AUTHOR AND EDITOR INFORMATION

Section 1 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

INTRODUCTION

Section 2 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

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.

CLINICAL

Section 3 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

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.

DIFFERENTIALS

Section 4 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

Other Problems to be Considered

Because of the overlap between treponematoses, endemic syphilis has to be correctly differentiated from venereal syphilis, yaws, and pinta. The differential diagnoses of the diseases are very similar, and some features, such as nasopharyngeal lesions, can be difficult to differentiate.

During the early stages, features in endemic syphilis resemble a number of dermatoses, mainly eczema, mycoses, psoriasis, leprosy, herpes simplex, perlèche, and condylomata acuminata.

In the late stages, the features overlap that of malignant tumors, including carcinoma, mycosis fungoides, lupus vulgaris, and lupus erythematosus.

The mutilating nasopharyngeal lesions can be mistaken for tertiary venereal syphilis, tuberculosis, leprosy, rhinoscleroma, and mucocutaneous leishmaniasis.

WORKUP

Section 5 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

Lab Studies

• The best and most reliable method to diagnose endemic syphilis is a thorough clinical history and a complete physical examination. Geographic data, including travel information, is invaluable in establishing the diagnosis.
• • Laboratory tests confirm the clinical based diagnosis. These laboratory tests are the same as those used for the diagnosis of venereal syphilis.
  • Serologic, morphologic, and biochemical tests are not useful in distinguishing between the types of treponemal infections.
• Serologic tests
• • Results of treponemal tests, such as the fluorescence treponemal antibody absorption (FTA-ABS) test, are positive in all stages of the disease.
  • Nontreponemal tests, such as the Venereal Disease Research Laboratory (VDRL) test and the rapid plasma reagent (RPR) test, are reactive 2-3 weeks after the onset of the primary lesion. These tests have a sensitivity of 80% in patients with a 2- to 3-month history of symptomatic primary syphilis. In the secondary stage, these tests have a sensitivity of near 100%.
• Dark-field microscopy
• • This is the best method to confirm a treponemal disease. Although it does not specify the correct species because of similarities in morphology, the test promptly confirms that the infection is caused by a treponeme.
  • Serum is obtained by squeezing the base of the lesion.

TREATMENT

Section 6 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

Medical Care

• Endemic syphilis responds well to penicillin and other treponemicidal drugs. Patients become noninfectious within 24 hours.
• After successful treatment, the titer of nontreponemal tests shows a gradual decline and eventually becomes negative.
• No vaccines for the disease are available.

MEDICATION

Section 7 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

The goals of pharmacotherapy are to eradicate the infection, to reduce morbidity, and to prevent complications.

Drug Category: Antibiotics

These agents have the capability to achieve a 100% cure rate. Empiric antimicrobial therapy must be comprehensive and should cover all likely pathogens in the context of the clinical setting.

FOLLOW-UP

Section 8 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

Prognosis

• Mortality is uncommon (see Mortality/Morbidity).

Patient Education

• Early detection and treatment campaigns aimed at endemic syphilis by health officials are extremely important. Improvement of social and medical conditions and continuing health education help halt the spread and facilitate in the eradication of the disease.
• For excellent patient education resources, visit eMedicine's Sexually Transmitted Diseases Center. Also, see eMedicine's patient education article Syphilis.

REFERENCES

Section 9 of 9

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

• Antal GM, Lukehart SA, Meheus AZ. The endemic treponematoses. Microbes Infect. Jan 2002;4(1):83-94. [Medline].
• Blount JH, Holmes KK. Epidemiology of syphilis and the non-venereal treponematoses. In: Johnson RC, ed. The Biology of Parasitic Spirochetes. New York: Academic;157-76.
• Cutler JC. Endemic syphilis, yaws, and pinta. In: Johnson RC, ed. The Biology of Parasitic Spirochetes. New York: Academic;365-73.
• Engelkens HJ, Niemel PL, van der Sluis JJ, et al. Endemic treponematoses. Part II. Pinta and endemic syphilis. Int J Dermatol. Apr 1991;30(4):231-8. [Medline].
• Falabella R. Nonvenereal treponematoses: yaws, endemic syphilis, and pinta. J Am Acad Dermatol. Dec 1994;31(6):1075. [Medline].
• Kanan MW, Kandil E. Bejel or non-venereal endemic syphilis. Br J Dermatol. May 1971;84(5):461-4. [Medline].
• Knox JM, Musher D, Guzick ND. The pathogenesis of syphilis and the related treponematoses. In: Johnson RC, ed. The Biology of Parasitic Spirochetes. New York: Academic;249-59.
• Koff AB, Rosen T. Nonvenereal treponematoses: yaws, endemic syphilis, and pinta. J Am Acad Dermatol. Oct 1993;29(4):519-35; quiz 536-8. [Medline].
• Meheus A, Antal GM. The endemic treponematoses: not yet eradicated. World Health Stat Q. 1992;45(2-3):228-37. [Medline].
• Morand JJ, Simon F, Garnotel E, et al. [Overview of endemic treponematoses]. Med Trop (Mars). Feb 2006;66(1):15-20. [Medline].
• Nsanze H, Lestringant GG, Ameen AM, et al. Serologic tests for treponematoses in the United Arab Emirates. Int J Dermatol. Nov 1996;35(11):800-1. [Medline].
• Parish JL. Treponemal infections in the pediatric population. Clin Dermatol. Nov-Dec 2000;18(6):687-700. [Medline].
• Sharma VK, Kumar B. Malignant syphilis or syphilis simulating malignancy. Int J Dermatol. Sep 1991;30(9):676. [Medline].

Article Last Updated: Feb 21, 2007