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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 29  |  Issue : 1  |  Page : 43-46

Coat-sleeve type giant congenital melanocytic nevus with intraoral blue nevus: A rare case report


1 Department of Oral Medicine and Radiology, Chhatrapati Shahu Maharaj Shikshan Sanstha Dental College and Hospital, Aurangabad, Maharashtra, India
2 Department of Oral Medicine and Radiology, Kusum Devi Sunderlal Dugar Jain Dental College and Hospital, Kolkata, West Bengal, India
3 Department of Oral Medicine and Radiology, Rural Dental College, Loni, Maharashtra, India

Date of Submission29-Jan-2016
Date of Acceptance28-May-2017
Date of Web Publication04-Aug-2017

Correspondence Address:
Sneha H Choudhary
Kusum Devi Sunderlal Dugar Jain Dental College and Hospital, Kolkata, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaomr.JIAOMR_14_16

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   Abstract 

Congenital melanocytic nevi (CMN) are visible hyperpigmented (melanocytic), benign, tumor like proliferations in the skin resulting from faulty development of pigment cell precursors in the embryo, and are composed of an abnormal mixture of skin elements. Giant congenital melanocytic nevus (GCMN) is usually defined as a melanocytic lesion present at birth that will reach a larger size in adulthood. GCMN is a rare variety of CMN which is characterized by its size (diameter ≥20 cm) and the potential for malignant transformation. It is infrequently associated with other findings, which makes the clinical picture complex. In this case, we report a rare association of GCMN with intraoral blue nevus in a 24-year-old male patient.

Keywords: Blue nevus, congenital melanocytic nevus, giant congenital melanocytic nevus, intraoral nevus


How to cite this article:
Kale LM, Choudhary SH, Muley PB, Mishra SS. Coat-sleeve type giant congenital melanocytic nevus with intraoral blue nevus: A rare case report. J Indian Acad Oral Med Radiol 2017;29:43-6

How to cite this URL:
Kale LM, Choudhary SH, Muley PB, Mishra SS. Coat-sleeve type giant congenital melanocytic nevus with intraoral blue nevus: A rare case report. J Indian Acad Oral Med Radiol [serial online] 2017 [cited 2019 Sep 18];29:43-6. Available from: http://www.jiaomr.in/text.asp?2017/29/1/43/212080


   Introduction Top


Giant congenital melanocytic nevus (GCMN) may be defined as a melanocytic lesion which is present usually at birth or appears within first few months after birth reaching a diameter ≥20 cm in adulthood.[1] These are also known as bathing trunk, coat sleeve, or stocking nevi. GCMN appears dark brown to black in color with irregular margins, verrucous surfaces, and satellite lesions which are present beyond the periphery of main lesion.[2] GCMN of size larger than 20 cm in diameter are very rare and occurs in 1 per 5 lakh newborns, with slight female predilection.[3],[4] Here, we report a case of GCMN with intraoral nevus in a 24-year-old male.


   Case Report Top


A 24-year-old male patient reported to the department of oral medicine and radiology with the complaint of pain in his right maxillary and mandibular posterior teeth since 1 week. Extraoral examination revealed a large grayish black hyperpigmented patch on the left hand with numerous hairs (hypertrichosis) [Figure 1]a, following which a detailed prenatal, natal, postnatal, and family history were recorded. Patient gave a history of presence of this hyperpigmented patch since birth, which was darker earlier but had become lighter over the period of 24 years. Patient gave no history of any associated symptoms with the patch. No significant prenatal or family history was reported. Patient had visited various medical hospitals for the same reason where he was advised plastic surgery, however, due to some financial reasons he did not undergo surgery.
Figure 1: (a and b) Grayish black hyperpigmented patch with numerous hairs on left hand and localized nodular surface on the side of the palm

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Detailed physical examination revealed an extensively pigmented patch of size approximately >70 cm with elevated and nodular surface [Figure 1]b over the left upper extremity extending from shoulder till the proximal interphalangeal joint [Figure 2]a and [Figure 2]b. Tufts of coarse and lusterless grayish white hair were scattered all over the lesion on left upper extremity [Figure 3]a and [Figure 3]b. Another similar single pigmented satellite lesion of size approximately 3 × 5 cm [Figure 4] was present over the lower back of the patient just lateral to the midline on the right side around the waist area.
Figure 2: (a and b) Hyperpigmented papular patch with elevated and nodular surface over the left upper extremity extending from shoulder till the proximal interphalangeal joint

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Figure 3: (a and b) Tufts of coarse and lusterless grayish white hair scattered all over the lesion on left upper extremity

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Figure 4: Single pigmented satellite lesion present over the back just lateral to the midline on right side around the waist area

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Intraoral examination revealed deep distal caries in right maxillary first premolar, right mandibular first molar, and left maxillary first premolar. Right maxillary first premolar and right mandibular first molar were tender on percussion. Also, single well-defined bluish gray pigmented lesion of size approximately 1 × 2 cm was present on the left side of hard palate in relation to left maxillary first and second molars extending mediolaterally from the level of approximately 1.5 cm medial to the marginal palatal gingiva of left maxillary first and second molars to the level approximately 2 cm lateral to the midline and anteroposteriorly from the level of an imaginary line joining the mesial aspect of maxillary first molars of both sides to the level of an imaginary line joining the distal aspect of maxillary second molars of both sides [Figure 5]. The lesion was nontender and firm on palpation.
Figure 5: Well-defined bluish grey pigmented patch present on left side of palate in relation to left maxillary first and second molars

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On the basis of history and clinical examination, a provisional diagnosis of apical periodontitis with maxillary right first premolar and mandibular right first molar and GCMN on upper left extremity with intraoral nevus was given. The differential diagnosis considered for GCMN included acanthosis nigricans, baker’s nevus, malignant melanoma. Intraoral periapical radiograph (IOPA) of right maxillary first premolar and right mandibular first molar was advised, which revealed periodontal ligament space widening in both the teeth. Maxillary cross-sectional occlusal radiograph was advised which showed no changes.

The biopsy of the lesion was taken from the forearm in the department of oral and maxillofacial surgery and was sent for histopathological examination. The histopathological slide showed elongated and spindle-shaped nevoid cells arranged in the form of nests and evident fibrous tissue, hair follicles, and nerve tissue along with areas of melanin incontinence. All these findings were consistent with those of congenital melanocytic nevus. No evidence of a malignant transformation was seen [Figure 6]. Thus, the final diagnosis of apical periodontitis with maxillary right first premolar and mandibular right first molar and GCMN on upper left extremity was confirmed. Biopsy of palatal lesion was not done because the patient was asymptomatic and not ready for the same. However, the patient was instructed to report back if any symptom develops within the intraoral lesion for further investigations.
Figure 6: Histopathological slide showing elongated and spindle shaped nevoid cells

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Root canal treatment in maxillary right first premolar and mandibular right first molar was advised and the patient was referred to the department of endodontics for further treatment. Consult with a dermatologist was also advised who recommended no treatment till any symptom develops or there are any signs of malignant transformation. Patient came for follow-up after his root canal treatment was completed. He was advised to report to the dermatologist on development of any symptoms pertaining to the lesions.


   Discussion Top


CMN are pigmented cutaneous lesions which are said to form by a combination of dermally and epidermally derived nevi cells which occurs in about 1% of the newborns.[3],[5] Most of the studies reported in literature showed a slightly higher prevalence in female than males, with the ratio ranging from 1.17:1 to 1.46:1.[4] However, in the present case we have reported GCMN in a male patient. These nevi have been categorized into three categories based on their size as: small (<1.5 cm), medium (1.5–19.9 cm) and giant (>20 cm).[6] These are also known as bathing trunk nevi, coat sleeve nevi, or stocking nevi.[3] In the present case, the size measured was >70 cm covering whole of the left upper extremity resembling a coat sleeve type of GCMN. GCMN usually begin as flat, brown, or brownish black patches but may become elevated and develop a mottled appearance and nodular surface later in life, as was seen in the present case. They appear darker at birth than a few weeks later.[3] Similarly, in the present case, the lesion was darker in color initially and became lighter in color as the age of the patient advanced. They are found most commonly on the trunk, followed by the limbs and the head. In the present case, whole of the left upper extremity was involved.

Giant nevi on the scalp in a posterior axial location and those with satellite lesions are at a greater risk of malignant transformations.[3] However, in the present case in spite of the presence of satellite lesion, no malignant transformation was observed. Various abnormalities associated with GCMN have been reported in literature which includes limb hypoplasia, ear deformities, and angiomas.[1] In the present case, we have reported an asymptomatic intraoral nevus in association with GCMN which has not been reported yet. One case of CMN of the oral mucosa had been reported in literature in a patient with a small CMN of inner thigh.[7]

The estimated risk of developing melanoma varies from 5 to 10% in the entire lifetime of the patient. Therefore, individualization in the management of giant congenital melanocytic nevus is needed.[4] Because GCMN have an increased risk of malignant melanoma and other neural crest malignancies, complete early surgical excision is the recommended treatment.[1] Tissue expanders, tissue grafts, and tissue flaps are often necessary to close the large defects following excision. The role of laser treatment is controversial in congenital melanocytic nevi.[5] However, Laser can be considered as a treatment option for the lesions which cannot be excised surgically. In cases where surgical excision is not feasible, close clinical, and dermoscopic follow-up with biopsy of clinically suspicious areas is recommended.[8]


   Conclusion Top


GCMN is a very rare clinical entity which may be present in association with other extraoral and intraoral findings. Because it has significantly increased risk for malignant transformation, patient should be kept under surveillance for the development of malignant changes. Oral CMN is a rare lesion, and hence, knowledge regarding its clinical features is required for its appropriate diagnosis and management. The case presented here contributes to the knowledge regarding the clinical, histopathological as well as immunohistochemical features of CMN, and is the first one to be reported in which oral CMN occurred along with GCMN.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Schaffer JV, Bolognia JL. The clinical spectrum of pigmented lesions. Clin Plast Surg 2000;27:391-408.  Back to cited text no. 1
[PUBMED]    
2.
Mackie RM (1993). In: Champion RH, Burton JL and Ebling FJG, editors. Rooks Textbook of Dermatology. 5th ed. Oxford: Blackwell Scientific Publication; 1993.  Back to cited text no. 2
    
3.
Malik G, Singh A, Singh K, Pannu MS, Singh P, Jain R. Giant congenital melanocytic nevus: A case report. International Journal of Basic and Applied Medical Sciences 2015;5:138-40.  Back to cited text no. 3
    
4.
Viana ACL, Gontijo B, Bittencourt FV. Giant congenital melanocytic nevus. An Bras Dermatol 2013;88:863-78.  Back to cited text no. 4
    
5.
Taksande A, Vilhekar K. Congenital giant pigmented nevus. J Mahatma Gandhi Inst Med Sci 2014;19:65-6.  Back to cited text no. 5
  [Full text]  
6.
Rhodes AR. Melanocytic precursors of cutaneous melanoma. Estimated risks and guidelines for management. Med Clin N Am 1986;70:33-7.  Back to cited text no. 6
    
7.
Gilbert ML, Hanna W, Ghazarian D, Dover D, Klieb HBE. Congenital melanocytic nevus of the oral mucosa: Report of a rare pigmented lesion and review of the literature. Clin Pract 2011;1:e17.  Back to cited text no. 7
    
8.
Hadja I, Mezianea M, Harmouchb T, Mernissia FZ. Giant congenital melanocytic nevus with neurofibroma like lesions and onset of vitiligo. J Dermatol Dermatol Surg 2015;19:58-61.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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