REVIEW ARTICLE

Eponyms, Morphology, and Pathogenesis of Some

Less Mentioned Types of Melanocytic Nevi
Angel Fernandez-Flores, MD, PhD

Synonyms
Abstract: Some types of melanocytic nevi are seldom mentioned in Halo eczema nevus; halo dermatitis nevus (not to be
the literature and are therefore less well known. In the current study, mistaken for halo nevus or Sutton nevus).
we focus on the concepts of eponyms, synonyms, clinical presentation,
and morphology of the following types: Meyerson nevus, cockarde Eponym
(or cockade) nevus, Hori nevus, Sun nevus, Hidanos nevus, Duperrat In 1971, Lawrence B. Meyerson published a report in
nevus, Spark nevus, nevus spilus, eclipse nevus, Kerl nevus, and the Archives of Dermatology on the cases of 2 men with
Kopf nevus. benign melanocytic nevi surrounded by eczema.1 The doctor,
Key Words: Meyerson nevus, cockarde nevus, ancient nevus, Hori from the Dermatology Service of the Brooke General Hospital
nevus, Duperrat nevus in San Antonio, Texas, remarked how ‘‘a similar disorder has
not been noted in the literature.’’ The cases corresponded with
(Am J Dermatopathol 2012;34:607–618) localized eczematous eruption surrounding an acquired nevi,
with parakeratosis, spongiosis, acanthosis, and a lymphocytic
perivascular infiltrate in the dermis. The lesions were resolved
under topical steroid therapy, leaving the unchanged nevi.1
INTRODUCTION
Medical literature is full of reports on many types of Clinical Presentation
melanocytic nevus. Some of them, such as the Spitz nevus, are This type of nevus is mainly found in young adults,1–3
the focus of modern studies on differential diagnosis with with an even distribution in both genders,2,4–6 although
malignant conditions. Some nevi, such as Clark nevus, are well pediatric cases,4 or cases in adolescents,7 have been reported.
known for controversial reasons, whereas others, like nevi of Meyerson’s phenomenon can also be found on nevi of aged
Ota and Ito, are so classic that although they are not common patients.2 Meyerson nevus mainly appears on the trunk or on
in clinical practice, they are known by all medical students. proximal extremities,1,2,4,5 although it can present on virtually
However, several other types of melanocytic nevi are not any location of the skin.2,4,6,8–11 Usually, it is asymptomatic,
so frequently mentioned in the literature and are, therefore, not although some cases are pruritic.2,3,11,12
so widely known by general pathologists. In some cases, even It presents as a symmetrical area of erythema and scale
the concepts regarding those nevi are not clearly approached in encircling a central lesion, which is the melanocytic nevus
every report mentioning those nevi. Some definitions of these (Fig. 1).12 The eczematoid changes can be seen either over the
nevi were introduced in the literature not so long ago, which nevus or on the adjacent skin.5 The phenomenon spontane-
contributes to certain lack of knowledge about them. The ously resolves in some months, although the nevus usually
current report focuses on several of these types of nevi and persists.1 However, depigmentation of the nevus, after the
aims to clarify some aspects of their definition, eponyms, eczema is gone, has also been described.4,5 Sometimes,
clinical presentation, and morphology. Meyerson nevus can progress to a Sutton nevus 3,9 or vice
versa.3 Sutton nevus and Meyerson nevus have also been
reported to coexist in the same patient.8,10,13 Some researchers
MEYERSON NEVUS even consider that Sutton nevus and Meyerson nevus are
opposite poles of the same immunologic reaction spectrum.10
Concept Although Meyerson’s phenomenon is usually an isolated
Meyerson nevus is generally caused by an eczematiza- finding,2,8,9,14,15 it is occasionally associated with atopic
tion of the center of the melanocytic nevus and/or of its dermatitis.4 Meyerson’s phenomenon has been associated
periphery. with congenital or acquired nevi, as well as with other
cutaneous nonmelanocytic lesions, such as seborrheic kera-
toses,16 basal cell carcinomas, squamous cell carcinomas,6
From the Department of Pathology, Hospital El Bierzo, Ponferrada, Spain. dermatofibromas,17 and insect bites.3
The author declares no conflicts of interest.
Reprints: Angel Fernandez-Flores, MD, PhD, Servicio de Anatomı́a Morphology
Patologica, Hospital El Bierzo, Medicos sin fronteras 7, 24411,
Fuentesnuevas, Leon, Spain (e-mail: [email protected]). Changes manifest themselves as a subacute eczematous
Copyright Ó 2012 by Lippincott Williams & Wilkins dermatitis with spongiosis, sometimes with vesiculation,

Am J Dermatopathol  Volume 34, Number 6, August 2012 www.amjdermatopathology.com | 607

Fernandez-Flores Am J Dermatopathol  Volume 34, Number 6, August 2012

COCKARDE (OR COCKADE) NEVUS

Concept
A benign targetoid nevus made up of a central
pigmented area, an intervening nonpigmented zone, and
a peripheral pigmented ring.
The Term
A cockarde (or cockade) is a rosette or knot of ribbons,
commonly used as an ornament worn on a hat as a badge. Such
ornaments have often had historical or military connotations
(Fig. 3). In the same way that ornaments commonly have more
than one color, the appearance of this nevus is targetoid.
The cockarde nevus was first described in the American
literature by Mehregan and King,20 who remarked how they
had been ‘‘unable to locate any reference to this clinical form
[of nevi]’’ and called them ‘‘target-like pigmented nevi.’’ In
1974, Happle (in German literature) referred to this type of
nevus as ‘‘Kokardennaevus.’’21
Synonyms
Target-like nevus.
Clinical Presentation
Cockarde nevus is usually reported in children or young
patients.21–23 The original description by Mehregan and King20
was on a 20-year-old woman. These nevi commonly present as
multiple lesions.22,23 In the report by Mehregan and King,20 the
patient had 24 lesions, but cases with up to 30 lesions have
been reported.22 The most common location has been the
trunk.20–24
It presents as a central dark papule with an intervening
nonpigmented zone and peripheral, pinpoint pigmented
papules arranged in a circle surrounding the central lesion.23
The entire lesion has a global target-like appearance
FIGURE 1. Clinical picture of Meyerson nevus with the mimicking a cockarde. However, it should be remarked that
eczematour erythematous area surrounding the central nevus. not all nevi with a target-like appearance are cockarde nevi.
Examples of clinically target-like blue nevi (either common or
cellular)25 or targetoid melanoma26 have been reported.
acanthosis, and parakeratosis, with an interstitial lymphohis- Curiously, some have presented as a series of cockarde nevus
tiocytic inflammatory infiltrate in the superficial dermis in with spinal dysraphism.27
contact with the nevus, and some exocytosis of the
lymphocytes (Fig. 2).5,8,11 Some eosinophils can be evidenced Morphology
as well.12 The infiltrate is mainly CD4+, with a small The central lesion is usually made up of a junctional or
population of CD8+ cells.18 This is in contrast with the compound nevus with junctional activity extending out to the
inflammatory reaction caused in Sutton nevus, which is edge and eventually losing its pigment.23 The periphery can
predominantly CD8+.12 It is also different from the reaction show increased pigment in the dermis.24
caused in contact dermatitis, which usually presents IL-2
receptor–positive cells; these latter cells are not present in Pathogenesis
Meyerson nevus.18 Happle21 proposed an immune phenomenon or
sporadic periods of junctional activity during the growth
Pathogenesis phase, but he failed to demonstrate antibodies against the
Meyerson (1971) himself postulated this eruption to be basement membrane or the melanocytes21 in the patients’
pityriasis rosea located around the melanocytic nevus.1 It is peripheral blood. Some researchers have demonstrated that
accepted that the nevus is directly related to the eczematous nevus cells in the central and in the peripheral portions more
reaction because the excision of the former is accompanied by actively produced melanin,23 and others have demonstrated
the disappearance of the latter.15 Triggering factors, such as that only the nevus cells and keratinocytes at the periphery of
ultraviolet radiation,8 chemotherapy, or alpha-2B-interferon, the tumors stained for melanin.22 The authors of the latter
have been suggested.19 Even if a triggering antigen exists, it report demonstrated the presence of tyrosinase in epi-
has not been yet identified. dermal melanocytes of cockarde nevi; therefore, they were

608 | www.amjdermatopathology.com q 2012 Lippincott Williams & Wilkins

Am J Dermatopathol  Volume 34, Number 6, August 2012 Less Mentioned Types of Melanocytic Nevi

FIGURE 2. The eczematous changes in this Meyerson nevus can easily be seen in the epidermis above the intradermal nevus. Such
changes are characterized by parakeratosis, spongiosis, and exocytosis of lymphocytes. A perivascular superficial dermatitis can also
be seen.

theoretically able to synthesize melanin.22 However, because College and professor at the Faculty of Medicine, Kyushu
such cells did not stain for melanin, they interpreted University, Japan.
a blockage in the melanin synthesis.22Finally, the darker Their findings were published in 1984 in the Journal of
peripheral ring is considered in some quarters to be the the American Academy of Dermatology. They described
result of increased pigment granules in the peripheral pigmented lesions in 22 patients, of which 21 were women.
junctional cells.23 These cases were collected during a period of 7 years.28 The
lesions were symmetrical and presented in ‘‘the forehead,
temples, eyelids, cheeks, and/or nose.’’28 Therefore, according
HORI NEVUS, SUN NEVUS, AND
to some, the term should not be used for lesions presenting in
HIDANOS NEVUS an asymmetric distribution.29 Hori et al28 also remarked how
Concept of Hori Nevus the lesions were ‘‘similar clinically to nevus of Ota, female
Hori nevus is described as a bilateral symmetric facial melanosis ., or melasma’’ but that they ‘‘. are not
acquired dermal melanocytosis with no mucosal involvement. observable in ocular and mucosal membranes.’’
However, a group led by Akira Hidano of the Tokyo
Eponym Metropolitan Police Hospital in Japan published a case of
In the early 1980s, a team of researchers led by Yoshiaki a ‘‘bilateral nevus of Ota’’ in 1965 on a Japanese man aged 23,
Hori presented a new type of nevus. Hori was chairman of with no mucosal involvement. Hidano et al30 noted that the
the Department of Dermatology at the Yamanashi Medical lesion extended to ‘‘the right cervical, supraclavicular and

q 2012 Lippincott Williams & Wilkins www.amjdermatopathology.com | 609

Fernandez-Flores Am J Dermatopathol  Volume 34, Number 6, August 2012

acquired bilateral nevus of Ota-like macules34,35; acquired

symmetrical dermal melanocytosis.29
Synonyms for Hidanos nevus: acquired dermal mela-
nocytosis of the face and extremities.36

Clinical Presentation
Hori nevus is usually found in young and middle-aged
Asian women. Sun et al31 found 102 females with the
phenomenon and only 8 males (male–female ratio of 1:12).
Some reports have curiously demonstrated a strong family
history.31,37 The age at onset ranges from 6 to 72 years,35,37–40
with a peak of incidence at 30 years.37
This phenomenon usually presents as blue, brown, or
gray speckled macules on the face on both sides of the
forehead, temple eyelids, malar area, alae of the nose, and the
root of the nose. Involvement of the upper lip has also been
described.37
The lesions become progressively more confluent and
gray over time. They also tend to get darker with chronic sun
exposure and during pregnancy.37,39 The color of the nevus
often varies with menstruation.41,42 Contrary to what happens
in other types of facial nevi, no ocular or mucosal involvement
has been seen.

Morphology
FIGURE 3. Detail of the painting ‘‘Farewell to an Insurgent’’ by Spindle-shaped c-kit–positive melanocytes with brown
Artur Grottger (1866), in which the woman is putting granules are evident in the middle and upper dermis33
a cockade to the man. (in contrast to Ota nevus, which has melanocytes in the upper
and lower dermis). In the ultrastructural study, melanosomes
are in stages II–IV of melanization,28 mostly in stage IV.29 This
scapular region and also on the upper third of the presternal has already been recognized by the original authors as
area.’’ The authors interpreted this as ‘‘an extremely extensive ‘‘actively melanin-synthesizing dermal melanocytes.’’28 The
case of pigmentary anomaly, associating aberrant and interactions have been described as between mast cells and the
persistent Mongolian spots, extensive nevus of Ito and melanocytes.29 Melanophages are also evidenced in smaller
bilateral nevus Ota [sic] with exceptional involvement of the numbers than melanocytes, which probably contributes to the
lips.’’30 However, in their histological description of the lesion, gray color.29
they mention ‘‘cord-like or spindle-shaped, heavily pigmented
cells in the upper corium,’’ which is not consistent with what is Pathogenesis
observed in an Ota nevus. Hori postulated 4 possible mechanisms: epidermal
Sun nevus is named after Chee-Ching Sun of the melanocyte migration (‘‘dropping off’’), hair bulb melanocyte
National Taiwan University Hospital in Taipei, who in 1987 migration, reactivation of immature resting dermal melano-
reported in the British Journal of Dermatology a study of cytes, and the manifestation of latent dermal melanocytosis
110 cases of a new entity called ‘‘naevus fusco-caeruleus in some areas of the face, triggered by inflammation, atrophy,
zygomaticus.’’31 Sun et al31 mentioned how this condition had or degeneration of epidermis and dermis by aging or some
probably been considered as ‘‘an unusual type of naevus of Ota unknown cause.28 Some of the observations made so far
by some Japanese dermatologists,’’ as they quoted the support some of these mechanisms: darkening with sun
publications of Hidano et al30 and Hori et al.28 None of the exposure or pregnancy would support a reactivation of latent
patients in the study by Sun et al31 showed mucosal melanocytes. Moreover, as some have remarked, immature or
involvement either. nonpigmented melanocytes are present in the dermis adjacent
With this information, it seems that the concept of the to Hori nevus29,43 and in the dermis of normal skin from people
Hori nevus has to be restricted to a symmetrical bilateral without this type of nevus.29
acquired dermal melanocytosis involving the face. Similar Several factors stand out as potential stimulants.
bilateral lesions with extrafacial involvement32,33 should Hormones might play a role, given the female predominance
probably be renamed as Hidanos nevus. Sun nevus seems to and the variation in nevus color with menstruation.41 Trauma
be the same condition as Hori nevus. or inflammation might also be potential triggers because
darkening of the nevus has sometimes been associated with
Synonyms such events.44 Also, sun damage might play an important role
Synonyms for Hori nevus and Sun nevus: naevus fusco- since many cases are located on sun-exposed areas of the
caeruleus zygomaticus31; acquired dermal melanocytosis29,33; body.45

610 | www.amjdermatopathology.com q 2012 Lippincott Williams & Wilkins

Am J Dermatopathol  Volume 34, Number 6, August 2012 Less Mentioned Types of Melanocytic Nevi

DUPERRAT NEVUS Pathogenesis

Some researchers believe that subnevic folliculitis and
Concept inflammation due to an epidermal cyst underneath the nevus
An inflammation underneath a melanocytic nevus caused should not be put in the same group.48 They cite the fact that
by a rupture of a folliculosebaceous unit or an epidermal cyst. subnevic folliculitis is not such a rare finding because it is
Eponym mainly located on the face and is often caused by plucking of
In 1954, Bernard Duperrat published 11 cases of the hairs. On the contrary, they argue that a true epidermal
intradermal nevi associated with ruptured epidermal cyst cyst underneath a nevus is a rare event.48 The extruded
(89 cases) and folliculosebaceous units, causing a ‘‘deep products from the cyst or from the hair follicle, induce an
subnevic inflammation.’’46 Duperrat studied medicine in Paris, inflammatory reaction. Some researchers have demonstrated
where he later became a dermatologist. There, he worked in that the contents of epidermal cysts occur mainly through the
the Laboratory of Dermatopathology under Professor alternative pathway. Chemotactic C5a anaphylatoxin pro-
H. Gougerot. In 1952, he joined the Dermatology Department duced by the cyst contents after contact with serum most
at l’Hôspital Saint Louis, where he became Associate likely plays a significant role in the initiation and aggravation
Professor of Dermatology in 1953 and Chairman of of inflammation in ruptured epidermal cysts.49 Microorgan-
Dermatology in 1964.47 Today, the term ‘‘Duperrat nevus’’ isms that are usually located in the follicle (Pityrosporum
is accepted for all melanocytic nevi, even if not intradermal. ovale, Propionibacterium acnes, and Staphylococcus epi-
dermidis) also play a role in the development of the
Morphology inflammation.49 Some recent studies have demonstrated that
Underneath the nevus, an inflammatory response can be in aerobic and anaerobic bacterial culture, the rate of bacterial
found that includes a granulomatous response to the products growth and the recovered anaerobes are significantly greater
extruded from the cyst or from the folliculosebaceous unit in the inflamed epidermal cysts than in the noninflamed
(Figs. 4, 5). epidermal cysts.50

FIGURE 4. Duperrat nevus showing a granulomatous reaction beside a folliculosebaceous unit. Keratin lamellae can be seen
attached to the giant cell reaction and the latter is close to the melanocytic intradermal nests.

q 2012 Lippincott Williams & Wilkins www.amjdermatopathology.com | 611

Fernandez-Flores Am J Dermatopathol  Volume 34, Number 6, August 2012

FIGURE 5. Variant of Duperrat nevus associates an infundibular cyst with a prominent inflammatory reaction intermingling with the
melanocytic population.

SPARK NEVUS Clinical Presentation

These nevi are more commonly found in young women,
Concept mainly in the trunk and in the lower extremities.52,56 The
A melanocytic lesion with features of both Clark nevus clinical appearance often seems like malignancy due to the
and Spitz nevus. evidence of one or more of the following features: irregular
border, mild asymmetry, more than one color, and big size
Synonyms (more than 5 mm).
Dysplastic Spitz nevus.51
Morphology
The Term The lesions are commonly symmetric and well circum-
The term ‘‘spark’’ is a contraction of ‘‘Spitz’’ and scribed (Fig. 6, top left), with melanocytes mainly found at
‘‘Clark’’ and was first attributed to A. Bernard Ackerman,52 the tips of the rete ridges. The melanocytic cells are present in
who is responsible for many revolutionary concepts in the nested and/or lentiginous array with focal pagetoid scatter
field of dermatopathology. Author of more than 60 books of melanocytes. The spindled and/or epithelioid melanocytes
and 700 scientific articles, he is also founder of 2 journals show bridging of nests (Fig. 6, middle). In contrast to classic
on dermatopathology.53,54 The term ‘‘Ackerman nevus’’ would Spitz nevus, Spark nevus shows a parallel arrangement of the
not, however, be appropriate because Ackerman himself spindle melanocytes to the surface of the epidermis. Some
promoted the eponym to refer to the superficial congenital clefts around the nests are found (Fig. 6, top right). Underlying
melanocytic nevus.55 fibroplasias is common (Fig. 6, bottom). The latter is not so

612 | www.amjdermatopathology.com q 2012 Lippincott Williams & Wilkins

Am J Dermatopathol  Volume 34, Number 6, August 2012 Less Mentioned Types of Melanocytic Nevi

FIGURE 6. Spark nevus as a symmetric well-demarked lesion (top left). Some clefts are found around the melanocytic nests (top
right). There is bridging between the melanocytic nests, but the cellular arrangement is parallel to the epidermis, opposite to what
is commonly seen in Spitz nevus (vertical arrangement). Underlying fibroplasias is common in this type of nevus (bottom).

q 2012 Lippincott Williams & Wilkins www.amjdermatopathology.com | 613

Fernandez-Flores Am J Dermatopathol  Volume 34, Number 6, August 2012

common when the epithelioid component is predominant.

Lateral extension of the junctional component56 also exists,
although the lesions usually end in nests at the periphery.
Kamino bodies are common. Occasional mitoses can be seen,
although they are never prominent. Upward scatter of single
melanocytes or clusters of melanocytes are evident in
a few foci.
Some of these nevi can be pigmented56 and they have
been called ‘‘pigmented spindle cell nevi with dysplastic
changes.’’57
In summary, this nevus combines features of Clark
nevus (lentiginous proliferation, bridging of nests, fibroplasia,
and lateral extension of the junctional component) with
features of Spitz nevus (spindle or epithelioid cells, clefts
around nests, and Kamino bodies).

NEVUS SPILUS
Concept and the Term
The term spilus comes from the Greek ‘‘spı́lo§
(spilos),’’ which means ‘‘spot.’’ However, the term must have
been used to refer to a uniformly pigmented macular lesion58,
that is better known as ‘‘café au lait’’ macule. In occidental
literature, in contrast to Japanese literature, the term was
more commonly used to refer to a ‘‘spots on a spot’’ nevus.
For instance, in 1928, Estabrook59 reported 5 generations of
a family with such speckled types of lesions. Ito and Hamada
in 1952,60 and Cohen in 1970,61 contributed to the
reinforcement of the term. However, some old descriptions
of nevus spilus with hypertrichosis could actually be what we
would currently know as Becker nevus.62 FIGURE 7. A typical example of a nevus spilus, with multiple
pigmented macules or papules within a lightly pigmented
Synonyms macular background on the back of a white patient.
Speckled lentiginous nevus; spots on a spot; zosteriform
lentiginous nevus.
ECLIPSE NEVUS
Clinical Presentation
It commonly appears in childhood, although it can Concept
appear at any age. The lesion, which has a predilection for A benign melanocytic nevus with a peculiar clinical
whites, can occur anywhere on the body, although it is more morphology evoking an eclipse of the sun.
common on the torso and extremities.63
This nevus commonly appears as a solitary lesion made The Term
of multiple pigmented macules, or papules, within a lightly The term ‘‘eclipse nevus’’ was first coined by Prof. Seth
pigmented macular background (Fig. 7). It commonly Orlow and described in an article by Schaffer et al,71 from
progresses to more noticeable red–brown macules and papules Yale, in 2001, referring to ‘‘a type of melanocytic naevus that
over the years.64 is two-tone and has an irregular outline, but is benign.’’ The
Three clinical types have been distinguished: small/ term was chosen ‘‘as means of evoking a visual image for
medium size, giant, and zosteriform.63 When linear, the lesion the clinician.’’71 The authors tried to emphasize the ‘‘irregular,
usually appears along the lines of Blaschko.65 A clinical form often stellate, appearance’’ of the brown rim, which looked
involving the upper and lower eyelids has also been reported.66 like the sun’s rays.
Morphology
The brownish back grown is due to skin with melano- Clinical Presentation
cytic hyperplasia, a lentigo simplex, or a nevus. The darker The most common location is the trunk but other
speckle areas show morphology of either junctional or com- locations are possible, such as the arms and legs. The nevus
pound nevus.61,63,67,69 Spitz nevi or blue nevi lesions can commonly presents in people in their 30s, although this type of
sometimes be seen.69 Cases with cytologic atypia have been nevus has also been described in the scalp of children.72
described70 and also cases in which the speckles were It shows a tan center and a brown peripheral rim. The latter
melanoma.64 shows an irregular outline, sometimes with a stellate

614 | www.amjdermatopathology.com q 2012 Lippincott Williams & Wilkins

Am J Dermatopathol  Volume 34, Number 6, August 2012 Less Mentioned Types of Melanocytic Nevi

appearance and other times with a discontinuous pattern. same patient74 and postulates a similar pathogenic process for
These features are usually clinically stable. both types.
Morphology
The nevus is fairly symmetrical and shows a flat KERL NEVUS
orientation, with a ‘‘junctional shoulder’’ and an extension Concept
of the junctional component beyond the intradermal compo-
A benign melanocytic neoplasia with degenerative
nent.71 The junctional shoulder shows prominent pigmenta-
cellular changes. Probably, the simplest definition is that
tion, although there is decreased junctional pigmentation
given by Kerl et al75: ‘‘it is fundamentally a long-standing
over the central intradermal component. Prominent fibrosis
Miescher’s nevus whose asymmetry at scanning magnification
of the papillary dermis in the central portion of the lesion is
is the result of the presence of two populations of melanocytes
sometimes seen. Maturation is evidenced, and there are no, or
and of various stromal changes.’’
only occasional, dermal mitoses. Apparently, some would have
noticed dysplastic changes in this type of nevus,73 but the
authors of the original report did not notice such a change.71 Eponym
Dr Helmut Kerl is a professor of dermatology at the
Pathogenesis Dermatology Department of the Medical School of Graz
The ultimate mechanism of development is unknown. University in Austria. He is also an invited professor at the
As in other cases of depigmentation in nevi, an immunologic Dermatology Department of the University of Zurich in
phenomenon has been suggested.74 Some of the literature Switzerland and a former president of the International Society
describes eclipse nevi associated with cockarde nevus in the of Dermatopathology.

FIGURE 8. Kerl nevus. The two populations of melanocytic cells are evident even at a low power (top left). The first population is
made of small round cells (top right). The second population is made of larger cells with certain atypia (bottom left). Concentric
fibrosis around blood vessels is a typical feature (bottom right).

q 2012 Lippincott Williams & Wilkins www.amjdermatopathology.com | 615

Fernandez-Flores Am J Dermatopathol  Volume 34, Number 6, August 2012

FIGURE 9. Kopf nevus is made up of a symmetrical melanocytic lesion with an inflammatory halo reaction. The melanocytic
population is similar to that found in a Spitz nevus.

In 1993, Dr Kerl was the first author of an abstract bluish color. As long as we know, these nevi show a benign
presented by Graz University at the 31st Annual Meeting of clinical behavior,77 the main concern is not to misdiagnose
the American Society of Dermatopathology and later them as melanoma.
published in the Journal of Cutaneous Pathology.76 The
concepts of the article were emphasized in later publications Morphology
on the subject.75,77 In the original abstract, they described
Commonly, they are exo-endophytic lesions involving
19 examples of an ‘‘unusual variant of melanocytic nevus.’’76
the dermis and sometimes the hypodermis. As a rule, the
Synonyms epidermis is spared.75 The lesion is well circumscribed but
Pleomorphic melanocytic nevus with degenerative asymmetric because it is made of 2 population of cells. One
changes; ancient nevus. population has large pleomorphic nuclei and shows senes-
cence changes, including atypia, hyperchromasia, prominent
Clinical Presentation nucleoli, abundant cytoplasm, and multinucleation (resem-
Kerl nevus is mainly located on the face, especially on bling Spitz cells). These large melanocytes are arranged in
the cheek or ear, trunk, and extremities of middle-aged to older nests, nodules, and sheets. The other population is made of
people of both sexes with a median age of 50 years.77 It is small monomorphous cells (Fig. 8). The small round cells
commonly a dome-shaped lesion of brownish, reddish, or mimic those found in congenital nevi and they are placed

616 | www.amjdermatopathology.com q 2012 Lippincott Williams & Wilkins

Am J Dermatopathol  Volume 34, Number 6, August 2012 Less Mentioned Types of Melanocytic Nevi

above, below, or at the lateral margins of the clusters with mainly made of small lymphocytes, occasional plasma cells,
pleomorphic cells. Sometimes, both populations are inter- and rare eosinophils. The lymphocytes are even CD4+ and
mingled. Some large cells in mitosis can be seen but CD8+ cells.81 Some cases may be accompanied by partial
commonly no more than 2 per section. regression of the nevus.
The nevus shows degenerative stromal changes, such as Contrary to Spitz nevi, Kopf nevi can show atypical
hemorrhage foci, dilated vessels, thrombi, pseudovascular cellular changes secondary to the inflammatory infiltrate, and
spaces, fibrosis, myxoid changes with occasional atypical less maturation.81
spindle cells, and the rims of sclerosis around vessels.
Due to the asymmetry, the atypias, and the presence of
mitoses, the main differential diagnosis is melanoma. CONCLUSIONS
However, the latter usually shows ‘‘more strikingly abnormal Accuracy with terms is crucial in any area but especially
melanocytes and many more mitotic figures, particularly in the in medicine. Therefore, the eponymic approach to nevi is one
lower portion of the neoplasm.’’75 of the most accurate ways to avoid unnecessary synonyms and
multiplicity of terms. In modern times, when social values are
Pathogenesis often exclusively centered on economical profit, eponyms are
It is not totally understood, but it has been speculated the least we can offer to those who altruistically committed
that the degenerative changes might be related to vascular their personal time and effort to medicine.
alterations because they are more intense around zones of
hemorrhage.77
ACKNOWLEDGMENTS
KOPF NEVUS The author would like to thank Dr J. M. Montero,
a dermatologist at the Clinica Ponferrada, for supplying the
Concept clinical pictures of nevus spilus and Meyerson nevus and
A Spitz nevus with halo reaction. would also like to thank Dr Suarez-Peñaranda, a dermatopa-
thologist from the University Hospital of Santiago de
Eponym Compostela, Spain, for lending a slide of a Kerl nevus.
Kopf et al78 in 1965 reported a case of ‘‘melanoma
juvenile’’ with halo reaction in a series of 59 cases of halo nevi.
Kopf was a member of the Skin Cancer Unit of the Department REFERENCES
of Dermatology at New York University Medical Center and 1. Meyerson LB. A peculiar papulosquamous eruption involving pigmented
Associate Professor at the New York University Schools of nevi. Arch Dermatol. 1971;103:510–512.
2. Cook-Norris RH, Zic JA, Boyd AS. Meyerson’s naevus: a clinical and
Medicine. histopathological study of 11 cases. Australas J Dermatol. 2008;49:
In 1968, Wayte and Helwig79 reported another case in 191–195.
a series of 105 cases of halo nevi. Some other single cases80 3. Elenitsas R, Halpern AC. Eczematous halo reaction in atypical nevi. J Am
have also appeared in literature. The main complete study of Acad Dermatol. 1996;34:357–361.
4. Rolland S, Kokta V, Marcoux D. Meyerson phenomenon in children:
the morphological evidenced in these nevi was published in observation in five cases of congenital melanocytic nevi. Pediatr
a series by Harvell et al.81 Dermatol. 2009;26:292–297.
5. Weedon D, Farnsworth J. Spongiotic changes in melanocytic nevi. Am J
Clinical Presentation Dermatopathol. 1984;6(suppl):257–259.
This type of nevus commonly presents in young adults, 6. Tegner E, Bjornberg A, Jonsson N. Halo dermatitis around tumours. Acta
mainly in teenagers.81 Clinical halo is only evident in Derm Venereol. 1990;70:31–34.
7. Schepis C, Siragusa M. The Meyerson phenomenon in a teenager.
a minority of the cases and only about a third of the patients Dermatol Online J. 2008;14:28.
refer to ‘‘certain changes’’ in the nevi,81 which usually 8. Petit A, Viney C, Gaulier A, et al. Coexistence of Meyerson’s with
corresponds to pigment variegation. The nevi are commonly Sutton’s naevus after sunburn. Dermatology. 1994;189:269–270.
located on the head, trunk, and upper extremities.81 9. Ramon R, Silvestre JF, Betlloch I, et al. Progression of Meyerson’s naevus
to Sutton’s naevus. Dermatology. 2000;200:337–338.
Morphology 10. Shifer O, Tchetchik R, Glazer O, et al. Halo dermatitis in children. Pediatr
They are symmetrical, well circumscribed, often wedge- Dermatol. 1992;9:275–277.
11. Tauscher A, Burch JM. Picture of the month–quiz case. Diagnosis:
shaped nevi, made of spindle and or epithelioid cells (Fig. 9).81 Meyerson phenomenon within a congenital melanocytic nevus. Arch
As in Spitz nevus, if mitoses are found, they are few and Pediatr Adolesc Med. 2007;161:471–472.
superficially located. 12. Rodins K, Byrom L, Muir J. Early melanoma with halo eczema
It should be mentioned that Spitz nevus can be (Meyerson’s phenomenon). Australas J Dermatol. 2011;52:70–73.
accompanied by a certain perivascular (or even focally 13. Brandt O, Christophers E, Folster-Holst R. Halo dermatitis followed by
the development of vitiligo associated with Sutton’s nevi. J Am Acad
arranged in a band) inflammatory infiltrate, which does not Dermatol. 2005;52:S101–S104.
imply an ‘‘halo’’ phenomenon. As is Spitz nevi, Kopf nevus 14. Kus S, Ince U, Candan I, et al. Meyerson phenomenon associated with
can display secondary epidermal changes, such as epidermal dysplastic compound nevi. J Eur Acad Dermatol Venereol. 2006;20:350–351.
hyperplasia, orthokeratosis or parakeratosis, wedge-shaped 15. Cox NH, Bloxham CA, Lawrence CM. Halo eczema—resolution after
excision of the central naevus alone. Clin Exp Dermatol. 1991;16:66–67.
hypergranulosis, and Kamino bodies. 16. Rosen R, Paver K, Kossard S. Halo eczema surrounding seborrhoeic
The inflammatory infiltrate of Kopf nevus is commonly keratoses: an example of perilesional nummular dermatitis. Australas J
symmetrical and permeates the full thickness of the nevus. It is Dermatol. 1990;31:73–76.

q 2012 Lippincott Williams & Wilkins www.amjdermatopathology.com | 617

Fernandez-Flores Am J Dermatopathol  Volume 34, Number 6, August 2012

17. Gallais V, Lacour JP, Perrin C, et al. Halo eczema around a histiocytofi- 49. Terui T, Rokugo M, Kato T, et al. Analysis of the proinflammatory
broma: the Meyerson phenomenon [in French]. Ann Dermatol Venereol. property of epidermal cyst contents: chemotactic C5a anaphylatoxin
1993;120:617–620. generation. Arch Dermatol Res. 1989;281:31–34.
18. Fernandez Herrera JM, Aragues Montanes M, Fraga Fernandez J, et al. 50. Kuniyuki S, Yoshida Y, Maekawa N, et al. Bacteriological study of
Halo eczema in melanocytic nevi. Acta Derm Venereol. 1988;68:161–163. epidermal cysts. Acta Derm Venereol. 2008;88:23–25.
19. Krischer J, Pechere M, Salomon D, et al. Interferon alfa-2b-induced 51. Toussaint S, Kamino H. Dysplastic changes in different types of
Meyerson’s nevi in a patient with dysplastic nevus syndrome. J Am Acad melanocytic nevi. A unifying concept. J Cutan Pathol. 1999;26:84–90.
Dermatol. 1999;40:105–106. 52. Ko CJ, McNiff JM, Glusac EJ. Melanocytic nevi with features of Spitz
20. Mehregan AH, King JR. Multiple target-like pigmented nevi. Arch nevi and Clark’s/dysplastic nevi (‘‘Spark’s’’ nevi). J Cutan Pathol. 2009;
Dermatol. 1972;105:129–130. 36:1063–1068.
21. Happle R. Cockade nevus. Unusual variation of nevus cell nevus [in 53. Kerl H, Burgdorf W. A. Bernard Ackerman (1936-2008): the most
German]. Hautarzt. 1974;25:594–596. important dermatopathologist of the 20th century, who transformed the
22. James MP, Wells RS. Cockade naevus: an unusual variant of the benign world of dermatopathology. Am J Dermatopathol. 2009;31:734–739.
cellular naevus. Acta Derm Venereol. 1980;60:360–363. 54. Weyers W. A. Bernard Ackerman-1936-2008. Am J Dermatopathol. 2009;
23. Guzzo C, Johnson B, Honig P. Cockarde nevus: a case report and review 31:740–761.
of the literature. Pediatr Dermatol. 1988;5:250–253. 55. Ackerman AB. Congenital nevus, superficial. In: www.derm101.com/
24. Warin AP. Cockarde naevus. Clin Exp Dermatol. 1976;1:221–224. quiz/study.aspx?id=q4001741. Accessed January 15, 2012.
25. Bondi EE, Elder D, Guerry Dt, et al. Target blue nevus. Arch Dermatol. 56. Shapiro M, Chren MM, Levy RM, et al. Variability in nomenclature used
1983;119:919–920. for nevi with architectural disorder and cytologic atypia (microscopically
26. Champion RH. An unusual melanoma. Br J Dermatol. 1964;76:347. dysplastic nevi) by dermatologists and dermatopathologists. J Cutan
27. Capella GL, Altomare G. Cockade nevi and spinal dysraphism. Int J Pathol. 2004;31:523–530.
Dermatol. 2000;39:318–320. 57. Barnhill RL, Barnhill MA, Berwick M, et al. The histologic spectrum of
28. Hori Y, Kawashima M, Oohara K, et al. Acquired, bilateral nevus of Ota- pigmented spindle cell nevus: a review of 120 cases with emphasis on
like macules. J Am Acad Dermatol. 1984;10:961–964. atypical variants. Hum Pathol. 1991;22:52–58.
29. Murakami F, Soma Y, Mizoguchi M. Acquired symmetrical dermal 58. Bulkley H. Lecture on the classification and diagnosis of disease of the
melanocytosis (naevus of Hori) developing after aggravated atopic skin. New York Med Gaz. 1842;2:97–100.
dermatitis. Br J Dermatol. 2005;152:903–908. 59. Estabrook A. A family with birthmarks (nevus spilus) for five generations.
30. Hidano A, Kajima H, Endo Y. Bilateral nevus Ota associated with nevus Eugen News. 1928;13:90–92.
Ito. A case of pigmentation on the lips. Arch Dermatol. 1965;91:357–359. 60. Ito M, Hamada Y. Nevus spilus en nappe. Tohuku J Exp Med 1952;55:44–48.
31. Sun CC, Lu YC, Lee EF, et al. Naevus fusco-caeruleus zygomaticus. Br J 61. Cohen HJ, Minkin W, Frank SB. Nevus spilus. Arch Dermatol. 1970;102:
Dermatol. 1987;117:545–553. 433–437.
32. Hidano A. Acquired, bilateral nevus of Ota-like macules. J Am Acad 62. Corsi H. Naevus spilus. Proc R Soc Med. 1936;29:928.
Dermatol. 1985;12:368–369. 63. Vaidya DC, Schwartz RA, Janniger CK. Nevus spilus. Cutis. 2007;80:
33. Mizoguchi M, Murakami F, Ito M, et al. Clinical, pathological, and 465–468.
etiologic aspects of acquired dermal melanocytosis. Pigment Cell Res. 64. Schaffer JV, Orlow SJ, Lazova R, et al. Speckled lentiginous
1997;10:176–183. nevus—classic congenital melanocytic nevus hybrid not the result of
34. Stanford DG, Georgouras KE. Dermal melanocytosis: a clinical spectrum. ‘‘collision’’. Arch Dermatol. 2001;137:1655.
Australas J Dermatol. 1996;37:19–25. 65. Welch ML, James WD. Widespread nevus spilus. Int J Dermatol. 1993;
35. Manuskiatti W, Sivayathorn A, Leelaudomlipi P, et al. Treatment of 32:120–122.
acquired bilateral nevus of Ota-like macules (Hori’s nevus) using 66. Sato S, Kato H, Hidano A. Divided nevus spilus and divided form of
a combination of scanned carbon dioxide laser followed by Q-switched spotted grouped pigmented nevus. J Cutan Pathol. 1979;6:507–512.
ruby laser. J Am Acad Dermatol. 2003;48:584–591. 67. Schaffer JV, Orlow SJ, Lazova R, et al. Speckled lentiginous nevus: within
36. Hidano A, Kaneko K. Acquired dermal melanocytosis of the face and the spectrum of congenital melanocytic nevi. Arch Dermatol. 2001;137:
extremities. Br J Dermatol. 1991;124:96–99. 172–178.
37. Ee HL, Wong HC, Goh CL, et al. Characteristics of Hori naevus: 68. Carmichael AJ, Tan CY. Speckled compound naevus. Clin Exp Dermatol.
a prospective analysis. Br J Dermatol. 2006;154:50–53. 1990;15:137–138.
38. Polnikorn N, Tanrattanakorn S, Goldberg DJ. Treatment of Hori’s nevus 69. Betti R, Inselvini E, Crosti C. Blue nevi and basal cell carcinoma within
with the Q-switched Nd:YAG laser. Dermatol Surg. 2000;26:477–480. a speckled lentiginous nevus. J Am Acad Dermatol. 1999;41:1039–1041.
39. Lam AY, Wong DS, Lam LK, et al. A retrospective study on the efficacy 70. Weinberg JM, Schutzer PJ, Harris RM, et al. Melanoma arising in nevus
and complications of Q-switched alexandrite laser in the treatment of spilus. Cutis. 1998;61:287–289.
acquired bilateral nevus of Ota-like macules. Dermatol Surg. 2001;27: 71. Schaffer JV, Glusac EJ, Bolognia JL. The eclipse naevus: tan centre with
937–941; discussion 941–932. stellate brown rim. Br J Dermatol. 2001;145:1023–1026.
40. Kunachak S, Leelaudomlipi P, Sirikulchayanonta V. Q-Switched ruby 72. Dysplastic nevi in children. Pediatr Dermatol. 1990;7:218–234.
laser therapy of acquired bilateral nevus of Ota-like macules. Dermatol 73. Rhodes AR. Dysplastic melanocytic nevi. In: Freedberg IM, Eisen AZ,
Surg. 1999;25:938–941. Wolff K, et al, eds. Dermatology in General Medicine. New York, NY:
41. Whitmore SE, Wilson BB, Cooper PH. Late-onset nevus of Ota. Cutis. McGraw-Hill Inc; 1999:1060–1079.
1991;48:213–216. 74. Yazici AC, Ikizoglu G, Apa DD, et al. The eclipse naevus and cockade
42. Mishima Y, Mevorah B. Nevus Ota and nevus Ito in American Negroes. naevus: are they two of a kind? Clin Exp Dermatol. 2006;31:596–597.
J Invest Dermatol. 1961;36:133–154. 75. Kerl H, Soyer HP, Cerroni L, et al. Ancient melanocytic nevus. Semin
43. Mizushima J, Nogita T, Higaki Y, et al. Dormant melanocytes in the Diagn Pathol. 1998;15:210–215.
dermis: do dermal melanocytes of acquired dermal melanocytosis exist 76. Abstracts of papers presented at the 31st Annual Meeting of American
from birth? Br J Dermatol. 1998;139:349–350. Society of Dermatopathology. J Cutan Pathol. 1993;20:529–579.
44. Stuart C. Naevus of Ota. Br J Dermatol. 1955;67:317–319. 77. Kerl H, Wolf IH, Kerl K, et al. Ancient melanocytic nevus: a simulator of
45. Mataix J, Lopez N, Haro R, et al. Late-onset Ito’s nevus: an uncommon malignant melanoma. Am J Dermatopathol. 2011;13:127–130.
acquired dermal melanocytosis. J Cutan Pathol. 2007;34:640–643. 78. Kopf AW, Morrill SD, Silberberg I. Broad spectrum of leukoderma
46. Duperrat B, Cuzin JC. [Gougerot-Hailey-Hailey’s benign familial acquisitum centrifugum. Arch Dermatol. 1965;92:14–33; discussion 33–15.
pemphigus]. Bull Soc Fr Dermatol Syphiligr. 1954;61:30–31. 79. Wayte DM, Helwig EB. Halo nevi. Cancer. 1968;22:69–90.
47. Mascaro JM. In memory of Professor Bernard Duperrat (1908-1982). Am 80. Yasaka N, Furue M, Tamaki K. Histopathological evaluation of halo
J Dermatopathol. 1984;6:515–517. phenomenon in Spitz nevus. Am J Dermatopathol. 1995;17:484–486.
48. Sanchez Yus E, Requena L. Nevus with cyst and nevus with folliculitis. 81. Harvell JD, Meehan SA, LeBoit PE. Spitz’s nevi with halo reaction:
Am J Dermatopathol. 1994;16:574–575. a histopathologic study of 17 cases. J Cutan Pathol. 1997;24:611–619.

618 | www.amjdermatopathology.com q 2012 Lippincott Williams & Wilkins