Phakomatosis Pigmentovascularis: Case Report of Type IIa
Phakomatosis Pigmentovascularis 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.
Phakomatosis Pigmentovascularis is a rare sporadic developmental disorder
characterized by coexistence of a cutaneus vascular malformation and pigmentary nevi.
Traditionally, there are 4 types of PPV. However, later on a fifth type has been described.
Happle proposed a new classification of PPV composed of 4 types.
The most common form of PPV is type II. A 12-year-old girl presented with a symptomatic
persistent skin lesions, since birth. The lesions were increasing in size in the first few years
of life but later on became stable.
Skin examination revealed mixture of non-scaly, bleachable erythematous patches,
greenish patches, and hypopigmented patches over her trunk only.
Dermatol Open J. 2016; 1(1): 19-21. doi: 10.17140/DRMTOJ-1-106
