Phakomatosis pigmentovascularis Phakomatosis pigmentovascularis (PPV) is a rare syndrome characterised by the association of a vascular naevus, usually a capillary naevus (naevus flammeus or port-wine stain), with an extensive pigmented naevus, most commonly Mongolian spot or blue/grey oculocutanous melanocytosis (naevus of Ota). PPV is also associated with other naevi such as naevus anaemicus (see above), epidermal naevus, telangiectatic naevus (reticulate vascular naevi), naevus spilus (speckled lentiginous naevus) or cutis marmorata telangiectatica congenita. Five types of PPV have been described. The most common is Type II. Each type is further divided in to subtypes A and B. Subtype A has skin manifestations only. In subtype B, there are associated systemic abnormalities. Systemic involvement usually consists of similar features to those seen in Sturge-Weber syndrome or Klippel-Trenaunay syndrome (see above), and these features usually related to the body surface affected by the vascular lesion. http://www.dermnetnz.org/vascular/steal.html#avm Совершенно случайно выявил данную патологию осматривая пациентку на предмет угревой болезни.