• Disease Overview
  • Synonyms
  • Signs & Symptoms
  • Causes
  • Affected Populations
  • Disorders with Similar Symptoms
  • Diagnosis
  • Standard Therapies
  • Clinical Trials and Studies
  • References
  • Programs & Resources
  • Complete Report

Bejel

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Last updated: May 15, 2009
Years published: 1986, 1993, 2004, 2009


Disease Overview

Bejel is an infectious disease that is rare in the United States but common in certain parts of the world. The infection is very similar to syphilis but is not sexually transmitted. Most frequently, transmission is by means of non-sexual skin contact or by common use of eating and drinking utensils. The organism that causes bejel belongs to the same family as the bacterium that causes syphilis, pinta and yaws and is known as treponema. Treponemas are spiral-shaped bacteria (spirochetes). Also known as endemic syphilis, bejel is characterized by lesions of the skin and bones that begin in the mouth and progress in gradual stages. The late stages are the most severe.

Bejel is very common in dry, hot climates especially in the countries of the eastern Mediterranean region and in Saharan West Africa.

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Synonyms

  • Dichuchwa
  • Endemic Syphilis
  • Frenga
  • Njovera
  • Nonvenereal Syphilis
  • Siti
  • Treponematosis, Bejel Type
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Signs & Symptoms

At the outset, children with bejel have patchy, slimy, ulcerations (lesions) on the mucous membranes, particularly in or near the mouth. Later, blisters appear on the back, arms, and legs. Further disease progression is indicated by infections of the bones, especially those of the legs. In these later stages, soft, gummy lumps (gummas) appear on the roof of the mouth and in the nasal passages.

Other symptoms may include swollen glands or lymph nodes (lymph-adenopathy), and/or skin ulcerations under the arms or around the rectum and groin.

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Causes

Bejel is a rare infectious disease caused by a spiral-shaped bacterium (spirochete), known as Treponema pallidum endemicum. Under a microscope, T. p. endemicum is virtually indistinguishable from Treponema pallidum, the bacterium that causes syphilis. The disease is not sexually transmitted.

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Affected populations

In the USA, bejel remains a rare disorder. It was brought to the USA via immigration of families from countries in which it is common (endemic), or by Americans who may have contracted the disease while in a hot, dry country.

Bejel exhibits no preferences or propensities as to gender or race. Children living in unsanitary conditions are most often affected. About 25% of cases involve children of six years or younger; about 55% involve children and adolescents of age 16 years or younger, while about 20% involve persons of adult age, especially those who are closely involved with children.

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Diagnosis

The diagnosis of bejel is based on the geographic history of the patient as well as laboratory testing of material from the lesions (darkfield examination). The responsible bacterium is readily identifiable on sight in a microscope as a treponema. Samples of the fluid that surrounds the lesions yield a positive VDRL (Venereal Disease Research Laboratory) and FTA-ABS (fluorescent treponemal antibody-absorption) test. However, additional history may be required to differentiate bejel from venereal syphilis.

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Standard Therapies

Treatment

The standard treatment for bejel is the antimicrobial drug benzathine penicillin G. Those individuals who are allergic to penicillin may be given doxycycline or tetracycline. The lesions caused by this disease typically heal after treatment. However, scarring may be permanent.

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Clinical Trials and Studies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

Research on tropical diseases is ongoing. The development of a treponemal vaccine is also being investigated. For more information about these disorders contact the World Health Organization (WHO) listed in the Resources section below.

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References

TEXTBOOKS

Beers MH, Berkow R, eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:1185-86.

Berkow R., ed. The Merck Manual-Home Edition.2nd ed. Whitehouse Station, NJ: Merck Research Laboratories; 2003:1099.

Bennett JC, Plum F, eds. Cecil Textbook of Medicine. 20th ed. W.B. Saunders Co., Philadelphia, PA; 1996:1714.

Mandell GL, Bennett JE, Dolan R, eds. Mandell, Douglas and Bennett’s Principles and Practice of Infectious Diseases. 4th ed. Churchill Livingstone Inc. New York, NY; 1995:2133, 2136-37.

JOURNAL ARTICLES

Rothschild BM, Coppa A, Petrone PP. “Like a virgin”: Absence of rheumatoid arthritis and treponematosis, good sanitation and only rare gout in Italy prior to the 15th century. Reumatismo. 2004;56:61-66.

Mitchell PD. Pre-Columbian treponemal disease from 14th centruy AD Safed, Israel, and implications for the medieval eastern Mediterranean. Am J Phys Anthropol. 2003;121:117-24.

Antal GM, Lukehart SA, Meheus AZ. The endemic treponematoses. Microbes Infect. 2002;4:83-94.

Klouman E, Masenga EJ, Sam NE. Serological markers for treponemal infection in children in rural Kilamanjaro, Tanzania: evidence of syphilis or non-venereal treponematoses? Genitourin Med. 1997;73:522-27.

FROM THE INTERNET

Galadari I. Endemic Syphilis. emedicine. Last Updated: February 23, 2004. 8pp.

www.emedicine.com/derm/topic117.htm

Fine S, Fine LS. Treponematosis (Endemic Syphylis). emedicine. Last Updated: June 13, 2002. 9pp.

www.emedicine.com/med/topic2305.htm

Bejel. Amersham Health. ©2004. 1p.

www.amershamhealth.com/medcyclopaedia/medical/volume%20III%201/BEJEL.ASP

Bejel, Yaws, and Pinta. Merck Manual of Medical Information – Second Home Edition. ©2004. 2pp.

www.merck.com/mmhe/sec17/ch190/ch190d.html?alt=pf

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Programs & Resources

RareCare® Assistance Programs

NORD strives to open new assistance programs as funding allows. If we don’t have a program for you now, please continue to check back with us.

Additional Assistance Programs

MedicAlert Assistance Program

NORD and MedicAlert Foundation have teamed up on a new program to provide protection to rare disease patients in emergency situations.

Learn more https://rarediseases.org/patient-assistance-programs/medicalert-assistance-program/

Rare Disease Educational Support Program

Ensuring that patients and caregivers are armed with the tools they need to live their best lives while managing their rare condition is a vital part of NORD’s mission.

Learn more https://rarediseases.org/patient-assistance-programs/rare-disease-educational-support/

Rare Caregiver Respite Program

This first-of-its-kind assistance program is designed for caregivers of a child or adult diagnosed with a rare disorder.

Learn more https://rarediseases.org/patient-assistance-programs/caregiver-respite/

Patient Organizations


National Organization for Rare Disorders