Fibromatosis is a skin disease characterized by proliferation of connective tissue. Unlike cancer, the growth is often benign. However, as generalized congenital fibromatosis, fibromatosis can lead to death.
What is fibromatosis?
People who have fibromatosis have growths in collagenous connective tissue, which are neoplastic formations. Neoplastic formations also include cancers and other forms of uncontrolled cell growth. Fibromatosis, however, does not have the same damaging potential, but in most cases is considered benign or at least only mildly malignant. However, certain forms of fibromatosis are a fundamental exception. Fibromatosis can manifest itself at different sites of the human body. Depending on the location of the growth, medicine distinguishes different forms of the disease. For example, a growth on the gums is a so-called fibromatosis gingivae.
Causes
Various causes can be considered for the development of fibromatosis. Often, various factors act together. One of the most common forms of fibromatosis is fasciitis nodularis. The proliferation of the tissue may be due to inflammation or local violence, among other factors. Due to an erroneous reaction, the affected tissue grows incorrectly, causing a bulge. The exact background is unclear in many cases.
Symptoms, complaints, and signs
The main feature of fibromatosis is the proliferation of connective tissue. Usually, the bulge smoothly merges into the surrounding tissue. Fibromatosis can occur in various forms; they differ mainly in the site of their development. The exact delineation of the various forms does not always prove easy in practice. The superficial fibromatoses can manifest in different parts of the body. Often, a person develops different superficial fibromatoses, for example, bulges on the bottom of the feet (Ledderhose’s disease) and fasciitis nodularis on the arms. Another form of fibromatosis is generalized congenital fibromatosis. This is, as the name suggests, a congenital disease. It manifests itself in countless tissue growths that form nodules. These nodules resemble hamartomas. Unlike most fibromatoses, generalized congenital fibromatosis can lead to death. Aggressive fibromatosis also forms growths of collagenous connective tissue. In aggressive fibromatosis, these growths continue to grow very slowly but steadily. In this way, the connective tissue develops into a bulge. Medicine also calls this type of growth a desmoid. In aggressive fibromatosis, they occur as extra-abdominal desmoids: they are frequently found on the trunk and less frequently on the extremities. This distinguishes them from abdominal desmoids, which can form on the abdomen. Aggressive fibromatosis often recurs and is also called highly differentiated fibrosarcoma because of its appearance.
Diagnosis
To diagnose fibromatosis, physicians first consult the external appearance of the growth. Unlike other skin phenomena, fibromatosis is not sharply demarcated from the surrounding tissue. The proliferation of tissue can often be removed with an outpatient procedure. A subsequent examination of the tissue will reveal whether it is benign or malignant. If the neoplastic formation turns out to be malignant, further testing is often needed to make sure that the growth has not yet spread and affected other organs.
Complications
Fibromatosis can cause several complications. In generalized congenital fibromatosis, severe malformations may occur in the tissues. Depending on the location and size of the growths, chronic paralysis, circulatory problems, and nerve damage may occur. In the long term, congenital fibromatosis can lead to death. If other organs are already affected, various dysfunctions may occur that progress as the tumor grows. Later, organ failure may occur. Treatment of fibromatosis also carries risks. Hair loss, diarrhea, headaches and other typical complications may occur during radiation therapy.Surgical removal of the tissue proliferation may be accompanied by temporary fatigue and depressive moods. Also typical are secondary bleeding, excessive scarring and the reappearance of growths in the connective tissue. In the case of aggressive fibromatosis, the removal of a growth may be followed by reactions of the immune system and hormonal balance, sometimes leading to sweating and palpitations. Drug therapy usually involves the prescription of antiestrogens, which are associated with various side effects. Typical of drugs such as tamoxifen are, for example, menstrual disorders, hot flashes and discharge. In addition, thrombosis, polyps, and tumors may rarely occur.
When should you see a doctor?
Since fibromatosis can lead to the death of the affected person in the worst case, it must be treated in any case. As a rule, a doctor should be consulted whenever there are growths in the tissues. These growths can occur in different parts of the body and lead to different skin complaints. However, in most cases, they are easy to recognize because they form large nodules. Should the formation of these nodules occur, a doctor must be consulted immediately. The growths rarely occur on the extremities. Diagnosis and treatment can be done by a dermatologist. The earlier fibromatosis is detected and treated, the higher the chances of a positive course of the disease. Treatment involves the removal of the nodules by surgery, so sufferers usually still require a stay in hospital. Further radiation therapy may also be necessary. After successful treatment of fibromatosis, the disease may recur, so that a doctor must then be consulted again and the disease treated.
Treatment and therapy
In many cases, close monitoring of fibromatosis is significant. This allows patients and doctors to see how the growth is behaving and whether it is spreading, for example. Doctors can surgically remove the tissue proliferation. To do this, they remove the superfluous tissue. To ensure that none of the proliferating tissue remains and spreads again, surgeons remove the affected tissue relatively generously. However, fibromatosis often grows back. Additional radiation therapy may prove useful under certain circumstances – for example, if complete removal of the tissue proliferation is not possible or was not successful for some reason. Radiation therapy is administered locally and exposes the affected skin area to high levels of radiation. Under normal circumstances, this is harmful to health because it destroys the cells. In radiotherapy, however, it is precisely this effect that is desired: ideally, the proliferating cells of the tissue die off completely, while the healthy tissue is spared as far as possible. However, just like the surgical removal of fibromatosis, radiation therapy always interferes a little with healthy tissue. If physicians have already completely removed the fibromatosis in surgery and there is a high probability that no proliferating tissue remained, additional radiation therapy is usually not necessary. Some fibromatoses turn out to be malignant and behave like other neoplastic formations that displace healthy tissue as cancer. When fibromatosis proves to be malignant, rapid action is often required – because otherwise the tissue can metastasize and affect other organs. As a result, the affected organs suffer functional impairment, which often progresses as the tumor grows and can lead to organ failure. However, such forms of fibromatosis are very rare.
Outlook and prognosis
If it is the malignant form of fibromatosis, the disease can be fatal in the worst case. There is a risk that the growths will spread throughout the body and eventually affect the organs. Aggressive fibromatosis can form recurrences years after recovery. The growths then often go unnoticed and are only discovered when the disease is already far advanced. The affected organs are limited in their function, which can lead to physical discomfort and eventually organ failure. Generalized congenital fibromatosis can lead to death if left untreated. If diagnosed and treated early, the chances of recovery are relatively good.Drug treatment is lengthy, but does not entail any health risks for the patient. If other organs are already affected at the time of treatment, radiation therapy must be performed. This always represents a considerable burden and can place a considerable physical and mental strain on the person affected. Malignant fibromatosis thus has a rather negative prognosis, while the benign form can normally be treated well. Both forms must be detected and treated promptly to avoid complications.
Prevention
The development of fibromatoses is very complex and often ambiguous. Several factors play a role in its development: local exposure to violence, genetic aspects, and inflammation are difficult to prevent. Avoidance of excessive and strong solar radiation and environmental toxins could contribute to general prevention.
Follow-up
Fibromatosis should be treated directly by a physician in the first place, since the options for aftercare are very limited in this case. In the worst case, this disease can also lead to the death of the affected person, if it is a malignant cancer, which spreads throughout the body. For this reason, regular examination of the body should always be performed, even if fibromatosis has been completely cured. In most cases, patients with this disease require surgical intervention, during which the growths are completely removed. In this case, the patient should always rest after an operation and also take care of his body. Radiation therapy or chemotherapy may also be necessary. It is not uncommon for those affected to depend on the support and help of friends and family during the treatment of fibromatosis, so that it does not lead to psychological upsets or depression. Contact with other people affected by fibromatosis can also be useful in this regard and may also make the affected person’s everyday life easier. Sometimes the disease reduces the life expectancy of the affected person.
What you can do yourself
Patients affected by fibromatosis should see a doctor regularly with the disease. This way, if the growth spreads, the appropriate measures can be taken to prevent further complications. Fibromatosis generally requires medical treatment. This is best supported by rest and bed rest. Sometimes it is possible to support the therapy with an individually tailored diet and physical exercise. In general, it is recommended to avoid too high and too strong sun radiation as well as environmental toxins. Other potentially irritating influences, for example skin care products or chlorinated water in indoor swimming pools, should also be avoided if possible. The doctor will tell the patient exactly what measures should be taken when the diagnosis is made. Patients who suffer severely from the disease should also seek out a self-help group. Talking to other sufferers can help them learn strategies that will make it easier to cope with the disease. If fibromatosis turns out to be malignant, rapid action is required. Affected individuals should have surgery or radiation therapy immediately to avoid a severe course and rule out serious complications.
