Cutis verticis gyrata is a medical condition usually associated with thickening of the

scalp The scalp is the area of the head where head hair grows. It is made up of skin, layers of connective and fibrous tissues, and the membrane of the skull. Anatomically, the scalp is part of the epicranium, a collection of structures covering th ...

. The condition is identified by excessive thickening of the soft tissues of the scalp and characterized by ridges and furrows, which give the scalp a cerebriform appearance. Clinically, the ridges are hard and cannot be flattened on applying pressure. Patients show visible folds, ridges or creases on the surface of the top of the scalp. The number of folds can vary from two to roughly ten and they are typically soft and spongy. The condition typically affects the central and rear regions of the scalp, but sometimes can involve the entire scalp. Hair loss can occur over time where the scalp thickens, though hair within any furrows remains normal. Thus far, due to the (apparent) rarity of the condition, limited research exists and causes are as yet undetermined. What is known, is that the condition is not exclusively

congenital A birth defect is an abnormal condition that is present at childbirth, birth, regardless of its cause. Birth defects may result in disability, disabilities that may be physical disability, physical, intellectual disability, intellectual, or dev ...

. The condition was first reported by

Jean-Louis-Marc Alibert Jean-Louis Marie Alibert (2 May 1768 – 4 November 1837) was a French dermatologist born in Villefranche-de-Rouergue, Aveyron. He was a pioneer of dermatology. Life and work Originally planning to enter the priesthood, Alibert did not begin ...

in 1837, who called it ''cutis sulcata''. A clinical description of the condition was provided by Robert in 1843 and it was named by

Paul Gerson Unna Paul Gerson Unna, (September 8, 1850, Hamburg – January 29, 1929, Hamburg) was a German physician specialized in dermatologist, dermatology and one of the pioneers in dermatopathology. Biography Paul Unna was born to German Jewish parents Mor ...

in 1907. It has also been called ''Robert-Unna syndrome'', ''bulldog scalp'', ''corrugated skin'', ''cutis verticis plicata'', and ''pachydermia verticis gyrata''.

Cause

At this time, causes are unknown, but it is believed to not be congenital.

Conditions and syndromes

CVG is a feature of several conditions: * Acromegaloid phenotype with cutis verticis gyrata and corneal leukoma * Beare-Stevenson cutis gyrata syndrome * Cutis verticis gyrata and

intellectual disability Intellectual disability (ID), also known as general learning disability (in the United Kingdom), and formerly mental retardation (in the United States), Rosa's Law, Pub. L. 111-256124 Stat. 2643(2010).Archive is a generalized neurodevelopmental ...

* Cutis verticis gyrata, thyroid aplasia, and intellectual disability *

Hypertrophic osteoarthropathy Hypertrophy is the increase in the volume of an organ or tissue due to the enlargement of its component Cell (biology), cells. It is distinguished from hyperplasia, in which the cells remain approximately the same size but increase in number. Al ...

, primary,

autosomal dominant In genetics, dominance is the phenomenon of one variant (allele) of a gene on a chromosome masking or overriding the Phenotype, effect of a different variant of the same gene on Homologous chromosome, the other copy of the chromosome. The firs ...

NDE1 Nuclear distribution protein nudE homolog 1 is a protein that in humans is encoded by the ''NDE1'' gene. Clinical significance Mutations In biology, a mutation is an alteration in the nucleic acid sequence of the genome of an organism, ...

-related

microhydranencephaly Microhydranencephaly (MHAC) is a severe abnormality of brain development characterized by both microcephaly and hydranencephaly. Signs and symptoms may include severe microcephaly, scalp rugae (a series of ridges), and profound intellectual disabil ...

* Necrotizing encephalomyelopathy, subacute, of Leigh, adult

Diagnosis

The diagnosis of CVG is primarily clinical, based on the physical appearance of the scalp. Dermatological examination reveals the presence of thickened skin folds and furrows that are most commonly found on the vertex and occipital regions of the scalp. In some cases, additional tests may be conducted to rule out underlying conditions, especially for secondary CVG, including hormonal assays, imaging studies, or biopsy to assess the scalp's histological features.

Classifications

CVG is classified according to the presence, or lack of underlying cause. Studies suggest that CVG often occurs in individuals in a secondary form to other ailments. However, the condition can also be present on its own. CVG can be classified into two forms: ‘primary’ (essential and non-essential) and ‘secondary’. The classifications are: :*Primary essential :*Primary non-essential :*Secondary Primary essential CVG is where the cause of the condition in unknown. It has no other associated abnormalities. This occurs mainly in men, with a male:female ratio of between 5:1 and 6:1, and develops during or soon after puberty. Because of the slow progression of the condition, which usually occurs without symptom, it often passes unnoticed in the early stage. Primary non-essential CVG can be associated with neuropsychiatric disorders including cerebral palsy, epilepsy, seizures, and ophthalmologic abnormalities, most commonly cataracts. Secondary CVG occurs as a consequence of a number of diseases or drugs that produce changes in scalp structure. These include:

acromegaly Acromegaly is a disorder that results in excess growth of certain parts of the human body. It is caused by excess growth hormone (GH) after the growth plates have closed. The initial symptom is typically enlargement of the hands and feet. There ...

(excessive

growth hormone Growth hormone (GH) or somatotropin, also known as human growth hormone (hGH or HGH) in its human form, is a peptide hormone that stimulates growth, cell reproduction, and cell regeneration in humans and other animals. It is thus important in ...

levels due to pituitary gland tumours), and theoretically, the use of growth hormone itself or the use of drugs that mimic the effect of growth hormone (such as

GHRP-6 Growth hormone-releasing peptide 6 (GHRP-6) (developmental code name SKF-110679), also known as growth hormone-releasing hexapeptide, is one of several synthetic met-enkephalin analogues that include unnatural D-amino acids, were developed for ...

and

CJC-1295 CJC-1295 DAC, also known as DAC:GRF (short for drug affinity complex:growth hormone-releasing factor), is a synthetic analogue of growth hormone-releasing hormone (GHRH) (also known as ''growth hormone-releasing factor'' (''GRF'')) and a growth ...

). It may also arise in association with melanocytic naevi (moles), birthmarks (including connective tissue naevi, fibromas and naevus lipomatosus), and inflammatory processes (e.g. eczema, psoriasis, Darier disease, folliculitis, impetigo, atopic dermatitis, acne).

Treatment

Options for medical treatment for this condition have been limited to plastic surgery with excision of the folds by means of scalp reduction/surgical resection. Scalp subcision has also been suggested as a treatment. Additional suggestions also include injections of a dermal filler (e.g. ( poly-L-lactic acid)). Two published medical journals appear to show

Hyaluronidase Hyaluronidases are a family of enzymes that catalyse the degradation of hyaluronic acid. Karl Meyer classified these enzymes in 1971, into three distinct groups, a scheme based on the enzyme reaction products. The three main types of hyaluroni ...

injections (Hyaluronidase is an enzyme used to dissolve

hyaluronic acid Hyaluronic acid (; abbreviated HA; conjugate base hyaluronate), also called hyaluronan, is an anionic, nonsulfated glycosaminoglycan distributed widely throughout connective, epithelial, and neural tissues. It is unique among glycosaminog ...

which is a component of our skin that contributes to its thickness) as a possible treatment.

References

Notes Bibliography *

External links

{{Medical resources , DiseasesDB = , ICD10 = , ICD9 = , ICDO = , OMIM = 219300 , OMIM_mult = {{OMIM, 605685, , none {{OMIM, 304200, , none , MedlinePlus = , eMedicineSubj = , eMedicineTopic = oh , MeshID = Genodermatoses Genetic disorders with OMIM but no gene