Full text of DOI:10.1111/j.1346-8138.2001.tb00089.x

The Journal of Dermatology
Vol. 28: 58–59, 2001
Short report
Linear and Whorled Nevoid Hypermelanosis
Vibhu Mendiratta, R.C. Sharma, Latika Arya and Kabir Sardana
Abstract
Linear and whorled nevoid hypermelanosis (LWNHM) is a reticulate pigmentary disor-
der with a sporadic occurrence, representing genetic mosaicism. It is characterised by hy-
perpigmented macules in a reticulate pattern along Blaschko’s lines, sparing the mucous
membranes and stabilising after one to two years. It may be associated with various neuro-
logical abnormalities. The disorder may resemble incontinentia pigmenti, epidermal
nevus, or zebra-like hyperpigmentation clinically. We report LWNHM in a 15-year-old girl
with progressively increasing streaks of reticulate hyperpigmented macules arranged in a
whorled pattern over the trunk and extremities, which appeared soon after birth. There
was no history of any preceding eruption or any associated systemic abnormality.
Histopathological examination revealed basal cell hyperpigmentation without any pig-
mentary incontinence. CT scan of the brain was normal.
Key words: linear and whorled nevoid hypermelanosis; lines of Blaschko; reticulate
hyperpigmentation; neurological abnormalities
palsy with abnormal EEG and brain abnor-
malities on CT scan (4), and severe delayed
Introduction
Linear and whorled nevoid hyperme-
lanosis (LWNHM) was first recognised as a
distinct entity by Kaltar et al. (1) in 1988,
who described it as a reticulate pigmentary
disorder probably representing genetic mo-
saicism, having a sporadic occurrence, ap-
pearing within a few weeks after birth, and
evolving along Blaschko’s lines in the form
of 1 to 5 mm, hyperpigmented macules in a
reticulate pattern, with sparing of mucous
membranes, palms, and soles. It becomes
stable or fades after 1 to 2 years. There is no
pigmentary incontinence.
psychomotor development (5). Familial oc-
currence of LWNHM has also been de-
scribed (6).
This disorder can be confused clinically
with incontinentia pigmenti, epidermal
nevus, and zebra-like hyperpigmentation,
which may explain the scarcity of reports in
Extracutaneous abnormal features may
be seen in up to 30% of cases (2). Various
neurological abnormalities have been re-
ported in association with LWNHM, includ-
ing cerebral anomalies on MRI (3), cerebral
Received March 28, 2000; accepted for publication
October 16, 2000.
Department of Dermatology and STD, Lady
Hardinge Medical College, New Delhi, India.
Reprint requests to: Dr. Vibhu Mendiratta, RA-24,
Inderpuri, New Delhi-110 012, India.
Fig. 1. Reticulate hyperpigmented macules in a
linear and whorled pattern on the trunk and
right arm

Linear and Whorled Nevoid Hypermelanosis
59
the literature. To the best of our knowledge,
this is the second case being reported from
India.
sence of any neurological anomaly is similar
to the earlier reports of LWNHM in an Indi-
an male patient (7) and that of Kaltar et al.
(1). A progressively increasing hyperpig-
mentation along lines of Blaschko has not
been previously reported and suggests that
the pigmentation not necessarily stabilise
within 1 to 2 years after birth. Since some
patients may be asymptomatic, a neurologi-
cal assesment with a cerebral MRI/ CT and
EEG should be done.
Case Report
A 15-year-old girl, born of a non-consan-
guinous marriage and an uneventful pregnancy,
presented with progressively increasing irregu-
lar streaks of hyperpigmentation over the trunk
and extremities, which appeared soon after
birth. Bizarre hyperpigmentation initially ap-
peared over the right hand, extended onto the
right arm in a linear fashion, and then was fol-
lowed by the appearance of similar streaks over
the limbs and trunk. There was no history of any
preceding eruption. There was no history of a
similar disorder in the family. Her growth and
development had been normal.
References
1) Kaltar DC, Friffths WA, Atherton DJ: Linear and
whorled nevoid hypermelanosis, J Am Acad Der-
matol, 19: 1037–1044, 1988.
2) Nehal KS, PeBenito R, Orlow SJ: Analysis of 54
cases of hypopigmentation and hyperpigmenta-
tion along the lines of Blaschko, Arch Dermatol,
132: 1167–1170, 1996.
Clinical examination revealed light brown,
hyperpigmented macules of 1 to 5 mm in size,
present in a reticulate configuration and
arranged linearly over the limbs and in a
whorled fashion over the abdomen and back,
following Blaschko’s lines (Fig. 1). The face,
palms, soles, and mucous membranes were
spared. Hair, teeth, and nails were normal. Sys-
temic examination did not reveal any abnormal-
ity. Hematological and biochemical investiga-
tions were non-contributory. CT scan of the
brain was normal. Skin biopsy revealed in-
creased epidermal pigmentation without any
basal cell degeneration, pigmentary inconti-
nence, or dermal melanophages.
3) Claudel P, Labbe L, Pedespan JM, Labreze C,
Taieb A: Linear and whorled nevoid hyperme-
lanosis: Report of two cases, Arch Pediatr, 5:
1098–1102, 1998.
4) Hassab El Neby HM, Alsaleh QA, Fathallah MA:
Linear and whorled nevoid hypermelanosis: Re-
port of a case associated with cerebral palsy, Pe-
diatr Dermatol, 13: 148–150, 1996.
5) Yim SY, Lee IY, Rah UW et al: Linear and
whorled nevoid hypermelanosis with delayed
psychomotor development, Yonsei Med J, 37:
290–294, 1996.
6) Akiyama M, Aranami A, Sasaki Y, Ebihara T, Sug-
iura M: Familial linear and whorled nevoid hy-
permelanosis, J Am Acad Dermatol, 30: 831–833,
1994.
7) Kanwar AJ, Dhar S, Ghosh S, Kaur S: Linear and
whorled nevoid hypermelanosis (letter), Int J
Dermatol, 32: 385–386, 1993.
Discussion
Our patient fulfils the diagnostic criteria
of LWNHM laid by Kaltar et al. (1). The ab-