Cutis verticis gyrata

Cutis verticis gyrata is a rare progressive dermatological condition characterized by excessive skin folds in the scalp, resembling the surface of the cerebral cortex.

Epidemiology

It occurs more commonly in males, with a male-to-female ratio of approximately 5:1 ¹.

Clinical presentation

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.

Pathology

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. 

Etiology

The etiologies of cutis verticis gyrata can be classified as either primary or secondary ^1-6:

• primary

  • primary essential: no other associated anomalies

  • primary non-essential: associated with neuropsychiatric or ophthalmologic anomalies

• secondary

  • acromegaly

  • scalp dermatologic conditions (e.g. benign or malignant tumors, nevi, inflammatory dermatoses, trauma, etc.)

  • hypothyroidism

  • malignancy (e.g. leukemia)

  • amyloidosis

  • type 2 diabetes mellitus

  • Ehlers-Danlos syndrome

  • tuberous sclerosis

  • pachydermoperiostosis

  • Beare-Stevenson cutis gyrata syndrome

  • syphilis

  • Noonan syndrome

  • various aneuploidies (e.g. Turner syndrome, Klinefelter syndrome, etc.)

Radiographic features

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/MRI

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 focussed 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.

Differential diagnosis

• cutis laxa

• lipedematous scalp