Cutis Verticis Gyrata Workup

Updated: May 09, 2018
  • Author: Malgorzata D Skibinska, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
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Workup

Laboratory Studies

In primary cases, no laboratory tests should be ordered. A low free testosterone level was observed in some patients with primary cutis verticis gyrata. [40]

In secondary cases, laboratory tests depend on the presentation and the associated disease.

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Imaging Studies

Perform magnetic resonance imaging (MRI) or computed tomography (CT) in cutis verticis gyrata presenting at birth or when associated with mental retardation and neurologic and/or ophthalmologic abnormalities to determine or exclude any structural brain abnormalities. [58, 59, 60] Some authors suggest a standard anteroposterior skull film with soft-tissue technique, which may be helpful for making the diagnosis in subtle cases.

In CT scanning, thickening of the skin and subcutaneous fat and irregularly distributed cutaneous folds can be observed. Changes, such as cortical-subcortical atrophy, a dilated ventricular system, abnormal brain calcifications, bone changes, or an intracranial tumor, were also demonstrated in patients with cutis verticis gyrata. In patients with acromegaly and cutis verticis gyrata, enlarged sellae with enhancing intrasellar masses were present. [5, 61, 62]

In MRI, severe abnormality of the occipital lobes, bilateral polymicrogyria, small frontal and anterior temporal lobes, parietal and occipital cortex atrophy, colpocephaly, hypoplastic splenium of the corpus callosum, and atrophy of the cerebellar cortex were described. [63, 64]

In essential primary cutis verticis gyrata, MRIs reveal thickened dermis and a slight increase in the volume of subcutaneous fat. [59, 65]

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Other Tests

Always obtain a skin biopsy specimen of the affected area of the scalp to identify the etiology of cutis verticis gyrata. [22, 23] Evaluate female infants with cutis verticis gyrata with or without peripheral lymphedema by chromosomal analysis to exclude Turner syndrome.

Obtain an EEG in all patients with mental deficiency and cutis verticis gyrata to establish the nature of the mental disorder. Among others, severe diffuse slowing of the background activity with very frequent right temporal spikes and spike-wave complexes that spread to the frontal area were described. [66]

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Histologic Findings

The histopathologic picture is of normal appearance in most cases of the primary type. In some cases, dermal collagen thickening, hypertrophy of the pilosebaceous structures, and multiple sweat glands and ducts may be present. [67] In the secondary form, the histopathologic picture depends on the underlying disease. [23]

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