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Year : 2016  |  Volume : 17  |  Issue : 1  |  Page : 45-47

Central nervous system involvement in a case of segmental nevus depigmentosus

1 Department of Pediatric Medicine, Institute of Child Health, Kolkata, West Bengal, India
2 Department of Pediatric Dermatology, Institute of Child Health, Kolkata, West Bengal, India

Date of Web Publication4-Jan-2016

Correspondence Address:
Ishita Majumdar
AG-104 Sourav Abasan, Flat No. 5/4, Sector - 2, Salt Lake City, Kolkata - 700 091, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2319-7250.173151

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Central nervous system involvement in segmental nevus depigmentosus (SND) is rare. A 7-month-old boy having convulsion and segmental hypopigmented patch in the right inguinal region. Magnetic resonance imaging of brain showed bilateral periventricular white matter hypoplasia with prominent subarachnoid spaces and mild dilation of ventricles with mild left cerebral hemispheric atrophy. Association of SND with seizure and white matter lesion has been rarely reported.

Keywords: Segmental nevus depigmentosus, seizure, white matter hypoplasia

How to cite this article:
Majumdar I, Mukherjee D, Dhar S, Kundu R. Central nervous system involvement in a case of segmental nevus depigmentosus. Indian J Paediatr Dermatol 2016;17:45-7

How to cite this URL:
Majumdar I, Mukherjee D, Dhar S, Kundu R. Central nervous system involvement in a case of segmental nevus depigmentosus. Indian J Paediatr Dermatol [serial online] 2016 [cited 2022 Jan 16];17:45-7. Available from: https://www.ijpd.in/text.asp?2016/17/1/45/173151

  Introduction Top

Nevus depigmentosus (ND syn. nevus achromicus) is described as a congenital, nonfamilial and nonprogressive hypopigmented macule or patch. This is a benign condition which is primarily limited to the skin.[1] However, extra-cutaneous manifestations in the form of seizures, mental retardation, and unilateral limb hypertrophy have been rarely reported. Here, we report a 7-month-old boy infant who presented with afebrile seizures and was found to have segmental ND (SND) in the right inguinal region and white matter hypoplasia in the brain.

  Case Report Top

A 7-month-old boy infant born out of nonconsanguinity by normal vaginal delivery, having normal developmental milestones as per age was admitted in our institution with complain of two consecutive episodes (within 6 h) of generalized tonic-clonic seizures lasting for approximately 30 min each. Seizures were terminated with injection midazolam and were followed by prolonged postictal drowsiness. There was no previous history of the seizure of the child or within the family.

On examination, the child was afebrile. There were no apparent neurological deficits. His pupils were reacting symmetrically to light. There was no bulging of anterior fontanelle. Examination of respiratory, cardiac, and gastrointestinal systems did not reveal any significant abnormality.

A hypopigmented nonprogressive patch (9 cm by 7 cm) with irregular margin without surrounding hyperpigmentation and not crossing the midline was noted in the right inguinal region [Figure 1]. This patch had no sensory deficit. There were no other neurocutaneous stigmata in the child.
Figure 1: A segmental hypopigmented lesion in right groin region with irregular margins and the lesion not crossing the midline

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Sepsis screen, blood sugar, and electrolytes on admission were normal. Liver and renal function tests were also normal. A study of cerebrospinal fluid revealed zero cells with normal protein and sugar. Electroencephalography of the brain was also normal.

Magnetic resonance imaging (MRI) of the brain showed features of bilateral periventricular white matter hypoplasia (possibly due to white matter injury) with prominent subarachnoid spaces and mild dilatation of the ventricles. There was mild left cerebral hemispheric atrophy. The myelination pattern was, however, normal.

Diascopy was found to be negative. Wood's lamp examination of the hypopigmented patch showed off white patch without fluorescence suggestive of SND.

The child is presently on oral phenobarbitone and is in the regular follow-up. Over the last 2 months, there have been no further seizures and no new pigmentary changes. The development of the child is being closely monitored.

  Discussion Top

ND, first described by Lesser in 1884[2] is a congenital disorder of pigmentation that remains stable in its relative size and distribution throughout the life and occurs equally in both sexes.

The pathogenesis behind ND is still controversial. It may occur as a result of defective functioning of melanosomes within the melanocytes or may occur as a result of cutaneous mosaicism with the melanocytes synthesizing less melanin.[3] ND has three clinical patterns: Localized (most common), segmental, and whorled, or systematized. SND is characterized by a unilateral band of hypopigmented lesion along the  Lines of Blaschko More Details, not crossing the midline.[4] Our child with SND had the nonprogressive, hypopigmented lesion localized to the right inguinal region.

ND usually appears by infancy, with the common sites of distribution being the trunk, neck, face, and upper part of the limbs.[3] 19.4% patients present with ND at birth, as our child did.

ND was diagnosed using Coupe's criteria.[3] The diagnosis was confirmed using diascopy which was negative in our patient (unlike nevus anemicus). Wood's lamp examination was used to differentiate from vitiligo. Measurement of relative melanin index by a mexameter can assist in differentiating vitiligo and ND better than the previous use of melanin index.[4] Since, the child did not have any other neurocutaneous stigmata, tuberous sclerosis was excluded.

The incidence of systemic abnormalities associated with SND has been rarely reported. The largest study in India on patients with SND has revealed a rare association with central nervous system involvement.[5] The largest study on 20 children has reported just one child to have neurological problems characterized by seizures, mental retardation, and computed tomography scan changes along with hemihypertrophy. The other child had only pes cavus.[6] Our child presented with seizures and changes in MRI.

The pigmentation in ND possibly occurs due to somatic mutation late in embryogenesis when organ development has already been completed. Hence extra-cutaneous manifestations are not commonly seen unlike in the systematized form of ND or Hypomelanosis of Ito (HOI) (62–94% chance of systemic involvement)[6] which is one of the closest differential diagnosis where the pigmentary changes occur in the initial phase of embryogenesis.[7] However, as our child had pigmentation localized to a segment, HOI was ruled out.

As ND is a nonprogressive skin lesion, the primary treatment involves counseling of parents. Seizures should be controlled with appropriate medications as we have done for our child. However, there have been advances in management options which include cosmetic camouflage, psoralen ultraviolet A therapy, suction blister grafting, melanocyte-keratinocyte transfer,[8],[9] and excimer laser.

The association of extra-cutaneous manifestations with SND has been highlighted in only two case series until date.[6],[10] This highlights the rarity of association and the need for screening for these problems when we diagnose a child with SND.

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Conflicts of Interest

There are no conflicts of interest.

  References Top

Sarma N, Chakraborty S. Birthmarks of clinical significance. In: Sarkar R, Inamadar AC, Palit A, editors. Advances in Pediatric Dermatology. 1st ed., Vol. 2. India: Jaypee Brothers Medical Publishers (P) Ltd.; 2014. p. 198-9.  Back to cited text no. 1
Lesser E. Hanbuchder hautkrankheiten. In: Ziemssen HV, editor. 2nd ed. Leipzig: Vogel; 1884. p. 183.  Back to cited text no. 2
Lee HS, Chun YS, Hann SK. Nevus depigmentosus: Clinical features and histopathologic characteristics in 67 patients. J Am Acad Dermatol 1999;40:21-6.  Back to cited text no. 3
Park ES, Na JI, Kim SO, Huh CH, Youn SW, Park KC. Application of a pigment measuring device – Mexameter – For the differential diagnosis of vitiligo and nevus depigmentosus. Skin Res Technol 2006;12:298-302.  Back to cited text no. 4
Dhar S, Kanwar AJ, Kaur S. Nevus depigmentosus in India: Experience with 50 patients. Pediatr Dermatol 1993;10:299-300.  Back to cited text no. 5
Di Lernia V. Segmental nevus depigmentosus: Analysis of 20 patients. Pediatr Dermatol 1999;16:349-53.  Back to cited text no. 6
Glover MT, Brett EM, Atherton DJ. Hypomelanosis of Ito: Spectrum of the disease. J Pediatr 1989;115:75-80.  Back to cited text no. 7
Mulekar SV, Al Issa A, Al Eisa A. Nevus depigmentosus treated by melanocyte-keratinocyte transplantation. J Cutan Aesthet Surg 2011;4:29-32.  Back to cited text no. 8
[PUBMED]  Medknow Journal  
van Geel N, Wallaeys E, Goh BK, De Mil M, Lambert J. Long-term results of noncultured epidermal cellular grafting in vitiligo, halo naevi, piebaldism and naevus depigmentosus. Br J Dermatol 2010;163:1186-93.  Back to cited text no. 9
Nehal KS, PeBenito R, Orlow SJ. Analysis of 54 cases of hypopigmentation and hyperpigmentation along the lines of Blaschko. Arch Dermatol 1996;132:1167-70.  Back to cited text no. 10


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