What is Bejel?

Bejel, also known as endemic syphilis or non-venereal syphilis, is a disease caused by the bacterium Treponema pallidum subspecies endemicum. It belongs to a group of infections known as endemic treponematoses, which also include yaws and pinta. Unlike venereal syphilis, which is sexually transmitted, bejel is spread through non-sexual contact, such as skin-to-skin contact or sharing contaminated eating and drinking utensils. This mode of transmission makes children particularly vulnerable to infection.

The bacterium Treponema pallidum subspecies endemicum is closely related to the bacterium that causes venereal syphilis. The World Health Organization (WHO) recognizes bejel as a neglected tropical disease (NTD). It primarily occurs in hot and dry regions, including parts of Africa, Asia, and the Middle East. Like the other endemic treponematoses, bejel is characterized by a multi-stage infection process that can affect the skin, bones, and cartilage.^1,2

Cutaneous manifestations by stage

The skin manifestation of bejel differs based on the stage of infection. Mainly, there are three stages: primary stage, secondary stage, and tertiary (late stage). Knowing these stages is important in recognising the disease so that the infected can receive prompt treatment. 

Primary stage

The initial lesions in primary bejel are small, superficial, and painless, typically appearing as lesions on the mucous membranes of the oropharynx (the middle part of the throat behind the mouth) and nasopharynx (the upper part of the throat). These lesions are often overlooked because they are small and located in less visible areas. Additionally, since they are usually painless, they often go unreported.^1,3

Secondary stage

In the secondary stage of bejel, the skin manifestations become more noticeable and widespread, similar to those seen in venereal syphilis. Lesions may appear on the mucous membranes, such as the inner lining of the mouth, tonsils, tongue, lips, and the upper throat (nasopharynx). These mucous patches can be accompanied by:

Regional lymphadenopathy: Swelling of the lymph nodes

Diffuse maculopapular rash: A widespread rash that can appear on various parts of the body, including the trunk (torso), palms, soles, and limbs. This rash is usually not itchy and can take different forms:

• Macular: Flat, discolored spots
• Papular: Raised, solid bumps
• Follicular: Involving hair follicles
• Papulosquamous: Raised lesions with scaly patches
• Pustular: Pus-filled bumps

Condyloma lata: These are wart-like lesions that typically occur in warm, moist areas where skin touches skin, such as the armpits, groin, and around the anal region. These lesions are highly infectious and play a significant role in spreading the bacteria.

Ulcers: Painless ulcers can develop on the skin, especially in areas prone to friction or trauma, such as under the arms or around the rectum and groin. These ulcers are usually shallow and well-defined, meaning they have clear edges.

Other possible symptoms during this stage include osteitis and periostitis, which are inflammations of the bones and the lining around the bones. These conditions can cause bone pain, particularly at night.

Some patients who are not treated may experience recurrences of the secondary stage in the first several years after infection. The secondary manifestations of bejel typically heal within 6-9 months. After this period, the disease may enter a latent stage, where symptoms are absent but the infection remains in the body.^4,5

Tertiary (late) stage

The latency period in bejel can last for many years. During this time, the patient carries infectious organisms, primarily in the spleen, lymph nodes, and other tissues, and blood tests for the infection remain positive. 

In the tertiary stage, the cutaneous manifestations become more severe and can lead to permanent disfigurement. The primary skin lesions in this stage are known as gumma.

A gumma is a highly destructive lesion that commonly affects the skin and bones but can also appear in other tissues, such as the liver and brain. It is characterized by a firm, nodular area with a necrotic (dead tissue) center, surrounded by inflamed tissue. Gummas may present as ulcerative plaques that are either painless or have a crater-like appearance. 

These lesions typically develop due to a delayed hypersensitivity response to the small number of treponeme bacteria present in the lesions. Gummatous nodules on the skin can evolve into infiltrated areas with abnormal pigmentation. When gummas occur in the nasopharynx, they can lead to a condition known as destructive rhinopharyngitis (gangosa), which involves severe damage to the nasal and throat structures.^6,7

Treatment 

• Antibiotic: For over 50 years, intramuscular benzathine penicillin has been the standard treatment for both venereal syphilis and endemic treponemal diseases, including bejel. This antibiotic is effective in eliminating the bacteria and resolving skin lesions. For individuals allergic to penicillin, alternative treatments include doxycycline or tetracycline. Skin lesions typically heal following antibiotic treatment. However, some scarring and deformities may persist, especially if the lesions were extensive or if treatment was delayed
• Household treatment: It is recommended to treat all household members to prevent reinfection and stop further transmission, as bejel can spread through close contact^4,7

Prevention and public health awareness

• Improved hygiene: Since bejel is transmitted through non-sexual skin contact and the sharing of utensils, improving personal and community hygiene is crucial. Regular hand washing and avoiding sharing personal items, especially in endemic areas, can significantly reduce the risk of transmission
• Early diagnosis: Detecting and treating bejel in its early stages can prevent the development of more severe skin manifestations and help reduce the spread of the disease
• Public health measures: Implementing community-wide treatment programs in endemic areas can significantly curb the spread of bejel. These programs ensure that everyone affected receives appropriate treatment, which helps lower the number of infections in the community
• Education: Raising awareness about bejel, including how it spreads and the importance of good hygiene, can help prevent new cases. Educational efforts should particularly target high-risk populations in regions where the disease is common^1,6

FAQ’s

What are the symptoms of bejel?

• Primary stage: small painless lesions or ulcers usually inside the mouth or throat
• Secondary stage: non-itchy rashes in the trunk and extremities, wart-like lesions (condiloma lata), and painless skin ulcers in areas like the armpit and groin area
• Tertiary stage: destructive lesions called gumma, which is a firm nodular lesions with a necrotic center that can cause scarring and permanent damage or disfigurement.

What is the difference between bejel and yaws?

Bejel and yaws are both nonvenereal endemic treponematoses caused by subspecies of Treponema pallidum; the differences between these two are:

Causative organism

• Bejel: Treponema pallidum subsp. endemicum
• Yaws: Treponema pallidum subsp. pertenue

Geographic distribution

• Bejel: Arid regions such as Africa, Asia, and the Middle East
• Yaws: Tropical regions including Southeast Asia, West Africa, and the Pacific Islands

Transmission

• Bejel: Non-sexual skin contact, shared utensils
• Yaws: Direct skin-to-skin contact with lesions

Skin manifestations

Bejel typically starts with painless, small lesions or ulcers in the mouth or throat, progressing to a non-itchy rash, condyloma lata, and painless skin ulcers in the secondary stage. Yaws begins with a "mother yaw," a highly infectious ulcerative papule, followed by multiple papillomatous skin lesions and painful bone and joint involvement. In the tertiary stage, bejel may cause gummas, which can lead to severe deformities, while yaws may result in destructive skin and bone lesions, often causing significant deformities like saber shins.⁸

What are the three stages of bejel?

Primary, secondary, and tertiary.

How do you treat bejel?

The treatment of bejel is similar to that of venereal syphilis and involves antibiotics, specifically benzathine penicillin. 

Summary

Bejel or non-venereal syphilis is an infection caused by bacteria called Treponema pallidum subspecies endemicum. This disease differs from venereal syphilis in that it is spread through non-sexual skin contact and the sharing of eating or drinking utensils. Once infected, a person may experience various symptoms, including skin manifestations that differ depending on the stage of the disease. These include painless lesions, skin rashes, bone pain, and in some cases, gummas. There may also be a latency period if untreated, when symptoms are resolved, but the infection still remains inside the body. Treatment with antibiotics is effective in curing the infection, but early detection is crucial for its management and the prevention of severe complications.