Cutis verticis gyrata
|Cutis verticis gyratann|
Cutis verticis gyrata is a medical condition usually associated with thickening of the scalp. People 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 are typically soft and spongy. These folds cannot be corrected with pressure. 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.
The condition was first reported by Jean-Louis-Marc Alibert in 1837, who called it cutis sulcata. A clinical description of the condition was provided by Robert[who?] in 1843 and it was named by Paul Gerson Unna in 1907. It has also been called Robert-Unna syndrome, bulldog scalp, corrugated skin, cutis verticis plicata, and pachydermia verticis gyrata.
At this time, causes are unknown, but it is believed to not be congenital.
There is no clinical diagnosis for CVG as cases are rarely seen and are often comorbid with other conditions.
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
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 5:1 or 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 (excessive growth hormone 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 and CJC-1295). 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).
The only current medical treatment for this condition is 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)).
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