Published on June 10, 2024. Last Updated on July 23, 2024.
What is cutis verticis gyrata (CVG)? CVG is a rare disorder common in males, characterized by deep skin folds on the scalp resembling those of the brain. Also known as the “bulldog scalp” syndrome, the disorder is classified into primary and secondary forms.
Primary Form
The primary form of bulldog scalp syndrome, or CVG, affects the vertex and occipital regions on the scalp, with folds running symmetrically in an anterior-posterior direction.
The two types are:
- Primary Essential CVG: This form is prevalent among young adults, especially postpubertal men. The cause is attributed to genetics and endocrine factors.
- Primary Non-essential CVG: Unlike the primary essential, the non-essential CVG is often associated with various abnormalities such as schizophrenia, epilepsy, retardation, ophthalmologic and cranial abnormalities.
Secondary Form
The secondary form of bulldog scalp syndrome, or CVG, is associated with inflammatory and underlying scalp diseases. These may include cutis laxa, syphilis, amyloidosis, intracranial aneurysms, and acromegaly.
What Causes Cutis Verticis Gyrata (CVG)?
Little research on the disorder shows that the cause is still undetermined. Understanding the etiology of CVG shows that it is not an individual disease entry. It is a manifestation of diverse causes:
Hormonal: The post-puberty onset of CVG and the male dominance suggest that various hormones can cause the disorder.
Imbalances of the growth hormone (GH) in some cases may lead to acromegaly or other endocrine disorders, which affect the overall physical growth of the body.
Genetics:
Genetics is often linked to the primary non-essential CVG, where the form is associated with neurodevelopmental disorders like epilepsy and retardation, among other genetic syndromes such as Turner Syndrome and Acromegaly.
Chronic Inflammation: Persistent scalp infections with recurrent inflammations may affect the skin, developing CVG folds and ridges.
Symptoms of Cutis Verticis Gyrata (CVG)
Although CVG symptoms are distinctive and visible ridges and furrows, here are others to look out for:
Primary Symptoms
- Ridges and Furrow: These are very noticeable from the onset, with soft folds varying in prominence and depth. The ridges and furrows grow gradually over time.
- Thickened Skin: During the early stages, the skin on the scalp thickens and becomes sponge-like to the touch.
Secondary Symptoms
In the case of secondary CVG form, one might experience additional symptoms related to underlying disorders or conditions:
- Hair loss or thinning on the affected areas.
- Discomfort and itching
- Other skin and hair conditions
- Symptoms related to underlying disorders may include weight loss or gain, fatigue, and enlarged body parts.
How is CVG Diagnosed?
Bulldog scalp syndrome is particularly easy and fast to diagnose. Dermatologists examine the scalp physically. The presence of furrows and ridges is sufficient to diagnose CVG.
In rare cases, a specialist will further assess other visible symptoms to classify them as primary or secondary CVG.
In the case of secondary CVG, diagnostic tests such as skin biopsy and blood tests may be ordered to check for underlying disorders such as thyroid, acromegaly, or other scalp inflammatory diseases.
Patients with CVG are advised to avoid self-treatment to ensure a correct diagnosis has been made by a certified dermatologist, who may, in turn, refer them to specialists based on the findings.
What does Bulldog scalp syndrome look like?
How Do You Treat CVG?
It is important to determine the form of Bulldog scalp syndrome/CVG diagnosed (primary or secondary) before proceeding with the treatment to ensure the methods used are effective.
To properly treat CVG, specialists are required to manage the cosmetic needs associated with changes in the scalp skin and the underlying conditions leading to the effects on the skin.
Cosmetic Treatment
- Surgical procedures
The most effective treatment method is surgical intervention, which depends on the location and size of the CVG folds. Big folds may require skin grafts and local flap reconstructions, while smaller furrows can be managed with simple cuts out of the affected area.
Surgical procedures include:
- Scalp reduction to flatten the surface.
- Laser Therapy to make the scalp skin smooth.
- Hair transplant
- Topical Treatments to manage dryness and itching.
- Use of corticosteroid injections to reduce inflammation.
Managing Underlying Conditions
- Hormonal therapy
- Treatment of associated dermatological conditions
- Preventing progressive skin changes through essential treatment of underlying disorders.
Self-Care
- Patients are encouraged to practice scalp hygiene by cleaning between furrows and ridges to prevent bad odors and secondary infections. Ensure products used are mild to the skin to reduce irritation.
- Avoid tight headgear or hairstyles to minimize scalp trauma.
CVG patients, especially those with associated conditions, require regular monitoring by specialists to assess progress and address complications or adjust treatment.
Relationship Between CVG (Cutis Verticis Gyrata) and AKN (Acne Keloidalis Nuchae (AKN)
CVG and AKN are both scalp conditions prevalent in men and characterized by small and large folds on the back region of the head. A 2022 research study on the relationship between dermal thickening and excessive folding in both disorders revealed that the spreading of AKN lesions preceded the onset of CVG by two years for patients affected by the two disorders.
In the wake of the research findings associating AKN and CVG, dermatologists have become more aware of the possibility of patients experiencing one after the other or both simultaneously. Specialists can now take precautions in addition to suggested treatment methods to manage the disorders.
Frequently Cutis Verticis Gyrata Asked Questions (FAQ)
Is Cutis Verticis Gyrata treatable?
Treatment options include managing underlying conditions, symptoms, and cosmetic needs.
Is Cutis Verticis Gyrata hereditary?
Some forms of CVG contain genetic components. However, the disease is rare and patternless.
What causes Cutis Verticis Gyrata/Bulldog Scalp Syndrome?
The exact cause is yet to be established. It can be either primary (spontaneous disease) or secondary (underlying conditions).
How can I treat Cutis Verticis Gyrata?
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References
Jeong, J., Daniela Tiemi Sano, Silvia Regina Martins, Antônio José Tebcherani, & Paula, A. (2014). Primary essential cutis verticis gyrata – Case report. Anais Brasileiros de Dermatologia, 89(2), 326–328. https://doi.org/10.1590/abd1806-4841.20142949
Koregol, S., Yatagiri, R., Warad, S., & Itagi, N. (2016). A rare association of scleromyxedema with cutis verticis gyrata. Indian Dermatology Online Journal, 7(3), 186. https://doi.org/10.4103/2229-5178.182365
Larsen, F., & Birchall, N. (2007). Cutis verticis gyrata: Three cases with different aetiologies that demonstrate the classification system. Australasian Journal of Dermatology, 48(2), 91–94. https://doi.org/10.1111/j.1440-0960.2007.00343.x
