Endemic syphilis is a nonvenereal variant of syphilis. The pathogen is the bacterium Trepnoma pallidum ssp. endemica. Treatment is by administration of penicillin for several weeks.
What is endemic syphilis?
Endemics are diseases that occur in relation to a specific population or limited area. For example, syphilis exists as an endemic variant in Africa, the Arabian Peninsula, and the Middle East. Each form of syphilis is a venereal disease resulting from infection with the bacterium Treponema pallidum. Unlike “common syphilis,” transmission in endemic syphilis does not occur venereally. Infections occur through smear infection and are the result of close social contact in socioeconomic conditions that are usually poor. The endemic form of syphilis is also known as Bejel or Njoverav and mainly affects children between the ages of four and ten. The non-venereal route of transmission in this case means infection without sexual contact. Endemic syphilis mainly affects nomadic populations living in close social association under poor hygienic conditions. The endemic form of syphilis is most widespread among the nomads of the Sahel, the pygmies between the Republic of Congo and Central Africa, and among Bedouin peoples in Saudi Arabia. Dry areas such as Iraq, Iran, Kazakhstan, Turkmenistan, Uzbekistan, Afghanistan, and Xinjiang have also been risk areas in the past. In-country names include Frenga, Dichuchwa, Siti, and Skerljevo.
Causes
As in “true syphilis,” a gram-negative screw bacterium of the family Spirochaetaceae is the causative agent of endemic syphilis. Initially, a separate bacterial species called Trepnoma endemica was determined to be responsible for endemic syphilis. However, since the pathogen shows high similarity to the pathogen of “true syphilis”, both bacterial species are now treated as subspecies of the species Trepnoma pallidum. For endemic syphilis, the subspecies is more precisely Trepnoma pallidum ssp. endemica. The subspecies lacks some pathogenicity factors that the causative agent of “true syphilis” possesses. Unlike “true syphilis,” endemic syphilis cannot infect nerve tissue or survive in scar tissue. Morphological differences between the two pathogens do not exist. Both are helical bacteria with a length between five and 20 µm and a width between 0.1 and 0.4 µm. The mode of locomotion of both pathogens consists of longitudinal rotational movements. Because of their environmental stability and sensitivity to dryness, both bacteria are transmitted primarily in direct skin or mucosal contact. Somewhat less frequently, transmission of bejel occurs during breastfeeding or via indirect contact, as occurs through flying or sharing of dishes.
Symptoms, complaints, and signs
Like all bacteria of the Trepnoma species, Bejel is characterized by a course of several phases. Open skin sores are the leading symptom. Primary lesions occur at the site of entry of the bacterium and are often not directly visible. After an incubation period of up to three months, the corners of the affected person’s mouth become inflamed. Smaller lesions occur on the oral mucosa. The ulcers bleed easily. At times, visible sores occur in the anogenital region or on the larynx. Skin lesions and bone involvement are a possible but not particularly common sign in the early stages. During the first stage, the general condition of patients is usually not affected. After about six to nine months, the latent stage of infection occurs. The long tubular bones of the tibia and the facial bones of the nasal bone are affected. Proliferative, deforming periostitis occurs. Ulcerative skin lesions or major skin defects are present. At times, the infection progresses for years and results in disfiguring tissue defects. The central nervous system and heart are spared, unlike in “true syphilis.”
Diagnosis
Diagnosis of endemic syphilis requires microbiologic evidence of the bacterium Trepnoma pallidum. Routine examination of the tissue or does not reveal the thin bacteria in most cases.Native dark-field microscopy or specific fluorescence microscopy is usually required to detect the pathogen. In the early phase of the disease, antibodies are present which are detected by Treponema pallidum hemagglutination assay and fluorescent Treponema antibody absorption test. Differentiation from “true syphilis” is only possible in a specialized laboratory. Patients with endemic syphilis have a much better prognosis than patients with “true syphilis.”
Complications
A number of complications can occur in the setting of endemic syphilis. For example, the pathogens spread throughout the body after only a few weeks and also affect the lymph nodes, among other places. In the secondary stage, there is a skin rash and sometimes also severe bone, intestinal and meningeal changes. In the tertiary stage, endemic syphilis causes further inflammation of the bones and heart. Possible inflammation of the aorta significantly increases the risk of aneurysm. Rarely, the formation of so-called gummas occurs. These tumors can appear externally on the skin as well as affect the internal organs and the skeleton. Characteristic is also a progressive personality deterioration, which often lasts for years and is irreversible. In the first and second stages, the disease is also highly contagious. In infected women, the pathogen can enter the embryo during pregnancy and lead to congenital syphilis in the child. If endemic syphilis is not treated, it can lead to death even years later. It is therefore recommended that a doctor be consulted immediately if syphilis is suspected.
When should you see a doctor?
If the affected person suffers from unusual sores on the skin, it is advisable to consult a doctor. If the sores occur repeatedly or the frequency of occurrence increases, a visit to the doctor is necessary. There is a risk that germs enter the organism through the open wounds, leading to further diseases. A doctor should be consulted as soon as a general feeling of indisposition, dizziness or physical weakness appears. Repeated inflammations of the corners of the mouth should be medically clarified, examined and treated. If the corners of the mouth tear again over a period of several weeks or months, it is advisable to seek medical attention. In case of complaints of the oral mucosa or changes in the appearance of the skin, a doctor should be consulted. Sores in the region of the larynx are considered unusual and should be clarified by a medical professional. Often, in a gradual process, the state of general well-being decreases. A visit to the doctor is advisable as soon as the changes in the lowered general condition are noticed in everyday life. If there is pain in the bones, an inner restlessness or a diffuse feeling of illness, a doctor should be consulted. If there are emotional problems, an increased experience of stress or a persistent depressed emotional state, it is advisable to consult a doctor.
Treatment and therapy
Endemic syphilis is a bacterial infection. Like most other infections, treatment requires primarily targeted antibiotic therapy. Antibiotic treatment extends for at least two weeks for patients with endemic syphilis. The drug used is penicillin. In mild disease in the initial phase, a single administration of depot preparations is often sufficient. Despite growing bacterial resistance, the pathogens of endemic syphilis are not yet resistant to penicillin. If the patient has hypersensitivity to penicillin, macrolides and tetracyclines are used instead of the drug. If the disease is severe at a later stage, conservative drug treatment of endemic syphilis is continued for a longer period of time. Mass decay of the bacteria often occurs during therapy. A Herxheimer reaction with acute symptoms of poisoning is conceivable for this reason. After antibiotic therapy, severe tissue defects are often present, which can later be corrected or reconstructed in the course of plastic surgery.
Outlook and prognosis
The prognosis for patients with endemic syphilis depends on various factors but cannot be predicted with certainty in individual cases. In general, starting treatment early has a positive effect on the disease prognosis for endemic syphilis.In addition, the patient’s general state of health and the hygienic conditions play a decisive role. If patients receive medical treatment at an early stage of endemic syphilis, the disease can often be kept under control with drugs. Since complications are possible at any time even during adequate therapy, close medical monitoring of patients is highly relevant. Once treatment with antibiotic agents has been completed, pronounced defects often remain in the tissue of the affected person. In such cases, plastic surgeons reconstruct the corresponding areas if desired and possible. However, scars or other externally visible defects often remain that cannot be repaired. The prognosis of endemic syphilis without appropriate medical therapy is comparatively poor. This is because the infectious disease attacks various parts of the organism over time and sometimes leads to the death of patients several years after infection. Prior to this, those affected by endemic syphilis suffer from numerous complaints that severely limit the quality of life and make sudden complications likely.
Prevention
A preventive vaccine against treponemas is not yet known. For this reason, prophylaxis in the case of endemic syphilis is limited to avoidance of close social contact in the areas described and improvement of hygienic circumstances. Education about the disease and the mode of transmission is considered by researchers to be the most important preventive step. Medically improved care should eliminate the pathogen from populations at risk.
Follow-up
The sufferer of this disease is primarily dependent on a very rapid diagnosis followed by treatment to alleviate the condition. If this does not result in an early diagnosis, severe complications and a significant worsening of the symptoms may occur, so the focus in this disease is usually the early detection of this complaint. The earlier the disease is detected, the better is usually the further course. In most cases, the disease is treated with the help of medications, mainly antibiotics are used. When taking antibiotics, care should be taken to ensure that they are taken in the correct dosage and on a regular basis. They should also not be taken together with alcohol, as this can significantly weaken the effect. In most cases, they must be taken for a few days after the symptoms have subsided in order to completely alleviate the symptoms permanently. In the case of children, it is above all the parents who must ensure that the medication is taken correctly and regularly. Further follow-up measures are usually not necessary, and the life expectancy of the affected person is also usually not reduced with timely treatment.
What you can do yourself
Endemic syphilis progresses in phases, with a variety of complications to be expected, especially in the later stages. In the early stages, the disease shows few symptoms, but it is highly contagious during this time. Since, in contrast to true syphilis, no sexual contact is required for transmission of the pathogens, the patient’s entire social environment is at risk. This circumstance is usually also emotionally very stressful for the person affected. In addition, the existence of a non-venereal form of syphilis is not generally known. The diagnosis of syphilis is therefore often accompanied by stigmatization. Patients are ashamed of their disease, which increases the psychological suffering. In this situation, those affected should join a self-help group. These can also be contacted online via the Internet. Patients who are suffering emotionally should also not be afraid to consult a therapist. There are not many self-help measures available against the physical consequences of the disease. However, it is extremely important that those affected take it easy and drink enough to compensate for the loss of fluids caused by diarrhea. In addition, medication must be taken as prescribed and all follow-up examinations must actually be attended.
