Cutis Verticis Gyrata - Causes, Symptoms, Diagnosis, and Treatment

What Is Cutis Verticis Gyrata?

Cutis verticis gyrata (CVG), also called cutis verticis plicata, "bulldog" scalp syndrome, or paquidermia verticis gyrata, is a rare benign skin condition marked by the presence of folded and creased scalp skin that resembles the convolutions of the brain's sulci and gyri. The condition was first documented in medical literature in 1837 by Alibert, and later, in 1907, Unna coined the term "cutis verticis gyrata," which has since been widely accepted as its name.

In 1953, Butterworth introduced a classification system for CVG, dividing it into two forms: primary and secondary. However, in 1984, the classification system was expanded to include primary essential, primary non-essential, and secondary CVG. Primary non-essential CVG, now known as cutis verticis gyrata-intellectual disability (CVG-ID), can be associated with neuropsychiatric and ophthalmological abnormalities. On the other hand, secondary and primary non-essential CVG is often accompanied by additional abnormalities, while primary essential CVG typically presents without any associated conditions.

Who Is Affected by Cutis Verticis Gyrata?

Cutis verticis gyrata affects men more frequently than it does women. The male-to-female ratio of the cutis verticis gyrata primary form is 5:1. This condition may be less common in women because longer hair may be able to hide the condition. The majority of primary cases start after puberty and usually manifest before the age of 30. Cutis verticis gyrata can manifest in some secondary forms at birth, such as cerebriform intradermal naevus. Secondary CVG is a little more frequent than primary CVG and can happen to anyone at any age without regard to gender.

What Are the Causes of Cutis Verticis Gyrata?

The overgrowth of the scalp skin leads to cutis verticis gyrata. It fits into the category of:

• Primary Essential Verticis Gyrata: No additional abnormalities are associated with primary essential verticis gyrata. Primary denotes that there is no known cause of the condition. However, the specific mechanisms leading to this excessive tissue growth and folding are still unclear.

• Primary Non-essential Verticis Gyrata: The primary nonessential cutis verticis gyrata is connected to some conditions, which include:

  1. Neuropsychiatric conditions like cerebral palsy (a neurological condition affecting movement and coordination), epilepsy (a condition that causes repeated seizures), and seizures.

  2. Ophthalmologic abnormalities, most frequently cataracts.

• Secondary Cutis Verticis Gyrata: This variant is a result of various conditions that alter the structure of the scalp, including:

  1. Acromegaly (a hormonal disorder resulting in the excessive growth of body tissues and bones).

  2. Mole melanocytic naevi (benign pigmented skin growths composed of melanocytes).

  3. Birthmarks, such as fibromas, connective tissue naevus (dermis-related hamartomas), and others.

  4. Inflammatory conditions (acne, eczema, psoriasis, Darier disease, folliculitis, impetigo).

What Are the Symptoms Of Cutis Verticis Gyrata?

The symptoms of cutis verticis gyrata primarily manifest as distinctive changes in the appearance and texture of the scalp. The most notable symptom of CVG is the thickening and folding of the scalp, which gives it a distinct corrugated or ridged appearance. The skin on the affected area develops deep furrows or ridges, resembling the convolutions of the brain.

The folds can be of varying depth, size, and pattern, and they typically occur on the top and back of the scalp. The forehead and sides of the scalp are usually spared from these changes.

In addition to the visible changes, individuals with CVG may experience other associated symptoms, including:

• Hair Loss: In some cases, CVG can be accompanied by hair loss in the affected areas. The hair follicles within the folded skin may become damaged, leading to thinning or complete loss of hair.

• Scalp Itching or Discomfort: The excessive folding and stretching of the scalp skin can cause itching or discomfort in some individuals. The constant tension and compression on the underlying tissues can result in sensations of tightness or irritation.

• Psychological Impact: The distinct appearance of the scalp can attract attention and may lead to self-consciousness, social stigma, or feelings of embarrassment.

The symptoms can be bilateral or asymmetric, affecting one side of the scalp more than the other. The condition usually develops gradually over time, and its progression can be stable or slowly progressive.

How Can Cutis Verticis Gyrata Be Diagnosed?

The following steps are involved in the diagnostic process of cutis verticis gyrata :

• Medical History: The healthcare professional will review the individual's medical history, including any known conditions or factors that could be associated with secondary CVG. This may involve discussing the onset and progression of symptoms, previous medical diagnoses, family history, and exposure to potential causative factors.

• Physical Examination: A dermatologist or healthcare professional will perform a thorough examination of the scalp. They will closely observe the characteristic appearance of thickened and folded skin, with ridges or furrows resembling brain convolutions. The examination helps in identifying the presence of CVG and assessing its severity. The following features are looked for during physical evaluation:

  1. In primary cutis verticis gyrata, the thickened skin folds usually appear symmetrically from anterior to posterior, primarily affecting the vertex and occiput. It can occasionally extend horizontally and involve the entire scalp.

  2. Secondary cutis verticis gyrata may have more asymmetrical folds and can even be observed on the forehead.

  3. The folds typically present in a mild corrugated pattern across the entire scalp in inflammatory and systemic disorders.

  4. When cutis verticis gyrata is secondary to a neoplasm, it initially manifests as a localized area of scalp furrowing, which may progress to involve the entire scalp.

• Scalp Biopsy: A small sample of skin from the affected area of the scalp is taken and examined under a microscope. A biopsy helps evaluate the skin's histopathological features and can rule out other conditions with similar presentations, such as benign or malignant tumors.

• Genetic Testing: Genetic testing may be recommended if secondary CVG is suspected due to an underlying genetic disorder. This involves analyzing the individual's DNA (deoxyribonucleic acid) to identify specific gene mutations associated with CVG or related conditions.

• Imaging Studies: In cases where secondary CVG is suspected to be caused by tumors or other structural abnormalities, imaging studies like magnetic resonance imaging (MRI) or computed tomography (CT) scans may be performed. These scans provide detailed images of the scalp and underlying structures, aiding in the identification and evaluation of potential causative factors.

What Is the Treatment for Cutis Verticis Gyrata?

Treatment of the underlying condition typically results in a regression of secondary cutis verticis gyrata, though an additional intervention may be needed occasionally. After secondary involvement has been controlled, there are several ways to treat primary CVG. In order to prevent secretion accumulation, maceration, unpleasant odor, and secondary infections, maintain local scalp hygiene. Even though primary cutis verticis gyrata is a benign skin condition, surgery is still the most effective treatment option due to related psychological and cosmetic issues. The patient's size, location, and expectations will all affect the type of surgical repair.

Surgical treatment options include modalities ranging from simple excisions to more complex procedures such as tissue expansion and skin grafts.

• In cases with larger folds, a staged approach may be used, utilizing techniques like tissue expansion and local flap reconstruction.

• Another option is to perform partial resection of the most prominent segment of the lesion.

• Complete resection of excess skin folds can be achieved through the use of myocutaneous flaps or free flaps, such as the latissimus dorsi muscle.

• Smaller localized folds can be excised and closed primarily, taking care to preserve the natural appearance of the brow, eyelid, and hairline, depending on the location of the folds.

Conclusion:

Cutis verticis gyrata is an unusual condition that affects the normal texture and appearance of the scalp, giving it a distinctive and often pronounced wrinkled or furrowed appearance. Living with CVG can have emotional and psychological implications. Individuals with CVG may experience decreased self-esteem or psychological distress due to their appearance. Psychological support and counseling can be beneficial for individuals to cope with the emotional impact of the condition.