Phacomatosis pigmentovascularis type II

Phacomatosis pigmentovascularis (PPV) is described as the association of cu taneous vascular malformations and different pigmentary disorders. The diff erent associations are classified into four types, with localized and syste mic forms. The constant alteration in each type is a news flammeus. Associa ted pigmented abnormalities are news pigmentosus and verrucosus in type I, blue spots in type II, news spilus in type III, blue spots and news spilus in type IV. Type II is the most frequently reported. In this type, half of the patients have visceral involvement, more often Klippel-Trenaunay or/and Sturge-Weber syndrome. We report two cases of phacomatosis pigmentovascula ris type II. The particularity of the first case is the association with a cranio-cervical junction malformation (Arnold-Chiari type I), which to our knowledge, has never been described with PPV. The second case presents with a bilateral scleral melanosis without visceral involvement. Definition of visceral involvement and ophthalmologic manifestations are discussed.