Definition
A port-wine stain, also called naevus flammeus, is a benign change of the skin, which takes on a dark red to reddish-purple color. The fire stain also owes its appearance to the widespread name “port wine stain”. This congenital malformation of the smallest vessels, so-called capillaries, is one of the rare malformations and can occur alone or as a partial symptom of a superordinate disease. Important examples of such diseases are the Sturge-Weber syndrome or the Klippel-Trénaunay syndrome.
Causes
port-wine stains are caused by a malformation of the finest blood vessels, which are called capillaries. These blood vessels are dilated and lead to the typical reddish-violet appearance of port-wine stain. It is therefore not, as is often suspected, a pigment disorder.
The port-wine stain is usually congenital or develops immediately after birth. Why some people are affected by a port-wine stain and others are not, is not conclusively clarified. However, there are genetic causes and peculiarities in the DNA of many people with a port-wine stain.
However, these variants have no disease value and are not worrying. Rarely is a port-wine stain a part of a symptom complex, which is based on a disease. Also in this case genetic changes are detectable.
One such disease is the Sturge Weber syndrome, the cause of which is largely unclear. Often spontaneous mutations in the DNA of the affected persons are assumed, rarely familial variants of the disease are also known. Another cause of port-wine stain is Klippel-Trénaunay syndrome.
This congenital disease is associated with multiple vascular malformations and growth disorders. Klippel-Trénaunay syndrome is also caused by DNA mutations. There is a widespread misconception that mothers can influence the appearance of port-wine stains in their children through their behavior.
This is not the case. Neither the wearing of tight clothes, nor the taking of medication or forceps delivery lead to birthmarks in the child. This misconception leads to unfounded feelings of guilt in many mothers.
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