Vascular Malformations

About vascular malformations

Vascular malformations may refer to a malformation of any vessel type (arterial, venous, lymphatic, capillary), but in dermatology it usually refers to a capillary malformation (a malformation of the smallest blood vessels).  A capillary malformation is often also called a port-wine stain or a nevus flammeus.  A capillary malformation is present at birth, often on the head or neck,  and grows proportionately with the child.  This is in distinction to a hemangioma, which has a rapid growth phase followed by involution.  Capillary malformations do not involute.  Capillary malformations tend to progress from a flat pink in infancy to red in childhood and purple in adulthood, often attaining a cobblestoned thickened texture.

An isolated capillary malformation typically does not result in any morbidity or mortality, but it can be disfiguring, especially on the face.  It is estimated to occur in 0.3-0.5% of newborns in the United States. Capillary malformation may be associated with other disorders.   Ocular (especially glaucoma) and/or CNS involvement may occur, notably for lesions that involve the V1 or V2 nerve distributions in the face.

There are a number of syndromes associated with capillary malformation, notably Sturge-Weber Syndrome (capillary malformations involving the upper face and the ipsilateral leptomeninges and  cerebral cortex), Klippel-Trenaunay Syndrome (capillary malformation, varicose veins, and hypertrophy of underlying tissues), and Cobb Syndrome (cutaneous vascular lesion overlying the spine associated with vascular malformations in the underlying spinal meninges), among several other associated syndromes.

Additional dermatological abnormalities may be present, a finding termed Phakomatosis pigmentovascularis. There are several varieties of associations, including various combinations of epidermal nevus, dermal melanocytosis, nevus anemicus, and nevus spilus.

With what can a vascular malformation be confused?

A capillary malformation may be misdiagnosed as an infantile hemangioma.

How is a vascular malformation diagnosed?

A capillary malformation is typically diagnosed by clinical exam and history.  If Sturge-Weber Syndrome or Cobb Syndrome is suspected, imaging is usually performed.

How is a vascular malformation treated?

The pulsed-dye laser is the treatment of choice for isolated capillary malformations.

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Hemangiomas

About Hemangiomas

A hemangioma is a benign proliferation of blood vessels, and in some cases supporting stroma, in the dermis. Hemangiomas are traditionally divided into two broad categories, cavernous and capillary,  thought classification schemes abound.  Capillary hemangiomas, in turn, have several subtypes: nevus flammeus, strawberry hemangioma, and cherry angioma. Cavernous hemangiomas are large, deep vessels, while the blood vessels of capillary hemangiomas are smaller and more superficially located. Hemangiomas are red, blue or purple in appearance, and soft or firm in consistency, depending on location and vessel size. They can be macular, papular or nodular. The lesions is often blanchable. They are asymptomatic unless large and obstructive, such as over an eye, where it can impede vision.

Nevus flammeus. In newborns, these common lesions occur on the eyelids and the back of the neck (“stork bite”). They are flat and pink. Nevus flammeus usually appear as only a faint pink macule at birth, which then enlarges rapidly over several weeks.

Strawberry hemangioma. This lesion protrudes from the skin surface and has a rough texture and bright red color. They can occur anywhere. They are far less common than nevus flammeus. These are more likely to present as a cosmetic concern.

Cherry angioma. Unlike the first two categories of capillary hemangiomas, the cherry angioma occurs in middle age and older individuals. They are extremely common. They are small, dome-shaped papules, red to blue in coloration and smooth in surface texture.

Cavernous hemangioma. As the name implies these uncommon lesions are composed of deep larger vessels; some also have a superficial capillary involvement. They appear as dome-shaped blue nodules.

With what can a hemangioma be confused?

The diagnosis is usually not in doubt. A cherry angioma, if dark in coloration, can sometimes be confused with a melanoma or a blue nevus. Kaposi’s sarcoma, a vascular tumor associated with AIDS, can be confused with a hemangioma.

A vascular malformation can be confused with a hemangioma.  However, vascular malformations are present at birth, do not proliferate, and do not involute.

How is a hemanioma diagnosed?

Hemangiomas are diagnosed clinically based upon appearance and history. Biopsy is typically unnecessary, unless there is suspicion of malignancy.

How is a hemangioma treated?

Childhood strawberry hemangiomas usually spontaneously involute over several years, though they may enlarge during the first two years of life. Often no treatment is necessary other than parental reassurance; however, laser treatment of enlarging hemangiomas is sometimes advocated.  Camouflage make-up can be helpful. Cavernous hemangiomas also spontaneously regress, but less often than strawberry hemangiomas. They can sometimes impinge upon structures such as the eye or pharynx, necessitating treatment. Oral steroids are often effective in arresting enlargement.  More recently, propanolol has been advocated.

What is the prognosis for hemangiomas?

Faint nevus flammeus lesions of the eyelid usually resolve within the first year of life. Strawberry hemangiomas will also spontaneously involute over several years if left untreated. Strawberry hemangiomas, due to their raised profile, are subject to ulceration and secondary infection.  Large cavernous hemangiomas can rarely give rise to the Kasabach-Merrit syndrome, in which platelets are destroyed in tortuous blood vessels. Cherry angiomas are persistent, but are amenable to laser treatment if of cosmetic concern.

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Birth Marks

About Birthmarks

Birthmarks are present at birth or arise,shortly thereafter.  They can be hyperpigmented, hypopigmented, or vascular in appearance, though other forms are possible.

Look for specific entries regarding birth marks:

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