Cutis Verticis Gyrata

Updated: Apr 14, 2016
  • Author: Malgorzata D Skibinska, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
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Overview

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 [1] . Polan and Butterworth [2] established the classification of cutis verticis gyrata in 1953, dividing cutis verticis gyrata into primary and secondary forms.

In 1984, Garden and Robinson [3] 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.

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

  • Amyloidosis [6]
  • Autosomal dominant insulin-resistant syndrome [7]
  • Beare-Stevenson syndrome
  • Connective tissue nevus
  • Cretinism
  • Cutaneous leiomyomatosis [8]
  • Diabetes mellitus
  • Fallopian tube carcinoma
  • Focal mucinosis
  • Graves disease [9]
  • Hereditary neuralgic amyotrophy [10]
  • HIV-related lipodystrophy [11]
  • Hyperimmunoglobulin E syndrome [12, 13]
  • Inflammatory processes (eg, eczema, psoriasis, Darier disease, folliculitis, impetigo, erysipelas, atopic dermatitis, acne conglobata) [14, 15, 16, 17]
  • Intracranial aneurysm [18]
  • Intraventricular ependymoma [18]
  • Leukemia [19]
  • Melanocytic nevi or hamartomas (cerebriform intradermal nevus, giant cellular blue naevus, cutaneous neurocristic hamartoma) [20, 21, 22, 23, 24]
  • Myxedema [9, 25]
  • Neurofibroma [26]
  • Nevus lipomatosus
  • Posttraumatic (eg, traction alopecia) [31]
  • Pseudoacromegaly [32]
  • Supernumerary X chromosome syndromes (including Klinefelter syndrome) [33]
  • Systemic T-cell lymphoma [34]
  • Turner syndrome
  • Vemurafenib and whole-brain radiotherapy combination treatment in melanoma patients [35, 36]
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Pathophysiology

In the primary essential form, the etiology is not known, and, though most of the cases seem sporadic, autosomal recessive and autosomal dominant inheritance with variable expression have been described. In the primary nonessential form, the pathogenesis (beside the genetic determination) may have an endocrinologic basis. [16, 37]

Cutis verticis gyrata mainly occurs in males, after puberty, and it may disappear after castration. [3] This may be due to increased peripheral use of testosterone, which was further supported by the results of the study in which the free testosterone level was reduced in patients with primary cutis verticis gyrata compared with controls. [38] Male predominance may also suggest an X-linked inheritance. An association with the fragile X syndrome or other fragile sites on chromosomes 9, 10, and 12, and, in a single case, breaks at bands 3p14 and 16q23, has been reported. [39, 40, 41, 42, 43] In the secondary form, the etiology depends on the underlying process (eg, inflammatory, neoplastic). Lymphedema is a postulated cause of cutis verticis gyrata in Turner and Noonan syndromes. [27, 44, 45, 46]

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Epidemiology

Frequency

United States

Cutis verticis gyrata occurs in 0.5% of people with mental retardation. [3]

International

In male patients residing in mental hospitals, the frequency varies from 0.71% to 3.4% in Scotland and Sweden. [47, 48, 49] Among the mentally retarded adult male patients in Italy, the prevalence was as much as 11.4%. [42] The only data available for the general population are from 1964, with an estimated prevalence of 1 case in 100 000 population for males and 0.026 case in 100,000 population for females. [47, 48]

Race

No data are available, though the higher prevalence in Italy may be associated with an ethnic factor as most patients were of Sicilian origin. [42]

Sex

In primary cutis verticis gyrata, a male-to-female ratio of 5:1 or 6:1 is observed. The incidence of cutis verticis gyrata may appear to be lower in women because longer hair may camouflage the condition. [3]

Age

Most primary cases develop after puberty and often (90%) before age 30 years. Some secondary forms, like cerebriform intradermal nevus, may be present at birth. [3]

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Prognosis

The prognosis of primary cutis verticis gyrata is good, although the condition does not regress without surgical intervention and progression of cutis verticis gyrata may be observed. In secondary cases, the prognosis depends on the underlying process. [30]

Cutis verticis gyrata is long lasting and progressive. It is often found to be unacceptable because of cosmetic reasons. It can be complicated by malignant melanoma developing within a congenital melanocytic nevus. [21, 50]

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Patient Education

Educate patients with cutis verticis gyrata that proper hygiene of the scalp is essential to avoid the accumulation of secretion in the furrows. Lack of hygiene may be the cause of an unpleasant smell and secondary infection.

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