Cutis Verticis Gyrata

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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[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. The latter has been named cutis verticis gyrata and mental retardation (OMIM 219300) or cutis verticis gyrata–intellectual disability (CVG-ID) syndrome.[4]

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

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.[17, 39]

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.[40] 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.[41, 42, 43, 44, 45] 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.[28, 46, 47, 48] A possible role of IGF-1 in the pathogenesis in acromegaly-associated cutis verticis gyrata was suggested.[49]

Etiology

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. Cutis verticis gyrata‒like lesions have been described in melanoma patients undergoing treatment with vemurafenib and whole-brain radiotherapy, but not with each of the treatments alone.[37, 38]

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.[50, 51, 52] Among the mentally retarded adult male patients in Italy, the prevalence was as much as 11.4%.[44] 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.[50, 51] A systematic review of all patients with CVG-ID syndrome reported in the last 60 years suggests that it is most likely under-recognized as a result of the deinstitutionalization of severely intellectually disabled people.[4]

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.[44]

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. Congenital cases of primary cutis verticis gyrata have been rarely reported.[53] Some secondary forms, like cerebriform intradermal nevus, may be present at birth.[3]

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.[31]

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.[22, 54]

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.

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 Examination

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.

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Cutis verticis gyrata. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/dermal-infiltrative/cvg2.jpg).

Laboratory Studies

In primary cases, no laboratory tests should be ordered. A low free testosterone level was observed in some patients with primary cutis verticis gyrata.[40]

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 cutis verticis gyrata presenting at birth or when associated with mental retardation and neurologic and/or ophthalmologic abnormalities to determine or exclude any structural brain abnormalities.[58, 59, 60] 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 cutis verticis gyrata. In patients with acromegaly and cutis verticis gyrata, enlarged sellae with enhancing intrasellar masses were present.[5, 61, 62]

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.[63, 64]

In essential primary cutis verticis gyrata, MRIs reveal thickened dermis and a slight increase in the volume of subcutaneous fat.[59, 65]

Other Tests

Always obtain a skin biopsy specimen of the affected area of the scalp to identify the etiology of cutis verticis gyrata.[22, 23] Evaluate female infants with cutis verticis gyrata with or without peripheral lymphedema by chromosomal analysis to exclude Turner syndrome.

Obtain an EEG in all patients with mental deficiency and cutis verticis gyrata 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.[66]

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.[67] In the secondary form, the histopathologic picture depends on the underlying disease.[23]

Medical Care

Properly establishing the diagnosis is very important. Separately exclude or treat any underlying process (see Background). Improvement of cutis verticis gyrata with topical treatment of causal dermatitis has been reported.[68] Primary essential cutis verticis gyrata 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 cutis verticis gyrata, surgical resection of the lesions is usually requested for psychological or esthetic reasons.[67, 39, 69, 70, 71, 72, 73]

In cases of cerebriform intradermal nevus, early diagnosis, wide surgical excision, and plastic reconstruction should be always considered.[21, 22, 55]

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. Additionally, consider a consultation with an ophthalmologist if any suspicion exists of an underlying ophthalmologic abnormality.

Complications

Congenital nevus of the scalp, which may clinically produce cutis verticis gyrata, can be the site of malignant transformation and it can occur very early in life.[21, 22, 23, 54]

Malignant melanoma occurs in up to 10% of patients with cerebriform melanocytic nevi; however, recent data suggest the rate of transformation may only be on the order of 4.5%.[21, 22, 57]

Long-Term Monitoring

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.

Patients with cerebriform melanocytic nevus require long-term clinical follow-up in order to detect malignant transformation.[22, 55]

Medication Summary

No drugs are available in the treatment of cutis verticis gyrata.

Author

Malgorzata D Skibinska, MD, PhD, Senior Lecturer, Department of Dermatology, Pediatric Dermatology and Oncology, Medical University of Łódź; Consultant Dermatologist and Allergist, Private Practice, Łódź, Poland

Disclosure: Nothing to disclose.

Coauthor(s)

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

Disclosure: Nothing to disclose.

Specialty Editors

David F Butler, MD, Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Pathology, Professor of Pediatrics, Professor of Medicine, Rutgers New Jersey Medical School

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Disclosure: Nothing to disclose.

Additional Contributors

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

Disclosure: Nothing to disclose.

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Cutis verticis gyrata. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/dermal-infiltrative/cvg2.jpg).

Cutis verticis gyrata. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/dermal-infiltrative/cvg2.jpg).