Dr. Johnston of Johns Hopkins University School of Medicine and Chief Medical Officer at Kennedy Krieger Institute has no relevant financial relationships to disclose.)
This article includes discussion of congenital syphilis, congenital lues, French malady, lues, mal de la Hispaniola, and mal napolitano. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.
Congenital syphilis, one of the transplacentally acquired preventable infections, can produce long-lasting morbidities in neonates. In this article, the authors describe the epidemiology of congenital syphilis in the United States and in developing countries as well as the clinical spectrum of the disease. Also discussed are the issues pertaining to prevention and diagnosis of congenital syphilis in the United States, as well as globally, and the treatment of presumed and proven congenital syphilis.
Historical note and terminology
Syphilis has been recognized as an infection since the beginning of the 16th century, when Ferdinand, the son of Christopher Columbus, described its skin manifestations among the sailors returning from the first voyage to the New World in 1493, and it was termed mal de la Hispaniola. The disease spread to the Spanish soldiers who fought at the side of the King of Naples and was then retermed morbo Napolitano, and the disease finally reached the French Troops of Charles VIII during their 3-week siege of the city of Naples in 1495. When this army returned to the north, the Italian Peninsula was invaded by syphilis, and the disease became known as morbo Gallico or "French malady."
Girolamo Fracastoro of Verona became famous for his poem “Syphilis sive Morbus Gallicus” in 1530, in which the main character, the shepherd Syphilus, is afflicted by this disease as punishment for cursing the Gods (Public Health Service 1968); this poem not only baptized the French malady with its present name, but also hinted on its venereal origin. From Spain the disease spread throughout Europe, and there exists exact documentation of its appearance in Asia, India (1498), China (1505), and Japan (1569) (Petrucelli 1978).
Congenital syphilis was first described in an English 17th century pediatric textbook, although Paracelsus, who lived from 1493 until 1541, first suggested in utero transmission (Public Health Service 1968). As a result of this knowledge, newborns with syphilis were abandoned by their mothers because of fear of contagion. Sir Jonathan Hutchinson described the triad of notched incisors, interstitial keratitis, and eighth cranial nerve deafness as a criterion for diagnosis of congenital syphilis (Ioannou et al 2016).
In 1905, Fritz Schaudin described the causative role of Treponema pallidum, and shortly afterwards in 1910 Paul Erhlich introduced a synthetic arsenical, salvarsan, as the main line of therapy. Although toxic, it had more efficacy than previous medications such as mercury salts and guaiac. The natural history of untreated syphilis is well-known because of an epidemiologic study initiated in Oslo, Norway by Boeck in 1891. This study documented the course of the disease in 1978 patients and included autopsies of many of them.
The “Tuskegee Study of Untreated Syphilis in the Negro Male” was started in 1932 by the United States Public Health Service to study the natural history of syphilis in hopes of justifying treatment programs for blacks (Centers for Disease Control and Prevention 2012). It has become an illustrative case of human research carried out without the benefit of the patients' informed consent.
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