Port-Wine Stain: Causes, Symptoms, and Treatment Options

1. What Is a Port-Wine Stain?

A port-wine stain (PWS) is a type of vascular birthmark that appears as a flat, pink, red, or purple mark on the skin. It occurs when groups of tiny blood vessels (capillaries) under the skin remain dilated instead of narrowing normally.

Unlike some birthmarks that fade with age, port-wine stains usually grow and darken over time. They are present at birth and can vary in size, shape, and location.

Key facts (Cleveland Clinic, AAD):

• Occurs in about 3 out of 1,000 newborns.

• Can appear anywhere on the body, most commonly on the face, neck, arms, or legs.

• Usually harmless but may be linked to certain medical conditions in rare cases.

2. Causes of Port-Wine Stain

Port-wine stains are caused by abnormalities in the capillaries of the skin. Instead of narrowing after birth, these small blood vessels stay open (dilated). This leads to the visible discoloration.

Researchers have found that some cases are associated with mutations in the GNAQ gene, which regulates blood vessel development.

PWS are not inherited and are not caused by anything parents did during pregnancy.

3. Symptoms and Appearance

• Color: Starts as pink or red at birth, often darkening to purple or deep red with age.

• Texture: The skin is smooth in early childhood but may become thicker or bumpy over time.

• Size and Shape: Can be small spots or cover large areas of the body.

• Progression: Without treatment, port-wine stains do not go away. They may darken, thicken, or develop nodules.

In some cases, port-wine stains located on the face may affect the eye or brain. This can be linked to rare conditions such as Sturge-Weber syndrome.

4. Possible Complications

Most port-wine stains are cosmetic. However, depending on location, they can cause medical complications:

• Ocular complications: Stains near the eye may increase the risk of glaucoma.

• Neurological complications: If associated with Sturge-Weber syndrome, may lead to seizures or developmental delays.

• Skin changes: Thickening and nodularity of the affected skin over time.

• Emotional impact: Visible port-wine stains may cause psychological or social stress.

5. Diagnosis

Port-wine stains are usually diagnosed at birth by physical examination. A dermatologist may recommend additional tests if the stain is on certain areas:

• Ophthalmologic exam: If near the eye, to check for glaucoma.

• Neurological imaging (MRI/CT): If associated with seizures or suspected Sturge-Weber syndrome.

• General skin evaluation: To assess progression and treatment options.

6. Treatment Options

Although port-wine stains are permanent, several treatments can lighten or improve appearance.

6.1 Laser Therapy (First-Line Treatment)

• Pulsed Dye Laser (PDL): The gold standard treatment.

• Works by targeting blood vessels under the skin without damaging surrounding tissue.

• Multiple sessions are often required.

• Most effective in early childhood when skin is thinner.

6.2 Other Laser and Light Treatments

• Nd:YAG laser may be used for deeper or resistant lesions.

• Intense Pulsed Light (IPL) may help in select cases.

6.3 Cosmetic Options

• Camouflage makeup can cover visible marks.

• Psychological support and counseling may help with self-esteem issues.

6.4 Medical Monitoring

• Regular check-ups if the stain is near the eyes, brain, or covers large areas.

• Monitoring for complications like glaucoma.

7. Living with a Port-Wine Stain

• Early intervention: The sooner laser treatment begins, the better the results.

• Protect the skin: Use sunscreen to prevent discoloration from worsening.

• Emotional support: Support groups and counseling may be beneficial.

• Ongoing care: Children and adults with port-wine stains should have regular check-ups with dermatologists and specialists.

8. Prognosis

• Port-wine stains are benign and not life-threatening.

• With modern laser therapy, many patients see significant lightening of the stain.

• Early treatment often leads to better cosmetic results.

• Regular medical care ensures any complications are detected early.

9. Conclusion

A port-wine stain is a vascular birthmark caused by dilated capillaries under the skin. While harmless in most cases, it can sometimes be linked to eye or neurological complications.

Today, pulsed dye laser therapy is the most effective treatment, especially when started in childhood. For many patients, treatment not only improves appearance but also boosts confidence and quality of life.

With proper medical care and support, individuals with port-wine stains can lead healthy, fulfilling lives.