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Last updated: 4/17/2025
Years published: 1986, 1993, 2004, 2009, 2025
NORD gratefully acknowledges Gioconda Alyea, MD (FMG), MS, National Organization for Rare Disorders, for assistance in the preparation of this report.
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Bejel, also known as endemic syphilis, is a chronic bacterial infection that mainly affects the skin and mucous membranes (the moist linings inside the mouth, nose and other parts of the body).1,2
Bejel is caused by a bacterium called Treponema pallidum subspecies endemicum (TEN) which is in the same family as the bacteria that cause syphilis, pinta and yaws.1
Bejel usually affects children between the ages of 2 and 15, mostly in hot, dry regions such as parts of the Middle East and Africa. In very rare cases, people in Europe and North America have also been diagnosed with bejel.1 Bejel spreads mainly through direct skin or mouth contact and by sharing eating or drinking utensils.2 Although it’s generally not considered a sexually transmitted infection, rare cases of sexual transmission have been reported.2,3
Treatment is with antibiotics.
Bejel signs and symptoms progress in three stages, similar to syphilis:1,2,,4,5
Bejel is not known to cause congenital infection; this may be because most new infections occur in children rather than in women of childbearing age.6
A milder version of bejel has been seen in some areas, where symptoms are fewer and less severe, such as leg pain with bone changes.
Bejel is part of the endemic treponematoses, a group of chronic bacterial infections caused by treponemes, spiral-shaped bacteria. Specifically, bejel is caused by the infection with the bacteria Treponema pallidum endemicum.
In addition to bejel, the endemic treponematoses include yaws, caused by Treponema pallidum subsp. pertenue and pinta (caused by T. carateum).1,3,4
Bejel, yaws and a more common disease, syphilis, are caused by bacteria belonging to the Treponema pallidum species. Syphilis is caused by Treponema pallidum subspecies pallidum.
T. pallidum belongs to a family of gram-negative spiral-shaped bacteria, the Spirochaetaceae,and has a length ranging from 10 to 15 microns and a diameter of 0.2 microns, making it invisible to light microscopy except under dark field illumination.
Treponemes penetrate the human host through epidermal micro-scrapes, adhere to the skin cells and multiply locally. After invasion, the organisms appear in lymph nodes within minutes and disseminate widely within hours, reaching and surviving in distant skin and organs.
Bejel spreads mainly through direct skin or mouth contact and by sharing eating or drinking utensils. Bejel has been detected in primary genital lesions and may be transmitted sexually, and rare cases of sexual transmission have been reported.2,3
A whole genome analysis of a limited number of T. p. pallidum, T. p. pertenue, and T. p. endemicum have shown that the overall genetic similarity between the three subspecies is very high (99.8%), meaning they are closely related. However, there are specific regions in the DNA where the sequences differ, and these regions are used to tell the different subspecies apart. These genetic differences allow for the development of tests that can identify and distinguish between syphilis and yaws strains, which is not possible with other tests. 6 It’s currently unknown whether these small genetic differences are directly linked to the distinct signs and symptoms of these diseases.
Bejel occurs mainly in the arid areas of the Sahel (southern border of the Sahara Desert) including Senegal, Burkina Faso and Niger. In these areas, high rates of seropositivity (10% to 20%) in children under 15 years of age have been reported. Bejel has also been described among the nomadic people of the Arabian Peninsula (Saudi Arabia, Iraq and Syria). There was a report of three cases of bejel in Turkey in 1995 (where the disease was considered eliminated) as well as one case report from Iran in 2012 and one from Pakistan in 2013.6
The status of bejel endemicity can be seen at the World Health Organization (WHO) Global Health Observatory website.
Doctors usually make the diagnosis by the signs and symptoms and by looking at the appearance of the sores or skin lesions, especially in people who live in or have traveled to areas where bejel is common.
However, diagnosing bejel can be very difficult, especially in the early stages of the illness, because it looks very similar to other diseases caused by related treponema bacteria, especially syphilis (a sexually transmitted infection) and yaws (a skin infection common in tropical regions).1,2,3
The medical history, specifically travel or exposure history, and where the affected person is from or has been, can be very helpful to make a diagnosis. For example, bejel tends to occur in dry areas like parts of the Middle East and North Africa, and it usually spreads through non-sexual contact like sharing utensils or close skin contact, often in children. Syphilis, on the other hand, is mostly sexually transmitted and found worldwide, especially in adults.1
The following are laboratory tests used to help to make the diagnosis, but the test results can be similar the other treponemal infections and are not specific to Treponema pallidum endemicum, the treponema that causes bejel:4,5,6
The test called PCR (polymerase chain reaction) can detect the DNA of the specific type of Treponema bacteria causing the infection. However, this test is more advanced and may not be available in all countries or areas.2,4,5,6
X-rays or other imaging tests are not useful for diagnosing these diseases, but in late stages, they might show changes in the bones if the infection has spread there.
Treatment
Bejel can be completely cured with antibiotics. The main treatment is an injection of penicillin G benzathine which is given as a single dose. Children under 10 years old receive a lower dose (1.2 million units), while those 10 and older receive a higher dose (2.4 million units).6
This is based on how similar infections like yaws are treated, since they share many features with bejel.
An alternative option is azithromycin, an antibiotic taken by mouth. The World Health Organization recommends a single dose of azithromycin based on body weight (30 mg per kilogram, up to a maximum of 2 grams). Both penicillin and azithromycin work equally well in most cases.7
If someone is allergic or can’t tolerate penicillin or azithromycin, doxycycline is another option. This is taken as a pill, 100 mg twice a day for 14 days.4,5,6
People who live with or frequently interact with someone who has bejel, whether they have symptoms or not, should also be treated. This helps prevent the infection from spreading. Even people who test positive but don’t show symptoms (called latent bejel) should receive treatment if they live in an area where bejel is common.
For people with more advanced (late) symptoms, a second injection of penicillin may be given one week after the first.4,5,6
Blood tests (RPR or VDRL) are used to track how well treatment is working. In most people, the antibody levels drop fourfold within a year. It’s recommended to repeat these blood tests at 6 and 12 months after treatment. If the levels don’t drop as expected a second round of treatment with penicillin may be needed.4,5,6
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For information about clinical trials conducted in Europe, contact: https://www.clinicaltrialsregister.eu/
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