PART III - VASCULAR MALFORMATIONS
Published online by Cambridge University Press: 07 September 2010
Summary
Introduction
Capillary malformations (CM) are hemodynamically inactive, slow-flow vascular malformations affecting the capillary network of skin and mucosa, sometimes invading deeper underlying structures specifically in the facial area. They include common CMs also known as “port-wine stains” and “telangiectasia.” Both can occur as a single isolated anomaly or in association with other abnormalities. Some are included in complex syndromes, the majority being sporadic and some being familial. We can also include the various “angiokeratomas”, localized, systematized or diffuse (angiokeratoma corporis diffusum), familial or not.
Common Capillary Malformations: Port-wine Stains (PWS)
Clinical Aspects
This is the most common type of vascular malformation. Typically a port wine stain (PWS) is present at birth and persists lifelong growing proportionately. However, rare acquired PWSs develop and progress in adolescents or adults; the possible role of trauma has been stressed (1), and reported as Fegeler syndrome (51).
A PWS is a more- or less-extensive well-demarcated red macular stain. Localized segmental PWSs are common on the face. On the face, CMs are commonly sub-classified as lateral CM (PWS) and medial CM (also known as salmon patch). Lateral PWSs of the face always persist whereas medial lesions usually become lighter and some disappear, particularly those of the mid-face. Metameric distribution occurs on the trunk and limbs; on the other hand it seems that PWSs never spread along Blaschko lines.
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- Color Atlas of Vascular Tumors and Vascular Malformations , pp. 123 - 286Publisher: Cambridge University PressPrint publication year: 2007
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