Cutis vertices gyrata (CVG) is characterized by thickening of the scalp, which becomes raised to form ridges and furrows resembling the cerebral gyri, which cannot be flattened by traction or pressure. The diagnosis is based on clinical features, and further investigations are needed to demonstrate the precise typical features. We describe how three-dimensional magnetic resonance imaging (3D MRI) can be used to visualize the extent of the lesions in primary non-essential CVG.
A 24-year-old man presented with deep furrows and convolutions on his forehead and scalp. Transverse furrows and convolutions on his forehead had developed 10 years earlier and vertical folds developed on the scalp 2 years later. The folds became deeper and increased in size to form ridges and furrows.
His medical history was not significant. He had no history of any disorders or inflammatory conditions of his skin or scalp besides the keloids on his anterior chest and both shoulders. He denied a family history of similar scalp condition.
Physical examination showed folds and furrows running transversely on the forehead (Fig. 1A) and anteroposteriorly on the scalp (Fig. 1B), which could not be corrected by pressure or traction. Laboratory examinations, such as complete blood-cell count, chemistry, venereal disease research laboratory (VDRL) test, urinalysis and chromosomal analysis, were all negative or normal. He had an amblyopia on his right eye which had gradually progressed and had conduction defect in the right visual pathway anterior to the chiasm by visual-evoked potential test. He had borderline intelligence on the Korean Wechsler Intelligence Scale. Pure-tone audiogram and skull X-ray were normal. Brain MRI revealed ischaemic changes: gliosis of both parietal lobes and periventricular leukomalasia of the left parietal lobe. A scalp skin biopsy showed an essentially normal histology. 3D MRI showed typical ridges and furrows of CVG on his forehead and scalp more clearly (Fig. 2).
Fig.1. (A) Transverse folds on the forehead, and (B) vertical folds and furrows on the scalp.
Fig. 2. Three-dimensional magnetic resonance imaging (3D MRI) showing typical scalp folds.
CVG may be classified into primary and secondary forms (1). In primary CVG, ridges and furrows usually occur after puberty and show symmetrical distribution. A skin biopsy specimen reveals a normal appearance or thickened connective tissue, hyperplasia of appendages. The primary type can be further divided into essential and non-essential forms. The former may exist as a solitary finding, with no association with neurological and ophthalmological disease and the latter may be associated with mental retardation, epilepsy and other brain or ophthalmologic abnormalities (1–4). Secondary CVG may appear at any age, skin folds are usually asymmetric and histology reveals various alterations depending on underlying causes such as tumours, neurofibromas, cerebriform intradermal nevi and inflammatory conditions. Systemic disorders associated with secondary CVG include acromegaly, myxedema, amyloidosis or pachydermoperiostosis (1–4).
The diagnosis of CVG can be made clinically, but the length and styling of hair makes it difficult to detect the characteristic features of CVG. A few reports of CVG used computed tomography or MRI as a diagnostic method, however, it is difficult to reveal the entire pattern of the scalp lesions by these methods (5–7). We found 3D MRI of the head could show the characteristic furrows and ridges of CVG more obviously.
1. Dayna GD, Tonny T, Sharon SR. Cutis verticis gyrata. Int J Dermatol 1991; 30: 710–712.
2. Leticia KS, Tantiane G, Lauro AC, Nelson R, Alberto S, Ricard H. Essential primary cutis verticis gyrate. J Pediatr 2002; 78: 75–80.
3. Filosto M, Tonin P, Vattemi G, Bongiovanni LG, Rizzuto N, Tomelleri G. Cutis verticis gyrata, mental retardation and Lennox-Gastaut syndrome: a case report. Neurol Sci 2001; 22: 253–256.
4. Chang GY. Cutis verticis gyrata, underrecognized neurocutaneous syndrome. Neurology 1996; 47: 573–575.
5. Okamoto K, Ito J, Tokiguchi S, Ishikawa K, Furusawa T, Sakai K. MRI in essential primary cutis verticis gyrate. Neuroradiology 2001; 43: 841–844.
6. Kolawole TM, Orainy IA, Patel PJ, Fathuddin S. Cutis vertices gyrata: its computed tomographic demonstration in acromegaly. Eur J Radiol 1998; 27: 145–148.
7. DePadova-Elder SM, Ditire CM, Kantor GR. Cutis verticis gyrata and pachydermoperiostosis: demonstration with computed tomograthy. Arch Dermatol 1992; 128: 276–277.
Doo Jin Oh, Jeong Hoon Park, Seung Hoon Kang, Seon Wook Hwang and Sung Wook Park
Department of Dermatology Inje University Busan-Paik Hospital, Gaegum-dong 633-165, Busanjin-gu, Busan, 614-735 South Korea. E-mail: firstname.lastname@example.org
Accepted April 20, 2006.