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Delineation of the various shapes and patterns of nevi


European Journal of Dermatology. Volume 15, Number 6, 439-50, November-December 2005, Genes and skin


Summary  

Author(s) : Antonio Torrelo, Eulalia Baselga, Eduardo Nagore, Antonio Zambrano, Rudolf Happle , Department of Dermatology, Hospital del Niño Jesús, Menéndez Pelayo 65 28009-Madrid, Spain, Department of Dermatology, Hospital Santa Creu i Sant Pau, Barcelona, Spain, Department of Dermatology, Instituto Valenciano de Oncología, Valencia, Spain, Department of Dermatology, University of Marburg, Marburg, Germany.

Summary : It has been proposed that all nevi reflect mosaicism, and this concept has been corroborated at the molecular level in a number of nevi. If the concept of mosaicism holds true, one would expect that the various types of nevi should be characterized by intrinsic shapes or patterns. A photographic review of 1,188 recognizable images of a list of nevi of the skin was undertaken in order to delineate a classification of the various shapes and patterns of nevi. We disclosed three distinct shapes and two so far undescribed archetypical patterns, in addition to the well-known Blaschko-linear, flag-like and extensive garment-like patterns, to which all lesions could be allocated. The newly delineated shapes are called round or oval shape\; patches with indented borders\; and teardrop or triangular shape. The newly proposed archetypical configurations are the agminated pattern and the diffuse patchy pattern. Other mosaic patterns, such as the phylloid pattern or the lateralization pattern, were not observed in any of the images reviewed. We conclude that the various types of nevi can be assigned to specific shapes or patterns. Admittedly, however, a given nevus may manifest itself in several different shapes, whereas its archetypical pattern tends to be the same. This ‘archetypical’ pattern can be taken as the perfect or ideal model of a given type of nevus, whereas the newly described shapes are subordinated to the archetypical patterns and are best considered to be ‘nonarchetypical’. Our work gives more consistency to the idea that nevi reflect mosaicism, because they display repetitive shapes and patterns.

Keywords : nevus, mosaicism, morphology, patterns, shapes

Pictures

ARTICLE

Auteur(s) : Antonio Torrelo1, Eulalia Baselga2, Eduardo Nagore3, Antonio Zambrano1, Rudolf Happle4

1Department of Dermatology, Hospital del Niño Jesús, Menéndez Pelayo 65 28009-Madrid, Spain
2Department of Dermatology, Hospital Santa Creu i Sant Pau, Barcelona, Spain
3Department of Dermatology, Instituto Valenciano de Oncología, Valencia, Spain
4Department of Dermatology, University of Marburg, Marburg, Germany

accepté le 15 Juillet 2005

Despite the widespread use of the term nevus, there was during the last century no generally accepted definition [1]. Nevi may be congenital or acquired, they may show increase or decrease of a given cellular structure, or they may reflect a functional disorder. In this situation it is understandable that some authors ironically stated that every lesion that once was called a nevus may be considered a nevus [2]. In 1995, a workable definition was proposed by Happle who put forward the idea that all nevi reflect mosaicism [1]. He proposed the following definition: “Nevi are visible, circumscribed, long-lasting lesions of the skin or the neighboring mucosa, reflecting genetic mosaicism. With the exception of melanocytic nevi, they do not show neoplastic growth. They never show malignant neoplasia” [1]. Although this concept has so far not been proven in every type of nevus, mosaicism has today indeed been documented in many nevi such as segmental hypermelanotic lesions [3], segmental hypomelanotic lesions (nevus depigmentosus) [4], segmental acne (“Munro nevus”) [5], epidermolytic epidermal nevus [6] and segmental Darier disease [7].Happle has described five standard patterns of cutaneous mosaicism in human skin, in the form of the Blaschko-linear, checkerboard, phylloid, midline patch, and lateralization pattern [8, 9]. These patterns reflect the arrangement of rather large lesions and visualize either genomic or epigenetic mosaicism. However, if the concept of nevi as mosaics holds true, an explanation should be offered to the fact that most nevi we regularly see in clinical practice apparently do not follow such archetypical patterns. Hence, two hypotheses might be put forward: 1) more clinical patterns of mosaicism may exist in human skin, and 2) every nevus follows one of those ‘archetypical’ mosaic patterns but in small or medium-sized lesions this arrangement cannot be recognized, although they may display one or more characteristic shapes. To assess which one of these hypotheses is more likely, we studied the shapes and patterns of nevi from photographic files. In this way we recognized that many of such lesions show a highly repetitive shape or pattern. Defining the specific shape or pattern of a given nevus would be helpful as a first step to elucidate the genetic basis of such lesions, since different clinical shapes or patterns might possibly reflect different genetic mechanisms such as different mutations or even different moments of appearance of such mutations (i.e., timing) during embryogenesis or later in life. Furthermore, a more thorough study of the shapes and patterns of nevi would help to further delineate the association of cutaneous nevi with extracutaneous manifestations. Finally, because some distinct types of nevi may appear simultaneously or in close apposition, their shapes and patterns may be useful to further depict this so called twin spotting phenomenon [8].In this article, we propose a new classification of the shapes and patterns of nevi, based on a retrospective review of photographic images.

Materials and methods

A photographic review of our image files was carried out. We reviewed all evaluable and recognizable images of every nevus of the skin. For the selection of images, we used Happle’s definition of nevus to consider a lesion eligible (table 1)( Table 1 ). For nevi with conflicting terminology, we decided arbitrarily to follow a denomination used in a classic reference textbook [10]. In this way, the conditions listed in table 1 were chosen for review. Some facts about the choice deserve further comment. Regarding melanocytic nevi, we decided to include only congenital melanocytic nevi, disregarding common acquired ones. Vascular nevoid conditions were troublesome because of different denominations by different countries or schools; thus, although in some classifications of vascular skin disorders the word “nevus” is no longer present [11], we only considered lesions diagnosed under the terms telangiectatic nevus and nevus flammeus amenable to our study. Nevi flammei affecting the face and neck were not used for this review because they will be the subject of another study. Salmon patches of the newborn were not considered because they are not included in the definition of nevus we used for our study [1]. When a picture showed more than one cutaneous lesion of the same clinical type of nevus, the most conspicuous one was selected; for example, in cases of giant melanocytic nevus with smaller “satellites”, only the largest lesion was chosen for assignment to a pattern.

For our review, two observers from different departments of dermatology (A.T. and E.B.) independently evaluated the selected conditions from their image files. Then, an agreement was reached regarding conceptual shapes or patterns of the skin lesions. In our evaluation process, no attention was paid to the cellular origin of the nevus, although this is the most usual way to refer to any of such nevi. We only assigned the nevi to one or other pattern by their shape, outlines, and distribution, regardless of features such as color, size, depth of involvement, association with other nevi or extracutaneous abnormalities, or supposed cellular components of the lesions.

After discussion and training, a second review of all of the selected cases was carried out, and all images were assigned to one of the shapes or patterns designed. In a third step, all images with conflicting assignment were reassessed, and every doubtful case was ultimately categorized by agreement after reasoning.
Table 1 List of nevi chosen for review

• Epidermal nevus (without further specification)

• Sebaceous nevus

• Comedo nevus

• Linear porokeratosis

• Porokeratotic eccrine ostial and dermal duct nevus

• Congenital triangular alopecia

• Becker’s nevus

• Collagen nevi

• Juvenile elastoma

• Nevus anelasticus

• Congenital smooth-muscle hamartoma

• Telangiectatic nevi (including nevus flammeus)

• Nevus anemicus

• Cutis marmorata telangiectatica congenita

• Segmental venous malformations

• Angiokeratoma

• Congenital melanocytic nevus

• Nevus spilus (syn. speckled nevus)

• Ota nevus

• Aberrant blue spot (dermal melanocytosis)

• Congenital blue nevus

• Nevus depigmentosus

• Hyperchromic nevus

Results

A total of 1,188 images were selected for the study. Only images where the whole lesion could be photographed were chosen but, for very extensive lesions, a set of images from a single patient was accepted to clarify the distribution and extent of the nevus. By observation of images of nevi, we were able to define three distinct shapes as well as two so far undescribed patterns, in addition to the well-established Blaschko-linear, block/flag-like and garment-like patterns, to which all lesions could be allocated. A single case was only assigned to one single shape or pattern. The configurations identified and the number of entities assigned to such shapes or patterns are shown in table 2( Table 2 ). In brief, the configurations were described and named as follows:

Assignment to three distinct shapes

Round or oval shape

Typically, a round or oval patch, papule or plaque, usually of small size, with regular borders is present at any part of the skin surface. In our review, this was the most frequently occurring shape of all nevi, and corresponded mostly to small or medium-sized congenital melanocytic nevi (table 2). Other examples of nevi with this common shape include nevus depigmentosus, small sebaceous nevi, and small patches of dermal melanocytosis (aberrant blue spots) ( (figure 1) ).
Table 2 Assignment of nevi to the different shapes or patterns

TYPE OF NEVUS

Round or oval

Patch with indented borders

Teardrop / triangular

Agminated

Blaschkolinear

Blocks or flags

Diffuse patchy

Garment-like

TOTAL

Epidermal nevus (without further specification)

3

1

128

132

Sebaceous nevus

74

4

37

115

Comedo nevus

16

16

Linear porokeratosis

15

15

PEODDN a

3

3

Triangular alopecia

15

15

Becker’s nevus

58

58

Collagen nevi

17

9

26

Juvenile elastoma

10

5

15

Nevus anelasticus

1

1

Congenital smooth-muscle hamartoma

35

4

1

2

42

Telangiectatic nevus (without further specification) b

5

38

31

14

88

Nevus anemicus

12

12

Cutis marmorata telangiectatica congenita

43

43

Congenital melanocytic nevus

148

3

4

6

1

70

232

Nevus spilus (speckled nevus)

38

1

13

52

Ota nevus

9

9

Aberrant blue spot (aberrant Mongolian spot, dermal melanocytosis)

36

4

1

37

78

Congenital blue nevus

7

5

2

14

Nevus depigmentosus

67

15

3

52

8

145

Hyperchromic nevus c

37

18

18

1

3

77

TOTAL

450

193

30

22

269

100

51

73

1188

aPEODDN: porokeratotic eccrine ostial and dermal duct nevus.

bExcluding nevi flammei of the face and neck.

cA hyperchromic, usually congenital macule, as counterpart of nevus depigmentosus (i.e., hyperchromic mosaic patches), excluding café-au-lait macules of NF1.

Patchy indented shape

This shape is characterized by irregular, usually serrated or indented borders, and it may be round, oval or rhomboidal. The size of the nevus may range from a small lesion to a larger one even measuring some centimeters in diameter. Nevi displaying this shape may appear on any part of the body, and some of them may ignore the midline ( (figure 2) ).

This shape was exhibited by some hyperpigmented epidermal macules or hyperchromic nevi (café-au-lait macules of NF1 and lentigines excluded), nevus anemicus, and aberrant blue spots, among others. Lesions adopting this shape may show areas of normal tissue within the patch, intermingled with involved areas, leading to a somewhat reticular pattern with preserved irregular borders. This was noted in cases of Becker’s nevus or connective tissue nevus.

Teardrop or triangular shape

This peculiar configuration is characterized by comma shaped lesions, with a sharply, angled end. Their size is rather variable, and the lesions do not extend in a Blaschko-linear distribution. This shape may resemble some lesions of the phylloid pattern, but we are describing isolated lesions here. Remarkably, a significant portion of skin lesions in our review adopting this teardrop shape consist of nevi of absent or decreased tissue, such as nevus anelasticus or congenital triangular alopecia, but other lesions such as small sebaceous nevus, melanocytic nevus, and nevus depigmentosus may also show this outline ( (figure 3) ).

Assignment to seven distinct archetypical patterns

Agminated or cannonball pattern

The Latin term ‘agminated’ means clustered. Under this term we include small patches or papules being grouped in a circumscribed area of the skin, with sparing of the normal skin between the individual lesions. These clusters are mostly solitary, and they may appear in every part of the skin. It is a rarely occurring pattern, and in our series, the most common skin lesions following this pattern were agminated melanocytic nevi and collagen nevi ( (figure 4) ).

Diffuse patchy pattern

This pattern consists of disseminated lesions, usually affecting a large portion of the trunk, which appear as multiple, discrete or confluent, patchy macules, showing no tendency to respect the midline. Such extensive patches leave areas of uninvolved skin. In our series, only two types of nevi, extensive aberrant blue spots and some extensive telangiectatic nevi were assigned to this pattern ( (figure 5) ).

Blaschko-linear pattern

Today this is the best known pattern of nevi or nevoid disorders. It is generally accepted that Blaschko lines are the lines of embryonic development of the skin, and that every Blaschko line is derived from a single cell forming a clone [12]. Dermatoses following these lines are indeed mosaic states of the disease, as has been demonstrated for epidermal nevi, systematized nevus depigmentosus, segmental hypermelanosis, segmental Darier disease, segmental Hailey-Hailey disease and linear lesions of child nevus. Other skin lesions following Blaschko lines still await genetic confirmation of its mosaic state. Blaschko lines originate from the dorsal midline, and have a fountain-like, V or S shape on the trunk, and a longitudinal shape on the limbs. Blaschko lines on the face and scalp have been recently addressed [13]. Examples of lesions following this well known pattern in our study were sebaceous nevus, epidermal nevus (without further specification), nevus depigmentosus, linear hyperchromic nevus (linear hyperpigmentation), porokeratotic eccrine nevus, nevus comedonicus, and linear porokeratosis ( (figure 6) ).

Block or flag-like pattern

Under the synonymic designation “checkerboard pattern” this arrangement was also depicted by Happle as an archetypical manifestation of mosaicism. Extensive areas of mutant tissue take the form of blocks or flags involving large areas of the skin, mostly located on the trunk. Typically, these blocks stop at the midline, and they may affect one side or both sides of the body; in this latter instance, alternating mutant blocks may give rise to a true ‘checkerboard pattern’, but if the same areas of both sides of the body are affected, the checkered appearance is blurred. On the limbs the checkers are more difficult to recognize because, instead of squares or rectangles they appear as broad ribbons or as an involvement of the whole extremity extending from the girdle, where the lesion usually has straight upper and medial margins. In many instances, patients with this pattern have been described with rather loose words such as “dermatomal”, “segmental”, “zosteriform” or others. The most common lesions following this pattern in our study were vascular nevi such as cutis marmorata telangiectatica congenita or nevus flammeus, but speckled nevus as well as hyper- or hypopigmented macules sometimes were likewise assigned to this pattern ( (figure 7) ).

Paradoxically, however, Becker’s nevus that was presented by Happle as exemplifying the flag-like or checkerboard pattern, failed to show this arrangement in our series of 58 cases. This may be explained by the fact that the Becker nevi included in our study tended to be rather small lesions ( (figure 8) ).

Extensive garment-like pattern

This pattern is almost exclusively described for pigmented lesions. It shows a typical involvement of large areas of the skin covering the trunk or limbs. The mutant tissue does not stop at the midline, and usually involves partially or completely, the trunk or a limb, in the form of a shirt, a bathing suit or a cuff. In general the extension of the lesion is wider on the back than on the abdomen, and the lesion may not embrace the whole circumference of the trunk, leaving abdominal areas uninvolved. In our series, this pattern was almost restricted to giant melanocytic nevi, but some hypermelanotic macules were likewise extensive enough to be included in this group ( (figure 9) ).

Failure to observe other patterns

We did not observe any clinical image consistent with a phylloid pattern of mosaicism or a lateralization pattern [15].

Discussion

In this study we have tried to specify the shapes and patterns that nevi may exhibit. According to our working definition of a nevus [1], all nevi reflect mosaicism. At least two other mosaic patterns exist, namely the phylloid and the lateralization patterns [8, 9], but we did not identify in our files any patient whose nevi followed such configurations. On the other hand, we are proposing two so far undescribed mosaic patterns in the form of the “agminated” and the “diffuse patchy” patterns. It is important to realize that we assigned a given case to one single shape or pattern only, although a limited number of cases might have been allocated to more than one category. In such instances, the most conspicuous configuration was chosen by agreement between both observers.

We are using the term “shape” for the configuration of an individual skin lesion that is usually small or medium-sized, whereas the term “pattern” denotes an archetypical arrangement of usually larger lesions that, in a given case, may or may not show a systematized distribution. For example, a small Becker’s nevus is assigned to the “patchy indented” shape, whereas a large Becker’s nevus, although likewise being both patchy and indented, can be assigned to the block/flag-like pattern. Hence, it should be noted that the categories of “shape” and “pattern” are not mutually exclusive.

We emphasize that the three newly described shapes in the form of round/oval, patchy indented, and teardrop/triangular configurations are categories that apply to small or medium-sized nevi. When the same types of nevi are of larger size or show a systematized arrangement, they may adopt an “archetypical” pattern. For example, in cases of non-organoid or organoid epithelial nevi we often observed a round or oval shape, and sometimes even a teardrop shape, but they never showed a patchy indented shape. From this we conclude that a round/oval or a teardrop-shaped patch indeed represents, at least in some types of nevi, a “point” on a Blaschko line. On the other hand, in the group of vascular nevi that often show a block or flag-like pattern, we find that smaller lesions may show a “patchy indented” shape. Similarly, large Becker nevi are patches with indented margins arranged in a block or flag-like pattern, whereas small lesions can only be assigned to the “patchy indented” shape (table 2). Thus, we infer that, at least for some types of nevi, a patch with indented borders is, indeed, a small part or “forme fruste” of a block or flag-like pattern.

The patterns of lesions diagnosed as nevus depigmentosus support this view. Accordingly, a nevus depigmentosus, when very widespread, may be arranged in “archetypical” patterns such as Blaschko’s lines or the flag-like pattern, whereas smaller lesions show a round/oval or triangular shape (representing a “point” or a short piece of a Blaschko line), or a patchy indented shape (representing rather small “blocks” or “flags”).

It should be noted that some types of nevi which commonly appear as a round or oval patches, never or virtually never follow the lines of Blaschko, as is the case for congenital melanocytic nevi, speckled nevi or aberrant blue spots. From this we conclude that a round/oval lesion may also represent, in principle, a rather small block/flag or garment-like lesion.

Other patterns we have described here, such as the “agminated” and the “diffuse patchy” patterns, need further comment. It seems conceivable that some agminated lesions, particularly the agminated melanocytic nevi, may represent in principle a small counterpart of garment-like lesions showing further modification by tissue expansion or other unknown factors. In such cases, “agminated” would perhaps be better taken as a shape rather than as a pattern. On the other hand, we tentatively introduced a pattern we have called “diffuse patchy” because we felt that this pattern differs from the garment-like arrangement. We do so far not know whether this is a new archetypical pattern of mosaicism or merely represents a variation of the garment-like pattern. Notably, lesions that were assigned to this pattern (extensive telangiectatic nevi and extensive aberrant blue spots) tend to appear as round/oval lesions or as patches with indented borders when lesions are smaller.

The fact that some types of nevi virtually always show an identical, repetitive shape or pattern from patient to patient suggests that a similar mutational event is responsible for such configurations. On the other hand, different types of nevi may exhibit identical shapes or patterns, suggesting that they originate from some common genetic pathomechanism. As a hypothesis, if mosaicism is responsible for a single nevus showing various configurations, we may hypothesize that postzygotic mutations are occurring at different states of embryonic development, or during extra-uterine life [14]. Thus, we infer that if a mutation occurs after the migration of skin components has been completed, a round/oval or patchy indented shape is expected, whereas “archetypical” patterns such as Blaschkolinear or flag-like arrangements would originate from a mutation occurring before the embryonic development of the skin components has been completed. So far, postzygotic mutations have been demonstrated to be involved in some, mostly Blaschkolinear, nevi, but demonstration of mosaicism in all types of nevi is still lacking [3-7]. However, the striking and usually under-recognized tendency of lesions with different clinical shapes or patterns to appear associated in the form of twin spotting or didymosis is yet another piece of indirect evidence of the mosaic nature of nevi [15]. The concept of mosaicism will help to further elucidate the pathogenesis of nevi, and will also permit us to avoid inappropriate terms such as “dermatomal” or “zosteriform”, and to develop a more accurate classification of the various shapes and patterns of nevi.

The classification of different archetypical patterns may yield some insight into the embryonic development of human skin. Although our morphological analysis was not designed as an epidemiological study on the prevalence of nevi, it is clear from our photographic files that certain nevi derived from embryonic ectodermal tissue such as non-organoid epidermal nevus, comedo nevus, linear porokeratosis, and others show a tendency to be arranged in a Blaschko-linear pattern, whereas pigmentary nevi, that are likewise derived from the ectodermal layer, may also show a block/flag-like, diffuse patchy or garment-like pattern, in addition to the Blaschklo-linear arrangement. On the other hand, the Blaschko-linear pattern is only rarely followed by nevi of mesodermal origin, such as vascular or collagen nevi. Such lesions show a preference for the block/flag-like arrangement and the diffuse patchy pattern. We should like to emphasize, however, that mesodermal lesions can likewise follow the lines of Blaschko, as is the case in focal dermal hypoplasia or linear atrophoderma of Moulin. Hence, the Blaschko lines can be visualized by cell types other than those derived from the ectoderm. Melanocytic nevi show an array of various patterns, but remarkably the garment-like pattern is almost completely restricted to them. We observed the indented shape in melanocytic nevi as well as in nevi of vascular origin. On the other hand, the teardrop shape and the agminated pattern were not assigned to any type of vascular nevus in the present study.

Some methodological aspects of our work deserve further explanations. Firstly, the lesions studied here do not reflect the true prevalence of skin nevi, because this was a retrospective analysis of photographic files, and it is conceivable that not all common lesions are photographed in the daily clinic. More importantly, we have defined shapes and patterns to include all photographic images in our files, but the configurations we chose were arbitrarily selected, and some minor differences may be difficult to recognize. Hence, theoretically it could be problematic to obtain a high inter- observer agreement when evaluating cutaneous nevi. However, both observers involved in the present study reached an almost complete agreement in the assignment of configurations, but we must acknowledge that the statistical reliability of these shapes and patterns has not been studied. In fact, the assessment of the inter-observer agreement was not a purpose of this study. Hence, further investigations specifically aimed at this matter are warranted. Nonetheless, it is our feeling that a high degree of agreement can be achieved with minimal training. It is still possible that some lesions are very difficult to assign and may defy our classification of shapes and patterns, and that other configurations may exist. Thus, some refinement of the shapes and patterns we have proposed is acceptable. Finally, regarding the nevi selected for our study, we acknowledge that some lesions we call nevi may be considered by others as “malformations” [11]. However, because such lesions tend to show consistent shapes or patterns, we think that the term “nevus” is more appropriate.

Another semantic problem may lie in the fact that proliferative lesions such as hemangiomas, that are definitely not nevi, may sometimes show a segmental arrangement [16]. In fact, many other neoplastic lesions can be arranged in a “nevoid” pattern, such as segmental nevoid basal cell carcinomas, segmental neurofibromas, linear multiple trichoepithelioma or syringoma, or segmental leiomyomas [14]. Such cases should be categorized as examples of “nevoid” arrangement reflecting mosaicism, rather than as representing true nevi.

The value of a new classification of the shapes and patterns of nevi might be questioned by those who think that the currently existing “archetypical” patterns described by one of the co-workers of this study are sufficient. In our opinion, the present delineation of shapes and patterns may be valuable for several reasons: 1) the proposed new shapes in the form of “round or oval”, “patchy indented” and “teardrop/triangular” configurations can be considered to reflect mosaicism, thus expanding our view of nevi as mosaic disorders; 2) this new classification reinforces the patterns initially described by Happle [8] and highlights the idea that nevi displaying these “archetypical” patterns may also appear as small lesions that can only be categorized by their shape; 3) there is a good correlation between different types of nevi and the shapes or patterns they assume; 4) new more complex mosaic configurations are proposed in the form of the agminated pattern and the diffuse patchy pattern; 5) all nevi reviewed could be assigned to one of these shapes or patterns; and 6) the concept that all nevi are mosaic lesions with a recognizable pattern may lead to a better understanding of the origin and nature of possibly associated extracutaneous anomalies.

Conclusion

In an attempt to better understand the mosaic nature of nevi, we have assigned such lesions to a new classification of shapes and patterns. From this study we conclude that the different patterns are “archetypical” in a way that they are the perfect or ideal model of certain types of nevi, whereas the newly delineated shapes are subordinated to these patterns and can be taken as “non-archetypical”. We emphasize that nevi derived from a particular cell type may become manifest in many different shapes and patterns. For example, nevi originating from the pigmentary cell line may show a round/oval, patchy indented, or teardrop shape, whereas larger lesions may be arranged in a Blaschko-linear, block/flag-like, phylloid, garment-like, agminated, or diffuse patchy pattern.

From a heuristic point of view, it seems worthwhile to assign a given dermatological entity to one “archetypical” pattern. For example, giant melanocytic nevi may then be assigned to the garment-like pattern, whereas nevus sebaceus would be categorized as belonging to the Blaschko-linear pattern.

Our work gives more consistency to the idea that nevi reflect mosaicism, because they have been shown to follow a repetitive, mosaic pattern, which can be archetypical or not. It is possible, and even likely, that some other distinct shapes or patterns of nevi will be delineated in the future. For the time being, however, it seems appropriate to assign a given nevus to the shapes and patterns as presented in this article. Most probably, further molecular studies performed in various nevi will provide additional evidence that all of these lesions reflect mosaicism.

References

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12 Happle R. Lyonization and the lines of Blaschko. Hum Genet 1985; 70: 200-6.

13 Happle R, Assim A. The lines of Blaschko on the head and neck. J Am Acad Dermatol 2001; 44: 612-5.

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