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CASE REPORT
Year : 2018  |  Volume : 11  |  Issue : 3  |  Page : 245-247  

One-sided brain over the head: Cutis verticis gyrata


Department of Skin and VD, MGIMS, Wardha, Maharashtra, India

Date of Web Publication29-Jun-2018

Correspondence Address:
Priyanka Date
Department of Skin and VD, MGIMS, Sewagram, Wardha, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/MJDRDYPU.MJDRDYPU_149_17

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  Abstract 


Cutis verticis gyrata (CVG) is a rare disease manifesting as convoluted folds and furrows, resembling sulci and gyri formed from thickened skin of the scalp. It can be congenital or acquired and primary or secondary. We report a rare case of 28-year-old male with a localized variant of primary essential CVG.

Keywords: Cutis verticis gyrata, pachydermoperiostosis, strabismus


How to cite this article:
Date P, Jain S. One-sided brain over the head: Cutis verticis gyrata. Med J DY Patil Vidyapeeth 2018;11:245-7

How to cite this URL:
Date P, Jain S. One-sided brain over the head: Cutis verticis gyrata. Med J DY Patil Vidyapeeth [serial online] 2018 [cited 2019 Jul 17];11:245-7. Available from: http://www.mjdrdypv.org/text.asp?2018/11/3/245/235553




  Introduction Top


Alibert was the first to mention about cutis verticis gyrata (CVG) which was described by Robert in 1843.[1] In 1907, Unna introduced the term CVG.[1] Polan and Butterworth classified it in 1953, into primary and secondary forms.[2] In 1984, Garden and Robinson [3] improved the classification by proposing new terms: primary essential and primary nonessential CVG. The former has no other abnormality, whereas the latter can be associated with ophthalmologic (cataract, strabismus, blindness, and retinitis pigmentosa), central nervous system (mental deficiency, cerebral palsy, epilepsy, and schizophrenia), cranial (microcephaly) abnormalities, and deafness. Secondary CVG can be associated with other diseases such as pachydermoperiostosis,[4] neurofibroma,[5] and acromegaly.[6]

Primary CVG is rare and is diagnosed by exclusion of secondary causes of the disease.[7]

Herein, we report a rare case of localized variant of primary nonessential CVG in a young male.


  Case Report Top


A 28-year-old male patient visited the dermatology outpatient department, complaining of itching and sticky discharge from the scalp and thickened and folded skin over the left side of scalp. On interrogation, the patient gave a history that it was since birth which had increased in size since last 2 years. There was no history of high-risk behavior, trauma, pain, hair loss over the affected area seizures, deafness, or blindness. There was no family history of similar complaints, diabetes, thyroid dysfunction, and hyperlipidemia.

On local examination, multiple convoluted anteroposterior folds were present over the left parieto-occipital area [Figure 1] which were soft and spongy and could not be corrected by pressure or traction. Rest of the scalp was normal [Figure 2]. Growth of hair over the affected area was normal. Greasy, foul-smelling scales were present between the folds. No acanthosis nigricans/skin tags were present. Distal extremities were normal [Figure 3]. Palms and soles and rest of the examination were normal.
Figure 1: Anteroposterior disposed hypertrophy of parieto-occipital region of the scalp

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Figure 2: Normal scalp over right side

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Figure 3: Normal upper and lower extremities

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ENT and ophthalmological examination were within normal limits. Histopathological examination of the excised biopsied tissue was consistent with CVG. Secondary causes of the disease were ruled out on the basis of detailed history and physical examination, computed tomography scan of the brain was normal. The patient was diagnosed to have primary essential CVG. He was treated symptomatically for greasy, foul-smelling scales and pruritus with selenium sulfide lotion and antihistamines. The patient refused surgical excision of the lesion as his problem of foul smell was solved, and the condition was harmless.


  Discussion Top


CVG was originally described by Jean-Louis-Marc Alibert in 1837, and it is also known as paquidermia verticis gyrata, cutis verticis plicata, and “bulldog” scalp syndrome.[2] Primary CVG is five times more frequent in men compared to women [7],[8] and is often associated with neuropsychiatric diseases, such as seizures and schizophrenia or mental retardation.[7] Age of onset is often after puberty, and the folds are usually symmetrical.[7] In our case, we found onset is since birth, and the lesion was localized. In primary essential CVG, there is no association with other comorbidities. Our case similar showed only localized increase in skin folds. No histopathologic abnormality was detected in our case, whereas the secondary CVG has been linked to local inflammatory skin conditions, such as eczema and psoriasis and to systemic illnesses, such as acanthosis nigricans, syphilis, myxedema, amyloidosis, Ehlers–Danlos syndrome, and insulin-resistance syndrome.[9] Skin folds in secondary CVG can appear at any age and may be more asymmetrical. Acromegaly and pachydermoperiostosis (idiopathic hypertrophic osteoarthropathy) often present with secondary CVG [9] were ruled out in our case. No exposure to drugs such as anabolic steroids [10] which may lead to the secondary form of CVG was present in our case.

The treatment of primary essential CVG may be palliative or surgical. Education regarding local hygiene is necessary to avoid foul smell due to the accumulation of sebaceous secretions. Surgical treatment can improve the clinical aspect, but the quality of life might be compromised due to unesthetical aspect.[11]


  Conclusion Top


Primary essential CVG, a rare condition, can only be diagnosed after excluding neurological, ophthalmological alterations and other diseases possibly associated with it. After careful examination, necessary investigations, and interspecialty consultation, we are reporting an extremely rare case of localized variant of primary essential CVG after excluding all the conditions associated with it. Many times physical appearance might be the only initial presenting complaint.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Unna PG. Cutis verticis gyrata. Monatschr Prakt Derm 1907;45:227-33.  Back to cited text no. 1
    
2.
Polan S, Butterworth T. Cutis verticis gyrata; a review with report of seven new cases. Am J Ment Defic 1953;57:613-31.  Back to cited text no. 2
    
3.
Garden JM, Robinson JK. Essential primary cutis verticis gyrata. Treatment with the scalp reduction procedure. Arch Dermatol 1984;120:1480-3.  Back to cited text no. 3
    
4.
Thappa DM, Sethuraman G, Kumar GR, Elangovan S. Primary pachydermoperiostosis: A case report. J Dermatol 2000;27:106-9.  Back to cited text no. 4
    
5.
Commens CA, Greaves MW. Cutis verticis gyrata due to an intradermal naevus with an underlying neurofibroma. Clin Exp Dermatol 1978;3:319-22.  Back to cited text no. 5
    
6.
Kolawole TM, Al Orainy IA, Patel PJ, Fathuddin S. Cutis verticis gyrata: Its computed tomographic demonstration in acromegaly. Eur J Radiol 1998;27:145-8.  Back to cited text no. 6
    
7.
Diven DG, Tanus T, Raimer SS. Cutis verticis gyrata. Int J Dermatol 1991;30:710-2.  Back to cited text no. 7
    
8.
Lasser AE. Cerebriform intradermal nevus. Pediatr Dermatol 1983;1:42-4.  Back to cited text no. 8
    
9.
Snyder MC, Johnson PJ, Hollins RR. Congenital primary cutis verticis gyrata. Plast Reconstr Surg 2002;110:818-21.  Back to cited text no. 9
    
10.
Ross JB, Tompkins MG. Cutis verticis gyrata as a marker of internal malignancy. Arch Dermatol 1989;125:434-5.  Back to cited text no. 10
    
11.
Dumas P, Medard de Chardon V, Balaguer T, Cardot-Leccia N, Lacour JP, Lebreton E. Primary essential cutis verticis gyrata: Case report and literature review. Ann Chir Plast Esthet 2010;55:243-8.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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