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A port-wine stain (nevus flammeus), also commonly called a firemark, is a discoloration of the human skin caused by a vascular anomaly (a capillary malformation in the skin). They are so named for their coloration, which is similar in color to port wine, a fortified red wine from Portugal.
A port-wine stain is almost always a birthmark; in rare cases it can develop in early childhood. Either way, port-wine stains ordinarily 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, particularly on the neck and upper trunk. Early stains are usually flat and pink in appearance. As the child matures, the color may deepen to a dark red or purplish color. In adulthood, thickening of the lesion or the development of small lumps may occur.
Port-wine stains may be part of a syndrome such as Sturge–Weber syndrome or Klippel–Trénaunay–Weber syndrome.
Nevus flammeus may be divided as follows:
- Nevus flammeus nuchae
- Midline nevus flammeus
Vascular tumors, often referred to as hemangiomas, are the most common tumors in infants, occurring in 1-2%. Prevalence is even higher (10%) in premature infants of very low birth weight. Vascular tumors are characterized by overgrowth of normal vessels, which show increased endothelial proliferation. It can be present at birth, but often appears within a couple of weeks after birth or during infancy. There are different kinds of vascular tumors, but the 4 most common types are: infantile hemangioma, congenital hemangioma, kaposiform hemangioendothelioma and pyogenic granuloma.
Pyogenic granuloma, also known as lobular capillary hemangioma, is a small benign vascular tumor that primarily involves the skin (88.2%) and mucous membranes. Pyogenic granuloma appears as a red macule that grows rapidly, turns into a papule and eventually becomes pedunculated, being attached to a narrow stalk. The average diameter of these lesions is 6.5 mm. Although these lesions are small, they are often complicated by bleeding, crusting and ulceration. Microscopically, pyogenic granulomas are characterized by vascular amidst granulation tissue and chronic inflammatory infiltrate.
Pyogenic granulomas are rarely congenital. It commonly develops in infants: 42.1% develops within the first 5 years of life. This vascular tumor is twice as common in males as in females and 25% of lesions seem to be associated with trauma, an underlying cutaneous condition, pregnancy, hormonal alterations and medications. Pyogenic granulomas can also arise within a capillary malformation. Of all pyogenic granulomas, 62% is distributed on the head or neck, occurring mainly on the cheek and in the oral cavity. Lesions on the face may cause visible deformity.
Numerous treament methods have been described for pyogenic granuloma. Lesions involving the reticular dermis, may be out of the reach of pulsed-dye laser, cautery or shave excision and therefore have a recurrence rate of 43.5%. Definitive management requires full-thickness skin excision. Other options are currettage or laser therapy. Furthermore, thorough currettage and cauterization are often used for small lesions and full-thickness excision for larger lesion.
A capillary hemangioma (also known as an Infantile hemangioma, Strawberry hemangioma, and Strawberry nevus) is the most common variant of hemangioma which appears as a raised, red, lumpy area of flesh anywhere on the body, though 83% occur on the head or neck area. These marks occur in about 10% of all births, and usually appear between one and four weeks after birth. It may grow rapidly, before stopping and slowly fading. Some are gone by the age of 2, about 60% by 5 years, and 90–95% by 9 years. Capillary hemangioma is a vascular anomaly.
Capillary hemangiomas occur 5 times more often in female infants than in males, and mostly in Caucasian populations. Additionally, low birthweight infants have a 26% chance of developing a hemangioma.
It is the most common tumor of orbit and periorbital areas in childhood. It may occur in the skin, subcutaneous tissues and mucous membranes of oral cavities and lips as well as in the liver, spleen and kidneys. While this birthmark may be alarming in appearance, physicians generally counsel that it be left to disappear on its own, unless it is in the way of vision or blocking the nostrils.
Clinical symptoms of CNS origin include recurrent headaches, focal neurological deficits, hemorrhagic stroke, and seizures, but CCM can also be asymptomatic. The nature and severity of the symptoms depend on the lesion's location.
Cherry angiomas are made up of clusters of capillaries at the surface of the skin, forming a small round dome ("papule") , which may be flat topped . They range in colour from bright red to purple. When they first develop, they may be only a tenth of a millimeter in diameter and almost flat, appearing as small red dots. However, they then usually grow to about one or two millimeters across, and sometimes to a centimeter or more in diameter . As they grow larger, they tend to expand in thickness, and may take on the raised and rounded shape of a dome. Multiple adjoining angiomas are said to form a "polypoid angioma". Because the blood vessels comprising an angioma are so close to the skin's surface, cherry angiomas may bleed profusely if they are injured.
One study found that the majority of capillaries in cherry hemangiomas are fenestrated because of staining for carbonic anhydrase activity.
Hyperkeratotic cutaneous capillary-venous malformation is a cutaneous condition characterized also by inherited cerebral capillary malformations.
Spider angiomas are found only in the distribution of the superior vena cava, and are thus commonly found on the face, neck, upper part of the trunk, and arms. They may also be present on the backs of the hands and fingers in young children.
A spider angioma (also known as a nevus araneus, spider nevus, vascular spider, and spider telangiectasia) is a type of telangiectasis (swollen blood vessels) found slightly beneath the skin surface, often containing a central red spot and reddish extensions which radiate outwards like a spider's web. They are common and may be benign, presenting in around 10–15% of healthy adults and young children. However, having more than three spider angiomas is likely to be abnormal and may be a sign of liver disease. It also suggests the probability of esophageal varices.
In the eye, it is known as orbital cavernous hemangioma and is found in women more frequently than men, most commonly between the ages of 20-40. This neoplasm is usually located within the muscle cone, which is lateral to the optic nerve. It is not usually treated unless the patient is symptomatic. Visual impairment happens when the optic nerve is compressed or the extraocular muscles are surrounded.
Phakomatosis pigmentovascularis is subdivided into five types:
- Type 1 PWS + epidermal nevus
- Type 2 (most common): PWS + dermal melanocytosis +/- nevus anemicus
- Type 3: PWS + nevus spilus +/- nevus anemicus
- Type 4: PWS + nevus spilus + dermal melanocytosis +/- nevus anemicus
- Type 5: CMTC (Cutis marmorata telangiectatica congenita) + dermal melanocytosis
They all can contain capillary malformation. Type 2 is the most common and can be associated with granular cell tumor. Some further subdivide each type into categories A & B; with A representing oculocutaneous involvement and subtype B representing extra oculocutaneous involvement. Others have proposed fewer subtypes but currently this rare entity is mostly taught as having five subtypes currently.
A tufted angioma (also known as an "Acquired tufted angioma," "Angioblastoma," "Angioblastoma of Nakagawa," "Hypertrophic hemangioma," "Progressive capillary hemangioma," and "Tufted hemangioma") usually develops in infancy or early childhood on the neck and upper trunk, and is an ill-defined, dull red macule with a mottled appearance, varying from 2 to 5 cm in diameter.
Central nervous system cavernous hemangioma is a cavernous hemangioma that arises in the central nervous system (CNS). It can be considered to be a variant of hemangioma, and is characterized by grossly large dilated blood vessels and large vascular channels, less well circumscribed, and more involved with deep structures, with a single layer of endothelium and an absence of neuronal tissue within the lesions. These thinly walled vessels resemble sinusoidal cavities filled with stagnant blood. Blood vessels in patients with cerebral cavernous malformations (CCM) can range from a few millimeters to several centimeters in diameter. Most lesions occur in the brain, but any organ may be involved.
Cavernous hemangiomas are the most common benign tumors of the liver. Usually one tumor exists, but multiple lesions can occur in the left or right lobe of the liver in 40% of patients. Their sizes can range from a few millimeters to 20 centimetres. Those over 5 cm are often referred to as "giant hemangiomas".
Cherry angiomas, also known as Campbell De Morgan spots or senile angiomas, are cherry red papules on the skin. They are a harmless (benign) tumor, containing an abnormal proliferation of blood vessels, and have no relationship to cancer. They are the most common kind of angioma, and increase with age, occurring in nearly all adults over 30 years.
Campbell de Morgan is the nineteenth-century British surgeon who first described them.
Sinus pericranii typically present as soft palpable masses along midline skull, which may fluctuate in size depending on body positioning. Classically, these lesions are not associated with color change of the overlying skin, such as with other vascular lesions such as hemangioma.
Phakomatosis pigmentovascularis is a rare neurocutanous condition where there is coexistence of a capillary malformation (port-wine stain) with various melanocytic lesions, including dermal melanocytosis (Mongolian spots), nevus spilus, and nevus of Ota.
DVA can be characterized by the Caput medusae sign of veins, which drains into a larger vein. The drains will either drain into a Dural venous sinuses or into a deep ependymal vein. It appears to look like a Palm tree.
A developmental venous anomaly (DVA, formerly known as venous angioma) is a congenital variant of the cerebral venous drainage. On imaging it is seen as a number of small deep parenchymal veins converging toward a larger collecting vein.
Angioma serpiginosum is characterized by minute, copper-colored to bright red angiomatous puncta that have a tendency to become papular.
The disease is present at birth, but clinical manifestations are often not seen until later in life. Patients typically experience the sudden onset of pain, numbness, or weakness in their extremities as children or young adults. These symptoms may remit or remain stable and often can be localized below a specific dermatome. Symptoms tend to worsen over time either by discrete steps or continuously. Early development of weakness may portend a more aggressive course. Less commonly, weakness or bowel and bladder dysfunction may be presenting symptoms.
The major debility from Cobb syndrome is the onset of weakness, paresis, sensory loss, and loss of bowel and bladder control. A possible complication if treatment is delayed is Foix-Alajouanine disease or subacute necrotic myelopathy due to thrombosis in the spinal angioma.
Cutaneous lesions may be distributed anywhere in the dermatome, from midline back to abdomen. Midline back lesions may be associated with spina bifida. The cutaneous lesion may be very faint and may be more pronounced when the patient performs a Valsalva maneuver which increases abdominal pressure and causes preferential filling of the cutaneous angioma. Neurological examination will reveal weakness or paralysis and numbness or decreased sensation with a sharp upper cutoff.
The appearance of pyogenic granuloma is usually a color ranging from red/pink to purple, and can be smooth or lobulated. Younger lesions are more likely to be red because of the high number of blood vessels. Older lesions begin to change into a pink color. Size commonly ranges from a few millimeters to centimeters, though smaller or larger lesions may occur. A pyogenic granuloma can be painful, especially if located in an area of the body where it is constantly disturbed. Pyogenic granulomas can grow rapidly and will often bleed profusely with little or no trauma. They may exude an oil like substance, causing the surface to be damp. This is especially true if the granuloma is located on the scalp.
Pyogenic granulomas may be seen at any age, and are more common in females than males. In pregnant women, lesions may occur in the first trimester with an increasing incidence up until the seventh month, and are often seen on the gums. Epulis granulomatosum is a variant of pyogenic granuloma that forms only on gingiva, and is often seen forming in a recent extraction socket. Pyogenic granulomas appear on the gingiva in 75% of cases, more often in the maxillary than mandibular jaw. Anterior areas are more often affected than posterior areas. It can also be found on the lips, tongue, and inner cheek. Poor oral hygiene or trauma are usually precipitating factors.
One study has suggested a correlation between pyogenic granulomas and Bartonella seropositivity. However, this association has been questioned by others. The microscopic appearance of a pyogenic granuloma consists of highly vascular granulation tissue. Inflammation is present. The lesion may have a fibrous character if it is older, and the surface may have ulcerations. Pyogenic granulomas rarely occur in the conjunctiva, cornea or connective tissue of the eye following minor local trauma. Grossly these mass lesions resemble those occurring at more common sites. The relationship of these lesion to lobular capillary hemangiomas of skin and oropharyngeal mucosa commonly referred to as pyogenic granuloma is uncertain.
Pyogenic granuloma (also known as a "eruptive hemangioma", "granulation tissue-type hemangioma", "granuloma gravidarum", "lobular capillary hemangioma", "pregnancy tumor", and "tumor of pregnancy") is a vascular lesion that occurs on both mucosa and skin, and appears as an overgrowth of tissue due to irritation, physical trauma, or hormonal factors. It is often found to involve the gums, the skin and nasal septum, and has also been found far from the head such as in the thigh.
Sinus pericranii (SP) is a rare disorder characterized by a congenital (or occasionally, acquired) epicranial venous malformation of the scalp. Sinus pericranii is an abnormal communication between the intracranial and extracranial venous drainage pathways. Treatment of this condition has mainly been recommended for aesthetic reasons and prevention of hemorrhage.