cutis verticis gyrata

Radiopaedia • CC-by-nc-sa 3.0 • de

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 .

Clinical presentation

Patients with cutis verticis gyrata have a thickened scalp with ridges and furrows that progress in severity over time . This characteristically resembles the gyriform surface of the cerebral cortex . The folds usually manifest after puberty in teenagers and young adults and are usually asymptomatic . In severe cases, skin secretions may accumulate in the furrows resulting in an unpleasant odor developing .

Depending on the etiology, the folds may be symmetric (seen more in primary disease) or asymmetric (seen more in secondary disease) . 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) .

Unlike patients with cutis laxa, who may have a similar scalp appearance, the ridges are usually not flattened by traction in cutis verticis gyrata .

Pathology

The exact etiology of cutis verticis gyrata is unknown . 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 . In particular, testosterone is thought to play an important role, and if implicated, may explain the male predilection .

Etiology

The etiologies of cutis verticis gyrata can be classified as either primary or secondary :

Radiographic features

Although the diagnosis is primarily a clinical one, cutis verticis gyrata can be demonstrated on cross-sectional imaging . Indeed, in unknowing patients with mild disease, detection of cutis verticis gyrata by imaging may be the first time the pathology is diagnosed .

CT/MRI

CT and MRI demonstrate diffuse thickening of the scalp with marked ridges and furrows . The thickening always involves the dermis, but often also involves the subcutis as well . The thickened dermis and subcutis demonstrate the same attenuation and signal intensity, on CT and MRI respectively, as normal dermis and subcutis .

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 . An exception to this anteroposterior orientation is in the occiput, where folds may be transverse . The ridges and furrows may be symmetric, seen more-so in primary disease, or asymmetric, seen more-so in secondary disease .

Patients with severe disease have a greater number of folds and ridges . The appearance of such disease has been given the description of having a 'cog-wheel' pattern radiographically .

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 .

Treatment and prognosis

Management depends on severity of the disease . In mild disease, good scalp hygiene is recommended to avoid accumulations of malodourous secretions in furrows . However, in more severe disease, surgical scalp reduction can be considered .

In patients with secondary cutis verticis gyrata, management should also be focused on the underlying cause .

History and etymology

Cutis verticis gyrata was first described by Jean-Louis-Marc Alibert (1768-1837), a French dermatologist, in 1837 . However the term "cutis verticis gyrata" was first coined many years later by Paul Gerson Unna (1850-1929), a German dermatologist, in 1907 .

Differential diagnosis

Siehe auch: