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AUTHOR AND EDITOR INFORMATION

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

Malgorzata D Skibinska is a member of the following medical societies: British Medical Association

Coauthor(s): Camila K Janniger, MD, Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, New Jersey Medical School

Editors: Daniel Hogan, MD, Chief of Dermatology, Professor, Departments of Internal Medicine and Pediatrics, Louisiana State University Medical Center; David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White Clinic; 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; Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center

Author and Editor Disclosure

Synonyms and related keywords: CVG, Robert-Unna syndrome, bulldog scalp, cutis sulcata, corrugated skin, cutis verticis plicata, pachydermia verticis gyrata, Klingon head

INTRODUCTION

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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 CVG in 1953, dividing CVG into primary and secondary forms.

In 1984, Garden and Robinson2 improved the classification by proposing new terms: primary essential CVG 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 CVG 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.

CVG 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 CVG 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 CVG in Turner syndrome.3, 4

Frequency

United States

CVG 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

CVG 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 CVG, a male-to-female ratio of 5:1 or 6:1 is observed. The incidence of CVG 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

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History

  • In primary CVG, skin plaques develop after puberty, usually in the vertex and occipital region.
  • In secondary CVG, 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 CVG due to intradermal nevus and in traction alopecia.
  • The progression of changes is visible.

Physical

  • In the primary form, only the scalp is involved.
  • CVG 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 CVG, folds are usually symmetric; in secondary CVG, 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.


DIFFERENTIALS

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Acromegaly
Cutis Laxa (Elastolysis)
Cylindroma
Pachydermoperiostosis

Other Problems to be Considered

Cerebriform intradermal nevus7


WORKUP

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Lab Studies

  • In primary cases, no laboratory tests should be ordered.
  • A low free testosterone level was observed in some patients with primary CVG.
  • In secondary cases, laboratory tests depend on the presentation and the associated disease.

Imaging Studies

  • Perform magnetic resonance imaging (MRI) or computed tomography (CT) in CVG presenting at birth or when associated with mental retardation and neurologic and/or ophthalmologic abnormalities to determine or exclude any structural brain abnormalities. 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 CVG. In patients with acromegaly and CVG, enlarged sellae with enhancing intrasellar masses were present.
    • 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.
    • In essential primary CVG, MRIs reveal thickened dermis and a slight increase in the volume of subcutaneous fat.

Other Tests

  • Always obtain a skin biopsy specimen of the affected area of the scalp to identify the etiology of CVG.
  • Evaluate female infants with CVG with or without peripheral lymphedema by chromosomal analysis to exclude Turner syndrome.
  • Obtain an EEG in all patients with mental deficiency and CVG 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

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. In the secondary form, the histopathologic picture depends on the underlying disease.


TREATMENT

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Medical Care

Properly establishing the diagnosis is very important. Separately exclude or treat any underlying process (see Background). Primary essential CVG is a cosmetic problem, but psychological repercussions are important.

Hygiene for folds and furrows is very important. In some patients, using medicated shampoos may be beneficial.

Surgical Care

  • In primary CVG, surgical resection of the lesions is usually requested for psychological or esthetic reasons.
  • In cases of intradermal nevus, early diagnosis, wide surgical excision, and plastic reconstruction are the most important procedures.

Consultations

  • Consult a surgeon to establish the possibility of surgical excision.
  • Consider a consultation with a neurologist if any suspicion exists of an underlying neurologic process.
  • Consider a consultation with an ophthalmologist if any suspicion exists of an underlying ophthalmologic abnormality.


MEDICATION

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No drugs are available in the treatment of CVG.


FOLLOW-UP

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Further Outpatient Care

  • If the patient does not agree to any surgical treatment or this procedure is contraindicated, the patient should be seen every 3-6 months to estimate the progression of the lesions.

Complications

  • Congenital nevus of the scalp, which may clinically produce CVG, can be the site of malignant transformation.
  • Malignant melanoma occurs in as many as 4-10% of patients with cerebriform intradermal melanocytic nevi, and it can occur very early in life.

Prognosis

  • The prognosis of primary CVG is good, although the condition does not regress without surgical intervention and progression of CVG may be observed.
  • In secondary cases, the prognosis depends on the underlying process.

Patient Education

  • Educate patients with CVG 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.


MISCELLANEOUS

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Medical/Legal Pitfalls

  • Failure to diagnose CVG on a routine examination is a pitfall.
  • Missing the diagnosis (especially if it is due to a cerebriform intradermal nevus) may prove fatal if malignant transformation has occurred.

Special Concerns

  • People who are mentally retarded are a group of patients among whom the condition is very common. CVG is easily missed because it is usually covered with hair.
  • Consider the diagnosis of Turner syndrome in female infants with CVG with or without peripheral lymphedema.8


REFERENCES

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Cutis Verticis Gyrata excerpt

Article Last Updated: Nov 14, 2007