Indian Journal of Pathology and Microbiology
Home About us Instructions Submission Subscribe Advertise Contact e-Alerts Ahead Of Print Login 
Users Online: 1276
Print this page  Email this page Bookmark this page Small font sizeDefault font sizeIncrease font size

  Table of Contents    
Year : 2011  |  Volume : 54  |  Issue : 3  |  Page : 624-625
A case of cutis verticis gyrata secondary to giant cerebriform intradermal nevus

Department of Pathology, MNR Medical College, Sangareddy, Andhra Pradesh, India

Click here for correspondence address and email

Date of Web Publication20-Sep-2011

How to cite this article:
Tagore KR, Ramineni AS. A case of cutis verticis gyrata secondary to giant cerebriform intradermal nevus. Indian J Pathol Microbiol 2011;54:624-5

How to cite this URL:
Tagore KR, Ramineni AS. A case of cutis verticis gyrata secondary to giant cerebriform intradermal nevus. Indian J Pathol Microbiol [serial online] 2011 [cited 2020 Nov 30];54:624-5. Available from: https://www.ijpmonline.org/text.asp?2011/54/3/624/85121

Giant cerebriform intradermal nevus is one of the underlying causes for the development of cutis verticis gyrata (CVG). Clinically, it manifests as a scalp deformity resembling the surface of the brain. Early diagnosis and treatment is more important as the risk of development of malignant melanoma is high.

A 14-year-old male presented with history of progressively increasing swelling over occipital region since birth. On examination, there was a single bosselated, non tender irregular swelling of 20 Χ 15 cm with deep folds over the occipital region and left parietal region with absence of hair over the swelling. Skin over the swelling was not pinchable. Routine investigations were within normal limits. No bony involvement. The lesion was excised with 0.5 cm skin margin and primary full thickness skin grafting was done. Postoperative period was uneventful, grafts taken up well and wound healed.

Grossly the mass was dome shaped with hypertrophied and irregular foldings of the skin showing gyriform appearance [Figure 1]. Cut section of the gyriform elevations show brownish black areas and non gyriform portions at the margin appear normal [Figure 2]. Microscopic examination from the pigmented gyriform elevations shows an intradermal nevus with neuroid elements [Figure 3]. The neuroid areas show considerable similarity to schwannoma in the form of nuclear palisading and verocay body formation with superimposed melanin pigment [Figure 4]. Margins were normal and free from neural nevus.
Figure 1: A 20 cm tumor with folded skin giving gyriform appearance

Click here to view
Figure 2: Under surface of the same tumor showing pigmented areas

Click here to view
Figure 3: Epidermis showing increased pigmentation in the basal layer. Deep dermis shows islands of nevus cells with cytoplasmic pigmentation. No atypical features (original magnification x40)

Click here to view
Figure 4: Another area showing neural differentiation with verocay bodies (H and E stain,x200)

Click here to view

Cutis verticis gyrata is a morphological syndrome in which there is hypertrophy and folding of the skin of the scalp to present a gyrate of cerebriform appearance. CVG classified into primary and secondary forms. The primary form associated with neurologic, ophthalmologic disorders and psychiatric manifestations. [1],[2] Secondary CVG has been described with a wide range of underlying causes. An underlying nevus is the most often found, [3] but other nevoid abnormalities such as naevus lipomatosis, connective tissue nevi and acquired lesions such as neurofibroma, may also cause CVG. Prolonged traction due to tying of hair in a knot in Sikhs has led to thickening and laxity of scalp skin and cutis verticis gyrata. Histologically cerebriform congenital nevus usually present as intradermal nevus with neuroid changes simulating those observed in neurofibroma. [4] These lesions have high risk for the development of malignant melanoma. The life time incidence of melanoma arising either in a giant nevus or, in one of the many smaller satellite nevi is 6.3% and 12%. [1] The melanoma may be present at birth, or it may arise in infancy or at any time later in life. The mortality of such lesions is high. The treatment of choice is surgical extirpation and scalp flap reconstruction.

   References Top

1.Jeanfils S, Tennstedt D, Lachapelle JM. Cerebriform intradermal nevus: A clinical pattern resembling cutis verticis gyrata. Dermatology 1993;186:294-7.  Back to cited text no. 1
2.Hamm JC, Argenta LC. Giant cerebriform intradermal nevus. Ann Plast Surg 1987;19:84-8.  Back to cited text no. 2
3.Lasser AE. Cerebriform intradermal nevus. Pediatr Dermatol 1983;1:42-4.  Back to cited text no. 3
4.Yazici AC, Ikizoglu G, Baz K, Polat A, Ustunsoy D. Cerebriform intradermal nevus. Pediatr Dermatol 2007;24:141-3.  Back to cited text no. 4

Correspondence Address:
Koyye Ravindranath Tagore
Department of Pathology, MNR Medical College, Narasapur Road, Sangareddy, Andhra Pradesh
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.85121

Rights and Permissions


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


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Email Alert *
    Add to My List *
* Registration required (free)  

    Article Figures

 Article Access Statistics
    PDF Downloaded77    
    Comments [Add]    

Recommend this journal