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  In this article
    Abstract
    Introduction
    Case Report
    Discussion
    References

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CASE REPORT
Year : 2002  |  Volume : 68  |  Issue : 6  |  Page : 367-368

Congenital cerebriform melanocytic naevus with cutis verticis gyrata


Department of Skin and VD, Kasthurba Medical College, Mangalore, India

Correspondence Address:
Department of Skin and VD, Kasthurba Medical College, Mangalore, India

   Abstract 

Congenital melanocytic naevus is hamortomatous or non-neoplastic proliferation of abnormal mixture of tissue's or non-neoplastic proliferation of abnormal mixture of tissue's normal components. Cutis verticis gyrata is hypertrophy with parallel or gyrate folds of skin of scalp. We report a young man who presented to us with hair loss on the back of the head and increased growth of hair over the upper back since birth. A biopsy proved the diagnosis of congenital melanocytic naevus.

How to cite this article:
Pai VG, Rao GS. Congenital cerebriform melanocytic naevus with cutis verticis gyrata. Indian J Dermatol Venereol Leprol 2002;68:367-8


How to cite this URL:
Pai VG, Rao GS. Congenital cerebriform melanocytic naevus with cutis verticis gyrata. Indian J Dermatol Venereol Leprol [serial online] 2002 [cited 2019 Nov 18];68:367-8. Available from: http://www.ijdvl.com/text.asp?2002/68/6/367/11198



   Introduction Top

Naevus refers to a variety of hamartomatous or non-neoplastic proliferation of abnormal mixtures of tissue's normal components.[1] Congenital melanocytic naevus of >20 cm is a rare occurrence.[2] It is one of the causes of cutis verticis gyrata which presents as hypertrophy with parallel folds of skin. The common sites of occurrence are the parietal, occipital and the temporal regions of the scalp.[3]

   Case Report Top

A 25- year-old man presented to us with decreased hair over back of scalp and increased growth of hair on upper back since birth. He gave a history of oozing and crusting at the age of two years for which he took treatment and the lesions healed. Various ayurvedic preparations and topical applications were used with no relief. There was no history of epilepsy or mental retardation.
Examination revealed 32 x 10 cm naevus from back of scalp to upper back, scarring alopecia in upper part, parallel folds of skin in middle part and hypertrophy and hypertrichosis in the lower part. [Figure - 1]. A 2. 5 cm hairy pigmented solitary plaque is seen over the right arm.
Bio­psy from the rugose area showed dermis with groups and nests of round to oval naevus cells, some with melanin pigment in their cytoplasm, few cells in the lower portion were spindle shaped [Figure:2].

   Discussion Top

Congenital melanocyfic naevus is seen in 1-2 % of newborns.[4] The naevus cells are derived from epidermal melanocytes. [5] According to the size there are three variants. 1. <1. 5 cm-small, 2. 1. 5-20 cm - intermediate, 3. > 20 cm - giant variety' A rare form of giant variety is cerebriform melanocytic naevus. A size of > 10 cms is seen in 1 out of 20,000 newborns.[6] The naevus may present as a convoluted mass over the scalp which may not be pigmented. They become larger prone f o r malignancy. Most common malignancy seen is malignant melanoma, in 1. 8-42% cases.[7] Cutis verticis gyrata is hypertrophy of scalp skin with parallel or gyrate folds. Various causes of cutis verticis gyrata are:- Hereditary, traumatic, endocrinal, inflammatory, tumours and in association with other conditions. The commonest cause of cutis verticis gyrata amongst the tumours is cerebriform melanocytic naevus.[1] The main modalities of treatment are tissue expanders, dermabrasjon excision and skin grafting.[8] We present this case for its extensive nature and the rare occurrence of the two conditions in the same patient.  

   References Top

1.Amladi ST, Jerajani HR. Nevi and development defects. In: IADVL Textbook and Atlas of Dermatology Vol . 1 Edited by R G Valia 2001 ; 143-150.   Back to cited text no. 1    
2.Kamlesh Harish. Giant congenitial melanocytic nevus. Indian Pediatrics 2001;38:560-560.   Back to cited text no. 2    
3.Mahakrishnon P Pandian I. Cerebriform intradermal nevus. Indian J Dermatol Venerol Leprol 1981;47:279-280.   Back to cited text no. 3    
4.David Elder, Rosalie Elenitsas. Benign pigmented lesions and malignant melanoma. In: Lever's Histopathology of Skin, Eighth Edition. Edited by David Elder et al; 644-645.   Back to cited text no. 4    
5.Metcalf JS, Maize JC. Melanocytic nevi and malignant melanoma. Dermatologic Clinics 1985;3:218.   Back to cited text no. 5    
6.Rhodes AR. Benign neoplasia and hyperplasis of melanocytes. In: Fitzpatrick's Dermatology in General Medicine 5"' Edition. Edited by Irwin M Freidberg, Arthur Z Eisen, Klaus Wolff; 1028.   Back to cited text no. 6    
7.Zitelli JA, Grant MG. Histologic pattern of congenital nevocytic nevi and implication for treatment. J Am Acad Dermatol 1984;11:402  Back to cited text no. 7    
8.Mackie RU. Melanocytic naevi and malignant melanoma. In: Rook/ Wilkinsons/Eblings Textbook of Dermatology 6th Edition Vol 2. Edited by RH Champion, JL Burton; 1734-1735.   Back to cited text no. 8    

 

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