Phakomatosis Pigmentovascularis

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Retrieved
2021-01-18
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Phakomatosis pigmentovascularis 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.

Types

Phakomatosis pigmentovascularis is subdivided into five types:

  • Type 1 PWS + epidermal nevus
  • Type 2 (most common): PWS + dermal melanocytosis +/- nevus anemicus
  • Type 3: PWS + nevus spilus +/- nevus anemicus
  • Type 4: PWS + nevus spilus + dermal melanocytosis +/- nevus anemicus
  • Type 5: CMTC (Cutis marmorata telangiectatica congenita) + dermal melanocytosis

They all can contain capillary malformation. Type 2 is the most common and can be associated with granular cell tumor. Some further subdivide each type into categories A & B; with A representing oculocutaneous involvement and subtype B representing extra oculocutaneous involvement. Others have proposed fewer subtypes but currently this rare entity is mostly taught as having five subtypes currently.

Diagnosis

Treatment

See also

  • List of cutaneous conditions