Nevus Flammeus

Key Points
Birthmark caused by capillary malformation
Red dilitation of blood vessels often on eyelid, face, or nape of neck
The facial patches (angel's kiss) tends to fade over the first year of life

Capillary vascular malformation is a birthmark caused by a malformation of capillaries, which become dilated, causing the visible appearance of the birthmark. The two most common types of capillary vascular malformation are salmon patches and port wine stains. Salmon patches are the most common, consisting of a light pink patch, usually found on the face or back of the neck. Salmon patches are often a temporary dilation of blood vessels and disappear within the first year. Those which form on the nape of the neck are more persistent, and in some cases remain for the life of the affect child. Port wine stains are much rarer, appearing in less than 1% of infants, and are a true malformation of the blood vessels in the affected area. Consisting of dark red patches with well-defined borders most often on the face, they initially are flat but over time can change in texture. Port wine stains often darken with age, and most persist throughout the life of the infected person. While very uncommon, bleeding which is difficult to stop may occur with minor trauma. Capillary vascular malformations of both types vary widely in size, and can appear anywhere on the body.

There is no link between genetics and capillary vascular malformation, and their cause and any determination of who is predisposed to be affected by them is unknown. In rare cases, the formation of these birthmarks can be associated with several rare syndromes which affect brain and body development. These disorders include Sturge-Weber syndrome, Parkes Weber syndrome, Klippel-Trénaunay syndrome, hyperkeratotic cutaneous capillary-venous malformation, and Proteus syndrome

Differential Diagnosis (Other conditions with similar appearance)
Haemangioma

Diagnosis
Key Points
Diagnosis based on skin appearance
Skin biopsy or ultrasound may be performed to confirm diagnosis
Other testing may be performed to ensure appearance is not due to associated conditions

Capillary vascular malformation is generally diagnosed based on appearance. Health care professionals may perform a skin biopsy or ultrasound to confirm diagnosis. Additionally, if the condition is suspected to be associated with the rare developmental syndromes listed above, additional testing may be necessary.

Treatment
Salmon patches do not require treatment
Port wine stain treatment depends on location and severity, and are often treated with laser therapy
*If an associated syndrome is present, other treatment options will be assigned accordingly

Salmon patches are usually temporary dilations, and therefore no treatment is required. In the case of port wine stains, treatment may be requested based on the location of the birthmark. In cases where the birthmark is complex in formation or location, an MRI may be performed to assist in planning treatment. The most effective treatment is therapy with a pulsed dye laser, which can improve the birthmark by 75% in approximately half of all affected persons. If a lesion does not respond to the pulsed dye laser, and may be treated with other laser therapies. If an associated syndrome is detected, health care professionals will plan treatment accordingly.