Cutis verticis gyrata (CVG) is a rare scalp condition where the skin forms deep folds and grooves, resembling the brain's surface. It primarily affects men. Treatment focuses on managing underlying causes and may include surgery for cosmetic or health reasons. Learn more about this rare skin condition.
Cutis verticis gyrata
Image by George M. Gould and Walter L. Pyle/Wikimedia
Summary
Cutis verticis gyrata
Image by G. E. Shuttleworth and W. A. Potts/Wikimedia
Cutis verticis gyrata
Congenital Imbecile with Convoluted Scalp.
Image by G. E. Shuttleworth and W. A. Potts/Wikimedia
What Is Cutis Verticis Gyrata?
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.
Source: Genetic and Rare Diseases (GARD) Information Center
Additional Materials (3)
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An extreme case of Cutis Verticis Gyrata
Le cutis verticis gyrata (CVG), ou pachydermie vorticellée du cuir chevelu, est une affection rare et évolutive de la peau du scalp et/ou du visage. Elle se caractérise par une hypertrophie et une hyperlaxité cutanée formant des plis semblables à la surface du cortex cérébral . Ces plis peuvent être à l'origine d'une gêne esthétique, sociale et fonctionnelle (macération, infection) [Cette pathologie était rapportée pour la première fois par Alibert en 1837 sous le terme de « cutis sulcata » Robert était le premier ayant donné la description clinique de cette pathologie en 1843 et en 1907, Unna lui donnait le nom de CVG, communément admis depuis . Il existe trois formes décrites de CVG: le CVG secondaire à des pathologies chroniques métaboliques, inflammatoires [3], respiratoires, cardiaques, endocriniennes épatobiliaires et formes paranéoplasiques ], ou encore iatrogène suite à un traitement par Minoxidil ]. Le CVG est également présent dans la pachydermoperiostose (ou ostéo-arthrite hypertrophique chronique) ], les lésions hamartomateuses, l'acromégalie, la syphilis, la leucémie, la neurofibromatose, le syndrome d'Ehlers- Danlos . Une traction chronique du scalp a même été décrite à l'origine d'un CVG . Cette forme est la plus fréquente et touche plus fréquemment le visage en association avec l'atteinte du cuir chevelu. Elle intéresse surtout les hommes avec un sex-ratio de 5/1 ; le CVG primitif, beaucoup plus rare, dont l'origine est génétique à transmission indéterminée [3]. Il peut être essentiel ou non-essentiel: a forme non-essentielle est présente chez 0,5% des patients ayant des anomalies neurologiques et/ou ophtalmiques (retard mental, épilepsie, microcéphalie, encéphalopathies, cataracte congénitale) la forme primitive essentielle est une forme extrêmement rare, seuls 15 cas de CVG primitif essentiel traités chirurgicalement sont rapportés dans la littérature. Elle touche surtout le sujet masculin et débute à la fin de l'adolescence. Cette forme n'est pas associée à des anomalies neurologiques ou ophtalmiques. Le type de transmission génétique est incertain
Image by Mohammad2018/Wikimedia
Turricephalie Atypique 2
Fig. 2. - De profil apparaisant tres nettement la surelevation du bregma et son report en avant.
Image by P. Michon et J. Harmand/Wikimedia
Cutis verticis gyrata
Abnormal development of scalp
Image by George M. Gould and Walter L. Pyle/Wikimedia
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This media may include sensitive content
An extreme case of Cutis Verticis Gyrata
Mohammad2018/Wikimedia
Turricephalie Atypique 2
P. Michon et J. Harmand/Wikimedia
Cutis verticis gyrata
George M. Gould and Walter L. Pyle/Wikimedia
Causes
Unknown
Image by GDJ/Pixabay
Unknown
Image by GDJ/Pixabay
What Causes Cutis Verticis Gyrata?
The underlying cause of cutis verticis gyrate (CVG) is not well understood. The condition may be divided into 3 forms, based on whether it is isolated or it's association with specific underlying conditions:
Primary essential - refers to cases in which no other abnormality is present. In this form the cause is not known. While most of these cases appear to be sporadic, autosomal recessive and autosomal dominant inheritance have been described.
Primary nonessential - can be associated with intellectual disability, cerebral palsy, epilepsy (seizures), schizophrenia, cranial abnormalities (microcephaly), deafness, eye or vision abnormalities, or a combination of these. In this form, the cause may have an endocrinologic basis (caused by abnormalities of the endocrine system).
Secondary - associated with a wide variety of underlying diseases and treatments. In these cases, the underlying cause depends on the associated disease or treatment.
Source: Genetic and Rare Diseases (GARD) Information Center
Statistics
Rare Genetic Diseases
Image by National Human Genome Research Institute (NHGRI)
Rare Genetic Diseases
Genomics is ending diagnostic odysseys for patients with rare diseases.
Image by National Human Genome Research Institute (NHGRI)
How Common Is Cutis Verticis Gyrata?
The estimated prevalence of cutis verticis gyrata (with data from 1964) is up to 1 in 100,000 people in the general population. It has been estimated to occur in 0.5% (1 in 200) of people with intellectual disability in the United States.
Source: Genetic and Rare Diseases (GARD) Information Center
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Cutis Verticis Gyrata
Cutis verticis gyrata (CVG) is a rare scalp condition where the skin forms deep folds and grooves, resembling the brain's surface. It primarily affects men. Treatment focuses on managing underlying causes and may include surgery for cosmetic or health reasons. Learn more about this rare skin condition.