Port-wine Stain: Causes, Symptoms & Treatment

A port-wine stain or nevus flammeus is a benign, congenital vascular malformation. The exact cause has not been definitively determined to date. It can also occur together with other diseases. Treatment of a port-wine stain should be started early. A port-wine stain can also be an indication of other vascular malformations. For example, the vessels of the meninges, as well as the bones, may also be affected.

What is a port-wine stain?

A port-wine stain is a malformation of the smaller skin vessels (capillaries). The malformation is benign and usually congenital. In rare cases, a port-wine stain may develop in early childhood. Port-wine stains can occur anywhere on the body. Two forms of the malformation are distinguished. The more common form is the symmetrical port-wine stain. It occurs in 30 to 50 percent of all children and in 70 to 80 percent of cases it recedes on its own during the first years of life. A special form is the so-called stork bite in the neck. The second, much rarer form is the asymmetrical port-wine stain. This affects only about 0.1 to 0.3 percent of newborns. About five percent of asymmetric port-wine stains are partial symptoms of other diseases. In these cases, there are other malformations of larger vessels or also of the bones or the fatty tissue. The asymmetric form can occur anywhere on the body and without treatment, the port-wine stain will persist for life.

Causes

The origin of a port-wine stain has not yet been comprehensively clarified. The only certainty is that the malformation is not genetically inherited, provided that it occurs as a single phenomenon and is not accompanied by other malformations. There are widespread rumors, but all of them are false. Port-wine stains are not caused by a wrong lifestyle of the mother during pregnancy, nor by special events during pregnancy or during birth. In rare cases, port-wine stains occur along with other malformations as part of a medical condition. These include Sturge-Weber syndrome, Proteus syndrome, and Klippel-Treaunay-Weber syndrome. In these cases, there is a genetic link between other malformations of various types and the port-wine stain.

Symptoms, complaints, and signs

A port-wine stain can lead to a variety of symptoms and can usually appear in different forms. In general, however, a port-wine stain always results in reddened skin, with the skin continuing to change color when pressure is applied. In some cases, pain also occurs, so that those affected are restricted in their everyday life. A port-wine stain can also significantly limit and reduce the aesthetics of the patient, so that most patients with this complaint no longer feel beautiful and also suffer from reduced self-esteem or inferiority complexes. This can also lead to psychological discomfort and depression. Furthermore, the complaint also leads to bullying in children or even teasing. Especially on the face, the port-wine stain can significantly limit the aesthetics. If the port-wine stain is located near the eyes, it can lead to cataracts and in the worst case to a complete loss of vision. Therefore, the port-wine stain must be treated in any case. Nodules can also form, which can lead to bleeding. However, the life expectancy of the affected person is not negatively affected by the complaint.

Diagnosis and course

A port-wine stain can be diagnosed based on its appearance without further examination. However, since port-wine stains can also occur together with other malformations, affected children should be carefully and thoroughly examined in this regard. If the port-wine stain is located in the facial region, both the brain waves and the eye pressure should be measured regularly, as it can also lead to glaucoma. When making a diagnosis, it is important to distinguish a port-wine stain from a hemangioma. If a port-wine stain is left untreated, tissue thickening and further vasodilatation will develop. Blackish nodules can form, which can burst open and bleed. In addition, many affected individuals often suffer severe psychological distress due to their altered appearance. Therefore, early diagnosis and treatment of a port-wine stain is absolutely advisable.

Complications

A port-wine stain can lead to various complications. These depend on the position and severity of the symptom. In most cases, the affected skin area is red in color. Likewise, the discoloration of the area changes when pressure is applied. If the port-wine stain is located on the face, it must be examined regularly. This can lead to the development of glaucoma. Dark knots can also form from the port-wine stain. These burst open and cause bleeding. In most cases, port-wine stains have a strong negative impact on the psychological situation of the affected person. Most people do not feel beautiful with the port-wine stain, which leads to decreased self-esteem. This can also lead to severe psychological problems and depression. For this reason, early treatment is necessary to avoid such consequential damage. However, a port-wine stain can be covered with the help of make-up, so that no further discomfort occurs. However, this is not a permanent solution. The port-wine stain can also be removed with the help of a laser. The removal proceeds without pain and without complications.

When should you go to the doctor?

It makes sense to see a doctor in connection with a port-wine stain from three points of view. In the first case, it is a matter of identifying possible concomitant diseases that may be associated with a port-wine stain. These include Sturge-Weber syndrome, Proteus syndrome and about two dozen other syndromes that may be associated with port-wine stain. Frequently, a genetic analysis is necessary to clarify this. Since in almost all cases the port-wine stain is conspicuous at birth, it is up to the parents to clarify a possible disease. The second case is about port-wine stains on the face. Due to the fact that they can be variable (accumulation of blood), regular eye control, for example, makes sense. After all, dilated vessels can affect the pressure in the eye and thus damage the eyes. The third aspect is an aesthetic one: depending on the location of the port-wine stain, it can lead to aesthetic and psychological impairments in the affected person. In such cases it may be advisable to go to a dermatological or cosmetic surgery practice specialized in this area. Overall, however, the nevus flammeus does not require treatment, as it is considered harmless. It itself will not limit the affected person physically or healthwise. Only possible accompanying circumstances come into question as a reason for treatment.

Treatment and therapy

There are two ways to treat a port-wine stain. The simplest method is to cover the mole with camouflage or makeup. However, this method is very time-consuming and brings only limited improvement. It is a non-invasive measure, i.e. it does not penetrate the tissue, which brings only a cosmetic improvement. Covering with camouflage is not only very time-consuming, but must also be learned through expert guidance. Alternatively, a port-wine stain can also be removed. This is done by laser therapy. The treatment is done with a flash lamp pumped dye laser, called FPDL for short. This uses short pulse lengths to cause the small vessels in the area of the port-wine stain to burst or rupture.

Outlook and prognosis

The prognosis for a small or large port-wine stain (nevus flammeus) is not particularly good. Although the port-wine stain may be harmless, it is usually a permanent disfigurement. This is due to a disorder in the prenatal formation of blood vessels. Since a port-wine stain remains on the skin for life, cosmetic concealment options are one way to deal with it, at least in the facial area. Since such port-wine stains are often extensive, bluish or dark red and somewhat raised, they are difficult to conceal inconspicuously. Therefore, the prospects of making it look less obvious are limited. The prognosis for laser treatment is also not always promising. It is true that laser treatment can reduce the extent of an extensive port-wine stain. However, it often cannot be completely removed by laser. The prognosis is more positive only for small port-wine stains. Here, too, there is a certain residual risk that laser treatment cannot counteract a recurrence of the port-wine stain. Extensive port-wine stains on the face in particular can place a heavy psychological burden on those affected.The prognosis therefore also includes the development of social avoidance behavior, depression or lack of self-esteem. If the port-wine stain is part of a Sturge-Weber or Klippel-Trenaunay syndrome, severe impairments of the skeleton or the vessels are the consequence. Here, the prognosis is particularly poor that there are serious medical abnormalities.

Prevention

So far, there are no preventive measures against the development of a port-wine stain. However, early treatment is advisable to avoid further complications. In this context, it is particularly important to avoid psychological stress that can result from an untreated port-wine stain. These can be counteracted by early removal or covering of the port-wine stain.

Aftercare

A port-wine stain cannot be prevented. Therefore, aftercare cannot aim to prevent recurrence. Rather, it is about avoiding complications and making everyday life seem worth living. This plays a role especially when laser treatment does not allow complete removal. Doctors can only try to take the psychological pressure off the patients. In psychotherapy, self-esteem can be strengthened. Remedies against depression and isolation can be discussed there. The use of make-up is also recommended. Light green color hides the dark red tone of the port-wine stain. If the mole is successfully sclerosed using laser technology or is in an inconspicuous location, there is usually no need for follow-up care at all. This is because there is no problem. In this context, however, it is important that the physician has previously differentiated the skin phenomenon from other diseases. In particular, it must be ensured that no hemangioma is present. This is a tumor. In addition, the marks rarely occur with another condition such as Sturge-Weber syndrome and Klippel-Trénaunay syndrome. It is important to have medical evidence that it is not these serious conditions.

What you can do yourself

In everyday life, there are no physical limitations due to a port-wine stain, but those affected often feel psychologically restricted, especially if the port-wine stain is on the face. The port-wine stain can become a burden once it is perceived as a stigma and self-esteem suffers as a result. The problems that accompany it can be severe and can go as far as severe depression. Social isolation and withdrawal into loneliness can be the result. However, these complications in everyday life develop insidiously, so that they can be counteracted well. It is important to strengthen the self-confidence of those affected in everyday life, to include them socially and to reduce tendencies of withdrawal or shame through encouragement, activities and support. Mindfulness and meditation techniques can contribute to self-acceptance, as can regular exercise. Port-wine stains can also be covered up in everyday life using special makeup. This, of course, is not a permanent solution, but is often perceived as a relief by those affected. The make-up techniques must first be learned, the procedure itself is time-consuming and the result is not always completely satisfactory. Depending on where a port-wine stain is located, it can also be covered up by clothing in everyday life.