Bejel

Definition

Bejel, also known as endemic syphilis, is a chronic but curable disease that is seen mostly in children in dry regions, such as parts of Africa (Sudan, southern Rhodesia, and South Africa), parts of the Middle East (among nomadic/Bedouin tribes of Saudi Arabia, Iraq, and Syria), and parts of Asia (Turkey, southeast Asia, and the western Pacific). Unlike venereal syphilis, endemic syphilis is not sexually transmitted. Similar to venereal syphilis, however, it begins with skin sores and has a latent period followed by a more severe stage, which includes bone infections and additional skin lesions.

Demographics

Bejel occurs predominately in children aged two to 15 years. Twenty-five percent of the cases occur in those younger than six years of age, and 55 percent of the cases occur before the age of 16 years. The remaining 20 percent of the cases occur in adults who are in close contact with infected children. Bejel is only rarely reported in the United States and then usually among immigrants and people arriving from areas where the disease is common. Both sexes are equally susceptible to bejel.

Description

Bejel has many other names depending on the locality, including siti (Gambia), njovera (southern Rhodesia), therlijevo (Croatia), and frenjak (Balkans). Bejel is related to yaws and pinta , and together the three diseases are referred to as treponematoses. Yaws, which also affects the skin and bones, occurs in the humid equatorial countries, while pinta, which only affects the skin, is common among the native peoples of Mexico, Central America, and South America.

Transmission

Treponema pallidum subspecies endemicum , the bacteria that causes bejel, is very closely related to the one that causes the sexually transmitted form of syphilis, but the method of transmission is different. In bejel, transmission is by direct contact, with broken skin or contaminated hands, or indirectly by sharing drinking vessels and eating utensils. T. pallidum subspecies endemicum is passed on mostly among children living in poverty in unsanitary environments and with poor hygiene.

Causes and symptoms

The skin, bones, and mucous membranes are all affected by bejel. The disease begins with slimy patches on the inside of the mouth, followed by blisters on the trunk, arms, and legs. Bone infections, mainly in the legs, develop later and cause pain deep within the bones. Eventually, the bones may become deformed because of bone and cartilage destruction. In later stages, soft gummy lesions may form in the nasal passages, destroying nasal cartilage and in the roof of the mouth, even breaking through the mouth palate. The lesions associated with bejel are destructive and may leave disfiguring scars.

Diagnosis

T. pallidum subspecies endemicum can be detected by microscopic study of samples taken from the sores or lymph fluid. However, since antibody tests do not distinguish between the types of syphilis, specific diagnosis of the type of syphilis depends on the child's history, symptoms, and environment.

When to call the doctor

The doctor should be called if symptoms of bejel develop in a child. Travel information is invaluable in diagnosis of the disease.

Treatment

Large doses of benzathine penicillin G given by injection into the muscle can cure this disease in any stage, although it may take longer and require additional doses in later stages. If penicillin cannot be given, alternative antibiotics are chloramphenicol and tetracycline. Since tetracycline can permanently discolor new teeth that are still forming, it is usually not prescribed for children unless no viable alternative is available.

Prognosis

Bejel is usually completely curable with antibiotic treatment. Death from bejel is uncommon. Follow-up care is recommended to detect treatment failures and reinfection.

Prevention

The World Health Organization (WHO) has worked with many countries to prevent this and other diseases, and the number of cases of bejel has been reduced somewhat. Widespread use of penicillin has been responsible for reducing the number of existing cases, but the only way to eliminate bejel is by improving living and sanitation conditions and through continuing health education. Since the disease is very contagious, public health personnel must seek out and treat infected children and their contacts in order to prevent additional cases.

Parental concerns

When traveling in areas where bejel is endemic, parents should ensure that their children avoid contact with children with lesions and avoid shared drinking and eating utensils.

KEY TERMS

Endemic disease —An infectious disease that occurs frequently in a specific geographical locale. The disease often occurs in cycles.

Lymph fluid —Clear, colorless fluid found in lymph vessels and nodes. The lymph nodes contain organisms that destroy bacteria and other disease causing organisms (also called pathogens).

Syphilis —This disease occurs in two forms. One is a sexually transmitted disease caused by A systemic infection caused by the spirochete Treponema pallidum . It is most commonly transmitted by sexual contact.

See also Pinta .

Resources

ORGANIZATIONS

National Organization for Rare Disorders Inc. 55 Kenosia Ave, PO Box 1968, Danbury, CT 06813–1968. Web site: http://www.rarediseases.org.

WEB SITES

"Endemic syphilis." eMedicine , October 28, 2004. Available online at http://www.emedicine.com/derm/topic117.htm (accessed December 6, 2004).

Judith Sims Jill S. Lasker