Bejel Disease or Nonvenereal endemic syphilis is a chronic, inflammatory childhood disease of the treponematosis group. The early disease is characterized by infectious mucocutaneous lesions and osseous manifestations resembling those of secondary syphilis. Following disappearance of early lesions and an undetermined latency period, late manifestations may develop. There are skin and bone lesions similar to those of late “benign” venereal syphilis. If they occur at all, cardiovascular, nervous system, and prenatally acquired manifestations are extremely rare.
Etiology of Bejel Disease
The bejel treponema is morphologically indistinguishable from T. pallidum, T. pertenue, and T. carateum. .It is present in early lesions or lymph node aspirate. The organism has not been cdkn rated in vitro. In laboratory animals Turner Hollander showed consistent differences in chemical reaction as compared with that of yaws venereal syphilis treponemes. The bejel :treponema is apparently an intermediate between the two (Paris-Hamelin et al., 1968). Like other :treponematosis, bejel is accompanied by antibody, formation with seroreactivity in reagin, e.g., VDRL,- and treponemal antibody (TPI/FTA) tests. Childhood infection with bejel protects against later infection with syphilis.
Epidemiology of Bejel Disease
Humans are the reservoir of bejel. Treponemes are most likely transmitted directly among children by skin-to-skin contact, or by hands moistened with treponema-containing saliva, or indirectly via drinking flasks, the spouts of which have been demonstrated to contain treponemes (Grin). Treponema implantation is generally facilitated by labial and oral fissures, occurring in dry climates, or by small mucosal lesions. Bejel is a household disease. In some instances 60 to 70 per cent of rural community populations have been reported to be infected. Narrow huts, crowded dwellings, unhygienic living conditions, and low socioeconomic standards favor transmission.
There are many scattered endemic centers of bejel in backward rural areas north and south of the tropics. Bejel occurs along the Kalahari and Sahara deserts in Africa, in the countries of the Balkans and the Eastern Mediterranean region, on the Arabian peninsula, in central Asian countries, and in Australia. It prevails in arid areas in contrast to yaws, which is prevalent in moist, tropical jungle regions. Bejel was first described as a disease of nomadic people, subsequently to occur in settled rural populations, e.g., among the Dogons of Mali, the Islamic descendants of Bosnia, and the Bakwaas of Botswana. Previously it was widespread in the Middle East and Europe. Bejel has not been observed in the Western Hemisphere, where pinta and yaws are the prevailing childhood treponematoses.
Bejel is in regression from its higher prevalence of two to three decades ago as a result of extensive mass penicillin campaigns and some improvement of health services. In certain areas the prevalence of bejel has remained higher than that of yaws because of occurrence in nomadic tribes, on account of geographic inaccessibility of endemic foci, and inadequacy of health services (Basset, 1963). It is likely to recur when mass treatment has been incomplete. Thus, in Niger infectious lesions (5 per cent) and seroreactivity (30 to 40 per cent) were found some years after a mass treatment campaign.
Clinical Manifestations and Diagnosis.
The experimental incubation period is approximately three weeks. Initial lesions are rarely encountered. The earliest lesions are “mucous patches” of the secondary type localized to the oral and facial mucosa. “Split papules” occur at the oral angles. Local papular condylomata lata or anal, genital, or other intertriginous skin areas were observed in 25 per cent of infected children in Iraq (Guth nd Luger). Generalized secondary rashes and alopecia are relatively rare. Regional lymphadenopathy is common. Polyadenitis is rare. The early disease is followed by a latency period of undetermined duration, with seroreactivity as the sole sign of infection. Late “benign” manifestations of the skin develop in some patients. They do not differ in character from those in late venereal syphilis. Superficial tubero ulcerative skin lesions and characteristic serpiginous nodular ulcers occur.
Nasopharyngeal ulcerations may occur and range from palate perforation to rhinopharyngitis mutilans as in yaws and syphilis (gangosa). Gummatous ulceration of the breast may occur in women previously infected with bejel who are suckling a child with oral lesions, a phenomenon which supports the concept that gumma may be delayed hypersensitivity reactions from repeated exposure (see Syphilis). Juxta-articular nodes have been described in some geographic areas.
Bone lesions are the most frequent manifestations of late bejel, affecting the clavicle, other long bones, and the frontal bones, giving rise to swelling, tenderness, and pain. There is periosteal endosteal proliferation, and deformities may result. Isolated cases of cardiovascular and neurologic system involvement have been described in Bosnia (Grin) and Botswana (Murray et al.). Incidental cases of prenatally acquired disease have also been reported. No case of prenatal or systemic disease was observed in several thousand examinations in a WHO project in Syria which included radiologic and cerebrospinal fluid examinations. When observed, the systemic manifestations may be due to the occurrence also of venereal syphilis in the geographic area concerned.
Diagnosis of Bejel Disease.
The diagnosis of early bejel is established on epidemiologic and clinical grounds. It may be confirmed by dark-field demonstration of treponemes in the lesions or in node aspirate and by serologic tests (VDRL), reactive in nearly 100 per cent of cases. Serodiagnostic tests cannot differentiate latent bejel from latent yaws or syphilis. Because late clinical lesions are similar to those of yaws and syphilis, the local epidemiologic situation is an important diagnostic consideration. As distinct from the manifestations of late prenatal syphilis, dental deformities and interstitial keratitis are not observed in bejel.
Treatment and Prevention of Bejel Disease.
Penicillin is as effective against bejel as it is against yaws, syphilis, and pinta. In the control campaigns initiated by the World Health Organization in several countries (1950 to 1960), one dose of 1.2 mega units of long-acting penicillin PAM or benzathine penicillin G was given intramuscularly in early cases, with two further doses at three- to seven-day intervals to patients with late manifestations. Half doses are used for contacts. The longer acting benzathine penicillin is preferred in contact treatment among nomadic tribes. Rapid healing of early lesions was followed by sero reversion within a year in a large proportion of cases. Late destructive lesions required more time. Healing with scars progressed slowly but definitely and with
no effect on treponemal antibody tests (TPI/FTA), as in syphilis.Seroepidemiologic studies undertaken in Bosnia, Yugoslavia, 20 years after the penicillin mass campaign—which was followed by systematic periodic surveillance over ten years —showed that childhood infection had been reduced to nil, the community seroimmunologic profiles indicating complete interruption of transmission.
During this period adequate basic health services were provided, health education promoted, and general socioeconomic development took place. Bosnia remains the only example of eradication of endemic treponematoses. However, eradication of childhood infection has resulted in a population susceptible to venereal syphilis in later life. The absence of protective cross-immunity has thus created a new epidemiologic situation in which sporadic cases of the venereal treponematosis occurs.