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Nevus roseus: a distinct vascular birthmark

European Journal of Dermatology. Volume 15, Number 4, 231-4, July-August 2005, Genes and skin

Summary

Author(s) : Rudolf Happle , University of Marburg, Deutschhausstr. 9, D-35033 Marburg, Germany.

Summary : The new term nevus roseus is proposed to denote a lateralized telangiectatic birthmark with a light-red or pale-pink color, unlike the dark hue of nevus flammeus. It appears to be a distinct entity rather than just a color variant of nevus flammeus. Remarkably, nevus flammeus is a characteristic component of phacomatosis pigmentovascularis type II (“phacomatosis cesioflammea”), whereas nevus roseus represents a distinguishing feature of phacomatosis pigmentovascularis type III (“phacomatosis spilorosea”). In analogy to “port-wine stain” that is used as a synonym for nevus flammeus, nevus roseus could also be called “rosé-wine stain”. This lateralized vascular birthmark should be distinguished from the salmon patch that always involves the midline of the body. Nevus roseus belongs to the rather broad category of telangiectatic nevi, as well as to the even larger group of “capillary malformations”. For obvious reasons these terms are not suitable to designate any specific type of vascular birthmark such as nevus roseus.

Keywords : lateralized vascular birthmarks, telangiectatic nevi, nevus flammeus, nevus roseus, phacomatosis cesioflammea, phacomatosis spilorosea, classification of capillary malformations

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ARTICLE

Auteur(s) :, Rudolf Happle^*

University of Marburg, Deutschhausstr. 9, D-35033 Marburg, Germany

accepté le 23 Mars 2005

The group of vascular nevi includes various disorders such as nevus flammeus, nevus anemicus, and cutis marmorata telangiectatica congenita [1]. The purpose of this article is to describe another distinct type of vascular nevus. Every experienced dermatologist may have seen it, but because this birthmark had no specific name it does not as yet exist in our minds. Up to now it has been categorized as “nevus flammeus” or “telangiectatic nevus” or “capillary malformation”.

Definition of nevus roseus

Nevus roseus is a lateralized telangiectatic birthmark characterized by a light-red or pale-pink color (figures 1 and 3), contrasting with the dark hue of nevus flammeus (figures 2 and 4). Similar to nevus flammeus it tends to be arranged in a checkerboard pattern, involving either one or both sides of the body. In analogy to nevus flammeus, for which the synonym “port-wine stain” is used, nevus roseus could also be called “rosé-wine stain”.

Clinical evidence for the existence of nevus roseus as a distinct entity

A major argument in favor of the existence of nevus roseus can be derived from considering the various types of phacomatosis pigmentovascularis [2].The most frequently occurring form, phacomatosis pigmentovascularis type II [3], is always associated with a typical nevus flammeus (“phacomatosis cesioflammea”). Conversely, the vascular birthmark associated with phacomatosis pigmentovascularis type III (“phacomatosis spilorosea”) is not dark-red but constantly shows the pale-pink color of nevus roseus (figures 5 and 6) [2]. This dichotomy supports the notion that nevus roseus represents a distinct entity.

Nevus roseus versus nevus flammeus

Nevus roseus shows a rather light-red color, whereas nevus flammeus is characterized by a dark-red hue. It is important to realize, however, that during early infancy a nevus flammeus may likewise show a pink color [5]. Hence, an appropriate differential diagnosis can only be established in older children and adults.

Furthermore, it should be borne in mind that a nevus flammeus may either darken or lighten later in life. A distinction from nevus roseus, however, should still be possible in most cases. In contrast to nevus flammeus that may become markedly elevated in adult patients, especially when the lesion involves the face (( figure 4 )) [6-8], nevus roseus remains flat throughout life.

Nevus roseus versus salmon patch

The salmon patch is likewise a pale-pink telangiectatic lesion but, contrasting with nevus roseus, it always involves the midline in the occipital area (“stork-bite”), glabellar region (“angel’s kiss”), or sacral area [6-8]. The salmon patch occurs in about half of neonates [7]. For obvious reasons, it does not reflect mosaicism and should, therefore, not be categorized as a true nevus but rather as a nevoid lesion [9], whereas the lateralized nevus roseus represents, beyond doubt, an authentic nevus.

Nevus roseus versus “telangiectatic nevus”

The term “telangiectatic nevus” has a rather broad meaning. It refers to various types of vascular birthmarks including nevus flammeus, nevus roseus, and cutis marmorata telangiectatica congenita (table 1)( Table 1 ). It may even be applied to a segmental manifestation of Rendu-Osler disease [10]. Hence, this term cannot be used as a synonym for nevus roseus.
Table 1 A proposed classification of capillary malformations

1. Vascular nevi
1.1 Telangiectatic nevi
1.1.1 Nevus flammeus
1.1.2 Nevus roseus
1.1.3 Cutis marmorata telangiectatica congenita
1.2 Nevus anemicus
2. Vascular lesions that do not represent nevi
2.1 Salmon patch (“stork-bite”, “angel’s kiss”)
2.2 Telangiectases of Rendu-Osler disease

Nevus roseus versus “capillary malformation”

Presently, many authors no longer speak of vascular nevi but tend to apply to all of these cutaneos lesions the fashionable name “capillary malformation” [7, 11-14]. It is important to realize, however, that this term does not refer to any specific vascular disorder. It includes the various types of telangiectatic nevi as well as nevus anemicus [15].

A proposed classification of capillary malformations is presented in table 1. It should be noted that this classification, which may still be incomplete, does not correspond to the presently prevailing inconsequential and confusing terminology. For example, nevus anemicus does not so far exist as a distinct vascular anomaly in the Mulliken classification for the inexplicable reason that it is not considered a “capillary malformation” [7, 12, 16].

Other authors use the term nevus flammeus as a synonym for the salmon patch and not for the port-wine stain [12, 17]. Such inconsistencies show that we are presently far from reaching the aim of a generally acceptable classification of vascular birthmarks.

Conclusion

Nevus roseus appears to be a distinct entity. This vascular lesion should not simply be taken as a color variant of nevus flammeus. So far it is not possible to determine the prevalence of nevus roseus, but it can be said that it certainly occurs less frequently than nevus flammeus.

It must be emphasized, however, that a nevus flammeus may be rather light-red in infants and that, during adolescence and adulthood, this lesion may either darken or lighten, which is why it may be confused with nevus roseus. If it is difficult to discriminate between the two types of vascular nevi, it seems worthwhile to use the neutral term “telangiectatic nevus”, thus leaving the diagnosis uncertain.

Today it seems too soon to determine the full nosological significance of nevus roseus. Future studies may address the following questions:

– Does the margin of nevus roseus tend to be less well demarcated as compared to nevus flammeus ( (figure 3) )?
– May the pink color of this lesion sometimes be more reticular ( (figure 3) )?
– Does the response to laser treatment differ as compared to nevus flammeus?
– And, most importantly, to which degree do the spectrum and prevalence of associated defects or complications differ from those observed in nevus flammeus [18, 19]?

A pale-pink vascular nevus should no longer be called “nevus flammeus” or “port-wine stain”, nor should it be described, in those cases where a classification is possible, by the indistinct terms “telangiectatic nevus” or “capillary malformation”. Nevus roseus is an additional example of “capillary malformation” and thus provides further evidence that this rather broad term is not suitable to denote any specific type of vascular birthmark.

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