Phakomatosis pigmentovascularis

Phakomatosis pigmentovascularis
Other names	Association of cutaneous vascular malformations and different pigmentary disorders [1]
Specialty	Dermatology

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: ^{[2] [3]}

• 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.^{[ citation needed ]}

See also

• List of cutaneous conditions

References

1. "Phacomatosis pigmentovascularis | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Archived from the original on 28 April 2019. Retrieved 28 April 2019.
2. Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). Page 1012. McGraw-Hill. ISBN   0-07-138076-0.
3. Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN   978-1-4160-2999-1.