In which Adenoma sebaceum there is new formation of body tissues in the face area. Numerous small knots form, mainly on the cheeks. The skin changes are benign tumors.
What is adenoma sebaceum?
Adenoma sebaceum was first reported by the scientist and English dermatologist John Pringle in 1890. For this reason the disease is also called Pringle tumor designated. It is characterized by changes in the complexion of the face.
There is no gender specific expression. Both sexes can get sick equally. The areas around the nose, cheeks and forehead are primarily affected. Reddish-brown nodules develop in these areas. These are roughly the size of the head of a pin and have a smooth, shiny appearance.
The sebaceous adenoma is a tuberous sclerosis. This is a congenital hereditary disease. It is inherited as an autosomal dominant trait. In this form of inheritance, a defective allele on one of the two chromosomes is enough to cause the disease to break out. Numerous fibroadenomas develop on the patient's face. These are small knots with a soft structure.
The benign tumors develop in the sebum glands. These are located all over the body and are multilayered piston-shaped glands. The main task of the sebum glands is to protect the skin from drying out. The sebum hyperplasia is diagnosed in the first few years of life. The noticeable skin changes rarely appear for the first time in later years of life.
causes
Adenoma sebaceum is a hereditary genetic defect. It can be passed on by one or both parents. The probability of inheritance is 50 percent. Changes in the genes Tuberous Sclerosis Gen 1 and Gen 2 are passed on from parents to children.
In addition, the disease can occur sporadically and be caused by a new mutation of the genes. The genetic changes lead to a disruption in the functioning of the proteins hamartin and tuberin. Both proteins are responsible for tumor suppression in the human body. Fully functional, they prevent a cell from developing into a tumor cell.
As a result of the abnormality of the proteins, their functional activity is disrupted. This manifests itself in the formation of a large number of tumors. Scientists are currently researching whether the proteins are involved in the regulation of growth during the development of the embryo. There are indications of this, but the thesis has not yet been scientifically proven or adequately proven.
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In sebaceous adenoma, red or brown nodules occur primarily on the cheeks and in the nasolabial folds. The nasolabial fold is located on both sides in the region between the nostrils and the corners of the mouth. The skin changes also occur on the forehead and can also be skin-colored.
The sebaceous adenoma can be unilateral or symmetrical on both halves of the face. Symptoms are usually noticed and manifest in early childhood, between three and ten years of age. With increasing age, the skin changes become more apparent, especially in post-pubertal age.
The upper layer of the skin is considered inconspicuous, as the skin changes occur in the sebum glands and not on the epidermis. Patients report an uncomfortable feeling of tension on the skin of the face and feeling impure. In individual cases, the benign tumors can also develop on the chin or in the upper neck area.
Once the area around the nose is affected, problems arise for those who wear glasses. The skin changes are usually not painful. When touched with the hand, the individual tumors can be moved easily with a little pressure without additional discomfort.
Diagnosis & course
The diagnosis of adenoma sebaceum is made in the first step through visual contact by a doctor. This is followed by a comprehensive diagnosis using various methods. As a rule, a genetic test ultimately shows the mutations in the genes.
Often the adenoma is part of an overall symptom complex. Usually it is part of the Bourneville-Pringle syndrome. This is described clinically as a triad. These include adenoma sebaceum, epilepsy and mental underdevelopment compared to the age group.
Complications
The sebaceous adenoma usually causes knots to form on the cheeks and sometimes in other regions of the face. These are mostly benign tumors. The lumps can also form in the corners of the mouth or on the forehead and will increase in number over the course of the patient's life.
Often this leads to inferiority complexes and reduced self-esteem, as the patients do not find themselves beautiful with the knots. This can also lead to social exclusion. The skin on the face is tight and may also feel unclean or itchy.
Furthermore, the adenoma sebaceum can also develop on the neck or chin. Especially people who wear glasses often suffer from adenoma sebaceum when it forms directly on the nose. This can lead to severe pain. Treatment takes the form of removing the knots.
These can usually be removed with the help of a laser or surgery. Since these are benign tumors, sebaceous adenoma does not lead to any further symptoms or complications. Scars usually do not appear and the life expectancy of the patient is not reduced.
When should you go to the doctor?
If adenoma sebaceum is suspected, a doctor should be consulted immediately. Typical warning signs for the disease are red or brown lumps on the cheeks and the corners of the mouth, as well as painful pustules on the forehead and the bridge of the nose. If one or more of these symptoms is noticed, a medical professional should clarify the cause. A visit to a doctor is particularly recommended if the skin changes occur between the ages of three and ten and persist longer than usual.
Usually, before puberty begins, the lumps tend to increase and are accompanied by an uncomfortable feeling of tension. The skin changes rarely develop into benign tumors - at the latest then a doctor must be consulted. Anyone who already suffers from another skin disease such as Bourneville-Pringle Syndrome should see a doctor immediately with the adenoma.
The same applies if there are previous illnesses such as neurodermatitis or the skin changes lead to a reduced self-esteem or inferiority complexes. In general, however, adenoma sebaceum is harmless and only needs to be clarified if there are other complaints.
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Treatment & Therapy
There is currently no therapeutic method to treat sebaceous adenoma. For this reason, the treatment is based on the cosmetic removal of facial nodules. Depending on the extent of the lumps, this is done via laser irradiation or an excision. With laser irradiation, the unwanted tissue is gradually removed in several sessions with a laser.
This takes place via thermal destruction of the diseased tissue by means of the radiation emitted by the laser. The tumor tissue is absorbed by the healthy one and falls off. With an excision, the unwanted tissue is removed in a surgical procedure. Depending on the individual extent of the nodules, the surgical procedure is performed with local anesthesia.
Depending on the severity, a skin graft may take place. In most cases, the skin heals without any problems or leaves small scars, which can be reduced in further cosmetic interventions if desired.
Outlook & forecast
In many cases, patients suffer from aesthetic complaints due to the sebaceous adenoma and feel subjectively disfigured by the neoplasms on the face. This can lead to severe depression and inferiority complexes and thereby promote social exclusion. Especially in children, these malformations can lead to teasing or bullying and thus considerably reduce the quality of life.
Sometimes pain and breathing difficulties occur if the adenoma sebaceum spreads in these areas. The skin on the face feels unclean to the patient. People who rely on glasses can experience pain because the glasses can press on the affected areas.
In most cases there are no life-threatening complications or complaints. The sebaceous adenoma can be removed with the help of a laser. In some cases, scars remain afterwards, but these can be covered with a skin graft. In severe cases, the sebaceous adenoma is removed by surgery. Here, too, the disease progresses positively and the patient's life expectancy is not affected.
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Preventive measures cannot be taken with adenoma sebaceum. As soon as a parent becomes ill with the genetic defect, the mutation is very likely passed on to the child through the dominant chain of inheritance. Only the degree of expression in the parent and the child can vary.
Aftercare
There are usually no special follow-up options available to those affected with adenoma sebaceum. The benign tumors must be removed from the skin in order to avoid further complications and complaints. In most cases, the treatment consists of a surgical procedure in which the tumors are removed from the skin.
After such an operation, the patient should rest and take care of the body. All strenuous activities or sporting activities should be avoided so that the body can rest and healing is not disturbed. Since complete treatment of adenoma sebaceum is usually not possible, the person affected depends on regular examinations.
This ensures that the lumps under the skin can be identified and treated at an early stage. In some cases, however, the patient will need a skin graft to relieve the symptoms. Since adenoma sebaceum not infrequently also leads to psychological complaints or depression, discussions with friends or with your own family are often very useful in order to alleviate these complaints. Contact with other people affected by the disease can also be useful, as this leads to an exchange of information.
You can do that yourself
The adenoma sebaceum cannot yet be treated causally. The most important self-help measure is to present the skin changes to the family doctor or a specialist and to have treatment carried out via excision or laser irradiation as soon as possible.
After such treatment, strict personal hygiene is required. The affected area must be well cared for, ideally with the care products prescribed by the doctor. A complaint diary should be kept to accompany this. Adenoma sebaceum often occurs in combination with other symptoms and complaints that need to be clarified and, if necessary, treated.
After a skin transplant, patients should rest for a few weeks. The affected area of the skin is very sensitive after an operation and must not be exposed to irritants such as dust or perfumed care products. Sun exposure should also be avoided if possible. Comprehensive treatment is necessary to avoid scarring.
If, in spite of everything, there are emotional complaints, a conversation with a therapist makes sense. Going to a self-help group may also help. Which measures are indicated in detail should always be discussed with the responsible dermatologist.
