Skin melanocytic tumor
Syndromes
Phacomatosis pigmentovascularis (PPV)


Topic Completed: 1 February 2013

Revised: 16 May 2019

Copyright: 2002-2019, PathologyOutlines.com, Inc.

PubMed Search: Phacomatosis pigmentovascularis (PPV)

Christopher S. Hale, M.D.
Page views in 2018: 277
Page views in 2019 to date: 580
Cite this page: Hale C. Phacomatosis pigmentovascularis (PPV). PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/skintumormelanocyticphakomatosis.html. Accessed December 14th, 2019.
Definition / general
  • Rare syndrome of widespread, aberrant and persistent nevus flammeus (vascular nevus, capillary malformation) and extensive pigment abnormalities (Arch Dermatol 2005;141:385, Pediatr Dermatol 1987;4:189)
  • Divided into three main types:
    • Phacomatosis cesioflammea: blue spots [caesius = bluish gray] plus nevus flammeus; also called types IIa / IIb; includes nevus of Ota; most common of 3 types (J Am Acad Dermatol 2008;58:88); often associated with Sturge-Weber and Klippel-Trenaunay syndromes
    • Phacomatosis spilorosea: nevus spilus [speckled lentiginous nevus] plus pale pink telangiectatic nevus; also called types IIIa / IIIb
    • Phacomatosis cesiomarmorata: blue spots plus cutis marmorata telangiectatica congenita; also called type V
    • Subtypes: a - cutaneous involvement only, b - cutaneous and system involvement
Terminology
  • "Phacomatosis" refers to any of several disorders with multiple hamartomas of nervous system, eye, skin, viscera
  • Sometimes spelled "phakomatosis"
Epidemiology
Sites
  • Any site; 50% have extracutaneous involvement
Case reports
Clinical images

Images hosted on other servers:

Aberrant mongolian spots affecting upper part of back

Extensive nevus flammeus involving back and left arm

  

Blue black macular
pigmentation on periorbital
areas (bilateral
scleral melanosis)

Extensive nevus flammeus involving lower limbs

  

Extensive nevus flammeus
involving face, thorax,
arms; nevus anemicus on
right pectoral area


Extensive nevus flammeus and livedo reticularis pattern

Left lower limb 3 cm longer

Diffuse bluish discoloration

Phacomatosis cesioflammea

Phacomatosis spilorosea

Phacomatosis cesiomarmorata

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