Letters to the Editor
Dermatoscopic features of incontinentia
pigmenti
Sir,
Dermatology consultation was sought by the parents of a
1‑month‑old Indian girl, born out of a non‑consanguineous
marriage, for pigmented lesions on her body, which they had
noticed since birth. Examination revealed linear streaks of
brownish hyperpigmentation on the trunk and limbs with a
striking blaschkoid distribution and showing mild atrophy on
lower limbs [Figure 1a and b]. Small hyperkeratotic plaques
of around 1 cm, which were preceded by bullous lesions at
birth, were present on thumbs. Ocular examination revealed
no abnormalities. Scalp hair was normal in both mother and
the infant. A diagnosis of incontinentia pigmenti was strongly
considered.
Parents were counselled regarding the need for
performing a skin biopsy. Since genetic testing was
planned simultaneously, they agreed for the latter; the
biopsy was deferred because of its invasive nature.
Dermatoscopic examination was performed on an
area having both normal skin and hyperpigmented
streaks using a Dermlite II hybrid M dermatoscope at
10  ×  magnification in polarized noncontact mode and
photographs were captured with the help of Dermlite app
using Apple Iphone 6 plus. The dermatoscopy from the
hyperpigmented streaks characteristically lacked sweat
Figure 1a: Linear streaks of brownish hyperpigmentation in blaschkoid
distribution
422 Indian Journal of Dermatology, Venereology and Leprology | Volume 86 | Issue 4 | July-August 2020
gland orifices  (visible as glowing white dots in normal
skin) and hair follicles, and demonstrated a few gray
dots suggestive of pigment incontinence; consistent with
the diagnosis of hyperpigmented stage of incontinentia
pigmenti [Figure 2a and b].
Investigations revealed peripheral eosinophilia  (absolute
eosinophil count 5400) and a mutation study of the blood
sample revealed mutation in nuclear factor κB essential
modulator  (NEMO) gene confirming the diagnosis of
incontinentia pigmenti.
Incontinentia pigmenti is an X-linked dominant
genodermatosis that characteristically presents in
female infants with typical skin lesions in blaschkoid
distribution, a pattern followed by migrating keratinocytes
during embryogenesis. Four types of lesions have been
described: vesicular, hyperkeratotic, hyperpigmented and
hypopigmented.1 Vesicular lesions reveal histopathological
changes typical of incontinentia pigmenti including
eosinophilic spongiosis and dyskeratosis. Hyperkeratotic
lesions usually follow vesiculobullous lesions and subside
in a few initial months of life.1
Sometimes the initial stages of incontinentia pigmenti occur
and subside during intrauterine life, and hyperpigmented
lesions in a blaschkoid pattern present at birth. Though
atrophy is usually apparent in hyperpigmented stages of
incontinentia pigmenti, histopathological evidence in the
form of loss of pilosebaceous and sudomotor appendages
is more readily demonstrated in hypopigmented lesions.
But, a similar histopathological finding has also been noted
in hyperpigmented streaks  (stage 3).2 Our dermatoscopic
findings in the form of readily apparent loss of sweat gland
orifices and hair, on hyperpigmented streaks, correlated well
with histopathological findings noted in a previous study.2
Figure 1b: Streaks of brownish hyperpigmentation on upper limbs

Figure 2a:Circular, polycyclic and U‑shaped areas of normal skin (red stars)
harboring glowing white dots representing sweat gland orifices (red arrows).
Hair is also visible interspersed between the sweat glands  (roots of hair
marked by brown arrows). The dermatoscopy from the pigmented streaks
demonstrate exaggerated normal reticulate pigmentation and a few gray dots
suggesting pigment incontinence (white arrows); and characteristically lack
the glowing white sweat gland orifices and hair follicles, consistent with the
diagnosis of hyperpigmented stage of incontinentia pigmenti, (yellow squares
bordered by blue lines). There are a few minute satellite areas of normal skin
islands (black stars) in these hyperpigmented streaks, demonstrating normal
sweat glands (Derm Lite II, hybrid M, ×10x, polarized)
Linear and whorled hypermelanosis is an important
clinical differential of hyperpigmented stage of IP. The
dermatoscopic features have been previously published and
demonstrate small hyperpigmented circles/rings, and curved
and parallel pigmented streaks.3,4 Reticulate pigmentation
was also described in one report.5 Unlike dermatoscopy of
incontinentia pigmenti, hypopigmented dots corresponding
to perifollicular areas and sweat gland openings are present
in both whorled and linear lesions. Since dermatoscopic
features of incontinentia pigmenti have not been reported
yet, our findings could help differentiate it from linear and
whorled hypermelanosis in forthcoming cases.
To conclude, these dermatoscopic findings should be actively
looked for in a neonate/infant presenting with streaky
hyperpigmentation and may preclude the need for an invasive
procedure like skin biopsy, especially in the later stages of the
incontinentia pigmenti.
Indian Journal of Dermatology, Venereology and Leprology | Volume 86 | Issue 4 | July-August 2020
Letters to the Editor
Figure 2b: Another area of normal skin (red stars), harboring glowing white
dots representing sweat gland orifices (red arrows). Hair is also visible (roots of
hair marked by brown arrows). The dermatoscopy from the pigmented streaks
demonstrate exaggerated normal reticulate pigmentation and a few gray dots
suggesting pigment incontinence (white arrows); and characteristically lack the
glowing white sweat gland orifices and hair follicles, (yellow squares bordered
by blue lines). Few minute satellite areas of normal skin islands (black stars)
can be seen floating in between these hyperpigmented streaks, demonstrating
normal sweat glands (Derm Lite II, hybrid M, ×10x, polarized)
Declaration of patient consent
The authors certify that they have obtained all appropriate patient
consent forms. In the form, the legal guardian has given his
consent for images and other clinical information to be reported
in the journal. The guardian understands that name and initial of
the child will not be published, and due efforts will be made to
conceal patient identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
Anuradha Bishnoi, Sendhil M. Kumaran,
Keshavamurthy Vinay
Department of Dermatology, Venereology and Leprology, Postgraduate
Institute of Medical Education and Research, Chandigarh, India
423
Letters to the Editor
Correspondence: Dr. Keshavamurthy Vinay,
Department of Dermatology, Venereology, and Leprology,
Postgraduate Institute of Medical Education and Research,
Chandigarh ‑ 160 012, India.
E‑mail: vinay.keshavmurthy@gmail.com
References
1. Poziomczyk CS, Recuero JK, Bringhenti L, Maria FD, Campos CW,
Travi  GM, et al. Incontinentia pigmenti. An Bras Dermatol
2014;89:26‑36.
2. Hadj‑Rabia  S, Rimella  A, Smahi  A, Fraitag  S, Hamel‑Teillac  D,
Bonnefont JP, et al. Clinical and histologic features of incontinentia
pigmenti in adults with nuclear factor‑κB essential modulator gene
mutations. J Am Acad Dermatol 2011;64:508‑15.
3. Ertam  I, Turk  BG, Urkmez  A, Kazandi  A, Ozdemir  F. Linear and
whorled nevoid hypermelanosis: Dermatoscopic features. J Am Acad
Dermatol 2009;60:328‑31.
4. Errichetti  E, Pegolo  E, Stinco  G. Linear and whorled nevoid
hypermelanosis: A  case report with dermoscopic findings. Indian J
Dermatol Venereol Leprol 2016;82:91‑3.
5. Naveen KN, Reshme P. Linear and whorled nevoid hypermelanosis
with dermatoscopic features. Dermatol Online J 2014;20. pii:
doj_21760.
Pagetoid dyskeratosis of hands: Report of
two cases and the usefulness of dermoscopy
Sir,
Pagetoid dyskeratosis  is an incidental pathologic finding
which can be noted in skin biopsies from different lesions and
body locations.1 Friction has been proposed as an inducing
factor.1 Actually, the real nature of these cells and their causes
are not well known. Pagetoid dyskeratosis has been described
in different benign skin lesions, such as naevi, soft fibromas,
acrochordons, lentigos, fibrous papules, milia and seborrheic
keratoses.1
Only two papers have been published describing the dermoscopic
findings of lesions with pagetoid dyskeratosis which consisted
of a parallel ridge pattern.2,3 Herein, we report two new cases of
pagetoid dyskeratosis in patients with brown pigmentation of
hands and parallel ridge pattern on dermoscopy.
Case 1
A 26‑year‑old woman presented with brown patches on her
right palm lasting for 1  year  [Figure  1a]. She referred that
she had started working as a housecleaner one year ago.
Dermoscopy showed a brown pigmentation with parallel
ridge pattern sparing the furrows [Figure 1b]. A skin biopsy
demonstrated some large keratinocytes in the upper layers
of the epidermis, with pale cytoplasm and pyknotic nucleus,
surrounded by a clear halo  [Figure  1c]. A  year later, she
started working as a salesperson and the pigmentation almost
completely disappeared [Figure 1d].
424 Indian Journal of Dermatology, Venereology and Leprology | Volume 86 | Issue 4 | July-August 2020
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How to cite this article: Bishnoi A, Kumaran SM, Vinay K.
Dermatoscopic features of incontinentia pigmenti. Indian J Dermatol
Venereol Leprol 2020;86:422-4.
Received: April, 2019. Accepted: December, 2019.
© 2020 Indian Journal of Dermatology, Venereology and Leprology | Published by
Wolters Kluwer - Medknow
Case 2
A 31‑year‑old woman presented with a 3‑year history of
brown spots on her fourth left finger  [Figures  2a], which
progressively increased in size. She did not recall any
suspected trigger. Dermoscopy showed a parallel ridge
pattern  [Figure  2b]. A  skin biopsy showed pagetoid cells
with no other findings, matching the description of pagetoid
dyskeratosis. A few months later, the discoloration had almost
disappeared spontaneously [Figure 2c].
Pagetoid dyskeratosis is thought to be a reactive keratinocyte
response to mechanical trauma which leads to premature
keratinization of a small part of the normal keratinocyte
population. 1,4 This is probably the reason why it is more
frequent in areas more commonly exposed to physical injury,
such as intertriginous areas, trunk, buttocks, face and limbs.1
Keratinocytes in pagetoid dyskeratosis have a size larger
than normal, usually a roundish shape, a pale eosinophilic
cytoplasm and a pyknotic nucleus surrounded by a clear halo.
These cells usually predominate in the upper layers of the
epidermis. They are similar to the cells present in Paget’s
disease, although the latter show large atypical nuclei.4
Clinically, pagetoid dyskeratosis tends to appear as
hyperpigmented lesions, probably because of its relation
to friction.3,4 Both our patients had brown discoloration of