ABSTRACT

Peripheral vascular anomalies are very common (Greene et  al. 2011). One in three infants has cutaneous birthmarks at birth or immediately after, but only 1% of these require subsequent monitoring and therapeutic management (Fevurly and Fishman 2012). Notwithstanding the high incidence of vascular anomalies, our understanding of this pathology is still incomplete and the large number and complexity of classication systems proposed has not helped improve our understanding (Behr and Johnson 2013a,b; Britney 2007). The major barrier to elucidating the clinical and pathophysiological mechanisms underlying the development and regression of vascular anomalies has been the lack of a denitive classication system with a universally accepted nomenclature that is recognised by all specialists involved in its management (El-Merhi et al. 2013; Enjolras et al. 2007; Garzon and Frieden 2007). This lack of an accepted nomenclature is evidenced by the terms frequently used incorrectly to describe vascular anomalies from cavernous, strawberry, and tuberous angioma to red eck and port-wine stain birthmarks (Redondo 2004).