Phakomatosis Pigmentovascularis: Case Report of Type IIa

Abstract

Phakomatosis Pigmentovascularis (PPV) is a rare sporadic developmental disorder
characterized by coexistence of a cutaneous vascular malformation and pigmentary nevi. There
are different classifications of PPV. When systemic involvement is there, a designation ‘b’ is
used, whereas if no systemic involvement, a designation ‘a’ is used. Herein, we reported a 12
years old girl presented with a symptomatic persistent progressive skin lesions since birth.
Systemic review and past medical history were all unremarkable. Skin examination revealed
mixture of diffuse non-scaly, bleachable erythematous patches, greenish patches, and hypopigmented
patches over her trunk. Ophthalmologist and neurologist consulations did not reveal
any abnormalities. Based on the above clinical findings, the patient was diagnosed to have
port-wine stains, Mongolian spots, and nevus anemicus. Constellation of these clinical findings
without presence of extracutaneous manifestations made the diagnosis of PPV type IIa.