What are the other Names for this Condition? (Also known as/Synonyms)

• Firemark
• Naevus Flammeus
• Nevus Flammeus

What is Port-Wine Stain? (Definition/Background Information)

• Port-Wine Stain is an uncommon form of capillary vascular malformation (CVM), which is a reddish birthmark on skin caused by the malformation of small blood vessels
• It occurs in newborn children as a flat patch of reddish discoloration on skin, usually on the face. Port-Wine Stains are frequently observed at birth. In very rare cases, some may form later in childhood too. They are benign and typically persist throughout one’s life
• The cause of Port-Wine Stain is unknown, but some studies show certain genetic abnormalities and gene mutations. A small percentage of them are associated with Sturge-Weber syndrome
• These benign growths may occur in any part of the body, but most are found on the face. They do not present any significant symptoms or complications
• Generally, no treatment is necessary for Port-Wine Stains. But, if required, they are occasionally treated for cosmetic purposes and removed through laser therapy. Some are known to completely disappear into adulthood

Who gets Port-Wine Stain? (Age and Sex Distribution)

• Port-Wine Stain is almost always present at birth; although, some may rarely develop during childhood
• The incident rate of the condition is 1 in 300 newborn children
• Both males and females are affected
• It may be observed in individuals of all races and ethnic backgrounds across the world

What are the Risk Factors for Port-Wine Stain? (Predisposing Factors)

• The risk factors for Port-Wine Stain are not yet well-established
• Some are occasionally associated with certain genetic disorders that include:
  • Klippel-Trénaunay-Weber syndrome
  • Sturge-Weber syndrome
  • Parkes-Weber syndrome

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Port-Wine Stain? (Etiology)

• The exact cause of development of Port-Wine Stain is unknown. It occurs from malformed and dilated blood vessels under the skin
• Some are observed in the presence of certain genetic disorders. Mutation on the following genes have also been reported: GNAQ gene and RASA1 gene
• They occur at birth and become more prominent with time i.e., as the baby gets older
• If untreated into adulthood, the affected small vessels become dilated, and show a darker red/blue/purple coloring, become elevated, and can cause local tissues to hypertrophy

What are the Signs and Symptoms of Port-Wine Stain?

Port-Wine Stains are generally asymptomatic and do not present any significant signs and symptoms. However, the following may be observed:

• Presence of an irregular patch on skin of varied size that appears like a ‘flat hemangioma’
• Some Port-Wine Stains are small in size and confined to an area of the body skin, while others may be big and distributed over larger body surfaces
• The skin patches or moles have well-defined borders and are red, pink, or purple in color (wine-colored)
• Normally, only one half of the body is involved
• The moles grow in size and become darker in color as the child grows into an adult
• In adults, the patch may thicken to form into a lesion or lump
• Port-Wine Stains may be present at any part of the body, but the most common location is the face or head. Very rarely, these may be present inside the mouth too

How is Port-Wine Stain Diagnosed?

A diagnosis for Port-Wine Stain includes evaluating the clinical history (physical exam) and a thorough family history. Other tools to help diagnose the skin condition may include:

• A visual assessment by the physician, which can help diagnose the condition in most cases
• Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
• Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
• Imaging studies and other tests may be necessary to rule out Klippel-Trénaunay-Weber syndrome or Sturge-Weber syndrome
• If necessary, skin biopsies may be performed to rule-out other conditions

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Port-Wine Stain?

No significant complications are generally reported from Port-Wine Stains.

• In some children and adults, it may present cosmetic (appearance) issues
• Birthmarks around the face or eye may cause psychological stress in some individuals
• The hypertrophy of underlying skin and vessels can cause functional changes around the mouth, eyes, etc.

How is Port-Wine Stain Treated?

• Port-Wine Stains are generally asymptomatic. Early laser treatment in infancy and childhood can minimize the dramatic increase in vessel size and visual attention known to occur in adulthood
• Children with facial or extensive Port-Wine-Stains should be examined and managed by a practitioner with experience in this condition

How can Port-Wine Stain be Prevented?

Currently, there are no specific methods available to prevent the formation of Port-Wine Stain.

• If there is a family history of the condition, then genetic counseling will help assess risks, before planning for a child
• Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic conditions

What is the Prognosis of Port-Wine Stain? (Outcomes/Resolutions)

• Port-Wine Stains are noncancerous and generally asymptomatic
• Some are known to disappear spontaneously after a period of time. However, many persist throughout an individual’s life
• A small study showed 11-24 % of the affected individuals develop thickening or nodularity in their Port-Wine-Stain in adulthood

Additional and Relevant Useful Information for Port-Wine Stain:

Please visit our Skin Care Health Center for more physician-approved health information:

https://www.dovemed.com/healthy-living/skin-disorders/