Abstract:

The rare clinical entity of cutis verticus gyrata (CVG) describes a lesion or process that causes excessive growth of the scalp and folding in the skin, which results in a striking and characteristic cerebriform pattern of growth, resembling brain cortex on the external surface of the skull. It may be primary or caused by an underlying systemic disease including pachydermoperiostosis, amyloidosis, psoriasis, or others, or by an underlying neoplasm. Here we present a 12-year-old otherwise healthy female who presented with a large cerebriform mass of the temporal region of the scalp, measuring approximately 10 x 10 cm. The lesion had been present since at least 5 years of age, but had increased in size over time and was felt to be disfiguring, protruding through her dense hair. Thus, the patient underwent excision of the lesion after tissue expander placement. Histologic examination revealed intradermal nevus cells with variable amounts of pigment appearing in nests, fascicles, and as single cells. The cells extended deep though the dermis and into the subcutis with dense neurotization and associated fibrosis. Features of malignancy were not seen. Given the clinical presentation and histologic appearance, a diagnosis of CIDN was rendered. CIDN is an uncommon cause of a rare clinical entity, CVG. However, it can have a devastating impact on the patient, as it is often disfiguring, and has a low but not insignificant risk of malignant transformation, estimated to be approximately 4% in some studies, occurring in patients as young as 6 years of age. Thus, surgical excision is generally recommended, although close clinical follow up is also an option.

Financial Disclosure:
No current or relevant financial relationships exist.

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Erin Alston, MD