Iranian Journal of Dermatology

Vol. 15, No. 60, Summer 2012
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Phakomatosis pigmentovascularis type IIa
Abbas Rasi, Mehdi Tabaie, Habib Hassannejad

Contact Info: Research Fellow of Photobiostimulation Research Group, Iranian Center for Medical Laser, Academic Center for Education, Culture and Research, Tehran, Iran, Email:

Date Received: 2012 / Apr / 10 Date Revised: 2012 / Apr / 10 Date Accepted: 2012 / Jun / 15

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 port-wine stains all over the left body side and left hard palate and a right plantar port-wine stain.

Keywords: nevus of Ota, phakomatosis pigmentovascularis, pigmentary anomalies, port-wine stains, vascular anomalies

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