Syphilis is a sexually transmitted disease resulting from infection by the spirochete Treponema pallidum. It is the oldest documented venereal disease, dating back to the 1400s. A more detailed discussion of syphilis appears in Chapter 15.

Epidemiology. The United States has seen cyclic rises in the incidence of syphilis since nearly eliminating the disease in the late 1950s. Since 1990, the rate of primary and secondary syphilis has decreased nearly 90% to 2.5 cases per 100,000 people. Of the cases, 80% are reported to the Centers for Disease Control (CDC) and there appears to be a regional predilection for the Southeast (9). The rate of congenitally acquired syphilis has also undergone a decline similar to primary/secondary infection since the early 1990s where it occurs in approximately 14 per 100,000 births nationally (10).

Pathogenesis. The infection by the spirochete occurs through contact of the lesion with traumatized or abraded mucous membrane or epidermis. This contact is most commonly associated with sexual transmission, with the exception of congenital syphilis. The risk of infection following sexual contact with a person with the disease is nearly 50%.

Clinical Manifestations. The clinical course of syphilis is separated into stages: primary, secondary, latent, and tertiary. Each stage has a characteristic set of clinical entities associated with infection, and not all patients go on to develop tertiary syphilis.

The head and neck, as well as systemic manifestations of syphilis are myriad. With reference to the topic of discussion, regional (cervical) lymphadenopathy generally occurs early in infection, during the primary stage of syphilis. This stage is characterized by the chancre, an ulcerated painless lesion noted at the site of inoculation. In the instance of oral inoculation, the chancre will appear within the oral mucosa accompanied by firm, nontender lymphadenopathy in the upper cervical or submandibular nodal basins. With spontaneous resolution of the chancre, lymphadenopathy clears and generally does not recur unless associated with the benign gummata of tertiary syphilis.

Other head and neck manifestations of infection include sensorineural hearing loss in otosyphilis; chancres of the oral cavity in primary syphilis and pharynx in secondary syphilis; laryngeal chancres; mucous patches or gummata encompassing the full spectrum of stages associated with syphilitic infection; and numerous effects of congenital syphilis such as palatal defects, saddle nose deformities, and congenital deafness.

Systemic manifestations may involve multiple organs and are predicated on the clinical stage. Serious and disabling systemic involvement is usually associated with tertiary syphilis. The stage is characterized by organ system symptom complexes divided into neurosyphilis, cardiosyphilis, and benign gummatous syphilis. Neurosyphilis is characterized by meningeal signs, possibly with cranial nerve involvement; meningovascular syphilis and associated CNS ischemia and stroke; parenchymatous neurosyphilis associated with the classic sensory ataxia; and autonomic dysfunction of tabes dorsalis (11,12). Cardiosyphilis occurs as a consequence of spirochete invasion of the coronary vessels and aorta, leading to endarteritis obliterans. Involvement of the aortic root produces scarring and necrosis of the aortic wall media, resulting in aneurysm formation. Benign gummatous syphilis is characterized by destructive lesions (gummata) that affect soft tissue and bone throughout the body. These lesions represent an intense inflammatory response to the spirochete.

Diagnosis. Studies established for diagnosis are separated into four categories, based in part upon their utility and distinct stages of the disease or with specific clinical manifestations. Dark field examination is used when spirochetes are plentiful and may be obtained from lesions. These include chancres, mucous patches, or fluid from FNA of enlarged lymph nodes. The use of fluorescent antibodies amplifies the utility of dark field examination. Serologic tests for the spirochete include venereal disease research laboratory test (VDRL), rapid plasma reagent test (RPR), unheated serum reagent test (USR), and toluidine red unheated serum test (TRUST), all of which are nontreponemal studies. These tests measure antibody response to treponemal particles and are somewhat nonspecific because other illnesses may cause release of similar antigens. Despite this, these tests may be used to follow treatment response. These tests are not positive following eradication of the disease.

Treponemal tests use the spirochete itself as the antigen and are used to complement the nontreponemal studies in establishing the diagnosis. These include the fluorescent treponemal antibody absorption test (FTA-ABS) and the microhemagglutinin-treponema pallidum test (MHA-TP). Both of these tests remain positive even following disease elimination; therefore, they cannot be used to measure response to treatment.

Treatment. Parenteral penicillin G is the treatment of choice for syphilis. Dosing schedule and duration of administration vary with the stage and the severity of clinical disease.

Complications and Prognosis. Untreated syphilis may be associated with significant lifelong disability. Progression of disease into a latent phase or tertiary syphilis occurs in approximately one-third of patients in each category. Untreated tertiary syphilis is associated with serious neurologic and/or cardiovascular complications. Appropriate and timely treatment is successful in the majority of patients diagnosed.

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