Chris Schach

Author Bio -

 Key points
* Condition consists of non-cancerous blotches of red or puprle skin discoloration on any part of the body, usually present at birth.
* Most common forms are the salmon patches — small flat blotches of pink/red skin with poorly defined borders — and port-wine stains — large flat patches of purple or dark red skin with well defined borders that become bumpy and unsightly over time.
* In rare cases, the vascular malformations can be associated with additional syndromes.

Capillary vascular malformations (telangiectatic naevi or nevi — sometimes referred to as flat hemangiomas) are damaged or malformed dialeted blood vessels in the skin. They are non-cancerous and appear as blotches of red or purplish skin discoloration on any part of the body. They are always present at birth, and become more obvious with time, varying in size from a small dot to occasionally involving a whole limb, and they grow in proportion to the child's growth.

The two most common capillary vascular malformations are the salmon patch (naevus simplex) and port wine stain (naevus flammeus). Salmon patches are very common, occurring in about 40% of all newborns. Salmon patches are small flat blotches of pink or red skin with poorly defined borders, commonly found at the nape of the neck (stork bites), on the forehead between the eyebrows (angel's kiss) or on the eyelids. The patches become more noticeable and intense in color when the child is crying. These patches often spontaneously disappear within the first year, with the exception of stork bites which may persist into the adult years. Port wine stains are less common than salmon patches, most commonly appearing on the face though they can occur anywhere on the body, generally appearing on one side of the body with a sharp mid-line cut-off. They are usually large flat patches of purple or dark red skin with well-defined borders. At birth, the stains are flat, but in time they become bumpy and more unsightly. The port-wine stains may fade over time, but most remain unchanged or may even deepen in color. They do not shrink by themselves or disappear spontaneously, and with facial or neck stains, can have a severe impact on the child' development. There are also two less common forms of vascular malformations: reticulate vascular naevi – port wine stains that usually spontaneously improve by fading over time — and Angiokeratoma circumscriptum — a raised warty dark red-purple patch usually found on the lower leg or foot, covered with rough scaly patches of skin.

Most capillary vscular malformations are not associated with other abnormalities but some rare syndromes may present themselves with this kind of birthmark, such as Sturge-Weber syndrome, Parkes Weber syndrome, Klippel-Trenaunay syndrome, hperkeratotic cutaneous capillary-venous malformation, and Proteus syndrome.

Differential Diagnosis (Other conditions with similar appearance)
Cobb Syndrome
Infantile Hemangioma
Klippel-Trenaunay-Weber Syndrome
Proteus syndrome
Rubinstein-Tavbi Syndrome

Key points
* Vascular malformations are usually diagnosed clinically with no further investigation needed.
* In uncertain or complicated circumstances, ultrasounds, MRIs or angiographys are also performed.

Capillary vscular malformations are usually diagnosed clinically and no investigations are necessary for the majority of patches. However, when there is uncertainty or concern, an ultrasound scan is often performed. In more complicated cases, Magnetic Resonance Imaging (MRI) or angiography can also be performed.

* Vascular malformations require no treatment.
* Cosmetic cover or laser treatment can be useful in clearing up the conditions.
* Vascular malformations may bleed uncontrollably after minor trauma.
* Extreme measures may need to be taken in extremely complicated and rare scenarios.

Salmon patches require no treatment, but treatment of port wine stains depends on the site, extent and nature of the patches. In the past, cosmetic cover was best, but now many can be successfully removed or their appearance improved by laser treatment, usually before school age. The pulsed dye laser (PDL) appears to be the treatment of choice for most port wine stains. Approximately 40-45% improve by at least 75% after 5 to 25 treatments. Patches that do not respond to PDL may improve when treated with other types of laser. Capillary vscular malformations may bleed after minor trauma and sometimes the bleeding can be difficult to stop, though this is uncommon.

In complicated scenarios, the following measures may rarely be necessary: compression stockings or bandaging to reduce swelling and protect from injury, aspirin and sometimes anticoagulants to prevent clots, resection or ligation (tying-off) of abnormal blood vessels, slerotherapy, bone shortening, and amputation.

More in this category: « Carcinoma cuniculatum Chilblains »