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Phakomatosis Pigmentovascularis

  • Ramón Ruiz-Maldonado
  • Carola Duràn-McKinster
  • Luz Orozco-Covarrubias
  • Marimar Saez-De-Ocariz
Chapter

Abstract

Phakomatosis pigmentovascularis (PPV) is defined as the coexistence of a widespread vascular (usually capillary) nevus (nevus flammeus) and an extensive pigmentary nevus (usually of the Mongolian spot type or blue/slate/grey oculo-cutaneous melanocytosis) associated to a variety of other cutaneous nevus (e.g., anaemicus, epidermal nevus, telangiectatic nevus, nevus spilus or cutis marmorata telangiectatica) and/or extracutaneous alterations (Happle 2005, Ruiz-Maldonado et al. 1987, Vidaurri-de la Cruz et al. 2003). All types of PPV so far described have been best explained as examples of twin spotting (Happle 1999, 2005).

Keywords

Moyamoya Disease Weber Syndrome Epidermal Nevus Capillary Malformation Mongolian Spot 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer-Verlag/Wien 2008

Authors and Affiliations

  • Ramón Ruiz-Maldonado
    • 1
  • Carola Duràn-McKinster
    • 1
  • Luz Orozco-Covarrubias
    • 1
  • Marimar Saez-De-Ocariz
    • 1
  1. 1.Department of DermatologyNational Institute of PaediatricsMexico CityMexico

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