Indian Journal of Dermatology
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CORRESPONDENCE
Year : 2021  |  Volume : 66  |  Issue : 4  |  Page : 430-431
Gradual Spontaneous Fading of Giant Congenital Melanocytic Nevi of the Scalp and Trunk


1 Department of Dermatology, Pontificia Universidad Católica de Chile, Santiago, Chile
2 ASM Dermatología; Hospital Alemán de Buenos Aires, Argentina
3 School of Medicine, Faculty of Medicine, Pontificia Universidad Católica de Chile, Santiago, Chile
4 ASM Dermatología, Hospital Alemán de Buenos Aires, Argentina
5 Pathology, Faculty of Medicine, Pontificia Universidad Católica de Chile, Santiago, Chile

Date of Web Publication17-Sep-2021

Correspondence Address:
María S Zegpi
Department of Dermatology, Pontificia Universidad Católica de Chile, Santiago
Chile
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_417_19

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How to cite this article:
Zegpi MS, Santos A, Hasbún C, Majluf P, Vergara V, Villarroel A. Gradual Spontaneous Fading of Giant Congenital Melanocytic Nevi of the Scalp and Trunk. Indian J Dermatol 2021;66:430-1

How to cite this URL:
Zegpi MS, Santos A, Hasbún C, Majluf P, Vergara V, Villarroel A. Gradual Spontaneous Fading of Giant Congenital Melanocytic Nevi of the Scalp and Trunk. Indian J Dermatol [serial online] 2021 [cited 2021 Dec 2];66:430-1. Available from: https://www.e-ijd.org/text.asp?2021/66/4/430/326123




Congenital melanocytic nevi (CMN) appear at birth or shortly after. Giant CMN (GCMN) arise in 1 in 20,000–500,000 births[1] and carry a higher risk of melanoma.

Spontaneous involution is a rare event, often reported in association with halo phenomenon or vitiligo.[2] Induration, ulceration, and residual sclerosis have also been described.[3] We present two cases of spontaneous regression of GCMN without a residual lesion.


   Case 1 Top


A 2-month-old boy presents for evaluation of a lesion on his scalp present at birth [Figure 1]a. A diagnosis of GCMN was made, and the findings of the magnetic resonance imaging (MRI) of the brain were normal. On a 3-year follow-up, the nevus had faded [Figure 1]b. Four biopsies were carried out, with the results showing dermal CMN [Figure 1]c.
Figure 1: Clinical presentation of a patient with a GCMN of the scalp. (a) 2 months of age: there is a pigmented, round macule involving the occipital area and vertex. (b) 3 years old: the CMN has clinically regressed with darker hair on the site of the nevus. (c) Histopathology of case 1: mature, medium- to small-sized nevus cells are seen with no inflammatory infiltrate (HE, 40×)

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   Case 2 Top


A 4-month-old girl is referred for a brownish-black lesion on the posterior trunk present at birth [Figure 2]a. A diagnosis of GCMN was made; brain and whole spine MRI was normal.
Figure 2: Clinical presentation of a patient with a GCMN of the trunk. (a) 4 months of age: there is a brownish-black macule on the posterior trunk with several satellite lesions. (b) 3 years old: mild hyperpigmentation is seen on the site of the nevus

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The patient received follow-up every 6 months, with progressive loss of pigment. There is no halo phenomenon, atrophy, or any other significant change [Figure 2]b. Several biopsies showed persistence of CMN on the dermis without cellular destruction, inflammation, or fibrosis.

Spontaneous regression rarely occurs in pigmented lesions and is mostly associated with the development of a depigmented halo; however, there are reports of CMN that show regression without a peripheral halo or vitiligo.[3],[4],[5] Current literature suggests that there is a decrease in melanin production rather than an immunologic reaction, since biopsy specimens show amelanotic cells without an inflammatory infiltrate.[3],[4],[5] We were able to find only one report that showed inflammation, the one by Gass et al.,[3] where a neonatal biopsy exhibits an inflammatory infiltrate that was no longer present when the nevus had regressed. However, the CMN in this report had a large ulceration and healed with a desmoplastic reaction; as these features were not present in either one of our cases, we cannot hypothesize as to this mechanism of involution of GCMN.

A report by Zack et al.[4] shows hyperpigmentation as a residual sign after the involution of a GCMN. A biopsy when pigmentary regression had not yet occurred showed no inflammation. The authors present several theories as to why the dermal nevus cells lose their ability to produce pigment, including aging and a hormonal factor; however, an early autoimmune response cannot be ruled out.

The biopsy samples in both our cases were taken when clinical involution had occurred, and the persistence of nevus cells without an inflammatory infiltrate was seen. Therefore, the evidence in our report supports the hypothesis that there is a decrease in melanin production rather than an autoimmune reaction that destroys dermal nevus cells. However, it is possible that there is an early immunologic process that has not been identified in biopsy samples. Further research is needed to elucidate the different mechanisms of pigmentary regression in CMN.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Ibrahimi OA, Alikhan A, 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. 1
    
2.
Lee NR, Chung H, Hong H, Lee JW, Ahn SK. Spontaneous involution of congenital melanocytic nevus with halo phenomenon. Am J Dermatopathol 2015;37:e137-9.  Back to cited text no. 2
    
3.
Gass JK, Grant JW, Hall PN, Atherton DJ, Burrows NP. Clinical resolution of a neonatally eroded giant congenital melanocytic nevus. Pediatr Dermatol 2006;23:567-70.  Back to cited text no. 3
    
4.
Zack LD, Stegmeier O, Solomon LM. Pigmentary regression in a giant nevocellular nevus: A case report and a review of the subject. Pediatr Dermatol 1988;5:178-83.  Back to cited text no. 4
    
5.
Kageshita T, Inoue Y, Ono T. Spontaneous regression of congenital melanocytic nevi without evidence of the halo phenomenon. Dermatology 2003;207:193-5.  Back to cited text no. 5
    


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