eMedicine Specialties > Dermatology > Bacterial Infections

Endemic Syphilis

Author: Hassan I Galadari, MD, MBBS, Assistant Professor of Dermatology, Faculty of Medicine and Health Sciences, United Arab Emirates University
Coauthor(s): Ibrahim Galadari, MD, MB, BCh, MSc, Professor of Dermatology, Department of Internal Medicine, Faculty of Medicine and Health Sciences, United Arab Emirates University
Contributor Information and Disclosures

Updated: Feb 13, 2009

Introduction

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).

Also see the related eMedicine articles Treponematosis (Endemic Syphilis), Neurosyphilis, and Ocular Manifestations of Syphilis.

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.¹

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

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.²

Clinical

History

Unlike venereal syphilis, endemic syphilis rarely involves the nervous and cardiovascular systems. The clinical manifestation of neurosyphilis is minor and not significant. Congenital syphilis is rarely encountered because the disease can be treated during pregnancy.

Physical

• Primary stage
  • The incubation period is 10-90 days.
  • Skin lesions resemble the chancres of venereal syphilis. A small, eroded or ulcerated papule is usually asymptomatic.
  • Observing a lesion on the nipple of a mother with a suckling child who is infected is not uncommon.
  • Primary lesions heal in 1-6 weeks and often go undiagnosed.
  • Generalized lymphadenopathy is uncommon since the inoculum is small.
• Secondary stage
  • This stage usually consists of macerated, eroded patches on the lips, the tongue, and the tonsils. Hypertrophic condyloma lata can appear in the anogenital area.
  • Nontender, generalized lymphadenopathy is common.
  • Painful osteoperiostitis in the long bones (eg, tibia) can occur.
  • This stage can persist for 6-9 months.
  • Angular stomatitis resembling that caused by vitamin B deficiency can be seen.
• Tertiary and late stages
  • Destruction of the bone and the cartilage in the formation of gummatous lesions (commonly in the nose) may occur. The gummas can ulcerate and develop chronic serpiginous tracts. Healing results in depigmented scars with a hyperpigmented border.
  • Saddle nose deformity and palate perforation can occur.
  • Rare atypical involvement of the cardiovascular and nervous systems can occur.

Causes

• T pallidum subsp endemicum, which is transmitted nonvenereally, is the pathogenic organism causing the disease.
• Endemic syphilis is a disease common in areas of poor economic status, education, and personal hygiene. Transmission occurs when skin or mucous membranes come in contact with infected skin lesions. Wearing gloves at all times is imperative for the physician who is examining the lesion.

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