Cutis verticis gyrata - Hair and Scalp
Synopsis

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.
Codes
L91.8 – Other hypertrophic disorders of the skin
Q82.8 – Other specified congenital malformations of skin
SNOMEDCT:
51603000 – Cutis verticis gyrata
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Last Updated:01/10/2022