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Cutis Verticis Gyrata and Neurofibromatosis: A Rare Dermatologic Association – Case Report and Literature Review

Year: 2025
Type: article
Study design: Case Report
Abstract: Background: Cutis verticis gyrata (CVG) is a rare dermatologic condition characterized by thickened, folded scalp skin resembling cerebral gyri. Its association with neurofibromatosis type 1 (NF1) is exceedingly rare, with fewer than 20 cases reported worldwide. Case Presentation: A 23-year-old woman presented with progressive CVG and NF1-related neurofibromas involving the scalp, retroauricular region, and lumbosacral area. Surgical excision of redundant scalp tissue and neurofibromas was performed, with histopathology confirming dermal fibrosis and sebaceous hyperplasia. Discussion: We explore the pathophysiological overlap between CVG and NF1, emphasizing the role of RAS/MAPK pathway dysregulation in both conditions. Surgical strategies for CVG in NF1 patients must address vascular preservation and recurrence risk. Conclusion: CVG-NF1 association warrants multidisciplinary management. Scalp reduction surgery provides functional and aesthetic improvement, but long-term surveillance for malignant transformation is critical.
Language: English
Subfield: Neurology
Field: Medicine
Open Access status: diamond
OpenAlex ID: W4410378157
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