[Phakomatosis pigmentovascularis ].

A case of phakomatosis pigmentovascularis type IIa in a, short stature, 6-year old girl with nevus flammeus of the face and palate, bilateral ocular melanosis, aberrant mongolian spots and minor dysmorphic traits is reported. The patient has been considered at risk for glaucoma and ocular melanoma and an ophthalmologic follow-up including a tonometry is yearly requested. Phakomatosis Pigmentovascularis (PP) is a rare disease characterized by coexistence of capillary hemangioma and pigment abnormalities. The authors review the literature and discuss clinical features which actually allow the distinction of PP into 4 types. The opportunity of a follow-up specifically planned on the patient's phenotype is emphasized.