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

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.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.

More on Endemic Syphilis

Overview: Endemic Syphilis
Differential Diagnoses & Workup: Endemic Syphilis
Treatment & Medication: Endemic Syphilis
Follow-up: Endemic Syphilis
References
[ CLOSE WINDOW ]

References

  1. Clyti E, dos Santos RB. [Endemic treponematoses in Maputo, Mozambique]. Bull Soc Pathol Exot. May 2007;100(2):107-8. [Medline].

  2. Parish JL. Treponemal infections in the pediatric population. Clin Dermatol. Nov-Dec 2000;18(6):687-700. [Medline].

  3. Sharma VK, Kumar B. Malignant syphilis or syphilis simulating malignancy. Int J Dermatol. Sep 1991;30(9):676. [Medline].

  4. Antal GM, Lukehart SA, Meheus AZ. The endemic treponematoses. Microbes Infect. Jan 2002;4(1):83-94. [Medline].

  5. Blount JH, Holmes KK. Epidemiology of syphilis and the non-venereal treponematoses. In: Johnson RC, ed. The Biology of Parasitic Spirochetes. New York, NY: Academic; 1976:157-76.

  6. Cutler JC. Endemic syphilis, yaws, and pinta. In: Johnson RC, ed. The Biology of Parasitic Spirochetes. New York, NY: Academic; 1976:365-73.

  7. Engelkens HJ, Niemel PL, van der Sluis JJ, Meheus A, Stolz E. Endemic treponematoses. Part II. Pinta and endemic syphilis. Int J Dermatol. Apr 1991;30(4):231-8. [Medline].

  8. Falabella R. Nonvenereal treponematoses: yaws, endemic syphilis, and pinta. J Am Acad Dermatol. Dec 1994;31(6):1075. [Medline].

  9. Farnsworth N, Rosen T. Endemic treponematosis: review and update. Clin Dermatol. May-Jun 2006;24(3):181-90. [Medline].

  10. Kanan MW, Kandil E. Bejel or non-venereal endemic syphilis. Br J Dermatol. May 1971;84(5):461-4. [Medline].

  11. 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, NY: Academic; 1976:249-59.

  12. 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].

  13. Meheus A, Antal GM. The endemic treponematoses: not yet eradicated. World Health Stat Q. 1992;45(2-3):228-37. [Medline].

  14. Morand JJ, Simon F, Garnotel E, Mahe A, Clity E, Morlain B. [Overview of endemic treponematoses]. Med Trop (Mars). Feb 2006;66(1):15-20. [Medline].

  15. Nsanze H, Lestringant GG, Ameen AM, Lambert JM, Galadari I, Usmani MA. Serologic tests for treponematoses in the United Arab Emirates. Int J Dermatol. Nov 1996;35(11):800-1. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

nonvenereal syphilis of children, sibbens, radseyege, siti, therlijevo, njovera, frenjak, Treponema pallidum subsp endemicum, T pallidum subsp endemicum, nonvenereal endemic syphilis Bejel, non-venereal endemic syphilis Bejel

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Hassan I Galadari, MD, MBBS, Assistant Professor of Dermatology, Faculty of Medicine and Health Sciences, United Arab Emirates University
Hassan I Galadari, MD, MBBS is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Medical Student Association/Foundation, and American Society for Dermatologic Surgery
Disclosure: Nothing to disclose.

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
Ibrahim Galadari, MD, MB, BCh, MSc is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Medical Editor

Craig A Elmets, MD, Director of Dermatology, Departments of Dermatology, Pathology, and Environmental Health Sciences; Professor, The Kirklin Clinic, University of Alabama at Birmingham
Craig A Elmets, MD is a member of the following medical societies: American Academy of Dermatology, American Association of Immunologists, American College of Physicians, American Federation for Medical Research, and Society for Investigative Dermatology
Disclosure: Palomar Medical Technologies Stock None; Merck Consulting fee Independent contractor; Tronox Consulting fee Independent contractor; Amgen Consulting fee Review panel membership; Astellas Consulting fee Review panel membership; Massachusetts Medical Society Salary Employment

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Paul Krusinski, MD, Director of Dermatology, Professor, Department of Internal Medicine, Fletcher Allen Health Care, University of Vermont
Paul Krusinski, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.