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CASE REPORT
Year : 2017  |  Volume : 18  |  Issue : 1  |  Page : 50-52

Cutis verticis gyrata secondary to giant cerebriform intradermal nevus in a pediatric patient


1 Department of Dermatology, Okmeydani Training and Research Hospital, Istanbul, Turkey
2 Department of Pathology, Istanbul Training and Research Hospital, Istanbul, Turkey
3 Department of Dermatology, Istanbul Training and Research Hospital, Istanbul, Turkey

Date of Web Publication12-Dec-2016

Correspondence Address:
Ilteris Oguz Topal
Department of Dermatology, Okmeydani Training and Research Hospital, Istanbul
Turkey
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2319-7250.172471

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  Abstract 


Cutis verticis gyrata (CVG) is a long-lasting and progressive condition in which there is hypertrophy and folding of the scalp skin. Cerebriform intradermal nevus (CIDN) is a rare cause of CVG. Here, we report a case with CIDN of the scalp in a 12-year-old girl. Dermatological examination showed a cerebriform appearance with accentuating folds and deep furrows, thickening in the temporal regions. We want to emphasize that CIDN should not be forgotten in the differential diagnosis of the causes of CVG.

Keywords: Cerebriform intradermal nevus, cutis verticis gyrata, scalp


How to cite this article:
Topal IO, Goncu OE, Leblebici C, Gungor S, Duman H, Erdemir AV. Cutis verticis gyrata secondary to giant cerebriform intradermal nevus in a pediatric patient. Indian J Paediatr Dermatol 2017;18:50-2

How to cite this URL:
Topal IO, Goncu OE, Leblebici C, Gungor S, Duman H, Erdemir AV. Cutis verticis gyrata secondary to giant cerebriform intradermal nevus in a pediatric patient. Indian J Paediatr Dermatol [serial online] 2017 [cited 2019 Dec 14];18:50-2. Available from: http://www.ijpd.in/text.asp?2017/18/1/50/172471




  Introduction Top


Cutis verticis gyrata (CVG) is a descriptive term for a condition of the scalp manifesting as convoluted folds and furrows formed from thickened skin of the scalp resembling cerebriform pattern.[1] It may occur by primary or secondary causes.[2] Herein, we describe a 12-year-old girl with CVG to giant cerebriform intradermal nevus (CIDN).


  Case Report Top


A 12-year-old girl presented with a 10-year history of an increasing thickness on her scalp. She had no history of significant disease such as seizures, or cerebral palsy, and she had no mental retardation. The lesion started as pigmented patch at birth, grew in size during subsequent years, and eventually attained the current size. There was no family history of the similar disorder. Dermatological examination of the scalp revealed a cerebriform appearance with accentuating folds and deep furrows. There were also millimetric papules and several nodules over the temporal scalp [Figure 1]. Neurologic, ophthalmologic examinations, magnetic resonance imaging of the brain, and routine blood tests were normal.
Figure 1: Folds and furrows with millimetric papules and nodules on the scalp

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Histopathological examination of a skin biopsy specimen showed clusters of nevus cells in the dermoepidermal junction and reticular dermis. The hair follicles and sebaceous glands surrounded by nevus cells without atypia [Figure 2].
Figure 2: Biopsy of the scalp shows clusters of nevus cells in the dermoepidermal junction, reticular dermis, and around the hair follicles and sebaceous glands (H and E, ×40)

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A diagnosis of giant CIDN was made on the clinical and histological findings. The patient refused to undergo any surgical procedure because of her concerns about the cosmetic outcomes of the surgery.


  Discussion Top


CVG is a rare entity with an estimated prevalence of 1 in 100,000.[1],[2] It is classified into two forms: Primary and secondary.[3] The primary form is often associated with neurologic, psychiatric and chromosomal disorders, and among others.[1] The secondary form may occur due to underlying disorders such as amyloidosis, syphilis, myxoedema, acromegaly, hamartomas, dermatofibromas, nevi, neurofibromatosis, and breast cancer.[2],[3]

Various cases of CVG due to CIDN have been reported in the English literature. Hammon and Ransom reported the first case with CVG secondary to intradermal nevus on the scalp in 1937.[4] Alcántara González et al. described another case who had CIDN on the hemicranial area with histology showing typical solitary and clusters of nevus cells in the dermis. They observed slight hyperpigmentation and alopecia on the left parietal and temporal areas of the scalp with folds accompanied by thickening of the skin.[3] CIDN is a rare disorder that is also called pseudo or secondary CVG. Clinically, CIDN is characterized by a well demarcated cerebriform skin surface, usually on the parietal area of the scalp.[4] Congenital melanocytic nevi are pigmented lesions caused by malformations of the neuroectoderm and occasionally neural elements, following deregulated growth, and arrest of melanocytes during migration from the neural crest to the skin.[5] Giant congenital nevi have been reported to cause CVG.[2] Some researchers consider giant congenital melanocytic nevi and CIDN to be related conditions and they believe that CIDN is a rare form of congenital melanocytic nevi because of histopathological similarities. However, other authors suggest that CIDN is a separate disorder which is usually not pigmented with a few nevus cell nests at the dermoepidermal junction, and of alopecia or sparse hair within the lesion is common.[2],[3] Giant congenital melanocytic nevi have intense pigmentation, an increased number of hair follicles and frequent nevus cell nests at the dermoepidermal junction.[5] We did not observe alopecia within the lesion on our patients scalp. The lesion was hairy and floppy. On the other hand, histopathological examination of the skin showed only a few nevus cell nests with no pigmentation and hair follicles which were compatible with a congenital intradermal nevus. We believe that CIDN is a type of congenital melanocytic nevus. These antities are similar and establishing the accurate diagnosis is very difficult.

The development of malignant melanoma in patients with congenital nevus has been reported.[5] We recommended annual follow-up of the presented case.


  Conclusion Top


CVG may occur by primary or secondary causes. Giant congenital nevi are rare secondary causes. Because of the possibility of progression to melanoma, follow-up of the patients with congenital nevus is essential.

Financial Support and Sponsorship

Nil.

Conflicts of Interest

There are no conflicts of interest.

 
  References Top

1.
Diven DG, Tanus T, Raimer SS. Cutis verticis gyrata. Int J Dermatol 1991;30:710-2.  Back to cited text no. 1
    
2.
Ghosh SK, Bandyopadhyay D, Ghoshal L, Sarkar S. Lobulated mass on the back of the scalp. Indian J Dermatol Venereol Leprol 2012;78:225-7.  Back to cited text no. 2
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3.
Alcántara González J, Truchuelo Díez MT, Carrillo Gijón R, Martín Diaz RM, Jaén Olasolo P. Letter: Cerebriform intradermal nevus presenting as secondary cutis verticis gyrata. Dermatol Online J 2010;16:14.  Back to cited text no. 3
    
4.
van Geest AJ, Berretty PJ, Klinkhamer PJ, Neumann HA. Cerebriform intradermal naevus (a rare form of secondary cutis verticis gyrata). J Eur Acad Dermatol Venereol 2002;16:529-31.  Back to cited text no. 4
    
5.
Lyon VB. Congenital melanocytic nevi. Pediatr Clin North Am 2010;57:1155-76.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2]



 

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