Framboesie: Causes, Symptoms & Treatment

Frambösie is a nonvenereal infectious disease of tropical countries caused by the bacterium Treponema pertenue. The disease, which is contagious through skin contact, is one of the treponematoses and is readily treatable with antibiotics. If left untreated, the skin disease develops in four stages, beginning with raspberry-like papules and leading to destruction of the bones and joints over a period of usually more than ten years in the fourth stage.

What is Frambösie?

Frambösie is a non-venereal skin disease of tropical countries that is contagious via skin contact and droplet infection and, like venereal and non-venereal syphilis, belongs to the treponematoses. The name Frambösie is derived from the French Framboise for raspberry, because in the first stage the disease is noticeable by raspberry-like papules on the skin. Many other names such as Framboesia tropica or raspberry epidemic are used synonymously for the disease. The pathogen, the bacterium Treponema pertenue, belongs to the spirochetes. They are thin, helical, gram-negative bacteria that can actively move themselves via internal flagella. The main distribution area of Frambösie is humid tropical countries of Southeast Asia, Africa and Latin America. Typically, the disease progresses – untreated – in four stages, with the third stage, which can last five to ten years, being symptom-free and can give the deceptive impression that the chronic disease has been overcome.

Causes

The causative agent of the chronic skin disease Frambösie is the bacterium Treponema pertenue, which particularly loves warm and humid tropical climates. Infection occurs mainly through skin contact and smear infections. In exceptional cases, infections through insect bites are also said to occur. The domestic confinement often found in tropical humid areas among the rural population, combined with inadequate hygienic conditions, promotes the risk of infection. In regions where Frambösie is endemic, most people become infected in childhood.

Symptoms, complaints, and signs

Frambösia has an incubation period of three to four weeks and typically presents with one or more “raspberry-like” papules on the skin, preferably on the lower leg. In breastfeeding women, such papules also develop on the breast. The itchy and weeping papules are painless, but cause swelling of the “responsible” lymph nodes. The papules heal again – even if untreated – and new papules develop after a few weeks. In this second stage, also called secondary stage, especially palms and soles are affected. These new papules also disappear after some time and are followed by a third, deceptive dormant stage, which provides freedom from symptoms for five to ten years. Only then does the fourth or tertiary stage appear. The term tertiary stage implies that the long symptomless phase is not counted as a separate phase. During the tertiary stage, changes occur in the bones and joints. Skin nodules develop, which feel rubbery, and there is inflammation of the bones and periosteum. Skeletal changes and so-called gangosa, a visually disfiguring remodeling of the nasopharynx, appear as particularly serious.

Diagnosis

An initial diagnosis is made on the basis of the patient’s history and the conspicuous external signs that accompany frambosia. Morphologic differentiation between the various four Treponema known to be pathogenic is very laborious and not always reliable. The symptomatic signs of the disease should be sufficient for a diagnosis that is considered certain, especially since treatment would be possible with less overall effort than a laboratory-based diagnosis. The course of the disease can be divided into four phases or three stages, as described above, with the last phase beginning five to ten years after the initial infection.

Complications

If Frambrosis is not treated, it can lead to severe complications and damage to the bones and joints. This damage usually occurs after several years and is not immediately visible. In frambosia, papules appear on the patient’s skin after about four weeks.Due to the relatively long incubation period, Frambösie can only be discovered relatively late by a doctor. The papules continue to spread on the hands and feet and usually disappear after a short time. Initially, no complications occur. However, Frambösie becomes noticeable again after about five years. Strong nodules appear on the skin and the bones become inflamed. This leads to severe pain in most patients. A normal everyday life is no longer possible due to the Frambösie. The skeleton changes and the affected persons are disfigured by malformations on the nose. Treatment can be done by adding penicillin and fights Frambösie if treated early. If Frambösie is not detected until the last stage, there is usually no cure and death results. Life expectancy is reduced by Frambösie.

When should you go to the doctor?

In the case of frambosia, treatment must always take place. There is no self-healing and usually a worsening of symptoms if the disease is not treated. A doctor should be consulted when there is the formation of papules on the skin. These take on a reddish color and may also be affected by itching. Furthermore, swelling can also indicate frambosia and should be examined. The papules themselves may disappear without treatment, but usually reappear after a few weeks. In the further course also inflammations at the nodules point to the Frambösie. These usually make themselves felt by very strong bone shards, which must be examined by a physician. The first diagnosis is made by the dermatologist or by the general practitioner. For further treatment, patients depend on taking antibiotics to relieve the symptoms of the disease. In most cases, there is a positive course of the disease and the disease can be well limited.

Treatment and therapy

As mentioned above, the chronic infectious disease occurs exclusively in warm and humid tropical regions, where the rural population often has to live under inadequate hygienic conditions and usually has very limited financial resources. The standard treatment, which has proven effective, is a single intramuscular injection of penicillin. This greatly reduced the disease within WHO campaigns in the 1950s and 1960s, and it fell out of focus in the years that followed until it is now on the rise again. In a small study conducted by the University of Barcelona in 2013/2014 in Papua New Guinea on over 200 sick children, it was shown that the effect between a single injection with penicillin did not differ from a single oral administration of the antibiotic azithromycin. In the “penicillin group” 105 of 113 patients were cured and in the “azithromycin group” 106 of 110 patients were cured. This could mean that in the future, broad-based cost-effective treatments with the antibiotic azithromycin could restore widespread control of the disease or even overcome it altogether.

Outlook and prognosis

Thanks to well-developed and modern medical options, Frambösia has a favorable prognosis. With early diagnosis and rapid initiation of treatment, complete recovery of the patient occurs. The causative agent of the disease can be killed by administering medication. It is then removed from the body. The affected person experiences an improvement in health shortly after the start of therapy and is considered to be recovered after a few weeks of the healing process. If no damage to the bone structure has occurred, no secondary damage is to be expected as a result of Frambösie. If left untreated, the bacterium can continue to spread unhindered in the organism. The prognosis worsens in these patients. There is a gradual increase in skin lesions and swelling. In an advanced stage of the disease, complaints of the bones and joints occur. If the affected person does not take advantage of treatment even after several weeks or months, permanent impairment of the skeletal system sets in. Inflammations develop, the immune system is weakened overall, and the patient’s health gradually deteriorates.Visual changes in the face occur that are irreparable. Existing pain increases in intensity and prevents participation in the usual daily life. Psychological problems and a drastic decrease in well-being are to be expected.

Prevention

The extremely long course of untreated frambosia means that sufferers are a constant source of infection, from which people within the immediate environment – especially children – can contract the disease. As a preventive measure, which can protect a little against infection, is the observance of a minimum standard of hygiene. Minor and major injuries to the skin, which the bacteria can use as a portal of entry, are particularly susceptible to infection. The best prevention would be if the infected persons could be treated with the appropriate antibiotic penicillin or azithromycin. This would not only cure the diseased, but also protect against reinfection by eliminating the sources of infection.

Follow-up

The options for aftercare are severely limited in the case of frambosia. The focus is on medical treatment of this disease by a physician, since self-healing cannot occur. For this reason, early diagnosis with early treatment is very important to prevent further complications and avoid premature death of the affected person. In most cases, Frambösie is treated with medication, usually using antibiotics. In this case, the affected person must ensure that the antibiotics are taken correctly and regularly and follow the doctor’s instructions. Furthermore, alcohol should not be taken together with the antibiotics, as alcohol can weaken the effect of the medication. Further measures of aftercare are not necessary if the disease is cured in time by the use of medication. In this case, if the cure is complete, the life expectancy of the affected person is also not reduced. During treatment, the patient should not exert himself unnecessarily and should take care of his body. A healthy lifestyle with a balanced diet can have a positive effect on the further course of the disease.

What you can do yourself

Frambösie is very contagious. If an infection is suspected, it is necessary to see a doctor immediately. As a rule, the disease is then easily treatable with an antibiotic. Education and compliance with hygiene measures are essential and prevent further spread. There are few things that can be done by oneself to make life with this disease a little easier. A general healthy diet, exercise in the fresh air and stress management strengthen the immune system and thus the defenses, which can then contribute to better health. Unfortunately, self-healing cannot occur; on the contrary, the symptoms only worsen and the infection continues to spread. Due to the long incubation period, it is advisable to get tested already in case of suspicion and to avoid physical contact during this time. After taking the antibiotic, a quick cure is usually achieved, with no need for flanking measures. If the disease is advanced, it depends on the degree of secondary disease and the corresponding limitations to determine what would facilitate everyday life. Because infectious disease usually spreads in impoverished regions, the range of options is very limited.