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 Table of Contents  
Year : 2022  |  Volume : 17  |  Issue : 1  |  Page : 137-139

A congenital melanocytic hairy nevus of the face and conjunctival nevus: A real management dilemma for the pediatrician?

1 Department of Pediatrics, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences, Wardha, Maharashtra, India
2 Department of Pediatrics, Datta Meghe Medical College, SMHRC, Wanadongri, Maharashtra, India

Date of Submission28-Feb-2022
Date of Decision12-Mar-2022
Date of Acceptance18-Mar-2022
Date of Web Publication25-Jul-2022

Correspondence Address:
Dr. Mahaveer Singh Lakra
Department of Pediatrics, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences, Sawangi (Meghe), Wardha - 442 004, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jdmimsu.jdmimsu_70_22

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How to cite this article:
Lakra MS, Vagha KJ, Vagha J, Lakra A. A congenital melanocytic hairy nevus of the face and conjunctival nevus: A real management dilemma for the pediatrician?. J Datta Meghe Inst Med Sci Univ 2022;17:137-9

How to cite this URL:
Lakra MS, Vagha KJ, Vagha J, Lakra A. A congenital melanocytic hairy nevus of the face and conjunctival nevus: A real management dilemma for the pediatrician?. J Datta Meghe Inst Med Sci Univ [serial online] 2022 [cited 2022 Aug 16];17:137-9. Available from: http://www.journaldmims.com/text.asp?2022/17/1/137/352248

  Case 1 Top

A 16-month-old female child, a product of a nonconsanguineous marriage, presented with a history of dark discoloration of the skin and a tuft of hair over the right side of her face. The mother sought treatment for disfigurement and expressed concern about the nature of the lesion. Since birth, the mother noticed skin changes and hair growth all over her face, which was gradually increasing in size. On cutaneous examination, a dark-colored pigmented patch was noticed measuring 6.5 cm × 4.8 cm in size with a tuft of hair over it as shown in [Figure 1]. The child was neurologically normal with a normal spine and skeletal examination. Based on the characteristic presentation of skin discoloration, the presence of a tuft of hair, and other factors, a diagnosis of pigmented congenital hairy nevus was made. After a dermatologist's opinion, the child was advised to take conservative treatment and no biopsy or excision was done and we decided to keep the child in follow-up.
Figure 1: Congenital melanocytic hairy nevus in a child consisting of tufts of hair and skin discoloration over the right side of the face. The surrounding skin is clear with well-defined margins

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  Case 2 Top

Another similar but variant case of a 6-year-old male child presented with small, nonprogressive dark discoloration over the conjunctiva of the right eye for 3 years of age. There were no other dark spots or discolorations present in any of the body parts. No history of any weakness of any body part and no visual difficulty or seizures. A well-defined spot of brownish discoloration of size 4 mm × 3 mm, with a well-defined margin was present over the palpebral conjunctiva in the right eye as shown in [Figure 2]. There was no redness, ecchymosis, or discharge in the eye. The rest examination of both eyes was normal. We decided to keep this child in follow-up after the ophthalmologist's opinion.
Figure 2: Well-defined dark brownish spot of a nevus on the medial side of palpebral conjunctiva of the right eye

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Both of these examples are melanocytic nevi, one of which is a hairy nevus across the face and the other is a conjunctival nevus. The kind, nature, association, and evolution of the nevus were conveyed to the parents. The nature of the disease was explained and we were advised to monitor strictly for the worsening of symptoms to rule out any transformation in malignancy. As it was not increasing in size, and the concern was cosmetic disfigurement, we decided to wait and watch both of the cases.

The prevalence of congenital hairy nevus varies and can reach 1 in 500,000 live births. These congenital melanocytic hairy nevi may be associated with malignancy of different tissues and central nervous system abnormalities. Before the adolescent period, there is a 2.5% chance of malignant development.[1] Congenital hairy melanocytic and conjunctival nevus are the types of birthmarks mostly seen at birth and are found in 1% of infants throughout the world. It is a pigmented nevus which is generally present at birth and grows as the age of the child increases. Pathogenesis is the defective migration, proliferation of melanocytes, and defects in cell maturation during the embryonic period.[2] Congenital hairy nevus is not generally inherited but occurs due to gene mutations in cells. It may be seen in any part of the body, at any age, and in any race. The characteristic features vary from asymptomatic, dry, scaly skin to dark, localized discoloration of the skin, tufts of hair, and cosmetic disfigurement. It is mostly seen in the back, abdomen, thighs, arms, and sometimes over the face and neck area. Some of the nevi may be associated with abnormal patchy hair growth, giving rise to the appearance of pigmented hairy nevi.[3]

They are classified into small, medium, and large sizes depending on the size of the nevus. Ours was a medium-sized nevus, measuring 6.5 cm × 4.8 cm. If the size of the congenital hairy nevus is >20 cm (8 inches), then they are called “giant congenital hairy nevi,” which are more prone to turning into malignant melanoma.[2],[3] These are generally benign but may turn into malignant if there is stellar, multiple, or neurological involvement, and the chances of turning into malignant mainly depend on the size, grade, depth, and area of localization.[3],[4]

Conjunctival melanocytic nevus is a rare entity seen in children, although benign nevus is more common. The darkly pigmented lesions of the conjunctiva include nevus, primary acquired melanosis, and complexion-associated melanosis. The dark melanocytic nevus forms 53% of the malignancy of the conjunctiva. Out of these, nevus is the most common lesion type, which is mostly benign but may turn malignant. Depending on where the proliferation occurs in the conjunctiva, there are three types: benign, junctional, and subepithelial.[5] In 70% of the cases, nevi are located in interpalpebral fissures and most of the lesions are benign. An unusual case of a juvenile nevus with underlying inflammation and atypical histopathological changes has been reported.[6] The periorbital site is usually not involved in congenital hairy nevus, but if it is, a full eye examination is recommended because it can sometimes extend into the deep structures of the eye.[7]

The treatment modalities for these melanomas mainly include close observation, derma abrasion, curettage, laser therapy, and surgical removal. Small- and medium-sized nevi should be excised surgically and the surrounding margins should be cut extensively to prevent further progression into malignancy. Benign lesions should be monitored for changes in malignancy and followed over time.[8] The latest guidelines say that even the prophylactic excision of facial hairy nevus is not recommended due to cosmetic facial issues. Closure of the defect by surrounding tissue and then primarily closure is possible but a difficult task. This can be excised if parents are concerned with cosmetics in adulthood, but it is better to leave if there are chances of disfigurement and difficulty in incising due to the involvement of deep structures.[9]

There are no current guidelines that specify which patients should undergo surgical excision and biopsy for the complete evaluation. It is always better to wait and watch in such cases to avoid facial disfigurement but simultaneously, there is always a risk of 1%–5% of transformation into a malignant state. This decision is very crucial and we should always keep a close watch on the progression of the symptoms in such cases. This is equally important from the psychosocial aspect of the family also as it can have an adverse impact due to the esthetic look of the lesion. These lesions are sometimes associated with other neurocutaneous syndromes and may progress to malignant melanoma, so the approach should be individualized.

  Conclusion Top

The family members of the child should be counseled about the nature of the disease and advised to self-monitor at home to detect early changes of malignancy. The question of whether these kids should get a biopsy and surgery at this point is still up for debate. This awareness will also encourage doctors to take the right decision at the right time for the benefit of the patient, although it can be challenging. The malignant transformation of such nevus should always be kept in mind and it must be investigated if symptoms become alarming.

Declaration of patient consent

The authors clarify that they have obtained all appropriate consent from patients' relatives. In the form, the relatives have given their consent for the image and other clinical information to be reported in the journal. They understand that their names and initials will not be published and due efforts will made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Hale C, Pernick N. Congenital Nevus. Pathology Outlines. Available from: https://www.pathologyoutlines.com/topic/skintumormelanocyticmelanomacongenital.html. [Last accessed on 2022 Jun 28].  Back to cited text no. 1
Tannous ZS, Mihm MC Jr., Sober AJ, Duncan LM. Congenital melanocytic nevi: Clinical and histopathologic features, risk of melanoma, and clinical management. J Am Acad Dermatol 2005;52:197-203.  Back to cited text no. 2
Pareekutty NM, Kattepur AK. Congenital nevus with metastatic melanoma. Indian J Surg Oncol 2020;11:306-7.  Back to cited text no. 3
Jiya FB, Sanni MA, Jiya NM, Aquib DM, Umar M, Abba MH, et al. Congenital melanocytic hairy nevi in a child from north-western Nigeria. Asian J Med Health 2020;18:28-34. [doi: 10.9734/ajmah/2020/v18i730221].  Back to cited text no. 4
Paro AM, de Barros GF, Passos JP, Burnier J, Abreu E, Burnier MN. A 10-Year Study of Melanocytic Lesions of the Conjunctiva. ARVO Annual Meeting. Abstract; July 2019.  Back to cited text no. 5
Colarossi C, Milazzo M, Paglierani M, Massi D, Memeo L, Canzonieri V. A juvenile case of conjunctival atypical nevus. Diagn Pathol 2013;8:64.  Back to cited text no. 6
Raina UK, Seth A, Gupta A, Batta S. Ocular findings in a case of periorbital giant congenital melanocytic nevus. Oman J Ophthalmol 2014;7:153-5.  Back to cited text no. 7
[PUBMED]  [Full text]  
Sakhiya J, Sakhiya D, Patel M, Daruwala F. Giant congenital melanocytic nevi successfully treated with combined laser therapy. Indian Dermatol Online J 2020;11:79-82.  Back to cited text no. 8
[PUBMED]  [Full text]  
Fahradyan A, Wolfswinkel EM, Tsuha M, Reinisch JF, Magee W 3rd, Hammoudeh JA, et al. Cosmetically challenging congenital melanocytic nevi. Ann Plast Surg 2019;82 5S Suppl 4:S306-9.  Back to cited text no. 9


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