Nail Melanoma In Situ: Clinical, Dermoscopic, Pathologic

Clues, and Steps for Minimally Invasive Treatment

Ana F. Duarte, MD,* Osvaldo Correia, PhD,*† Ana M. Barros, MD,*
Filipa Ventura, MD,* and Eckart Haneke, PhD*‡xk

BACKGROUND Nail unit melanoma (NUM) is a variant of acral lentiginous melanoma. The differential
diagnosis is wide but an acquired brown streak in the nail of a fair-skinned adult person must be considered
a potential melanoma. Dermoscopy helps clinicians to more accurately decide if a nail apparatus biopsy is
necessary.

OBJECTIVE Detailed evaluation of clinical and dermoscopy features and description of conservative surgery
of in situ NUM.

METHODS Retrospective study of in situ NUM diagnosed and treated with conservative surgical manage-
ment in the authors’ center from 2008 to 2013.

RESULTS Six cases of NUM were identified: 2 male and 4 female patients, age range at diagnosis of 44 to 76
years. All patients underwent complete nail unit removal with at least 6-mm security margins around the
anatomic boundaries of the nail. The follow-up varies from 4 to 62 months.

CONCLUSION Nail unit melanomas pose a difficult diagnostic and therapeutic challenge. Wide excision is
sufficient, whereas phalanx amputation is unnecessary and associated with significant morbidity for patients
with in situ or early invasive melanoma. Full-thickness skin grafting or second-intention healing after total nail
unit excision is a simple procedure providing a good functional and cosmetic outcome.

The authors have indicated no significant interest with commercial supporters.

N ail unit melanoma (NUM), localized either

under (subungual) or around (periungual) the
nail, is considered a variant of acral lentiginous
the problem that the proximal nail fold and nail plate
cover the underlying structures, but a lack of awareness
for ungual melanomas. Also, it is often asymptomatic
melanoma that represents approximately 1% to 2.5% for a prolonged period, and many patients only notice
of all melanomas in white, 15% to 35% in dark- pigmentation after trauma to the area and two thirds
skinned, and 50% to 58% in Asian individuals.1–4 seek medical advice very late because of the appearance
of the lesion.6,7
Late diagnosis of ungual melanomas is still a sad reality.
This is the reason for the poorer prognosis and lower Longitudinal pigmentation of the nail in light-skinned
survival of nail melanoma patients as compared with individuals has a wide range of differential diagnoses,
those with melanomas of other localizations.5 Despite including onychomycosis, drugs, subungual hematoma,
an obvious brown streak in the nail, both physicians nonmelanoma tumors, and striated melanonychia
and patients often neglect this important sign for years. because of functional activation of matrix melanocytes,
Thus, apart from amelanotic nail melanoma, the diffi- a lentigo, or a matrix nevus. It is necessary to be aware
culty of establishing an early diagnosis is not so much that an acquired brown streak in the nail of a fair-skinned

*Centro de Dermatologia Epidermis, Instituto CUF, Porto, Portugal; †Faculty of Medicine, University of Porto, Portugal;
‡
Department of Dermatology, Inselspital, University of Bern, Bern, Switzerland; xDermatology Clinic Dermaticum,
Freiburg, Germany; kDepartment of Dermatology, University of Ghent, Ghent, Belgium

· · ·
© 2014 by the American Society for Dermatologic Surgery, Inc. Published by Lippincott Williams & Wilkins
ISSN: 1076-0512 Dermatol Surg 2015;41:59–68 DOI: 10.1097/DSS.0000000000000243
·
59

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 NAIL MELANOMA IN SITU

adult person must be considered a potential melanoma.8 prolongation of disease-free survival, in particular in
The spread of the pigmentation into the periungual skin early cases. The use of tissue-sparing Mohs micro-
(true Hutchinson sign) is indicative of the extension of graphic surgery for thin melanomas of the nail unit is
the disease beyond the nail organ. an alternative that has potential benefit in avoiding
amputation. However, it is not available in all
Although histopathologic diagnosis remains the gold centers.6
standard for NUM,6,9 the clinical diagnosis of longi-
tudinal melanonychia (LM) can be assisted by der- Full-thickness skin grafting after total nail unit exci-
moscopy, which can help clinicians to more accurately sion is a simple procedure that can be performed in
decide if a nail apparatus biopsy is required. cases of NUM in situ. It provides a good cosmetic and
excellent functional outcome and does not produce
Management of melanoma requires consideration of significant donor site morbidity.1,7,12,13
oncologic and reconstructive surgical principles to
optimize the chances of cure and quality of life.10 Nail
Materials and Methods
unit melanomas pose a difficult surgical challenge
because of the close relationship between the nail Six cases of NUM in situ from 2008 to 2013 were
tissues and underlying bone and extensor tendon. retrieved from the dermatopathologic database of
Wide excision with phalanx amputation was once the authors’ department. Clinical (Figure 1) and
considered the first-line therapy10,11; however, it is not dermoscopic (Figure 2) photographs of all cases
satisfactory for the patients and has not shown were examined by 3 dermatologists, and pathology

Figure 1. Nail unit melanoma clinical features—LM (A–F) and Hutchinson sign (arrows).

60 DERMATOLOGIC SURGERY

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 DUARTE ET AL

Figure 2. Nail unit melanoma dermoscopic features—brown bands irregular in color, width, and spacing, and Hutchinson
sign (arrows). (A–F) Correspond to Figure 1A–F, respectively.

slides (Figure 3) were reviewed by 1 pathologist and Surgical Management

1 dermatopathologist.
In all patients, a tangential biopsy specimen was taken
from the matrix and nail bed after reflection of the
Demographic data, including age at the time of
proximal nail fold and separation of the nail plate from
diagnosis, sex, localization, family and personal his-
tory of melanoma, history of trauma of the affected the matrix and proximal nail bed (Figure 4). After
finger, delay to diagnosis, clinical appearance with histopathologic confirmation of in situ nail mela-
presence or absence of periungual pigmentation noma, surgical excision of the whole nail unit
(Hutchinson sign), dermoscopy features, histopath- including the 2 lateral nail folds, the proximal nail
ologic study, treatment modalities, immediate com- fold, and the distal pulp of the finger/toe with an extra
plications of treatment, if any, and follow-up events margin of 6 mm, was performed. The deep margin
were recorded (Table 1). was the cortical bone. The excisional defect produced

Figure 3. Histopathology of in situ matrix melanomas. Hematoxylin and eosin stain. (A) Almost invisible changes (patient
from Figures 1E and 2E). (B) A few cells are obvious (arrow) (patient from Figures 1F and 2F). (C) The entire matrix epitelium
shows pagetoid spread of atypical melanocytes (arrows) (patient from Figures 1B and 2B). (D) Lentiginous (small arrows)
and nest-like (large arrow) proliferation of atypical melanocytes (patient from Figures 1A and 2A).

41:1:JANUARY 2015 61

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 NAIL MELANOMA IN SITU

Two lesions were located on the fingers (1 thumb and 1

TABLE 1. Demographic Data
fifth finger) and 4 lesions on the toes (all on the hallux).
Number of Patients (n = 6), The right side was involved in 4 patients, and the left
Features n (%) side in 2 patients.
Sex
Male 2 (33.3) One patient had a personal history of melanoma in
Female 4 (66.7) another location, and none gave a family history of
Age at diagnosis, years
melanoma. Only 1 patient reported a previous trauma
Range 44–76
Mean 61
of the affected nail. The mean delay of diagnosis was
Phototype 3.5 years, ranging from 3 months to 7 years. The lesion
II 3 (50) was detected by a dermatologist in 3 cases, by the
III 3 (50) patient in 2 cases, and by the family in 1 case.
Location
Finger 2 (33.3)
All patients presented an LM. There were no amela-
Toe 4 (66.7)
Personal history of
notic cases. The width of the LM was between 3 and 6
melanoma mm in 4 patients, narrower than 3 mm in 1, and larger
Yes 1 (16.7) than 6 mm in another one. A clinically apparent
No 5 (83.3) Hutchinson sign was present in 2 patients.
Family history of
melanoma
Yes 0 (0) The dermoscopic features were suspicious in all cases.
No 6 (100) All patients had a dark brown background, 5 of them
Local trauma history had brown bands with irregular lines in width, spac-
Yes 1 (16.7) ing, and color. One patient had regular lines. Two
Unknown 5 (83.3)
cases showed ill-defined edges. Haphazard pigmenta-
Lesion duration
Mean 3.5 years
tion on the hyponychium, called micro-Hutchinson
Range 3 months–7 years sign, was observed in 4 cases.
<1 years 1 (16.7)
1–3 years 3 (50) A tangential excisional biopsy was performed in all
3–5 years 0 (0) patients. All were diagnosed as in situ melanoma.
5–10 years 2 (33.3)
Clinical, dermoscopic, and pathology features are
Who suspected the
lesion outlined in Table 2.
Patient himself 2 (33.3)
Dermatologist 3 (50) Despite clinical suspicion, the pathology may be
Family 1 (16.7) unclear, that is why 2 patients needed a second biopsy
before the diagnosis was reached.
on the dorsal side of the distal phalanx was covered
by a full-thickness skin graft taken from the internal The entire lesion was totally removed in all patients, with
aspect of an arm or thigh (depending if the defect was safe margins confirmed by histopathology. The entire
in the finger or toe, respectively), or left for second- nail unit was completely removed with an additional
intention healing (Figure 5). margin all around it of at least 6 mm and down to the
bone of the terminal phalanx including the periosteum.
Results

Of 6 patients, 4 were women (66.7%) and 2 were men The defect was immediately closed in 4 patients with
(33.3%). The mean age at the time of diagnosis was 61 a full-thickness skin graft from the inner arm or the
years, ranging from 44 to 76 years. All patients were inner thigh, depending on the defect location (finger or
white people, 50% of Phototype II and 50% Phototype toe, respectively). Two patients healed by second
III. The demographic features are shown in Table 1. intention.

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 DUARTE ET AL

Figure 4. Nail unit melanoma biopsy. (A) Proximal nail fold (arrow) and nail plate (#) elevation. (B) Tangential nail matrix (*)
and nail bed (♦) biopsy.

The healing was good in all patients, both cosmetically radical surgery, namely phalanx amputation. Non-
and functionally (Figure 6). In 2 patients, the defect amputative excision, described by Haneke and Binder
was left for second-intention healing. Although they in 1978, is now being advocated in the surgical and
had the longest healing period of approximately 2 particularly the dermatosurgical literature.6,7,11,14,15
months, their cosmetic outcome was superior because
there was less tissue depression over the surgical In the authors’ series, there was a female pre-
defect (Figure 7). dominance, similar to the literature. This may be
related to either genuine gender predominance or to
Treatment and follow-up details are given in Table 3. women seeking earlier medical advice. The authors’
The mean time of follow-up was 25.2 months, population was exclusively white, Phototype II or III.
ranging from 4 to 62 months. There was no evidence Unlike most of the reports, NUM was more frequent
of recurrence or metastases in any of the patients. on toes than on fingers (4 and 2, respectively). A
traumatic etiology suggested as an NUM risk factor
was reported by only 1 of the authors’ patients, but
Discussion
this remains controversial in the literature.
Nail unit melanomas are frequently more advanced
than other melanomas at the time of diagnosis. Con- The authors’ series includes only 6 patients, from
sequently, traditional surgical intervention for this a small single center, who were diagnosed and treated
type of malignancy has focused predominantly on in a period of 5 years. The latest and largest published

Figure 5. Surgical procedure. (A) An incision is carried around the entire nail organ with a safety margin of 6 mm. (B) The
entire nail organ is dissected from the terminal phalanx bone; the undersurface of the surgical specimen is shown. (C) End of
surgery; a running locked suture of the wound margin reduces bleeding. This wound is left for second-intention healing.

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 NAIL MELANOMA IN SITU

ethnic groups and closely examine a patient’s nail16 to

TABLE 2. Clinical, Dermoscopic, and Pathologic
Features decrease the often appalling delay in correct diagnosis.

Number (%) of In this series using dermoscopy, two-thirds of the patients

Features Patients (n = 6)
had an LM 3 to 6 mm in width and 5 had brown bands
Clinical features with irregular lines in width, spacing, and color. Sur-
LM width
prisingly, 1 patient had regular lines in color. A clinically
0–3 mm 1 (16.6)
apparent Hutchinson sign was present only in 2 patients,
6–6 mm 4 (66.6)
>6 mm 1 (16.6) but a haphazard pigmentation on the hyponychium,
Amelanotic lesion 0 (0) called micro-Hutchinson sign, was observed in 4 cases,
Hutchinson sign underlining the importance of dermoscopy.
Yes 2 (33.3)
No 4 (66.6)
The delay of diagnosis averaged 3.5 years, ranging from
Dermoscopic features
3 months to 7 years, less than the 5 years of the largest
Dark background, brown bands 5 (83.3)
with lines irregular in color series of in situ melanomas published. This may also
Dark background, brown bands 1 (16.6) reflect that the authors’ policy to biopsy all acquired
with lines regular in color
LMs in adults. In contrast, the most recent series of
Brown bands with lines irregular 5 (83.3)
in width and spacing 124 NUMs of all levels claimed a delay of 2.2 years.17
Haphazard pigmentation on the 4 (66.6)
hyponychium–Hutchinson sign In this series, the lesion was detected during a routine
Ill-defined edges 2 (33.3)
check for pigmented lesions in 3 cases, by the patient in
Pathologic features
2 cases and by the patient’s family in 1 case.
Irregular crowding of 3 (50)
melanocytes in the basal and
suprabasal layers Clinical suspicion and close inspection are most
Confluence of melanocytes, 3 (50)
important to recognize early NUM. A persistent
multinucleated cells, and
a lichenoid infiltrate search, sometimes culminating in several biopsies, is
Few melanocytes in the basal 1 (16.6) needed until an accurate diagnosis is made.18,19 The
and suprabasal layers with
presence of a brown pigmentation wider than 5 mm
irregular distribution. Some
hyperchromatic nuclei and overlaid by longitudinal lines irregular in their
Occasional melanocyte in high 1 (16.6) thickness, spacing, color, or parallelism is highly
spinous layer
suspicious for a melanoma. On Table 4, the main
Irregular distribution with dense 3 (50)
melanocytes in the matrix, differences between early NUM and advanced clear-
hyponychium, and adjacent cut NUM are outlined.
skin of the tip of the digit but
only few cells in the nail bed
Number of biopsies needed until The etiology, pathogenesis, and natural history of
the diagnosis of melanoma in situ NUMs remain poorly understood. Although most
Mean 1.3 patients do not recall a previous trauma, the majority
1 4 (66.7)
of NUM lesions are on the thumb and hallux that are
2 2 (33.3)
typically locations of minor chronic or repeated
trauma. Thus, the authors cannot exclude a role of
series reported 11 patients in 13 years.15 The incidence recurrent trauma on melanoma development.20,21 One
of melanoma is increasing every year, although this is patient only could remember a previous trauma to the
not clearly the case for NUM. A population-based affected nail unit.
study would help to better understand the epidemiol-
ogy of NUM. Although NUM is rare, given its atypical Unlike cutaneous melanoma, UV exposure does not
locations and poor survival rates, it is important that seem to play a relevant role in NUM because the nail
physicians maintain a high index of suspicion in all plate is too dense for significant light penetration.2,22

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 DUARTE ET AL

Figure 6. Grafting of the defect. (A) Full-thickness skin graft designed on an inner thigh. (B) The graft is sutured into place
with a running locked suture.

Dermoscopic examination of the free edge of the nail and the dermoscope.24 This provides information that
plate gives information on the lesion location: pig- can help the surgeon to determine whether the lesion
mentation of the dorsum of the nail plate is in favor of should be excised or can simply be submitted for
a proximal nail matrix lesion, whereas pigment in the biopsy, which is especially important in the case of
lower part of the nail edge indicates a lesion of the large bands that cannot be excised without subsequent
distal matrix.23 The International Study Group on nail scarring. However, intraoperative nail matrix
Melanonychia stated that in the adult, a brown dermoscopy, performed directly on the matrix after
background associated with longitudinal lines that nail plate avulsion, is an invasive procedure that cannot
are irregular in color, width, spacing, and parallelism routinely be performed in all centers.25
is suggestive of malignant melanoma. Also an indi-
vidual line that shows irregularity in color or width Recently, an intraoperative ex vivo diagnosis by
along its length and a dark background with the areas reflectance confocal laser microscopy has been
of different hues of pigmentation even in the absence described. It is an expensive technique used in inves-
of irregular lines, are considered suspicious of mela- tigation and not available for the majority of centers,
noma.19 Widening of the band proximally stands for but this type of examination of the nail matrix is an
enlargement of the width of the lesion.2 Although efficient diagnostic approach of LM that is believed to
dermoscopy can help, particularly in micro-Hutch- permit an extemporaneous diagnosis of malignancy
inson and slightly irregular melanonychias, the and a 1-step surgical treatment of in situ or minimally
problem with dermoscopy is that in melanonychia it invasive melanoma, dramatically reducing the dura-
only gives a unidimensional picture of the result of tion of postoperative disability.26
a melanocytic lesion in the matrix in contrast to
dermoscopy of the skin, which gives a bi-dimensional Histopathology of clinically typical nail melanoma is
picture of the lesion itself. Thus, dermoscopy may usually unequivocal2; however, very early cases may
often help but can be misleading as shown by 1 of the pose extreme difficulties. Amin and colleagues27
authors’ cases. proposed the melanocyte number per 1 mm stretch of
the dermo-epithelial junction as a means to distin-
Direct dermoscopic examination of the nail bed and guish between melanomas and benign melanocytic
matrix enables the visualization of pigmentation directly lesions. Some authors stress that there is a lympho-
in its original site, revealing aspects not observed when cytic infiltrate associated with ungual melanoma, but
the nail plate is interposed between the pigmented lesion the authors have not seen this in any of the authors’

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 NAIL MELANOMA IN SITU

Figure 7. Nail unit melanoma after surgical outcome—good aesthetic and functional outcome. (A) Corresponds to the same
patient as in Figures 1A and 2A, (B) corresponds to Figures 1B and 2B, (C) corresponds to Figures 1C and 2C, (D) corresponds
to Figures 1D and 2D, (E) corresponds to Figures 1E and 2E, and (F) corresponds to Figures 1F and 2F. (C and F) Were allowed
to heal secondarily.

cases. Intraungual melanocytes as a sign of pagetoid In a person of color, whether black or Asian, longi-
spread are seen in roughly one third of the cases and tudinal brown striation is more and more frequent
are strong evidence for a melanoma. Further, the with age.4,16,30 The melanonychias range from single
shape of the melanocyte dendrites is plumper in light brown bands to almost entirely black nails.
melanomas.28 Occasionally, hardly any melanocyte is Most of these discolorations are due to melanocyte
detected despite an acquired wide brown band in an activation, which is said to yield rather a grayish color
elderly person. Nevertheless, this clinical scenario is than brown.19 However, this may be extremely dif-
particularly suspicious, with a high risk of nail mel- ficult to differentiate, and it has therefore been pro-
anoma despite lack of obvious morphologic features posed to look for the “ugly duckling sign,” which
histologically.28,29 The number of atypical melano- means that in dark-skinned individuals with many
cytes and the severity of atypia seem to be correlated pigmented nails, one should look for a particularly
with the overall number of melanocytes in the matrix. black streak that stands out from the rest of the

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 DUARTE ET AL

The follow-up of this series is short, mean time was

TABLE 3. Treatment and follow-up
25.2 months, ranging from 4 to 62 months. Longer-
Number (%) of term data, of at least 5 years, are necessary for stronger
Features Patients (n = 6) conclusions.
Treatment
Excisional biopsy 6 (100)
Total nail unit excision, until the 4 (66.7) Conclusion
phalanx and immediately full-
thickness skin grafting A high index of suspicion is mandatory to diagnose
Total nail unit excision, until the 2 (33.3) NUM. A biopsy is recommended for suspicious LM
phalanx and second-intention acquired after puberty in fair-skinned individuals or in
healing
Follow-up
the presence of LM showing rapid and progressive
Recurrence growth.12 The use of noninvasive techniques such as
Yes 0 (0) dermoscopy is valuable for the better selection of
No 6 (100) cases, in which a pathologic examination is
Metastasis indicated.32
Yes 0 (0)
No 6 (100)
Follow-up time, months
Because dermoscopy gives finer details, it can be of
Mean 25.2 help to identify slightly irregular melanonychias and
Range 4–62 micro-Hutchinson, which would not be seen other-
wise by the naked eye. However, nail dermoscopy does
brown bands.31 Any nail dystrophy in conjunction not allow the melanocytic lesion to be observed
with a melanonychia is suspicious for ungual mela- directly; this is only possible with intraoperative direct
noma unless otherwise proven. matrix dermoscopy.

TABLE 4. Nail Unit Melanoma Clinical, Dermoscopic, and Pathology Features, Early Versus Late Changes

Early NUM Late NUM

Clinical features2
Narrow LM (<5 mm) Large, LM rapidly changing (>5 mm)
Brown discoloration Black discoloration
Discrete nail defect Nail dystrophy
Discrete Hutchinson sign Evident Hutchinson sign
Oozing or bleeding lesion
Nodular lesion
Dermoscopic features19
Dark background Black background
Brown bands with lines irregular Structureless pattern
in width and/or spacing and/or color
Discrete Hutchinson sign Evident Hutchinson sign
Ill-defined edges
Pathology features2
Irregular crowding of melanocytes, Dense aggregations of melanocytes (39–136
mostly in the basal and melanocytes/mm basal layer) with large and
suprabasal layers atypical nuclei, often long dendrites, and
pagetoid spread of cells to the upper layers of
the matrix epithelium and into the nail plate
Confluence of melanocytes,
multinucleated
cells, and a lichenoid
infiltrate even
when only focal, are
in favor of a melanoma

41:1:JANUARY 2015 67

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 NAIL MELANOMA IN SITU

The clinical diagnosis should be supported by tools 16. Bradford PT, Goldstein AM, McMaster ML, Tucker MA. Acral
lentiginous melanoma: incidence and survival patterns in the United
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18. Miranda BH, Haughton DN, Fahmy FS. Subungual melanoma: an

Acknowledgments The authors thank Filipa Tomás important tip. J Plast Reconstr Aesthet Surg 2012;65:1422–4.
and Teresa Meireles for secretary work and
19. Di Chiacchio ND, de Farias DC, Piraccini BM, Hirata SH, et al.
Dr. Esmeralda Vale for the pathology consultation. Consensus on melanonychia nail plate dermoscopy. An Bras Dermatol
2013;88:309–13.

20. Fanti PA, Dika E, Misciali C, Vaccari S, et al. Nail apparatus melanoma:
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11 cases and review of the literature. J Eur Acad Dermatol Venereol Instituto CUF, Apartado 55031, Porto 4050-401,
2014;28:550–7. Portugal, or e-mail: [email protected]

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