Hutchinsons sign: A reappraisal

Robert Bat-an, MD; and Paul Kechijian, MDb Cannes, France, and Neu, York, New York
Hutchinsons sign, periungual extension of brown-black pigmentation from longitudinal
melanonychia onto the proximal and lateral nailfolds, is an important indicator of subungual
melanoma. However, experience has demonstrated that Hutchinsons sign, although valuable,
is not an infallible predictor of melanoma. Periungual pigmentation is present in a variety of
benign disorders and, therefore, may lead to overdiagnosis of subungual melanoma. Periun-
gual hyperpigmentation occurs in at least one nonmelanoma skin cancer, Bowens disease
of the nail unit. Hyperpigmentation of the nail bed and matrix may reflect through the
transparent nailfolds simulating Hutchinsons sign. Pseudc+Hutchinsons sign is a
phrase coined to encompass these three simulants of Hutchinsons sign. Each represents a
misleading clue to the diagnosis of subungual melanoma. Total reliance on the (apparent)
presence or absence of periungual pigmentation may lead to overdiagnosis or underdiagno-
sis of subungttal melanoma. All relevant clinical and historical information, including the
presence or absence of periungual pigmentation, must he carefully evaluated in a patient sus-
pected of having subungual melanoma. Ultimately, the diagnosis of subungual melanoma is
made histologically. (J AM ACAD DERMATOL 1996;34:87-90.)
One hundred years ago, Sir Jonathan Hutchinson
wrote a succinct landmark report (described in
Kopf). Melanotic Whitlow. There is a rare form of
disease of the nail bed that is malignant, and usually
presents as melanotic sarcoma. It is generally attrib-
uted in the first instance to injury, and its diagnosis
is always missed in the early stages. Because it re-
sembles whitlow, and is usually so named at first, I
prefer to give it that name. It is, however, from the
beginning, malignant. Careful observation will find
at the edge of the inflamed nail a little border of
coal-black colour, and this, however slightly marked,
must be allowed to make the diagnosis. I have seen
at least half a dozen of these cases. Early amputation
is demanded.2
This sign remains an important clue to the diag-
nosis of subungual melanoma. Hutchinsons sign is
characterized by extension of brown-black pigment
from the nail bed, matrix, and nail plate onto the ad-
jacent cuticle and proximal and/or lateral nailfolds
(Fig. 1). It may present as a dark circle resembling
a ring originating at the base of the tumor, as a com-
plete or incomplete circle, or as a large spot, dash, or
rod. In addition, Hutchinsons sign may be subtle or
From the Nail Disease Center. Cannes,3 and the Nail Section, Depar-
ment of Dermatology. New York University Medical Center.b
Reprint requests: Paul Kechijian, MD, 935 Northern Blvd., Great Neck,
NY llO?l.
Copyright 0 1996 by the American Academy of Dermatology. Inc.
019@96'2/96 $5.00+0 16/l/66878
even absent in subungual melanoma. When accom-
panied by ulceration of the nail bed or obliteration of
the nail plate with a pyogenic granuloma-like tumor,
the sign is essentially pathognomonic of subungual
melanoma. However, the presence of periungual
pigmentation by itself is not pathognomonic of sub-
ungual melanoma. This review attempts to reevalu-
ate this sign in light of the experience gleaned since
Hutchinsons report.
Hutchinsons sign is the clinical counterpart of the
radial growth phase of subungual melanoma.36
During its radial growth phase, subungual melanoma
may distribute its black color into the entire nail plate
and surrounding tissues. Dystrophic changes usually
occur during the vertical growth phase. Hutchinsons
sign is best regarded as a clinical sign that is accom-
panied by the histologically confirmed presence of
subungual melanoma. In the absence of histologicly
confirmed melanoma, the diagnosis of Hutchinsons
sign (and the implicit diagnosis of subungual mela-
noma) must be considered presumptive.
Often, a 3 to 4 mm punch biopsy specimen of pe-
riungual tissue provides insufftcient tissue to distin-
guish a junctional nevus from subungual melanoma.
The only histologic clue to the radial growth phase
of acral lentiginous melanoma may be intense
inflammation characterized by the presence of lym-
phocytes and macrophages at the dermoepidermal
interface.(j A profusion of host epidermal lympho-
cytes is present in intimate association with intraepi-
87
88 Bat-an and Kechijian
Journal of the American Academy of Dermatology
January 1996
Fig. 1. Hutchinsons sign in a subungual melanoma.
Band of longitudinal melanonychia extends from cuticle
to distal nail tip. Margins are fuzzy, not sharp. Centrally,
it is dark brown; laterally it is lighter in color. Proximal
to the band, the nailfold is infiltrated with light tan pig-
mentation.
Fig. 3. Multiple, poorly defined patches of brown pig-
ment are scattered most prominently in the hyponychium
and distal pulp in this patient with AIDS, who was not
treated with zidovudine. (Courtesy J. Lacour, MD, Nice,
France.)
Fig. 2. In this patient with Laugier-Hunziker syndrome,
a tan, well-circumscribed band of longitudinal melanony-
chia abuts focus of pigmentation in adjacent cuticle and
proximal nailfold.
Fig. 4. Congenital nevus of the toe in an g-year-old
child. The entire nail plate is pigmented. Faintly pig-
mented hyponychium and lateral and proximalnail folds
form a pale, dusky annulus around the darkened nail plate.
dermal melanoma cells. Careful examination of ad- however, is the presence of prominent, usually pig-
jacent areas in the basilar region may reveal in- mented, long dendrites that traverse a distance span-
creased numbers of large melanocytes. Recognition ning the width of three to four adjacent keratinocytes.
of these distinctive large melanocytes is necessary Commonly, large melanocytes are numerous, com-
for histologic diagnosis of the radial growth phase. prising every second or third cell in the anatomic
The prototypic melanocyte has a large nucleus with basal layer of the epidermis. The abnormal melano-
a prominent nucleolus. Its most important feature, cytes may form large nests that protrude into the
Journal of the American Academy of Dermatology
Volume 34, Number 1 Bat-an and Kechijian 89
Table I. Benign conditions accompanied by pseudc+Hutchinsons sign
Condition Clinical features
Ethnic pigmentation
L,augier-Hunziker syndrome
Peutz-Jegher syndrome
Radiation therapy Reported after treatment of finger dermatitis, psoriasis, and
chronic paronychia9
Malnutrition Polydactylous involvement
Minocycline Polydactylous involvement
Patients with AIDS Polydactylous involvement: zidovudiie produces similar pig-
Trauma-induced
Congenital nevus
After biopsy
Regressing nevoid melanosis in childhood
Subungual hematoma
Pigmentation of proximal nailfold in dark-skinned persons; lat-
eral nailfolds not involved; longitudinal melanonychia not al-
ways present; often exaggerated in thumbs
Macular pigmentation of lips, mouth, and genitalia7,$ one or
several fingers involved (Fig. 2)
Hyperpigmentation of fingers and toes; macular pigmentation of
buccal mucosa and lips7
mentation (Fig. 3).7, I2
Friction, nail biting and picking, and boxing3-5
See Fig. 4.16
Pigment recurrence after biopsy of longitudinal melanonychia in
acquired and congenital melanocytic nevi17; often striking cyto-
logic atypia*
Monodactylic; initial increase in dyschromia followed by subse-
quent pigment regression; perplexing disordert9s 2o
Exceptionally, blood spreads to nailfolds and hyponychial areas
(Fig. 5).
dermis and extend profusely along eccrine ducts.
Even when the histologic diagnosis is obvious,
pagetoid growth may be absent or minimal. To as-
sist the pathologists interpretation of the biopsy
specimen, the clinician should provide the patholo-
gist with a detailed history and clinical description.
In addition, the clinician must be wary of a histologic
report of a benign lesion if Hutchinsons sign is
present.
When present, Hutchinsons sign is an important
presumptive clue to the diagnosis of subungual mel-
anoma. There are, however, three exceptions to
consider in the evaluation of patients with periungual
pigmentation who are suspected to have subungual
melanoma.
1. Several benign conditions are accompanied by
Hutchinsons sign (Table I, Figs. 2-5). These 12
examples represent benign disorders occurring in
association with pseudoHutchinsons sign. Mono-
dactylous and polydactylous longitudinal melanony-
chia and periungual hyperpigmentation are present
in all.
2. Sau et al* described seven cases of Bowens
disease of the nail unit. Among their patients was one
with periungual hyperpigmentation and features
clinically typical of subungual melanoma (Fig. 6).
This case represents a second type of pseudo
Hutchinsons sign, this one arising in a nonmela-
noma skin cancer.
3. The appearance of Hutchinsons sign may be
illusory. Benign disorders such as subungual he-
matomas and melanocytic nevi produce pigment
confined exclusively to the nail matrix and bed. Be-
cause the nailfold and cuticular tissues are relatively
transparent, brown-black coloration may appear to
arise within the periungual tissues when, in fact,
pigmentation is confined to the nail bed and matrix.
Dark color traverses the transparent cuticle and nail-
fold simulating Hutchinsons sign. This not uncom-
mon simulant represents a third example of pseudo-
Hutchinsons sign.
Benign, nonmelanoma, and illusory
variants of pseudoutchinsons sign occur in the
absence of subungual melanoma. Each is character-
ized by periungual hyperpigmentation occurring in
association with longitudinal melanonychia. Each
represents a potentially misleading clue to the diag-
nosis of subungual melanoma. These variants do vl~t
negate the importance of Hutchinsons sign. Rather,
they oblige the clinician to consider diagnostic pos-
sibilities other than subungual melanoma. Likewise,
the absence of periungual pigmentation does not
preclude the diagnosis of subungual melanoma. The
clinician must carefully evaluate the individual pa-
90 Bar-an and Kechijian
Journal of the American Academy of Dermatology
January 1996
Fig. 5. Blood from this subungual hematoma is present
under the nail plate and within the hyponychium and ad-
jacent pulp.
Fig. 6. Striking, coal black pigmentation extends
broadly into the proximal and lateral nail folds and
hyponychium of this patient with Bowens disease and
longitudinal melanonychia. (Prom Sau P, McMarlin SL,
Sperling LC, et al. Arch Dermatol 1994;130:204-9.)
tient for clues to the diagnosis of subungual mela-
noma. In addition to a detailed history of the present
illness, careful clinical examination of the lesion;
general physical examination; the history of the pa-
tient including drug ingestion, past treatments, hob-
bies and illnesses, family history, and racial origin;
and general physical appearance must be evaluated.
If the diagnosis of subungual melanoma seems
likely, an adequate biopsy of involved nail unit is
performed. In this manner, the pathologist is able to
examine tissue sufficient to rule in or out the diag-
nosis of subungual melanoma.
The relevance of Hutchinsons sign to the diag-
nosis of subungual melanoma has withstood the test
of time. If the possibility of pseud~Hutchinsons
variants is kept in mind, the clinician is less Likely to
overdiagnose this important malignancy and more
likely to address the problem with confidence and
precision.
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