What is Cutis Verticis Gyrata?
Cutis verticis gyrata (CVG), also known as paquidermia verticis gyrata, cutis verticis plicata, or “bulldog” scalp syndrome, is a rare, non-dangerous skin condition. It creates complex folds and deep ridges in the scalp that look like the grooves and bumps of a brain. This condition was first mentioned in a medical paper in 1837 by Alibert.
The name ‘cutis verticis gyrata’ was proposed by Unna in 1907 and has been in use ever since. In 1953, Butterworth classified this condition into two types: primary and secondary. This classification was updated in 1984 and now includes primary essential, primary non-essential, and secondary CVG.
The primary non-essential cutis verticis gyrata can be connected with issues affecting mental health and vision. Nowadays, this type is also known as cutis verticis gyrata-intellectual disability (CVG-ID). While secondary and primary non-essential cutis verticis gyrata are linked with other abnormalities, primary essential CVG stands alone with no associated issues.
What Causes Cutis Verticis Gyrata?
Cutis verticis gyrata (CVG) is not a disease itself, but rather a condition that can result from a variety of other underlying causes. It’s important to understand these causes in order to classify and manage CVG effectively. Some people think CVG might be caused by hormones, since it usually affects males and doesn’t start until after puberty. However, a study that tested the levels of various hormones like thyroid, cortisol, sex hormones, and prolactin in patients with CVG didn’t find any abnormalities.
As for a genetic link, it’s currently unclear whether CVG can be passed down through families.
Risk Factors and Frequency for Cutis Verticis Gyrata
Cutis verticis gyrata, a rare disorder, is most often seen in men. Typically, this condition appears in males after puberty but before age 30. The disorder is more common in individuals with mental health issues. There are two types of this condition: primary and secondary. Secondary cutis verticis gyrata is a little more common and can occur at any age in either gender. However, it’s rare to see this disorder in young children.
- Cutis verticis gyrata is a rare disorder that’s most common in men.
- About 1 in 100,000 males and 0.026 in 100,000 females have the disorder.
- The primary type usually occurs in males between puberty and age 30.
- It’s more common in patients with mental health issues.
- Secondary cutis verticis gyrata is a bit more common and can occur at any age in both genders.
- This condition is rarely seen in young children.
Signs and Symptoms of Cutis Verticis Gyrata
Cutis verticis gyrata is a condition where the scalp displays a unique pattern of deep grooves and folds, which is similar to the look of brain folds. In primary cases of this condition, these thick and symmetrical skin folds typically start from the front of the head and extend to the back, often affecting the top and back of the skull. In some cases, these folds might run horizontally and cover the whole scalp. On the other hand, secondary cutis verticis gyrata may display more uneven skin folds, and can even show up on the forehead.
When this condition is a result of an inflammatory or a systemic disorder, the scalp may display a mild wavy pattern covering the entire area. However, if this condition arises due to a tumor, it generally starts as a localized area of scalp ridges and can eventually affect the entire scalp.
Testing for Cutis Verticis Gyrata
Primary cutis verticis gyrata (CVG) can only be diagnosed after careful examination to rule out any other conditions that might cause the same symptoms. This means checking that there are no local or systemic diseases causing secondary CVG. Testing methods might include taking a small sample of skin for examination (skin biopsy), blood tests, and various scans or imaging technologies. The specific tests used for this could depend on the person’s symptoms. This is the best way to determine whether the CVG is a primary or secondary condition.
Treatment Options for Cutis Verticis Gyrata
If the skin condition known as cutis verticis gyrata (CVG) is secondary, meaning it’s caused by another underlying disorder, treatment of that disorder can often lead to regression, or improvement, of CVG. If that’s not sufficient, further intervention might be necessary.
If the condition is primary, meaning it originates on its own, it can be addressed in several ways. The most basic involves maintaining scalp hygiene to avoid accumulation of secretions, which can lead to softening of the skin, unpleasant smell, and potential infections. However, given the possible psychological and aesthetic issues linked to primary CVG, surgical removal may often be the best choice.
The specific surgical method depends on factors like the size and location of the skin folds and patient expectations. These techniques can range from just cutting away skin folds to expanding tissue and grafting skin, especially for cases with a larger area of scalp involved.
For larger folds, treating in stages using tissue expansion and local flap reconstruction is an option. Surgeons might also opt for partial removal of the most pronounced part of the lesion, or abnormal skin. In some cases, flaps of muscle and skin (myocutaneous flaps) or free flaps, such as from the latissimus dorsi muscle in the back, can be used for full removal of excessive skin folds.
For smaller, located folds, they can be removed and the area closed directly, with care to avoid distorting nearby features like the brow, eyelid, and hairline. Research has shown that partial removal with immediate closure is the safest and most effectiveness way to treat CVG. Further removal can safely occur after a one-year recovery period following the initial surgery.
What else can Cutis Verticis Gyrata be?
Cutis Verticis Gyrata (CVG) usually has a distinctive appearance which makes it easy to identify, but it’s a challenge for doctors to correctly identify accompanying or underlying conditions. CVG is now divided into three categories: primary essential, primary nonessential, and secondary.
Primary essential CVG is not linked with any other abnormalities. On the other hand, Primary nonessential CVG could be linked with conditions such as intellectual disability, cerebral palsy, and mental health issues, among others, as well as vision related problems like cataracts or even blindness. The intelligence quotient (IQ) of patients with this form of CVG usually do not exceed 35, and common symptoms may include small head size, static brain disorders, and seizures.
Secondary CVG can be associated with a broad range of conditions, from minor inflammations of the scalp to more severe conditions like leukemia or lymphoma, diabetes, skin disorders and even misuse of anabolic steroids. It can also be linked to genetic abnormalities, including Turner syndrome, Klinefelter syndrome, and fragile X syndrome among others.
What to expect with Cutis Verticis Gyrata
Although Cutis verticis gyrata can change a person’s appearance, it’s primarily a harmless condition and the outlook for those with it is generally good. There are no documented cases of this skin condition turning into cancer.
Possible Complications When Diagnosed with Cutis Verticis Gyrata
Cutis verticis gyrata, a condition that causes a wrinkly or wavy appearance of the scalp, is generally harmless. However, it’s necessary to treat any issues that come along with it and any complications that arise.
Preventing Cutis Verticis Gyrata
It’s important for patients to understand the different types of cutis verticis gyrata, often shortened to CVG. It’s particularly crucial for them to be aware of what it’s related to. It should be clearly communicated that this condition is generally harmless.
