[Quiz] A man with crooked scalp

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A 50 year old man presented with such scalp changes. What is your spot diagnosis?

Answer

Cutis verticis gyrata (CVG) is a superficial medical condition usually associated with thickening of the scalp.
Sufferers show visible folds, ridges or creases on the surface of the top of the scalp. The number of folds can vary from 2 to roughly 10 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 can sometimes can involve the entire scalp.
Hair loss can occur over time where the scalp thickens, though hair within any furrows remains normal. 

Classification
CVG is classified according to the presence, or lack of underlying cause. 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 5 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), excessive drug use that mimics acromegaly (including the injection of growth hormone itself and drugs that stimulate growth hormone output, such as GHRP-6 and CJC-1295), 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
There is no cure for this condition and currently, medical treatment is limited to plastic surgery with excision of the folds by means of scalp reduction and more recently scalp subcision.



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