Cutis Verticis Gyrata 

  • Author: Malgorzata D Skibinska, MD, PhD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jul 15, 2010
 

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:

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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.[21, 29]

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.[30] 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.[31, 32, 33, 34, 35, 36] 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.[24, 37, 38, 39]

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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.[40, 41, 42] Among the mentally retarded adult male patients in Italy, the prevalence was as much as 11.4%.[35] 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.[40, 41]

Mortality/Morbidity

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.[8, 43]

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.[35]

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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Contributor Information and Disclosures
Author

Malgorzata D Skibinska, MD, PhD  Locum Consultant Dermatologist, Department of Dermatology, Basildon University Hospital, UK

Malgorzata D Skibinska, MD, PhD is a member of the following medical societies: British Association of Dermatologists, British Medical Association, European Society for Dermatological Research, Royal College of Physicians of the United Kingdom, and Royal Society of Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Camila K Janniger, MD  Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, New Jersey Medical School

Camila K Janniger, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Daniel J Hogan, MD  Clinical Professor of Internal Medicine (Dermatology), NOVA Southeastern University; Investigator, Hill Top Research, Florida Research Center

Daniel J Hogan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Contact Dermatitis Society, and Canadian Dermatology Association

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

Glen H Crawford, MD  Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital

Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

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