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Year : 2016  |  Volume : 82  |  Issue : 3  |  Page : 298--303

Phacomatosis pigmentovascularis: Report of four new cases

1 As'ad Al-Hamad Dermatology Center, Al-Sabah Hospital, Kuwait
2 Department of Pediatrics, Mubarak Al-Kabeer Hospital, Kuwait
3 Department of Pediatrics, Al-Adan Hospital, Kuwait
4 Al-Bahar Eye Center, Kuwait

Correspondence Address:
Dr. Arti Nanda
P.O. Box: 6759, Salmiya 22078
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0378-6323.178905

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Phacomatosis pigmentovascularis is a rare group of syndromes characterized by the co-existence of a vascular nevus and a pigmentary nevus with or without extracutaneous systemic involvement. The existing classifications of phacomatosis pigmentovascularis are based on phenotypic characteristics. We report four new cases of phacomatosis pigmentovascularis, three with phacomatosis cesioflammea demonstrating phenotypic variability, and one with phacomatosis cesiomarmorata. Extracutaneous manifestations were observed in three patients (75%) that included central nervous system involvement in three, bilateral congenital glaucoma in two, and cardiovascular system involvement in one. The molecular basis of phacomatosis pigmentovascularis is yet to be elucidated. Whether the various subtypes of phacomatosis pigmentovascularis are separate molecular entities or phenotypic variants of the same disease needs to be settled.


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Online since 15th March '04
Published by Wolters Kluwer - Medknow