change without notice!

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