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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]
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).
It is commonly referred to as "port-wine stain", and is thought to affect approximately 0.5% of the population. [1] Typically capillaries in the papillary dermis are involved, and this gives rise to pink or violaceous colored lesions. [2] The majority of DCMO lesions are diffuse, reticulated pale-colored stains. [3]
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.
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]
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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.
Port-wine stain visible on the head of Mikhail Gorbachev, one of the most famous individuals with such a birthmark Port-wine stains, also known as nevus flammeus and sometimes mistaken for strawberry marks, are present at birth and range from a pale pink in color, to a deep wine-red.