The phakomatoses have been traditionally defined as a group of hereditary diseases with variable expressivity characterized by multisystem tumors with possible malignant transformation. The Sturge-Weber syndrome, Klippel-Trenaunay syndrome, and the phakomatosis pigmentovascularis have the facial port-wine stain in common. Numerous pathophysiogenetic mechanisms have been suggested such as venous...

Common birthmarks can be an indicator of underlying genetic disease but are often overlooked. Mongolian blue spots (dermal melanocytosis) are usually localized and transient, but they can be extensive, permanent, and associated with extracutaneous abnormalities. Co-occurrence with vascular birthmarks defines a subtype of phakomatosis pigmentovascularis, a group of syndromes associated with neur...

We report phakomatosis pigmentovascularis (PPV) detected in a 40yearold male characterized by the presence of a port-wine stain in the background of aberrant Mongolian spots covering the back, nevus of Ota, ocular melanosis, epidermal nevus and a scaly patch with the diagnosis of discoid lupus erytematosus. These associations have not been reported yet.

Temporal triangular alopecia (TTA), also known as congenital triangular alopecia, is a nonscarring, noninflammatroy, circumscribed form of alopecia. TTA has been associated with several disorders, such as Phakomatosis Pigmentovascularis. Hair restoration surgery using follicular unit transplantation has been a successful treatment modality for TTA. Herein we report such a success that was susta...

Phacomatosis pigmentovascularis is a rare syndrome, defined as the simultaneous presence of vascular nevus and melanocytic nevus in the same patient. We report the case of a 53-year-old woman presented with dermal melanosis and extensive vascular nevus, which match the typical manifestations of phakomatosis pigmetovascularis of cesioflammea type, according to Happle's classification. The rare o...

Sturge-Weber syndrome can be associated with facial port-wine stains and intracranial calcification, and concurrent Klippel-Trenaunay-Weber syndrome has been reported. Klippel-Trenaunay-Weber syndrome is a rare congenital mesodermal phakomatosis characterized by cutaneous hemangiomas, venous varicosities and soft tissue or bone hypertrophy of the affected extremities. This report is presented a...

The association of a vascular lesion with melanocytic nevi was first described by Ota et, al in 1947, and given the name phakomatosis pigmentovascularis(PPV). Later on this term was used for the cases with simultaneous occurrence of congenital vascular and pigmented (epidermal or melanocytic) anomalies. Our first case is a 12-year-old Kuwaiti boy who had extensive port-wine stain (nevus flammeu...

Phakomatosis Pigmentovascularis (PPV) is a rare cutaneous congenital malformation syndrome, defined as simultaneous occurrence of congenital cutaneous vascular and pigmentary anomalies. As most of the reported cases are from Far East countries, especially Japan, we reported a case of PPV type IIa in an Iranian patient. This case was unusual because of the coexistence of unilateral extensive por...