The three main features are nevus flammeus (port-wine stain), venous and lymphatic malformations, and soft-tissue hypertrophy of the affected limb. (wikipedia.org)
[ 1 ] Noninvoluting capillary hemangiomas are termed port-wine stains or nevus flammeus. (medscape.com)
Phakomatosis pigmentovascularis represents the association of widespread, aberrant, and persistent nevus flammeus and pigmentary abnormalities. (pediatricneurosciences.com)
SWS also known as encephalotrigeminal angiomatosis includes naevus flammeus, also known as port-wine stain (PWS), and ipsilateral leptomeningeal angiomatosis as the main features [ 6 ]. (hindawi.com)
Capillary1
An improperly developed lymph system In some cases, port-wine stains (capillary port wine type) may be absent. (wikipedia.org)
Vascular2
In 1879, Sturge reported on a case with bilateral facial nevus, vascular deformity, and congenital glaucoma in the right eye and spasms affecting the patient's left side of the body [ 9 ]. (hindawi.com)
The pathogenesis of the port-wine stain (PWS) is still not completely understood, but it is linked to progressive ectasia of the superficial cutaneous vascular network [ 13 , 14 ]. (hindawi.com)
Hemangiomas1
Phakomatosis pigmentovascularis is a distinctive association of cutaneous hemangiomas and melanocytic nevi. (pediatricneurosciences.com)
Bilateral1
The patient had bilateral facial nevus as well as unilateral buphthalmos [ 8 ]. (hindawi.com)
Naevus1
Sturge-Weber syndrome is a mesodermal phakomatosis characterized by port-wine naevus covering face and cranium supplied by first division of trigeminal nerve along with atrophy and calcification of cerebral hemisphere homolateral to the skin lesion. (pediatricneurosciences.com)
The birth defect is diagnosed by the presence of a combination of these symptoms (often on approximately 1⁄4 of the body, though some cases may present more or less affected tissue):[citation needed] One or more distinctive port-wine stains with sharp borders Varicose veins Hypertrophy of bony and soft tissues, that may lead to local gigantism or shrinking, most typically in the lower body/legs. (wikipedia.org)
Bony and soft4
The birth defect is diagnosed by the presence of a combination of these symptoms (often on approximately 1⁄4 of the body, though some cases may present more or less affected tissue):[citation needed] One or more distinctive port-wine stains with sharp borders Varicose veins Hypertrophy of bony and soft tissues, that may lead to local gigantism or shrinking, most typically in the lower body/legs. (wikipedia.org)
Klippel-Trenaunay-Weber syndrome (KTWS) is characterized by a triad of port-wine stain, varicose veins, and bony and soft tissue hypertrophy involving an extremity. (medscape.com)
In 1900, noted French physicians Klippel and Trenaunay first described a syndrome in 2 patients presenting with a port-wine stain and varicosities of an extremity associated with hypertrophy of the affected limb's bony and soft tissue. (medscape.com)
Most patients demonstrate all 3 signs of the clinical syndrome: port-wine stain, varicose veins, and bony and soft tissue hypertrophies. (medscape.com)
Parkes Weber1
Today, conflicting opinion exists in the literature as whether to separately designate the original triad as Klippel-Trenaunay syndrome and the triad with the addition of arteriovenous malformation as Parkes Weber syndrome. (medscape.com)
Hypertrophy of the affected1
The three main features are nevus flammeus (port-wine stain), venous and lymphatic malformations, and soft-tissue hypertrophy of the affected limb. (wikipedia.org)
Syndrome1
basal cell nevus syndrome an autosomal dominant syndrome characterized by the development in early life of numerous basal cell carcinomas, in association with abnormalities of the skin, bone, nervous system, eyes, and reproductive tract. (topgrowupclinic.eu)
Skin2
Birthmarks such as congenital nevi are benign pigmented patches of skin that can range in color from tan and brown to red, pink or purple and are present at birth. (advanced-dermcare.com)
[3] The entity was classically defined by the triad of abnormal skin pigmentation, nail dystrophy , and leukoplakia of the oral mucosa , but these components do not always occur. (mdwiki.org)