Phacomatosis pigmentovascularis type II

Phacomatosis pigmentovascularis (PPV) is described as the association of cutaneous vascular malformations and different pigmentary disorders. The different associations are classified into four types, with localized and systemic forms. The constant alteration in each type is a nevus flammeus. Associated pigmented abnormalities are nevus pigmentosus and verrucosus in type I, blue spots in type II, nevus spilus in type III, blue spots and nevus 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 pigmentovascularis 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.