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Cutis Verticis Gyrata

Background

Cutis verticis gyrata (CVG) is a descriptive term for a condition of the scalp manifesting as convoluted folds and furrows formed from thickened skin of the scalp resembling cerebriform pattern. Although Alibert first mentioned it, Robert described the condition in 1843. Unna introduced the term cutis verticis gyrata in 1907
. Polan and Butterworth
established the classification of cutis verticis gyrata in 1953, dividing cutis verticis gyrata into primary and secondary forms.

In 1984, Garden and Robinson
improved the classification by proposing new terms: primary essential cutis verticis gyrata for cases in which no other abnormality was found (rare) and primary nonessential, which can be associated with mental deficiency, cerebral palsy, epilepsy, schizophrenia, cranial abnormalities (microcephaly), deafness, ophthalmologic abnormalities (cataract, strabismus, blindness, retinitis pigmentosa), or a combination of these. The latter has been named cutis verticis gyrata and mental retardation (OMIM 219300) or cutis verticis gyrata–intellectual disability (CVG-ID) syndrome.

Secondary cases of cutis verticis gyrata are associated with the following underlying diseases and treatments:

Acanthosis nigricans

Acromegaly

Amyloidosis

Autosomal dominant insulin-resistant syndrome

Beare-Stevenson syndrome

Connective tissue nevus

Cretinism

Cutaneous leiomyomatosis

Cylindroma

Dermatofibroma

Diabetes mellitus

Ehlers-Danlos syndrome

Fallopian tube carcinoma

Fibroma

Focal mucinosis

Graves disease

Hereditary neuralgic amyotrophy

HIV-related lipodystrophy

Hyperimmunoglobulin E syndrome

Inflammatory processes (eg, eczema, psoriasis, Darier disease, folliculitis, impetigo, erysipelas, atopic dermatitis, acne conglobata)

Intracranial aneurysm

Intraventricular ependymoma

Leukemia

Melanocytic nevi or hamartomas (cerebriform intradermal nevus, giant cellular blue naevus, cutaneous neurocristic hamartoma)

Myxedema

Neurofibroma

Nevus lipomatosus

Noonan syndrome

Pachydermoperiostosis

Posttraumatic (eg, traction alopecia)

Pseudoacromegaly

Scleromyxedema without monoclonal gammopathy

Supernumerary X chromosome syndromes (including Klinefelter syndrome)

Syphilis

Systemic T-cell lymphoma

Tuberous sclerosis

Turner syndrome

Vemurafenib and whole-brain radiotherapy combination treatment in melanoma patients

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