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JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION VOL. 100, NO.

6, JUNE 2008 743


C A S E R E P O R T
INTRODUCTION
M
elanonychia striata is caused by an increase in
the activity of melanocytes in the nail matrix,
which produces an increase in melanin depo-
sition in the nail plate.
1
The clinical presentation can
range from a narrow, brown or black longitudinal streak
to an almost complete pigmentation of the nail plate.
1

There is a marked racial variation in the occurrence of
melanonychia striata.
2
Although melanonychia striata is
not uncommon in dark-skinned individuals, the condi-
tion is uncommon in Chinese individuals, especially in
those aged <20 years.
2
The occurrence is usually spo-
radic. We describe a Chinese mother and her two chil-
dren with melanonychia striata. To our knowledge, the
familial occurrence of melanonychia striata has not
been reported.
CASE REPORT
A 44-year-old Chinese female presented with a lon-
gitudinal brownish streak in her right index nger. The
pigmentation was rst noted eight years ago. She con-
sulted the rst author (Leung) because she read about
his previous publications on melanonychia striata on a
website. She was convinced that the condition she and
her children had matched what the author had published
previously. The patient had two children, a 15-year-old
daughter and a 12-year-old son, who had similar con-
ditions rst noted at 7 and 8 years of age, respectively.
There was no other family member with melanonychia
striata. There was no history of trauma to the digits in
any of the family members, and none of them were on
any medication. There was no family history of Peutz-
Jeghers syndrome or Laugier-Hunziker syndrome.
On physical examination, the patient was noted to
have a vertical, well-demarcated brownish discoloration
of the nail plate of the right index ngernail (Figure 1).
No nodularity or nail deformity was noted. The rest of
the physical examination was normal. In particular, there
was no periungual pigmentation, oral/labial/perioral pig-
mentation, axillary lymphadenopathy or involvement of
the toenails. The patient was right handed.
Physical examination of the 15-year-old daughter
showed brownish discoloration of the nail plates of the
left thumbnail, left index ngernail, right thumbnail,
right index ngernail and right middle ngernail (Fig-
ure 2). The rest of the examination was unremarkable.
The patient was right handed.
Physical examination of the 12-year-old son showed
a brownish streak of discoloration in the nail plate of the
left thumbnail (Figure 3). The rest of the examination
was normal. The patient was right handed.
A diagnosis of melanonychia striata was made. The
family was reassured of the benign nature of the condi-
tion. The mother and the children were advised to seek
medical attention if there was a sudden increase in pig-
mentation or size of the lesion(s).
DISCUSSION
Melanonychia striata is rare in whites but not uncom-
mon in African Americans, Hispanics and other rel-
atively dark-skinned individuals.
2-6
Kawamura et al.
reported that 11% of 3,102 Japanese individuals aged
1670 years had pigmented bands of the nails.
3
Tasaki
examined 800 Japanese individuals and found that 20%
of the males and 23% of females had melanonychia stri-
ata of the ngernails.
6
Leyden et al. evaluated the nails of
200 black individuals and reported that the presence of
longitudinal pigmented bands increased progressively
with age from 2.5% of those aged <4 years to 75% of
2008. From the Department of Pediatrics (Leung, clinical associate pro-
fessor; McLeod, associate professor), and Department of Medical Genet-
ics (McLeod, associate professor), the University of Calgary and the Alberta
Childrens Hospital, Calgary, Alberta, Canada. Send correspondence and
reprint requests for J Natl Med Assoc. 2008;100:743745 to: Dr. Alexander
K.C. Leung, #200, 233 16th Ave. NW, Calgary, Alberta, Canada T2M 0H5;
fax: (403) 230-3300; e-mail: aleung@ucalgary.ca
The occurrence of melanonychia is usually sporadic. We
describe a Chinese family with three affected members in
two generations, suggesting a dominant mode of inheri-
tance. The familial occurrence of melanonychia striata has
not been reported previously.
Key words: fingernails n genetics
Familial Melanonychia Striata
Alexander K.C. Leung, MBBS, FRCPC, FRCP(UK & Irel), FRCPCH and D. Ross McLeod, MD, FRCPC, FCCMG
744 JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION VOL. 100, NO. 6, JUNE 2008
MELANONYCHIA STRIATA
those aged 4049 years, and to 96% of those aged 50
years.
4
Kopf et al. examined 632 Caucasians and did not
nd a single case of melanonychia striata.
7
Leung et al.
examined 2,457 Chinese individuals for the presence of
melanonychia striata.
2
The authors found that 20 indi-
viduals had melanonychia striata, for a prevalence rate
of 0.8%. The condition was not identied in individu-
als aged <20 years. Melanonychia striata was present in
0.6% of individuals aged between 2029 years of age.
Thereafter, the prevalence increased gradually to 1.7%
in individuals aged 50 years. None of the affected indi-
viduals had involvement of >2 digits, and none had a
family history of melanonychia striata that they were
aware of.
The occurrence of melanonychia striata is usually
sporadic and spontaneous. Melanonychia striata may
also occur as a result of trauma, irradiation, gold ther-
apy, medications (mepacrine, phenolphthalein and cyto-
toxic agents), arsenic intoxication, hemochromatosis,
Addison disease or vitamin-B
12
deciency.
5,8
In all the
reported series, no familial occurrence of melanonychia
striata has been described. Carson did report an Indian
48-year-old mother and her 21-year-old daughter with
pigmented nevi involving the right big toenail and right
thumbnail, respectively.
8
Melanonychia striata is more common in frequently
used ngers.
5
As such, the thumbs are most frequently
affected. The index, middle, fourth and fth ngers
are employed with decreasing frequency for grasping
objects and demonstrate a corresponding lower inci-
dence of melanonychia striata. The condition is usually
solitary with exception of bilateral involvement of the
thumbs.
2
Involvement of multiple digits is rare.
2,5
In the
present series, the 15-year old daughter had ve digits
affected with melanonychia striata.
Melanonychia striata has to be differentiated from
other causes of melanonychia. Probably the most com-
mon cause of melanonychia is a subungual hemor-
rhage.
5
A subungual hemorrhage will migrate distally
as the nail grows, whereas most of the other causes of
melanonychia will remain stationary. Other differential
diagnosis includes a subungual nevus and subungual
melanoma.
9
Rarely, pigmentary nail changes have been
reported in association with Peutz-Jeghers syndrome
and Laugier-Hunziker syndrome. However, our patients
did not have features of either syndrome.
Melanonychia presents a challenging diagnostic and
therapeutic problem. Most authors suggest histologic
diagnosis in suspicious cases only; otherwise, the treat-
ment is a wait-and-see approach, as the condition is usu-
ally benign.
10
In the present series, the characteristic lin-
ear streaks suggest the diagnosis of melanonychia striata.
The multiple digital involvement makes the diagnosis
of subungual nevus and subungual melanoma highly
unlikely and biopsy of the lesions unnecessary.
Our report is unique in that three family members
had melanonychia striata, and two of the affected mem-
bers were children. As melanonychia is rare in Chinese
Figure 1. A 44-year-old female with melanonychia
striata affecting the right index fingernail
Figure 3. Son with melanonychia striata
affecting the left thumbnail
Figure 2. Daughter with melanonychia striata
affecting the left thumbnail, left index fingernail,
right thumbnail, right index fingernail and right
middle fingernail
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION VOL. 100, NO. 6, JUNE 2008 745
MELANONYCHIA STRIATA
patients, especially those <20 years of age, the existence
of melanonychia striata in the present three affected
family members is more than a chance occurrence. The
inheritance pattern is consistent with dominant inheri-
tance and, as the son was less affected than his mother
and sister, it is mostly likely autosomal. A multifactorial
mode of inheritance is also a reasonable explanation with
both genetic and environmental factors involved.
REFERENCES
1. Leung AK, Kao CP. An atlas of nail disorders. Consultant. 2001;41:58-64.
2. Leung AK, Robson WL, Liu EK, et al. Melanonychia striata in Chinese chil-
dren and adults. Int J Dermatol. 2007;46:920-922.
3. Kawamura T, Nishihara K, Kawasakiya S, et al. Pigmentatio longitudinalis
striata unguium and the pigmentation of nail plate in Addison disease. Jpn
J Dermatol. 1958;68:76-88.
4. Leyden JJ, Spott DA, Goldschmidt H. Diffuse and banded melanin pig-
mentation in nails. Arch Dermatol. 1972;105:548-550.
5. Leung AK, Woo T. Melanonychia striata with multiple toenail involvement
in a child. J Natl Med Assoc. 2004;96:1232-1234.
6. Tasaki K. On band or linear pigmentation of the nail. Jpn J Dermatol.
1933;33:568.
7. Kopf A, Waldo E. Melanonychia striata. Aust J Dermatol. 1980;21:59-70.
8. Carson GA. Familial congenital pigmented naevi of the nails. Lancet.
1962;1:508-509.
9. Leung AK, Woo TY. A subungual nevus in a Filipino child. Pediatr Derma-
tol. 2004;21:462-465.
10. Leung AK. Melanonychia striata. In: Lang F, ed. The Encyclopedia of
Molecular Mechanisms of Disease. Berlin: Springer-Verlag (in press). n
C A R E E R O P P O R T U N I T I E S
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