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 Table of Contents  
THE CLINICAL PICTURE
Year : 2019  |  Volume : 6  |  Issue : 2  |  Page : 113-114

Giant congenital melanocytic nevus with neurofibroma-like papules


Department of Dermatology and Venereology, Institute of Medicine, Tribhuvan University, Kathmandu, Nepal

Date of Web Publication19-Dec-2019

Correspondence Address:
MBBS, Resident M. M Aarif Syed
Department of Dermatology and Venereology, Institute of Medicine, Tribhuvan University, Kathmandu
Nepal
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/Pigmentinternational.Pigmentinternational_

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How to cite this article:
Shrestha DP, Aarif Syed MM. Giant congenital melanocytic nevus with neurofibroma-like papules. Pigment Int 2019;6:113-4

How to cite this URL:
Shrestha DP, Aarif Syed MM. Giant congenital melanocytic nevus with neurofibroma-like papules. Pigment Int [serial online] 2019 [cited 2020 Apr 3];6:113-4. Available from: http://www.pigmentinternational.com/text.asp?2019/6/2/113/273459



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A 4-month-old male child presented to us with extensive black pigmented lesions on his back, upper chest, neck, and bilateral shoulders. The lesions were present since birth. The antenatal history was normal. There was no delay in achieving developmental milestones till date and without any history of seizure or focal neurological deficits. The child was the first product of a nonconsanguineous marriage. On physical examination, large hyperpigmented plaque covering major portion of back, neck, upper chest, and axilla with multiple satellite lesions involving lower back, buttock, and abdomen were noticed [Figure 1]. The plaques were studded with multiple neurofibroma-like soft nodules and papules ranging in sizes 1 × 1 cm to 3 × 3 cm soft on the back along with hair on the shoulder and neck [Figure 1]. No associated congenital anomaly was noted on examination. A diagnosis of giant congenital melanocytic nevus with neurofibroma-like papules was made. The patient was offered biopsy for histopathological examination (to rule out melanoma), Magnetic Resonance Imaging (MRI) scan of the brain (to rule out neurocutaneous melanosis), and X-ray of the chest and lumbosacral spine that was declined, despite counselling about the prognosis.
Figure 1 Large hyperpigmented patch covering major portion of back, neck, upper chest, and axilla with multiple satellite lesions involving lower back, buttock, and abdomen. The patch is studded with multiple soft swellings on the back along with hair on the shoulder and neck.

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Giant congenital melanocytic nevus (GCMN) is defined as a melanocytic lesion in excess of size 20 cm diameter.[1] It is a rare disorder and occur in 1% to 2% of newborns. It causes severe cosmetic distress to patient and has the propensity to develop into malignant melanoma with a lifetime risk of 5% to 10%.[2] GCMN may be a part of neurocutaneous melanosis in 2.5% to 7% of cases,[3] so a thorough neurological examination with MRI scan of brain is imperative. Association of GCMN with neurofibromatosis is also documented,[2] which could have been our case also. Chemical peels, dermabrasion, skin curettage, and shave excision are effective for superficial lesions, but with limited utility. Excision and debulking the melanocytic mass are the treatment of choice. Laser can be used, where surgical management is difficult.

Declaration of patient consent

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


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Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Leite VAC, Bernardo G, Vasques BF. Giant congenital melanocytic nevus. An Bras Dermatol 2013;88:863-78.  Back to cited text no. 1
    
2.
Silfen R, Skoll PJ, Hudson DA. Congenital giant hairy nevi and neurofibromatosis: the significance of their common origin. Plast Reconstr Surg 2002;110:1364-5.  Back to cited text no. 2
    
3.
Slutsky JB, Barr JM, Femia AN, Marghoob AA. Large congenital melanocytic nevi: associated risks and management considerations. Semin Cutan Med Surg 2010;29:79-84.  Back to cited text no. 3
    


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