Cutis Verticis Gyrata (CVG) – Classification, Causes and Treatment

Definition

Cutis verticis gyrata (CVG) refers to deep folds on the scalp that look similar to the folds of the brain. It occurs more commonly in males, and most commonly develops after puberty, but before age 30. It may occur alone (isolated CVG) or in association with a variety of underlying conditions or treatments, including neuropsychiatric disorders, eye abnormalities, or inflammatory conditions. While most isolated cases of unknown cause are sporadic, autosomal recessive and autosomal dominant inheritance with varying degrees of severity have been described. In cases associated with underlying conditions, the cause depends on the underlying condition. Management may include keeping areas within the folds clean, and/or surgery if requested for psychological or cosmetic reasons.

Classification of Cutis Verticis Gyrata

It is the result of overgrowth of the scalp skin. It can be classified as:

Primary essential cutis verticis gyrata: No other associated abnormalities. This type means the cause of the condition is unknown.

Primary nonessential cutis verticis gyrata: Associated with neuropsychiatric disorders including cerebral palsy, epilepsy, seizures, and ophthalmologic abnormalities, most commonly cataracts.

Secondary cutis verticis gyrata: Consequence of a number of diseases that produce changes in scalp structure including:

• Acromegaly
• Melanocytic naevi (moles)
• Birthmarks, including connective tissue naevus, fibromas and naevus lipomatosus
• Inflammatory processes (eczema, psoriasis, Darier disease, folliculitis, impetigo, atopic dermatitis, acne)

Cutis Verticis Gyrata Epidemiology

It is a rare disorder, predominately seen in men, with a reported prevalence of about 1 in 100000 males and 0.026 in 100000 females. Primary CVG usually occurs in postpubertal men before the age of 30, with an increased incidence seen in patients with mental disorders. Secondary CVG, which is slightly more common than primary and can occur at any age with no gender predilection. CVG rarely presents in young children.

Pathophysiology

The pathogenesis of primary cutis verticis gyrata remains unclear. A hormonal influence has been postulated as this disorder usually manifests in postpubertal men. Secondary cutis verticis gyrata is considered to be a manifestation of a variety of underlying causes, and the pathophysiology, in this case, can be correlated to the specific underlying condition.

Causes and risk factors of Cutis Verticis Gyrata

The causes can be diverse and are often associated with underlying conditions or factors that contribute to the thickening and folding of the scalp skin. While the precise cause may vary among individuals, some common factors include:

1. Primary Essential Cutis Verticis Gyrata (PECVG): In some cases, cutis verticis gyrata is considered idiopathic, meaning it occurs without an apparent underlying cause. This form is referred to as Primary Essential Cutis Verticis Gyrata (PECVG).
2. Genetic Factors: There is evidence to suggest a genetic predisposition to cutis verticis gyrata. Some cases may have a familial component, indicating a potential genetic influence on the development of the condition.
3. Inflammatory Conditions: Chronic inflammation of the scalp, such as in conditions like seborrheic dermatitis or psoriasis, can contribute to the thickening of the skin and the development of cutis verticis gyrata.
4. Neurological Disorders: Some cases of cutis verticis gyrata have been associated with underlying neurological disorders. The relationship between the nervous system and the skin’s structural changes is not fully understood, but certain neurological conditions may contribute to the development of CVG.
5. Trauma: Physical trauma to the scalp, such as repeated friction or pressure, can lead to the thickening and folding of the skin, contributing to the appearance of cutis verticis gyrata.
6. Endocrine Disorders: Disorders affecting the endocrine system, such as acromegaly (excessive growth hormone production), have been linked to cutis verticis gyrata in some cases.

It’s important to note that the specific cause of cutis verticis gyrata can vary among individuals, and in many cases, a combination of factors may contribute to its development.

Symptoms of cutis verticis gyrata

• Main Symptom: Thickening and folding of the skin on the scalp, giving it a corrugated or brain-like appearance.
• Appearance: The scalp texture changes gradually and can vary from mild to more pronounced folds.
• Progression: CVG typically develops slowly over time.
• Cosmetic Impact: The main concern is the cosmetic appearance, which may lead to self-consciousness or social concerns.
• Hair: Some individuals may experience associated hair loss or thinning, but it’s not always present.
• Discomfort: Itching or discomfort may occur in some cases.
• Benign Condition: CVG is generally considered a benign condition, meaning it’s not usually associated with serious health issues.
• Localized to Scalp: The symptoms are primarily on the scalp and don’t affect internal organs or overall health.

Complications of Cutis Verticis Gyrata

It is generally considered a benign condition, and it is not associated with severe complications or significant health risks. However, there are certain aspects related to the condition that individuals may find challenging:

1. Psychosocial Impact: The most significant complication of CVG is often the psychological and social impact. The altered appearance of the scalp may lead to self-consciousness, reduced self-esteem, or concerns about social acceptance.
2. Quality of Life: The cosmetic nature of CVG can affect an individual’s quality of life, potentially leading to emotional distress or avoidance of social situations.
3. Hair-Related Issues: While not a universal symptom, some individuals with CVG may experience associated hair loss or thinning in the affected areas. This can contribute to additional cosmetic concerns.
4. Potential for Discomfort: In some cases, individuals with CVG may experience itching or discomfort related to the changes in the scalp’s skin texture.

It’s important to note that CVG itself does not pose direct health threats, and the complications mentioned are more related to the impact on well-being and quality of life.

Diagnosis

The diagnosis of cutis verticis gyrata (CVG) typically involves a combination of clinical evaluation, medical history review, and, in some cases, additional tests. Here’s an overview of the diagnostic process:

Clinical Examination

A dermatologist or healthcare provider will conduct a thorough examination of the scalp to assess the characteristic thickening and folding of the skin. The distinctive corrugated or cerebriform appearance is a key visual clue for diagnosing CVG.

Medical History

Gathering a detailed medical history is crucial. This may include information about the onset and progression of symptoms, any associated symptoms like itching or discomfort, and family history.

Differential Diagnosis

Since it is a rare condition, healthcare professionals may consider other dermatological conditions with similar symptoms during the diagnostic process. These may include certain forms of hyperplasia, pachydermoperiostosis, or other scalp conditions.

Imaging Studies

In some cases, imaging studies such as magnetic resonance imaging (MRI) or computed tomography (CT) scans may be used to examine the thickness and structure of the scalp tissues. This is not always necessary but can be employed if the diagnosis is unclear or if there is a need to rule out other underlying conditions.

Biopsy (Rarely)

A skin biopsy may be performed in some cases, especially if there is uncertainty about the diagnosis. This involves taking a small sample of skin tissue for laboratory analysis.

Underlying Conditions

Since cutis verticis gyrata can be associated with underlying medical conditions such as inflammatory disorders or neurological issues, additional tests may be conducted to identify or rule out these contributing factors.

The healthcare provider will tailor the diagnostic approach based on the individual’s specific symptoms and medical history. Once diagnosed, the healthcare team can discuss appropriate management options and address any cosmetic or psychological concerns.

Treatment for Cutis Verticis Gyrata

The treatment depends on the underlying cause, as CVG can be primary (idiopathic) or secondary to other conditions.

Topical Treatments

Emollients and moisturizers can be applied to the affected area to help manage dryness and reduce scaling. Topical steroids may be prescribed to reduce inflammation and control symptoms.

Oral Medications

Oral antibiotics may be prescribed if there is an associated bacterial infection. In some cases, oral retinoids may be considered to help regulate skin cell growth and reduce thickening.

Surgery

Surgical intervention may be an option in severe cases or when there is a significant cosmetic concern. Surgical procedures may include dermabrasion, scalp reduction, or skin excision to reshape the affected area.

Scalp Reduction

This surgical procedure involves removing a portion of the affected scalp and repositioning the remaining tissue to create a smoother appearance.

Laser Therapy

Laser therapy may be used to reduce excess tissue and improve the appearance of the scalp.

Treatment of Underlying Conditions:

If cutis verticis gyrata is secondary to an underlying medical condition, such as acromegaly, neurofibromatosis, or inflammatory disorders, treating the primary condition may help manage CVG.

It’s crucial to consult with a dermatologist or a healthcare professional to determine the most appropriate treatment plan based on the specific characteristics and underlying cause of cutis verticis gyrata in each individual case. The treatment approach may vary, and a combination of therapies may be recommended for optimal results.

Prevention of Cutis Verticis Gyrata

It is a rare condition, and in many cases, its underlying cause is not fully understood. As a result, preventing cutis verticis gyrata may not always be possible. However, there are general skin care practices that can help maintain a healthy scalp and may contribute to overall skin health:

Good Hygiene:

Keep the scalp clean by regularly washing your hair with a mild shampoo. Proper hygiene can help prevent bacterial and fungal infections that may contribute to skin conditions.

Moisturize:

Use a mild moisturizer on the scalp if it tends to be dry. Moisturizing can help prevent excessive dryness and scaling of the skin.

Avoid Trauma:

Be gentle when combing or brushing your hair to avoid scalp trauma. Trauma to the scalp may exacerbate certain skin conditions or contribute to inflammation.

Protect from Sun Exposure:

Protect your scalp from excessive sun exposure by wearing a hat or using sunscreen. Prolonged sun exposure can damage the skin and may contribute to certain skin conditions.

Manage Underlying Conditions:

If you have an underlying medical condition associated with cutis verticis gyrata, work closely with your healthcare provider to manage and treat that condition effectively.

Regular Dermatological Check-ups:

Schedule regular check-ups with a dermatologist, especially if you notice any changes in your skin or scalp.

It’s important to note that cutis verticis gyrata is often a result of an underlying condition, and preventing the underlying cause may not always be within your control. If you have concerns about your skin or scalp health, it’s advisable to seek guidance from a healthcare professional or dermatologist who can provide personalized advice based on your specific situation.