Diagnosis and management of nail pigmentations

Ralph Peter Braun, MD,

a
Robert Baran, MD,
b
Frederique Anne Le Gal, MD, PhD,
a
Stephane Dalle, MD,
c
Sandra Ronger, MD,
c
Roberta Pandolfi, MD,
c
Olivier Gaide, MD,
a
Lars Einar French, MD,
a
Paul Laugier, MD,
a
Jean Hilaire Saurat, MD,
a
Ashfaq Ahmed Marghoob, MD,
d
and Luc Thomas, MD, PhD
c
Geneva, Switzerland; Cannes and Lyon, France; and New York, New York
Longitudinal pigmentation of the nail is very common. The differential diagnosis varies from subungual
hematoma, to a fungal infection, to a melanocytic lesion (lentigo, nevus melanoma, etc.) to others. Often,
dermatologists do not feel at ease with these pathologies and management is often not clear. In many cases,
a biopsy is not helpful because an inadequate technique was chosen. The use of noninvasive techniques
such as dermoscopy has been described to be useful for the preoperative evaluation and the management
decision. Using these technique, one will be able to reduce the number of unnecessary surgeries and to
choose the most adequate biopsy technique. In this article, we will review the management, including
diagnosis as well as differential diagnosis of nail pigmentations and propose a new algorithm for the non
invasive diagnosis of nail pigmentation. ( J Am Acad Dermatol 2007;56:835-47.)
L
ongitudinal pigmentation of the nail is a
common presenting problem in general der-
matology. The differential diagnosis varies
from subungual hematoma to a fungal infection to
a melanocytic lesion (ie, lentigo, nevus, melanoma)
among others.
1
Dermatologists are often unsure
regarding their clinical diagnosis and lack confidence
in managing this condition. Furthermore, many
physicians are also reluctant to perform biopsies of
the nail matrix because the procedure is painful and
can result in permanent nail dystrophy. To compli-
cate matters, when a decision to biopsy is finally
rendered it is not uncommon for inadequate biopsy
specimens (eg, biopsy specimen of the nail plate
instead of the nail matrix) to be submitted to the
pathologist. This in turn compromises the ability
of the pathologist to render an accurate diagnosis.
Fortunately, the use of noninvasive techniques such
as dermoscopy can assist clinicians in correctly
evaluating and diagnosing nail pigmentation.
1
This
in turn will reduce the number of unnecessary
surgeries while helping physicians determine the
most appropriate biopsy technique and site of biopsy
if it were deemed necessary to biopsy. In this article,
we will review diagnosis and management of nail
pigmentation and propose a new algorithm for the
noninvasive diagnosis of nail pigmentation using
dermoscopy. We will describe and review the most
common biopsy techniques and the management of
melanoma of the nail matrix.
PRACTICAL APPROACH
Personal history
The physician should try and elicit a history as
to whether the pigment was present since birth or
acquired. Furthermore, the history of its duration,
history of what made the patient rst become aware
of it, and whether the patient has observed any
change in the nail lesion may provide clues for the
correct diagnosis. Many patients nd it difcult to
verbally describe what the nail looked like and how
it had changed over time. To overcome this problem
we have found it useful to allow the patient to draw
their impression of what the lesion initially looked
like and to draw the perceived change on a simple
schematic of a nail.
Specic questions pertaining to athletic activities,
recent trauma, physical exertion such as long hiking
trips, or the use of blood thinners such as aspirin and
warfarin may help in differentiating subungual hem-
orrhage fromother causes of nail pigment. Obtaining
a thorough history of any medications the patient is
ingesting may help identify drugs that may contrib-
ute to abnormal nail pigmentation.
2,3
Clinical examination
A complete examination must include the inspec-
tion of all 20 nails, periungual skin, and the oral
From the Pigmented Skin Lesion Unit, Department of Dermatol-
ogy, University Hospital Geneva
a
; Nail Disease Center, Cannes
b
;
and Departments of Dermatology at Ho tel Dieu, Lyon,
c
and
Memorial Sloan Kettering Cancer Center, New York.
d
Funding sources: None.
Conflicts of interest: None declared.
Reprint requests : Ralph P. Braun, MD, Department of Dermatology,
University Hospital Geneva, 24, rue Micheli-du-Crest, CHe1211
Geneva 14, Switzerland. E-mail: braun@melanoma.ch.
Published online March 6, 2007.
0190-9622/$32.00
2007 by the American Academy of Dermatology, Inc.
doi:10.1016/j.jaad.2006.12.021
835
mucosa. Examination of the oral and genital mucosa
can aid in correctly identifying Peutz-Jeghers syn-
drome
4
or the Laugier Hunziker syndrome.
5-8
The clinician should record the diameter of the
pigmentation at both the distal and proximal ends of
the nail. In addition, the color or colors and whether
the lesion has an overall homogeneous or heteroge-
neous appearance should be recorded.
Dermoscopy examination
Dermoscopy is a noninvasive method that helps
to narrow the differential diagnosis of pigmented
lesions of the skin and can assist in the recognition of
early melanoma.
9,10
It has been shown that dermo-
scopy increases the diagnostic accuracy compared
with clinical visual inspection. Dermoscopy uses an
immersion technique to render the stratum corneum
translucent. It also provides optical magnification.
11
Handheld devices called dermoscopes are easy to
use, and relatively inexpensive. Recently, dermo-
scopy has been described to be useful for the
evaluation and diagnosis of longitudinal melanony-
chia.
1,12
For the examination of the nail apparatus we
recommend the use of a gel such as ultrasound gel as
immersion medium
11
because its decreased viscosity
permits it to stay on the nail plate and fill any
concavities without rolling off. For the evaluation
of the pigmentation and to appreciate the thin lines
within the nail band, it is useful to vary the focus
of the device during the examination.
The following criteria can be evaluated by
dermoscopy.
1
Gray pigmented band composed of multiple
thin homogeneous grayish lines. This pattern is
usually a result of epithelial hyperpigmentation
without accompanying melanocytic hyperplasia
such as is seen in a lentigo, drug-induced pigmen-
tation, and ethnic pigmentation.
Brown pigmented band. The brown pig-
mented band is usually composed of multiple thin
brown lines. These lines can be regular or irregular
and are caused by melanocytic hyperplasia as seen in
a nevus or melanoma. There are two main patterns
seen within brown pigmented bands.
Regular pattern: Brown longitudinal parallel
lines with regular spacing and thickness. This pat-
tern is usually associated with a brown homogenous
color of the background band. The color of the
individual lines within the band can vary from light
brown to black. However, within any given band, the
lines will be composed of similar shades of brown
throughout the lesion. The spacing between the lines
is regular and the thickness of the lines is relatively
uniform throughout the band. The lines comprising
the band are oriented parallel to each other.
Irregular pattern: The band comprises multiple
longitudinal brown to black lines with irregular
spacing and thickness and disruption of
parallelism. This pattern is also associated with a
homogeneous brown pigmentation of the back-
ground nail band. However, the color of the indi-
vidual lines varies from light brown to black. In fact it
is common to observe many different colored lines
within the nail band. The lines vary in their thickness
and spacing. These lines, normally arranged parallel
to each other, can lose their parallelism and cross
into each other.
Dermoscopy of the free edge of the nail
Apart from the examination of the nail plate,
perionychium, and hyponychium, dermoscopy can
also be used to examine the free edge of the nail
plate. Examination of the free edge of the nail plate
can help determine whether the origin of the pig-
mentation is from the proximal or distal nail matrix.
13
As a result of the natural growth of the nail, pigment
produced in the proximal nail matrix will be incor-
porated into the upper portion of the nail plate and
pigment produced in the distal nail matrix will
appear in the lower part of the nail plate.
14
Thus,
when examining the free edge of the nail plate
particular attention should be placed on determining
whether the pigment is located in the upper or lower
portion the nail plate. This information, in turn, will
help isolate which part of the matrix may need to be
biopsied. If doubt exists as to the origin of the
pigment one can always obtain a nail clipping and
stain it with Fontana-Masson stain. This will help
determine the location of melanin within the nail
plate, which in turn will help determine whether the
pigment originated in the proximal or distal nail
matrix.
DIFFERENTIAL DIAGNOSIS OF
NAIL PIGMENTATION
The rst and most important task necessary to
diagnose the cause of nail pigmentation is to differ-
entiate whether the pigment is of melanocytic or
nonmelanocytic origin.
2
In most cases this task can
be accomplished by clinical inspection and exami-
nation with dermoscopy. In nonmelanocytic lesions
such as fungal infections or subungual hematoma,
the pigment tends to be distributed homogenously.
In melanocytic lesions, the melanin is found in
cellular inclusions,
10
which can be easily identified
as small granules less than 0.1 mm in diameter under
dermoscopy. Thus, if such granules are detected
under dermoscopy, the lesion should be considered
to be of melanocytic origin. A summary of the causes
of nail pigmentation is shown in Table I.
J AM ACAD DERMATOL
MAY 2007
836 Braun et al
Nail pigment of nonmelanocytic origin
(nail hyperchromia)
Exogenous pigmentation. There are multiple
causes that can lead to exogenous nail pigment.
Among the most frequent causes are contact with
chemical agents, topical application of therapeutic
agents such as silver nitrate or ethacridinlactat,
tobacco, and cosmetics such as henna and hair
dyes.
14-16
Subungual hematoma. Subungual hematoma
usually appears as a reddish to reddish-black pig-
ment depending on the age of the bleed. Dermo-
scopically one is usually able to identify small
reddish to reddish-black globules along the proximal
and lateral margins of the pigment (Fig 1). The distal
part will often have streaks of pigment. The pigment
is homogenous and no melanin granules can be
observed. The hematoma will progressively grow
out distally as the nail plate grows. It is important to
remember that a subungual hematoma can on rare
occasions be caused by an episode of hemorrhage or
caused by neovascularization within a tumor and,
thus, the presence of subungual blood should not be
used to rule out the diagnosis of melanoma. Hence,
any subungual hemorrhage that does not grow out
with the nail or that recurs at the same place requires
special attention and further examination including
radiograph of the distal phalange to exclude exostosis.
If the diagnosis cannot be determined by radiologic
studies then the lesion should be biopsied.
Fungal or bacterial infection. Nails infected
with Trichophyton rubrum (var nigricans) or with
dermatiaceous fungi such as Scytalidium dimidia-
tumare pigmented (Fig 2). Although difficult to treat,
the pigmentation will fade after successful treatment.
Nail pigmentation of melanocytic origin
As mentioned earlier, melanonychia striata will
reveal tiny melanin granules in the nail plate under
dermoscopy. Fig 3 shows melanin inclusions of the
nail plate at high magnification.
Nail pigmentation caused by epithelial
hyperpigmentation. The dermoscopy correlate
of epithelial hyperpigmentation is the presence of a
gray band composed of homogenous grayish lines
(Fig 4). Some authors prefer touse the termmelanotic
macule of the nail for this entity.
17
There are multiple
causes for epithelial hyperpigmentation and their
corresponding dermoscopic findings are listed below.
Histopathology of these entities shows a normal
number of melanin-containing melanocytes, which
is approximately 6.5 cell/mm of the basal layer
length.
18,19
Fig 1. Subungual hematoma. A, Clinical image. B, Der-
moscopic image showing homogenous brownish to red-
dish pigmentation without evidence of melanin inclusions.
Lack of melanin inclusions suggests that the condition is
caused by a nonmelanocytic lesion that has resulted in
nail hyperchromia. It is also common in subungual hem-
orrhage to see multiple reddish globules at the lateral and
proximal edge of the pigmented area. In addition, one can
see streaklike pigment at distal portion of pigmented area.
Table I. The most frequent causes for
longitudinal melanonychia
Focal melanocytic activation
Drug- and radiation-induced LM
Endocrine LM
LM associated with HIV infection
LM associated with inflammatory nail disorders
Laugier Hunziker syndrome
Nonmelanocytic nail tumors
Nutritional LM
Traumatic LM
Ethnic (racial) LM
Systemic lupus erythermatosus
Scleroderma
Melanocytic hyperplasia
Nevi of the nail matrix
Melanoma of the nail matrix
Other, less common causes
LM, Longitudinal melanonychia.
J AM ACAD DERMATOL
VOLUME 56, NUMBER 5
Braun et al 837
Longitudinal melanonychia as a result of focal
melanocytic activation: Drug- and radiation-
induced longitudinal melanonychia. On clinical ex-
amination, drug- and radiation-induced longitudinal
melanonychia can be seen as horizontal or longitu-
dinal bands or as diffuse nail darkening. It may affect
the nails of the hands and feet and it can involve one
or more digits.
19
Hyperpigmentation of the nail can
also be associated with a hyperpigmentation of the
skin. Dermoscopically homogenous grayish bands
composed of gray lines can be observed. It has been
reported to occur during or after treatment with
some drugs, chemotherapy, or radiation therapy.
This type of nail pigmentation is more commonly
seen in individuals with phototypes IV, V, and VI skin
than in individuals with fair skin. It usually appears
1 to 2 months after the beginning of the offending
agent. For example, it is estimated that 67% of
patients receiving AZT for the treatment of HIV
Fig 2. Fungal infection with Trichophyton rubrum.
A, Clinical image. B, Dermoscopy reveals a homogenous
brown to black pigmented band that is devoid of visible
melanin inclusions thus helping to correctly identify
hyperchromia to be of nonmelanocytic origin.
Fig 3. Dermoscopy of nail plate reveals, under high
magnication, tiny granules that correspond to melanin
inclusions. This feature suggests that the band is of
melanocytic origin.
Fig 4. Lentigo of the nail matrix. A, Clinical image. B,
Dermoscopy shows parallel homogenous grayish lines
that are juxtaposed to each other resulting in appearance
of homogenous gray pigmented band. The band does
have melanin inclusions that identifies it to be of melano-
cytic origin. However, gray color suggests that the diag-
nosis of this nail pigmentation is a result of epithelial
hyperpigmentation, which is occurring in the absence of
an accompanying melanocytic proliferation.
J AM ACAD DERMATOL
MAY 2007
838 Braun et al
infection will develop this form of nail pigmentation.
AZT-induced nail pigmentation usually develops be-
tween 8 weeks and 1 year after the beginning of the
therapy.
20
After the treatment is completed, the pig-
mentation will progressively fade over several months
but it may never completely disappear.
Endocrine longitudinal melanonychia. Nail pig-
mentation is a common feature of Addisons disease
(diffuse pigmentation and/or LM). It may also be
seen in Cushings syndrome, after adrenalectomy,
21,22
hyperthyroidism,
23
acromegaly, and during preg-
nancy.
19
The clinical and dermoscopy findings are
very similar to drug- and radiation-induced longitu-
dinal melanonychia. Endocrine longitudinal melano-
nychia mayaffect multipledigits. InAddisons disease,
longitudinal melanonychia is typically associatedwith
cutaneous and mucosal hyperpigmentation.
Longitudinal melanonychia associated with HIV
infection. In patients who are HIV positive, nail
pigmentation unrelated to AZT treatment has been
described.
24-26
The nail pigmentation usually affects
multiple nails and is associated with pigmented mac-
ules of the palms, soles, and mucous membranes. In
most reported cases the patients died within months
after the pigmentation appeared.
Longitudinal melanonychia associated with in-
ammatory nail disorders. This type of nail pig-
mentation occasionally develops in nails affected
by lichen planus,
27-29
onychomycosis,
12
chronic
radiodermatitis, pustular psoriasis, or Hallopeaus
disease. The pigmentation can be subtle at the
beginning and become clearly clinically evident as
the inflammatory process progresses. It is usually
associated with nail scarring and abnormalities of the
surface of the nail plate. The clinical and dermo-
scopy aspect of this entity is not specific.
Laugier Hunziker syndrome. Laugier Hunziker
syndrome is an acquired disorder of the pigment
system. The characteristic ndings include longitu-
dinal melanonychia, and macular pigmentation of
the lips, mouth, and anogenital area.
5,8
The nail
pigmentation may appear either as a single or double
band with a diameter between 1 to 2 mm or as
homogeneous pigmentation of the lateral nail plate.
Although the entire nail can become hyperpig-
mented, this is rather rare. This form of pigmentation
can be associated with a pseudo-Hutchinsons sign.
Longitudinal melanonychia as a result of
nonmelanocytic nail tumors. In rare cases non-
melanocytic nail tumors can activate melanocytes
resulting in the formation of a longitudinal pig-
mented nail band. This entity is most commonly
seen in association with Bowens disease.
30,31
Any
longitudinal melanonychia in the presence of a
nonmelanocytic tumor requires biopsy.
Nutritional longitudinal melanonychia. Pig-
mentation of the nails and the skin can be seen in
vitamin B12 or folate deciency.
32,33
The pigmenta-
tion tends to have a bluish-black color. The pigmen-
tation on the skin is accentuated over the knuckles
and the distal phalanges. Usually the diagnosis is
made based on laboratory examination. This type of
nail pigmentation is completely reversible after vita-
min B12 or folate administration.
Traumatic longitudinal melanonychia. Re-
peated trauma such as nail biting may induce nail
pigmentation. This is predominantly seen after re-
peated minor trauma such as picking, chewing,
breaking, or rubbing
34,35
of the proximal nailfold.
This entity is usually associated with visible abnor-
malities of the surface of the nail plate as a result of
the repetitive nail matrix injuries.
Frictional longitudinal melanonychia is mainly
seen on the toes and is often associated with foot
deformities, unsatisfactory footwear, or both.
36
This
type of nail pigmentation may gradually fade once
the repetitive trauma has subsided.
Ethnic nail pigmentation. Ethnic or racial nail
pigmentations are physiological longitudinal pig-
mentations of the nail or nails observed in dark-
skinned individuals with skin type V and VI.
37,38
They can present as single or more often multiple
bands involving one or more digits.
Systemic lupus erythematosus and scleroderma.
Longitudinal melanonychia has been reported in
Fig 5. Algorithm for dermoscopy diagnosis of nail pig-
mentation that is of melanocytic origin.
1,12
In the first step,
pigmentation should be evaluated for its color. Grayish
color is highly suggestive of focal melanocytic activation
in the absence of any melanocytic hyperplasia. Brownish
color is suggestive for melanocytic hyperplasia. For the
latter, lesions should be evaluated further to determine
whether the pigmented lines comprising the band are
regular, thus suggesting diagnosis of nevus or irregular
suggesting possibility of melanoma.
J AM ACAD DERMATOL
VOLUME 56, NUMBER 5
Braun et al 839
rare cases of systemic lupus erythematosus
39
and
scleroderma.
40
Nail pigmentation as a result of melanocytic
proliferation. The term melanocyte hyperplasia
should be reserved only for cases in which there is
an increased number of melanin-containing melano-
cytes ([6.5 cell/mm of basal membrane length)
withinthe basal andsuprabasal layer.
18
It is important
to remember that the architecture of the nail matrix
together with the dendritic morphology and the
suprabasal location of the nail matrix melanocytes
can histologically simulate an in situ melanoma.
Therefore, it is often difficult to differentiate between
melanocytic hyperplasia and an in situ melanoma
based on a partial biopsy specimen (ie, punch biopsy
specimen) of the nail matrix. Whenever possible it is
preferable to perform a complete longitudinal exci-
sion of the band and submit it to the pathologist.
Clinically longitudinal melanonychia appears as
a single dark pigmented band.
Dermoscopically the brown background pig-
mented band is composed of multiple thin lines
1,10
(Fig 5). Depending on the dermoscopy pattern
Fig 6. Nevus of nail matrix. A, Clinical image. B, Dermo-
scopy reveals melanin inclusions that correctly identifies
lesion to be of melanocytic origin. Thin lines within nail
band have regular pattern, are parallel to each other, and
have uniform thickness and spacing, all of which helps to
correctly diagnose this as a benign nevus.
Fig 7. Heavily pigmented nevus of nail matrix. A, Clinical
image revealing pseudo-Hutchinsons sign that results
from pigment in matrix becoming visible through rela-
tively translucent cuticule. B, Dermoscopy shows melanin
inclusions and regular dermoscopy pattern.
J AM ACAD DERMATOL
MAY 2007
840 Braun et al
(regular or irregular) the following diagnoses should
be considered.
Regular dermoscopy pattern: Nevi of the nail
matrix. Nail matrix nevi may be congenital or ac-
quired and are often seen in children and young
adults. Clinically they appear as longitudinal parallel
and homogenous pigmentation of the nail. Its color
varies fromlight brown (Fig 6) to dark brown to black
(Fig 7). Usually nevi of the nail matrix are heavily
pigmented and can clinically simulate melanoma.
A pseudo-Hutchinson sign in which one can see the
nail matrix pigment through a translucent cuticula is
very common in nevi of the nail matrix (Fig 7). The
most common conditions that can be associated with
a pseudo-Hutschinsons sign are listed in Table II.
Before dermoscopy the standard of care for many
cases of melanonychia striata was excisional biopsy
and histopathologic examination. However, because
the dermoscopic criteria for nail pigmentation have
now been well dened many cases can be managed
without biopsy. Dermoscopically, nevi of the nail
matrix show brown longitudinal parallel lines with
regular spacing and thickness. This has been de-
scribed as regular pattern.
1,10
Histopathology shows nests of melanocytes at the
dermoepidermal junction. Junctional nevi are more
frequently found than compound nevi. In general,
nail matrix nevi occur more often on ngernails than
on toenails.
Irregular dermoscopy pattern: Melanoma of the
nail matrix. Nail matrix melanoma has the same
incidence across all races. However, they are the
most frequent melanoma subtype in black and Asian
populations. It usually affects middle-aged or elderly
individuals. Nail matrix melanoma affects both n-
gernails and toenails and is most frequently found on
the index nger, the thumb, the large toe, or all of
them.
41
Approximately 50% of patients with nail
melanoma recollect preceding trauma.
1,2,41-43
A melanoma of the nail matrix usually presents
as a broad band, dark brown to black in color with
rather fuzzy or blurred lateral borders (Fig 8).
However, it is not uncommon to encounter subun-
gual melanoma with a light brown and thin band
(Fig 9). In melanomas that are growing rapidly the
lines within the band are not parallel and the diam-
eter of the entire band is wider at the proximal end
(Fig 10). In advanced stages dystrophy of the nail
plate or even loss of the nail plate can be observed
(Figs 11 and 12). This is a direct result of the
progressive destruction of the nail matrix by the
expanding tumor. Pigmentation of the nailfold cuti-
cle and the surrounding skin, known as Hutchinsons
sign, can frequently be observed (Fig 13).
On dermoscopic examination, a nail matrix mel-
anoma is characterized by the presence of brown
background coloration of the band. However, the
individual lines forming the band are irregular
Fig 8. Melanoma of nail matrix. A, Clinical image. B,
Dermoscopy shows melanin inclusions, brown to black
color, and irregular dermoscopy pattern with longitudinal
brown to black lines with variable spacing and thickness.
In addition, parallel line pattern is disrupted, which is
called disruption of parallelism.
Table II. Conditions that may produce
a pseudo-Hutchinsons sign
Addisons disease
AIDS
Bowens disease
Drug-induced pigmentation
Laugiers syndrome
Malnutrition
Nail matrix nevi (heavily pigmented)
Peutz-Jeghers syndrome
Racial pigmentations (phototypes V and VI)
Radiation therapy
Trauma
J AM ACAD DERMATOL
VOLUME 56, NUMBER 5
Braun et al 841
(irregular pattern). Irregular lines tend to have dif-
ferent degrees of pigmentation, varying thicknesses
and spacing
1
(Figs 8 to 10). In some areas they might
end abruptly and in others their parallelism is dis-
rupted. Blood spots can also be found in melanoma
and, thus, their presence does not automatically
suggest the diagnosis of a subungual hemorrhage.
The prognosis of nail matrix melanoma is generally
worse than for melanoma in other sites.
44
This may, in
great part, be because of delays in diagnosis. We have
the impression that nail matrix melanoma will lend
itself well to sentinel node biopsy, however, there is
currently no literature available addressing this issue.
45
WHICH LESIONS SHOULD BE BIOPSIED?
Any lesion with an irregular dermoscopy pattern
should be biopsied.
Independent from the morphology seen under
dermoscopy the following situations should alert the
physician to the possibility of malignancy
14,19
:
1. Isolated pigmented band on a single digit that
develops during the fourth to sixth decade of life.
Although melanoma can be seen in children it is
a very rare event.
2. Nail pigmentation that develops abruptly in a
previously normal nail plate.
3. Pigmentation that suddenly becomes darker or
larger or when the pigment becomes blurred
near the nail matrix.
4. Acquired pigmentation of the thumb, index fin-
ger, or large toe.
Fig 9. Melanoma of nail matrix. A, Clinical image. B,
Dermoscopy shows melanin inclusions, brown lines with
irregular dermoscopy pattern in which lines have varia-
bility in thickness, color, and spacing. In addition, there is
one line that disrupts normal parallelism.
Fig 10. Melanoma of nail matrix. A, Clinical image shows
that diameter of band is wider at proximal end as
compared with distal end. This usually occurs in rapidly
growing lesions. B, Dermoscopy reveals melanin inclu-
sions at periphery of lesion, brown to black color, which
suggests melanocytic proliferation, and irregular dermo-
scopy pattern with variability in line pigment, thickness,
and spacing. Parallelism is also disrupted.
J AM ACAD DERMATOL
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842 Braun et al
5. Pigment that develops after a history of digital
trauma
41
and in which subungual hematoma has
been ruled out.
6. Any acquired lesion in patients with a personal
history of melanoma.
7. If the pigmentation is associated with nail dys-
trophy including partial nail destruction or ab-
sence of the nail plate (Figs 11 and 12).
8. If pigmentation of the periungual skin (including
lateral nail folds) is found to be present (Hutch-
insons sign) (Fig 13). This includes pigment of
the cuticle or hyponychium.
Although we fully agree with most of the conclu-
sions made by Husain et al,
17
our opinions differ on
the importance of dermoscopy. Besides the 8 points
mentioned, we are of the opinion that dermoscopy
can greatly assist in the management decisions
regarding nail pigmentations.
BIOPSY TECHNIQUES FOR NAIL
PIGMENTATION
The choice of the biopsy technique for longitudi-
nal melanonychia depends on many factors such as
location, degree of suspicion,
14,19,46,47
and:
d
Presence of periungual pigmentation or Hutch-
insons sign (Fig 13).
d
Location of the band within the nail.
d
Origin of the band in the matrix. In other words,
is the origin of the pigment in the proximal or
distal nail matrix?
d
Width of the band.
1. Periungual pigmentation or pigmentation of
the nailfold should be viewed as a sign of
malignancy unless proven otherwise (Fig 13).
If there are no other factors to account for this
pigmentation, an adequate biopsy specimen,
Fig 12. Amelanotic melanoma of nail matrix associated
with complete nail dystrophy.
Fig 13. Melanoma of nail matrix. A, Clinical image with
pigment noted on nailfold, surrounding skin and hypo-
nychium (Hutchinsons sign). B, Dermoscopy shows dif-
fuse pigmentation with variable shades of brown; feature
commonly seen in acral melanoma.
Fig 11. Melanoma of nail matrix with destruction of the
nail plate.
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VOLUME 56, NUMBER 5
Braun et al 843
sometimes requiring en bloc excision down to
the bone, is necessary to rule out melanoma.
2. When the lateral third of the nail plate is in-
volved, then a lateral longitudinal excisional
biopsy specimen will provide adequate tissue
for the pathologist while giving the best chance
for a reasonable cosmetic outcome (Fig 14, C).
The incision should begin in the lateral nail
groove and should reach a depth close to the
underlying bone. The incision should extend the
entire length of the lateral nail margin and should
include 3 to 4 mm of the nail plate. This ensures
that a full-thickness specimen of the nail bed and
the nail matrix with its lateral horn is obtained.
The excision should extend from the just below
the distal interphalangeal joint to the hyponych-
ium. Slightly curved scissors or a surgical blade are
useful for separating the tissue from the bone. It is
preferred that this separating process should
begin at the distal (hyponychium) and proceed
proximally toward the nail matrix while always
maintaining contact with the bone. Sutures are
placed on the proximal nailfold and the hypo-
nychium. The lateral nailfold is sutured to the nail
plate with the nail equivalent of a half-burried (in
plate) horizontal matress suture so as to recon-
struct the lateral nailfold.
3. When the mid portion of the nail plate is in-
volved, the potential risk of postoperative nail
dystrophy is high. Most of the nail pigmentations
have their origin in the distal nail matrix, but to
choose the optimal biopsy method, it is neces-
sary to determine the origin of the melanocytes
responsible for the nail pigmentation. This infor-
mation may be obtained by sampling the free
edge of the nail plate and using a Fontana-
Masson stain as described previously. However,
dermoscopy can also be helpful in such cases. By
placing the dermoscope against the free edge of
the nail it may be possible to determine whether
the pigment is located in the upper part or the
lower part of the nail plate as was described
previously.
13
This noninvasive approach can
provide the desired information instantaneously,
often obviating the need for a nail clipping.
Pigment localized to the lower nail plate reflects
the presence of melanocytes in the distal nail
matrix. For the best cosmetic outcome, the bi-
opsy should only involve the distal matrix and
the proximal matrix should be preserved as
much as possible. In contrast, pigment localized
in the upper nail plate reflects melanocyte activ-
ity in the proximal nail matrix. In this scenario
the biopsy specimen must include the proximal
nail matrix and, thus, the risk of scarring and
nail dystrophy after excision is unavoidable. The
ultimate biopsy method decided on may also
depend on the width of the longitudinal
melanonychia.
14,19,46,47
d
A double punch biopsy is recommended for a
pigmented band that is less than 3 mm in diam-
eter (Fig 14, A). The first and larger punch, usually
6 mm, removes a circular defect in the nail plate.
The second and smaller punch, usually 3 to 4 mm,
biopsies the nail matrix through the circular nail
plate defect created by the first and larger punch.
The first 6-mm nail disk is then put back into its
originating place and serves as a dressing and
allows for quicker wound healing.
d
For a pigmented band with a diameter of 3 to
6 mm and in which the pigment is found to arise
from the distal nail matrix, a transverse matrix
biopsy is recommended (Fig 14, B). However, if
the pigment is found to arise from the proximal
nail matrix then an en bloc removal and repair
using a U flap is required (Fig 14, D).
Transverse matrix biopsy: when performing a
nail matrix biopsy it is important to try and main-
tain the distal curvature of lunula. To provide
access to the nail matrix necessitates the need for
two small oblique incisions to be made on each
side of the proximal nailfold. The fold is then
retracted to expose the matrix. The proximal third
Fig 14. Different biopsy techniques (after Haneke, with
permission). A, Punch biopsy of nail matrix, which can be
used for pigmented bands that are less than 3 mm in
diameter. B, Transverse biopsy of distal nail matrix, which
can be used for pigmented bands occurring in middle of
nail plate and having diameter of between 3 and 6 mm.
C, Longitudinal nail biopsy, which can be used to biopsy
lateral nail pigmented lesions. D, Median longitudinal nail
biopsy withUflap, whichcanbe usedtobiopsy mediannail
pigmentation resulting fromlesions originating in proximal
nail matrix and have diameter between 3 and 6 mm.
J AM ACAD DERMATOL
MAY 2007
844 Braun et al
of the nail plate is then dissected and separated
from the nail bed while the distal two thirds of the
nail plate remains pristine and attached to the nail
bed. This will insure that the distal rosette appear-
ance of the nail bed is preserved. The specimen is
then removed by a fusiform-shaped wedge with the
convex portion of the crescent matching the contour
of the lunula (Fig 14, B). The incision should extend
to the level of the bone. The subsequent repair
requires that the surrounding matrix be undermined
so as to allow for primary closure. The closure can be
accomplished by using interrupted stitches using a
monofilament synthetic absorbable suture material
(p.e. PDS 6.0). If care is taken and the proximal nail
matrix is not disturbed the transverse biopsy will
merely result inthinning of the involvednail plate but
will not leave a permanent fissure as would result
from a central longitudinal biopsy.
Recently, tangential matrix excision has been
suggested (Fig 15). This technique is similar to the
transverse matrix biopsy but provides a specimen
that is less than 1-mm thick. However, this technique
may lower the risk of postoperative nail dystrophy.
Obviously if the lesion is diagnosed as malignant,
more extensive surgery is mandated.
Scherenbergs releasing U-ap method: This
method requires a rectangular bloc excision of the
involved nail plate, nail bed, nail matrix, and prox-
imal nailfold. The ap is created by a curved incision
running along the lateral wall and extending from
the distal end of the incision to the proximal edge of
the matrix (Fig 14, D). The nail bed and matrix are
separated from the underlying bone so as to provide
complete mobility. The flap is then rotated into
position and closed with 5-0 nylon sutures. The
defect created in the lateral wall is allowed to heal by
secondary intention. Efforts should be taken to keep
the matrix from adhering to the overlying proximal
nailfold so as to reduce the risk of adhesion and
postoperative nail splitting. A modified technique
has been described by Haneke
2
: a rectangular exci-
sion that includes all of the affected tissue is removed
en bloc. A flap, which includes 3 to 4 mm of the
lateral nail wall, is raised by a curved incision that
runs parallel to the excised rectangle and extends
from the proximal edge of the matrix to just short of
the distal edge of the excised tissue. This will then
provide a bridge that will enhance the viability of the
flap and facilitate its subsequent rotation. The rotated
flap is then approximated to the remaining nail. The
defect created by rotating the flap is allowed to heal
by secondary intention. This technique enables the
removal of the proximal portionof the nail matrix and
results in acceptable postoperative changes in the
nail apparatus. The resulting nail plate is diminished
in its width but is otherwise normal except for a slight
longitudinal ridge. However, despite meticulous sur-
gical techniques and care it is sometimes impossible
to avoid postoperative nail splitting.
For pigmented bands greater than 6 mm in diam-
eter, a matrix punch or transverse biopsy are usually
adequate biopsy techniques that can be used during
the preliminary investigation process (Fig 14, A).
SURGICAL MANAGEMENT OF
MELANOMAS OF THE NAIL APPARATUS
Melanoma in situ
The surgical treatment of an in situ melanoma
requires the complete surgical removal of the nail
apparatus including nail plate, nail bed, and nail
matrix. The resulting defect can be repaired either
with a split- or full-thickness skin graft.
48-52
Although
it is possible to allow wound healing by secondary
intention, this will often result in a surface that is not
smooth and can have spicules of keratinizing tissue
and, thus, is not as comfortable for the patient as the
results obtained with a skin graft.
Invasive melanomas
The treatment of invasive melanoma of the nail
unit often requires amputation of the effected digit.
The choice between simple distal amputation of a
Fig 15. Schematic showing technique of matrix shave
biopsy (from Haneke,
2
with permission). LM, Longitudinal
melanonychia; NP, nail plate; RNP, reflected NP; RPNF,
reflected proximal nailfold.
J AM ACAD DERMATOL
VOLUME 56, NUMBER 5
Braun et al 845
portion of the digit versus more aggressive and
extensive surgical procedures is dictated, to some
extent, by the thickness of the tumor. In addition, the
relative functional importance of a given digit may
also inuence the extent of surgery. For example,
if the melanoma is located on the large toe it is
important, if at all possible, to preserve the proximal
phalange because this is the insertion site for many
muscles and is important for balance. Melanomas
located on the thumb also require special surgical
consideration. The thumb is the most important digit
because it is the only one that is fully opposable to the
tips of the ngers and gives us the ability to grasp and
hold objects. Thus, needless to say, the level at which
the amputation is performed has major implications
for the dexterity of the involved hand. In summary,
the primary priority regarding melanoma remains the
complete surgical removal of the tumor followed by
attempts to preserve functionality. Lastly, one needs
to take the aesthetics into consideration.
48-51,53
CONCLUSION
Longitudinal nail pigmentation occurs frequently
enough that every dermatologist will be confronted
with this condition in his or her practice. The
differential diagnosis is fairly broad and includes
entities listed in Table I. Each one of these conditions
poses particular management issues. The use of
dermoscopy can assist in the evaluation and possible
diagnosis of nail pigmentation. It should be per-
formed to help identify the type of pigment and to
determine the origin of the pigment. This informa-
tion in turn will help determine the most appropriate
biopsy technique to use so as to provide maximal
information while attempting to maintain cosmesis.
It has been our experience that dermoscopy has
greatly impacted our management decisions and has
clearly reduced the number of unnecessary opera-
tions performed on banal nail pigmented bands.
However, it is important to remember that dermo-
scopy cannot and should not be used in a vacuum
that is devoid of patient history and physical exam-
ination findings. Thus, independent from the dermo-
scopic morphology, it is important to take into
consideration other high-risk features, which were
discussed earlier, which may also impact the deci-
sion to perform a biopsy. If nail biopsy is required, it is
incumbent on the physician to select the most appro-
priatesurgical procedurethat will providethenecessary
pathological information while avoiding permanent
postoperative nail dystrophia, if at all possible.
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