Cutis verticis gyrata as an incidental finding: Revisiting a rare dermatological entity

INTRODUCTION

Cutis verticis gyrata (CVG) is a rare skin disease, affecting 1 of 100,000 males and 0.026 of 100,000 females in the general population.¹ CVG can be classified into two broad categories- Primary and Secondary. Primary CVG is further classified as Primary essential CVG and Primary non-essential. CVG is mostly asymptomatic and a cosmetic problem. The treatment, if requested, is surgical.² We report 2 cases of primary essential CVG that were incidentally noticed by the patients themselves after a tonsure.

CASE REPORT

CASE 1

A young man in his early twenties presented with complaints of a thickened scalp that was noticed following a tonsure. Physical examination revealed a thickened scalp with multiple folds arranged in a symmetric manner, anteroposteriorly on the vertex. The scalp was otherwise normal [Figure 1]. The systemic examination did not reveal any neurologic, ophthalmologic, or metabolic abnormalities. Patient did not give a history of taking any anabolic steroids or any topical application on the scalp. Laboratory investigations were within normal range. Thus, the diagnosis of Cutis Verticis Gyrata, primary (essential) type was made.

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Figure 1: Symmetric skin folds running in anteroposterior direction over the scalp

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CASE 2

A middle-aged man, a known case of generalized plaque-type psoriasis on treatment since January 2024, presented with multiple skin folds on the scalp. The patient became aware of the skin folds following a tonsure. The psoriasis is under control with oral methotrexate. The scalp folds were seen on the vertex of the scalp, in a symmetric pattern, and running in an anteroposterior direction. A few psoriatic plaques were present on the occipital scalp [Figure 2]. No other neurologic, ophthalmic, or metabolic abnormalities were found on physical exam. Laboratory investigations were within normal range. Since the folds were symmetric in distribution and did not involve psoriatic patches along with a short duration of psoriasis (~ 1 year) before CVG was first identified, a diagnosis of primary cutis verticis gyrata with psoriasis was made.

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Figure 2: Plaques of psoriasis sparing the skin folds of cutis verticis gyrata.

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DISCUSSION

CVG is a rare benign condition first described by Alibert in 1837 and characterized by proliferation and hypertrophy of the skin of the scalp or forehead. The affected skin then exhibits folds and furrows similar to the cerebral cortex convolutions. Unna introduced the term cutis verticis gyrata in 1907.³ The entity is classified into primary and secondary subtypes. Primary (essential) is the rarest form of CVG, characterized by an isolated cutaneous involvement without any associated pathology. It usually begins post-puberty and is more common in males than females. The primary (non-essential) has been described in association with several neuropsychiatric disorders like epilepsy, intellectual disability, microcephaly, schizophrenia, ophthalmologic disorders like congenital cataract, strabismus, blindness, etc. Secondary CVG is slightly more common than the primary forms and is associated with multiple underlying conditions like eczema, psoriasis, impetigo, erysipelas, pemphigus, Dariers disease, pachydermoperiostosis, acne conglobata, cutaneous focal mucinosis, dermatofibroma, collagenoma, neurofibroma, cerebriform intradermal nevus, fibromas, cylindromas, nevus lipomatosus,connective tissue nevi;Malignancies like pituitary tumours, leukemia-cell lymphoma;Systemic disorders like acromegaly, tuberous sclerosis, amyloidosis, myxoedema, acanthosis nigricans, diabetes mellitus; Syndromes like Noonan syndrome, Beare-Stevenson syndrome, Ehlers-Danlos Syndrome, “Michelin tire baby” syndrome, Apert syndrome, Turner syndrome, Fragile X syndrome; Infections like syphilis and HIV AIDS and medications like anabolic steroids and minoxidil.⁴

In the primary form, as the disease is usually seen in postpubertal individuals an endocrine etiology has been postulated. Trophic hormones such as growth hormone (GH) and insulin-like growth factor 1 (IGF1) are thought to be causative for skin modifications like collagen thickening and sebaceous gland hypertrophy. The exact pathogenesis behind secondary CVG is unknown but it is hypothesized to occur as a consequence of several diseases or drugs that produce structural changes in scalp. These include acromegaly, the use of growth hormone or the use of drugs that mimic the effect of growth hormone (such as GHRP-6 and CJC-1295). It is also observed to occur in association with melanocytic nevi, connective tissue nevi, fibromas, and nevus lipomatosus and inflammatory processes (e.g., eczema, psoriasis, Darier disease, folliculitis, impetigo, atopic dermatitis, and acne).⁵ All the reported cases of secondary CVG due to scalp psoriasis have consistently shown the psoriatic plaques to be present over scalp folds of cutis verticis gyrata, and the presence of scalp psoriasis for multiple years before changes of CVG appeared.

Although the pathophysiology of CVG remains unclear, on a structural level, it is suggested that the skin folds are secondary to connective tissue septa formation between the skin and the galea. These septa prevent the expansion of the skin, resulting in skin folds.⁶

In primary essential CVG, histopathology shows a normal epidermis. Hyperplasia of pilosebaceous units with or without increase in collagen bundles can be seen in the dermis. Radiological investigations like X-ray, computed tomography, and magnetic resonance imaging are used to rule out secondary causes of cutis verticis gyrata, like intracerebral aneurysms, pachydermoperiostosis, intracranial tumors, and scalp tumors.⁴ The key differences between primary and secondary types of CVG have been listed in [Table 1].⁴

No medical treatments have been proven to be effective in CVG. All cases of CVG should be advised to practice maintenance of local hygiene to prevent skin maceration and secondary infection. Surgery is indicated only for cosmetic appeal, psychological concerns, and in case of any complications.

CONCLUSION

These cases highlight the importance of thorough scalp examination, as CVG may be overlooked when asymptomatic. Early recognition is essential for appropriate classification, exclusion of underlying disorders, and patient reassurance. Dermatologists should maintain a high index of suspicion, especially in patients with subtle scalp contour abnormalities.