Clinical Manifestations and Pathology

When William Osier asserted that "who knows syphilis, knows medicine," he was doing no more than stressing the extreme clinical variety of this disease, which is capable of affecting any system of the human body. As noted previously, the natural course of venereal syphilis includes three consecutive clinical stages, each stage separated by a latent period with no visible signs of infection (Perine et al. 1984; Csonka 1987; Holmes and Lukehart 1987).

Primary Syphilis

T. pallidum penetrates intact mucous membranes and abraded skin. After an incubation period ranging from 2 to 6 weeks (average 3 weeks), the primary lesion — the chancre - appears at the site of entry, it is a single, small, and painless ulcer with undurated edges, usually appearing in the genitalia (penis, vulva, labia, cervix) and, less frequently, in other regions such as the anus, mouth, buttocks, and fingers. Chancres of the penis and vulva are usually accompanied by moderate bilateral enlargement of inguinal lymph nodes. The chancre heals spontaneously over a period of 2 to 6 weeks.

Secondary Syphilis

In most patients, after a brief latent period (6 to 8 weeks), there is a secondary clinical stage characterized by the appearance of disseminated lesions on the skin and in the internal organs. In women, these lesions are often the first overt clinical sign of syphilis. Secondary lesions consist of a symmetrical, evolutive, and painless rash, very variable in appearance and localization, and usually accompanied by fever, malaise, aches in the bones (often worse at night), and generalized enlargement of lymph nodes. After a few weeks - generally 2 to 6 - secondary lesions and symptoms spontaneously disappear. In 25 percent of untreated patients, however, there is a recurrence of secondary lesions during the first 2 years of infection.

Tertiary or Late Syphilis

The tertiary stage develops only in about one-third of untreated cases, and only after another latent period lasting from 1 to 20 years, or even longer. This stage is characterized by progressively destructive lesions of the skin and mucous membranes, bones, and internal organs. The most typical lesion is the gumma, a small rubbery tumor that is a benign manifestation of tertiary syphilis, which can develop in any part of the body.

Particularly serious forms of late syphilis involve the cardiovascular and central nervous systems. Cardiovascular syphilis may cause aneurism of the thoracic aorta, and dilatation of the aortic valve. Neurosyphilis includes a loss of positional sense and sensation (tabes dorsalis, locomotor ataxia) or a form of insanity (general paresis [GPI], dementia paralytica).

As a result of the introduction of antibiotic therapy, tertiary syphilis has almost disappeared. Thus the most reliable information available on it today has been provided by two major studies on the course of untreated syphilis, the Oslo Study (1891-1951) and the Tuskegee Study (1932-72). The former surveyed retrospectively a group of nearly 2,000 patients with primary and secondary syphilis diagnosed clinically before immunologic tests came into use. The latter studied prospectively 431 black men with seropositive latent syphilis of 3 or more years' duration, who were deliberately kept untreated. Because of the ethical issues raised by this racist experiment, the Tuskegee Study has been crucial in formulating the present guidelines concerning medical experimentation on humans (Jones 1981; Holmes and Lukehart 1987).

Congenital Syphilis

The risk of congenital syphilis to the fetus is high during the first 2 years after the mother has acquired the infection. An infected fetus may die during pregnancy, be stillborn, or be born prematurely. Secondary-type lesions are present at birth or appear within the first 6 months of life (Perine et al. 1984; Csonka 1987; Holmes and Lukehart 1987).

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