It occurs more commonly in males, with a male-to-female ratio of approximately 5:1 1.
Patients with cutis verticis gyrata have a thickened scalp with ridges and furrows that progress in severity over time 2-4. This characteristically resembles the gyriform surface of the cerebral cortex 2-4. The folds usually manifest after puberty in teenagers and young adults and are usually asymptomatic 2-4. In severe cases, skin secretions may accumulate in the furrows resulting in an unpleasant odor developing 2.
Depending on the etiology, the folds may be symmetric (seen more in primary disease) or asymmetric (seen more in secondary disease) 2-4. Furthermore, some patients with primary disease may additionally demonstrate a wide array of neuropsychiatric (e.g. epilepsy, cerebral palsy) or ophthalmologic anomalies (e.g. retinitis pigmentosa, cataracts) 2-4.
Unlike patients with cutis laxa, who may have a similar scalp appearance, the ridges are usually not flattened by traction in cutis verticis gyrata 2.
The exact etiology of cutis verticis gyrata is unknown 3. However, because it typically manifests after puberty, it has been postulated that various endocrine factors might play a role in the pathogenesis of primary disease 3. In particular, testosterone is thought to play an important role, and if implicated, may explain the male predilection 1,3.
The etiologies of cutis verticis gyrata can be classified as either primary or secondary 1-6:
- primary essential: no other associated anomalies
- primary non-essential: associated with neuropsychiatric or ophthalmologic anomalies
- scalp dermatologic conditions (e.g. benign or malignant tumors, nevi, inflammatory dermatoses, trauma, etc.)
- malignancy (e.g. leukemia)
- type 2 diabetes mellitus
- Ehlers-Danlos syndrome
- tuberous sclerosis
- Beare-Stevenson cutis gyrata syndrome
- Noonan syndrome
- various aneuploidies (e.g. Turner syndrome, Klinefelter syndrome, etc.)
Although the diagnosis is primarily a clinical one, cutis verticis gyrata can be demonstrated on cross-sectional imaging 1,4-6. Indeed, in unknowing patients with mild disease, detection of cutis verticis gyrata by imaging may be the first time the pathology is diagnosed 6.
CT and MRI demonstrate diffuse thickening of the scalp with marked ridges and furrows 1,4-6. The thickening always involves the dermis, but often also involves the subcutis as well 1,4-6. The thickened dermis and subcutis demonstrate the same attenuation and signal intensity, on CT and MRI respectively, as normal dermis and subcutis 1,4-6.
The width of the ridges and depth of the furrows vary depending on the severity, however, the direction of folds and ridges is usually anterior to posterior 1,4-6. An exception to this anteroposterior orientation is in the occiput, where folds may be transverse 1. The ridges and furrows may be symmetric, seen more-so in primary disease, or asymmetric, seen more-so in secondary disease 1,4-6.
Patients with severe disease have a greater number of folds and ridges 1,4-6. The appearance of such disease has been given the description of having a 'cog-wheel' pattern radiographically 1.
Additionally, cross-sectional imaging should be used to interrogate the brain and orbits in the instance of primary non-essential disease, and also has a role in detecting an underlying etiology in secondary diseases, such as a pituitary adenoma in acromegaly 6.
Treatment and prognosis
Management depends on severity of the disease 2. In mild disease, good scalp hygiene is recommended to avoid accumulations of malodourous secretions in furrows 2. However, in more severe disease, surgical scalp reduction can be considered 2.
In patients with secondary cutis verticis gyrata, management should also be focused on the underlying cause 2.
History and etymology
Cutis verticis gyrata was first described by Jean-Louis-Marc Alibert (1768-1837), a French dermatologist, in 1837 6. However the term "cutis verticis gyrata" was first coined many years later by Paul Gerson Unna (1850-1929), a German dermatologist, in 1907 6,7.
- 1. Kolawole TM, Al Orainy IA, Patel PJ, Fathuddin S. Cutis verticis gyrata: its computed tomographic demonstration in acromegaly. (1998) European journal of radiology. 27 (2): 145-8. Pubmed
- 2. Diven DG, Tanus T, Raimer SS. Cutis verticis gyrata. (1991) International journal of dermatology. 30(10):710-2. doi:10.1111/j.1365-4362.1991.tb02615.x/abstract
- 3. Palazzo R, Schepis C, Ruggeri M, Baldini L, Pizzimenti A, Arcoraci V, Spina E. An endocrinological study of patients with primary cutis verticis gyrata. (1993) Acta dermato-venereologica. 73 (5): 348-9. Pubmed
- 4. Okamoto K, Ito J, Tokiguchi S, Ishikawa K, Furusawa T, Sakai K. MRI in essential primary cutis verticis gyrata. (2016) Neuroradiology. 43 (10): 841. doi:10.1007/s002340100591 - Pubmed
- 5. Alorainy IA. Magnetic resonance imaging of cutis verticis gyrata. (2008) Journal of computer assisted tomography. 32 (1): 119-23. doi:10.1097/RCT.0b013e31805d08a9 - Pubmed
- 6. do Amaral LLF, Bag AK, Gonçalves FG, Hanagandi PB. Advanced Neuroradiology Cases. ISBN: 9781316692394
- 7. Unna PG. Cutis verticis gyrata. (1907) Monatsh Prakt Derm. 45:227–233.