eMedicine Specialties > Dermatology > Pediatric Diseases

Cutis Verticis Gyrata

Author: Malgorzata D Skibinska, MD, PhD, Locum Consultant Dermatologist, Department of Dermatology, Basildon University Hospital, UK
Coauthor(s): Camila K Janniger, MD, Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, New Jersey Medical School
Contributor Information and Disclosures

Updated: Dec 8, 2008

Introduction

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 Butterworth1 established the classification of cutis verticis gyratain 1953, dividing cutis verticis gyratainto primary and secondary forms.

In 1984, Garden and Robinson2 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:

  • Pachydermoperiostosis
  • Melanocytic nevi or hamartomas (cerebriform intradermal nevus)
  • Neurofibroma
  • Fibroma
  • Dermatofibroma
  • Cylindroma
  • Nevus lipomatosus
  • Connective tissue nevus
  • Acromegaly
  • Diabetes mellitus
  • Autosomal dominant insulin-resistant syndrome
  • Myxedema
  • Cretinism
  • Amyloidosis
  • Posttraumatic (eg, traction alopecia)
  • Inflammatory processes (eg, eczema, psoriasis, Darier disease, folliculitis, impetigo, erysipelas, atopic dermatitis, acne conglobata)
  • Syphilis
  • Focal mucinosis
  • Leukemia
  • Fallopian tube carcinoma
  • Acanthosis nigricans
  • Beare-Stevenson syndrome
  • Tuberous sclerosis
  • Ehlers-Danlos syndrome
  • Noonan syndrome
  • Turner syndrome
  • Supernumerary X chromosome syndromes (including Klinefelter syndrome)
  • Hereditary neuralgic amyotrophy
  • Intracranial aneurysm
  • Intraventricular ependymoma

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.

Cutis verticis gyrata mainly occurs in males, after puberty, and it may disappear after castration. 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. 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. 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 syndrome.3,4

Frequency

United States

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

International

In male patients residing in mental hospitals, the frequency varies from 0.71-3.4% in Scotland and Sweden. Among the psychiatric adult male population in Italy, the prevalence was as much as 13.4%. 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.5,6

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.

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.

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.

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.

Clinical

History

  • In primary cutis verticis gyrata, skin plaques develop after puberty, usually in the vertex and occipital region.
  • In secondary cutis verticis gyrata, skin plaques can be present at birth.
  • The skin gradually becomes thicker, and folds and furrows are formed.
  • No other symptoms are usually present; however, pain was observed in cases with cutis verticis gyrata due to intradermal nevus and in traction alopecia.
  • The progression of changes is visible.

Physical

  • In the primary form, only the scalp is involved.
  • Cutis verticis gyrata typically affects the vertex and occipital region, but some forms can involve the entire scalp.
  • Folds are soft and spongy and cannot be corrected by pressure or traction.
  • In primary cutis verticis gyrata, folds are usually symmetric; in secondary cutis verticis gyrata, folds are asymmetric.
  • In most cases, the direction of the folds is anterior to posterior, but it may be transverse in the occipital region.
  • The number of folds varies from 2-12, although some atypical cases with 1 fold have been described.
  • The skin color is unchanged.
  • The hair over the folds may be sparse but normal in the furrows.
  • Maceration and an unpleasant smell may be present in patients with secondary infections in the furrows.

Causes

  • The cause is unknown in primary cases, although genetic and endocrinologic factors are suspected to participate in the etiology.
  • Systemic diseases, inflammatory dermatoses, underlying nevoid abnormalities, and trauma are most common in secondary cases.

More on Cutis Verticis Gyrata

Overview: Cutis Verticis Gyrata
Differential Diagnoses & Workup: Cutis Verticis Gyrata
Treatment & Medication: Cutis Verticis Gyrata
Follow-up: Cutis Verticis Gyrata
References
[ CLOSE WINDOW ]

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Further Reading

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Keywords

CVG, Robert-Unna syndrome, bulldog scalp, cutis sulcata, corrugated skin, cutis verticis plicata, pachydermia verticis gyrata, Klingon head

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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 Medical Association
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.

Medical Editor

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.

Pharmacy Editor

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
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: 3M Pharmaceutical Grant/research funds Other; Graceway Pharmaceuticals Grant/research funds Other

Managing Editor

Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of 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.

CME Editor

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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