Primary Cutis Verticis Gyrata Research Articles

CLINICAL CASE OF CUTIS VERTICIS GYRATA

Cutis verticis gyrata (CVG) is a descriptive term for a rare benign skin condition characterized by the formation of tortuous folds and deep furrows that mimic the gyri of the brain because of excessive skin growth. It is observed mainly in men. The first clinical case of the disease was described in 1837 by Alibert J.
 There are primary and secondary forms of CVG. Among the cases of primary CVG in 1984, Garden JM and Robinson JK further distinguished the essential and combined subtypes. The reason for the appearance of the essential form is not known. Primary combined CVG can accompany neuropsychiatric disorders, cerebral palsy, epilepsy, seizures, and ophthalmological abnormalities, most commonly cataracts. Secondary CVG occurs as a result of chronic inflammation, such as hysteria, lymphostasis, elephantiasis, eczema, psoriasis, folliculitis, atopic dermatitis, etc. In case of secondary CVG, the lower extremities are more often affected, the skin is congested-red, cyanotic, dense, swollen. Papillomatous growths, layering of crusts and scales are often visible against the background of changed skin.
 Given the rarity of this disease (it was diagnosed for the first time in our more than 50 years of dermatological practice), we report a clinical case of CVG.
 Case Presentation. Patient P., 27 years old, complained of wrinkles and increased mobility of the skin of the scalp. He noticed the first manifestations of the disease at the age of 17, but did not pay attention, believing that these were individual features of the skin structure. For the last 3-4 years in a row, he noticed a thickening and increase in the size of the folds of the skin on the scalp, growing mobility. The skin, according to the patient, had the appearance of ribs.
 During the dermatological examination, the skin is of normal color, turgor and elasticity are preserved, sweat and sebum secretion is moderate. Skin appendages are without pathological changes. The skin of the scalp is uniformly covered with thick hair, hyperelastic, pronounced transverse deep linear furrows resembling brain convolutions are visible.
 When using dermoscopy without a light-conducting liquid, the vascular pattern is hardly visible, on the surface there is small lamellar peeling. However, after its application, areas of clearly visible vessels of the superficial vascular plexus of the skin, which coincide with the location of the furrows, are determined.
 The patient was examined; no pathology was detected on the part of internal organs and heart. The results of ultrasound of the thyroid gland and organs of the abdominal cavity were without pathological changes. General analysis of blood, urine, blood sugar within the normal range. Biochemical indicators of liver and kidney function without deviations. The patient is recommended to have a periodic examination by a dermatologist. With increasing clinical manifestations - surgical removal of skin folds.
 Conclusions. The described clinical case of CVG is of considerable interest to dermatologists, pediatricians, and geneticists as a rare new developmental disease. In our report, the clinical picture of changes on the skin is illustrated, and differential diagnosis with dermatoses that are closest in clinical manifestations is briefly carried out.

54. Primary cutis verticis gyrata associated to chronic migraine treated with ultrasound guided onabotulinum injections. A case report

Cutis verticis gyrata (CVG), also known as “bulldog” scalp syndrome was originally described in 1837 by Jean-Louis-Marc Alibert. 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. In 1953 Polan and Butterworth classified CVG into two forms: primary and secondary. The primary form can be divided in essential and non-essential, where the essential form do not present other abnormality (while primary nonessential can be associated with other pathologies or a combination of this. 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. In the secondary form, the etiology depends on the underlying process (eg,inflammatory, neoplastic). Our patient was a 25 year old caucasian male who presented for three years scalp skin redundancy, with deep cerebriform folds and wrinkles, located in frontoparietal regions He denied comorbidities. He referred fronto temporal headache often unilateral with casual trigeminal diffusion, described as chronic and burning pain with allodinia. For those he performed a 3T MRI of head with a 3D reconstraction of scalp that excluded abnormality of the brain lobes and essentially revealed thickened dermis and a slight increase in the volume of subcutaneous fat and irregularly distributed cutaneous folds characteristic of CVG. Because neurologist considered patient with CM and inadequate response or intolerance to previous two prophylactic treatments he was treated with pericranial injections of about 150 UI of BoNT-A with the proposal to repeat treatment every 3–4 months and the purpose to increase dose up to 200 U in case of no response. After 3 months unfortunately patient achieved minimal reduction of pain of about 20–30% in headache frequency and intensity and during the 3 months follow-up period. The first weeks after BonT-A treatment he reduced NSAIDs and analgesics consumption but unfortunately after first stage he preferred to avoid further botulinum injections due to mini invasive procedure on scalp. Primary essential cutis verticis gyrata firstly because is a cosmetic problem, but also because eventual pain symptoms and psychological repercussions are very important issues. In this case both pain relief and reduction of folds width could be beneficials and therefore we encouraged the patient to treat both CM and CVG with BonT-A Injections.

Case report: Primary cutis verticis gyrata associated with psoriasis

Case report: Primary cutis verticis gyrata associated with psoriasis

Pachydermoperiostosis vs. Acromegaly in a Patient with Cutis Verticis Gyrata

Cutis Verticis Gyrata (CVG) is a rare condition associated with thickening of the skin folds of the scalp and forehead that resembles the appearance of gyri of the brain cortex. These skin lesions can lead to local skin infections as well as cosmetic complaints. Primary CVG has no obvious cause and is often associated with neurological conditions while secondary CVG is due to inflammation or infiltration of the scalp and is associated with endocrine or neoplastic conditions including acromegaly and pachydermoperiostosis. We discuss a complicated case of a 34 year-old male with cutis verticis gyrata presenting simultaneously with hypertrophic osteoarthropathy and a pituitary macroadenoma. The patient was initially suspected of having acromegaly secondary to the pituitary adenoma but further workup showed normal insulin growth factor-1 and growth hormone levels inconsistent with acromegaly. Subsequent workup was consistent with a diagnosis of the complete form of primary hypertrophic osteoarthropathy also known as pachydermoperiostosis.

Cutis Verticis Gyrata and Alopecia Areata: A Synchronous Coincidence?

Cutis verticis gyrata (CVG) is a descriptive term for a scalp condition that is convoluted folds and deep furrows that resemble the surface of the cerebral cortex. It is categorized by the underlying etiology, as primary essential, primary non-essential and secondary. Alopecia areata (AA) is a common, organ specific autoimmune disease, and most AA cases are sporadic. There is clearly a strong genetic component. There is no established relationship between CVG and AA. We report one case which was affected with essential primary CVG and alopecia areata, and suggest a possibility of genetic association between CVG and AA, possibly both being related to mutations in the fibroblast growth factor receptor 2 (FGFR2).

Using novel subcision technique for the treatment of primary essential cutis verticis gyrata

Primary essential cutis verticis gyrata (CVG) is characterized by folds and furrows of the scalp, resembling the convoluted appearance of the brain, and is not associated with other abnormalities. Most patients with CVG are treated with surgical methods, such as scalp reduction; however, surgery may not be suitable for patients in whom the area involved is large. A 37-year-old man with primary essential CVG was treated with a scalp subcision technique under local anesthesia. A successful cosmetic improvement was obtained. The scalp subcision technique is a safe procedure and should be a treatment option for primary CVG.

Primary Cutis Verticis Gyrata and Scalp Reduction in One Stage with Multiple Pinwheel Flaps (Revisited)

Primary Cutis Verticis Gyrata and Scalp Reduction in One Stage with Multiple Pinwheel Flaps (Revisited)

Primary Cutis Verticis Gyrata and Scalp Reduction in One Stage with Multiple Pinwheel Flaps (Revisited)

Primary Cutis Verticis Gyrata and Scalp Reduction in One Stage with Multiple Pinwheel Flaps (Revisited)

An unusual association of cutis verticis gyrata with empty sella

Cutis verticis gyrata (CVG) is a condition in which excessive growth of the scalp skin leads to the formation of furrows that resemble a cerebriform pattern and cannot be corrected by external pressure. There is no evidence of an endocrine influence in the primary form. In the literature, only three reports of primary essential CVG have been found, and all reported cases involved males [1,2]. Secondary CVG has been linked to paraneoplastic syndromes, to local inflammatory skin conditions (such as eczema and psoriasis), and to systemic illnesses (such as amyloidosis, syphilis, Ehlers–Danlos syndrome, acanthosis nigricans, and insulin resistance syndrome) [3]. Endocrine disorders associated with secondary CVG include acromegaly, cretinism, and myxedema [4]. The familial form of CVG is extremely rare. We report a female patient with primary CVG and partial empty sella without any hormonal deficiency. Her brother's and father's scalps were reported to have the same appearance and they had no known neurological or ophthalmologic disease. We were not able to examine her father, who had died years before, and she could not convince her brother to come to the hospital for further investigation.

Zerebriforme, asymptomatische Furchungen der Kopfhaut

Zerebriforme, asymptomatische Furchungen der Kopfhaut

Giant Primary Cutis Verticis Gyrata Successfully Treated with Surgical Excision and Tissue Expansion

Giant Primary Cutis Verticis Gyrata Successfully Treated with Surgical Excision and Tissue Expansion

Giant Primary Cutis Verticis Gyrata Successfully Treated with Surgical Excision and Tissue Expansion

Giant Primary Cutis Verticis Gyrata Successfully Treated with Surgical Excision and Tissue Expansion

Congenital Primary Cutis Verticis Gyrata

Congenital Primary Cutis Verticis Gyrata

Essential primary cutis verticis gyrata

OBJECTIVE: To report a rare case of a child with essential primary Cutis verticis gyrata. REPORT: Nine-year-old boy with extensive hypertrophy of scalp skin, with a cerebriform appearance. No underlying neurologic and ophthalmologic disorders were found, and no other cases were described in his family. COMMENTS: The diagnosis of primary Cutis verticis gyrata was established by thickening of the scalp and absence of neurologic and ophthalmologic abnormalities. Differential diagnosis comprises secondary conditions such as: cerebriform intradermal nevus, pachydermoperiostosis, acromegaly, and inflammatory diseases of the scalp. This is the first report of a child with this form of Cutis verticis gyrata.

MRI in essential primary cutis verticis gyrata.

We report MRI findings in four patients with essential primary cutis verticis gyrata (CVG). The patients were all men, aged 49-81 years (mean 65 years). They had no symptoms related to this rare scalp condition, preceding scalp disease or associated systemic disease. Coronal images showed a characteristic corrugated appearance of the thickened scalp at the vertex, diagnostic of CVG. Neuroradiologists should be aware of this condition.

Microcephaly, cutis verticis gyrata of the scalp, retinitis pigmentosa, cataracts, sensorineural deafness, and mental retardation in two brothers

We describe the cases of two brothers with microcephaly, primary cutis verticis gyrata of the scalp, prominent supraorbital ridges, large nose, hypertelorism, exotropia, progressive retinitis pigmentosa, cataracts, sensorineural hearing loss, kyphoscoliosis, and mental retardation. A review of the literature focusing on the major clinical findings suggests that our cases may represent a hitherto unreported new syndrome.

Primary Cutis verticis gyrata or Pachydermia verticis gyrata: A Peculiar Scalp Disorder of Mentally Retarded Adult Males

Primary or idiopathic cutis verticis gyrata (CVG) is often associated with mental retardation or chronic schizophrenia. The aim of the present study was to evaluate the prevalence of primary CVG and its histological aspects in a psychiatric adult population. Eighty-three hospitalized psychiatric adult patients were examined for the primary form of CVG. Forty-nine (44 males and 5 females) out of them were schizophrenic, 16 (14 males and 2 females) were mentally retarded and the remaining 18 were affected by different psychiatric disorders. Three males, 2 mentally retarded and 1 schizophrenic, were found to be affected and the clinical diagnosis was confirmed histologically. The high prevalence of primary CVG in males with mental retardation might be explained by ethnic factors or by the use of an adequate diagnostic methodology, improved by shaving the scalp hair in patients suspected to be affected. The term pachydermia verticis gyrata is suggested for the primary CVG, which is the most common scalp disorder in such a population.

An endocrinological study of patients with primary cutis verticis gyrata.

An endocrinological study of 15 psychiatric patients with primary cutis verticis gyrata (CVG) and 7 control patients was carried out. The investigation of the pituitary-gonadal axis, pituitary-adrenal axis, pituitary-thyroid axis, prolactin and human growth hormone (basal values and circadian biorhythms) did not show any significant difference between the CVG and the control patients. Only levels of free testosterone were significantly lower in patients with CVG than in the control group (p < 0.05), probably reflecting an increased peripheral use of testosterone.

Prevalence of primary cutis verticis gyrata in a psychiatric population: association with chromosomal fragile sites.

The prevalence of cutis verticis gyrata was studied in a psychiatric institutional population of 494 patients, the majority of whom were mentally retarded or had chronic schizophrenia. Twenty-two subjects (21 males and 1 female) were found to have primary cutis verticis gyrata, yielding a prevalence of about 4.5%. The frequency of the scalp disorder was 11.4% among mentally retarded patients and 1.7% in schizophrenic subjects. A cytogenetic study was performed on patients with primary cutis verticis gyrata. In 9 out of 21 subjects there was evidence of chromosomal fragile sites: 5 patients had fragile sites on the X-chromosome, in 2 there was fragility of chromosome 12 and in 2, fragility of chromosome 9. The fragile X-site is the genetic marker of the 'fragile X-syndrome', a sex-linked inherited disorder often associated with mental retardation and other neuropathological findings.

Prevalence of primary cutis verticis gyrata in a psychiatric population: association with chromosomal fragile sites.

The prevalence of cutis verticis gyrata was studied in a psychiatric institutional population of 494 patients, the majority of whom were mentally retarded or had chronic schizophrenia. Twenty-two subjects (21 males and 1 female) were found to have primary cutis verticis gyrata, yielding a prevalence of about 4.5%. The frequency of the scalp disorder was 11.4% among mentally retarded patients and 1.7% in schizophrenic subjects. A cytogenetic study was performed on patients with primary cutis verticis gyrata. In 9 out of 21 subjects there was evidence of chromosomal fragile sites: 5 patients had fragile sites on the X-chromosome, in 2 there was fragility of chromosome 12 and in 2, fragility of chromosome 9. The fragile X-site is the genetic marker of the 'fragile X-syndrome', a sex-linked inherited disorder often associated with mental retardation and other neuropathological findings.