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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Phacomatosis pigmentovascularis


Other Names for this Disease

  • Association of cutaneous vascular malformations and different pigmentary disorders
  • Phakomatosis pigmentovascularis
  • PPV
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Treatment

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How might phacomatosis pigmentovascularis be treated?

If phacomatosis pigmentovascularis (PPV) is not associated with systemic complications (e.g., Sturge-Weber syndrome, Klippel-Trenaunay syndrome, eye conditions) it requires no treatment, however pulsed dye laser may improve the appearance of port wine stains and Q-switched laser the appearance of pigmentary nevus.[1] Medical treatment of PPV with systemic complications requires individualized plans and often assistance from a team of specialists (e.g., opthamologist, neurologist, and vascular specialist).
Last updated: 2/1/2011

References
  1. Fernández-Guarino M, Boixeda P, de Las Heras E, Aboin S, García-Millán C, Olasolo PJ. Phakomatosis pigmentovascularis: Clinical findings in 15 patients and review of the literature. J Am Acad Dermatol.. 2008 Jan; Epub 2007 Nov 28;


Other Names for this Disease
  • Association of cutaneous vascular malformations and different pigmentary disorders
  • Phakomatosis pigmentovascularis
  • PPV
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.