What is cutis verticis gyrata

Cutis verticis gyrata (CVG) refers to a scalp condition with convoluted folds and deep furrows look similar to the folds of the brain ¹. Cutis verticis gyrata is a progressive condition. It is mainly troublesome due to the cosmetic appearance. Rarely, it can be complicated by malignant melanoma developing within a melanocytic nevus ². Primary cutis verticis gyrata occurs more commonly in males, and most commonly develops after puberty, but before age 30. The primary form of cutis verticis gyrata has a reported male-to-female ratio of 5:1. The incidence of cutis verticis gyrata may be reported as lower in women because longer hair may camouflage the condition. The estimated prevalence of cutis verticis gyrata is 1 in 100,000 in males 0.026 in 100,000 in females ³. Cutis verticis gyrata may occur alone where it is called isolated cutis verticis gyrata or in association with a variety of underlying conditions or treatments, including neuropsychiatric disorders, eye abnormalities, or inflammatory conditions ². Some secondary forms of cutis verticis gyrata, like cerebriform intradermal nevus, can be present at birth. 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 ¹.

Figure 1. Cutis verticis gyrata

Classification of cutis verticis gyrata

Cutis verticis gyrata is classified according to the underlying cause as primary essential, primary nonessential and secondary.

Primary means the cause of the condition is unknown. Primary essential cutis verticis gyrata has no other associated abnormalities. Primary nonessential cutis verticis gyrata can be associated with neuropsychiatric disorders including cerebral palsy, epilepsy, seizures and ophthalmologic abnormalities, most commonly cataracts.

Secondary cutis verticis gyrata occurs as a consequence of a number of diseases that produce changes in scalp structure. These conditions include:

• Acromegaly (excessive growth hormone, due to pituitary gland tumour)
• Melanocytic nevi (moles)
• Birthmarks, including Connective tissue nevi, fibromas and nevus lipomatosus
• Inflammatory processes (eczema, psoriasis, Darier disease, folliculitis, impetigo, atopic dermatitis, acne).

Cutis verticis gyrata causes

The cause of cutis verticis gyrata is unknown in primary cases, although genetic and endocrinologic factors are suspected to participate in the etiology. Systemic diseases, inflammatory dermatoses, underlying nevoid abnormalities, and trauma are most common in secondary cases. Cutis verticis gyrata‒like lesions have been described in melanoma patients undergoing treatment with vemurafenib and whole-brain radiotherapy, but not with each of the treatments alone ⁴.

Secondary causes of cutis verticis gyrata are associated with the following underlying diseases and treatments:

• Acanthosis nigricans
• Acromegaly ⁵
• Amyloidosis ⁶
• Autosomal dominant insulin-resistant syndrome ⁷
• Beare-Stevenson syndrome
• Connective tissue nevus
• Cretinism
• Cutaneous leiomyomatosis ⁸
• Cylindroma
• Dermatofibroma
• Diabetes mellitus
• Ehlers-Danlos syndrome
• Fallopian tube carcinoma
• Fibroma
• Focal mucinosis
• Graves disease ⁹
• Hereditary neuralgic amyotrophy ¹⁰
• HIV-related lipodystrophy ¹¹
• Hyperimmunoglobulin E syndrome ¹²
• Inflammatory processes (eg, eczema, psoriasis, Darier disease, folliculitis, impetigo, erysipelas, atopic dermatitis, acne conglobata) ¹³
• Intracranial aneurysm ¹⁴
• Intraventricular ependymoma ¹⁴
• Leukemia ¹⁵
• Melanocytic nevi or hamartomas (cerebriform intradermal nevus, giant cellular blue naevus, cutaneous neurocristic hamartoma) ¹⁶
• Myxedema ⁹
• Neurofibroma ¹⁷
• Nevus lipomatosus
• Noonan syndrome ¹⁸
• Pachydermoperiostosis ¹⁹
• Posttraumatic (eg, traction alopecia) ²⁰
• Pseudoacromegaly ²¹
• Scleromyxedema without monoclonal gammopathy ²²
• Supernumerary X chromosome syndromes (including Klinefelter syndrome) ²³
• Syphilis
• Systemic T-cell lymphoma ²⁴
• Tuberous sclerosis
• Turner syndrome
• Vemurafenib and whole-brain radiotherapy combination treatment in melanoma patients ²⁵

Cutis verticis gyrata symptoms

Cutis verticis gyrata typically affects the central and back of the scalp, but some forms can involve the entire scalp. The folds are typically soft and spongy. The folds are unable to be corrected with pressure. The skin color is not affected. The number of folds can vary from 2 to more than 10.

Cutis verticis gyrata treatment

The management of cutis verticis gyrata includes good scalp hygiene to avoid accumulations of secretions in the furrows of the scalp. Definitive treatment by surgery may be requested for appearance reasons. Small localized lesions can be excised in one procedure. Larger lesions can be removed with serial excision, requiring multiple procedures.

In cases of cerebriform intradermal nevus, early diagnosis, wide surgical excision, and plastic reconstruction should be always considered ²⁶.

Cutis verticis gyrata prognosis

The prognosis of primary cutis verticis gyrata is good, although the condition does not regress without surgical intervention and progression of cutis verticis gyrata may be observed. In secondary cases, the prognosis depends on the underlying process ²⁷.

Cutis verticis gyrata is long lasting and progressive. It is often found to be unacceptable because of cosmetic reasons. It can be complicated by malignant melanoma developing within a congenital melanocytic nevus ²⁶.

References

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