Phacomatosis pigmentovascularis

Common Name(s)

Phacomatosis pigmentovascularis

Phacomatosis pigmentovascularis (PPV) is a skin and blood vessel disorder that is present from birth. Common signs and symptoms include 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.
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Phacomatosis pigmentovascularis" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
Top

How do you compare to others with this condition?

Privately answer questions about your health. Let resources, you select, come to you.

Anonymously share and see how your answers compare with others with this condition while privately providing key pieces of information to medical researchers, disease advocacy groups, and others ONLY YOU select to help speed up cures and better alternatives.

 
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Phacomatosis pigmentovascularis" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
 
 
Top

Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Phacomatosis pigmentovascularis" returned 2 free, full-text research articles on human participants. First 3 results:

Phacomatosis pigmentovascularis type IIa--case report.
 

Author(s): Majoriê Mergen Segatto, Eloísa Unfer Schmitt, Laura Netto Hagemann, Roberta Castilhos da Silva, Cristiane Almeida Soares Cattani

Journal: An Bras Dermatol. ;88(6 Suppl 1):85-8.

 

Phacomatosis Pigmentovascularis is a rare syndrome characterized by capillary malformation and pigmentary nevus. A case of a 2-year-old patient is reported, who presented extensive nevus flammeus and an aberrant Mongolian spot, without systemic disease, manifestations that allow us ...

Last Updated: 18 Dec 2013

Go To URL
Intramedullary and retroperitoneal melanocytic tumor associated with congenital blue nevus and nevus flammeus: an uncommon combination of neurocutaneous melanosis and phacomatosis pigmentovascularis--case report.
 

Author(s): Ryu Kurokawa, Phyo Kim, Toshiki Kawamoto, Hadzki Matsuda, Shujiro Hayashi, Soji Yamazaki, Atsushi Hatamochi, Shozo Mori, Mitsugi Shimoda, Keiichi Kubota

Journal: Neurol. Med. Chir. (Tokyo). 2013 ;53(10):730-4.

 

Neurocutaneous melanosis (NCM) is a rare condition characterized by central nervous system melanocytic tumors associated with congenital melanocytic nevi. Phacomatosis pigmentovascularis (PPV) is an association of vascular nevus with pigmentary nevus. Aberrant maturation of neural ...

Last Updated: 28 Oct 2013

Go To URL

Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Phacomatosis pigmentovascularis" returned 0 free, full-text review articles on human participants.

 
 
Top

Symptoms, Diagnosis, and Treatment

There are currently no related results available in Genetics Home Reference.

There are currently no related results available in GeneReviews.

There are currently no related results available in Genetic Testing Registry.

 
 
Top

Clinical Trial Information This information is provided by ClinicalTrials.gov

There are currently no open clinical trials for this condition.