Flat hemangioma
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Flat hemangioma (Latin: naevus flammeus), also referred to as a "port-wine stain", is a congenital vascular malformation of the skin resulting from permanent dilation of the superficial capillary vessels within the dermis. The lesion appears as a patch ranging in color from light pink to dark red or purple, usually well demarcated from the surrounding skin. Unlike infantile hemangioma, it does not exhibit a proliferation phase or spontaneous regression. With age it may darken and thicken. Flat hemangiomas most commonly occur on the face and neck, but can appear on any part of the body. In some cases they coexist with vascular syndromes that require specialist diagnostic evaluation.
Flat hemangioma – what is it?
Port-wine stain belongs to the group of low-flow vascular malformations, in which there is no uncontrolled proliferation of endothelial cells, but a permanent ectasia (dilation) of existing capillaries. On histopathological examination, dilated capillaries are observed in the upper layers of the dermis, lined by normal endothelium.
Clinical features:
- present at birth,
- no elevation above the skin surface during infancy,
- no tendency to spontaneous regression,
- gradual darkening and possible thickening in adulthood,
- no signs of inflammation.
The most common location is the area innervated by branches of the trigeminal nerve (V1–V3). Lesions involving the V1 distribution (ophthalmic branch) require exclusion of Sturge–Weber syndrome – a rare neurocutaneous disorder associated with vascular malformations of the central nervous system and the eye.
Port-wine stain should be differentiated from:
- infantile hemangioma,
- telangiectasias,
- persistent erythema,
- arteriovenous malformations.
The diagnosis is clinical, and imaging studies (Doppler ultrasound, magnetic resonance imaging) are performed when deeper vascular components are suspected.
Flat hemangioma – causes
The pathogenesis of a port-wine stain is associated with a developmental disturbance of the embryonic vascular network. In recent years a link has been shown with a somatic mutation of the GNAQ gene, leading to disturbances of intracellular signaling and a persistent dilation of capillary vessels.
The mechanism of formation includes:
- a defect in the regulation of vascular wall tone,
- disturbances of the autonomic innervation of the vessels,
- abnormal control of angiogenesis during fetal life.
The lesion is not the result of trauma, infection or environmental factors. No typical familial inheritance has been demonstrated – the mutation is mosaic in nature and arises spontaneously during embryonic development.
In rare cases a port-wine stain is a component of congenital syndromes such as:
- Sturge–Weber syndrome,
- Klippel–Trénaunay syndrome.
Therefore extensive or segmental lesions, especially those involving the face, require dermatological, neurological and ophthalmological consultation.
Flat hemangioma – will it disappear?
Flat hemangioma does not undergo spontaneous regression. Unlike infantile hemangioma, it does not enter an involution phase. On the contrary — in its natural course it may:
- darken (from pink to purple),
- increase in color intensity,
- thicken and form nodular overgrowths in adulthood,
- cause soft tissue hypertrophy in the affected area.
These changes result from chronic dilation of vessels and progressive remodeling of the dermis.
Lack of treatment does not lead to malignant transformation — a flat hemangioma is a benign lesion. However, its location, extent and visibility (especially on the face) can pose a significant aesthetic and psychosocial problem.
Early initiation of therapy, particularly in childhood, is associated with a better response to treatment and a lower risk of secondary hypertrophic changes.
Flat hemangioma – what to apply
There are no topical preparations capable of removing a port-wine stain. Ointments, creams, or herbal preparations do not affect the permanent dilation of capillary vessels.
Local management is solely supportive and includes:
- sun protection (SPF 50 sunscreens) – UV radiation can exacerbate erythema,
- soothing preparations and products that strengthen the epidermal barrier,
- in selected cases – masking dermocosmetics (medical camouflage).
It is not recommended to:
- use irritating products,
- perform chemical peels within the lesion without medical qualification,
- attempt self-treatment with alternative methods.
The only method with documented efficacy in reducing the lesion is procedural treatment aimed at selectively closing the pathologically dilated vessels.
Port-wine stain – treatment
Treatment of capillary malformation is procedural and should be carried out in centers with experience in the therapy of vascular malformations. The goal of therapy is not to "remove the lesion" in the surgical sense, but selective closure of pathologically dilated capillaries, reduction of color intensity and prevention of secondary skin thickening.
Laser therapy – the gold standard
The basis of treatment is the use of the phenomenon of selective photothermolysis, in which light energy of a specific wavelength is absorbed by oxyhemoglobin. This causes controlled damage to the vessel wall and its gradual resorption without significant injury to surrounding tissues.
Most commonly used systems:
- pulsed dye laser (PDL, 585–595 nm) – the first-line method for superficial lesions, especially in children,
- Nd:YAG laser (1064 nm) – more effective in deeper and darker lesions,
- intense pulsed light (IPL) systems – in selected, lighter forms.
Treatment requires a series of procedures (usually 4–10, sometimes more), at intervals of 4–8 weeks. Response to treatment depends on:
- patient's age (better outcomes in infancy and childhood),
- location of the lesion (best response – lateral face),
- depth and color of the lesion,
- presence of hypertrophic changes.
Early initiation of therapy reduces the risk of skin thickening and the development of nodular hypertrophy in adulthood.
Treatment of hypertrophic changes
In adults who have developed:
- skin thickening,
- formation of nodular lesions,
- soft tissue hypertrophy,
combined treatment may be necessary, including:
- laser therapy with greater penetration,
- ablative fractional lasers (to reduce hypertrophy),
- surgical management in selected cases.
The therapeutic decision should be preceded by clinical assessment and – if necessary – Doppler ultrasound examination to exclude a high-flow component.
Therapeutic expectations
It should be emphasized that:
- complete removal of the lesion is not always possible,
- the goal of treatment is significant lightening and aesthetic improvement,
- efficacy averages 50–80% reduction in color intensity after a full series of treatments.
Adverse effects are usually transient and include swelling, erythema, purpura (particularly after PDL). Permanent complications, such as scarring or pigmentary changes, are rare with properly selected parameters.
Management in children
Current guidelines recommend considering initiation of therapy in the first months of life. Infant skin is thinner and vessels are more superficial, which increases effectiveness and reduces the number of sessions required.
For lesions involving areas innervated by the ophthalmic branch of the trigeminal nerve (V1), parallel neurological and ophthalmological care is required.
Treatment of capillary malformation requires a realistic approach: it is a permanent malformation, but modern laser therapy allows, in the vast majority of cases, achievement of a clear, clinically significant improvement with a high level of safety.
