Phakomatosis pigmentovascularis

Phakomatosis pigmentovascularis
Classification and external resources
ICD-10 Q85.8
Orphanet 2875

Phakomatosis pigmentovascularis refers to the 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 five types. 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.

Phakomatosis pigmentovascularis by type: 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

[1][2]

See also

References

  1. ↑ Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). Page 1012. McGraw-Hill. ISBN 0-07-138076-0.
  2. ↑ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.


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