Phakomatosis pigmentovascularis

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Phakomatosis pigmentovascularis
Other names: Association of cutaneous vascular malformations and different pigmentary disorders[1]
a) Increased melanin pigment in basal layer b) increased melanin pigment in basal layer/scattered spindle-shaped melanocytes

Phakomatosis pigmentovascularis is a group of skin conditions that have a combination of a blood vessel malformation and blue marks.[2]

It is a rare neurocutanous condition where there is coexistence of a capillary malformation (port-wine stain) with various melanocytic lesions, including dermal melanocytosis (Mongolian spots), nevus spilus, and nevus of Ota.


Phakomatosis pigmentovascularis is subdivided into four types; phakomatosis cesioflammea, phakomatosis spilorosa, phakomatosis cesiomamorata, and unclassifiable.[2]

See also


  1. "Phacomatosis pigmentovascularis | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". Archived from the original on 28 April 2019. Retrieved 28 April 2019.
  2. 2.0 2.1 James, William D.; Elston, Dirk; Treat, James R.; Rosenbach, Misha A.; Neuhaus, Isaac (2020). "28. Dermal and subcutaneous tumors: Phakomatosis pigmentovascularis". Andrews' Diseases of the Skin: Clinical Dermatology (13th ed.). Edinburgh: Elsevier. p. 603. ISBN 978-0-323-54753-6.

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