Primary essential cutis verticis gyrata: A case report

Cutis verticis gyrata (CVG) is a rare disease characterized by the excessive growth of the skin of the scalp, resulting in furrows and folds that resemble the gyri of the brain cortex [1,2]. Depending on its form, CVG can be identified as primary—essential or nonessential—or secondary [2]. Primary nonessential CVG is associated with neurological and ophthalmological manifestations while primary essential CVG is not. The secondary form of CVG is much more frequent and accompanies other pathologies [3]. There have been around 28 cases of primary essential CVG described in the literature. We report a case of primary essential CVG in a young female.


INTRODUCTION
Cutis verticis gyrata (CVG) is a rare disease characterized by the excessive growth of the skin of the scalp, resulting in furrows and folds that resemble the gyri of the brain cortex [1,2]. Depending on its form, CVG can be identified as primary-essential or nonessential-or secondary [2]. Primary nonessential CVG is associated with neurological and ophthalmological manifestations while primary essential CVG is not. The secondary form of CVG is much more frequent and accompanies other pathologies [3]. There have been around 28 cases of primary essential CVG described in the literature. We report a case of primary essential CVG in a young female.

CASE REPORT
A 20-year-old female presented herself with a history of thickened and prominent skin folds over the vertex and parietal region of the scalp persistent for one year prior (Fig. 1). No other symptoms were reported. A physical examination revealed enlargement of the skin folds of the scalp arranged in a vertical position.
There was no history of comorbidity, no family history of similar lesions, and no history of consanguinity. The patient reported no neurological or ophthalmological symptoms. Neurological, psychiatric, ophthalmological, and endocrine consultations were obtained and, to exclude secondary causes, a complete blood count, a thyroid function test, and syphilis screening were done and the levels of growth hormone, follicle-stimulating hormone, luteinizing hormone, and serum cortisol were checked, all of which reported normal values. MRI of the brain revealed a thickened scalp with marked ridges and furrows of a gyriform appearance in the parietooccipital region, suggestive of cutis verticis gyrata with no underlying defects. As the clinical picture was pathognomonic of CVG, a skin biopsy and histopathology were deemed unnecessary.
Primary CVG refers to cases with no underlying cause [4], beginning after puberty, usually before the age of thirty, and occurring predominantly in males, with a male-to-female ratio of 5:1. Primary CVG is divided further into two types: essential and nonessential (Fig. 2) [4,5].
The essential form, exhibited by our patient, is a rare disorder characterized by the thickening of the scalp, where the excess skin folds are symmetrical and take the shape of the cerebral gyri. There is no underlying neurological or ophthalmological association [1].
The nonessential form is associated with neurological (epilepsy, intellectual disability, microcephaly), psychiatric (schizophrenia), and ophthalmic (cataract, strabismus, blindness, retinitis pigmentosa) disorders [1,4]. Secondary CVG is more common than primary CVG, can occur at any age, and displays no gender predilection. Secondary CVG has a number of underlying causes [5]. Several sources explain that the secondary form may develop after inflammatory dermatosis, endocrine and genetic disorders, and internal malignancies as paraneoplastic syndrome (Table 1) [1,6].
CVG usually affects the vertex and the occipital scalp, and may sometimes involve the entire scalp. Generally, the folds are arranged in an anteroposterior direction but may be transverse on the occiput [5]. CVG is usually asymptomatic but the patient may sometimes complain of pruritus, a burning sensation, malodor, and thinning of the hair in the folded areas due to aggregation of debris and skin secretions in the folds [7].
The diagnosis of CVG is based on clinical findings but investigations are necessary to distinguish between primary and secondary CVG [5].
The treatment of primary essential CVG depends on the patient's aesthetic expectations, since no organic function   needs to be corrected. Local hygiene is important to prevent the accumulation of secretions with an unpleasant odor [8]. Surgical treatment may be considered to remove excess skin folds with the aim to achieve a more aesthetically satisfying appearance of the scalp. In our case, CVG was easily concealed by the patient's long hair. She was counseled regarding the benign nature of her condition and informed about the possibility of secondary infection due to the trapping of debris in the depressions. Explained to her was also the importance of local hygiene and attending regular follow-ups.

CONCLUSION
Primary essential CVG is an extremely rare condition, with around 253 cases of CVG and 28 cases of primary essential CVG reported in the literature. Because it is a rare condition, our case appears as a valuable educational opportunity. A dermatologist finding cases such as ours should take a proper medical history and perform careful examinations and complementary investigations to reach a definitive diagnosis.

Consent
The examination of the patient was conducted according to the principles of the Declaration of Helsinki.