Nevus roseus Q82.5

Happle R 2005

The term nevus roseus is propagated to be an independent entity and not just a color variant of the nevus flammeus. Remarkably, the nevus flammeus is a characteristic component of phacomatosis pigmentovascularis type II ("phacomatosis cesioflammea"), while the nevus roseus is a distinguishing feature of phacomatosis pigmentovascularis type III ("phacomatosis spilorosea").

The independence is postulated by the exclusion of postzygotic mutations in the genes: GNAQ, GNA11, AKT1, PIK3CA. Mutations in these genes are characteristics of lateralized capillary hamartomas (nevi).

Clinically, this capillary vascular nevus is characterized by an asymptomatic, lateralized, extensive bright red patch formation on the trunk. The nevus roseus is a hallmark of phacomatosis spilorosea and phacomatosis melanorosea in which the nevus roserus is associated with lateralized café-au-lait spots. These are different types of didymoses (twin spots) caused by the coexistence of a large pigmented nevus, such as a Mongolian spot, a café-au-lait spot or a macular nevus spilus, and an extensive telangiectatic nevus.

The term nevus roseus has been proposed to describe a lateralized telangiectatic malformation with a light red or pale pink color in contrast to the dark hue of the classic nevus flammeus.

In analogy to the "port wine stain", a term that is used synonymously for the nevus flammeus, the nevus roseus could also be called a "rosé wine stain". This lateralized vascular birthmark must be distinguished from the completely harmless salmon-coloured spot (Unna spot - old name Naevus flammeus medialis or Naevus Unna-Pollitzer), which always affects the midline of the body. The nevus roseus belongs to the rather broad category of telangiectasiaevi as well as to the even larger group of "capillary malformations".

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