Excerpt from Endemic Syphilis

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Background

Features of endemic syphilis have been noted in ancient Africa since history was first recorded. Originally, endemic syphilis was thought to be spread throughout a large geographic area. Through the passage of time, this disease has affixed itself to regions of dry, arid climates.

Endemic syphilis is also known as sibbens (Scotland), radseyege (Scandinavia), siti (Gambia), therlijevo (Croatia), njovera (Southern Rhodesia), frenjak (Balkans), and nonvenereal endemic syphilis (Bejel).

Pathophysiology

Different species of the spirochete Treponema cause diverse infections in humans. Treponema pallidum causes venereal syphilis. Treponema carateum and Treponema pertenue cause pinta and yaws, respectively. Endemic syphilis is caused by a spirochete closely related to T pallidum, which is T pallidum subsp endemicum.

Endemic syphilis is transmitted through direct or indirect skin-to-skin or mouth-to-mouth contact of the infected lesion. It occurs predominantly in children aged 2-15 years. Because children are the active transmitters of the disease, infection of all members of a household is very common. The common housefly, Musca domestica, has not been established as a potential vector.

Endemic syphilis has similar pathology and histology as venereal syphilis. However, the effects on the organ systems are different.

The disease has 2 stages, an early stage and a secondary stage. The early stage consists of primary and secondary lesions very similar to those of venereal syphilis. The secondary stage consists of late latent disease and tertiary lesions. Each stage affects different tissues and organs. The primary lesions usually manifest in the oropharynx. Secondary stage lesions can appear as mucous patches on the lips, the palate, and the larynx. Angular stomatitis, condylomata, oral ulcers, and generalized adenopathy can also be seen in the secondary stage. Tertiary and late-stage disease usually develops 6 months to years after inoculation and may manifest as gummas of the skin, the bones, or the cartilage. Neurologic involvement and cardiac involvement are rare.

Frequency

United States

Rare cases of endemic syphilis have been reported in the United States. When reported, the cases are typically seen in immigrants and people coming from endemic areas. Owing to its mode of transmission, endemic syphilis is easily transmitted to new areas. Hygiene; living conditions; and environmental factors, such as the weather, make the disease fastidiously endemic in the United States.

International

Endemic syphilis is extremely common in areas of dry, hot climates. It is also widely spread in rural areas of poor hygiene and education.

Parts of Africa (eg, Sahel countries [Sudan, Southern Rhodesia, South Africa]), parts of the Middle East (eg, Nomadic/Bedouin tribes of Saudi Arabia, Iraq, and Syria), and parts of Asia (eg, Turkey, Southeast Asia, the Western Pacific) are affected. In these areas, seropositivity in children reaches as high as 40%, and early lesions reportedly affect 2-20% of children.

Mortality/Morbidity

Because the disease rarely manifests clinically significant cardiovascular and neurologic symptoms, mortality is uncommon unless the disease state is highly exaggerated, through either a large inoculum or a devastating immune reaction.

Race

Endemic syphilis can affect anyone. Because it is endemic in certain areas of the world, the disease mostly affects ethnicities of those geographic regions.

Sex

Both sexes are equally affected, especially in the pediatric population. This varies with the geographic region. However, in adults, women are slightly more susceptible, probably because they are the primary interactants with children, either as a caregiver or during breastfeeding.

Age

Children aged 2-15 years are most commonly affected; 25% of cases occur before age 6 years, and 55% of cases occur before age 16 years. The remaining 20% of cases occur in adults who are in close contact with children who are infected.

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