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A port-wine stain (nevus flammeus) is a discoloration of the human skin caused by a vascular anomaly (a capillary malformation in the skin). [1] 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 a capillary malformation, seen at birth. [2]
A port-wine stain is visible under the nose. On the right side of a cheek, capillary malformations are present. Macrocephaly-capillary malformation ( M-CM ) is a multiple malformation syndrome causing abnormal body and head overgrowth and cutaneous , vascular , neurologic , and limb abnormalities.
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, intellectual disability, and ipsilateral leptomeningeal angioma (cerebral malformations and tumors).
Nevus flammeus in other contexts refers to a port-wine stain, which is a more permanent mark. The term "stork bite" refers to the folklore idea that storks bring newborn babies to parents . See also
Over time, port-wine stains may become thick or develop small ridges or bumps, and do not fade with age. [2] Such birthmarks may have emotional or social repercussions. [2] Port-wine stains occur in 0.3% of the population, equally among males and females.
Because of the staining color they are sometimes referred to as "port-wine stains". "Port-wine stain" or discoloration of the skin due to vascular malformation is also referred as nevus flammeus. [5] [8] Hypertrophy: Hypertrophy refers to excessive growth of the bone and soft tissue. In PWS patients a limb is overgrown and hypertrophy is ...
Port-wine stain on leg. Capillary malformations involve the capillaries, and are the most common type. They used to refer only to port-wine stains but now include others. [2] Capillary malformations are limited to the superficial layers of the skin but they can thicken, become nodular, and sometimes become disfiguring. [5]
The three main features are nevus flammeus (port-wine stain), venous and lymphatic malformations, and soft-tissue hypertrophy of the affected limb. [2] It is similar to, though distinct from, the less common Parkes Weber syndrome. The classical triad of Klippel–Trenaunay syndrome consists of: [3]