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CASE IMAGE
Year : 2021  |  Volume : 1  |  Issue : 4  |  Page : 270

A neonate born with natural garment: Congenital melanocytic nevus


Department of Neonatology, Government Medical College and Hospital, Chandigarh, India

Date of Submission23-Jun-2021
Date of Decision01-Nov-2021
Date of Acceptance06-Nov-2021
Date of Web Publication29-Nov-2021

Correspondence Address:
Dr. Supreet Khurana
D Block, Level IV, Department of Neonatology, Government Medical College and Hospital, Sector 32-B, Chandigarh - 160 030
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ipcares.ipcares_198_21

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How to cite this article:
Siddiqui A, Khurana S. A neonate born with natural garment: Congenital melanocytic nevus. Indian Pediatr Case Rep 2021;1:270

How to cite this URL:
Siddiqui A, Khurana S. A neonate born with natural garment: Congenital melanocytic nevus. Indian Pediatr Case Rep [serial online] 2021 [cited 2022 Jan 20];1:270. Available from: http://www.ipcares.org/text.asp?2021/1/4/270/331371

Monochorionic diamniotic twin girls were born to a primigravida at 34+2 weeks gestation via vaginal delivery. Both cried immediately at birth. The second twin was a girl with a birth weight of 1900 g. A large, black, skin lesion covering her back was noted at birth. Her twin and other family members had no similar lesions. Examination revealed a hyperpigmented nevus, measuring 13 cm × 13 cm, with well-demarcated margins, papular surface, and hypertrichosis [Figure 1]. Smaller hyperpigmented satellite lesions of variable size were present over the buttocks, right foot, left leg, and knee. A clinical diagnosis of giant congenital melanocytic nevus (GCMN), with “bathing trunk” distribution, was made. The cranial sutures and spine were normal. Systemic examination was unremarkable. Magnetic resonance imaging of the brain was normal. Parents were counseled regarding the nature, prognosis, and management of the disease.
Figure 1: Giant congenital melanocytic nevus over the back measuring 13 cm × 13 cm with satellite lesions over the buttocks and thighs

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The overall occurrence of congenital melanocytic nevus is 1%–3%, with slight female preponderance. Large or giant GCMN is reported to occur in 1:20,000 live births, with the variety “GCMN in garment” being even rarer, 1:500,000.[1] Lesions are classified with reference to the projected adult size:[2] small (<1.5 cm); medium (1.5–20 cm); large (>20–40 cm, >9 cm on the head and >6 cm on the body); and giant (>40 cm). These lesions are hypothesized to occur due to gain-of-function somatic mutations in either proto-oncogenes, BRAF V600 variant, or NRAS Q6. These affect the microtubule-associated protein kinase signal transduction pathway, leading to abnormal proliferation of embryonic melanoblasts. Apart from the significant cosmetic disfigurement, GCMN is associated with neurocutaneous melanosis (in 3%–10% of neonates at high-risk category i.e., with lesions >40 cm, multiple satellite lesions or >2 medium-sized lesions);[3] melanomas (in 2%–5% GCMN, particularly >60 cm or with satellite lesions);[4] or malignancies such as liposarcoma and rhabdomyosarcomas.

Management is individualized, the aim being close monitoring for malignancy and neurological involvement, cosmetic rehabilitation, and providing psychological support. Parents need to be counseled about the need for regular local checks of any change in color, texture, and/or size. Earlier, surgical excision was the mainstay to avoid malignant transformation. These included serial resection, excision followed by skin grafts, and tissue expanders followed by resection. However, nowadays, the role of surgery is no longer certain, considering the inability to remove the lesion in entirety, the risk of extensive postsurgical scarring, the absence of actual reduction in risk, especially since there are reports of malignancy occurring in surgically excised areas.[5] Less invasive procedures such as curettage, dermabrasion, and laser ablation are also being used. There are ongoing clinical trials with NRAS inhibitors such as trametinib in individuals with known NRAS mutations and some speculation regarding the use of endothelin-1 receptor antagonists.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's parents have given consent for images and other clinical information to be reported in the journal. The patient's parents understand that the names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Acknowledgments

We would like to acknowledge Prof. Suksham Jain and Prof. Deepak Chalwa in acknowledgments for their role in conducting patient management and content analysis.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Viana AC, Gontijo B, Bittencourt FV. Giant congenital melanocytic nevus. An Bras Dermatol 2013;88:863-78.  Back to cited text no. 1
    
2.
Krengel S, Scope A, Dusza SW, et al. New recommendations for the categorization of cutaneous features of congenital melanocytic nevi. J Am Acad Dermatol 2013;68:441-51.  Back to cited text no. 2
    
3.
Alikhan A, Ibrahimi OA, Eisen DB. Congenital melanocytic nevi: Where are we now? Part I. Clinical presentation, epidemiology, pathogenesis, histology, malignant transformation, and neurocutaneous melanosis. J Am Acad Dermatol 2012;67:495.  Back to cited text no. 3
    
4.
Vourc'h-Jourdain M, Martin L, Barbarot S, et al. Large congenital melanocytic nevi: therapeutic management and melanoma risk: A systematic review. J Am Acad Dermatol 2013;68:493-8.e1.  Back to cited text no. 4
    
5.
Manganoni AM, Belloni Fortina A, Pavoni L, et al. The controversial management of giant congenital melanocytic nevi. When would it be better “to wait and see”? G Ital Dermatol Venereol 2013;148:203-7.  Back to cited text no. 5
    


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