Cutis verticis gyrata - Hair and Scalp
Primary essential CVG presents only as scalp folds and has no associated findings. It has a male predominance with onset during or after puberty. Ninety percent of patients diagnosed are older than 30.
In primary nonessential CVG, patients have neurologic, psychiatric, or ophthalmologic comorbidities (eg, intellectual disability, seizures, schizophrenia, and cataracts). This form accounts for approximately 0.5% of cases, and there is also a male predominance by a ratio of 6:1. Most patients develop symptoms after puberty.
Secondary CVG is due to inflammatory, neoplastic, or systemic conditions that lead to a cellular infiltration of the dermis, which gives rise to the corrugated appearance of the scalp. Scalp folds and furrows tend to be more asymmetric and disordered in distribution. Local inflammatory conditions of the scalp that can give rise to CVG include eczema, psoriasis, and folliculitis. Systemic conditions that are associated with secondary CVG include acromegaly, myxedema, Graves disease, amyloidosis, and syphilis. Secondary CVG has also been associated with Turner syndrome, Klinefelter syndrome, and fragile X syndrome. Secondary CVG affects both sexes equally and can appear at any age, although it rarely presents in childhood.
L91.8 – Other hypertrophic disorders of the skin
Q82.8 – Other specified congenital malformations of skin
51603000 – Cutis verticis gyrata
Differential Diagnosis & Pitfalls
- Dissecting cellulitis of the scalp – Look for firm nodules that form boggy and fluctuant ridges.
- Acanthosis nigricans of the neck – Presents as a velvety, hyperpigmented plaque with fine ridges and furrows.
- Cerebriform intradermal nevus
- Pachydermoperiostosis – Thickening and furrowing tends to appear on the forehead and around the eyes. Look for associated acropachy, including clubbing of the fingers. Hyperhidrosis and arthralgias may also be present. Subperiosteal deposits at the ends of long bones may be seen on radiographic studies.
- Beare–Stevenson cutis gyrata syndrome – The association of widespread CVG (including face, neck, trunk, palms, and soles) with acanthosis nigricans and craniosynostosis. Inherited in an autosomal dominant fashion.
- Goeminne syndrome – Furrows of the scalp are associated with torticollis, keloids, cryptorchidism, and renal abnormalities.