Definition
Birthmarks are areas of discolored and raised spots found on the skin. Birthmarks are groups of malformed pigment cells or blood vessels.
Description
Vascular birthmarks are benign (noncancerous) skin growths comprised of rapidly growing or poorly formed blood vessels or lymph vessels. Found at birth (congenital) or developing later in life (acquired) anywhere on the body, they range from faint spots to dark swellings covering wide areas.
Birthmarks are most often found on the head or neck but can be anywhere on the body. The common appearing birthmark is a tiny red or purple mark. A specific group of birthmarks, called "strawberry spot," "portwine stain," and "stork bite," are medically called hemangiomas. These birthmarks are essentially an overgrowth of blood vessel tissue in a specific area on the body.
Many birthmarks disappear without any special treatment, but some remain the same size or enlarge. In rare cases, the strawberry mark may cover large area of the face and body.
Demographics
About one in every three infants has a birthmark. Twice as many girls as boys have birthmarks. For appearance or cosmetic reasons, medical treatment may be necessary if the birthmark does not disappear on its own. Treatment for most birthmarks is delayed until the child is older.
About 10 in every 100 babies have vascular birthmarks. Skin angiomas, also called vascular nevi (marks), are overgrown blood vessel tissue (hemangiomas) or lymph vessel tissue (lymphangiomas) beneath the skin's surface. Hemangiomas are on the face and neck (60%), trunk (25%), or the arms and legs (15%). Congenital hemangiomas, 90 percent of which appear at birth or within the first month of life, grow quickly and disappear over time. They occur in 110 percent of full-term infants, and 25 percent of premature infants. About 65 percent are capillary hemangiomas (strawberry marks), 15 percent are cavernous (deep) hemangiomas, and the rest are mixtures.
Vascular malformations are poorly formed blood or lymph vessels that appear at birth. One type, the salmon patch (nevus simplex), is a pink mark comprised of dilated capillaries (also called a stork bite). It appears on the back of the neck in 40 percent of newborns and on the forehead and eyelids (also called an angel's kiss) in 20 percent. Stork bites appear in 70 percent of white and 60 percent of black newborns.
Fewer than 1 percent of newborns have port-wine stains (nevus flammeus), birthmarks. These vascular malformations of dilated capillaries appear in the upper and lower layers of the skin on the face, neck, arms, and legs.Nevus flammeus are often permanent; these flat pink to red marks develop into dark purple bumpy areas in later life; 85 percent appear on only one side of the body.
Causes and symptoms
As of 2004 there were no known causes for congenital skin angiomas or birthmarks. Most birthmarks do not hurt; most do not cause any health problems and do not need treatment. Birthmarks may be an inherited weakness of vessel walls.
The birthmark is discoloration of the skin that starts before or just after birth. These marks can appear to be a red rash or lesion. Birthmarks tend to be different color from the skin. They are mostly flat, but some are raised, bumpy, and hairy. Many birthmarks fade or disappear altogether during the preschool years, but some never disappear completely.
Diagnosis
Patients are treated by pediatricians, dermatologists (skin disease specialists), plastic surgeons (doctors who specialize in correcting abnormalities of the appearance), and ophthalmologists (eye disease specialists), depending on the type and severity of the birthmark.
Angiomas and vascular malformations are not difficult to diagnose. The doctor takes a medical history and performs a physical examination, including visual inspection and palpation (feeling with the hands) of the marks. The skin is examined for discoloration, scarring, bleeding, infection, or ulceration. The type, location, size, number, and severity of the marks are recorded. The doctor may empty the mark of blood by gentle pressure. Biopsies or specialized x rays or scans of the abnormal vessels and their surrounding areas may confirm the diagnosis. Patients with port-wine stains near the eye may need skull x rays, computed tomography scans, and vision and central nervous system tests. Most insurance plans pay for diagnosis and treatment of these conditions.
Types of birthmarks
There are many types of birthmarks. Certain types of raised or flat red, pink, or bluish birthmarks need close watching by a qualified medical expert as the child grows. Description of common variations in skin color and birthmarks is as follows:
- Port-wine stains: These flat, pink, red, or purple colored birthmarks are caused by a concentration of dilated tiny blood vessels call capillaries. The stains usually occur on the head, face, and neck. They may be small, or they may cover large areas of the child's body. Port-wine stains do not change color when gently pressed and do not disappear over time. They may become darker and may bleed when the child is older or as an adult. Skin-colored cosmetics will cover small port-wine stains. The most effective way of treating port-wine stains is with a special laser when the child is older.
- Stork bites or salmon patches (called angel kisses when occurring on forehead or eyelids): These small pink or red patches are often found on the baby's eyelids or forehead, between the eyes, on the upper lip, and back of the neck. The name comes from the marks on the back of the neck where, as the myth goes, a stork may have picked up the baby. This concentration of immature blood vessels is most visible when the baby is crying. Most of these fade and disappear.
- Strawberry hemangiomas: These bright or dark red, raised or swollen, bumpy areas look like a strawberry. Hemangiomas are a concentration of tiny, immature blood vessels. Most of these occur on the head. They may not appear at birth but often develop in the first two months. Strawberry hemangiomas are more common in premature babies and in girls. These birthmarks often grow in size for several months (they stop growing around the first birthday), then the birthmarks gradually begin to fade. By age five, the birthmarks fade in half the children affected, and they disappear by age nine.
- Mongolian spots: These blue or purple-colored splotches on the baby's lower back or buttocks occur on over 80 percent of African-American, Asian, and Indian babies. They also occur in dark-skinned babies of all races. The spots, a concentration of pigmented cells, usually disappear in the first four years of life.
Treatment
Treatment choices for skin angiomas and vascular malformations depend on their type, location, severity, and degree of disfigurement.
Watchful waiting
Birthmarks are regularly examined until they disappear or require treatment. This approach is appropriate for most hemangiomas, since many eventually shrink by themselves.
Complications
When birthmarks (hemangiomas) form in an area that can interfere with the baby's normal development (for example, blocking vision or causing difficulty breathing or hearing), treatment may be necessary. If the mark begins bleeding, parents should apply pressure firmly to control the bleeding. About 5 percent ofhemangiomas become ulcerated, especially if they are in an area that is under pressure or touched often.
Port-wine stains are on the forehead, sides of the face, or eyelids. They are occasionally linked with an increase of blood vessels in the brain or glaucoma. An increase in pressure in the eye can lead to blindness if not treated. Port-wine stains on the legs or arm may be linked to an overgrowth of that extremity.
Other complications, including congestive heart failure from large lesions, Kippel-Trenaunay-Weber syndrome, and Kasabach-Merrit syndrome, are rare.
Drugs
CORTICOSTEROIDS Parents may consider treatment for hemangiomas that do not shrink or fade by the time the child is four years old. The treatments include the use of high doses of steroids (either orally or injected into the lesion) to stop the growth. Steroids prevent the marks from growing; they do not make the birthmarks smaller. Other treatments include interferon alpha, laser therapy, and surgical removal.
INTERFERON ALPHA-2A This drug reduces cell growth in vascular marks that affect vision and that are unresponsive to corticosteroids. Given in daily injections under the skin, the response rate is 50 percent after seven months. Side effects include fever , chills, muscle and joint pain , vision disorders, low white and red blood cell counts, fatigue, elevated liver enzymes, nausea , blood clotting problems, and nerve damage.
ANTIBIOTICS Oral or topical (applied to the skin) antibiotics are prescribed for infected marks.
Surgery
Birthmarks may be removed by laser surgery. Lasers create intensive heat that destroys abnormal blood vessels beneath the skin, without damaging normal skin. Laser surgery is not usually painful but can be uncomfortable. Children are usually sedated or anesthetized. Healing occurs within two weeks. Side effects include bruising, skin discoloration, swelling, crusting, and minor bleeding.
In some cases, the birthmark can be surgically excised, or removed. Under local or general anesthesia, the skin is cut and vascular marks or their scars are removed. The cut is repaired with stitches or skin clips.
Cryosurgery is another technique used to remove small birthmarks. Vascular marks can be frozen with a substance that is sprayed onto the skin. Wounds heal with minimal scarring.
Birthmarks can also be treated by electrodesiccation. In this procedure, affected vessels are destroyed with the current from an electric needle.
Other treatments
Other treatments include the following:
- Sclerotherapy: Injection of a special solution causes blood clotting and shrinkage with little scarring. Side effects include stinging, swelling, bruising, scarring, muscle cramping, and allergic reactions. This treatment is used most commonly for spider angiomas.
- Embolization: A special material is injected into the vessel blocks blood flow, which helps control blood loss from a bleeding birthmark or reduces the size of inoperable growths. A serious side effect, stroke , can occur if a major blood vessel becomes blocked.
- Make-up: Special brands designed to cover birthmarks are sold. Two of these are Covermark and Derma blend.
- Cleaning and compression: Bleeding marks are cleaned with soap and water or hydrogen peroxide, and compressed with a sterile bandage for five to 10 minutes.
Prognosis
Many birthmarks fade or disappear before the child is school age. Some may never go away. Most of them are benign and do not need treatment. Babies with birthmarks are examined and diagnosed by the doctor. Those birthmarks that cause complications in normal childhood growth and development may require medical and surgical treatment.
The various types of birthmarks have different prognoses:
KEY TERMS
Angioma A tumor (such as a hemangioma or lymphangioma) that mainly consists of blood vessels or lymphatic vessels.
Benign In medical usage, benign is the opposite of malignant. It describes an abnormal growth that is stable, treatable, and generally not life-threatening.
Capillaries The tiniest blood vessels with the smallest diameter. These vessels receive blood from the arterioles and deliver blood to the venules. In the lungs, capillaries are located next to the alveoli so that they can pick up oxygen from inhaled air.
Corticosteroids A group of hormones produced naturally by the adrenal gland or manufactured synthetically. They are often used to treat inflammation. Examples include cortisone and prednisone.
Cutaneous Pertaining to the skin
Cutaneous angiolipomas Benign growths consisting of fat cells and blood vessels just underneath the skin.
Hemangioma A benign skin tumor composed of abnormal blood vessels.
Hereditary Something which is inherited, that is passed down from parents to offspring. In biology and medicine, the word pertains to inherited genetic characteristics.
Incidence The rate of development of a disease in a given population over time.
Kasabach-Merrit syndrome A combination of rapidly enlarging hemangioma and thrombocytopenia; it is usually clinically evident during early infancy, but occasionally the onset is later. The hemangiomas are large and may increase in size rapidly and may cause severe anemia in infants.
Lymphangioma A benign skin tumor composed of abnormal lymph vessels.
Lymphatic vessels Part of the lymphatic system, these vessels connect lymph capillaries with the lymph nodes. They carry lymph, a thin, watery fluid resembling blood plasma and containing white blood cells. Also called lymphatic channels.
Nevus Any pigmented blemish of the skin present at birth, including moles and various types of birthmarks.
Seizure A sudden attack, spasm, or convulsion.
Subcutaneous Referring to the area beneath the skin.
Syndrome A group of signs and symptoms that collectively characterize a disease or disorder.
Ulcer A site of damage to the skin or mucous membrane that is characterized by the formation of pus, death of tissue, and is frequently accompanied by an inflammatory reaction.
Vascular malformation Abnormally formed blood or lymph vessels.
- Capillary hemangiomas: Fewer than 10 percent need treatment. Without treatment, 50 percent disappear by age five; 70 percent by age seven; and 90 percent by age nine. No skin changes are found in half, while others have some discoloration, scarring, or wrinkling. From 30 to 90 percent respond to oral corticosteroids, and 45 percent respond to injected corticosteroids; 50 percent respond to interferon Alpha-2a. About 60 percent improve after laser surgery.
- Cavernous hemangiomas: Many do not disappear and are complicated by ulceration or infection. About 75 percent respond to laser surgery but have scarring.
- Spider angiomas: These fade in children but may recur. About 90 percent respond to sclerotherapy, electrodesiccation, or laser therapy.
- Cherry angiomas: These are easily removed by electrodesiccation.
- Lymphangiomas: These marks require surgery.
- Salmon patches on eyelid marks: These marks disappear by six to 12 months of age, and forehead marks fade by age six; however, 50 percent of stork bites on the neck persist into adulthood.
- Port-wine stains (flat birthmarks): These marks are easily covered with make-up. Treatment during infancy or childhood improves results. About 95 percent of the stains respond to FPDL surgery with minimal scarring; 25 percent will disappear, and70 percent will partially disappear. For unknown reasons, 5 percent show no improvement.
Prevention
Birthmarks are congenital hemangiomas or vascular malformations and cannot be prevented.
Parental concerns
Though no treatment is needed in many of these cases, a child with a hemangiomas should be watched carefully by a doctor skilled in pediatric skin disorders. The hardest part for parents is to wait until the birthmarks begin to fade on their own or, in the case of a birthmark that does not fade, waiting until the child is old enough for surgical management.
When to call the doctor
Parents should report any birthmarks they notice to the child's pediatrician. They should call the pediatrician if they notice bleeding from the birthmark, if a sore develops on the birthmark, if the mark is growing larger.
Resources
BOOKS
Birthmarks: A Medical Dictionary, Bibliography, and Annotated Research Guide to Internet References. San Diego, CA: Icon Group International, 2004.
Thompson, June. Spots, Birthmarks, and Rashes: The Complete Guide to Caring for Your Child's Skin. Westport, CT: Firefly Books, 2003.
WEB SITES
"Birthmarks: Red." MedlinePlus, April 17, 2003. Available online at <www.nlm.nih.gov/medlineplua/ency/article/001440.htm> (accessed December 12, 2004).
Aliene S. Linwood, RN, DPA, FACHE
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