A Case Series of Variants in Nevus Depigmentosus
ISSN: 2320-5407 Int. J. Adv. Res.
11(12), 940-943
Journal Homepage: - www.journalijar.com
Article DOI: 10.21474/IJAR01/18061
DOI URL: http://dx.doi.org/10.21474/IJAR01/18061
RESEARCH ARTICLE
A CASE SERIES OF VARIANTS IN NEVUS DEPIGMENTOSUS
Dr. Sanjay Agrawal1, Dr.Virendra Saoji2 and Dr. Rashmi Pote3
1. Professor & Head, Department of Skin&VD, DrPanjabrao Memorial Medical College, Amravati.
2. Professor, Department of Skin &VD, DrPanjabrao Memorial Medical College, Amravati.
3. Junior Resident, Department of Skin&VD, DrPanjabrao Memorial Medical College, Amravati.
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Manuscript Info Abstract
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Manuscript History This case series presents unique variantsof Nevus Depigmentosus in
Received: 31 October 2023 pediatric patients with widespread distribution. Nevus Depigmentosus,
Final Accepted: 30 November 2023 well known as Achromic nevus, is a rare hypopigmentary skin disorder
Published: December 2023 that usually presents in childhood. Variants of this condition exhibit
diverse clinical presentations and distinct distribution pattern, thereby
Key words:-
Generalized Nevus Depigmentosus, posing diagnostic challenges. By exploring this rare dermatological
Segmental Nevus Depigmentosus phenomenon in pediatric context, we aim to contribute to the limited
literature available on Generalized and Segmental Nevus
Depigmentosus in this age group, ultimately improving clinical
awareness and patient care.
Copy Right, IJAR, 2023,. All rights reserved.
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Introduction:-
Nevus Depigmentosus (ND) is a rare and intriguing pigmentary skin lesion characterized by areas of hypopigmented
or depigmented skin, resulting from the absence of melanocytes. This condition was firstly described by Lesser in
1884.1Variants of Nevus Depigmentosusare been documented in various age groups, but its occurrence in children
as young as 3 years old, with generalized distribution is underreported in medical literature. This condition while
uncommon presents with a variety of clinical manifestations that contribute to its complexity and challenge in
diagnosis. Nevus Depigmentosusneeds to be differentiated from nevus anemicus, hypomelanosis of Ito, vitiligo,
albinism etc.Among the diverse spectrum of nevus depigmentosus variants, the generalized distributed presentation
stands out as a distinct and infrequent manifestation.
Cases Description:
Case1, a 3-year-old female child came to the dermatologic clinic with complaints of hypopigmented skin lesions.
The parents reported that these lesions were first noticed a year back andhave gradually increased in size and
number. There were no associated symptoms. There is no history of similar complaints in family. Upon clinical
examination, the child presented with extensive hypopigmented asymptomatic macules of varied sizes over trunk
and extremities. The macules were distributed bilaterally, and crossed the midline. The lesions measured of 1mm
diameter. There were few hypopigmented macules of approximately 3 cm diameter over right shoulder, lower
abdomen, gluteal region. The borders were well defined with irregular serrated margins that contrast with the
surrounding normal skin. There were no signs of scaling, erythema or atrophy. Mucous membrane, Oral cavity, Hair
and Nail appear normal. The overall development and general health were normal.Patientdid not show cutaneous
lesions other than nevus depigmentosus, and no apparent congenital anomalies were observed.
Corresponding Author:- Dr. Sanjay Agrawal
Address:- Professor & Head, Department of Skin &VD, DrPanjabrao Memorial Medical 940
College, Amravati.
ISSN: 2320-5407 Int. J. Adv. Res. 11(12), 940-943
Case 2 was a 3-year-old male child presented withhypopigmentedpatch over face and neck. The lesions presented
since 6 months. Thepatch over forehead is arranged in a linear pattern with irregular border.They were
asymptomatic with no signs of scaling, erythema or atrophy.Mucous membrane, Oral cavity, Hair and Nail appear
normal. The overall development and general health were normal.Patient did not show cutaneous lesions other than
nevus depigmentosus, and no apparent congenital anomalies were observed.
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ISSN: 2320-5407 Int. J. Adv. Res. 11(12), 940-943
Case 3 was a 1.5-year-old female child presented with hypopigmented patchoverabdomen.The borders were well
defined with irregular and serrated margins.They were asymptomatic.There were no signs of scaling, erythema or
atrophy. Mucous membrane, Oral cavity, Hair and Nail appear normal.
Diagnostic Evaluation:
Under Wood’s lamp examination, the lesion of ND showed an off-white accentuation without fluorescence.On
Diascopy, the lesions were non-blanchable with intact border. To confirm the clinical suspicion a skin punch biopsy
was performed on a hypopigmented macules. The histopathological analysis revealed reduced number of
melanocytes along with reduced melanin pigment. Although complete loss was not evident, the biopsy was
suggestive of Nevus Depigmentosus.
Discussion:-
Nevus Depigmentosus (ND) presents as a stable, discrete, hypopigmented macule or a patch, whose size and
distribution remains the same throughout life. 2-4El-Sayed S. Hewedy et al5 found age incidence mostly present
before 3 years (73.33% cases had the initial lesion before the age of 3 years) as in our cases (100%).Our cases had
lesions with serrated borders which is in line with El-Sayed S. Hewedy et al.5So, the diagnosis of ND can be
considered if patient develops hypopigmented macule with irregular or serrated border at an early age.Nevus
Depigmentosusmay be focal, segmental or generalized in distribution most commonly on the trunk.The main
pathology is secondary to aggregation of melanosomes in the melanocytes and a defective transfer of the same to the
surrounding keratinocytes.There is reduced melanosomalcontentoflesional keratinocytes and melanocytes. Immature
and aggregated melanosomes are present more in lesional keratinocytes.Given the benign nature of Nevus
Depigmentosus and absence of associated symptoms, a conservative management was done initially. Counselling
regarding photoprotection had been given. Regular follow up was done to monitor any changes. No specific
treatment was effective or was necessary. But for cosmetic purpose 308 nm excimer laser and surgical procedures
like blister roof grafting, cultured epidermal grafting, autologous epidermal cell grafts had been used with good
results which has been consistent with Abhijeet Kumar Jha et al.⁶ Park et al.⁷ also stimulated melanogenesis in ND by
using a 308-nm excimer laser and observed marked improvement of the ND lesion.
Conclusion:-
This case series highlights the clinical presentation, diagnosis, and management of Generalized Nevus
Depigmentosus in Case1,Segmental Variant in Case 2 and Case3 in paediatric context. The patients hypopigmented
macules remained stable during 12 months since presentation. So, though conservative management would be a
better option in management of cases ofnevus depigmentosus, excimer laser and surgical procedures like blister roof
grafting, cultured epidermal grafting, autologous epidermal cell grafts can be used with good cosmetic results.
Further research is needed to understand the genetic factors and potential treatments for Nevus Depigmentosus.
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ISSN: 2320-5407 Int. J. Adv. Res. 11(12), 940-943
References:-
1. Lesser E. Anomalien der Hautfärbung. In: von Zeimssen H, editor. Handbuch der Hautkrankheiten. Leipzig,
Germany: FCW Vogel; 1884. p. 161-200.
2. Shim JH, Seo SJ, Song KY, Hong CK. Development of multiple pigmented nevi within segmental nevus
depigmentosus. J Korean Med Sci 2002; 17:133-6.
3. Bolognia JL, Lazova R, Watsky K. The development of lentigines within segmental achromic nevi. J Am
AcadDermatol 1998; 39:330-3.
4. Khumalo NP, Huson S, Burge S. Development of lentigines within naevoid hypopigmentation. Br J Dermatol
2001; 144:188-9.
5. Jha AK, Sinha R, Prasad S. Letters to the Editor Nevus depigmentosus with unilateral bluish sclera, a rare entity
Phaeohyphomycosis presenting as a solitary nodulocystic lesion in a renal transplant patient. 2015;6(5):358–9.
6. Jha AK, Sinha R, Prasad S. Letters to the Editor Nevus depigmentosus with unilateral bluish sclera, a rare entity
Phaeohyphomycosis presenting as a solitary nodulocystic lesion in a renal transplant patient. 2015;6(5):358–9.
7. Park YK, Kim DY, Lee KY. Use of the 308-nm excimer laser for nevus depigmentosus: a promising treatment
for either nevus depigmentosus or vitiligo. Journal of Dermatology 2007; 34:217–8.
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