Port-wine stain
Encyclopedia
A port-wine stain or naevus flammeus is a vascular anomaly
consisting of superficial and deep dilated capillaries
in the skin
which produce a reddish to purplish discoloration of the skin. They are so called for their colour, resembling that of port wine
. It is part of the family of disorders known as vascular malformations, specifically an arteriovenous malformation.
The two terms are not always equated. Sometimes the term "naevus flammeus" is divided into two categories: port-wine stain and salmon patch.
Port-wine stains are present at birth and persist throughout life. The area of skin affected grows in proportion to general growth. Port-wine stains occur most often on the face
but can appear anywhere on the body. Early stains are usually flat and pink in appearance. As the child matures, the color may deepen to a dark red or purplish colour. In adulthood, thickening of the lesion or the development of small lumps may occur.
PWS may be one of a group of symptoms and signs, in which case it is considered to be part of a syndrome
such as Sturge-Weber syndrome
or Klippel-Trenaunay-Weber syndrome
.
may be needed to confirm the diagnosis. Depending on the location of the birthmark and other associated symptoms, a physician may choose to order a measurement of intraocular pressure or X-ray
of the skull
.
, radiation
, and tattooing
; port-wine stains can also be covered with cosmetics
. Lasers have made the biggest impact on treatment, because they are the sole method of destroying the cutaneous capillaries without significant damage to the overlying skin.
The flashlamp pumped dye laser
, a yellow light laser, has been the most successful at destroying stains in infants and young children.
The neodymium YAG laser
is used to treat the nodules that may develop in some adult port-wine stains.
Treatment of infants with the flashlamp pumped dye laser generally produces marked improvement in appearance.
However, complete disappearance is rare. In approximately 20% of cases there may be no improvement at all. Stains on the face respond better than those on the trunk or limbs. Older stains may be more difficult to treat.
. These complications are usually seen later in life. If the PWS is on the face or other highly visible part of the body, the presence of PWS can also cause emotional and social problems for the affected person because of their cosmetic appearance.
Vascular anomaly
A vascular anomaly is a kind of birthmark caused by a disorder of the vascular development, although it is not always present at birth. A vascular anomaly is a localized defect in blood vessels that can affect each part of the vasculature...
consisting of superficial and deep dilated capillaries
Capillary
Capillaries are the smallest of a body's blood vessels and are parts of the microcirculation. They are only 1 cell thick. These microvessels, measuring 5-10 μm in diameter, connect arterioles and venules, and enable the exchange of water, oxygen, carbon dioxide, and many other nutrient and waste...
in the skin
Skin
-Dermis:The dermis is the layer of skin beneath the epidermis that consists of connective tissue and cushions the body from stress and strain. The dermis is tightly connected to the epidermis by a basement membrane. It also harbors many Mechanoreceptors that provide the sense of touch and heat...
which produce a reddish to purplish discoloration of the skin. They are so called for their colour, resembling that of port wine
Port wine
Port wine is a Portuguese fortified wine produced exclusively in the Douro Valley in the northern provinces of Portugal. It is typically a sweet, red wine, often served as a dessert wine, and comes in dry, semi-dry, and white varieties...
. It is part of the family of disorders known as vascular malformations, specifically an arteriovenous malformation.
The two terms are not always equated. Sometimes the term "naevus flammeus" is divided into two categories: port-wine stain and salmon patch.
Port-wine stains are present at birth and persist throughout life. The area of skin affected grows in proportion to general growth. Port-wine stains occur most often on the face
Face
The face is a central sense organ complex, for those animals that have one, normally on the ventral surface of the head, and can, depending on the definition in the human case, include the hair, forehead, eyebrow, eyelashes, eyes, nose, ears, cheeks, mouth, lips, philtrum, temple, teeth, skin, and...
but can appear anywhere on the body. Early stains are usually flat and pink in appearance. As the child matures, the color may deepen to a dark red or purplish colour. In adulthood, thickening of the lesion or the development of small lumps may occur.
PWS may be one of a group of symptoms and signs, in which case it is considered to be part of a syndrome
Syndrome
In medicine and psychology, a syndrome is the association of several clinically recognizable features, signs , symptoms , phenomena or characteristics that often occur together, so that the presence of one or more features alerts the physician to the possible presence of the others...
such as Sturge-Weber syndrome
Sturge-Weber syndrome
Sturge–Weber syndrome, sometimes referred to as encephalotrigeminal angiomatosis, is a rare congenital neurological and skin disorder. It is one of the phakomatoses and is often associated with port-wine stains of the face, glaucoma, seizures, mental retardation, and ipsilateral leptomeningeal...
or Klippel-Trenaunay-Weber syndrome
Klippel-Trénaunay-Weber syndrome
Klippel-Trénaunay-Weber syndrome, often simply Klippel-Trénaunay syndrome and sometimes angioosteohypertrophy syndrome and hemangiectatic hypertrophy, is a rare congenital medical condition in which blood vessels and/or lymph vessels fail to form properly...
.
Types
Nevus flammeus may be divided as follows:- Nevus flammeus nuchaeNevus flammeus nuchaeNevus flammeus nuchae, also known as a stork bite, is a congenital capillary malformation present in 25%-50% of newborns. It is a common type of birthmark in a newborn and is usually temporary.-Causes:...
- Midline nevus flammeusMidline nevus flammeusMidline nevus flammeus is a vascular birthmark which may be found on the glabellar region or on one upper eyelid, and presents in approximately 15% of newborns....
Diagnosis
A physician can usually diagnose a port wine stain based entirely upon the history and appearance. In unusual cases, a skin biopsyBiopsy
A biopsy is a medical test involving sampling of cells or tissues for examination. It is the medical removal of tissue from a living subject to determine the presence or extent of a disease. The tissue is generally examined under a microscope by a pathologist, and can also be analyzed chemically...
may be needed to confirm the diagnosis. Depending on the location of the birthmark and other associated symptoms, a physician may choose to order a measurement of intraocular pressure or X-ray
X-ray
X-radiation is a form of electromagnetic radiation. X-rays have a wavelength in the range of 0.01 to 10 nanometers, corresponding to frequencies in the range 30 petahertz to 30 exahertz and energies in the range 120 eV to 120 keV. They are shorter in wavelength than UV rays and longer than gamma...
of the skull
Human skull
The human skull is a bony structure, skeleton, that is in the human head and which supports the structures of the face and forms a cavity for the brain.In humans, the adult skull is normally made up of 22 bones...
.
Treatment
Many treatments have been tried for port-wine stains including freezing, surgerySurgery
Surgery is an ancient medical specialty that uses operative manual and instrumental techniques on a patient to investigate and/or treat a pathological condition such as disease or injury, or to help improve bodily function or appearance.An act of performing surgery may be called a surgical...
, radiation
Radiation therapy
Radiation therapy , radiation oncology, or radiotherapy , sometimes abbreviated to XRT or DXT, is the medical use of ionizing radiation, generally as part of cancer treatment to control malignant cells.Radiation therapy is commonly applied to the cancerous tumor because of its ability to control...
, and tattooing
Tattoo
A tattoo is made by inserting indelible ink into the dermis layer of the skin to change the pigment. Tattoos on humans are a type of body modification, and tattoos on other animals are most commonly used for identification purposes...
; port-wine stains can also be covered with cosmetics
Cosmetics
Cosmetics are substances used to enhance the appearance or odor of the human body. Cosmetics include skin-care creams, lotions, powders, perfumes, lipsticks, fingernail and toe nail polish, eye and facial makeup, towelettes, permanent waves, colored contact lenses, hair colors, hair sprays and...
. Lasers have made the biggest impact on treatment, because they are the sole method of destroying the cutaneous capillaries without significant damage to the overlying skin.
The flashlamp pumped dye laser
Dye laser
A dye laser is a laser which uses an organic dye as the lasing medium, usually as a liquid solution. Compared to gases and most solid state lasing media, a dye can usually be used for a much wider range of wavelengths. The wide bandwidth makes them particularly suitable for tunable lasers and...
, a yellow light laser, has been the most successful at destroying stains in infants and young children.
The neodymium YAG laser
Nd:YAG laser
Nd:YAG is a crystal that is used as a lasing medium for solid-state lasers. The dopant, triply ionized neodymium, typically replaces yttrium in the crystal structure of the yttrium aluminium garnet , since they are of similar size...
is used to treat the nodules that may develop in some adult port-wine stains.
Treatment of infants with the flashlamp pumped dye laser generally produces marked improvement in appearance.
However, complete disappearance is rare. In approximately 20% of cases there may be no improvement at all. Stains on the face respond better than those on the trunk or limbs. Older stains may be more difficult to treat.
Prognosis
In the absence of successful treatment, hypertrophy (increased tissue mass) of the stains may produce deformity, loss of function (especially near the eye or mouth), bleeding, and increasing disfigurementDisfigurement
Disfigurement is the state of having one's appearance deeply and persistently harmed medically, as from a disease, birth defect, or wound.Disfigurement, whether caused by a benign or malignant condition, often leads to severe psychosocial problems such as negative body image; depression;...
. These complications are usually seen later in life. If the PWS is on the face or other highly visible part of the body, the presence of PWS can also cause emotional and social problems for the affected person because of their cosmetic appearance.
External links
- Information about Port Wine Stains from Children's Hospital, Seattle.
- Birthmarks.com
- Sturge-Weber Syndrome Community