Introduction

Syphilis, "The Great Imitator," with its myriad manifestations, is a disease with a rich background, colorful history, and never-ending speculation. The very origin remains controversial. The Columbian theory proposes that syphilis is a disease of the New World that was carried back to the European continent by Columbus and his sailors. Chronologically, this is supported by an outbreak of the disease in Naples in 1494 with documentary evidence linking Columbus's crew to the event. Additionally, syphilitic lesions have been noted in discovered remains of precontact Native Americans. The alternative suggests that the disease was present, at least in some form, pre-Columbus and spread through Europe with developing urbanization. Proponents refer to sporadic documentation of syphilitic symptoms throughout Europe prior to Columbus' s era and note descriptions of similar symptoms described by Hippocrates and possibly dating back to biblical passages. Furthermore, it has been suggested that systemic syphilis evolved from a microbiological cousin, Treponema pertenue, a subspecies of Treponema pallidum. This organism certainly existed in the early European era and was the source of yaws, a localized soft-tissue infection primarily afflicting children. It has also been proposed that both theories have some truth to them, and in fact, the Old World and New World strains of T. pallidum merged, resulting in the entity later known as syphilis.

Regardless of the origin, the first major outbreak of what is believed to be modern-day syphilis spread through the late fifteenth and into the sixteenth century. This was dubbed the Great Pox and produced significant morbidity and mortality with the spread of armies and population throughout Europe. It was first named morbus gallicus, or the "French disease," due to an outbreak in the French army; however, finger-pointing was prevalent, with Italians and Dutch also calling it the "Spanish disease," Germans and Russians calling it the "Polish disease," French naming it the "English disease" and "Italian disease," and the Arab population referring to the "Disease of the Christians."

While the lack of microbiology in that era precludes the confirmation of T. pallidum as the source of the outbreak, the disease process, including its sexual transmission, was well documented in the sixteenth century. In 1530, it received its current moniker, syphilis, at the hand of the Italian physician and scientist Girolamo Fracastoro. In his poem entitled "Syphilis, sive Morbus Gallicus," or "Syphilis, or the French Disease," he describes a shepherd by the name of Syphilis who is afflicted with the disease after disobeying the commands of Apollo.

From its original outbreak, which has been described as having an impact similar to the twentieth-century development of AIDS, syphilis continued with epidemic waves. However, while the original affliction was much more virulent, with rapid progression and more severe symptoms, further epidemics resembled the more indolent disease process with which we are now familiar. These were particularly pronounced during the Napoleonic wars, nineteenth-century industrialization, and World Wars I and II. Over the years, many notable figures are alleged or speculated to have been afflicted with syphilis, including Franz Schubert, Friedrich Nietzsche, Paul Gauguin, Vincent van Gogh, Oscar Wilde, Joseph Stalin, James Joyce, Al Capone, and Howard Hughes. While not often considered a disease of the modern era, it remained the leading cause of neurological and cardiovascular disease of middle-aged individuals as late as the early 1900s (1). At the turn of the twentieth century, syphilis picked up the nickname "lues" from the Latin phrase "lues venereum," which translates as "disease" or "pestilence." The term was applied generally to sexually transmitted diseases but then was used primarily as a substitute for syphilis, although the phrase is found infrequently in modern literature.

During the twentieth century, our understanding of syphilis rapidly progressed with a few key events. Fritz Schaudinn and Erich Hoffmann identified the spirochete T. pallidum from serum in 1905. One year later, August von Wassermann developed a complement fixation test to detect antibodies against the bacteria, allowing identification of millions of previously undiagnosed individuals. Paul Ehrlich then began his work with arsenic derivatives and in 1910 patented arsphenamine, which was found to be a significant improvement in treatment, though plagued by recurrences. The discovery of penicillin by Alexander Fleming in 1928 led to John Mahoney's breakthrough demonstration of this antibiotic's utility in treating syphilis in 1943. Penicillin was widely available by the post-World-War-II era, and in combination with public health measures, it facilitated the near-eradication of this historical plague. However, this effective treatment has left most current health-care providers relatively unfamiliar with the disease, with many never having seen a case of syphilis. Recent epidemiological data demonstrate an increasing incidence, particularly among the population of men who have sex with men. As such, a review of syphilis including its head and neck manifestations is of timely benefit.

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