P1.9 PRIMARY NON-ESSENTIAL CUTIS VERTICIS GYRATA WITH 3 DIMENSIONAL MAGNETIC RESONANCE IMAGING (3D MRI) finding

Sung Wook Park, Deborah Lee, Yoon Deok Choi, Sang Seok Lee, Si Hyung Cho

Department of Dermatology, Inje University School of Medicine, Busan, Korea

We describe a case of primary nonessential cutis verticis gyrata (CVG) with 3D MRI finding and review the classification.

A 24-year-old man presented with deep furrows and convolutions on his forehead and scalp. They had developed 10 years ago and gradually became larger and deeper. His past 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 consanguinity or similar scalp condition. Physical examination showed folds and furrows running anteroposteriorly on the scalp and transversely on the forehead. Laboratory exams such as complete blood-cell count, chemistry, VDRL test and urinalysis were all negative or normal. He had an amblyopia on his right eye which had gradually progressed and had conduction defect in right visual pathway anterior to the chiasm by visual evoked potential test. And he had borderline intelligence on Korean Wechsler Intelligence Scale. Pure tone audiogram and skull X-ray were normal. Brain MRI revealed ischemic changes: gliosis of both parietal lobes and periventricular leukomalacia of left parietal lobe. 3D MRI showed typical ridges and furrows of CVG on his forehead and scalp more clearly. A scalp biopsy showed an essentially nomal histology.

CVG is an unusual condition which typically occurs on the scalp and is characterized by ridges and furrows which cannot be flattened by traction or pressure. CVG may be classified into primary and secondary forms. Primary CVG may exist as a solitary finding or may be associated with mental retardation, epilepsy and other brain or ophthalmologic abnormalities. The former is called essential types and the latter nonessential. Secondary CVG is largely associated with other disorders of the scalp, such as tumors, neurofibromas, cerebriform intradermal nevi and inflammatory conditions. And systemic disorders associated with CVG include acromegaly, myxedema, amyloidosis or pachydermoperiostosis.

We recount a case of primary nonessential CVG associated with amblylopia, borderline intelligence and ischemic brain injury. The diagnosis of CVG can be made clinically; a few reports describe CT or MRI findings. We found that 3D MRI of the head demonstrated the characteristic furrows and ridges of CVG more obviously in our case, so we include 3D MRI findings in our report.