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CASE REPORT
Year : 2000  |  Volume : 66  |  Issue : 6  |  Page : 316-317

Giant Congenital Nevomelanocytic Nevus with Satellite Lesions, Vitiligo and Lipoma : a Rare Association




Correspondence Address:
Ram Gulati


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Source of Support: None, Conflict of Interest: None


PMID: 20877115

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  Abstract 

A case of giant congential nevomelanocytic nevus with satellite lesions, vitiligo and lipoma is described, owing to the rarity of the association. The constellation of finding can be explained on the basis of a defect in the neural crest, which is considered to be a common origin of melanoblasts, Schwann cells, sensory ganglia, bone, fat, muscle and blood vessels.


Keywords: Congenital nevomelanocytic nevus, Vitiligo, Lipoma


How to cite this article:
Gulati R, Jain D, Mehrania K, Kulde. Giant Congenital Nevomelanocytic Nevus with Satellite Lesions, Vitiligo and Lipoma : a Rare Association. Indian J Dermatol Venereol Leprol 2000;66:316-7

How to cite this URL:
Gulati R, Jain D, Mehrania K, Kulde. Giant Congenital Nevomelanocytic Nevus with Satellite Lesions, Vitiligo and Lipoma : a Rare Association. Indian J Dermatol Venereol Leprol [serial online] 2000 [cited 2020 Jun 4];66:316-7. Available from: http://www.ijdvl.com/text.asp?2000/66/6/316/4960



  Introduction Top


Congenital nevomelanocytic nevi (CNN) are viewed as benign neoplasms resulting from abnormalities of neural crest or its derivatives.[1] Giant CNN is a rare variety and is infrequently associated with other findings which make the clinical picture complex.[2],[3] We report a case of giant CNN associated with multiple small satellite CNN, vitiligo and lipoma.


  Case Report Top


A-17- year-old girl presented with a large asymptomatic, well-defined, dark brown patch extending over the entire upper back, chest and abdomen, since birth. Its smooth surface was covered with sparse hair and multiple irregular black speckles were dispersed over it. Other congenital features included multiple light to dark brown macular lesions measuring 0.5 to 2.5 cm in diameter distributed over rest of the body. Their surface characteristics resembled the giant lesion except for the evidence of coarse, long and dark hairs over most of them [Figure - 1], [Figure - 2]. Also, multiple discrete, white macular lesions measuring 0.5 to 1.5 cm in diameter were distributed symmetrically over the distal part of both upper and lower extremities, including palms and soles [Figure - 2].

Additionally, a well- defined, skin colored, I o b u I ate d swelling, 20cm 18 cm in diameter, was situated over the left buttock [Figure - 1]. It was soft, nonpulsatile, non tender and freely mobile on palpation. There was no impulse on coughing and transillumination test was negative. Two similar smaller swellings were present over the lower back. Tissue histopathology from those distinct lesions revealed a composite picture of giant CNN with multiple satellite lesions, vitiligo and lipoma.


  Discussion Top


Giant CNN has been reported by many authors. The above case appeared atypical because of the co-existence of congenital giant CNN, multiple satellite lesions, vitiligo and lipoma. This constellation of findings may be probably due to a defect in neural crest, which is the origin not only of melanoblasts but also of Schwann cells, sensory ganglia, bone, fat, muscle and blood vessles.[4],[5] The defect might lead to abnormal development (excess or deficiency) of any of its derivatives. Even though this view is highly dogmatized, the primary etiology is still a matter of debate.

 
  References Top

1.Reed RJ. The neural crest, its migrants and cutaneous malignant neoplasms related to neurocristic derivatives. In: Lynch HT, Fusaro RM, editors. Cancer Associated Genodermatoses. New York: Van Nostrand Reinhold, 1982:171.  Back to cited text no. 1    
2.Kopf AW, Bart RS.A congenital pigmented nevus associated with leucoderma. J Dermatol Surg Oncol 1981;7:547.  Back to cited text no. 2    
3.Hendrickson MR, Ross JC. Neoplasms arising in congenital giant nevi. Am J Surg Pathol 1981; 5:109.  Back to cited text no. 3  [PUBMED]  
4.Le Dourarin NM. The neural crest in the neck and other parts of the body. Birth Defects 1975;11:19.  Back to cited text no. 4    
5.Le Leviere CS, Le Dourarin NM. Mesenchymal derivatives of the neural crest: Analysis of chimeric quail and chick embryos, J Embryol Exp Morphol 1975;34:124.  Back to cited text no. 5    


Figures

[Figure - 1], [Figure - 2]



 

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