Trichoepithelioma is a tumor found on the skin. The disease is also synonymously called hair follicle nodules or epithelioma adenoides cysticum Brooke. Basically, the trichoepithelioma represents a benign tumor of the interdermal layers. The trichoepithelioma arises from the follicles of the hair.
What is trichoepithelioma?
Trichoepithelioma is a benign tumor found on the skin that is either sporadic or hereditary. In the sporadic form of trichoepithelioma, individual, isolated skin tumors develop, which are also known as solitary trichoepithelioma. The hereditary form of trichoepithelioma occurs in numerous cases in the families affected. For campomelic dysplasia basics, please visit theinternetfaqs.com.
There is an autosomal dominant inheritance of trichoepitheliomas. The trichoepithelioma develops in the hereditary variant in the form of multiple skin tumors or so-called multiple trichoepithelioma. This results in numerous trichoepitheliomas, which are distributed over the entire skin surface. The trichoepithelioma is always located inside the dermis and arises from cell types that belong to the hair follicles.
In the majority of cases, sporadic trichoepithelioma manifests itself particularly in adolescents and young adults. On the other hand, hereditary trichoepitheliomas often appear in patients as children or during the pubertal phase. In addition, trichoepithelioma occurs more frequently in females than in males. The exact frequency of trichoepitheliomas is not known.
Causes
There is currently still a need for research regarding the triggering factors of sporadic trichoepithelioma. The circumstances that favor the development of this form of skin tumors have not yet been clarified. The causes of the hereditary variant of trichoepitheliomas are much better researched.
Genetic mutations are present here that affect certain genes for tumor suppression. The corresponding genes are located on the 9th chromosome. Patients with genetic mutations of this type also develop other skin tumors with above-average frequency. Sometimes there is a connection between trichoepithelioma and Brooke-Spiegler syndrome. This often results in superfluous structures that are attached to the skin, such as spiradenomas and dermal cylindromas.
Symptoms, Ailments & Signs
The sporadic and the hereditary trichoepithelioma both appear more frequently on the forehead, the upper lip, the nose and nasolabial folds and the scalp. The tumors of the skin are therefore primarily concentrated in the area of the face. In some cases, the neck is also affected by the trichoepitheliomas.
A trichoepithelioma is similar in shape to a papule, which is rounded in shape and is between two and eight millimeters in size. The coloring hardly deviates from the natural color of the skin. In histological examinations, the trichoepitheliomas are recognizable as complexes of cells.
These are relatively strictly separated from the environment, have an approximately symmetrical shape and only occur in the upper layers of the skin. The papules lie in a stroma, sometimes with papillae of hair or follicles. In principle, no necrosis develops in the context of trichoepitheliomas.
Diagnosis & course of disease
Patients with the papules typical of trichoepithelioma visit a dermatologist who, after an initial patient consultation, first takes a family history. This enables the doctor to classify skin tumors into sporadic or hereditary types. The characteristic appearance of trichoepithelioma indicates the disease.
A reliable diagnosis of the trichoepithelioma is usually only possible through histological examinations, so that the trichoepithelioma must first be removed. It is also important to carry out a thorough differential diagnosis in trichoepithelioma.
Trichoepithelioma is sometimes confused with dermal nevi or angiofibromas that occur in Rombo syndrome or Gorlin-Goltz syndrome. In addition, the doctor excludes a trichofolliculoma, [tuberous sclerosis|tuberous sclerosis]], a microcystic adnexal carcinoma and basaloid follicular hemartomas.
Complications
Since trichoepithelioma is a tumour, in the worst case it can lead to the death of the affected person. As a rule, this case only occurs if the tumor is not treated and metastases have already formed. Those affected suffer from papules, which can form on different parts of the body.
Especially on the face and other visible areas, these papules are very uncomfortable and can lead to inferiority complexes or a significantly reduced self-esteem in those affected. Most patients are ashamed of the symptoms and feel uncomfortable with them. Necrosis can also develop.
Since trichoepithelioma does not heal itself, those affected are dependent on medical treatment for this disease in any case. Trichoepithelioma is usually treated relatively quickly and without complications. The papules can be removed surgically or with the help of a laser.
Furthermore, however, the patients are dependent on regular examinations in order to prevent a recurrence of the trichoepithelioma. With early diagnosis and successful treatment, the life expectancy of the patient with this disease is not negatively affected.
When should you go to the doctor?
Trichoepithelioma should always be examined and treated by a doctor. Since this disease does not heal itself and the symptoms usually worsen if no treatment is initiated, a doctor should be consulted as soon as the first symptoms and signs of the disease appear. The earlier the treatment begins, the better the further course of the trichoepithelioma. A doctor should be contacted if there are various complaints on the skin.
Pustules or papules can form, which affect the aesthetics of the affected person. The symptoms usually appear on the forehead and other regions of the face. Necrosis can also indicate a trichoepithelioma and should always be examined by a doctor. A trichoepithelioma is usually treated by a dermatologist. In most cases, this does not lead to a reduced life expectancy, although regular examinations by a doctor are useful. In some cases, however, this disease can also lead to psychological upsets or depression, so that a visit to a psychologist may also be necessary.
Treatment & Therapy
Treatment options for sporadic and hereditary trichoepithelioma are relatively limited. They are currently mainly limited to the removal of skin tumors as part of surgical interventions. Problems arise above all with multiple trichoepitheliomas, which are distributed in large numbers over the entire body surface or the main locations.
In these cases, a skin tissue transplant may be used to minimize the cosmetic impact on the patient. In addition, removal of the trichoepitheliomas by means of laser beams can sometimes be considered. In addition, some trichoepitheliomas are removed by superficial ablation or dermabrasion.
However, the trichoepitheliomas often form again after a few months or years. In principle, the prognosis of trichoepitheliomas is comparatively good. Benign tumors grow very slowly. If the skin tumors are not completely removed, recurrences may occur. Malignant degeneration of the trichoepitheliomas or the development of carcinomas are rather rare. For example, mixed epithelial-mesenchymal tumors sometimes develop.
Prevention
Prevention of trichoepitheliomas is currently not possible. The hereditary trichoepitheliomas are fixed by genetic mutations, so that there is no possibility of influence. It may be possible to prevent the formation of sporadic trichoepitheliomas by minimizing the relevant risk factors.
The problem, however, is that the causes of this form of the disease are not known, so that no preventive measures can be derived. Thanks to modern therapeutic measures, the treatment of benign tumors is usually relatively successful these days.
Aftercare
Those affected should avoid any risks that could cause a tumor. This includes the mandatory refraining from the consumption of alcohol, drugs or nicotine. Likewise, the diet should be adapted to the disease immediately after diagnosis. Excessive sugar and fat should be avoided. Sick people should urgently maintain contact with their social environment.
In this way, the help and support of family and relatives can be taken advantage of at any time. In addition, those affected should pursue any leisure activities that they did before the disease. This significantly increases the quality of life. However, those affected should only undertake these activities in the presence of family or friends.
Joining a support group can also be helpful. There, those affected can learn how others live and deal with the disease and get advice and help for themselves. They can also talk to other sick people there and do not feel alone. Perhaps they can learn new ways of dealing with the disease from other sufferers.
It also makes sense to undergo permanent psychological counseling. Psychologists can also teach sufferers how to deal with the disease. Sick people should have a balanced everyday life and allow themselves a lot of rest. This is very important and affects life expectancy.
You can do that yourself
Those affected can only rule out general risk factors that increase the likelihood of tumor formation. These include avoiding nicotine, alcohol and a diet high in fat and sugar. However, the success of these measures has not yet been scientifically proven. It can also be the case that genetic causes rule out an influence from the outset.
At least psychological consequences can be discussed in an understanding environment. Contact with other cancer patients also helps some patients. Without medical treatment, the disease progresses. Anyone who only relies on their own measures and alternative therapies risks losing many years of life.
After therapy, patients usually carry on with their previous lives. However, new formations can arise. Adherence to agreed follow-up appointments is essential for continued quality of life. It helps many sufferers to set achievable goals. Since most patients are still young at the time of diagnosis, they have plenty of time to realize their private and professional ideas. A positive awareness reduces the susceptibility to mental illness. Physical exercise in particular awakens the spirits and ensures a balanced and rested everyday life.
