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© 2017 Indian Journal of Dermatology, Venereology, and Leprology | Published by Wolters Kluwer - Medknow
Sir,
Speckled lentiginous nevus (SLN) is a relatively uncommon
melanocytic nevus clinically characterized by the presence of dark
colored macules and/or papules scattered over the pale brown
base.1 Vitiligo has also been reported with this nevus but in “halo”
conguration in the surrounding normal skin.2 Here, we report
a unique case of co‑localization of trichrome vitiligo “within”
the congenital segmental speckled lentiginous nevus on face in a
9‑year‑old girl.
A 9‑year‑old girl came to the outpatient department of dermatology
in a government medical college and hospital, Haldwani. She
presented with a well demarcated, evenly pigmented brownish
patch (base) superimposed with multiple scattered, dark brown
colored macules on left cheek and periocular area since her infancy.
She had also two depigmented patches within the brownish base
since 2 years. Parents revealed that pale brown larger patch was
present at birth but smaller dark colored scattered macules started
developing since the age of 2 years and ceased to grow in number
by the age of 7 years. There was no history of any pre‑existing
dermatosis. Personal and family history of any autoimmune
disorders was non‑contributory.
Cutaneous examination showed a well dened, pale brown or
tan patch of size 8 cm × 12 cm, speckled with numerous small
darkly pigmented macules of size 1 mm–3 mm. One vitiligo patch
affected the skin of inner canthus, medial side of upper eyelid
and infra‑orbital region, showing ill‑dened borders and some
hypopigmented area in contact with normal skin suggestive of the
three colors of trichrome vitiligo [Figure 1]. The other depigmented
patch was present over left cheek. Rest of the mucocutaneous and
systemic examination was unremarkable and routine investigations
were within normal limits.
Histopathological examination (HPE) of punch biopsy specimen
taken from small dark speckled macular component of nevus and
depigmented patch was done. Dark coloured speckled small macule
revealed melanocytic hyperplasia in basal layer, dermal aggregation
of naevomelanocytic cells along with junctional activity, while
depigmented patch demonstrated complete absence of melanocytes
in the basal layer of the epidermis and no nevomelanocytic cells in
dermis [Figures 2 and 3]. Common to these two lesions were the
presence of mononuclear inammatory inltrates in the papillary
dermis suggesting some immunological basis of these lesions. Due
to the poor infrastructure and nancial constraints of the patient,
staining for melanocytes and immunohistochemistry for inltrating
dermal mononuclear cells could not be done.
Based on typical history of evolution, characteristic clinical
appearance and the histopathological ndings, it was concluded as a
case of “Congenital segmental speckled lentiginous nevus,” which
was co‑localized with trichrome vitiligo.
Because of the very young age of patient, no aggressive treatment
was offered except topical application of tacrolimus and basic
broblast growth factor solution (bFGF) for vitiligo but patient did
not turn up further.
It is usually noticed during infancy or childhood with the
development of tan or brown patch as base on which multiple
dark flat or elevated lesions arise over the time due to
nevomelanocytic aggregations at dermoepidermal junction or
dermis and tends to persist for life although its spontaneous
regression is possible.3‑5
There is an interesting and complex association between
melanocytic nevus (congenital or acquired) and vitiligo as
evidenced by occasional development of vitiligo lesions around
Vitiligo developing in congenital segmental
speckled lentiginous nevus: Another example
of immunocompromised cutaneous district
due to immunological assault on aberrant
melanocytes?
Leer to the Editor
Figure 1: Large, pale brown patch speckled with numerous, small darkly
pigmented macules on left side of face
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Letter to the Editor
Indian Journal of Dermatology, Venereology, and Leprology | Volume XX | Issue XX | Month 20172
melanocytic nevus. The relative conguration and chronological
sequence of development of the melanocytic nevus and vitiligo may
vary. The depigmented patch usually follows the pre‑existing nevus
and encircle it like “halo,” classically seen in halo nevi. Speckled
lentiginous nevus is no exception as it was seen to be encircled by
vitiligo lesions in one such case reported previously in literature.2
Of note, we were unable to nd any previous reports describing the
development of vitiligo “within” the speckled lentiginous nevus as
in our case.
The co‑occurrence of melanocytic nevus and vitiligo is fascinating
to dermatologists. It has been proposed that both melanocytic nevi
and vitiligo occur due to mosaicism of abnormal melanocytes
leading to the stimulation of cellular immunity. In vitiligo,
melanocytorrhagic apoptosis of abnormal melanocytes ultimately
leads to depigmentation while in cases of melanocytic nevi,
immune reactions against nevomelanocytes may face both outcome;
regression of nevi or development of depigmented patche/s, “within”
or “around” the nevus due to immunological clearance of normal or
abnormal melanocytes, respectively.6
In recent few years, a new concept has been put forward to
explain many isotopic development of dermatoses as “locus
minoris resistentiae” which was collectively termed as
“Immunocompromised cutaneous district (ICD).” According
to the current concept of ICD, a complex and altered interplay
between immune cells, nerve bres and neuromediators occurs
within the primary lesion, resulting in localized immune
dysregulation which may lead to the development of secondary
lesion manifesting as opportunistic infections, tumors, or immune
reactions/disorders.7
Development of secondary lesion in the form of depigmented
patch of vitiligo (immune reaction) over the speckled lentiginous
nevus (primary lesion) justies it as a new example of ICD.
Histopathology of such melanocytic nevus, in addition to basal
melanocytic hyperplasia and nevomelanocytic aggregations
in papillary dermis, shows the presence of supercial dermal
lymphocytic inltrates in both the biopsy specimen, depigmented
patch of vitiligo and melanocytic nevus further supporting the
role of immunological reactivity against shared melanocytic
antigens.
With all such clinicopathological descriptions of this fascinating
association and based on newer concept of ICD, our case also
justies to be considered as another ICD caused by immunological
assault on aberrant melanocytes of mosaic origin.
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 will not be
published and due efforts will be made to conceal patient identity,
but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conicts of interest
There are no conicts of interest.
Anup Kumar Tiwary, Piyush Kumar1
Department of Dermatology and Venereology, Government Medical
College and Hospital, Haldwani, Uttarakhand, 1Department of
Dermatology, Katihar Medical College and Hospital, Katihar, Bihar, India
Correspondence: Dr. Anup Kumar Tiwary,
House No. 12, Diamond Building, Government Medical College,
Haldwani, Uttarakhand, India.
E‑mail: anup07tunnu07@gmail.com
References
1. Thappa DM, Garg BR, Bansal D, Ratnakar C. Speckled lentiginous
naevus. Indian J Dermatol Venereol Leprol 1995;61:224‑5.
2. Kim YY, Kim MY, Kim TY. Development of halo nevus around nevus
spilus as a central nevus, and the concurrent vitiligo. Ann Dermatol
2008;20:237‑9.
3. Corradin MT, Cacitti V, Giulioni E, Patriarca MM, Vettorello A. Nevus
spilus: A review of the literature. SM J Dermatol 2015;1:1003.
4. Kalla G, Purohit S, Purohit A, Vyas M. Speckled lentiginous naevus.
Indian J Dermatol Venereol Leprol 1996;62:329‑30.
5. Cecchi R, Fancelli L, Troiano M. Melanoma arising in giant zosteriform
nevus spilus. Indian J Dermatol Venereol Leprol 2012;78:643‑5.
Figure 3: Depigmented patch of vitiligo with complete absence of melanocytes
in the basal layer of the epidermis and lymphocytic collections in papillary
dermis (H and E, ×100)
Figure 2: Small, dark coloured scattered macule revealing melanocytic
hyperplasia in basal layer and dermal aggregation of naevomelanocytic cells
along with junctional activity (black arrow) (H and E, ×400)
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Letter to the Editor
3Indian Journal of Dermatology, Venereology, and Leprology | Volume XX | Issue XX | Month 2017
6. Attili VR, Attili SK. Anatomical segmentations in all forms of vitiligo:
A new dimension to the etiopathogenesis. Indian J Dermatol Venereol
Leprol 2016;82:379‑88.
7. Caccavale S, Kannangara AP, Ruocco E. The immunocompromised
cutaneous district and the necessity of a new classication of its
disparate causes. Indian J Dermatol Venereol Leprol 2016;82:227‑9.
How to cite this article: Tiwary AK, Kumar P. Vitiligo developing in
congenital segmental speckled lentiginous nevus: Another example of
immunocompromised cutaneous district due to immunological assault
on aberrant melanocytes?. Indian J Dermatol Venereol Leprol 0;0:0.
Received: January, 2017. Accepted: May, 2017.
© 2017 Indian Journal of Dermatology, Venereology, and Leprology | Published by
Wolters Kluwer - Medknow
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