Phakomatosis Pigmentovascularis: Case Report of Type IIa
Journal Title: Dermatology – Open Journal - Year 2016, Vol 1, Issue 1
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 findingswithout presence of extracutaneous manifestations made the diagnosis of PPV type IIa.
Authors and Affiliations
Khalid Al Hawsawi
Keywords
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- EP ID EP550627
- DOI 10.17140/ DRMTOJ-1-106
- Views 116
- Downloads 0
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