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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.
Complications: Neonatal hypoglycemia, Wilms tumor: Causes: CDKN1C mutation 11p15.5 CNV [2] Differential diagnosis: ... Nevus flammeus (port-wine stain) is a flat, red ...
The port-wine stains and physical examination are enough to diagnose PWS. [22] But additional testing is necessary to determine the extent of the PWS syndrome. The following tests may be ordered by physicians to help determine the appropriate next steps: MRI , ultrasound , CT/CAT scan, angiogram , and echocardiogram .
The greatest risk is associated with port-wine stains that appear to involve the entire V1 distribution followed by partial V1 involvement. [43] There is controversy as to whether or not the distribution of port-wine stains truly follows trigeminal nerve branches per se. [44] Port-wine stains are most often unilateral but can be bilateral ...
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]