Other Names for this Disease
- Association of cutaneous vascular malformations and different pigmentary disorders
- Phakomatosis pigmentovascularis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.
port wine stain and pigmentary lesions, such as melanocytic nevi or epidermal nevi. A variety of classification systems have been proposed for PPV, largely depending on what type of pigmentary lesion is present. Around half of individuals with PPV have systemic disease, meaning that body systems other than the skin are affected. Systemic symptoms can vary greatly from person to person. PPV is not inherited, but is thought to be caused by a genetic phenomenon called twin spotting.Phacomatosis pigmentovascularis (PPV) is a skin and blood vessel disorder that is present from birth. Common signs and symptoms include
Last updated: 2/1/2011
- Moutray T, Napier M, Shafiq A, Fryer A, Rankin S, Willoughby CE. Monozygotic twins discordant for phacomatosis pigmentovascularis: evidence for the concept of twin spotting. Am J Med Genet A. 2010 Mar;
- 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;
- Chekroun-Le Du L et al.,. Phacomatosis pigmentovascularis type II. European Journal of Dermatology. 1998; http://www.john-libbey-eurotext.fr/en/print/e-docs/00/01/87/6B/article.phtml. Accessed 2/1/2011.
- Narchi H et al.,. Picture of the month. Arch Pediatr Adolesc Med. 2001; http://archpedi.ama-assn.org/cgi/content/full/155/2/191. Accessed 2/1/2011.
On this page
- MeSH® (Medical Subject Headings) is a terminology tool used by the National Library of Medicine. Click on the link to view information on this topic.
- The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Phacomatosis pigmentovascularis. Click on the link to view a sample search on this topic.