Cutis Verticis Gyrata Across the Diagnostic Spectrum: Two Cases Highlighting Challenges in Clinical Classification

Cutis verticis gyrata (CVG) is an uncommon disorder characterized by cerebriform thickening of the scalp that may occur as a primary condition or secondary to a variety of systemic disorders. We report two patients with clinically distinct presentations of CVG illustrating the diagnostic challenges encountered during classification. A 40-year-old man presented with progressive scalp thickening and visual impairment, raising suspicion for primary non-essential or secondary CVG; however, definitive classification was not possible because further ophthalmological and systemic investigations could not be completed after he was lost to follow-up. A 28-year-old man presented with asymptomatic scalp folds accompanied by seborrhea and comedonal acne, initially suggesting pachydermoperiostosis. However, normal laboratory and endocrine investigations, including normal serum growth hormone levels, together with the absence of digital clubbing, periostosis, and radiographic abnormalities, favored primary essential CVG. These cases emphasize that CVG should be regarded as a clinical sign requiring systematic neurological, ophthalmological, endocrine, skeletal, and dermatological evaluation before definitive classification.