What is an adenoma sebaceum?The adenoma sebaceum is a tuberous sclerosis. This is a congenital hereditary disease. It is inherited as an autosomal dominant. In this form of inheritance, a defective allele extends to one of the two chromosomes.
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. It is characterized by changes in the appearance of the skin on the face.
There is no gender specificity. Both sexes can become equally ill. The areas around the nose, cheeks and forehead are primarily affected. Reddish-brown nodules appear at these spots. These are about the size of a pinhead and have a smooth-looking appearance.
The adenoma sebaceum is a tuberous sclerosis. This is a congenital hereditary disease. It is inherited as an autosomal dominant. In this form of inheritance, a defective allele on one of the two chromosomes is sufficient to lead to the onset of the disease. Numerous fibroadenomas develop in the patient's face. These are small nodes with a soft structure.
The benign tumors develop in the sebaceous glands. These are found throughout the body and are multi-layered piston-shaped glands. The main task of the sebaceous glands is to protect the skin from drying out. Sebaceous gland hyperplasia is diagnosed in the first years of life. Only rarely the eye-catching skin changes occur for the first time in the higher years of life.
Adenoma sebaceum is a hereditary genetic defect. It can be shared by one or both parents. The probability of inheritance is 50 percent. The alteration of genes tuberous sclerosis gene 1 and gene 2 are inherited from the parents to the child.
In addition, the disease can occur sporadically and be caused by a re-mutation of genes. The gene changes lead to a disruption in the function 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.
Due to the anomaly of the proteins disturbances of their function occur. This manifests itself in the formation of a variety of tumors. Scientists are currently investigating whether the proteins are involved in the growth of the embryo during the development of the embryo. Evidence exists, but the thesis has not yet been scientifically proven or sufficiently documented.
Symptoms, complaints & signs
Red or brown nodules occur in the adenoma sebaceum primarily on the cheeks and in the nasolabial folds. The nasolabial fold is located bilaterally 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 adenoma sebaceum may be formed on one side or symmetrically on both sides of the face. Symptoms are most commonly found in early childhood at the age of three to ten years and manifest. With increasing age, the skin changes appear more frequently, especially in post-pubertal age.
The upper layer of the skin is considered inconspicuous, as the skin changes occur in the sebaceous glands and not on the epidermis. Patients report an uncomfortable feeling of tension on the facial skin and feel impure. In individual cases, the benign tumors can also develop on the chin or in the upper neck area.
As soon as the area around the nose is affected, problems arise for spectacle wearers. The lesions are usually not painful. When touched by hand, the individual tumors can be easily moved with a little pressure without additional discomfort.
Diagnosis & History
The diagnosis of adenoma sebaceum is made in the first step of the visual contact by a physician. Subsequently, a comprehensive diagnosis with various methods is found. As a rule, a genetic test ultimately shows the mutations of the genes.
Often, adenoma is part of a whole symptom complex. Mostly it belongs to the Bourneville-Pringle syndrome. This is clinically described with trias. These include adenoma sebaceum, epilepsy and a mental underdevelopment compared to the age group.
Adenoma sebaceum usually results in nodule formation on the cheeks and sometimes in other areas of the face. These are usually benign tumors. The nodules can also form in the corners of the mouth or on the forehead and increase their number over the life of the patient.
This often leads to inferiority complexes and reduced self-esteem, as the patients with the nodules do not find themselves beautiful. This can also lead to social exclusion. The skin on the face tenses and may also feel unclean or itching.
Furthermore, the adenoma sebaceum can also form on the neck or on the chin. Especially people who wear glasses often suffer from Adenoma sebaceum when it forms directly on the nose. This can lead to severe pain. The treatment takes the form of removal of the nodes.
These can usually be removed with the help of a laser or through surgery. Because they are benign tumors, adenoma sebaceum does not cause any further discomfort or complications. Scarring usually does not occur and the life expectancy of the patient is not reduced.
When should you go to the doctor?
In case of suspected adenoma sebaceum, a doctor should be consulted promptly. Typical signs of the disease are red or brown spots on the cheeks and over the corners of the mouth, as well as sore pustules on the forehead and on the bridge of the nose. If one or more of these symptoms is noticed, a physician must clarify the cause. A doctor's visit is especially recommended if the skin changes between the third and the tenth year of life occur and persist longer than usual.
Usually, the nodes increase before the onset of puberty and are accompanied by an unpleasant sense of tension. Rarely, the skin changes to benign tumors develop - at the latest then a doctor must be consulted. Anyone who already suffers from another skin disease, such as Bourneville-Pringle syndrome, should go to the doctor immediately with adenoma.
The same applies if previous illnesses such as atopic dermatitis are added or the skin changes lead to reduced self-esteem or inferiority complexes. In general, however, adenoma sebaceum is harmless and must be clarified only with further complaints.
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Treatment & Therapy
There is currently no therapeutic method to treat adenoma sebaceum. For this reason, the treatment is based on a cosmetic removal of the facial nodules. This happens depending on the extent of the nodes via a laser irradiation or an excision. In laser irradiation, the unwanted tissue is gradually removed in several sessions with a laser.
This is done via a thermal destruction of the diseased tissue by means of the emitted radiation through the laser. The tumor tissue is absorbed by the healthy and falls off. In an excision, the unwanted tissue is removed in a surgical procedure. Depending on the individual extent of the nodules, surgery is performed with local anesthesia.
Depending on the severity, a skin graft may occur. In most cases, the skin heals easily or leaves small scars, which can be reduced on request in other cosmetic procedures.
Outlook & Forecast
In many cases, patients suffer from aesthetic complaints due to adenoma sebaceum and feel subjectively disfigured by the neoplasms on the face. This can lead to severe depression and inferiority complexes, thereby promoting social exclusion. Especially in children, these malformations can lead to teasing or bullying and thus significantly limit the quality of life.
Some pain and difficulty in breathing also occur if adenoma sebaceum spreads in these areas. The skin on the face feels impure to the patient. People who rely on glasses can suffer from pain as the glasses can press on the affected regions.
In most cases, there are no life-threatening complications or complaints. The adenoma sebaceum can be removed with the help of a laser. In some cases scars remain after that, but they can be covered with a skin graft. In severe cases, the adenoma sebaceum is removed by surgery. Again, there is a positive course of the disease and the life expectancy of the patient is not affected.
Preventive measures can not be taken with Adenoma sebaceum. Once a parent has the genetic defect, the mutation is most likely passed on to the child through the dominant inheritance chain. Only the degree of expression of the parent and the child can vary.
Special options for aftercare are usually not available to the person concerned with the disease Adenoma sebaceum. The benign tumors must be removed from the skin to avoid further complications and discomfort. In most cases, the treatment is performed by surgery in which the tumors are removed from the skin.
The patient should rest after such an intervention and protect the body. All strenuous activities or physical activities should be avoided so that the body can rest and the healing is not disturbed. Since complete treatment of Adenoma sebaceum is usually not possible, the person is dependent on regular examinations.
This ensures that the knots under the skin can be detected and treated at an early stage. However, in some cases, the patient may need a skin graft to relieve the symptoms. Since Adenoma sebaceum not infrequently also leads to mental discomfort or depression, conversations with friends or with one's own family are often meaningful to alleviate these symptoms. In this case, the contact with other victims of the disease can be useful, since it comes to an exchange of information.
You can do that yourself
The adenoma sebaceum can not 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 a timely treatment via excision or laser irradiation.
After such treatment, strict personal hygiene applies. The affected area must be well cared for, ideally with the care products prescribed by the doctor. Accompanying this, a complaint diary should be created. Adenoma sebaceum often occurs in combination with other symptoms and complaints that need to be clarified and, if necessary, treated.
After a skin graft, patients should take some time off for a few weeks. The affected area of skin is very sensitive after surgery and should not be exposed to irritants such as dust or perfumed care products. Also solar radiation should be avoided if possible. Comprehensive treatment is necessary to prevent the formation of scars.
If despite all emotional complaints, a conversation with a therapist makes sense. Under certain circumstances, even going to a self-help group can help. Which measures are indicated in detail should always be discussed with the responsible dermatologist.